WorldWideScience

Sample records for congenital tracheal agenesis

  1. CT of tracheal agenesis

    International Nuclear Information System (INIS)

    Strouse, Peter J.; Hernandez, Ramiro J.; Newman, Beverley

    2006-01-01

    Tracheal agenesis is a rare and usually lethal anomaly. In the past, opaque contrast medium was injected via the esophagus to demonstrate the anatomy. To demonstrate the utility of helical and multidetector CT in delineating the aberrant anatomy in newborns with tracheal agenesis. Four newborns with tracheal agenesis were identified from three institutions. Imaging studies and medical records were reviewed. Each child was imaged with chest radiography. One child was imaged on a single-detector helical CT scanner and the other three on multidetector scanners. Helical and multidetector CT with 2D and 3D reconstructions clearly delineated the aberrant tracheobronchial and esophageal anatomy in each infant. Minimum intensity projection reformatted CT images were particularly helpful. One infant each had type I and type II tracheal agenesis. Two infants had type III tracheal agenesis. All four infants died. CT is a useful tool for delineating the aberrant anatomy of newborns with tracheal agenesis and thus helps in making rational clinical decisions. (orig.)

  2. Securing the airway in a child with tracheal agenesis – an alternative perspective

    Directory of Open Access Journals (Sweden)

    Slavomir Nosal

    2011-03-01

    Full Text Available Tracheal agenesis is an extremely rare, typically fatal congenital anomaly characterised by typical clinical manifestation, failed endotracheal intubation and difficult emergency management. The prognosis and possibility of surgical correction rest on early diagnosis, anatomy, birth weight, and associated anomalies. Tracheal agenesis causes considerable resuscitation difficulties immediately after birth, as tracheal intubation is impossible. Although emergency management, by either bag and mask ventilation or oesophageal intubation, provides some pulmonary gas exchange through tracheo-oesophageal connection, they are associated with overdistension of the stomach and the risk of stomach perforation. We have performed selective intubation of the distal trachea in a child with tracheal agenesis through the tracheo-oesophageal connection with the help of a fibroendoscope. A standard guide wire was passed through the working port of the flexible endoscope and under direct vision was directed through the tracheo-oesophageal connection into the distal trachea. At this point, the endoscope was removed, leaving the guide wire in place. The endotracheal tube size 2 Fr was passed over the guide wire into the distal trachea. The infant showed dramatic improvement and maintained good saturations on mechanical ventilation. The infant was taken for a computed tomography (CT scan, which showed Floyd’s type II tracheal agenesis, the oesophagus connecting with the distal trachea before it bifurcates. The endotracheal tube was seen ending in the carina. Transoesophageal intubation of the short distal segment of the trachea through the tracheo-oesophageal connection might offer a new perspective for short-term securing of the airway in a child with tracheal agenesis and should be considered in any child with suspected tracheal agenesis to buy the time while evaluating the exact anatomy of the anomaly and the possibility of tracheal reconstruction in selected

  3. Congenital lumbar vertebrae agenesis in a lamb.

    Science.gov (United States)

    Farajli Abbasi, Mohammad; Shojaei, Bahador; Azari, Omid

    2017-01-01

    Congenital agenesis of lumbar vertebrae was diagnosed in a day-old female lamb based on radiology and clinical examinations. There was no neurological deficit in hindlimb and forelimb associated with standing disability. Radiography of the abdominal region revealed absence of lumbar vertebrae. Necropsy confirmed clinical and radiographic results. No other anomaly or agenesis was seen macroscopically in the abdominal and thoracic regions as well as vertebral column. Partial absence of vertebral column has been reported in human and different animal species, as an independent occurrence or associated with other organs anomalies. The latter has been designated as caudal regression syndrome. Vertebral agenesis may arise from irregularity in the differentiation of somites to the sclerotome or sclerotome to the vertebral primordium. Most of the previously reported cases of agenesis were related to the lumbosacral region, lonely or along with other visceral absences. This case was the first report of congenital agenesis of lumbar vertebrae in a lamb.

  4. Congenital Complete Tracheal Ring in a Neonate: A Case Report

    Directory of Open Access Journals (Sweden)

    Esra ARUN ÖZER

    2017-09-01

    Full Text Available Abnormalities of the upper airway tract lead to congenital high airway obstruction and may complicate neonatal airway management in the delivery room. Congenital complete tracheal rings are a rare and unusual tracheal anomaly, usually presenting in the neonate or infant as respiratory distress. The clinical presentation can vary from almost asymptomatic patients to near-fatal airway obstruction. It may exist as an isolated entity, or in association with other congenital malformations, in particular, cardiac anomalies along with vascular rings and pulmonary slings. Other associated anomalies have also been reported, for example, chromosomal anomalies, malformation of other parts of the respiratory tract, esophagus and skeletal systems. Here, we report an extreme case of VACTERL/TACRD association presented with congenital complete tracheal ring, encephalocele, bilateral radial agenesis with absent thumbs, equinovalgus deformity on right foot, low-set ears and micrognathia.

  5. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

    International Nuclear Information System (INIS)

    Moon, W.-J.; Porto, L.; Lanfermann, H.; Zanella, F.E.; Weis, R.

    2002-01-01

    We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

  6. Tracheal quadrifurcation associated with congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Bhat, Venkatraman; Gadabanahalli, Karthik; Ahmad, Ozaire [Narayana Multispeciality Hospital and Mazumdar Shaw Cancer Center, Department of Radiology, Bangalore (India)

    2015-08-15

    Tracheal anomalies are known in association with congenital cardiac defects. Some of the well-described anomalies include accessory (displaced) tracheal bronchus with variants, tracheal trifurcation and accessory cardiac bronchus. Here we describe a case of tracheal quadrifurcation associated with complex congenital heart disease. Illustration of complex airway anatomy was simplified by the use of multidetector CT using a variety of image display options. Awareness of this complex anomaly will expand our knowledge of tracheal anomalies and equip the anesthesia and surgical team for better airway management. (orig.)

  7. Isolated bilateral congenital lacrimal gland agenesis – Report of two cases

    Directory of Open Access Journals (Sweden)

    Manar Alwohaib

    2017-10-01

    Full Text Available Congenital lacrimal gland agenesis, also called congenital alacrima, is a rare cause of dry eye and is characterized by aplasia or hypoplasia of lacrimal glands. We present two 5-year old children with congenital lacrimal gland agenesis. The two cases had the final diagnosis of isolated bilateral congenital lacrimal gland agenesis and we document the clinical aspects, treatment and present a literature review related to this rare condition. Keywords: Alacrima, Lacrimal gland, Lacrimal gland agenesis, Punctal plugs

  8. Congenital tracheal defects: embryonic development and animal models

    Directory of Open Access Journals (Sweden)

    Zenab Arooj Sher

    2016-03-01

    Full Text Available Tracheal anomalies are potentially catastrophic congenital defects. As a newborn begins to breathe, the trachea needs to maintain an appropriate balance of elasticity and rigidity. If the tracheal cartilages are disorganized or structurally weak, the airways can collapse, obstructing breathing. Cartilage rings that are too small or too rigid can also obstruct breathing. These anomalies are frequently associated with craniofacial syndromes, and, despite the importance, are poorly understood. In this review, we summarize the spectrum of pathological phenotypes of the trachea and correlate them with the molecular events uncovered in mouse models.

  9. Laparoscopic Colectomy for a Patient with Congenital Renal Agenesis

    African Journals Online (AJOL)

    We present a very rare case of laparoscopic colectomy for a patient with ascending colon cancer and an agenetic right kidney. A 57‑year‑old man visited our institute for further evaluation for a positive fecal occult blood test. Approximately, 20 years earlier, the right kidney of the patient was found to be congenitally absent.

  10. Laparoscopic Colectomy for a Patient with Congenital Renal Agenesis

    African Journals Online (AJOL)

    2013-10-07

    the tumor could not be elevated by injection of saline into ... on Monday, October 07, 2013, IP: 41.135.175.93] || Click here to download free Android application for this journal .... the ureter facilitate the safe treatment of patients with congenital ...

  11. Congenital tricuspid valve disease and testicular agenesis: a case ...

    African Journals Online (AJOL)

    This is a report of a case of congenital tricuspid valve disease presenting with heart failure and pulmonary hypertension. Cardinal clinical features include breathlessness, easy fatigability since childhood, stunted growth, cyanosis, finger clubbing, a pansystolic murmur loudest at the left sternal edge in the fourth intercostal ...

  12. Congenital scoliosis associated with agenesis of the uterine cervix. Case report

    Directory of Open Access Journals (Sweden)

    Monica Matteo

    2004-06-01

    Full Text Available Abstract Background Alterations in the normal sequence of development of müllerian ducts lead to a wide spectrum of reproductive tract abnormalities. A rare form of lack of development, regarding a short tract of the müllerian ducts, leads to the isolated agenesis of the uterine cervix. Anomalies identified among patients with müllerian agenesis include skeletal deformities (i.e., scoliosis of the spine and Klippel-Feil anomaly. Case presentation A 46 years old woman presenting cyphoscoliosis and very low stature (120 cm – 3,93 feet, came to our observation for acute pelvic pain; she also reported primary amenorrhoea associated with cyclic pelvic pain. Clinical and imaging evaluation, evidenced a blind vaginal duct of normal length, left cystic adnexal mass, and enlarged uterus with hematometra. FSH, LH, 17β estradiol and CA-125, karyotype and radiographic study of limbs and vertebral column were also evaluated. At laparotomy, a left ovarian cyst was found. Uterus ended at the isthmus; under this level a thin fibrous tissue band was found, joining the uterus to the vagina. Uterine cervix was replaced by fibrous tissue containing some dilated glands lined with müllerian epithelium. Karyotype resulted 46, XX. The described skeletal deformity, were consistent with Klippel-Feil syndrome. Conclusion We report a case of congenital scoliosis associated with müllerian agenesis limited to uterine cervix, association thus far seen only among patients with Mayer-Rokitansky-Kuster-Hauser syndrome (utero-vaginal agenesis. This case report supports the necessity to evaluate, for accompanying müllerian anomalies, all cases of congenital structural scoliosis in view of the possibility for many müllerian development abnormalities, if timely diagnosed, to be surgically corrected.

  13. Pulmonary agenesis

    OpenAIRE

    Oyola, Mercedes; Pontificia Universidad Javeriana; Gordillo, Gisel; Pontificia Universidad Javeriana; García, Carlos A.; Pontificia Universidad Javeriana; Torres, David; Pontificia Universidad Javeriana

    2009-01-01

    Pulmonary agenesis is an infrequent pathology which occurs predominantly among females with no lateral preference. We report on the case of a newborn male diagnosed with prenatal diaphragm hernia though at birth seemed more likely either to be a congenital cystic adenomatoid malformation (congenital pulmonary airway malformation) or pulmonary agenesis. The patient died six days after birth and necropsy confirmed pulmonary agenesis. La agenesia pulmonar es una alteración poco frecuente, con...

  14. Fetoscopic tracheal occlusion for severe congenital diaphragmatic hernia: retrospective study

    Directory of Open Access Journals (Sweden)

    Angélica de Fátima de Assunção Braga

    Full Text Available Abstract Background and objectives: The temporary fetal tracheal occlusion performed by fetoscopy accelerates lung development and reduces neonatal mortality. The aim of this paper is to present an anesthetic experience in pregnant women, whose fetuses have diaphragmatic hernia, undergoing fetoscopic tracheal occlusion (FETO. Method: Retrospective, descriptive study, approved by the Institutional Ethics Committee. Data were obtained from medical and anesthetic records. Results: FETO was performed in 28 pregnant women. Demographic characteristics: age 29.8 ± 6.5; weight 68.64 ± 12.26; ASA I and II. Obstetric: IG 26.1 ± 1.10 weeks (in FETO; 32.86 ± 1.58 (reversal of occlusion; 34.96 ± 2.78 (delivery. Delivery: cesarean section, vaginal delivery. Fetal data: Weight (g in the occlusion and delivery times, respectively (1045.82 ± 222.2 and 2294 ± 553; RPC in FETO and reversal of occlusion: 0.7 ± 0.15 and 1.32 ± 0.34, respectively. Preoperative maternal anesthesia included ranitidine and metoclopramide, nifedipine (VO and indomethacin (rectal. Preanesthetic medication with midazolam IV. Anesthetic techniques: combination of 0.5% hyperbaric bupivacaine (5-10 mg and sufentanil; continuous epidural predominantly with 0.5% bupivacaine associated with sufentanil, fentanyl, or morphine; general. In 8 cases, there was need to complement via catheter, with 5 submitted to PC and 3 to BC. Thirteen patients required intraoperative sedation; ephedrine was used in 15 patients. Fetal anesthesia: fentanyl 10-20 mg.kg-1 and pancuronium 0.1-0.2 mg.kg-1 (IM. Neonatal survival rate was 60.7%. Conclusion: FETO is a minimally invasive technique for severe congenital diaphragmatic hernia repair. Combined blockade associated with sedation and fetal anesthesia proved safe and effective for tracheal occlusion.

  15. Association of two respiratory congenital anomalies: tracheal diverticulum and cystic adenomatoid malformation of the lung

    International Nuclear Information System (INIS)

    Restrepo, S.; Villamil, M.A.; Rojas, I.C.; Lemos, D.F.; Echeverri, S.; Angarita, M.; Triana, G.

    2004-01-01

    Many associations of congenital anomalies of the respiratory system have been reported, but the combination of tracheal diverticulum and cystic adenomatoid malformation (CCAM) is unique. We present a patient with these two anomalies and analyze their embryological correlation. (orig.)

  16. Polyflex stenting of tracheomalacia after surgery for congenital tracheal stenosis.

    Science.gov (United States)

    Tibballs, James; Fasulakis, Stephen; Robertson, Colin F; Berkowitz, Robert G; Massie, John; Brizard, Christian; Rose, Elizabeth; Bekhit, Elhamy; Eyres, Robert; Ragg, Philip

    2007-01-01

    Polyflex self-expanding stents (Rüsch, Germany) were used in three young children who had presented with life-threatening long-segment tracheal stenosis with bronchial stenosis in two cases. Two children had slide tracheoplasties and subsequently aortic homografts and another tracheal resection and autotracheoplasty. However, in all cases persistent lower tracheal malacia necessitated stenting. Complications of granuloma, stent migration or dislodgement occurred in all cases. A fatal tracheo-aortic fistula occurred in one child. Granuloma in one was treated successfully with steroids. One child survives.

  17. Prematurity and fetal lung response after tracheal occlusion in fetuses with severe congenital diaphragmatic hernia.

    Science.gov (United States)

    Sananes, Nicolas; Rodo, Carlota; Peiro, Jose Luis; Britto, Ingrid Schwach Werneck; Sangi-Haghpeykar, Haleh; Favre, Romain; Joal, Arnaud; Gaudineau, Adrien; Silva, Marcos Marques da; Tannuri, Uenis; Zugaib, Marcelo; Carreras, Elena; Ruano, Rodrigo

    2016-09-01

    To evaluate the independent association of fetal pulmonary response and prematurity to postnatal outcomes after fetal tracheal occlusion for congenital diaphragmatic hernia. Fetal pulmonary response, prematurity (prematurity (prematurity was not statistically associated with mortality after controlling for fetal pulmonary response (aOR 0.52, 95% CI 0.12-2.30, p=0.367). Fetal pulmonary response after FETO is the most important factor associated with survival, independently from the gestational age at delivery.

  18. Lumbosacral agenesis in a cat

    Directory of Open Access Journals (Sweden)

    Gabrielle C Hybki

    2016-01-01

    Full Text Available Case summary Lumbosacral agenesis is a rare congenital condition reported in children. We report a 17-week-old female domestic shorthair cat with lumbosacral agenesis on whole-body radiographs. The cat was euthanized shortly thereafter presentation. A necropsy was not permitted. Relevance and novel information This is the first reported feline case of lumbosacral agenesis.

  19. Learning From Experience: Improving Early Tracheal Extubation Success After Congenital Cardiac Surgery.

    Science.gov (United States)

    Winch, Peter D; Staudt, Anna M; Sebastian, Roby; Corridore, Marco; Tumin, Dmitry; Simsic, Janet; Galantowicz, Mark; Naguib, Aymen; Tobias, Joseph D

    2016-07-01

    The many advantages of early tracheal extubation following congenital cardiac surgery in young infants and children are now widely recognized. Benefits include avoiding the morbidity associated with prolonged intubation and the consequences of sedation and positive pressure ventilation in the setting of altered cardiopulmonary physiology. Our practice of tracheal extubation of young infants in the operating room following cardiac surgery has evolved and new challenges in the arena of postoperative sedation and pain management have appeared. Review our institutional outcomes associated with early tracheal extubation following congenital cardiac surgery. Inclusion criteria included all children less than 1 year old who underwent congenital cardiac surgery between October 1, 2010, and October 24, 2013. A total of 416 patients less than 1 year old were included. Of the 416 patients, 234 underwent tracheal extubation in the operating room (56%) with 25 requiring reintubation (10.7%), either immediately or following admission to the cardiothoracic ICU. Of the 25 patients extubated in the operating room who required reintubation, 22 failed within 24 hours of cardiothoracic ICU admission; 10 failures were directly related to narcotic doses that resulted in respiratory depression. As a result of this review, we have instituted changes in our cardiothoracic ICU postoperative care plans. We have developed a neonatal delirium score, and have adopted the "Kangaroo Care" approach that was first popularized in neonatal ICUs. This provision allows for the early parental holding of infants following admission to the cardiothoracic ICU and allows for appropriately selected parents to sleep in the same beds alongside their postoperative children.

  20. Two cases of sacral agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Choi, J Y; Bae, Y K; Hahm, C K; Kang, S R [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1979-06-15

    Sacral agenesis is a central component in the spectrum of anomalies comprising the caudal regression syndrome. Sacral agenesis occurs more frequently in an infant born to mother of diabetes, rubella infection in first trimester than normal mothers. In a patient of sacral agenesis, it is important to recognize the neurologic deficit, neurogenic bladder dysfunction and other congenital anomalies. A case of partial sacral agenesis of 10 years old girl and another case of complete total agenesis associated with multiple anomalies in autopsied newborn are reported.

  1. Two cases of sacral agenesis

    International Nuclear Information System (INIS)

    Choi, J. Y.; Bae, Y. K.; Hahm, C. K.; Kang, S. R.

    1979-01-01

    Sacral agenesis is a central component in the spectrum of anomalies comprising the caudal regression syndrome. Sacral agenesis occurs more frequently in an infant born to mother of diabetes, rubella infection in first trimester than normal mothers. In a patient of sacral agenesis, it is important to recognize the neurologic deficit, neurogenic bladder dysfunction and other congenital anomalies. A case of partial sacral agenesis of 10 years old girl and another case of complete total agenesis associated with multiple anomalies in autopsied newborn are reported.

  2. Complete dorsal pancreatic agenesis and unilateral renal agenesis.

    Science.gov (United States)

    Moreira, Adriana; Carvalho, André; Portugal, Inês; Jesus, José Miguel

    2018-02-01

    Dorsal pancreatic agenesis is a very rare congenital anomaly. Unilateral renal agenesis, on the other hand, is a relatively common congenital anomaly, although its etiology is not fully understood. Renal and pancreatic embryologic development appears to be nonrelated. We report a case of a 34-year-old man who was referred to our hospital for evaluation of cholestasis and microalbuminuria. Ultrasound and magnetic resonance imaging examinations showed empty right renal fossa and absence of the pancreatic neck, body, and tail. Our case report is the second case of a dorsal pancreatic agenesis and unilateral renal agenesis in a young male patient.

  3. Tracheal compression due to an elongated aortic arch in patients with congenital heart disease: evaluation using multidetector-row CT

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, Noriko; Hayabuchi, Yasunobu; Inoue, Miki; Sakata, Miho; Nabo, Manal Mohamed Helmy; Nakagawa, Ryuji; Saijo, Takahiko; Kagami, Shoji [University of Tokushima, Department of Pediatrics, Tokushima (Japan)

    2009-10-15

    The airway can become obstructed as a result of compression by an elongated aortic arch. In this study we evaluated tracheal compression using multidetector-row CT in patients with congenital heart disease and an elongated aortic arch. The trachea was measured at the level of the aortic arch in 205 children and young adults and then the severity of tracheal compression was determined by measuring the tracheal diameter ratio (short axis diameter/long axis diameter). Patients were divided as follows: group I (normal aortic arch; n=166), group II (transversely running aortic arch; n=22), and group III (elongated aortic arch; n=17). From the viewpoint of the relationship of the great arteries, group II had D-malposition, and group III had L-malposition. Age, height, weight and body surface area were significantly correlated with the short and long axis diameter in group I. There was a negative correlation between tracheal diameter ratio and the physical size parameters. The tracheal diameter ratio in group III was 0.50{+-}0.13, which was significantly lower than in groups I and II (P<0.01 and 0.05, respectively). Even apparently asymptomatic patients with an elongated aortic arch can have tracheal compression. An elongated aortic arch may be a useful predictor of tracheal compression. (orig.)

  4. Agenesis of the internal carotid artery with a trans-sellar anastomosis: CT and MRI findings in late-onset congenital hypopituitarism

    International Nuclear Information System (INIS)

    Mellado, J.M.; Merino, X.; Ramos, A.; Salvado, E.; Sauri, A.

    2001-01-01

    A 29-year-old woman with a history of hypothyroidism since early childhood developed hypopituitarism. CT and MRI revealed anterior pituitary hypoplasia, an ectopic posterior lobe, a Chiari I malformation and agenesis of the right internal carotid artery with a trans-sellar anastomosis. This constellation of findings constitutes a previously unreported association in congenital hypopituitarism of late onset. The usefulness of imaging modalities and the pathogenic implications are also discussed. (orig.)

  5. Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    McHugh, Kieran; Afaq, Asim; Roebuck, Derek J. [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom); Broderick, Nigel [Nottingham University Hospitals, Radiology Department, Nottingham (United Kingdom); Gabra, Hany O.; Elliott, Martin J. [Great Ormond Street Hospital for Children, Department of Cardiothoracic Surgery, London (United Kingdom)

    2010-05-15

    Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition. (orig.)

  6. Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia

    International Nuclear Information System (INIS)

    McHugh, Kieran; Afaq, Asim; Roebuck, Derek J.; Broderick, Nigel; Gabra, Hany O.; Elliott, Martin J.

    2010-01-01

    Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition. (orig.)

  7. Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    McHugh, Kieran; Afaq, Asim; Roebuck, Derek J [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom); Broderick, Nigel [Nottingham University Hospitals, Radiology Department, Nottingham (United Kingdom); Gabra, Hany O; Elliott, Martin J [Great Ormond Street Hospital for Children, Department of Cardiothoracic Surgery, London (United Kingdom)

    2010-05-15

    Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition. (orig.)

  8. Congenital agenesis of unilateral parotid gland with ipsilateral type I first branchial cleft anomaly: A rare presentation

    OpenAIRE

    Tripti Maithani; Apoorva Pandey; Seema Acharya

    2014-01-01

    Aim: To report a rare case of unilateral parotid agenesis with ipsilateral type I first branchial cleft anomaly. Material and methods: A case study with special emphasis on the embryology, outlining the complex developmental process of parotid and branchial arches and highlighting the probable reason for development of such anomalies. Results: The literature states that unilateral parotid agenesis is a rare entity with few reported cases occurring solely or in conjunction with other hea...

  9. Does the serum uric acid level have any relation to arterial stiffness or blood pressure in adults with congenital renal agenesis and/or hypoplasia?

    Science.gov (United States)

    Yazici, Raziye; Guney, İbrahim; Altintepe, Lutfullah; Yazici, Mehmet

    2017-01-01

    The relationship between serum uric acid and arterial stiffness or blood pressure is not clear. The serum uric acid level and its association with cardiovascular risk is not well known in patients with reduced renal mass. We aimed to investigate the relation between serum uric acid levels and arterial stiffness and also blood pressure in patients with congenital renal agenesis and/or hypoplasia. In this single center, cross-sectional study, a total of 55 patients (39 (% 70.9) with unilateral small kidney and 16 (%29.1) with renal agenesis) were included. The median age was 35 (21-50) years. The study population was divided into tertiles of serum uric acid (according to 2.40-3.96, 3.97-5.10, and 5.11-9.80 mg/dl cut-off values of serum uric acid levels). Official and 24-h ambulatory non-invasive blood pressures of all patients were measured. The arterial stiffness was assessed by pulse wave velocity (PWV). PWV values were increased from first to third tertile (5.5 ± 0.6, 5.7 ± 0.8, 6.1 ± 0.7, respectively), but this gradual increase between tertiles did not reach significance. Linear regression analyses showed a positive correlation between serum uric acid levels and PWV (β = 0.40, p = 0.010), but no correlation was found between uric acid and daytime systolic blood pressure (β = 0.24, p = 0.345). In congenital renal agenesis/hypoplasia, the serum uric acid level was positively correlated with arterial stiffness, but there was no correlation with blood pressure.

  10. Tracheal web

    International Nuclear Information System (INIS)

    Legasto, A.C.; Haller, J.O.; Giusti, R.J.

    2004-01-01

    Congenital tracheal web is a rare entity often misdiagnosed as refractory asthma. Clinical suspicion based on patient history, examination, and pulmonary function tests should lead to its consideration. Bronchoscopy combined with CT imaging and multiplanar reconstruction is an accepted, highly sensitive means of diagnosis. (orig.)

  11. Gallbladder Agenesis with Refractory Choledocholithiasis.

    Science.gov (United States)

    Tjaden, Jamie; Patel, Kevin; Aadam, Aziz

    2015-01-01

    Congenital agenesis of the gallbladder is a rare anomaly which is usually asymptomatic and found incidentally. In some cases, however, patients are symptomatic. Common symptoms include right upper quadrant abdominal pain, nausea, and vomiting. Jaundice is present in some symptomatic cases and is due to associated choledocholithiasis (Fiaschetti et al. 2009). In this case, a 63-year-old female presents with jaundice and episodic right upper quadrant abdominal pain with nausea and vomiting. Bilirubin and alkaline phosphatase were found to be markedly elevated. Upper endoscopic ultrasound (EUS) revealed choledocholithiasis, and the patient required multiple endoscopic retrograde cholangiopancreatography (ERCP) sessions before successful extraction of all stones. Subsequent surgical exploration revealed congenital agenesis of the gallbladder. Although this is a rare finding, patients with agenesis of the gallbladder are at increased risk of developing de novo choledocholithiasis which may be challenging to extract.

  12. Gallbladder Agenesis with Refractory Choledocholithiasis

    Directory of Open Access Journals (Sweden)

    Jamie Tjaden

    2015-01-01

    Full Text Available Congenital agenesis of the gallbladder is a rare anomaly which is usually asymptomatic and found incidentally. In some cases, however, patients are symptomatic. Common symptoms include right upper quadrant abdominal pain, nausea, and vomiting. Jaundice is present in some symptomatic cases and is due to associated choledocholithiasis (Fiaschetti et al. 2009. In this case, a 63-year-old female presents with jaundice and episodic right upper quadrant abdominal pain with nausea and vomiting. Bilirubin and alkaline phosphatase were found to be markedly elevated. Upper endoscopic ultrasound (EUS revealed choledocholithiasis, and the patient required multiple endoscopic retrograde cholangiopancreatography (ERCP sessions before successful extraction of all stones. Subsequent surgical exploration revealed congenital agenesis of the gallbladder. Although this is a rare finding, patients with agenesis of the gallbladder are at increased risk of developing de novo choledocholithiasis which may be challenging to extract.

  13. Pulmonary agenesis: two cases reported

    Directory of Open Access Journals (Sweden)

    Denis Yaraví Solano-Vázquez

    2014-11-01

    Full Text Available Background: Pulmonary agenesis is a rare anomaly (1 in 15 000 live births which consists in a total absence or severe hypoplasia of one or both lungs. The clinical spectrum of the unilateral agenesis could vary from early and severe respiratory distress, recurrent pneumonia to being an incidental finding. The prognosis is based on the presence of associated congenital abnormalities. Material and methods: We present two cases of unilateral pulmonary agenesis in patients at Tlaxcala’s Children Hospital during 2012. Results: Report details the case of a one-month old boy with left pulmonary agenesis and interatrial communication and mild pulmonary arterial hypertension. He had two resolved pneumonia incidents. The other case was a one-month old girl with right pulmonary agenesis, associated to multiple heart malformations who evolved to respiratory failure, heart failure and death.Conclusions: Pulmonary agenesis is a rare anomaly. Its outcome and prognosis varies with the hemodynamics related to its location and associated malformations.

  14. Gallbladder Agenesis with Refractory Choledocholithiasis

    OpenAIRE

    Tjaden, Jamie; Patel, Kevin; Aadam, Aziz

    2015-01-01

    Congenital agenesis of the gallbladder is a rare anomaly which is usually asymptomatic and found incidentally. In some cases, however, patients are symptomatic. Common symptoms include right upper quadrant abdominal pain, nausea, and vomiting. Jaundice is present in some symptomatic cases and is due to associated choledocholithiasis (Fiaschetti et al. 2009). In this case, a 63-year-old female presents with jaundice and episodic right upper quadrant abdominal pain with nausea and vomiting. Bil...

  15. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

    Directory of Open Access Journals (Sweden)

    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  16. Recurrent meningitis in a case of congenital anterior sacral meningocele and agenesis of sacral and coccygeal vertebrae Meningite recorrente em um paciente com meningocele sacral anterior e agenesia sacral e coccigea

    Directory of Open Access Journals (Sweden)

    Carolina A. R. Funayama

    1995-12-01

    Full Text Available A rare case of recurrent meningitis due to congenital anterior sacral meningocele and agenesis of the sacral and coccygeal vertebrae is described. An autosomal dominant inheritance is demonstrated for lower cord malformation, and environmental factors (chromic acid or fumes are discussed.Um caso raro de meningite recorrente devido a meningocele sacral anterior e agenesia das vértebras sacras coccígeas é descrito. Herança autossômica dominante para malformação medular caudal é demonstrada e, possíveis fatores ambientais (ligados ao cromo, são discutidos.

  17. Agenesia pulmonar unilateral Unilateral pulmonary agenesis

    OpenAIRE

    Maura Cavada Malcon; Claudio Mattar Malcon; Marina Neves Cavada; Paulo Eduardo Macedo Caruso; Lara Flório Real

    2012-01-01

    A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital mal...

  18. Agenesis of the dorsal pancreas

    Science.gov (United States)

    Schnedl, Wolfgang J; Piswanger-Soelkner, Claudia; Wallner, Sandra J; Krause, Robert; Lipp, Rainer W

    2009-01-01

    During the last 100 years in medical literature, there are only 54 reports, including the report of Pasaoglu et al (World J Gastroenterol 2008; 14: 2915-2916), with clinical descriptions of agenesis of the dorsal pancreas in humans. Agenesis of the dorsal pancreas, a rare congenital pancreatic malformation, is associated with some other medical conditions such as hyperglycemia, abdominal pain, pancreatitis and a few other diseases. In approximately 50% of reported patients with this congenital malformation, hyperglycemia was demonstrated. Evaluation of hyperglycemia and diabetes mellitus in all patients with agenesis of the dorsal pancreas including description of fasting blood glucose, oral glucose tolerance test, glycated hemoglobin and medical treatment would be a future goal. Since autosomal dominant transmission has been suggested in single families, more family studies including imaging technologies with demonstration of the pancreatic duct system are needed for evaluation of this disease. With this letter to the editor, we aim to increase available information for the better understanding of this rare disease. PMID:19140241

  19. Treatment of cervical agenesis with minimally invasive therapy: Case report

    Directory of Open Access Journals (Sweden)

    Azami Denas Azinar

    2017-11-01

    Full Text Available Cervical agenesis is very rare congenital disorder case with cervical not formed. Because of cervical clogged so that menstruation can not be drained. We Report the case of a19 years old women still single with endometrioma, hematometra, cervical agenesis and perform surgery combination laparoscopy and transvaginally with laparoscopic cystectomy, neocervix, and use catheter no 24f in the new cervix. And now she can currently be normal menstruation. Minimally invasive theraphy of congenital anomalies case is recommended to save reproductive function. Keywords: Cervical agenesis, minimal invasive theraphy

  20. Pulmonary Artery Agenesis: A Case Series

    Directory of Open Access Journals (Sweden)

    Meltem Ağca

    2015-04-01

    Full Text Available Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis.The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.

  1. Evaluation of tracheal bronchus in Chinese children using multidetector CT

    International Nuclear Information System (INIS)

    Ming, Zhu; Lin, Zhang

    2007-01-01

    Tracheal bronchus is a congenital bronchial anomaly. The diagnosis should be considered early in intubated patients. Multidetector computed tomography (MDCT) is the newest modality for evaluating tracheal bronchus. To evaluate the utility of 16-slice MDCT in children with tracheal bronchus and to characterize the frequency of tracheal bronchus in children with congenital heart disease. From June 2005 to May 2007, 3,187 consecutive children (1,124 with congenital heart disease and 2,063 without congenital heart disease) underwent MDCT examination. Minimum-intensity projection reconstruction was performed to show the tracheobronchial tree in every case. Tracheal bronchus was found in 42 children (3.74%) with congenital heart disease but in only 6 children (0.29%) without congenital heart disease. Among the 48 children with tracheal bronchus, 45 had right-side tracheal bronchus and 3 had bilateral tracheal bronchi with heterotaxy syndrome. The diagnostic sensitivity of MDCT was 100% (48/48). MDCT is a reliable imaging technique for the diagnosis of tracheal bronchus. Our data showed that right-side tracheal bronchus was more common and bilateral tracheal bronchi usually occurred with heterotaxy syndrome. In addition, tracheal bronchus often occurred with congenital heart disease. The angle between the tracheal bronchus and the trachea is important and should be measured. (orig.)

  2. Pilot Study Comparing Closed Versus Open Tracheal Suctioning in Postoperative Neonates and Infants With Complex Congenital Heart Disease.

    Science.gov (United States)

    Tume, Lyvonne N; Baines, Paul B; Guerrero, Rafael; Hurley, Margaret A; Johnson, Robert; Kalantre, Atul; Ramaraj, Ram; Ritson, Paul C; Walsh, Laura; Arnold, Philip D

    2017-07-01

    To determine the hemodynamic effect of tracheal suction method in the first 36 hours after high-risk infant heart surgery on the PICU and to compare open and closed suctioning techniques. Pilot randomized crossover study. Single PICU in United Kingdom. Infants undergoing surgical palliation with Norwood Sano, modified Blalock-Taussig shunt, or pulmonary artery banding in the first 36 hours postoperatively. Infants were randomized to receive open or closed (in-line) tracheal suctioning either for their first or second study tracheal suction in the first 36 hours postoperatively. Twenty-four infants were enrolled over 18 months, 11 after modified Blalock-Taussig shunt, seven after Norwood Sano, and six after pulmonary artery banding. Thirteen patients received the open suction method first followed by the closed suction method second, and 11 patients received the closed suction method first followed by the open suction method second in the first 36 hours after their surgery. There were statistically significant larger changes in heart rate (p = 0.002), systolic blood pressure (p = 0.022), diastolic blood pressure (p = 0.009), mean blood pressure (p = 0.007), and arterial saturation (p = 0.040) using the open suction method, compared with closed suctioning, although none were clinically significant (defined as requiring any intervention). There were no clinically significant differences between closed and open tracheal suction methods; however, there were statistically significant greater changes in some hemodynamic variables with open tracheal suctioning, suggesting that closed technique may be safer in children with more precarious physiology.

  3. Agenesis of pancreas

    DEFF Research Database (Denmark)

    Voldsgaard, P; Kryger-Baggesen, N; Lisse, I

    1994-01-01

    Complete agenesis of pancreas is a rare and lethal condition. Four cases have previously been reported in combination with other malformations, such as severe intrauterine growth retardation, hyperglycaemia and meconium ileus. We report a case of pancreatic agenesis as a single anomaly. The child...

  4. Agenesia pulmonar unilateral Unilateral pulmonary agenesis

    Directory of Open Access Journals (Sweden)

    Maura Cavada Malcon

    2012-08-01

    Full Text Available A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital malformations. When the patient presented symptoms, including cough, wheezing, and dyspnea, with no clinical improvement after a period of 30 days, imaging studies were conducted and the diagnosis was made.

  5. Tracheal Atresia with Segmental Esophageal Duplication: An Unusual Anatomic Arrangement.

    Science.gov (United States)

    Gaerty, Kirsten; Thomas, Joseph T; Petersen, Scott; Tan, Edwin; Kumar, Sailesh; Gardener, Glenn; Armes, Jane

    2016-01-01

    An unusual anatomic configuration of segmental tracheal agenesis/atresia with esophageal duplication on autopsy in a fetus that demised in utero at 29 weeks is reported. The mother was scanned initially for a cardiac anomaly at 20 weeks and on follow-up scan at 27 weeks had polyhydramnios and underwent amnioreduction. The final autopsy diagnosis was vertebral, ano-rectal, cardiac, tracheoesophageal, renal, and limb malformations (VACTERL). We discuss the autopsy findings along with the embryological mechanisms and compare the configuration with Floyd's classification for tracheal agenesis. The difficulties in prenatal diagnosis are discussed.

  6. Prophylactic use of the Arabin cervical pessary in fetuses with severe congenital diaphragmatic hernia treated by fetoscopic endoluminal tracheal occlusion (FETO): preliminary experience.

    Science.gov (United States)

    Dobrescu, Oana; Cannie, Mieke M; Cordier, Anne-Gael; Rodó, Carlota; Fabietti, Isabella; Benachi, Alexandra; Carreras, Elena; Persico, Nicola; Hurtado, Ivan; Gucciardo, Léonardo; Jani, Jacques C

    2016-01-01

    The aim of this study was to describe whether the prophylactic use of a cervical pessary decreases the rate of premature birth in congenital diaphragmatic hernia (CDH) fetuses treated with fetoscopic tracheal occlusion (FETO). The study concerns a consecutive series of cases with CDH and FETO and a group of CDH without FETO. In a subgroup of the FETO group, a prophylactic cervical pessary was inserted the day following the procedure. Gestational age (GA) at birth was the primary outcome. Fifty-nine fetuses with FETO and 47 expectantly managed were included. The last 15 FETO had a cervical pessary inserted. The median GA at delivery in the FETO group with pessary was 35.1 weeks and was not different from that in the FETO group without a pessary (34.3 weeks; p = 0.28) but was below that in the expectantly managed group (38.3 weeks; p cervical pessary does not prolong gestation of CDH fetuses treated with FETO. © 2015 John Wiley & Sons, Ltd. © 2015 John Wiley & Sons, Ltd.

  7. Rare and Common Variants Conferring Risk of Tooth Agenesis

    DEFF Research Database (Denmark)

    Jonsson, L; Magnusson, T E; Thordarson, A

    2018-01-01

    We present association results from a large genome-wide association study of tooth agenesis (TA) as well as selective TA, including 1,944 subjects with congenitally missing teeth, excluding third molars, and 338,554 controls, all of European ancestry. We also tested the association of previously ...

  8. Prenatal exposure to antiepileptic drugs and dental agenesis.

    Directory of Open Access Journals (Sweden)

    Pernille E Jacobsen

    Full Text Available OBJECTIVE: The aim of the study was to investigate the association between prenatal exposure to AEDs and the risk of dental agenesis and to differentiate between the possible effects of the different drugs used. METHODS: Data on 214 exposed and 255 unexposed children, aged 12-18 years, were extracted from the Prescription Database of the Central Denmark Region and North Denmark Region and the Danish Medical Birth Registry. The children's dental charts were examined for the presence of dental agenesis. RESULTS: Overall, children exposed to AED in utero had an increased risk of developing dental agenesis, but as a group, the difference was not significant (OR = 1.7; [95% CI: 0.8-3.6]. The risk of developing dental agenesis was three-fold increased (OR = 3.1; [95% CI: 1.3-7.4] in children exposed to valproate in mono- or in poly-therapy with other AEDs than carbamazepine or oxcarbazepine. The risk was further increased (OR = 11.2; [95% CI: 2.4-51.9] in children exposed to valproate and carbamazepine or oxcarbazepine in combination. CONCLUSIONS: The present study shows that dental agenesis is a potential congenital abnormality that is related to prenatal exposure to valproate, and dental agenesis may be considered a sensitive marker for the teratogenicity of valproate.

  9. Prenatal Diagnosis of Bilateral Pulmonary Agenesis: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung A; Cho, Jeong Yeon; Lee, Seung Mi; Jun, Jong Kwan; Kang, Ji Eun; Seo, Jeong Wook [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2010-02-15

    We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.

  10. Postmortem MRI of bladder agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Barber, Brendan R. [St George' s Hospital, Radiology Department, London (United Kingdom); Weber, Martin A. [Great Ormond Street Hospital for Children, Department of Histopathology, London (United Kingdom); Bockenhauer, Detlef [Great Ormond Street Hospital for Children, Department of Nephrology, London (United Kingdom); Hiorns, Melanie P.; McHugh, Kieran [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom)

    2011-01-15

    We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy. We are unaware of another postmortem MRI study demonstrating bladder agenesis. (orig.)

  11. Postmortem MRI of bladder agenesis

    International Nuclear Information System (INIS)

    Barber, Brendan R.; Weber, Martin A.; Bockenhauer, Detlef; Hiorns, Melanie P.; McHugh, Kieran

    2011-01-01

    We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy. We are unaware of another postmortem MRI study demonstrating bladder agenesis. (orig.)

  12. [A case of lingual agenesis].

    Science.gov (United States)

    Carinci, F; Felisatti, P; Curioni, C

    1996-01-01

    Lingual agenesis is a rare anomaly caused by failed morphogenesis of the lateral lingual swellings during embriogenesis. Most reported cases have been part of oromandibular limb hypogenesis syndrome (OLHS). A case of lingual agenesis associated with micrognathia and alteration of the hands is reported.

  13. Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

    Directory of Open Access Journals (Sweden)

    Blesneac Cristina

    2016-06-01

    Full Text Available Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH, that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

  14. MRI findings of type II sacral agenesis: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang A; Kim, Myung Soon; Kwon, Woo Cheol [Dept. of Radiology, Yonsei University Wonju College of Medicine, Wonju Severance Christian Hospital, Wonju (Korea, Republic of)

    2016-07-15

    Sacral agenesis (or caudal regression syndrome) is a rare congenital anomaly involving various levels of coccygeal, sacral, and even lumbar or lower thoracic vertebral dysgenesis, as well as spinal cord abnormalities. A few cases have been previously reported in Korea, especially based upon MRI findings. We describe a case of a 4-year-old girl with partially bilateral agenesis of the sacrum (type II), and club-shaped (chisel-shaped) spinal cord disruption. We also review MRI findings of sacral agenesis, focused on classification and radiological findings.

  15. Agenesis of the corpus callosum and autism: a comprehensive comparison.

    Science.gov (United States)

    Paul, Lynn K; Corsello, Christina; Kennedy, Daniel P; Adolphs, Ralph

    2014-06-01

    The corpus callosum, with its ∼200 million axons, remains enigmatic in its contribution to cognition and behaviour. Agenesis of the corpus callosum is a congenital condition in which the corpus callosum fails to develop; such individuals exhibit localized deficits in non-literal language comprehension, humour, theory of mind and social reasoning. These findings together with parent reports suggest that behavioural and cognitive impairments in subjects with callosal agenesis may overlap with the profile of autism spectrum disorders, particularly with respect to impairments in social interaction and communication. To provide a comprehensive test of this hypothesis, we directly compared a group of 26 adults with callosal agenesis to a group of 28 adults with a diagnosis of autism spectrum disorder but no neurological abnormality. All participants had full-scale intelligence quotient scores >78 and groups were matched on age, handedness, and gender ratio. Using the Autism Diagnostic Observation Schedule together with current clinical presentation to assess autistic symptomatology, we found that 8/26 (about a third) of agenesis subjects presented with autism. However, more formal diagnosis additionally involving recollective parent-report measures regarding childhood behaviour showed that only 3/22 met complete formal criteria for an autism spectrum disorder (parent reports were unavailable for four subjects). We found no relationship between intelligence quotient and autism symptomatology in callosal agenesis, nor evidence that the presence of any residual corpus callosum differentiated those who exhibited current autism spectrum symptoms from those who did not. Relative to the autism spectrum comparison group, parent ratings of childhood behaviour indicated children with agenesis were less likely to meet diagnostic criteria for autism, even for those who met autism spectrum criteria as adults, and even though there was no group difference in parent report of current

  16. A Case of Distal Vaginal Agenesis Presenting with Recurrent Urinary Tract Infection and Pyuria in a Prepubertal Girl.

    Science.gov (United States)

    Dural, Ozlem; Ugurlucan, Funda Gungor; Yasa, Cenk; Bastu, Ercan; Eren, Hulya; Yuksel, Bahar; Celik, Serdal; Akhan, Suleyman Engin

    2017-02-01

    Isolated distal vaginal agenesis is a rare anomaly and mostly becomes symptomatic after menarche. We describe an unusual presentation of this anomaly in a prepubertal girl. An 11-year-old prepubertal girl presented with recurrent urinary tract infection, pyuria, and right-sided renal agenesis. The findings of perineal inspection, ultrasonography, and magnetic resonance imaging were consistent with a distal vaginal agenesis with pyometrocolpos. Discharging pyometrocolpos with dissection of the atretic portion and a pull-through vaginoplasty were performed. A cystoscopy showed no sign of a vesicovaginal or uterine fistula. This rare presentation of distal vaginal agenesis reminds us that congenital malformations of the female genital tract should be considered in patients with congenital anomalies of the urinary system and/or recurrent urinary tract infection, even during the prepubertal period. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  17. Deep venous thrombosis and agenesis of the intrahepatic segment of inferior vena cava

    International Nuclear Information System (INIS)

    Velasco, J.; Fernandez, M.M.; Manzanares, R.; Hernando, A.

    1997-01-01

    We present a case of agenesis of the intrahepatic segment of inferior vena cava (IVC) with drainage through the azygos and hemiazygos systems. The presenting sign was deep venous thrombosis (DVT) in both lower extremities. The different imaging studies led to the diagnosis of both the congenital and acquired venous abnormalities, which are discussed. (Author) 14 refs

  18. Maxillary sinus agenesis - report of two cases

    International Nuclear Information System (INIS)

    Pierre, Jorge Henrique Arraes de Alencar; Santana, Expedito Araujo

    2000-01-01

    Agenesis or aplasia of the maxillary sinuses is an extremely rare condition, and only eight cases have been reported in the world medical literature. These malformations may arise as a result of developmental defects. Two cases of unilateral agenesis of the maxillary sinus are presented and the radiological abnormalities and the embryology are discussed. The literature is also reviewed. (author)

  19. Iatrogenic tracheal tear.

    LENUS (Irish Health Repository)

    Dias, A

    2010-10-01

    Large post intubation tracheal tears are usually detected intra-operatively due to unstable signs namely impaired ventilation and mediastinal emphysema and often require surgical management. Smaller tracheal tears are often missed during anaesthesia and recognized during the postoperative period. Conservative management should be considered in these latter cases.

  20. Gallbladder agenesis in a Chihuahua.

    Science.gov (United States)

    Kamishina, Hiroaki; Katayama, Masaaki; Okamura, Yasuhiko; Sasaki, Jun; Chiba, Satoshi; Goryo, Masanobu; Sato, Reeko; Yasuda, Jun

    2010-07-01

    A 4-year-old neutered male Chihuahua was presented with a history of anorexia and vomiting. Continuous elevation of liver enzymes was found on repeated blood examinations and the dog was referred to us for further evaluation. The absence of gallbladder was suspected on ultrasonography. Exploratory laparotomy and retrograde cholangiography confirmed gallbladder agenesis and a possible hypoplasia of the right medial and lateral liver lobes. Histologically, proliferation of bile ductules associated with portal fibrosis and pseudolobular formation were apparent in the liver lobes.

  1. Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

    Directory of Open Access Journals (Sweden)

    V Govindaraj

    2017-01-01

    Full Text Available Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

  2. Agenesis of the posterior arch of the atlas

    Directory of Open Access Journals (Sweden)

    Torriani Martin

    2002-01-01

    Full Text Available PURPOSE: To illustrate the radiological findings and review the current literature concerning a rare congenital abnormality of the posterior arch of the atlas. CASE REPORT: An adult female without neurological symptoms presented with an absent posterior arch of the atlas, examined with plain films and helical computerized tomography. Complete agenesis of the posterior arch of the atlas is a rare entity that can be easily identified by means of plain films. Although it is generally asymptomatic, atlantoaxial instability and neurological deficits may occur because of structural instability. Computerized tomography provides a means of assessing the extent of this abnormality and can help evaluate the integrity of neural structures. Although considered to be rare entities, defects of the posterior arch of the atlas may be discovered as incidental asymptomatic findings in routine cervical radiographs. Familiarity with this abnormality may aid medical professionals in the correct management of these cases.

  3. Tooth agenesis in osteogenesis imperfecta related to mutations in the collagen type I genes.

    Science.gov (United States)

    Malmgren, B; Andersson, K; Lindahl, K; Kindmark, A; Grigelioniene, G; Zachariadis, V; Dahllöf, G; Åström, E

    2017-01-01

    Osteogenesis imperfecta (OI) is a heterogeneous group of disorders of connective tissue, mainly caused by mutations in the collagen type I genes (COL1A1 and COL1A2). Tooth agenesis is a common feature of OI. We investigated the association between tooth agenesis and collagen type I mutations in individuals with OI. In this cohort study, 128 unrelated individuals with OI were included. Panoramic radiographs were analyzed regarding dentinogenesis imperfecta (DGI) and congenitally missing teeth. The collagen I genes were sequenced in all individuals, and in 25, multiplex ligation-dependent probe amplification was performed. Mutations in the COL1A1 and COL1A2 genes were found in 104 of 128 individuals. Tooth agenesis was diagnosed in 17% (hypodontia 11%, oligodontia 6%) and was more frequent in those with DGI (P = 0.016), and in those with OI type III, 47%, compared to those with OI types I, 12% (P = 0.003), and IV, 13% (P = 0.017). Seventy-five percent of the individuals with oligodontia (≥6 missing teeth) had qualitative mutations, but there was no association with OI type, gender, or presence of DGI. The prevalence of tooth agenesis is high (17%) in individuals with OI, and OI caused by a qualitative collagen I mutation is associated with oligodontia. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  4. Neurological presentations, imaging, and associated anomalies in 50 patients with sacral agenesis.

    Science.gov (United States)

    Emami-Naeini, Parisa; Rahbar, Ziba; Nejat, Farideh; Kajbafzadeh, Abdolmohammad; El Khashab, Mostafa

    2010-10-01

    Sacral agenesis is an uncommon congenital disorder that involves multiple organs. We studied neurological manifestations of the disease, common associated disorders, and their relation with extent of bony malformation. We investigated neurological manifestations of 50 patients with sacral agenesis. Patients were evaluated for previous procedures, ambulation, limb abnormalities, vertebral alignment, recurrent urinary tract infection, urinary incontinence, dribbling, dimple, lower extremities weakness, myelomeningocele (MMC), and lipomyelomenangocele. Weakness of lower extremities was seen in 37 (74%) patients. Concurrent weakness of proximal and distal muscles of the lower limb was statistically associated with a type of bony aplasia (P = .001). However, paraplegia was seen in only 2 of 44 children over the age of 1, and the rest could walk. Myelodysplastic syndromes were seen in 21 patients. Sacral agenesis is diagnosed in children with concomitant MMC at younger ages and reveals more severe symptoms. Progression of neurological disorders was seen in 19 patients, in all of whom MRI showed tethering of the spinal cord. Urinary disorders including diurnal urinary incontinence (in 30 of 35 children over age 4) and recurrent urinary tract infections (in 37) were also common. Imperforate anus was seen in 11 patients. Twelve children over age 4 reported fecal incontinence, a problem that had statistically significant association with imperforate anus (P = .013). Different disorders can concurrently affect patients with sacral agenesis that may have profound impressions on patients and their families. Early diagnosis, thorough evaluation, and proper intervention are of utmost importance as they can prevent or lessen future complications.

  5. Tracheal collapse in two cats

    International Nuclear Information System (INIS)

    Hendricks, J.C.; O'Brien, J.A.

    1985-01-01

    Two cats examined bronchoscopically to discover the cause of tracheal collapse were found to have tracheal obstruction cranial to the collapse. Cats with this unusual sign should be examined bronchoscopically to ascertain whether there is an obstruction, as the cause in these 2 cats was distinct from the diffuse airway abnormality that causes tracheal collapse in dogs

  6. Klippel-Feil syndrome with associated agenesis of lung and gall bladder presenting with asthma and allergic rhinitis

    International Nuclear Information System (INIS)

    Khanna, Puneet; Panjabi, Chandramani; Shah, Ashok

    2005-01-01

    Klippel-Feil syndrome (KFS), a triad of short neck, limitation of neck movement and low posterior hairline, is characterized by the presence of congenitally fused cervical vertebrae and is often associated with multiple congenital anomalies. A 35-year-old male was referred for evaluation of an 'opaque hemithorax'. This led to a diagnosis of KFS, agenesis of left lung and gall bladder. The patient had history of wheezing dyspnea with nasal symptoms, which were diagnosed as asthma and allergic rhinitis. A high index of suspicion is required to recognize such a patient, and efforts should be made to seek other congenital anomalies. (author)

  7. Primary tracheal adenocystic carcinoma and tracheal tumors during pregnancy

    Directory of Open Access Journals (Sweden)

    Cem Gundogdu

    2011-07-01

    Full Text Available Cancer complicates approximately 0.1% of all pregnancies. Primary tracheal carcinoma is one of very rarely seen tumors and the rate of its being seen makes up approximately % 0,2 of all tumors of respiratory tract. The patient, 28 years old, who has 28-weeks-pregnant, was diagnosed with primary tracheal adenocystic carcinoma. Patient was made operation as thoracotomy and tracheal tumor was removed at the 28th week of pregnancy. Patient was delivered with sectio abdominale at the 39th week of pregnancy. Primary tracheal adenocystic carcinoma is very rarely seen tumors and it is the first tracheal ACC with pregnancy case in literature to have been detected and surgically treated during pregnancy. We discussed primary tracheal adenocystic carcinoma and tracheal tumors during pregnancy with literature.

  8. Sirenomelia with agenesis of corpus callosum.

    Science.gov (United States)

    Shirani, Shapour; Rekabi, Vahab; Kamalian, Naser

    2006-07-01

    Sirenomelia is a very rare anomaly presented with fusion of the lower limbs. Genitourinary, neural tube, and vertebral anomalies are found in most cases. We report a case of sirenomelia with agenesis of corpus callosum, which has not been reported previously.

  9. Uterus bicornis bicollis, imperforate hemivagina and ipsilateral renal agenesis: case report and literature review.

    Science.gov (United States)

    DaCosta, V; Christie, L; Wynter, S; Harriott, J; Frederick, J

    2009-09-01

    We present a case of a patient with Uterus bicornis bicollis, imperforate hemivagina and ipsilateral renal agenesis. This group of congenital malformations is often asymptomatic until puberty, when it presents as cyclic dysmenorrhoea, leucorrhoea or a pelvic mass. Magnetic resonance imaging is becoming the preferred modality for delineation of uterine malformations. When congenital abnormalities of the reproductive tract are encountered, a search should also be made for renal anomalies. Patients with Uterus bicornis bicollis and unilateral imperforate vagina are often seen with pain severe enough to mimic an acute abdomen. It is important to keep this unusual congenital malformation in mind in the differential diagnosis of vaginal discharge, pelvic mass and/or abdominopelvic pain in young women so as to avoid inappropriate surgical procedures.

  10. Laparoscopic Colectomy for a Patient with Congenital Renal Agenesis

    African Journals Online (AJOL)

    The right colon was mobilized by lateral‑to‑medial extension of a retroperitoneal dissection between the fusion fascia and the anterior renal fascia. The right testicular vessels were preserved without injury to the anterior renal fascia; however, the right ureter could not be detected. The operation was performed safely. Thus ...

  11. Cryopreserved irradiated tracheal homograft reconstruction for subglottic-tracheal stenosis

    International Nuclear Information System (INIS)

    Somyos Kunachak; Yongyudh Vajaradul; Boonchu Kulapaditharom

    1999-01-01

    Subglottic-tracheal stenosis is a common clinical entity. Handling on severe case is often problematic. Various tracheal replacement techniques have been used with varying degree of success and dispute. In this study we worked on cryopreserved irradiated tracheal homograft, of which its use in human has not been reported. The tracheas were harvested from donor cadavers within 24 hours of death in a sterile condition. After 1-2 weeks of preservation at -70 degree C, the grafts were irradiated at 25 kGy, then stored at -70 degree C until used. Four patients, 2 males and 2 females (aged 2-40 years, mean 16 years) with severe subglottic-tracheal stenosis underwent segmental tracheal graft reconstruction using this graft. Immunosuppressant was not given in any patient. The follow up period ranged from 11-1 5 months. Three patients were successfully decapulated, 1 patient developed local infection and dislodgement of intraluminal stent with subsequent restenosis. Postoperative tracheal lumen appeared near normal with histologic evidence of normal respiratory epithelium at the grafted site. In conclusion, cryopreserved irradiated tracheal homograft is a valuable alternative for tracheal transplant or reconstruction, without the need of immunosuppression

  12. Cryptophthalmos and Bilateral Renal Agenesis with Cleft Lip and Palate: Fraser Syndrome: Case Report

    Directory of Open Access Journals (Sweden)

    Emre Pabuçcu

    2012-12-01

    Full Text Available Fraser syndrome is a rare autosomal recessive disorder consisting of multiple anomalies including variable expression of cryptophthalmos, syndactyly, abnormal genitalia, malformations of the nose, ear and larynx, renal agenesis, oro-facial clefts, skeletal defects, umbilical hernia and mental retardation. Antenatally detected multiple congenital fetal anomalies during 22nd week of gestation is reported in this paper. Fraser Syndrome was diagnosed according to major and minor criteria. Early antenatal detection is mandatory and clinician should be awere of the high recurrence rates of this syndrome among siblings threatening subsequent pregnancies and should inform affected families.

  13. Bilateral renal agenesis, a severe anomaly in a premature infant with VACTERL association: A case report

    Directory of Open Access Journals (Sweden)

    Erol Basuguy

    2017-11-01

    Full Text Available We report on a preterm male (birth weight 1,100 g with bilateral renal agenesis, a lethal malformation. Additionally, the child suffered from an atrial septal defect, ventricular septal defect, right aortic arch anomaly, a high type of anal atresia, vertebral anomalies, limbs defects (VACTERL association. The infant during first day of life was treated with an emergency sigmoid ostomy and peritoneal dialysis because of increasing abdominal dilatation and high urea and creatinine levels in blood. Important congenital anomalies associated with VACTERL association and prematurity are very serious causes of mortality in the early period

  14. Genetic epidemiology of tooth agenesis in Japan: a population- and family-based study.

    Science.gov (United States)

    Machida, J; Nishiyama, T; Kishino, H; Yamaguchi, S; Kimura, M; Shibata, A; Tatematsu, T; Kamamoto, M; Yamamoto, K; Makino, S; Miyachi, H; Shimozato, K; Tokita, Y

    2015-08-01

    Tooth agenesis is one of the most common congenital anomalies in humans. However, the etiology of tooth agenesis remains largely unclear, as well as evidence base useful for genetic counseling. Therefore, we estimated the prevalence and sibling recurrence risk, and investigated agenetic patterns systematically. Tooth agenesis was classified into two subtypes: hypodontia (one to five missing teeth) and oligodontia (six or more missing teeth). The prevalence of these two subtypes were 6.8% [95% confidence interval (CI): 6.1-7.7%] and 0.1% (95% CI: 0.04-0.3%), respectively, and sibling recurrence risk of these were 24.5% (95% CI: 13.8-38.3%) and 43.8% (95% CI: 26.4-62.3%), respectively. This result suggests that the severe phenotype, oligodontia, might be mostly transmitted in a dominant fashion. Using a simple statistical modeling approach, our data were found to be consistent with a bilateral symmetry model, meaning that there was equal probability of missing teeth from the right and left sides. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  15. Vermian agenesis without posterior fossa cyst

    International Nuclear Information System (INIS)

    Adamsbaum, C.; Moreau, V.; Bulteau, C.; Burstyn, J.; Lair Milan, F.; Kalifa, G.

    1994-01-01

    We report 11 cases of vermian partial agenesis without posterior fossa cyst or hemispheric abnormalities. Characteristic MR signs were: absence of the posterior lobe, hypoplasia of the anterior lobe, a narrow sagittal cleft separating the hemispheres (''buttocks sign'') and fourth ventricle deformity. The main clinical signs were complex oculomotor dysfunction and developmental delay. None of the patients had respiratory symptoms. Consideration is given to the relationship between Joubert syndrome and this entity as well as to embroyological data. (orig.)

  16. Total agenesis of the left pericardium

    Directory of Open Access Journals (Sweden)

    J. Glauco Lobo Fº

    1999-09-01

    Full Text Available This is the report of a 46-year-old patient with the preoperative diagnosis of an atrial septal defect (ASD of the ostium secudum type. After sternectomy, partial agenesis of the left pericardium was diagnosed. It is our opinion that, if the radiographic picture is suggestive of this entity, a clinical search for cardiopulmonary anomalies should be performed, because the majority of these associated anomalies can and should be surgically corrected.

  17. Diagnosis and Management of a Patient with Congenitally Missing Maxillary First Permanent Molars: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Megha Gupta

    2016-01-01

    Full Text Available Congenitally missing teeth are the most commonly seen dental anomalies. Agenesis of the permanent first molar has the least frequency of all the tooth types, and it usually occurs in association with oligodontia or anodontia. Thus, agenesis of the bilateral maxillary first permanent molar is an extremely rare occurrence, and no such case has been reported in ethnic Saudi Arabian population. We hereby report a case of nonsyndromic bilateral congenitally missing maxillary first permanent molar in an eight-year-old Saudi female patient. Comprehensive oral rehabilitation was done for the patient. The implications of the tooth agenesis are also discussed. The prognosis of this case is presented.

  18. Congenital anomalies of the neonatal head

    International Nuclear Information System (INIS)

    Benson, C.B.; Teele, R.L.; Dobkin, G.R.; Fine, C.; Bundy, A.L.; Doubilet, P.M.

    1987-01-01

    US is a noninvasive modality that has proved highly valuable in the assessment of congenital intracranial anomalies in the neonate. The patterns of malformation must be familiar to those who obtain and interpret neonatal cranial sonograms. The authors present a variety of cases of congenital anomalies studied with US, including agenesis of the corpus callosum, Dandy-Walker malformation, occipital enecphalocele, congenital hydrocephalus, vein of Galen aneurysm, hydranencephaly, holoprosencephaly, absence of the septum pellucidum, schizencephaly, and hypoplastic cerebellum. Correlation with other radiographic modalities and pathologic follow-up, available in a majority of cases, is included

  19. Isolated Unilateral Pulmonary Artery Agenesis complicated by Symptomatic Aspergilloma

    LENUS (Irish Health Repository)

    Daly, A

    2017-11-01

    Isolated unilateral pulmonary artery agenesis is a rare diagnosis. Poor blood flow to the lung parenchyma renders the tissue susceptible to opportunistic infections. We present the unusual case of isolated unilateral pulmonary artery agenesis complicated by aspergilloma. Management options and considerations are discussed.

  20.  Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report

    Directory of Open Access Journals (Sweden)

    Hansa Dhar

    2011-11-01

    Full Text Available  Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional referral hospital presented with history of dysmenorrhoea and foul vaginal discharge with right cystic pelvic mass. She was diagnosed as a case of double uterus with obstructed right hemivagina and right pyocolpos with ipsilateral renal agenesis after routine ultrasonography in the clinic followed by MRI. Excision of the right vaginal septum with drainage of 200 ml of purulent discharge was performed. She was relieved of her symptoms and conceived promptly after the surgical excision of the partial vaginal septum.

  1. Agenesis of maxillary lateral incisors and associated dental anomalies.

    Science.gov (United States)

    Garib, Daniela Gamba; Alencar, Bárbara Maria; Lauris, José Roberto Pereira; Baccetti, Tiziano

    2010-06-01

    The objectives of this study were to evaluate the prevalence of dental anomalies in patients with agenesis of maxillary lateral incisors and to compare the findings with the prevalence of these anomalies in the general population. A sample of 126 patients, aged 7 to 35 years, with agenesis of at least 1 maxillary lateral incisor was selected. Panoramic and periapical radiographs and dental casts were used to analyze other associated dental anomalies, including agenesis of other permanent teeth, ectopia of unerupted permanent teeth, microdontia of maxillary lateral incisors, and supernumerary teeth. The occurrence of these anomalies was compared with prevalence data previously reported for the general population. Statistical testing was performed with the chi-square test (P <0.05) and the odds ratio. Patients with maxillary lateral incisor agenesis had a significantly increased prevalence rate of permanent tooth agenesis (18.2%), excluding the third molars. The occurrence of third-molar agenesis in a subgroup aged 14 years or older (n = 76) was 35.5%. The frequencies of maxillary second premolar agenesis (10.3%), mandibular second premolar agenesis (7.9%), microdontia of maxillary lateral incisors (38.8%), and distoangulation of mandibular second premolars (3.9%) were significantly increased in our sample compared with the general population. In a subgroup of patients aged 10 years or older (n = 115), the prevalence of palatally displaced canines was elevated (5.2%). The prevalences of mesioangulation of mandibular second molars and supernumerary teeth were not higher in the sample. Permanent tooth agenesis, maxillary lateral incisor microdontia, palatally displaced canines, and distoangulation of mandibular second premolars are frequently associated with maxillary lateral incisor agenesis, providing additional evidence of a genetic interrelationship in the causes of these dental anomalies. 2010 American Association of Orthodontists. Published by Mosby, Inc. All

  2. Presentations of agenesis of the hemidiaphragm in an adult

    International Nuclear Information System (INIS)

    Izeldin, Osama M.; Ahmed, Mohamed E.

    2008-01-01

    A 67-year-old man was presented with chest tightness and vomiting of one-week duration. On physical examination bowel sounds were heard on the left chest. Plain chest x-ray, barium swallow and CT gastric scan suggested volvulus with diaphragmatic hernia. At laparotomy there was complete agenesis of the left hemidiaphragm with no diaphragmatic remnants seen and no associated lung hypoplasia. This is an extremely rare condition and careful assessment is needed to differentiate between diaphragmatic hernia and agenesis of diaphragm as more cases could be diagnosed in the future as a result of good perinatal care of agenesis of hemidiaphragm (AHD) and long term survival to adulthood. (author)

  3. Tracheobronchial Branching Anomalies

    International Nuclear Information System (INIS)

    Hong, Min Ji; Kim, Young Tong; Jou, Sung Shick; Park, A Young

    2010-01-01

    There are various congenital anomalies with respect to the number, length, diameter, and location of tracheobronchial branching patterns. The tracheobronchial anomalies are classified into two groups. The first one, anomalies of division, includes tracheal bronchus, cardiac bronchus, tracheal diverticulum, pulmonary isomerism, and minor variations. The second one, dysmorphic lung, includes lung agenesis-hypoplasia complex and lobar agenesis-aplasia complex

  4. Tracheobronchial Branching Anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Min Ji; Kim, Young Tong; Jou, Sung Shick [Soonchunhyang University, Cheonan Hospital, Cheonan (Korea, Republic of); Park, A Young [Soonchunhyang University College of Medicine, Asan (Korea, Republic of)

    2010-04-15

    There are various congenital anomalies with respect to the number, length, diameter, and location of tracheobronchial branching patterns. The tracheobronchial anomalies are classified into two groups. The first one, anomalies of division, includes tracheal bronchus, cardiac bronchus, tracheal diverticulum, pulmonary isomerism, and minor variations. The second one, dysmorphic lung, includes lung agenesis-hypoplasia complex and lobar agenesis-aplasia complex

  5. Isolated Left Pulmonary Artery Agenesis: A Case Report

    Directory of Open Access Journals (Sweden)

    Tansel Ansal Balcı

    2012-08-01

    Full Text Available Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation- perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. (MIRT 2012;21:80-83

  6. Agenesis of the corpus callosum with associated inter-hemispheric ...

    African Journals Online (AJOL)

    Adele

    developing countries should be alert to organic disorders presenting with ... CT Scan (Figures 1 and 2) of the head revealed agenesis ... co-existing lipoma is often seen with this type of ... childhood behavioural disorders, bipolar disorders,.

  7. An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome

    Directory of Open Access Journals (Sweden)

    Haldar M

    2014-08-01

    Full Text Available Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. Sometime it presents in unusual way with prolonged vaginal bleeding and profuse vaginal discharge. Here we report case of an 18 years old married female with OHVIRA syndrome presenting late with symptoms of recurrent pyocolpos following dilatation and evacuation for midtrimester miscarriage that was diagnosed on the basis of MRI and managed by vaginal septotomy.

  8. Radiographic Assessment of Dental Maturation in Children With Dental Agenesis.

    Science.gov (United States)

    Medina, Aida Carolina; Pozo, Rodrigo Del; de Cedres, Lucila Blanco

    Dental agenesis is the most common developmental anomaly in humans, frequently associated with disorders in dental development and maturation. The purpose of this study is to determine radiographic variations in dental maturation in a group of Venezuelan children with dental agenesis. 1,188 panoramic radiographs, from healthy patients ages 5 to 12 years old were studied for agenesis of permanent teeth. Dental maturation was assessed by relative eruption and dental age according to Nolla, comparing children affected with dental agenesis to a stratified control group selected from the same population, excluding children with premature loss of primary teeth in the left quadrants and unclear radiographs. Descriptive analysis, and differences between means and medians (Student t test, Kruskall-Wallis p=0.05) were performed. Medians for Nolla stages were similar between groups, with delay in tooth formation in the agenesis group for second molars (p<0.05) and maxillary lateral incisors and second premolars. Dental age was significantly underestimated for both groups, -0.89 (±0.78) for the control group and -1.20 (±0.95) for the study group. Tooth eruption was similar between groups. Dental age was significantly delayed in Venezuelan children with dental agenesis, with variable significance for tooth formation of studied teeth.

  9. Congenital Absence of the Internal Carotid Artery

    International Nuclear Information System (INIS)

    Florio, Francesco; Balzano, Silverio; Nardella, Michele; Strizzi, Vincenzo; Cammisa, Mario; Bozzini, Vincenzo; Catapano, Giuseppe; D'Angelo, Vincenzo

    1999-01-01

    We report three cases of congenital absence of an internal carotid artery (ICA), diagnosed incidentally by digital subtraction angiography. The analysis of the cases is based on the classification of segmental ICA agenesis proposed by Lasjaunias and Berenstein. Usually the patients with this rare vascular anomaly are asymptomatic; some may have symptoms related to cerebrovascular insufficiency, compression by enlarged intracranial collateral vessels, or complications associated with cerebral aneurysms. Diagnosis of congenital absence of ICA is made by skull base computed tomography (CT) scan, CT and magnetic resonance angiography, and conventional or digital subtraction angiography

  10. MURCS Association with Partial Duplication of the Distal Long Chromosome 5 and Unilateral Ovarian Agenesis

    Directory of Open Access Journals (Sweden)

    Anna Dabkowska-Huc

    2013-01-01

    Full Text Available A combination of the congenital abnormalities, Müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia, is defined as the MURCS association. Various genetic defects have been described in the MURCS association so far, yet the unambiguous molecular basis of these disorders has not been established. We report the case of an 18-year-old woman who presented with primary amenorrhea, right kidney, Arnold-Chiari malformation, and Klippel-Feil syndrome. In addition, the patient showed the following unusual features: right ovarian and Skenes gland agenesis, cubitus valgus with hyperextension and decreased range of motion at elbows, and facial changes. Moreover, the performed DNA analysis showed interstitial duplication in chromosome 5 (5q35.1. In the duplicated region, there are genes whose function is not well known. It is thought that they have an influence on the early stages of development and their joining in the later period can lead to neoplastic disorders, especially leukemias.

  11. Fetal MRI in experimental tracheal occlusion

    Energy Technology Data Exchange (ETDEWEB)

    Wedegaertner, Ulrike [Department of Diagnostic and Interventional Radiology, Universitaetsklinikum Hamburg-Eppendorf, Martinistrasse 52, 20251 Hamburg (Germany)]. E-mail: wedegaer@uke.uni-hamburg.de; Schroeder, Hobe J. [Experimental Gynecology, Department of Obstetrics and Prenatal Medicine, Universitaetsklinikum Hamburg-Eppendorf, Hamburg (Germany); Adam, Gerhard [Department of Diagnostic and Interventional Radiology, Universitaetsklinikum Hamburg-Eppendorf, Hamburg (Germany)

    2006-02-15

    Congenital diaphragmatic hernia (CDH) is associated with a high mortality, which is mainly due to pulmonary hypoplasia and secondary pulmonary hypertension. In severely affected fetuses, tracheal occlusion (TO) is performed prenatally to reverse pulmonary hypoplasia, because TO leads to accelerated lung growth. Prenatal imaging is important to identify fetuses with pulmonary hypoplasia, to diagnose high-risk fetuses who would benefit from TO, and to monitor the effect of TO after surgery. In fetal imaging, ultrasound (US) is the method of choice, because it is widely available, less expensive, and less time-consuming to perform than magnetic resonance imaging (MRI). However, there are some limitations for US in the evaluation of CDH fetuses. In those cases, MRI is helpful because of a better tissue contrast between liver and lung, which enables evaluation of liver herniation for the diagnosis of a high-risk fetus. MRI provides the ability to determine absolute lung volumes to detect lung hypoplasia. In fetal sheep with normal and hyperplastic lungs after TO, lung growth was assessed on the basis of cross-sectional US measurements, after initial lung volume determination by MRI. To monitor fetal lung growth after prenatal TO, both MRI and US seem to be useful methods.

  12. Vaginal agenesis: Experience with sigmoid colon neovaginoplasty

    Directory of Open Access Journals (Sweden)

    Rawat Jiledar

    2010-01-01

    Full Text Available Aim: Objective of this study is to report our experience with sigmoid vaginoplasty in adolescents. Materials and Methods: A retrospective study of children with vaginal atresia and Mayer-Rokitansky-Kuster-Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the cases. Results: Eight children were studied over a period of 7 years. The postoperative complications were ileus in 2, mucosal prolapse of the neovagina in 1, and minor wound infection in 1 patient. Seven patients are on regular follow-up. All the neovaginas were patent and functional. One patient had unacceptable perineal appearance, that is, badly scarred perineum as a late complication. None of the patients had vaginal stenosis or excessive mucus discharge, during follow-up visits. Out of the 7 patients, 2 patients are sexually active and satisfied. Conclusions: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, and short recovery time.

  13. Bilateral Deep Vein Thrombosis Associated with Inferior Vena Cava Agenesis in a Young Patient Manifesting as Low Back Pain

    Directory of Open Access Journals (Sweden)

    Felipe Langer

    2017-04-01

    Full Text Available Congenital absence of the inferior vena cava is a rare vascular anomaly, and most cases are asymptomatic. Nevertheless, patients with inferior vena cava malformations may have increased risk of deep venous thrombosis. Particularly, cases of bilateral deep venous thrombosis may arise owing to an insufficient collateral venous drainage from the lower limbs. We hereby describe a case of a previously healthy young male patient presenting with bilateral lower limb deep venous thrombosis as the initial clinical manifestation of congenital inferior vena cava agenesis. We conclude that in young patients presenting with deep venous thrombosis, especially when thrombosis occurs spontaneously, bilaterally, or recurrently, inferior vena cava anomalies should be thoroughly investigated and ruled out as appropriate.

  14. Agenesis of internal carotid artery associated with isolated growth hormone deficiency: a case report and literature review.

    Science.gov (United States)

    Stagi, Stefano; Traficante, Giovanna; Lapi, Elisabetta; Pantaleo, Marilena; Becciani, Sabrina; Mortilla, Marzia; Seminara, Salvatore; de Martino, Maurizio

    2015-10-19

    Agenesis of the internal carotid artery (ICA) is a rare congenital abnormality, sporadically reported to be associated with a combined congenital hypopituitarism. Nevertheless, only a few cases have been extensively described, and none of these have been characterized by an isolated growth hormone (GH) deficiency. Here, we describe a 17-year old boy referred to our hospital for fatigue, decreased muscle strength and severe headache reported after the cessation of rhGH treatment for a GH deficiency diagnosed at the age of 2 years and 3 months. Magnetic resonance imaging (MRI) showed an adenohypophyseal hypoplasia with a lack of posterior pituitary hyperintensity, whereas MRI angiography indicated the absence of a normal flow void in the left ICA. Endocrinological tests confirmed the GH deficiency (GH peak after growth-hormone-releasing hormone (GHRH) + arginine: 2.42 ng/mL) with a very low IGF-I value (31 ng/mL) and normal function of other pituitary axes. To the best of our knowledge this is the first confirmed case of an isolated GH deficiency in a patient with ICA agenesis. The presence of an isolated pituitary deficit is unlike to be considered only as an effect of hemodynamic mechanism, suggesting a role for genetic factor(s) as a common cause of these two rare birth defects. Further studies could clarify this issue and the underlying mechanisms to better understand the etiopathogenetic characteristics of this disorder.

  15. Tracheal replacement by autogenous aorta

    Directory of Open Access Journals (Sweden)

    Anoosh Farhad

    2009-06-01

    Full Text Available Abstract Background Tracheal defects may occur after trauma or prolonged intubation. Resection of tracheal tumors also poses a major challenge for substitution. In an effort to solve this problem, different techniques have been tried with little success. We report on a new animal model which showed acceptable results with fewer complications. Methods We replaced 5 cm of cervical trachea in 10 dogs with harvested infra-renal aorta and repaired the aortic defect with Dacron graft. Results Necropsy of the grafted aorta and anastomotic site revealed well healed anastomosis in all animals together with ciliated columnar epithelium coverage of grafted aorta and neovascularization of aortic wall. Conclusion Aortic graft is preferable to other substitutes because of less antigenicity, less vascularity, and no mucous secretions or peristalsis

  16. Tracheal replacement by autogenous aorta.

    Science.gov (United States)

    Anoosh, Farhad; Hodjati, Hossain; Dehghani, Seifollah; Tanideh, Nader; Kumar, Perikala V

    2009-06-09

    Tracheal defects may occur after trauma or prolonged intubation. Resection of tracheal tumors also poses a major challenge for substitution. In an effort to solve this problem, different techniques have been tried with little success. We report on a new animal model which showed acceptable results with fewer complications. We replaced 5 cm of cervical trachea in 10 dogs with harvested infra-renal aorta and repaired the aortic defect with Dacron graft. Necropsy of the grafted aorta and anastomotic site revealed well healed anastomosis in all animals together with ciliated columnar epithelium coverage of grafted aorta and neovascularization of aortic wall. Aortic graft is preferable to other substitutes because of less antigenicity, less vascularity, and no mucous secretions or peristalsis.

  17. Tracheal stoma necrosis: a case repor

    Directory of Open Access Journals (Sweden)

    Pak S

    2017-04-01

    Full Text Available Acute tracheal dilatation, due to an overinflated cuff, has been reported early in the course of mechanical ventilation through an endotracheal tube. Tracheal stoma necrosis is a rare complication, but such can accompany acute tracheal dilation. Herein, we report a case of tracheal necrosis 9 days following tracheostomy placement in a 71-year old woman associated with overinflation of the tracheal tube cuff. This case report aims to 1 add to the scant body of knowledge about the diagnosis and management for the patients with tracheal stoma necrosis and 2 raise awareness for error-traps in interpreting diagnostic images, specifically satisfaction of search error, inattentional blindness error, and alliterative error.

  18. Effects of tracheal occlusion with retinoic acid administration on normal lung development.

    Science.gov (United States)

    Delabaere, Amélie; Marceau, Geoffroy; Coste, Karen; Blanchon, Loïc; Déchelotte, Pierre-Jean; Blanc, Pierre; Sapin, Vincent; Gallot, Denis

    2017-05-01

    Tracheal occlusion (TO) is an investigational therapy for severe congenital diaphragmatic hernia that decreases pulmonary hypoplasia, but sustained TO also induces deficient surfactant synthesis. Intramuscular maternal administration of retinoic acid (RA) in a surgical rabbit model of congenital diaphragmatic hernia showed a beneficial effect on lung maturation. We evaluated the potential of RA delivery into the trachea and studied the combined effects of TO and RA on normal lung development. Experiments were performed on normal rabbit fetuses. Liposomes and capric triglyceride (Miglyol ® ), alone and with RA, were administered in the trachea just before TO (d26). Lung morphology and surfactant production were studied at term (d30). Tracheal occlusion increased lung weight and enhanced alveolar development but increased apoptotic activity and decreased surfactant expression. Tracheal injection of RA improved surfactant production to levels of normal controls. We established the potential of liposome and Miglyol as RA vehicle for delivering this bioactive molecule in the fetal airways. Tracheal RA injection seems to oppose the effects of TO in fetuses with normal lungs. © 2017 John Wiley & Sons, Ltd. © 2017 John Wiley & Sons, Ltd.

  19. Müllerian agenesis with hypohidrotic ectodermal dysplasia syndrome.

    Science.gov (United States)

    Whaley, Katie; Winter, Jordan; Eyster, Kathleen M; Hansen, Keith A

    2012-04-01

    To describe the association of müllerian agenesis with hypohidrotic ectodermal dysplasia. Case report. University medical center. A 17-year-old woman with hypohidrotic ectodermal dysplasia referred for evaluation of primary amenorrhea. History, physical examination, and ultrasound. Physical findings of these two syndromes. Physical examination and ultrasound demonstrated müllerian agenesis with findings of hypohidrotic ectodermal dysplasia. This is the first description of the association of müllerian agenesis with ectodermal dysplasia. This rare case might provide further insight into the development of the uterus and the ectoderm as well as its derivatives. Copyright © 2012 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  20. A Comfortable Solution To Tracheal Anastomosis Protection: Tracheal Retention Sutures.

    Science.gov (United States)

    Karapolat, Sami; Turkyilmaz, Atila; Seyis, Kubra Nur; Tekinbas, Celal

    2018-04-01

    Fixation of the chin to the anterior chest wall is the most commonly used method of reducing anastomotic tension following a segmental resection of the trachea and reconstruction with primary anastomosis. However, the sutures required for this method may lead to various organic and psychological problems. In five patients who underwent tracheal resection and primary anastomosis, retention sutures were placed on the proximal and distal-lateral edges of the anastomotic line rather than placing a Guardian chin stitch. All patients were mobilised in the early postoperative period and were able to perform their routine daily activities without restrictions. During their average 14.4 months of follow-up, no complications were found in their anastomotic lines during their clinical, radiological, and bronchoscopic assessments. The placement of tracheal retention sutures proved an inexpensive and reliable method to reduce anastomotic tension without additional surgical burden, and was effective in terms of patient comfort. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  1. Bochdalek Hernia with Adult Diaphragmatic Agenesis

    Directory of Open Access Journals (Sweden)

    Erkan Akar

    2013-10-01

    Full Text Available    Diaphragmatic hernia arises from pleuro-peritoneal membranes inability to close pericardioperitoneal membranes. Diaphragmatic defect may be located in esophageal hiatus (hiatal hernia, nearby the hiatus (paraesophageal, retrosternal (Morgagni or posterolateral (Bochdalek. Congenital diaphragmatic hernias (CDH diagnosed after neonatal period are defined as late presenting CDH. This group of patients consist 5-31% of CDHs and lead to diagnostic difficulties. A case of adult type Bochdalek hernia who was admitted to our clinic with respiratory problems and recognized late with the absence of left diaphragm was discussed in the light of clinical and surgical methods.

  2. Bicornuate unicollis uterus with left renal agenesis | Adeyinka ...

    African Journals Online (AJOL)

    This is a case report of a 30 year old lady with 8 years history of 2 Infertility. Radiological findings by Hystero-salpingography (HSG) revealed a Bicornuate Unicolis uterus. This was further confirmed by Ultrasound, which also demonstrated a Left Renal agenesis inclusive. Radiological features, clinical presentation and ...

  3. Bilateral agenesis of the anterior cruciate ligament: MRI evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Bedoya, Maria A.; Jaramillo, Diego [The Children' s Hospital of Philadelphia, Radiology Department, Philadelphia, PA (United States); McGraw, Michael H. [Hospitalof theUniversityof Pennsylvania, Divisionof Orthopaedics, Philadelphia, PA (United States); Wells, Lawrence [The Children' s Hospital of Philadelphia, Division of Orthopaedics, Philadelphia, PA (United States)

    2014-09-15

    Bilateral agenesis of the anterior cruciate ligament (ACL) is extremely rare. We describe a 13-year-old girl who presented with bilateral knee pain without history of trauma; she has two family members with knee instability. Magnetic resonance imaging showed bilateral absence of the ACL, and medial posterior horn meniscal tears. Bilateral arthroscopic partial meniscectomy and anterior cruciate ligament reconstruction was performed. (orig.)

  4. Mutations in PTF1A cause pancreatic and cerebellar agenesis

    NARCIS (Netherlands)

    Sellick, GS; Barker, KT; Stolte-Dijkstra, [No Value; Fleischmann, C; Coleman, RJ; Garrett, C; Gloyn, AL; Edghill, EL; Hattersley, AT; Wellauer, PK; Goodwin, G; Houlston, RS

    2004-01-01

    Individuals with permanent neonatal diabetes mellitus usually present within the first three months of life and require insulin treatment(1,2). We recently identified a locus on chromosome 10p13-p12.1 involved in permanent neonatal diabetes mellitus associated with pancreatic and cerebellar agenesis

  5. Agenesis of the dorsal pancreas: a rare cause of insulin-dependent diabetes without abdominal pain: Case report.

    Science.gov (United States)

    Liang, Kaiyong; Ou, Xiaojuan; Huang, Xukai; Lan, Qunfang

    2018-03-01

    Agenesis of the dorsal pancreas is a very rare condition with an unknown pathology and etiology, although it may be associated with autosomal dominant or X-linked dominant inheritance or retinoic acid and hedgehog signaling pathway alterations. This condition usually manifests with abdominal pain or pancreatitis, although some cases are asymptomatic. Approximately 50% of affected patients with this disorder present with hyperglycemia or various other anomalies. We report the case of a 23-year-old Chinese woman who visited the Department of Endocrinology and Metabolism with insulin-dependent diabetes but no specific symptoms, signs, or other deformities. Severe diabetic retinopathy indicated a long period of hyperglycemia. Agenesis of the dorsal pancreas was observed incidentally during the common diagnosis of diabetes, and the diagnosis was established using magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance cholangiopancreatography. Following the diagnosis of diabetes, insulin replacement therapy was initiated at a dosage of up to 45 U per day. The patient's blood glucose level was monitored, and the insulin dosage was adjusted accordingly. The patient's blood glucose levels gradually normalized after insulin treatment and were subsequently maintained with intensive insulin therapy. Treatment for diabetic retinopathy was provided by the Ophthalmology Department. Agenesis of the dorsal pancreas should be considered in a young patient diagnosed with diabetes who presents with obvious diabetes-related complications (e.g., renal, retinal, or neurological) inconsistent with the course of the disease or a history of other congenital anomalies. We recommend the routine use of computed tomography or magnetic resonance imaging when examining young patients with diabetes.

  6. First-line tracheal resection and primary anastomosis for postintubation tracheal stenosis.

    Science.gov (United States)

    Elsayed, H; Mostafa, A M; Soliman, S; Shoukry, T; El-Nori, A A; El-Bawab, H Y

    2016-07-01

    Introduction Tracheal stenosis following intubation is the most common indication for tracheal resection and reconstruction. Endoscopic dilation is almost always associated with recurrence. This study investigated first-line surgical resection and anastomosis performed in fit patients presenting with postintubation tracheal stenosis. Methods Between February 2011 and November 2014, a prospective study was performed involving patients who underwent first-line tracheal resection and primary anastomosis after presenting with postintubation tracheal stenosis. Results A total of 30 patients (20 male) were operated on. The median age was 23.5 years (range: 13-77 years). Seventeen patients (56.7%) had had previous endoscopic tracheal dilation, four (13.3%) had had tracheal stents inserted prior to surgery and one (3.3%) had undergone previous tracheal resection. Nineteen patients (63.3%) had had a tracheostomy. Eight patients (26.7%) had had no previous tracheal interventions. The median time of intubation in those developing tracheal stenosis was 20.5 days (range: 0-45 days). The median length of hospital stay was 10.5 days (range: 7-21 days). The success rate for anastomoses was 96.7% (29/30). One patient needed a permanent tracheostomy. The in-hospital mortality rate was 3.3%: 1 patient died from a chest infection 21 days after surgery. There was no mortality or morbidity in the group undergoing first-line surgery for de novo tracheal lesions. Conclusions First-line tracheal resection with primary anastomosis is a safe option for the treatment of tracheal stenosis following intubation and obviates the need for repeated dilations. Endoscopic dilation should be reserved for those patients with significant co-morbidities or as a temporary measure in non-equipped centres.

  7. [Tracheobronchial and pulmonary parenchymatous congenital abnormalities requiring surgical treatment in adults].

    Science.gov (United States)

    Mordant, P; De Dominicis, F; Berna, P; Riquet, M

    2012-04-01

    Most tracheobronchial and parenchymatous congenital abnormalities of the respiratory system are diagnosed in early life. However, some lesions may be initially silent and diagnosed only in adulthood. These cases included congenital abnormalies of the tracheobronchial tract (tracheal and/or bronchial stenosis, bronchogenic cysts, bronchial atresia, oesotracheal fistula, oesobronchial fistula, and tracheal diverticulum), and lung parenchyma itself (pulmonary sequestration, congenital cystic adenomatoïd malformation, lobar emphysema, lobar or lung hypoplasia). To avoid dreadful complications, these rare cases deserve surgical management, and must be known by chest physicians and surgeons. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  8. A case of congenital unilateral absence of the vas deferens

    Directory of Open Access Journals (Sweden)

    Mo B

    2013-04-01

    Full Text Available Bi Mo,1 Vishnu Garla,2 Lawrence M Wyner1 1Department of Surgery, 2Department of Internal Medicine, Marshall University, Huntington, WV, USA Background: Congenital unilateral absence of the vas deferens occurs in 0.5%–1.0% of males. It has been associated with various genitourinary abnormalities, including renal agenesis. We report a case of congenital unilateral absence of the vas deferens found incidentally during vasectomy in a patient with known unilateral renal agenesis. Case presentation: A 24-year-old male presented to our urology clinic requesting vasectomy. His past history was significant for left renal agenesis. Following successful right vasectomy, several attempts to locate the left vas deferens were unsuccessful. We diagnosed congenital unilateral absence of the vas deferens. Follow-up semen analysis showed azoospermia. Conclusion: As vasectomies are increasingly performed in outpatient settings, it is imperative that physicians be aware of this condition, which can be recognized by a simple physical exam. Recognition could prevent unnecessary surgery and prompt providers to investigate for associated abnormalities. Keywords: vas deferens, embryology, abnormalities, surgery

  9. Triplets with growth failure, microcephaly, mental retardation, nail hypoplasia and corpus callosum agenesis: is it a variant of Coffin-Siris or a new syndrome?

    Science.gov (United States)

    Kirel, B; Kural, N; Yakut, A; Adapinar, B

    2000-01-01

    We report eight-year-old triplet girls whose clinical features included microcephaly, severe mental retardation, hypoplasia of distal phalanges of both fifth and second fingers and nail hypoplasia on second fingers, dysmorphic facial features, and partial corpus callosum agenesis. During infancy, a Pavlik harness was used for congenital hip dislocation, and they had difficulty in feeding. One had been operated for patent ductus arteriosus. To our knowledge, this rare combination has not been previously reported in triplets whose clinical features closely resemble those of Coffin-Siris syndrome. The other diagnostic possibilities are also reviewed.

  10. The therapeutic effects and experience of tracheal stent implantation in managing severe tracheal stenosis

    International Nuclear Information System (INIS)

    Lv Weifu; Zhang Xingming; Zhang Xuebing; Wang Weiyu; Hou Changlong

    2006-01-01

    Objective: To evaluate the therapeutic effects and experience of the tracheal stent implantation for the management of severe tracheal stenosis. Materials: Thirteen patients with severe tracheal stenosis of various causes underwent high kilovoltage radiography and computed tomography for evaluating the site, form and extent of the stenosis including 10 at the trachea, 1 at the right main bronchus and 2 at left main bronchus. The C2 catheter assisted with ultra-slipping guide wire was inserted into the trachea under fluoroscopy and then a replaced high shoring guide wire was pushed through the stenotic segment and retained the stent. Results: All stents were implanted successfully with successful rate 100% together with dyspnoeic improvements. The mean survival time was 6.2 months for patients with malignant neoplasm. One patient with benign tracheal stenosis has been followed-up for 5 years without restenosis. Conclusions: The tracheal stent implantation is an effective means for severe tracheal stenosis. (authors)

  11. Tracheal resection and anastomosis after traumatic tracheal stenosis in a horse.

    Science.gov (United States)

    Barnett, Timothy P; Hawkes, Claire S; Dixon, Padraic M

    2015-02-01

    To report a resection and anastomosis technique to treat trauma-induced tracheal stenosis. Case report. A 9-year-old Warmblood gelding. Endoscopy, radiography, and ultrasonography were used to diagnose a single ring tracheal stenosis; the stenotic region was resected and adjacent tracheal rings anastomosed with an end-to-end technique. The anastomosis healed completely despite formation of a unilateral partial mucosal stenosis "web," which was subsequently removed by transendoscopic laser surgery. During tracheal anastomosis, the left recurrent laryngeal nerve was damaged, causing laryngeal hemiplegia, later treated successfully by laryngoplasty. The horse returned to its previous level of work. This tracheal resection and anastomosis technique successfully provided the horse with a large tracheal lumen, and despite major complications, allowed a return to full athletic work. © Copyright 2014 by The American College of Veterinary Surgeons.

  12. Tracheal resection and anastomosis in dogs.

    Science.gov (United States)

    Lau, R E; Schwartz, A; Buergelt, C D

    1980-01-15

    Resection and end-to-end anastomosis of the trachea is a practical procedure for the correction of various forms of tracheal stenosis. Preplacing retention sutures facilitates manipulation of the trachea and rapid apposition of the tracheal ends. These same sutures then relieve tension on the primary suture line, assuring early epithelialization. Two dogs with tracheal stenosis were treated by use of this technique. Slight narrowing of the trachea was evident postoperatively in both dogs, but neither dyspnea nor coughing occurred during the follow-up period.

  13. Laryngeal morbidity after tracheal intubation

    DEFF Research Database (Denmark)

    Sørensen, M K; Rasmussen, N; Kristensen, M S

    2013-01-01

    BACKGROUND: Tracheal intubation may cause vocal fold damage. The trial was designed to assess laryngeal morbidity comparing the Endoflex(®) tube with a conventional endotracheal tube with stylet. We hypothesised that laryngeal morbidity within the first 24 h after extubation would be lower...... with the Endoflex tube than with the conventional endotracheal tube with stylet because of less rigidity. METHODS: This randomised trial included 130 elective surgical patients scheduled for general anaesthesia with endotracheal intubation. Pre- and post-operative assessment of hoarseness, vocal fold pathology......% with the Endoflex tube and 55% with the endotracheal tube with stylet at 24 h after extubation (P = 0.44). Post-operative vocal fold injury was present in 23% in the Endoflex tube group and in 36% in the endotracheal tube with stylet group (P = 0.13). The increase in shimmer, the voice analysis variable reflecting...

  14. Nitinol memmo-alloy stents for benign and malignant tracheal stensis

    International Nuclear Information System (INIS)

    Wang Zhongmin; Wu Chaoxian; Gong Ju; Zheng Yunfeng; Gu Xuezhang

    2005-01-01

    Objective: To observe the therapeutic and side effects of tracheal stents in tracheal stenosis caused by tracheal malacia and malignant tumours. Methods: Twenty-five patients treated with tracheal stents were retrospectively analyzed. Results: Tracheal stents have been implanted successfully in twenty-five patients through fiberbronchoscope or tracheal intubation, and dyspnoea improved greatly after the procedure. Conclusions: Tracheal stents can relieve symptoms and improve the life quality in patients with tracheal stenosis caused by malignant tumors or tracheal malacia. (authors)

  15. Dental anomalies in an orthodontic patient population with maxillary lateral incisor agenesis.

    Science.gov (United States)

    Citak, Mehmet; Cakici, Elif Bahar; Benkli, Yasin Atakan; Cakici, Fatih; Bektas, Bircan; Buyuk, Suleyman Kutalmış

    2016-01-01

    The purpose of this study was to evaluate the prevalence of dental anomalies in a subpopulation of orthodontic patients with agenesis of maxillary lateral incisors (MLI). The material of the present study included the records of the 1964 orthodontic patients. Panoramic radiographs and dental casts were used to analyze other associated eight dental anomalies, including agenesis of other teeth, dens invaginatus, dens evaginatus, peg shaped MLI, taurodontism, pulp stone, root dilaceration and maxillary canine impaction. Out of the 1964 patients examined, 90 were found to have agenesis of MLI, representing a prevalence of 4.6%. The most commonly found associated-anomalies were agenesis of other teeth (23.3%), peg-shaped MLIs (15.6%), taurodontism (42.2%), and dilacerated teeth (18.9%). Permanent tooth agenesis, taurodontism, peg-shaped maxillary lateral incisor, and root dilacerations are frequently associated with maxillary lateral incisor agenesis.

  16. 21 CFR 878.3720 - Tracheal prosthesis.

    Science.gov (United States)

    2010-04-01

    ...) Identification. The tracheal prosthesis is a rigid, flexible, or expandable tubular device made of a silicone... of the trachea or trachealbronchial tree. It may be unbranched or contain one or two branches. The...

  17. Corpus callosum agenesis: Role of fetal magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Achour Radhouane

    2016-05-01

    Full Text Available Corpus callosum agenesis (CCA was evaluated by ultrasound examination and magnetic resonance imaging (MRI with many studies. Ultrasonography was able to suspect CCA by indirect signs but a definitive diagnosis of CCA was achieved in rare cases. MRI was able to diagnose complete CCA in majority of cases. Additional neurological abnormalities including heterotopia, gyration anomaly, asymmetry of the cerebral hemispheres, and Dandy-Walker variant were documented, as well as an ocular anomaly which was described, by MRI examination. Prenatal counseling for fetal agenesis of the corpus callosum is difficult as the prognosis is uncertain. The association with other cerebral abnormalities increases the likelihood of a poor outcome and ultrasonographic assessment of the fetal brain is limited. We found MRI to be a safe and useful additional procedure to complement ultrasonographic diagnosis or suspicion of CCA.

  18. Tooth agenesis: from molecular genetics to molecular dentistry

    Czech Academy of Sciences Publication Activity Database

    Matalová, Eva; Fleischmannová, Jana; Sharpe, P. T.; Tucker, A. S.

    2008-01-01

    Roč. 87, č. 7 (2008), s. 617-623 ISSN 0022-0345 R&D Projects: GA AV ČR KJB500450802; GA MŠk OC B23.001; GA ČR GC524/08/J032 Institutional research plan: CEZ:AV0Z50450515 Keywords : tooth agenesis Subject RIV: FF - HEENT, Dentistry Impact factor: 3.142, year: 2008

  19. and Non-Operable Tracheal Stenosis

    Directory of Open Access Journals (Sweden)

    Reza Bagheri

    2013-05-01

    Full Text Available   Introduction: Tracheal stenosis is normally caused by trauma, infection, benign and malignant tumors, prolonged intubation or tracheostomy. The best treatment for tracheal stenosis is resection and anastomosis of trachea. Yet the major surgical complication of tracheal surgery is postoperative stenosis. The goal of this paper is to study the result of tracheal stenting as a replacement therapy for patients suffering from tracheal stenosis who are not good candidates for surgery.   Materials and Methods: This study presents the results of stenting in patients with: Inoperable tumoral stenosis,Non-tumoral stenosis being complicated due to prior surgeries,Inability to undergo a major surgery.The study was performed between September 2002 and July 2011 and poly flex stents were used by means of rigid bronchoscopy. Results: A total of 25 patients received stents during this study. Among them 15 patients suffered from benign and 10 suffered from malignant tracheal stenosis. The patients were followed up for at most 12 months after the stenting operation. The mean age of the patients was 35 years. The most common cause of stenosis was prolonged intubation (75%. The most common indication for stenting was the history of multiple tracheal operations. The most common complication of stenting and cause of stent removal was formation of granulation tissue. 30% of patients with benign tracheal stenosis were cured and about 10% improved until they could stand a major operation. Ten patients in benign group and 2 patients in malignant group (20% needed T-Tube insertion after stent removal but other patientcure by stenting. Conclusions: In benign cases stenting is associated with recurrence of symptoms which requires other therapeutic techniqus, so the stenting may not be named as a final solution in benign cases. However, this technique is the only method with approved efficacy for malignant cases with indication.

  20. Post intubation tracheal stenosis in children

    Directory of Open Access Journals (Sweden)

    Marco Caruselli

    2014-12-01

    Full Text Available Many authors have reported that tracheal stenosis is a complication that can follow tracheal intubation in both adults and children. The symptoms, when they do appear, can be confused with asthma, with subsequent treatment providing only mild and inconsistent relief. We report here the case of an 8 year old girl admitted to our hospital for whooping cough that was not responding to therapy.

  1. Waardenburg syndrome with familial unilateral renal agenesis: a new syndrome variant?

    Science.gov (United States)

    Webb, Katie M; Smith, Alisha J; Dansby, Linda M; Diskin, Charles J

    2015-06-01

    A 64-year-old man with Waardenburg syndrome presented with anuria and was subsequently discovered by renal ultrasound to have unilateral renal agenesis. The patient is one of three generations with incidental finding of renal agenesis also marked by the presence of Waardenburg syndrome. To our knowledge, there has been no mention elsewhere in the scientific literature of a variant of Waardenburg syndrome with associated renal agenesis. © 2014 The Authors. Therapeutic Apheresis and Dialysis © 2014 International Society for Apheresis.

  2. A case of total agenesis of the corpus callosum

    International Nuclear Information System (INIS)

    Sakamoto, Masanobu; Takeda, Katsuhiko; Bandou, Mitsuaki; Murayama, Shigeo; Sakuta, Manabu

    1985-01-01

    We have reported a case of agenesis of the corpus callosum, in which NMR-CT revealed a complete defect of it, and have examined the localization of the speech center of this patient. The patient is a right-handed 26-year-old man who has complained of headache on the parietal region. His neurological examination revealed only a mild mental difficulty (IQ 77). X-ray CT showed the lateral ventricles to be separated widely and the posterior horns dilated, which were compatible with the agenesis of the corpus callosum. Further, NMR-CT has revealed a total agenesis of the corpus callosum. NMR-CT seems to be highly useful for the detection of the degree of the callosal defect. We have carried out the intracarotid amobarbital injection (Wada's test) for the determination of the lateralization of cerebral speech dominance. It had been reported by some authors that when it comes to the cerebral speech dominance, acallosal patients had no difference between each hemisphere. However, our results have demonstrated a left sided dominance. (author)

  3. Hypohyperdontia: Agenesis of three third molars and mandibular centrals associated with midline supernumerary tooth in mandible

    Directory of Open Access Journals (Sweden)

    Sivakumar Nuvvula

    2010-01-01

    Full Text Available Agenesis of teeth in a patient who also presents with a supernumerary tooth is one of the rare numerical anomalies in human dentition. Agenesis of third molars was shown to be associated with other missing permanent teeth. A review of literature on hypodontia including third molar agenesis, hyperdontia and a concomitant presence of these two conditions which is termed as hypohyperdontia is presented along with a case showing agenesis of three third molars, both mandibular central incisors and a midline supernumerary tooth.

  4. Patterns of third-molar agenesis and associated dental anomalies in an orthodontic population.

    Science.gov (United States)

    Celikoglu, Mevlut; Bayram, Mehmet; Nur, Metin

    2011-12-01

    The aim of this study was to investigate the frequency of dental anomalies in orthodontic patients with different patterns of third-molar agenesis, comparing them with patients without third-molar agenesis. A sample of 374 patients with agenesis of at least 1 third molar was divided into 4 groups according to the third-molar agenesis pattern, and a control group of 98 patients without third-molar agenesis was randomly selected from the patient archives. Panoramic radiographs and cast models were used to determine the associated dental anomalies, such as hypodontia, hyperdontia, impaction, dilaceration, microdontia, ectopic eruption, transposition, and transmigration. The Pearson chi-square and Fisher exact tests were used to determine the differences in the distribution of the associated dental anomalies among the groups. The prevalence of agenesis of other teeth (11.2%, n = 42) was significantly greater in our study sample (groups 1-4) than in the control group (group 5) (4.1%, n = 4; P dental anomalies in patients with agenesis of 3 and 4 third molars compared with the control group. Permanent tooth agenesis, microdontia of maxillary lateral incisors, and total dental anomalies are more frequently associated with agenesis of 4 third molars than with the presence of third molars. Copyright © 2011 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.

  5. The role of Msx1 and Pax9 in pathogenetic mechanisms of tooth agenesis

    Directory of Open Access Journals (Sweden)

    Yani Corvianindya Rahayu

    2009-09-01

    Full Text Available Background: Tooth agenesis is one of the most common developmental anomalies in human, which one or a few teeth are absent because they have never formed, may cause cosmetic or occlusal harm, while severe agenesis which are relatively rare require clinical attention to support and maintain the dental function. Molecular studies have demonstrated that tooth development is under strict genetic control. Purpose: This article want to review the genetic regulating that are responsible for tooth agenesis especially the role of Msx1 and Pax9 in pathogenetic mechanisms of tooth agenesis. Review: Tooth agenesis is a consequence of a qualitatively or quantitatively impaired function of genetic networks, which regulate tooth development. Mutations in Msx1 and Pax9 genes are dominant for tooth agenesis in humans. The Pax9 gene, which codes for a paired domain-containing transcription factor that plays an essential role in the development of mammal dentition, has been associated with selective tooth agenesis in humans and mice. Conclusion: Reduced amount of functional Msx1 or Pax9 protein in the tooth forming cells is able to cause severe and selective tooth agenesis. There are differences in the frequency of agenesis of specific teeth associated with the defects in Msx1 and defects in Pax9.

  6. Congenital uronephropathy pattern in children

    Directory of Open Access Journals (Sweden)

    Husein Alatas

    2001-10-01

    Full Text Available To obtain the basic data of congenital uronephropathy pattern and the affecting factors in children, we conducted a cross-sectional study at the Department of Child Health Cipto Mangunkusumo (CM Hospital Jakarta from 1995 to 1999 and 9 teaching hospitals throughout Indonesia. During the study period 134 patients were obtained, 116 patients from the CM Hospital and 18 patients from other teaching hospitals. Most patients (48.8% were below 1 year of age; male were affected more than female (2.4:1. The disorder was classified into two groups, i.e., congenital nephropathy and uropathy. There were 10 children with nephropathy, i.e., 4 with unilateral renal hypoplasia, 3 with polycystic kidney, and 3 with renal agenesis. In the uropathy group, 43 were with hypospadia, 22 with primary reflux vesicoureter, 18 with neurogenic bladder, and 17 with ureteropelvic junction obstruction. The complications found were urinary tract infection (71.2%, chronic renal failure (15.7%, hypertension (3.7%, and acute renal failure (1.5%. Consanguinity, familial disorders, maternal diseases, x-ray exposure and abortion efforts were found in a small proportion of patients. History of drug or herbs use in the first trimester of pregnancy was found in a large proportion of patients, mostly took analgesics (especially acetaminophen. In conclusion, uropathy disorders were much more common than congenital nephropathy. The most common complication was urinary tract infection, followed by chronic renal failure, hypertension, and acute renal failure.

  7. Congenital Hypothyroidism

    Science.gov (United States)

    ... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  8. Effects of successive tracheal resection and anastomosis on tracheal diameter and position of lobar bronchi in dogs.

    Science.gov (United States)

    de Mello Souza, Carlos Henrique; Reinero, Carol R

    2016-06-01

    OBJECTIVE To evaluate the effects of successive large-segment tracheal resection and anastomosis on tracheal diameter and position of lobar bronchi in dogs. ANIMALS 5 adult Beagles. PROCEDURES Right lateral radiographs were obtained for all dogs and used to measure tracheal length. Dogs were then euthanized, and successive segmental tracheal resections (intervals of 10% from 20% to 50% of the tracheal length), each of which was followed by anastomosis, were performed in each cadaver. Tracheobronchoscopy was performed before the first tracheal resection and after each of the anastomoses to evaluate tracheal diameter and changes in position of lobar bronchi. RESULTS Tracheal diameter was minimally affected by resections up to 50% of the tracheal length. Diameter of the trachea and position of bronchi were not affected by resection of 20% of the tracheal length. Changes in the position of various lobar bronchi were detected after resection of 30% of the tracheal length. CONCLUSIONS AND CLINCIAL RELEVANCE In this study, tracheal resections of 20% of the tracheal length were accommodated, possibly as a result of stretching of the annular ligament. Resections of ≥ 30% of the tracheal length altered the position of lobar bronchi. Clinical effects, if any, attributable to these changes in bronchial position remain to be elucidated.

  9. Indications and interventional options for non-resectable tracheal stenosis

    Science.gov (United States)

    Bacon, Jenny Louise; Patterson, Caroline Marie

    2014-01-01

    Non-specific presentation and normal examination findings in early disease often result in tracheal obstruction being overlooked as a diagnosis until patients present acutely. Once diagnosed, surgical options should be considered, but often patient co-morbidity necessitates other interventional options. Non-resectable tracheal stenosis can be successfully managed by interventional bronchoscopy, with therapeutic options including airway dilatation, local tissue destruction and airway stenting. There are common aspects to the management of tracheal obstruction, tracheomalacia and tracheal fistulae. This paper reviews the pathogenesis, presentation, investigation and management of tracheal disease, with a focus on tracheal obstruction and the role of endotracheal intervention in management. PMID:24624290

  10. Congenital Malformations Associated with Maternal Diabetes

    Directory of Open Access Journals (Sweden)

    Chih-Ping Chen

    2005-03-01

    Full Text Available Maternal diabetes has toxic effects on the development of the embryo and significantly increases the risk of congenital malformations in humans. The incidence of fetal structural defects caused by maternal pregestational diabetes is three- to fourfold higher than that caused by non-diabetic pregnancy. The congenital malformations associated with diabetic pregnancy arise before the seventh gestational week. Diabetic embryopathy can affect any developing organ system, including the central nervous system (CNS (anencephaly, spina bifida, microcephaly, and holoprosencephaly, skeletal system (caudal regression syndrome, sacral agenesis, and limb defects, renal system (renal agenesis, hydronephrosis, and ureteric abnormalities, cardiovascular system (transposition of the great vessels, ventricular septal defects, atrial septal defects, coarctation of the aorta, cardiomyopathy, and single umbilical artery, and gastrointestinal system (duodenal atresia, anorectal atresia, and small left colon syndrome. Pregnant women with fetuses with diabetic embryopathy may have chronic or unrecognized hyperglycemia and elevated levels of glycerated hemoglobin. This review emphasizes the necessity to consider hyperglycemia-induced teratogenesis during genetic counseling of parents with prenatally detected fetal malformations. Successful preconception counseling for women with diabetes mellitus and metabolic control will reduce birth defects and maternal morbidity.

  11. [Segmental tracheal resection and anastomosis for the treatment of cicatricial stenosis in cervical tracheal].

    Science.gov (United States)

    Cui, P C; Luo, J S; Liu, Z; Bian, K; Guo, Z H; Ma, R N

    2016-02-01

    To evaluate the efficacy of segmental tracheal resection with end-to-end anastomosis for cicatricial cervical tracheal stenosis. The clinical outcomes of 40 patients treated with tracheal resection were retrospectively reviewed. There were 28 male patients and 12 female patients with the age ranged from 6 to 64 years (mean 33.7 years). The degree of stenosis was classified according to Myer-Cotton classification as follows: grade Ⅱ (n=7), grade Ⅲ (n=22) and grade Ⅳ (n=11). The stenosis extension ranged from 1.0 to 4.3 cm (mean 2.5 cm). The causes of the stenosis were postintubation (n=33), cervical trauma (n=6) and resection of tracheal neoplasm (n=1). Thirty-four(85.0%) patients were decannulated and 6 failed. Of the 6 patients failed, 4 were decannulated after reoperation with the sternohyoid myocutaneous flap or thyroid alar cartilage graft. Complications occurred in 10 patients. In 8 patients granulation tissues formed at the site of the tracheal anastomosis, which needed endoscopic resction, and in 2 patients anastomosic dehiscence occurred. No injury to recurrent laryngeal nerve or trachoesophageal fistula occurred. Segmental tracheal resection with end-to-end anastomosis is an effective surgical method for tracheal stenosis, which has a higher successful rate for primary operation and shorter therapeutic period.

  12. Does asymptomatic septal agenesis exist? A review of 34 cases

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Ouardia; Andre, Christine; Kalifa, Gabriel; Adamsbaum, Catherine [St Vincent de Paul Hospital, Radiology Department, Paris (France)

    2005-04-01

    Primary septal agenesis (PSA) is a rare brain malformation that can be isolated or part of developmental brain abnormalities (holoprosencephaly, septo-optic dysplasia or cortical malformation). Such associated malformation can be subtle, leading to difficulties in the prenatal management of PSA. Moreover, the neurological prognosis of isolated PSA remains debatable. The aims of the study were to specify the patterns and frequency of brain malformations associated with septal agenesis (SA), to identify the clinical prognosis, and to discuss the aetiology of PSA with the new insights provided by molecular genetics. The study consisted of a 14-year retrospective review of brain MRI in 34 patients having PSA (mean age, 5 years). Chiasm and optic nerves were not evaluated. Post-hydrocephalus SA or incomplete data were excluded. The clinical data were correlated to the MRI patterns. The study disclosed 82.5% associated lesions with MRI (28/34): 11 neuronal migration disorders, 9 holoprosencephalies (HP), 7 pituitary stalk interruptions, 1 corpus callosum partial agenesis; 17.5% (6/34) of cases were apparently isolated PAS. Clinically, the patients had motor dysfunction in 68% (23/34), mental retardation in 65% (22/34), blindness in 24% (8/34), endocrinological defects in 21% (7/34) and epilepsy in 18% (6/34) of cases. Nine percent of patients (3/34) were neurologically normal (including one with scoliosis and two infants younger than 2 years at the last follow-up). Patients with bilateral cortical anomalies and HP (even if mild) had the worst neurological prognosis. A severe motor impairment was present without evidence of hemispheric anomaly in 12% of patients (4/34). Interestingly, the frontal lobes were involved in 90% of cortical anomalies and HP, supporting the malformative aetiology of PSA. PSA rarely appears isolated and severe psychomotor impairment may occur in apparently isolated forms. These unfavourable results should be highlighted and need to be confirmed

  13. Bilateral multicystic renal dysplasia with potter sequence: A case with penile agenesis

    International Nuclear Information System (INIS)

    Dursun, Ahmet; Ermis, Bahri; Bahadir, Burak; Seckiner, Ilker

    2006-01-01

    Hereditary renal adysplasia (HRA) is rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low set ears and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thin skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association and Coloboma, heart anomaly, choanal atresia, retardation, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. (author)

  14. Prevalence of Third Molar Agenesis: Associated Dental Anomalies in Non-Syndromic 5923 Patients.

    Science.gov (United States)

    Sujon, Mamun Khan; Alam, Mohammad Khursheed; Rahman, Shaifulizan Abdul

    2016-01-01

    The aim of this study was to investigate the prevalence of third molar agenesis and other associated dental anomalies in Bangladeshi population and to investigate the relationship of other dental anomalies with the third molar presence/agenesis. A retrospective study was performed using panoramic radiographs of 5923 patients, who ranged in age from 10 to 50 years. All radiographs were analyzed by Planmeca Romexis® 3.0 software (Planmeca Oy, Helsinki, Finland). Pearson chi-square and one way ANOVA (Post Hoc) test were conducted. The prevalence of third molar agenesis was 38.4%. The frequency of third molar agenesis was significantly higher in females than males (p dental anomalies was 6.5%, among them hypodontia was 3.1%. Prevalence of third molar agenesis varies in different geographic region. Among the other dental anomalies hypodontia was more prevalent.

  15. Agenesis of the dorsal mesentery presenting in an adolescent

    Directory of Open Access Journals (Sweden)

    Anith Chacko

    2013-03-01

    Full Text Available Agenesis of the dorsal mesentery is a rare occurrence that usually presents in children. It is associated with proximal small bowel malrotation as well as high jejunal atresia with discontinuity of the small bowel. We present a case report of an adolescent presenting with clinical features of proximal small bowel obstruction (confirmed on imaging as well as acute pancreatitis. At laparotomy, he was found to have no dorsal mesentery, without small bowel atresia, and the duodenum was fixed to the posterior abdominal wall. The patient recovered well and remained symptom-free.

  16. Scintigraphic evaluation of congenital anomalies of the thyroid gland

    International Nuclear Information System (INIS)

    Paul, A. K.; Hassan, M.A.; Miah, M.S.R.

    2001-01-01

    Total 2287 patients who undergone thyroid scintigraphy in Nuclear Medicine Centre, Khulna from January 1998 to December 2000 were retrospectively studied to evaluate the congenital anomalies of the thyroid gland. Scintigraphy showed thyroid anomalies in 11 patients (0.48%). The presenting features and thyroid function tests were analyzed and a detailed study was performed. Of these 11 cases, 7 patients (63.6%) had ectopic thyroid gland and site of ectopic was found to be lingual in 4 cases, sublingual in 2 cases and pre laryngeal in 1 case, 3 patients (27.3%) with hemi agenesis of thyroid and 1 patient (9.1%) with athyreosis. Biochemically 3 cases were hypothyroid of which 2 had ectopic thyroid and 1 with athyreosis. 1 patient was hyperthyroid with hemi agenesis of thyroid and 7 were euthyroid. Females were more affected than males, the ratio being 4.5:1. (authors) 2 tabs. 12 refs

  17. Multivariate analysis of factors affecting presence and/or agenesis of third molar tooth.

    Directory of Open Access Journals (Sweden)

    Mohammad Khursheed Alam

    Full Text Available To investigate the presence and/or agenesis of third molar (M3 tooth germs in orthodontics patients in Malaysian Malay and Chinese population and evaluate the relationship between presence and/or agenesis of M3 with different skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. Pretreatment records of 300 orthodontic patients (140 males and 160 females, 219 Malaysian Malay and 81 Chinese, average age was 16.27±4.59 were used. Third-molar agenesis was calculated with respect to race, genders, number of missing teeth, jaws, skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. The Pearson chi-square test and ANOVA was performed to determine potential differences. Associations between various factors and M3 presence/agenesis groups were assessed using logistic regression analysis. The percentages of subjects with 1 or more M3 agenesis were 30%, 33% and 31% in the Malaysian Malay, Chinese and total population, respectively. Overall prevalence of M3 agenesis in male and female was equal (P>0.05. The frequency of the agenesis of M3s is greater in maxilla as well in the right side (P>0.05. The prevalence of M3 agenesis in those with a Class III and Class II malocclusion was relatively higher in Malaysian Malay and Malaysian Chinese population respectively. Using stepwise regression analyses, significant associations were found between Mx (P<0.05 and ANB (P<0.05 and M3 agenesis. This multivariate analysis suggested that Mx and ANB were significantly correlated with the M3 presence/agenesis.

  18. The Prevalence of Palmaris longus agenesis among the Ghanaian population

    Directory of Open Access Journals (Sweden)

    Osonuga A

    2012-10-01

    Full Text Available Objective: Background: Studies have documented the agenesis of Palmaris longus muscle in different populations but none has included the Ghanaian population. Methods: The study involved 226 subjects (130 females and 96 males who are students of the University of Cape Coast, Cape Coast, Ghana. The presence of the PLM was clinically determined using the Schaeffer’s test. Subjects in which the tendon was not visualized or palpable, two other tests were performed to confirm the absence. Results: The total prevalence of absence of PLM was 3.1%; absence on the left hand was commoner than on the right hand. The frequency of PLM absence was also slightly higher in females than in males representing 1.8% and 1.3% respectively. One female had the PLM absent bilaterally whiles a male subject had a trifid tendon on the right forearm with a bifid on the left. Conclusion: The prevalence of PLM agenesis in the Ghanaian population is lower compared to values coated in standard textbook on surgery. PLM is not diminishing as fast as observed in some population hence it is can be readily used as donor tendon by Ghanaian surgeons.

  19. [Cervical tracheal resection with primary anastomosis for benign tracheal stricture in adult].

    Science.gov (United States)

    Ye, Jin; Hu, Yan-Ming; Liu, Hui; Li, Jing-Jia; Wang, Zhi-Yuan; Li, Yuan

    2013-07-01

    To introduce the outcomes of tracheal resection with primary end to end anastomosis for benign cervical tracheal stenosis, and to discuss the strategy for prevention of surgical complications. A retrospective analysis was performed in 12 patients diagnosed as benign cervical tracheal stenosis from October 2009 to June 2012. Laryngo-tracheal endoscopic examination and computed tomography (CT) were used to assess the degree of stenosis, the grade of inflammation and edema of the subglottis and trachea, and the extent of stenosis and the remaining linear amount of normal airway. The Meyer and Cotton grading system was used to categorise the clinical severity of the stenoses. All patients underwent tracheal resection with primary end to end anastomosis. The length of cervical tracheal stenosis ranged from 2.3 to 4.1 cm. Grade II stenosis was present in three patients, Grade III stenosis was present in seven patients and grade IV stenosis in two patients. Successful extubation was achieved in all 12 cases. After surgery, temporary hoarseness occurred in 1 patient (8.3%); unilateral pulmonary atelectasis with pleural effusion occurred in 1 patient (8.3%); subcutaneous emphysema with infection occurred in 1 patient (8.3%); mild dysphagia occurred in 3 patients (25.0%); a slight deepening of the tone of voice occurred in 5 patients (41.7%), granulation tissue growth near the suture occurred in 3 patients (25.0%), and suture dehiscence did not occur in any patient. The follow-up period ranged from 6 months to 38 months, no patient developed restenosis. It presents a high success rate and good functional result of tracheal resection with primary end-to-end anastomosis. Therefore, it is an effective and reliable approach for the management of benign cervical tracheal stenosis. To avoid complications, the preoperative assessment, patients selection and postoperative management should be emphasized.

  20. Viscoelastic Properties of Human Tracheal Tissues.

    Science.gov (United States)

    Safshekan, Farzaneh; Tafazzoli-Shadpour, Mohammad; Abdouss, Majid; Shadmehr, Mohammad B

    2017-01-01

    The physiological performance of trachea is highly dependent on its mechanical behavior, and therefore, the mechanical properties of its components. Mechanical characterization of trachea is key to succeed in new treatments such as tissue engineering, which requires the utilization of scaffolds which are mechanically compatible with the native human trachea. In this study, after isolating human trachea samples from brain-dead cases and proper storage, we assessed the viscoelastic properties of tracheal cartilage, smooth muscle, and connective tissue based on stress relaxation tests (at 5% and 10% strains for cartilage and 20%, 30%, and 40% for smooth muscle and connective tissue). After investigation of viscoelastic linearity, constitutive models including Prony series for linear viscoelasticity and quasi-linear viscoelastic, modified superposition, and Schapery models for nonlinear viscoelasticity were fitted to the experimental data to find the best model for each tissue. We also investigated the effect of age on the viscoelastic behavior of tracheal tissues. Based on the results, all three tissues exhibited a (nonsignificant) decrease in relaxation rate with increasing the strain, indicating viscoelastic nonlinearity which was most evident for cartilage and with the least effect for connective tissue. The three-term Prony model was selected for describing the linear viscoelasticity. Among different models, the modified superposition model was best able to capture the relaxation behavior of the three tracheal components. We observed a general (but not significant) stiffening of tracheal cartilage and connective tissue with aging. No change in the stress relaxation percentage with aging was observed. The results of this study may be useful in the design and fabrication of tracheal tissue engineering scaffolds.

  1. Congenital tuberculosis

    African Journals Online (AJOL)

    Prof Ezechukwu

    2012-06-20

    Jun 20, 2012 ... Key words: Congenital tuberculo- sis, case report, miliary tuberculosis. Introduction. Congenital tuberculosis defines tuberculosis in infants of .... tary TB and otitis media, resulting in seizures, deafness, and death. It is therefore not surprising that the index case who presented at twelve weeks of age, had ...

  2. Congenital Abnormalities

    Science.gov (United States)

    ... tube defects. However, there is also a genetic influence to this type of congenital anomaly. Unknown Causes The vast majority of congenital abnormalities have no known cause. This is particularly troubling for parents who plan to have more children, because there is no way to predict if ...

  3. Massive aspiration past the tracheal tube cuff caused by closed tracheal suction system.

    Science.gov (United States)

    Dave, Mital H; Frotzler, Angela; Madjdpour, Caveh; Koepfer, Nelly; Weiss, Markus

    2011-01-01

    Aspiration past the tracheal tube cuff has been recognized to be a risk factor for the development of ventilator-associated pneumonia (VAP). This study investigated the effect of closed tracheal suctioning on aspiration of fluid past the tracheal tube cuff in an in vitro benchtop model. High-volume low pressure tube cuffs of 7.5 mm internal diameter (ID) were placed in a 22 mm ID artificial trachea connected to a test lung. Positive pressure ventilation (PPV) with 15 cm H₂O peak inspiratory pressure and 5 cm H₂O positive end-expiratory pressure (PEEP) was used. A closed tracheal suction system (CTSS) catheter (size 14Fr) was attached to the tracheal tube and suction was performed for 5, 10, 15, or 20 seconds under 200 or 300 cm H₂O suction pressures. Amount of fluid (mL) aspirated along the tube cuff and the airway pressure changes were recorded for each suction procedure. Fluid aspiration during different suction conditions was compared using Kruskal-Wallis and Mann-Whitney test (Bonferroni correction [α = .01]). During 10, 15, and 20 seconds suction, airway pressure consistently dropped down to -8 to -13 cm H₂O (P aspiration was never observed under PPV + PEEP but occurred always during suctioning. Aspiration along the tube cuff was higher with -300 cm H₂O than with -200 cm H₂O suction pressure (P aspiration of fluid occurs along the tracheal tube cuff during suction with the closed tracheal suction system. © SAGE Publications 2011.

  4. [The cause and efficacy of benign tracheal stenosis].

    Science.gov (United States)

    Su, Zhu-quan; Wei, Xiao-qun; Zhong, Chang-hao; Chen, Xiao-bo; Luo, Wei-zhan; Guo, Wen-liang; Wang, Ying-zhi; Li, Shi-yue

    2013-09-01

    To analysis the causes of benign tracheal stenosis and evaluate the curative effect of intraluminal bronchoscopic treatment. 158 patients with benign tracheal stenosis in our hospital from September 2005 to September 2012 were collected to retrospectively analysis the causes and clinic features of tracheal stenosis. Interventional treatments through bronchoscopy were used to treat the benign tracheal stenosis and the curative effects were evaluated. 158 cases of benign tracheal stenosis were recruited to our study, 69.6% of them were young and middle-aged. The main causes of benign tracheal stenosis were as follows: secondary to postintubation or tracheotomy in 61.4% (97/158), tuberculosis in 16% (26/158), benign tumor in 5.1% (8/158) and other 27 cases. 94.3% patients improved in symptoms with alleviation immediately after bronchoscopic treatment, the average tracheal diameter increased form (4.22 ± 2.06) mm to (10.16 ± 2.99) mm (t = 21.48, P benign tracheal stenosis were increasing year by year. The most common cause of benign tracheal stenosis was postintubation and tracheotomy. Interventional treatments through bronchoscopy is effective in treating benign tracheal stenosis, but repeated interventional procedures may be required to maintain the favorable long-term effects.

  5. Agenesis of dorsal pancreas with eventration of diaphragm and intrapancreatic pseudocyst: a rare entity

    Directory of Open Access Journals (Sweden)

    Poras Chaudhary

    2013-02-01

    Full Text Available A case of acute pancreatitis in a 43-year-old male patient, which was eventually diagnosed as agenesis of dorsal pancreas with eventration of left hemidiaphragm and intrapancreatic pseudocyst is being reported.

  6. Pancreas and gallbladder agenesis in a newborn with semilobar holoprosencephaly, a case report.

    Science.gov (United States)

    Hilbrands, Robert; Keymolen, Kathelijn; Michotte, Alex; Marichal, Miriam; Cools, Filip; Goossens, Anieta; Veld, Peter In't; De Schepper, Jean; Hattersley, Andrew; Heimberg, Harry

    2017-05-19

    Pancreatic agenesis is an extremely rare cause of neonatal diabetes mellitus and has enabled the discovery of several key transcription factors essential for normal pancreas and beta cell development. We report a case of a Caucasian female with complete pancreatic agenesis occurring together with semilobar holoprosencephaly (HPE), a more common brain developmental disorder. Clinical findings were later confirmed by autopsy, which also identified agenesis of the gallbladder. Although the sequences of a selected set of genes related to pancreas agenesis or HPE were wild-type, the patient's phenotype suggests a genetic defect that emerges early in embryonic development of brain, gallbladder and pancreas. Developmental defects of the pancreas and brain can occur together. Identifying the genetic defect may identify a novel key regulator in beta cell development.

  7. Radiation response of perfused tracheal sections

    International Nuclear Information System (INIS)

    Ford, J.R.; Maslowski, A.J.; Braby, L.A.

    2003-01-01

    Full text: A model of respiratory tissue using a perfusion culture system is being developed. We are using this system to quantify the effects of normal tissue architecture and the interaction of epithelial cells with other cell types on radiation-induced bystander effects. Tracheal tissue taken from young adult male Fischer 344 rats is imbedded in a growth factor enriched agarose matrix. The chamber is designed to allow growth medium to periodically wash the epithelial surface of the tracheal lumen while maintaining the air-interface that is necessary for the normal differentiation of the epithelium. In preliminary experiments with rat trachea we have shown that a differentiated epithelial lining can be maintained for several days. Cells can be obtained for a number of different cell culture assays for endpoints such as survival and preneoplastic transformation after irradiation

  8. Middle fossa arachnoid cyst with temporal lobe agenesis accompanying isodense subdural hematoma -a case report-

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Soo; Choi, Hyung Sik; Kim, Myung Joon; Yang, Seoung Oh; Kim, Chang Jin [Capital Armed Forces General Hospital, Seongnam (Korea, Republic of)

    1987-12-15

    Cysts overlying the temporal lobes have been well described in literature. These are often associated with agenesis of the temporal lobes, and of major neurosurgical interest due to their frequent association with subdural hematoma, a combination that is rarely seen with cysts in other regions. Full features of plain, angiographic, and CT findings of arachnoid cyst with temporal lobe agenesis accompanying isodense subdural hematoma are presented, being very rare in radiologic literature.

  9. Agenesis of premolar associated with submerged primary molar and a supernumerary premolar: An unusual case report

    Directory of Open Access Journals (Sweden)

    S. V. S. G. Nirmala

    2012-01-01

    Full Text Available The combination of submerged primary molar, agenesis of permanent successor with a supernumerary in the same place is very rare. The purpose of this article is to report a case of submerged mandibular left second primary molar with supernumerary tooth in the same region along with agenesis of second premolar in an 11-year-old girl, its possible etiological factors, and a brief discussion on treatment options.

  10. Heterotaxy syndrome with associated agenesis of dorsal pancreas and polysplenia: A case report

    Directory of Open Access Journals (Sweden)

    Syed Althaf Ali1

    2015-01-01

    Full Text Available Heterotaxy syndrome is a rare embryological disorder comprising of polysplenia, partial agenesis of dorsal pancreas, malrotation of gut, cardiac and vascular anomalies resulting from failure of development of the usual left–right asymmetry of organs. We report a rare case of heterotaxy syndrome with polysplenia, partial agenesis of dorsal pancreas and malrotation of gut in a 28 year female presenting with subacute intestinal obstruction along with imaging illustrations, brief discussion and thorough review of literature.

  11. Tracheal Constriction In A Growing Dog

    OpenAIRE

    DeArmond, Christina; Niimura del Barrio, Maria Chie; Rosati, Tomasso; McAllister, Hester; Ryan, John

    2017-01-01

    A six-month-old castrated male German Shepherd/Husky cross was presented for investigation of a progressive dyspnoea and regurgitation. A circumferential cervical wound had been identified and closed following debridement by the primary care veterinarian when the dog was three months old. There was an extraluminal tracheal compression on radiographs. Surgical exploration revealed a plastic band tied and embedded in the dorsal musculature of the neck. The band had been increasingly constrictin...

  12. Recurred Post-intubation Tracheal Stenosis Treated with Bronchoscopic Cryotherapy

    Science.gov (United States)

    Jung, Ye-Ryung; Taek Jeong, Joon; Kyu Lee, Myoung; Kim, Sang-Ha; Joong Yong, Suk; Jeong Lee, Seok; Lee, Won-Yeon

    2016-01-01

    Post-intubation tracheal stenosis accounts for the greatest proportion of whole-cause tracheal stenosis. Treatment of post-intubation tracheal stenosis requires a multidisciplinary approach. Surgery or an endoscopic procedure can be used, depending on the type of stenosis. However, the efficacy of cryotherapy in post-intubation tracheal stenosis has not been validated. Here, we report a case of recurring post-intubation tracheal stenosis successfully treated with bronchoscopic cryotherapy that had previously been treated with surgery. In this case, cryotherapy was effective in treating web-like fibrous stenosis, without requiring more surgery. Cryotherapy can be considered as an alternative or primary treatment for post-intubation tracheal stenosis. PMID:27853078

  13. Congenital rubella

    Science.gov (United States)

    ... that usually closes shortly after birth remains open ( patent ductus arteriosus ) Narrowing of the large artery that ... prior to pregnancy can prevent congenital rubella. Pregnant women who have not had the vaccine should avoid ...

  14. Tooth agenesis and craniofacial morphology in pre-orthodontic children with and without morphological deviations in the upper cervical spine

    DEFF Research Database (Denmark)

    Jasemi, Ashkan; Sonnesen, Liselotte

    2016-01-01

    AIM: To analyze differences in prevalence and pattern of tooth agenesis and craniofacial morphology between non syndromic children with tooth agenesis with and without upper cervical spine morphological deviations and to analyze associations between craniofacial morphology and tooth agenesis...... in the two groups together. METHODS: One hundred and twenty-six pre-orthodontic children with tooth agenesis were divided into two groups with (19 children, mean age 11.9) and without (107 children, mean age 11.4) upper spine morphological deviations. Visual assessment of upper spine morphology...... and measurements of craniofacial morphology were performed on lateral cephalograms. Tooth agenesis was evaluated from orthopantomograms. RESULTS: No significant differences in tooth agenesis and craniofacial morphology were found between children with and without upper spine morphological deviations (2.2 ± 1.6 vs...

  15. Airflow in Tracheobronchial Tree of Subjects with Tracheal Bronchus Simulated Using CT Image Based Models and CFD Method.

    Science.gov (United States)

    Qi, Shouliang; Zhang, Baihua; Yue, Yong; Shen, Jing; Teng, Yueyang; Qian, Wei; Wu, Jianlin

    2018-03-01

    Tracheal Bronchus (TB) is a rare congenital anomaly characterized by the presence of an abnormal bronchus originating from the trachea or main bronchi and directed toward the upper lobe. The airflow pattern in tracheobronchial trees of TB subjects is critical, but has not been systemically studied. This study proposes to simulate the airflow using CT image based models and the computational fluid dynamics (CFD) method. Six TB subjects and three health controls (HC) are included. After the geometric model of tracheobronchial tree is extracted from CT images, the spatial distribution of velocity, wall pressure, wall shear stress (WSS) is obtained through CFD simulation, and the lobar distribution of air, flow pattern and global pressure drop are investigated. Compared with HC subjects, the main bronchus angle of TB subjects and the variation of volume are large, while the cross-sectional growth rate is small. High airflow velocity, wall pressure, and WSS are observed locally at the tracheal bronchus, but the global patterns of these measures are still similar to those of HC. The ratio of airflow into the tracheal bronchus accounts for 6.6-15.6% of the inhaled airflow, decreasing the ratio to the right upper lobe from 15.7-21.4% (HC) to 4.9-13.6%. The air into tracheal bronchus originates from the right dorsal near-wall region of the trachea. Tracheal bronchus does not change the global pressure drop which is dependent on multiple variables. Though the tracheobronchial trees of TB subjects present individualized features, several commonalities on the structural and airflow characteristics can be revealed. The observed local alternations might provide new insight into the reason of recurrent local infections, cough and acute respiratory distress related to TB.

  16. MR findings of congenital anorectal malformation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yoo Kyung; Kim, Hyae Young; Kwag, Hyon Joo; Chung, Eun Chul; Lee, Jung Sik; Suh, Jeong Soo [Ewha Womens University, medical College, Seoul (Korea, Republic of)

    1995-05-15

    To assess the usefulness of MRI in preoperative diagnosis of congenital anorectal malformation. MR findings of 11 cases with surgically proved anorectal malformations were retrospectively reviewed and compared with operative findings, according to the level of atresia, the development of sphincter muscle, fistula and associated anomalies of other organs. Four of 11 cases were low type of anorectal atresia, 3 cases were intermediate type, and 3 cases were high type. There was one case of Currarino triad with low type of anorectal stenosis. MRI demonstrated the levels of atresia correctly in all cases and revealed fistulas in all high type of anomalies. Degrees of the development of the sphincter muscles were good in all cases of low types and fair in a case of intermediate type and an anorectal stenosis, whereas the development was poor in 2 cases of intermediate type and all 4 cases of high type. The associated anomalies in anorectal malformation were renal agenesis, congenital hip dysplasia and sacral defect with presacral teratoma in Currarino triad. MRI was a simple and useful study to confirm the level of atresia, fistula and associated anomalies in the diagnosis of the congenital anorectal malformation.

  17. MR findings of congenital anorectal malformation

    International Nuclear Information System (INIS)

    Kim, Yoo Kyung; Kim, Hyae Young; Kwag, Hyon Joo; Chung, Eun Chul; Lee, Jung Sik; Suh, Jeong Soo

    1995-01-01

    To assess the usefulness of MRI in preoperative diagnosis of congenital anorectal malformation. MR findings of 11 cases with surgically proved anorectal malformations were retrospectively reviewed and compared with operative findings, according to the level of atresia, the development of sphincter muscle, fistula and associated anomalies of other organs. Four of 11 cases were low type of anorectal atresia, 3 cases were intermediate type, and 3 cases were high type. There was one case of Currarino triad with low type of anorectal stenosis. MRI demonstrated the levels of atresia correctly in all cases and revealed fistulas in all high type of anomalies. Degrees of the development of the sphincter muscles were good in all cases of low types and fair in a case of intermediate type and an anorectal stenosis, whereas the development was poor in 2 cases of intermediate type and all 4 cases of high type. The associated anomalies in anorectal malformation were renal agenesis, congenital hip dysplasia and sacral defect with presacral teratoma in Currarino triad. MRI was a simple and useful study to confirm the level of atresia, fistula and associated anomalies in the diagnosis of the congenital anorectal malformation

  18. Incidence of agenesis of palmaris longus in the Andhra population of India

    Directory of Open Access Journals (Sweden)

    K Devi Sankar

    2011-01-01

    Full Text Available Background : The knowledge of Palmaris longus (PL is a growing interest for its wide role in reconstructive plastic surgeries as a donor tendon for transfer or transplant. The prevalence of the PL agenesis has been well-documented by many authors in different ethnic groups or populations. Many conventional tests for determining the presence of the PL has been described, but lamentably there are many discrepancies in confirming its presence or absence. Slight modifications of the prevailing methods can still give authenticate results. Aim : This prospective study was conducted to determine the incidence of unilateral and bilateral agenesis of PL and its association with sex and side of the limb in the Andhra population of India. Materials and Methods : A total of 942 subjects of both sexes belonging to 18-23 years were used to access the PL using various tendon examination techniques including our modified Schaeffer′s test. The data collected were analyzed by Pearsons χ2 test using SPSS software. Results : Overall agenesis of muscle in both sexes was 264 (28.0%, out of which 40.2% was seen in females and 14.7% in males with the ratio of 3:1. The unilateral agenesis was seen in 70.5% and bilateral agenesis in 29.5% subjects. The left side agenesis was seen in 51.6% and right side in 48.4% subjects. Conclusions : The prevalence of bilateral and unilateral agenesis was more common on left side with a greater likelihood in the female subjects. The proposed technique could bring better results in all subjects and can be implemented in manual examination of PL.

  19. Circumferential tracheal resection with primary anastomosis for post-intubation tracheal stenosis: study of 24 cases.

    Science.gov (United States)

    Negm, Hesham; Mosleh, Mohamed; Fathy, Hesham

    2013-09-01

    The objective of this study is to evaluate the results of circumferential tracheal and cricotracheal resection with primary anastomosis for the treatment of post-intubation tracheal and cricotracheal stenosis. This is a retrospective analytical study. A total number of 24 patients were included in this study. The relevant preoperative, operative and postoperative records were collected and analyzed. Twenty patients were finally symptom-free reflecting an anastomosis success rate of 83.3 %. Variable grades of anastomotic restenosis occurred in 11 (45.8 %) patients, three patients were symptom-free and eight had airway obstructive symptoms. Four out of the eight patients with symptomatic restenosis were symptom-free with endoscopic dilatation while the remaining four patients required a permanent airway appliance (T-tube, tracheostomy) for the relief of airway obstruction and this group was considered as anastomotic failure. Cricoid involvement, associated cricoid resection and the type of anastomosis were the variables that had statistical impact on the occurrence of restenosis (P = 0.017, 0.017, 0.05; respectively). Tracheal resection with primary anastomosis is a safe effective treatment method for post-intubation tracheal stenosis in carefully selected patients. Restenosis does not always mean failure of the procedure since it may be successfully managed with endoscopic dilatation.

  20. Agenesis of the corpus callosum and gray matter heterotopia in three patients with constitutional mismatch repair deficiency syndrome.

    Science.gov (United States)

    Baas, Annette F; Gabbett, Michael; Rimac, Milan; Kansikas, Minttu; Raphael, Martine; Nievelstein, Rutger Aj; Nicholls, Wayne; Offerhaus, Johan; Bodmer, Danielle; Wernstedt, Annekatrin; Krabichler, Birgit; Strasser, Ulrich; Nyström, Minna; Zschocke, Johannes; Robertson, Stephen P; van Haelst, Mieke M; Wimmer, Katharina

    2013-01-01

    Constitutional mismatch repair deficiency (CMMR-D) syndrome is a rare inherited childhood cancer predisposition caused by biallelic germline mutations in one of the four mismatch repair (MMR)-genes, MLH1, MSH2, MSH6 or PMS2. Owing to a wide tumor spectrum, the lack of specific clinical features and the overlap with other cancer predisposing syndromes, diagnosis of CMMR-D is often delayed in pediatric cancer patients. Here, we report of three new CMMR-D patients all of whom developed more than one malignancy. The common finding in these three patients is agenesis of the corpus callosum (ACC). Gray matter heterotopia is present in two patients. One of the 57 previously reported CMMR-D patients with brain tumors (therefore all likely had cerebral imaging) also had ACC. With the present report the prevalence of cerebral malformations is at least 4/60 (6.6%). This number is well above the population birth prevalence of 0.09-0.36 live births with these cerebral malformations, suggesting that ACC and heterotopia are features of CMMR-D. Therefore, the presence of cerebral malformations in pediatric cancer patients should alert to the possible diagnosis of CMMR-D. ACC and gray matter heterotopia are the first congenital malformations described to occur at higher frequency in CMMR-D patients than in the general population. Further systematic evaluations of CMMR-D patients are needed to identify possible other malformations associated with this syndrome.

  1. Agenesia de veia cava inferior associada à trombose venosa profunda Agenesis of inferior vena cava associated with deep venous thrombosis

    Directory of Open Access Journals (Sweden)

    Clovis Luis Konopka

    2010-09-01

    Full Text Available A agenesia da veia cava inferior é uma anomalia congênita rara, que foi recentemente identificada como um importante fator de risco para o desenvolvimento e a recorrência de trombose venosa profunda de membros inferiores em jovens. O objetivo deste trabalho foi relatar o caso de uma paciente que apresentou trombose venosa profunda dois meses após a realização de cirurgia de varizes. A angiotomografia computadorizada demonstrou a presença de anomalia venosa complexa com ausência da veia cava inferior.The agenesis of the inferior vena cava is a rare congenital anomaly, which was recently identified as an important risk factor for the development and recurrence of deep venous thrombosis especially in young people. The goal of this work was to report the case of a patient who presented deep venous thrombosis approximately two months after varicose vein surgery. The computerized angiotomography demonstrated the presence of a complex venous anomaly with absence of the inferior vena cava.

  2. Emotional Intelligence in Agenesis of the Corpus Callosum.

    Science.gov (United States)

    Anderson, Luke B; Paul, Lynn K; Brown, Warren S

    2017-05-01

    People with agenesis of the corpus callosum (AgCC) with normal general intelligence have deficits in complex cognitive processing, as well as in social cognition. It is uncertain the extent to which impoverished processing of emotions may contribute to social processing deficiencies. We used the Mayer-Salovey-Caruso Emotional Intelligence Test to clarify the nature of emotional intelligence in 16 adults with AgCC. As hypothesized, persons with AgCC exhibited greater disparities from norms on tests involving more socially complex aspects of emotions. The AgCC group did not differ from norms on the Experiential subscale, but they were significantly below norms on the Strategic subscale. These findings suggest that the corpus callosum is not essential for experiencing and thinking about basic emotions in a "normal" way, but is necessary for more complex processes involving emotions in the context of social interactions. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  3. Bronchoscopic Treatment in the Management of Benign Tracheal Stenosis: Choices for Simple and Complex Tracheal Stenosis.

    Science.gov (United States)

    Dalar, Levent; Karasulu, Levent; Abul, Yasin; Özdemir, Cengiz; Sökücü, Sinem Nedime; Tarhan, Merve; Altin, Sedat

    2016-04-01

    Bronchoscopic treatment is 1 of the treatment choices for both palliative and definitive treatment of benign tracheal stenosis. There is no consensus on the management of these patients, however, especially patients having complex stenoses. The aim of the present study was to assess, in the largest group of patients with complex stenoses yet reported, which types of tracheal stenosis are amenable to optimal management by bronchoscopic treatment. The present study was a retrospective cohort study including 132 consecutive patients with benign tracheal stenoses diagnosed between August 2005 and January 2013. The mean age of the study population was 52 ± 18 years; 62 (47%) were women and 70 (53%) were men. Their lesions were classified as simple and complex stenoses. Simple stenoses (n = 6) were treated with 12 rigid and flexible bronchoscopic procedures (mean of 2 per patient); 5 stents were placed. The total success rate was 100%. Among the 124 complex stenoses, 4 were treated directly with surgical intervention. In total, 481 rigid and 487 flexible bronchoscopic procedures were performed in these patients. In this group, the success rate was 69.8%. From the present study, we propose that after accurate classification, interventional bronchoscopic management may have an important role in the treatment of benign tracheal stenosis. Bronchoscopic treatment should be considered as first-line therapy for simple stenoses, whereas complex stenoses need a multidisciplinary approach and often require surgical intervention. However, bronchoscopic treatment may be a valid conservative approach in the management of patients with complex tracheal stenosis who are not eligible for operative treatment. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  4. Synchrotron imaging of the grasshopper tracheal system: morphological and physiological components of tracheal hypermetry

    International Nuclear Information System (INIS)

    Greenlee, K.J.; Henry, J.R.; Kirkton, S.D.; Westneat, M.W.; Fezzaa, K.; Lee, W.; Harrison, J.F.

    2009-01-01

    As grasshoppers increase in size during ontogeny, they have mass specifically greater whole body tracheal and tidal volumes and ventilation than predicted by an isometric relationship with body mass and body volume. However, the morphological and physiological bases to this respiratory hypermetry are unknown. In this study, we use synchrotron imaging to demonstrate that tracheal hypermetry in developing grasshoppers (Schistocerca americana) is due to increases in air sacs and tracheae and occurs in all three body segments, providing evidence against the hypothesis that hypermetry is due to gaining flight ability. We also assessed the scaling of air sac structure and function by assessing volume changes of focal abdominal air sacs. Ventilatory frequencies increased in larger animals during hypoxia (5% O 2 ) but did not scale in normoxia. For grasshoppers in normoxia, inflated and deflated air sac volumes and ventilation scaled hypermetrically. During hypoxia (5% O 2 ), many grasshoppers compressed air sacs nearly completely regardless of body size, and air sac volumes scaled isometrically. Together, these results demonstrate that whole body tracheal hypermetry and enhanced ventilation in larger/older grasshoppers are primarily due to proportionally larger air sacs and higher ventilation frequencies in larger animals during hypoxia. Prior studies showed reduced whole body tracheal volumes and tidal volume in late-stage grasshoppers, suggesting that tissue growth compresses air sacs. In contrast, we found that inflated volumes, percent volume changes, and ventilation were identical in abdominal air sacs of late-stage fifth instar and early-stage animals, suggesting that decreasing volume of the tracheal system later in the instar occurs in other body regions that have harder exoskeleton.

  5. Synchrotron imaging of the grasshopper tracheal system : morphological and physiological components of tracheal hypermetry.

    Energy Technology Data Exchange (ETDEWEB)

    Greenlee, K. J.; Henry, J. R.; Kirkton, S. D.; Westneat, M. W.; Fezzaa, K.; Lee, W.; Harrison, J. F.; North Dakota State Univ.; Arizona State Univ.; Union Coll.; Field Museum of Natural History

    2009-11-01

    As grasshoppers increase in size during ontogeny, they have mass specifically greater whole body tracheal and tidal volumes and ventilation than predicted by an isometric relationship with body mass and body volume. However, the morphological and physiological bases to this respiratory hypermetry are unknown. In this study, we use synchrotron imaging to demonstrate that tracheal hypermetry in developing grasshoppers (Schistocerca americana) is due to increases in air sacs and tracheae and occurs in all three body segments, providing evidence against the hypothesis that hypermetry is due to gaining flight ability. We also assessed the scaling of air sac structure and function by assessing volume changes of focal abdominal air sacs. Ventilatory frequencies increased in larger animals during hypoxia (5% O{sub 2}) but did not scale in normoxia. For grasshoppers in normoxia, inflated and deflated air sac volumes and ventilation scaled hypermetrically. During hypoxia (5% O{sub 2}), many grasshoppers compressed air sacs nearly completely regardless of body size, and air sac volumes scaled isometrically. Together, these results demonstrate that whole body tracheal hypermetry and enhanced ventilation in larger/older grasshoppers are primarily due to proportionally larger air sacs and higher ventilation frequencies in larger animals during hypoxia. Prior studies showed reduced whole body tracheal volumes and tidal volume in late-stage grasshoppers, suggesting that tissue growth compresses air sacs. In contrast, we found that inflated volumes, percent volume changes, and ventilation were identical in abdominal air sacs of late-stage fifth instar and early-stage animals, suggesting that decreasing volume of the tracheal system later in the instar occurs in other body regions that have harder exoskeleton.

  6. Welded tracheal stent removal in a child under cardiopulmonary bypass.

    Science.gov (United States)

    Kao, S C; Chang, W K; Pong, M W; Cheng, K W; Chan, K H; Tsai, S K

    2003-08-01

    Metallic tracheal stents have been used in the treatment of paediatric tracheomalacia for more than a decade. We describe a case in which critical airway obstruction occurred during removal of a welded tracheal stent using a rigid bronchoscope under general anaesthesia. Life-saving cardiopulmonary bypass was instituted urgently, and the welded stent was then removed successfully by directly opening the trachea.

  7. Tracheal schwannoma: Completely resected with therapeutic bronchoscopic techniques

    Directory of Open Access Journals (Sweden)

    Barney Thomas Jesudason Isaac

    2015-01-01

    Full Text Available Tracheal schwannomas are rare benign tumors of the trachea. There are only a few reported cases in the literature. Surgeons have generally resected these tumors, whereas bronchoscopists have attempted to remove them bronchoscopically. We report a case of tracheal schwannoma which was completely resected using bronchoscopic techniques.

  8. 21 CFR 868.5795 - Tracheal tube cleaning brush.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Tracheal tube cleaning brush. 868.5795 Section 868.5795 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES ANESTHESIOLOGY DEVICES Therapeutic Devices § 868.5795 Tracheal tube cleaning brush. (a...

  9. What's in a name? Expiratory tracheal narrowing in adults explained

    International Nuclear Information System (INIS)

    Leong, P.; Bardin, P.G.; Lau, K.K.

    2013-01-01

    Tracheomalacia, tracheobronchomalacia, and excessive dynamic airway collapse are all terms used to describe tracheal narrowing in expiration. The first two describe luminal reduction from cartilage softening and the latter refers to luminal reduction from exaggerated posterior membrane movement. Expiratory tracheal narrowing is a frequent occurrence that can cause symptoms of airway obstruction, such as dyspnoea, wheeze, and exercise intolerance. The accurate diagnosis and quantification of expiratory tracheal narrowing has important aetiological, therapeutic, and prognostic implications. The reference standard for diagnosis has traditionally been bronchoscopy; however, this method has significant limitations. Expiratory tracheal disorders are readily detected by four-dimensional dynamic volume multidetector computed tomography (4D-CT), an emerging, non-invasive method that will potentially enable detection and quantification of these conditions. This review discusses the morphological forms of expiratory tracheal narrowing and demonstrates the utility of 4D-CT in the diagnosis, quantification, and treatment of these important conditions

  10. Complete agenesis of the dorsal pancreas: Case report with ...

    African Journals Online (AJOL)

    pancreatic head and uncinate process were normal, but the distal neck, body ... The neck, body, tail, and cephalic aspects of the head of the pancreas originate from the .... Embryology, normal variation, and congenital anomalies of the pancreas. ... M. A 3D reconstruction of pancreas development in the human embryos.

  11. Maxillary Hypoplasia With Congenital Oligodontia Treated by Maxillary Distraction Osteogenesis.

    Science.gov (United States)

    Mishima, Sayaka; Yamaguchi, Takako; Watanabe, Takuma; Komatani, Toru; Nakao, Kazumasa; Takahashi, Katsu; Bessho, Kazuhisa

    2018-02-27

    It is known that congenitally missing teeth can often cause differences in craniofacial morphology; however, there are few reported cases of orthognathic surgical treatment for these patients. Herein, the authors report a rare case of maxillary hypoplasia with congenital oligodontia treated by maxillary distraction osteogenesis with internal device. A 17-year-old male presenting with multiple tooth agenesis and maxillary recession was referred to our hospital for orthognathic surgical treatment. Preoperative simulation surgery was performed using Full-Color 3-dimensional salt model. After surgery, improvement in maxillary recession and occlusal stability was observed. This report demonstrates the advantages of the method used herein, which includes reduction in operating time with increase in the safety of the procedure.

  12. Endoscopically placed nitinol stents for pediatric tracheal obstruction.

    Science.gov (United States)

    Prasad, Mukesh; Bent, John P; Ward, Robert F; April, Max M

    2002-11-11

    To provide preliminary clinical data regarding endoscopically placed nitinol stents for children with tracheal obstruction as a temporizing measure to allow for trach tube decannulation while awaiting growth to allow for tracheal resection. This case series describes the experiences of two children (ages 5 and 15) who were dependent upon tracheotomy because of acquired tracheal obstruction. Both patients had combined tracheomalacia and tracheal stenosis. After failing tracheoplasty with rib graft augmentation both patients suffered from extensive tracheal disease, which was too long to allow for immediate tracheal resection. Endoscopic placement of nitinol stents in the obstructed tracheal segment using fluoroscopic guidance. All tracheotomy tubes were removed immediately after successful stent deployment with the patient still under general anesthesia. Four stents were placed in total. The first patient's initial stent was too narrow and was, therefore, removed and replaced at a later date with a larger diameter stent. The second patient experienced distal migration of his initial stent requiring stent removal and replacement at a later date. Both patients remain successfully decannulated (follow-up, 25 and 26 months) and are currently living more normal lives as they grow and await tracheal resection. Preliminary use of nitinol stents for pediatric tracheal obstruction has enabled successful decannulation in two children with complicated airways. Our results with this series of patients suggest that nitinol stents can be safely used in children as a temporizing measure until tracheal resection can be safely performed. With this approach children can live free from the hassles of trach care, social isolation and peer ridicule. Limited pediatric experience exists in the literature about nitinol stents. Thus, our experience with stent selection and placement will help others avoid problems encountered in this initial series. Copyright 2002 Elsevier Science Ireland

  13. Tracheal resections and anastomosis for benign tracheal stricture. A seven-year experience in a single tertiary institute

    Directory of Open Access Journals (Sweden)

    Ahmed Mostafa

    2017-09-01

    Conclusions: TRA proved to be a safe and reliable solution for post-intubation tracheal stenosis. This procedure should be considered first in developing countries where there is frequent rush for tracheostomies and tracheal stenting. Quality of life improved dramatically following this definitive surgery.

  14. Tracheal transplantation for carinal reconstruction in dogs.

    Science.gov (United States)

    Kawahara, K; Inutsuka, K; Hiratsuka, M; Makihata, S; Okabayashi, K; Shiraishi, T; Shirakusa, T

    1998-09-01

    Experimental carinal allotransplantation has been performed with tracheocarinal Y-shaped allografts in dogs. In this study we tried canine carinal reconstruction with cylindrical allografts. Carinal reconstruction was performed with allotransplantation of cylindrical trachea in dogs, and graft healing was evaluated by bronchoscopic observation, mucosal blood flow measurement, and histologic examination. A section of the recipient carina containing five tracheal rings and two main stem bronchi was removed, and a donor trachea seven rings long was inserted between the recipient trachea and the left main stem bronchus; then side-to-end anastomosis was performed between the graft midportion and recipient right main stem bronchus (new carina). The grafts were wrapped with pedicled omentum. Fresh grafts were transplanted into one group of dogs (n=8 ), and grafts cryopreserved for 1 week were transplanted into another group (n=7). No anastomotic leakage occurred in any dog. Excellent healing of grafts and graft anastomoses was observed by fiberoptic bronchoscopy in six dogs (75%) in the fresh graft group and in four dogs (57%) in the cryopreserved graft group. The mucosal blood flow in the new carina decreased remarkably and, although it recovered, mucosal blood flow remained under the preoperative level on day 28 after the operation. Cylindrical tracheal allotransplantation is useful for carinal reconstruction, and the method of side-to-end anastomosis between the donor trachea and recipient bronchus is a feasible and accessible procedure in dogs.

  15. Congenital amusias.

    Science.gov (United States)

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

  16. Evaluation of magnesium-yttrium alloy as an extraluminal tracheal stent.

    Science.gov (United States)

    Luffy, Sarah A; Chou, Da-Tren; Waterman, Jenora; Wearden, Peter D; Kumta, Prashant N; Gilbert, Thomas W

    2014-03-01

    Tracheomalacia is a relatively rare problem, but can be challenging to treat, particularly in pediatric patients. Due to the presence of mechanically deficient cartilage, the trachea is unable to resist collapse under physiologic pressures of respiration, which can lead to acute death if left untreated. However, if treated, the outcome for patients with congenital tracheomalacia is quite good because the cartilage tends to spontaneously mature over a period of 12 to 18 months. The present study investigated the potential for the use of degradable magnesium-3% yttrium alloy (W3) to serve as an extraluminal tracheal stent in a canine model. The host response to the scaffold included the formation of a thin, vascularized capsule consisting of collagenous tissue and primarily mononuclear cells. The adjacent cartilage structure was not adversely affected as observed by bronchoscopic, gross, histologic, and mechanical analysis. The W3 stents showed reproducible spatial and temporal fracture patterns, but otherwise tended to corrode quite slowly, with a mix of Ca and P rich corrosion product formed on the surface and observed focal regions of pitting. The study showed that the approach to use degradable magnesium alloys as an extraluminal tracheal stent is promising, although further development of the alloys is required to improve the resistance to stress corrosion cracking and improve the ductility. Copyright © 2013 Wiley Periodicals, Inc.

  17. Gallbladder Agenesis in 17 Dogs: 2006-2016.

    Science.gov (United States)

    Sato, K; Sakai, M; Hayakawa, S; Sakamoto, Y; Kagawa, Y; Kutara, K; Teshima, K; Asano, K; Watari, T

    2018-01-01

    Gallbladder agenesis (GBA) is extremely rare in dogs. To describe the history, clinical signs, diagnosis, treatment, and outcomes of dogs with GBA. Seventeen client-owned dogs with GBA. Medical records from 2006 through 2016 were retrospectively reviewed. Dogs were included when GBA was suspected on abdominal ultrasonography and confirmed by gross evaluation. Signalment, clinical signs, clinicopathological data, diagnostic imaging, histopathology, treatment, and outcome were recorded. Dogs were of 6 different breeds, and Chihuahuas (10 of 17) were most common. Median age at presentation was 1.9 (range, 0.7-7.4) years. Clinical signs included vomiting (5 of 17), anorexia (2 of 17), ascites (2 of 17), diarrhea (1 of 17), lethargy (1 of 17), and seizures (1 of 17). All dogs had increased serum activity of at least 1 liver enzyme, most commonly alanine aminotransferase (15 of 17). Fifteen dogs underwent computed tomography (CT) cholangiography; common bile duct (CBD) dilatation was confirmed in 12, without evidence of bile duct obstruction. Gross evaluation confirmed malformation of the liver lobes in 14 of 17 dogs and acquired portosystemic collaterals in 5 of 17. Ductal plate malformation was confirmed histologically in 16 of 17 dogs. During follow-up (range, 4-3,379 days), 16 of 17 dogs remained alive. Dogs with GBA exhibit clinicopathological signs of hepatobiliary injury and hepatic histopathological changes consistent with a ductal plate abnormality. Computed tomography cholangiography was superior to ultrasound examination in identifying accompanying nonobstructive CBD distention. Computed tomography cholangiography combined with laparoscopic liver biopsy is the preferable approach to characterize the full disease spectrum accompanying GBA in dogs. Copyright © 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  18. Proverb comprehension in individuals with agenesis of the corpus callosum.

    Science.gov (United States)

    Rehmel, Jamie L; Brown, Warren S; Paul, Lynn K

    2016-09-01

    Comprehension of non-literal language involves multiple neural systems likely involving callosal connections. We describe proverb comprehension impairments in individuals with isolated agenesis of the corpus callosum (AgCC) and normal-range general intelligence. Experiment 1 compared Gorham Proverb Test (Gorham, 1956) performance in 19 adults with AgCC and 33 neurotypical control participants of similar age, sex, and intelligence. Experiment 2 used the Proverbs subtest of the Delis-Kaplan Executive Function System (D-KEFS, 2001) to compare 19 adults with AgCC and 17 control participants with similar age, sex, and intelligence. Gorham Proverbs performance was impaired in the AgCC group for both the free-response and multiple-choice tasks. On the D-KEFS proverbs test, the AgCC group performed significantly worse on the free-response task (and all derivative scores) despite normal levels of performance on the multiple-choice task. Covarying verbal intelligence did not alter these outcomes. However, covarying a measure of non-literal language comprehension considerably reduced group differences in proverb comprehension on the Gorham test, but had little effect on the D-KEFS group differences. The difference between groups seemed to be greatest when participants had to generate their own interpretation (free response), or in the multiple choice format when the test included many proverbs that were likely to be less familiar. Taken together, the results of this study clearly show that proverb comprehension is diminished in individuals with AgCC compared to their peers. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Tracheal Compression Caused by a Mediastinal Hematoma After Interrupted Aortic Arch Surgery.

    Science.gov (United States)

    Hua, Qingwang; Lin, Zhiyong; Hu, Xingti; Zhao, Qifeng

    2017-08-03

    Congenital abnormalities of the aortic arch include interrupted aortic arch (IAA), coarctation of the aorta (CoA), and double aortic arch (DAA). Aortic arch repair is difficult and postoperative complications are common. However, postoperative tracheobronchial stenosis with respiratory insufficiency is an uncommon complication and is usually caused by increased aortic anastomotic tension. We report here a case of tracheal compression by a mediastinal hematoma following IAA surgery. The patient underwent a repeat operation to remove the hematoma and was successfully weaned off the ventilator.In cases of tracheobronchial stenosis after aortic arch surgery, airway compression by increased aortic anastomotic tension is usually the first diagnosis considered by clinicians. Other causes, such as mediastinal hematomas, are often ignored. However, the severity of symptoms with mediastinal hematomas makes this an important entity.

  20. Maternal reproductive history and the risk of isolated congenital malformations.

    Science.gov (United States)

    Materna-Kiryluk, A; Więckowska, B; Wiśniewska, K; Borszewska-Kornacka, M K; Godula-Stuglik, U; Limon, J; Rusin, J; Sawulicka-Oleszczuk, H; Szwałkiewicz-Warowicka, E; Walczak, M

    2011-03-01

    We examined the relationship between maternal reproductive history and the newborn's risk of isolated congenital malformations in a large case-control cohort from the Polish Registry of Congenital Malformations. Congenital malformations were classified into four categories: isolated congenital heart defects (n=1673), isolated cleft palate (n=255), cleft lip with or without cleft palate (n=448) and renal agenesis (n=103). The case groups were compared with a shared group of 2068 controls recruited in the same time period and geographic area. Multivariable logistic regression was used to assess the risk associated with maternal gravidity and of previous miscarriages after accounting for maternal age and other potential risk factors. In unadjusted analyses, maternal gravidity was significantly associated with increased risk of all four classes of congenital malformations. After adjustment, a significant association persisted for congenital heart defects [odds ratio (OR)=1.22, [95% confidence interval (CI) 1.09, 1.36], P=0.0007] and cleft lip with or without cleft palate (OR=1.21, [95% CI 1.09, 1.36], P=0.0005). A similar trend existed for isolated cleft palate (OR=1.18, [95% CI 1.02, 1.37], P=0.03). There was no appreciable increase in the risk of congenital malformations associated with a maternal history of miscarriages, but a trend for a protective effect on the occurrence of cleft lip with or without cleft palate was observed (OR=0.72, [95% CI 0.52, 0.99], P=0.045). Based on our data, maternal gravidity represents a significant risk factor for congenital heart defects and cleft lip with or without cleft palate in the newborn infant. Our data do not support an increase in risk because of past history of miscarriages. © 2011 Blackwell Publishing Ltd.

  1. Treatment of a case of tracheal stenosis in a dog with tracheal resection and anastomosis

    International Nuclear Information System (INIS)

    Mutlu, Z.; Acar, S.E.; Perk, C.

    2003-01-01

    A case of tracheal stenosis in the cervical portion of the trachea was encountered in a 5.5-month-old St. Bernard-Rottweiler cross dog. Breathing difficulty was seen in the clinical examination and presence of an obvious narrowing between the 3rd-5th cervical tracheal rings was determined in the radiological examination. Under general anesthesia the portion with stenosis was resected and the healthy trachea ends were anastomosed using the split cartilage technique. In the postoperative period the breathing difficulty disappeared and there was no development of a new stenosis in the anastomosis region. In the late period check-up the patient was seen to lead a healthy life

  2. Effects of acute respiratory virus infection upon tracheal mucous transport

    International Nuclear Information System (INIS)

    Gerrard, C.S.; Levandowski, R.A.; Gerrity, T.R.; Yeates, D.B.; Klein, E.

    1985-01-01

    Tracheal mucous velocity was measured in 13 healthy non-smokers using an aerosol labelled with /sup 99m/Tc and a multidetector probe during respiratory virus infections. The movement of boluses of tracheal mucous were either absent or reduced in number in five subjects with myxovirus infection (four influenza and one respiratory syncytial virus) within 48 hr of the onset of symptoms and in four subjects 1 wk later. One subject with influenza still had reduced bolus formation 12-16 wk after infection. Frequent coughing was a feature of those subjects with absent tracheal boluses. In contrast, four subjects with rhinovirus infection had normal tracheal mucous velocity at 48 hr after the onset of symptoms (4.1 +/- 1.3 mm/min). Tracheal mucous velocity was also normal (4.6 +/- 1.1 mm/min) in four subjects in whom no specific viral agent could be defined but had typical symptomatology of respiratory viral infection. During health tracheal mucous velocity was normal (4.8 +/- 1.6 mm/min) in the eleven subjects who had measurements made. Disturbances in tracheal mucous transport during virus infection appear to depend upon the type of virus and are most severe in influenza A and respiratory syncytial virus infection

  3. MR diagnosis of penile agenesis: is it just absence of a phallus?

    Energy Technology Data Exchange (ETDEWEB)

    Goenka, Ajit; Jain, Vaibhav; Sharma, Raju; Gupta, Arun K. [All India Institute of Medical Sciences, Department of Radiodiagnosis, New Delhi (India); Bajpai, Minu [All India Institute of Medical Sciences, Department of Paediatric Surgery, New Delhi (India)

    2008-10-15

    Penile agenesis is an extremely rare anomaly that results from absence of the genital tubercle or its failure to develop into a penis during embryonic life. Associated anomalies of the genitourinary and distal gastrointestinal tracts are frequently present. Imaging modalities, particularly MRI and cystography, play a crucial role in establishing the diagnosis and guiding further management. We report a child with penile agenesis with a urethroanal fistula and a blind-ending anterior urethra who had been reared as a male until presentation. (orig.)

  4. Chronic renal failure due to unilateral renal agenesis and total renal dysplasia (=aplasia)

    International Nuclear Information System (INIS)

    Kroepelin, T.; Ziupa, J.; Wimmer, B.

    1983-01-01

    Three adult patients with unilateral renal agenesis/total dysplasia (= aplasia) and with an early chronic renal failure are presented. One patient had renal agenesis without ureter bud and ureteric ostium on one side, and reflux pyelonephritis on the other; one had small compact total renal dysplasia (= aplasia) on one side, while chronic uric acid nephropathy (chronic renal disease as a cause of gout) was diagnosed on the other; the third patient had a total large multicystic dysplasia on one side, and on the other a segmental large multicystic dysplasia. Radiological steps and radiodiagnostic criteria are discussed and the combination of urogenital and extraurogenital anomalies is referred to. (orig.)

  5. Tracheal growth after resection and anastomosis in puppies.

    Science.gov (United States)

    Blanchard, H; Brochu, P; Bensoussan, A L; Lagacé, G; Khan, A H

    1986-09-01

    Tracheal morphology, morphometric changes, and growth and histologic changes were studied in puppies submitted to tracheal resection and anastomosis. Fifteen mongrel puppies about 12 weeks old and weighing on an average 5.5 kg were operated under general anesthesia using fluothane. A median cervicotomy incision was made in ten puppies (experimental group, EG) and the proximal 14 tracheal rings were resected (average length 5.08 cm or about 35% to 38% of total tracheal length). One layer anastomosis was done using vicryl 4.0 maintaining the average tension of 1,450 g. Five puppies (control group, CG) were submitted to tracheal transection and anastomosis and the following parameters were studied. Tracheal morphology the trachea of the EG was a rounded triangle whereas in the CG it was oval in shape, there was increase in the intercartilageneous spaces in the EG, no granulation tissue was present, two mucous webs were seen in the EG and one in the CG. Morphometric changes average tracheal length EG 13 cm, CG 17.7 cm, intercartilagenous space EG 3.08 mm, CG 1.3 mm, intercricothyroid space EG 1.2 cm, CG 0.53 cm, sagittal and transverse tracheal thickness at the anastomosis EG 2.6 and 3.3 mm, CG 2 and 1.5 mm, sagittal and transverse diameter reduced on an average 2 mm in EG. Histology Moderate fibrosis was found at the level of anastomosis with no modification of chondrocytes at the cartilagenous rings in the EG. Even with high anastomotic tension, the dogs had normal tracheal growth without stenosis; the sagittal and transverse growth at the anastomosis in the EG was 90% and 85%, respectively, when compared with the CG.

  6. A Case of Multiple Cardiovascular and Tracheal Anomalies Presented with Wolff-Parkinson-White Syndrome in a Middle-aged Adult.

    Science.gov (United States)

    Shi, Hyejin; Sohn, Sungmin; Wang, SungHo; Park, Sungrock; Lee, SangKi; Kim, Song Yi; Jeong, Sun Young; Kim, Changhwan

    2017-12-01

    Congenital cardiovascular anomalies, such as dextrocardia, persistent left superior vena cava (SVC), and pulmonary artery (PA) sling, are rare disorders. These congenital anomalies can occur alone, or coincide with other congenital malformations. In the majority of cases, congenital anomalies are detected early in life by certain signs and symptoms. A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia. After synchronized cardioversion, an electrocardiogram revealed Wolff-Parkinson-White (WPW) syndrome. Persistent left SVC, PA sling, and right tracheal bronchus were also detected by a chest computed tomography (CT) scan. He was diagnosed with paroxysmal supraventricular tachycardia (PSVT) associated with WPW syndrome, and underwent radiofrequency ablation. We reported the first case of situs solitus dextrocardia coexisting with persistent left SVC, PA sling and right tracheal bronchus presented with WPW and PSVT in a middle-aged adult. In patients with a cardiovascular anomaly, clinicians should consider thorough evaluation of possibly combined cardiovascular and airway malformations and cardiac dysrhythmia. © 2017 The Korean Academy of Medical Sciences.

  7. Congenital Hydrocephalus.

    Science.gov (United States)

    Estey, Chelsie M

    2016-03-01

    There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Congenital diplopodia

    International Nuclear Information System (INIS)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam

    2003-01-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  9. Snake Envenomation Causing Distant Tracheal Myonecrosis

    Directory of Open Access Journals (Sweden)

    Amina Khimani

    2013-01-01

    Full Text Available Snakebites are often believed to be poisonous. However, this is not always the case. In fact, each bite differs from snake to snake, depending on if the snake is poisonous and if there is envenomation. Venom in pit viper snakebites is often associated with local necrosis. The abundant literature selections and research articles justify local myonecrosis due to envenomation, but there is not much in the literature regarding myonecrosis at a site distant from the snakebite. We hereby present a case of a 42-year-old man who was transferred to our emergency department after a rattlesnake bit him twice. The patient, besides developing local myonecrosis at the site of the snakebite, developed necrosis of the scrotum as well as tracheal pressure myonecrosis at the site of the endotracheal tube balloon. In this review, we will attempt to discuss the myonecrosis pathophysiology and management related to the rattle snakebite.

  10. Esophageal trachealization: A feature of eosinophilic esophagitis

    International Nuclear Information System (INIS)

    AlHussaini, Abdulrahman A; Semaan, Toufic; ElHag, Imad A

    2009-01-01

    Eosinophilic esophagitis (EE) is an inflammatory condition characterized by intense eosinophilic infiltration of the esophagus. EE is frequently misdiagnosed as gastroesophageal reflux disease. Here, we present a child with EE and a characteristic endoscopic finding, r inged esophagus . An 11-year-old Saudi boy presented with dysphagia for 1 year. He had experienced an intermittent sensation of solid food sticking in his chest, which was relieved by drinking liquids. A barium swallow excluded anatomical causes of dysphagia, but revealed multiple-ringed esophagus. Endoscopy showed a furrowing and trachealizing appearance of the entire esophagus. Hisologically, extensive eosinophilic infiltration was a feature in biopsies obtained from the esophagus. The child responded well to a 2-month course of inhaled fluticasone. Symptoms recurred 3 months after discontinuation of therapy, which necessitated resumption of inhaled fluticasone. The endoscopic appearance of multiple esophageal rings should raise suspicion of EE and be confirmed by esophageal biopsies. (author)

  11. Congenital toxoplasmosis.

    Science.gov (United States)

    Kieffer, François; Wallon, Martine

    2013-01-01

    Congenital toxoplasmosis results from the transplacental transmission of the parasite Toxoplasma gondii after a maternal infection acquired in pregnancy. Prevalence of congenital infection ranges from 0.1 to 0.3 per 1000 live births. The maternal-fetal transmission rate increases with gestational age at maternal seroconversion, from less than 15% at 13 weeks of gestation to over 70% at 36 weeks. Conversely, the later the maternal infection, the lower the risk of symptomatic congenital infection (infections acquired during the third trimester are most often asymptomatic at birth). Prenatal diagnosis is currently performed by PCR analysis in amniotic fluid. Antenatal management and treatment vary considerably among countries. In some European countries, maternal infections are detected through serological screening allowing a prompt treatment with spiramycin, which is expected to reduce the risk of vertical transmission. If PCR analysis in amniotic fluid is positive or if maternal infection was acquired in the third trimester of pregnancy, a combination with pyrimethamine and sulphonamide is given until delivery. Benefits of antenatal treatments remain controversial. Infected newborns are prescribed pyrimethamine and sulphonamide for 12 months. Despite antenatal and postnatal treatment, chorioretinitis can occur at any age (prevalence>20% at 10 years of age): long-term ophthalmological follow-up remains necessary. Copyright © 2013 Elsevier B.V. All rights reserved.

  12. Unilateral renal agenesis associated with ovarian cysts in a 19 year ...

    African Journals Online (AJOL)

    MJP

    2015-10-28

    379. doi: http://dx.doi.org/10.14194/ijmbr.4.3.2. How to cite this article: Anyabolu E.N,. Chukwuonye I.I, Anyabolu A.E, Enwere O.O.. Unilateral renal agenesis associated with ovarian cysts in a 19 year old woman in Orlu,.

  13. Evidence-based, ethically justified counseling for fetal bilateral renal agenesis

    Science.gov (United States)

    Thomas, Alana N.; McCullough, Laurence B.; Chervenak, Frank A.; Placencia, Frank X.

    2017-01-01

    Background Not much data are available on the natural history of bilateral renal agenesis, as the medical community does not typically offer aggressive obstetric or neonatal care asbilateral renal agenesis has been accepted as a lethal condition. Aim To provide an evidence-based, ethically justified approach to counseling pregnant women about the obstetric management of bilateral renal agenesis. Study design A systematic literature search was performed using multiple databases. We deploy an ethical analysis of the results of the literature search on the basis of the professional responsibility model of obstetric ethics. Results Eighteen articles met the inclusion criteria for review. With the exception of a single case study using serial amnioinfusion, there has been no other case of survival following dialysis and transplantation documented. Liveborn babies die during the neonatal period. Counseling pregnant women about management of pregnancies complicated by bilateral renal agenesis should be guided by beneficence-based judgment informed by evidence about outcomes. Conclusions Based on the ethical analysis of the results from this review, without experimental obstetric intervention, neonatal mortality rates will continue to be 100%. Serial amnioinfusion therefore should not be offered as treatment, but only as approved innovation or research. PMID:28222038

  14. Professional and patient-based evaluation of oral rehabilitation in patients with tooth agenesis

    DEFF Research Database (Denmark)

    Dueled, Erik; Gotfredsen, Klaus; Trab Damsgaard, Mogens

    2009-01-01

    OBJECTIVES: The outcome of oral rehabilitation is usually monitored with clinical tests rather than by patient's perception of change. The aim of this study was to describe the objective measure and subjective perception of oral rehabilitation in patients with tooth agenesis. MATERIAL AND METHODS...

  15. Novel PAX9 gene polymorphisms and mutations and susceptibility to tooth agenesis in the Czech population

    Czech Academy of Sciences Publication Activity Database

    Hloušková, A.; Bonczek, Ondřej; Izakovičová Hollá, L.; Lochman, J.; Šoukalová, J.; Štembírek, Jan; Míšek, Ivan; Černochová, P.; Krejčí, P.; Vaněk, J.; Šerý, Omar

    2015-01-01

    Roč. 36, č. 5 (2015), s. 101-106 ISSN 0172-780X R&D Projects: GA MZd(CZ) NT11420 Institutional support: RVO:67985904 Keywords : odontogenesis * tooth agenesis * PAX9 gene Subject RIV: FF - HEENT, Dentistry Impact factor: 0.946, year: 2015

  16. Didelphic uterus with imperforate unilateral vagina and ipsilateral renal agenesis: Magnetic resonance

    International Nuclear Information System (INIS)

    Rodrigo, R.M.; Saez, F.; Astigarraga, E.; Rodriguez, O.

    1993-01-01

    We present two cases of didelphic uterus with the clinical appearance of a pelvic mass produced by the hematometra-hematocolpos retained by an imperforate unilateral hemivagina. In both cases, this anomaly was accompanied by ipsilateral renal agenesis. We basically review the diagnosis of this disorder, focusing on magnetic resonance as the technique of choice. (Author) 10 refs

  17. Aicardi's syndrome: (agenesis of the corpus callosum, infantile spasms, and ocular anomalies).

    Science.gov (United States)

    Dinani, S; Jancar, J

    1984-06-01

    A case of Aicardi's syndrome is reported. A 12-year-old mentally handicapped female has all the characteristics of the syndrome: agenesis of the Corpus callosum; female infant with mental handicap, epilepsy, characteristic eye lesions, vertebral anomalies and abnormal EEG pattern. The results of supporting examination and investigations are noted.

  18. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    International Nuclear Information System (INIS)

    Griffin, N.; Mansfield, L.; Redmond, K.C.; Dusmet, M.; Goldstraw, P.; Mittal, T.K.; Padley, S.

    2007-01-01

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution

  19. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Mansfield, L. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Redmond, K.C. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Dusmet, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Goldstraw, P. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Mittal, T.K. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-03-15

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

  20. Pancreas-specific deletion of mouse Gata4 and Gata6 causes pancreatic agenesis

    Science.gov (United States)

    Xuan, Shouhong; Borok, Matthew J.; Decker, Kimberly J.; Battle, Michele A.; Duncan, Stephen A.; Hale, Michael A.; Macdonald, Raymond J.; Sussel, Lori

    2012-01-01

    Pancreatic agenesis is a human disorder caused by defects in pancreas development. To date, only a few genes have been linked to pancreatic agenesis in humans, with mutations in pancreatic and duodenal homeobox 1 (PDX1) and pancreas-specific transcription factor 1a (PTF1A) reported in only 5 families with described cases. Recently, mutations in GATA6 have been identified in a large percentage of human cases, and a GATA4 mutant allele has been implicated in a single case. In the mouse, Gata4 and Gata6 are expressed in several endoderm-derived tissues, including the pancreas. To analyze the functions of GATA4 and/or GATA6 during mouse pancreatic development, we generated pancreas-specific deletions of Gata4 and Gata6. Surprisingly, loss of either Gata4 or Gata6 in the pancreas resulted in only mild pancreatic defects, which resolved postnatally. However, simultaneous deletion of both Gata4 and Gata6 in the pancreas caused severe pancreatic agenesis due to disruption of pancreatic progenitor cell proliferation, defects in branching morphogenesis, and a subsequent failure to induce the differentiation of progenitor cells expressing carboxypeptidase A1 (CPA1) and neurogenin 3 (NEUROG3). These studies address the conserved and nonconserved mechanisms underlying GATA4 and GATA6 function during pancreas development and provide a new mouse model to characterize the underlying developmental defects associated with pancreatic agenesis. PMID:23006325

  1. The Efficacy of Polydioxanone Monofilament Absorbable Suture for Tracheal Anastomosis

    OpenAIRE

    Kawahara, Katsunobu; Yamasaki, Naoya; Yamamoto, Satoshi; Nagayasu, Takeshi; Kusano, Hiroyuki; Akamine, Shinji; Takahashi, Takao; Tomita, Masao

    1994-01-01

    To evaluate the efficacy of polydioxanon absorbable suture for tracheal anastomoses, we performed an experimental study using dose. Eight adult mongrel dogs underwent sleeve resection of the mediastinal trachea. A length of ten to twelve cartilage rings was resected. An end-to-end anastomosis was performed using either interrupted or continuous running 4-0 polydioxanone (PDS) suture. There was no detectable difference bronchoscopically, microangiografically, or histologically, in tracheal ana...

  2. Perawatan Teknik Begg Pada Maloklusi Klas I Dengan Kaninus Impaksi dan Insisivus Lateral Agenesis

    Directory of Open Access Journals (Sweden)

    Kristina Wijaya Gunawan

    2013-06-01

    Full Text Available Impaksi kaninus maksila sering dijumpai pada sisi palatal daripada labial. Agenesis adalah anomali pertumbuhan akibat tidak ada satu atau lebih benih gigi. Laporan kasus ini bertujuan memaparkan kemajuan perawatan kasus Maloklusi klas I dengan kaninus impaksi dan insisivus lateral agenesis menggunakan alat cekat teknik Begg. Seorang pasien usia 20 tahun datang mengeluhkan gigi-gigi depan atas dan bawah yang berjejal sehingga mengganggu penampilan. Perawatan bertujuan untuk koreksi Maloklusi Angle klas I tipe dentoskeletal dengan deepbite, crossbite gigi 25 terhadap 35, pergeseran midline dental maksila dan mandibula ke kanan sebesar 2,5 mm dan 3,0 mm, 13 impaksi vertikal pada sisi labial, 42 agenesis, dan edentulous parsial regio 36. Koreksi dilakukan dengan pencabutan 14, 25, pemanfaatan ruang bekas pencabutan 36 dan exposure gigi kaninus yang impaksi. Tahap pertama teknik Begg adalah leveling, unraveling, dan bite opening, diikuti dengan koreksi midline dan penutupan sisa ruang bekas pencabutan. Kesimpulan: perawataan ortodontik menggunakan teknik Begg yang dilakukan simultan dengan exposure kaninus impaksi labial dengan closed eruption technique dapat memberikan hasil yang memuaskan. Treatment for Class I Malocclusion with Impacted Canine and Agenesis Lateral Incisor Using Begg Technique. Maxillary canine impaction occurs commonly on the palatal than labial side. Agenesis is a developmental anomaly condition because of the absence of one or more tooth buds. This case report aims to explain the treatment progress of class I malocclusion with impacted canine and agenesis lateral incisor using fixed appliance through Begg technique. A 20-year-old female patient complained about her upper and lower anterior dental crowding that disturbed her appearance. The treatment aims to correct the Angle class I malocclusion dentoskeletal types with deepbite, crossbite 25 to 35, maxillary and mandibulary dental midline shift to the right by 2.5 mm and 3.0 mm

  3. Congenital syphilis

    International Nuclear Information System (INIS)

    Lee, Sang Wook; Kim, Kyung Soo; Hur, Don

    1983-01-01

    In recent years, marked increase in incidence of congenital syphilis has occurred throughout the world due to changes in social norms and development of penicillin-resistant strains. Early diagnosis plays an important role in congenital syphilis as the clinical manifestations may simulate many other conditions in the paediatric age group. The authors analyzed 52 cases of congenital syphilis admitted to the department of paediatrics, Chosun University Hospital, clinically and radiologically. Among them, 18 cases were born in this hospital and 34 cases were admitted from OPD, during the period of 8 years from January, 1975 to December, 1982. The results obtained were as follows; 1. In 28 of 34 cases (82%), the first clinical manifestations were below the age of 3 months. 2. Among the 52 cases, a male predominance was observed with a male to female ratio of 2 : 1. 3. The serologic test (VDRL) of the 52 studied cases showed reactive response in 49 cases (94%), and that of syphilitic mothers except 6 cases, reactive in all studied cases. 4. The major manifestations of the 52 cases were bone tenderness (12%) and swelling of the joints (7%) in skeletal system, hepatosplenomegaly (79%) and skin lesions (73%) in extraskeletal one. 5. The radiological skeletal changes were detected in 45 of 52 cases (87%), and the commonest findings were detected in 45 of 52 cases (87%), and the commonest findings were metaphysitis (83%) and periostitis (81%). The most characteristic type of metaphysitis were transverse trophic line (74%) and zone of rarefaction (65%). 6. The commonest bones to be affected were growing metaphyses of the long bones, particulary about the wrist and the knee. The order of frequency were radius (80%), uina (80%), tibia (77%), femur (69%) and humerus (40%)

  4. [Congenital anomalies of poor prognosis. Genetics Consensus Committee].

    Science.gov (United States)

    Pardo Vargas, Rosa A; Aracena, Mariana; Aravena, Teresa; Cares, Carolina; Cortés, Fanny; Faundes, Víctor; Mellado, Cecilia; Passalacqua, Cristóbal; Sanz, Patricia; Castillo Taucher, Silvia

    The Genetic Branch of the Chilean Society of Paediatrics, given the draft Law governing the decriminalisation of abortion on three grounds, focusing on the second ground, which considers the "embryo or foetus suffering from a congenital structural anomaly or a genetic disorder incompatible with life outside the womb", met to discuss the scientific evidence according to which congenital anomalies (CA) may be included in this draft law. Experts in clinical genetics focused on 10 CA, reviewed the literature evidence, and met to discuss it. It was agreed not to use the term "incompatible with life outside the womb", as there are exceptions and longer survivals, and change to "congenital anomaly of poor prognosis (CAPP)". Ten CA were evaluated: serious defects of neural tube closure: anencephaly, iniencephaly and craniorachischisis, pulmonary hypoplasia, acardiac foetus, ectopia cordis, non-mosaic triploidy, "limb body wall" complex, "body stalk" anomaly, trisomy 13, trisomy 18, and bilateral renal agenesis. Findings on the prevalence, natural history, prenatal diagnostic methods, survival, and reported cases of prolonged survival were analysed. Post-natal survival, existence of treatments, and outcomes, as well as natural history without intervention, were taken into account in classifying a CA as a CAPP. A CAPP would be: anencephaly, severe pulmonary hypoplasia, acardiac foetus, cervical ectopia cordis, non-mosaic triploidy, limb body wall complex, body stalk anomaly, non-mosaic trisomy 13, non-mosaic trisomy 18, and bilateral renal agenesis. For their diagnosis, it is required that all pregnant women have access to assessments by foetal anatomy ultrasound and occasionally MRI, and cytogenetic and molecular testing. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Neuroimaging findings of congenital Zika virus infection: a pictorial essay.

    Science.gov (United States)

    Zare Mehrjardi, Mohammad; Poretti, Andrea; Huisman, Thierry A G M; Werner, Heron; Keshavarz, Elham; Araujo Júnior, Edward

    2017-03-01

    Zika virus (ZIKV) is a mosquito-borne arbovirus from the Flaviviridae family. It had caused several epidemics since its discovery in 1947, but there was no significant attention to this virus until the recent outbreak in Brazil in 2015. The main concern is the causal relationship between prenatal ZIKV infection and congenital microcephaly, which has been confirmed recently. Moreover, ZIKV may cause other central nervous system abnormalities such as brain parenchymal atrophy with secondary ventriculomegaly, intracranial calcification, malformations of cortical development (such as polymicrogyria, and lissencephaly-pachygyria), agenesis/hypoplasia of the corpus callosum, cerebellar and brainstem hypoplasia, sensorineural hearing-loss, and ocular abnormalities as well as arthrogryposis in the infected fetuses. Postnatal (acquired) ZIKV infection usually has an asymptomatic or mildly symptomatic course, while prenatal (congenital) ZIKV infection has a more severe course and may cause severe brain anomalies that are described as congenital Zika syndrome. In this pictorial essay, we aim to illustrate the prenatal and postnatal neuroimaging findings that may be seen in fetuses and neonates with congenital Zika syndrome, and will discuss possible radiological differential diagnoses. A detailed knowledge of these findings is paramount for an early correct diagnosis, prognosis determination, and counseling of the affected children and families.

  6. Brain MRI findings in infants with primary congenital glaucoma

    International Nuclear Information System (INIS)

    Dai, A. Ibrahym; Saygili, O.

    2007-01-01

    Congenital glaucoma appears in the first months of life, eventually at birth. Isolated congenital glaucoma is characterized by minor malformations of the irido-corneal angle of the anterior chamber of the eye. Clinical manifestations include tearing, photophobia and enlargement of the globe appearing in the first months of life. Imaging technology such as optical coherence tomography and measurement of central corneal thickness may play an important role in the assessment of children with suspected or known glaucoma. However, no MRI findings of the CNS in patients with primary congenital glaucoma (PCG) were reported in the literature. The purpose of this study was to investigate MRI findings of the brain in infants with PCG. We reviewed the radiological and histopathological and clinical characteristics of infants with primary congenital glaucoma. The records of 17 patients with PCG were reviewed and the MRIs of the brain and associated manifestations were analyzed. Three patients with PCG had abnormal MRI findings suggesting agenesis of the corpus callosum. Two infants had delayed myelinization of the brain. Significant abnormal optic nerve excavation and increased corneal diameters in 2 patients with delayed myelinization may suggest that intraocular pressure can be more striking and more severe, revealing a close relationship with PCG and abnormal myelinization in white matter. Studies with more patients are needed to confirm these results. (author)

  7. Brain lesions in congenital nystagmus as detected by computed tomography

    International Nuclear Information System (INIS)

    Lo, Chin-Ying

    1982-01-01

    Computed tomography (CT) was performed in a series of 60 cases with congenital nystagmus. The type of nystagmus was pendular in 20 and jerky in 40 cases. The age ranged from 3 months to 13 years. Abnormal CT findings of the central nervous system were detected in 31 cases (52%). There were 5 major CT findings: midline anomalies, cortical atrophy, ventricular dilatation, brain stem atrophy and low density area. The midline anomalies involved cavum septi pellucidi, cavum Vergae, cavum veli interpositi and partial agenesis of corpus callosum. The abnormal CT findings were more prominent in pendular type than in jerky type. The incidence of congenital nystagmus and positive CT findings were the same in the first and the second birth. There was a history of abnormalities during the prenatal or perinatal period in 28 out of the 60 cases (47%). This feature seemed to play a significant role in the occurrence of congenital nystagmus. The observed organic lesions in the central nervous system by CT would contribute to the elucidation of pathomechanism of congenital nystagmus. (author)

  8. The Role of Tracheal Stenting As an Alternative Treatment in Cicatricial and Non-Operable Tracheal Stenosis

    Directory of Open Access Journals (Sweden)

    Reza Bagheri

    2013-05-01

    Full Text Available Introduction: Tracheal stenosis is normally caused by trauma, infection, benign and malignant tumors, prolonged intubation or tracheostomy. The best treatment for tracheal stenosis is resection and anastomosis of trachea. Yet the major surgical complication of tracheal surgery is postoperative stenosis. The goal of this paper is to study the result of tracheal stenting as a replacement therapy for patients suffering from tracheal stenosis who are not good candidates for surgery. Materials and Methods: This study presents the results of stenting in patients with: Inoperable tumoral stenosis,Non-tumoral stenosis being complicated due to prior surgeries,Inability to undergo a major surgery.The study was performed between September 2002 and July 2011 and poly flex stents were used by means of rigid bronchoscopy. Results: A total of 25 patients received stents during this study. Among them 15 patients suffered from benign and 10 suffered from malignant tracheal stenosis. The patients were followed up for at most 12 months after the stenting operation. The mean age of the patients was 35 years. The most common cause of stenosis was prolonged intubation (75%. The most common indication for stenting was the history of multiple tracheal operations. The most common complication of stenting and cause of stent removal was formation of granulation tissue. 30% of patients with benign tracheal stenosis were cured and about 10% improved until they could stand a major operation. Ten patients in benign group and 2 patients in malignant group (20% needed T-Tube insertion after stent removal but other patientcure by stenting. Conclusions: In benign cases stenting is associated with recurrence of symptoms which requires other therapeutic techniqus, so the stenting may not be named as a final solution in benign cases. However, this technique is the only method with approved efficacy for malignant cases with indication.

  9. Canine tracheal epithelial cells are more sensitive than rat tracheal epithelial cells to transforming growth factor beta induced growth inhibition

    International Nuclear Information System (INIS)

    Hubbs, A.F.; Hahn, F.F.; Kelly, G.; Thomassen, D.G.

    1988-01-01

    Transforming growth factor beta (TGFβ) markedly inhibited growth of canine tracheal epithelial (CTE) cells. Reduced responsiveness to TGFβ-induced growth inhibition accompanied neoplastic progression of these cells from primary to transformed to neoplastic. This was similar to the relationship between neoplastic progression and increased resistance to TGFβ-induced growth inhibition seen for rat tracheal epithelial (RTE) cells. The canine cells were more sensitive than rat cells to TGFβ-induced growth inhibition at all stages in the neoplastic process. (author)

  10. Experimental use of new absorbable tracheal stent.

    Science.gov (United States)

    Schopf, Luciano F; Fraga, José Carlos; Porto, Rodrigo; Santos, Luis A; Marques, Douglas R; Sanchez, Paulo R; Meyer, Fabíola S; Ulbrich, Jane M

    2017-11-16

    Silicone and metallic stents are not effective in children with tracheobronchial stenosis or tracheomalacia. Herein, we aimed to evaluate the clinical manifestations and histological reaction of rabbit trachea to the presence of a new poly(lactic-co-glycolic acid) with polyisoprene (PLGA/PI) polymer absorbable stent. Fourteen adult white rabbits (weight, 3.0-3.5kg) were randomly assigned to three groups: Group I (n=6): PLGA/PI spiral stent; Group II (n=6): PLGA/PI fragment; and Group III (n=2): controls. After a longitudinal incision on three cervical tracheal rings, the stents and fragments were inserted into the trachea and fixed onto the lateral wall with nonabsorbable sutures. The stented group showed significantly more stridor at rest (p=0.0041), agitation (p=0.014), and use of accessory muscles (p=0.0002) and required more emergency endoscopies than the fragment group. Further, it showed significantly more remarkable histological inflammatory damage than the fragment and control groups (p=0.002). The new PLGA/PI polymeric stent implanted into the trachea of rabbits caused more clinical manifestations and histologically verified inflammatory reaction than the PLGA/PI polymeric fragment. Future studies should be aimed at reducing the stent-wall thickness. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Successful Treatment of Anterior Tracheal Necrosis after Total Thyroidectomy Using Vacuum-Assisted Closure Therapy

    Directory of Open Access Journals (Sweden)

    Grégory Philippe

    2012-01-01

    Full Text Available Total thyroidectomy involving the adjacent structures of the trachea can cause tracheal damage such as early tracheal necrosis. The authors describe the first case of anterior tracheal necrosis following total thyroidectomy treated using vacuum-assisted closure device. After two weeks of VAC  therapy, there was no evidence of ongoing infection and the trachea was partially closed around a tracheotomy cannula, removed after 3 months. The use of a VAC  therapy to reduce and close the tracheal rent and to create a rapid granulation tissue over tracheal structure appeared as a good opportunity after anterior tracheal necrosis.

  12. [Self-expanding nitinol stents in proximal tracheal stenosis].

    Science.gov (United States)

    Hafner, B; Mann, W; Heussel, C P; Scherhag, A; Schlegel, J; Kauczor, H U

    2000-03-01

    The use of silicone- or metal stents in stenosis of the distal trachea and the bronchial system is a customary procedure [1-4], for example after tumor invasion or cicatricial stenosis after transplantation. In the proximal part of the trachea, on smaller, short and soft strictures we try to stabilise the trachea by the implantation of rings. Other methods are tracheal plasty or transverse tracheal resectomy [5-7]. In the case of longer or nearer subglottal stenoses the positioning of self-expanding nitinol stents has proven a simple, gentle and well-tolerated alternative procedure even in very serious disorders [1, 8, 9]. These stents can be placed in short narcosis under endoscopic control without great strain on the patient. We placed nitinol-stents in the proximal part of the trachea in eleven cases. In five cases dyspnoea caused by a tracheal collapse improved. In two further cases a tracheal stenosis with massive granulation tissue and cicatricial pull under an inlaid tracheal cannula was removed and the tracheostoma was closed. In four cases a solid, scarred and cartilaginous stenosis in the area of the cricoid and the upper tracheal rings was widened with laser and later on stented. Over an observation time of two years no complications showed safe one case in which a directly postoperative dislocation was repositioned quickly. The patients live without restrictions through the tracheal stenosis or a tracheostoma. In the best possible case epithelialization over the metal meshes develops so that a nearly normal mucus transportation is possible [1, 10-12].

  13. Stenting of the right ventricular outflow tract after thrombosis of the modified Blalock-Taussig shunt in a 8 month old infant with tetralogy of Fallot and right pulmonary artery agenesis

    Directory of Open Access Journals (Sweden)

    I. A. Soynov

    2017-01-01

    Full Text Available Pulmonary artery agenesis combined with tetralogy of Fallot is the most rarely seen congenital heart disease. Children with this anomaly are an especially problematic category of patients undergoing staged surgical repair. The postoperative period quite often is complicated with shunt thrombosis while a redo open surgery is associated with a very high risk; therefore, endovascular repair is a preferred procedure. We describe a case of right ventricular outflow tract stenting in a 8 month old girl with tetralogy of Fallot and right pulmonary artery agenesis. The patient was admitted at 3 month after performing of a left-sided modified Blalock-Taussig shunt with severe signs of heart failure and desaturation caused by shunt thrombosis. Assessments performed at 2 months after stenting of the outflow tract demonstrated good oxygen saturation in arterial blood (80% and above and improvement of heart failure symptoms to NYHA II class.

  14. Congenital amusia.

    Science.gov (United States)

    Williamson, Victoria J; Stewart, Lauren

    2013-01-01

    For most people, music, like language, is acquired effortlessly in early life. But a few percent of the population have lifelong difficulties in the perception and production of music. In this chapter we discuss psycho-acoustic and behavioral studies that have attempted to delineate the nature of the auditory perceptual deficits in this group and consider whether these difficulties extend outside the musical domain. Finally, we review structural imaging studies in this group which point to subtle anomalies in temporal and frontal areas. We suggest that amusia can be considered a disorder of neural development, which has relatively specific consequences at the behavioral level. Studies of congenital amusia provide a unique window on the neurocognitive architecture of music processing. Copyright © 2013 Elsevier B.V. All rights reserved.

  15. Vardenafil inhibiting parasympathetic function of tracheal smooth muscle.

    Science.gov (United States)

    Lee, Fei-Peng; Chao, Pin-Zhir; Wang, Hsing-Won

    2018-07-01

    Levitra, a phosphodiesterase-5 (PDE5) inhibitor, is the trade name of vardenafil. Nowadays, it is applied to treatment of erectile dysfunction. PDE5 inhibitors are employed to induce dilatation of the vascular smooth muscle. The effect of Levitra on impotency is well known; however, its effect on the tracheal smooth muscle has rarely been explored. When administered for sexual symptoms via oral intake or inhalation, Levitra might affect the trachea. This study assessed the effects of Levitra on isolated rat tracheal smooth muscle by examining its effect on resting tension of tracheal smooth muscle, contraction caused by 10 -6  M methacholine as a parasympathetic mimetic, and electrically induced tracheal smooth muscle contractions. The results showed that adding methacholine to the incubation medium caused the trachea to contract in a dose-dependent manner. Addition of Levitra at doses of 10 -5  M or above elicited a significant relaxation response to 10 -6  M methacholine-induced contraction. Levitra could inhibit electrical field stimulation-induced spike contraction. It alone had minimal effect on the basal tension of the trachea as the concentration increased. High concentrations of Levitra could inhibit parasympathetic function of the trachea. Levitra when administered via oral intake might reduce asthma attacks in impotent patients because it might inhibit parasympathetic function and reduce methacholine-induced contraction of the tracheal smooth muscle. Copyright © 2018. Published by Elsevier Taiwan LLC.

  16. Failed tracheal intubation using a laryngoscope and intubating laryngeal mask.

    Science.gov (United States)

    Asai, T; Hirose, T; Shingu, K

    2000-04-01

    To report unexpected failed tracheal intubation using a laryngoscope and an intubating laryngeal mask, and difficult ventilation via a facemask, laryngeal mask and intubating laryngeal mask, in a patient with an unrecognized lingual tonsillar hypertrophy. A 63-yr-old woman, who had undergone clipping of an aneurysm seven weeks previously, was scheduled for ventriculo-peritoneal shunt. At the previous surgery, there had been no difficulty in ventilation or in tracheal intubation. Her trachea remained intubated nasally for 11 days after surgery. Preoperatively, her consciousness was impaired. There were no restrictions in head and neck movements or mouth opening. The thyromental distance was 7 cm. After induction of anesthesia, manual ventilation via a facemask with a Guedel airway was suboptimal and the chest expanded insufficiently. At laryngoscopy using a Macintosh or McCoy device, only the tip of the epiglottis, but not the glottis, could be seen, and tracheal intubation failed. There was a partial obstruction during manual ventilation through either the intubating laryngeal mask or conventional laryngeal mask; intubation through each device failed. Digital examination of the pharynx, after removal of the laryngeal mask, indicated a mass occupying the vallecula. Lingual tonsillar hypertrophy (1 x 1 x 2 cm) was found to be the cause of the failure. Awake fibrescope-aided tracheal intubation was accomplished. Unexpected lingual tonsillar hypertrophy can cause both ventilation and tracheal intubation difficult, and neither the laryngeal mask nor intubating laryngeal mask may be helpful in the circumstances.

  17. [The source and factors that influence tracheal pulse oximetry signal].

    Science.gov (United States)

    Fan, Xiao-hua; Wei, Wei; Wang, Jian; Mu, Ling; Wang, Li

    2010-03-01

    To investigate the source and factors that influence tracheal pulse oximetry signal. The adult mongrel dog was intubated after anesthesia. The tracheal tube was modified by attaching a disposable pediatric pulse oximeter to the cuff. The chest of the dog was cut open and a red light from the tracheal oximeter was aligned with the deeper artery. The changes in tracheal pulse oxygen saturation (SptO2) signal were observed after the deeper artery was blocked temporarily. The photoplethysmography (PPG) and readings were recorded at different intracuff pressures. The influence of mechanical ventilation on the signal was also tested and compared with pulse oxygen saturation (SpO2). The SptO2 signal disappeared after deeper artery was blocked. The SptO2 signal changed with different intracuff pressures (P signal appeared under 20-60 cm H2O of intracuff pressure than under 0-10 cm H2O of intracuff pressure(P signal under a condition with mechanical ventilation differed from that without mechanical ventilation (P signal is primarily derived from deeper arteries around the trachea, not from the tracheal wall. Both intracuff pressures and mechanical ventilation can influence SptO2 signal. The SptO2 signal under 20-60 cm H2O of intracuff pressure is stronger than that under 0-10 em H2O of intracuff pressure. Mechanical ventilation mainly changes PPG.

  18. http://www.bioline.org.br/js 104 Penetrating Tracheal Injuries – A ...

    African Journals Online (AJOL)

    jen

    airway and prompt repair is the mainstay of management of penetrating tracheal injuries. Introduction ... tracheal injury was made in the 16th century ... Pearson 7 Harrington8 Sheely9 and Hood10 ..... Ed Am Col Surg (Chicago) .p.391. 12.

  19. Airway injury during emergency transcutaneous airway access: a comparison at cricothyroid and tracheal sites.

    LENUS (Irish Health Repository)

    Salah, Nazar

    2009-12-01

    Oxygenation via the cricothyroid membrane (CTM) may be required in emergencies, but inadvertent tracheal cannulation may occur. In this study, we compared airway injury between the tracheal and CTM sites using different techniques for airway access.

  20. Efficacy of Lower-Extremity Venous Thrombolysis in the Setting of Congenital Absence or Atresia of the Inferior Vena Cava

    International Nuclear Information System (INIS)

    Ganguli, Suvranu; Kalva, Sanjeeva; Oklu, Rahmi; Walker, T. Gregory; Datta, Neil; Grabowski, Eric F.; Wicky, Stephan

    2012-01-01

    Purpose: A rare but described risk factor for deep venous thrombosis (DVT), predominately in the young, is congenital agenesis or atresia of the inferior vena cava (IVC). The optimal management for DVT in this subset of patients is unknown. We evaluated the efficacy of pharmacomechanical catheter-directed thrombolysis (PCDT) followed by systemic anticoagulation in the treatment of acute lower-extremity DVT in the setting of congenital IVC agenesis or atresia. Materials and Methods: Between November of 2005 and May of 2010, six patients (three women [average age 21 years]) were referred to our department with acute lower-extremity DVT and subsequently found to have IVC agenesis or atresia on magnetic resonance imaging. A standardized technique for PCDT (the Angiojet Rheolytic Thrombectomy System followed by the EKOS Microsonic Accelerated Thrombolysis System) was used for all subjects. Successful thrombolysis was followed by systemic heparinization with transition to Coumadin or low molecular-weight heparin and compression stockings. Subjects were followed-up at 1, 3, and then every 6 months after the procedure with clinical assessment and bilateral lower-extremity venous ultrasound. Results: All PCDT procedures were technically successful. No venous stenting or angioplasty was performed. The average thrombolysis time was 28.6 h (range 12–72). Two patients experienced heparin-induced thrombocytopenia, and one patient developed a self-limited knee hemarthrosis, No patients were lost to follow-up. The average length of follow-up was 25.8 ± 20.2 months (range 3.8–54.8). No incidence of recurrent DVT was identified. There were no manifestations of postthrombotic syndrome. Conclusions: PCDT followed by systemic anticoagulation and the use of compression stockings appears to be safe and effective in relatively long-term follow-up treatment of patients who present with acute DVT and IVC agenesis or atresia.

  1. Uterine conserving surgery in a case of cervicovaginal agenesis with unicornuate uterus

    Directory of Open Access Journals (Sweden)

    Vineet Mishra

    2016-01-01

    Full Text Available The presence of cervicovaginal agenesis with unicornuate uterus is a very rare mullerian anomaly. Its true incidence is still unknown. The presence of functioning unicornuate uterus poses a great challenge for a gynecologist because a successful repair could restore normal menses and may preserve a patient′s fertility. Hence, we report a case of 16-year-old unmarried female who presented with chief complaints of primary amenorrhea with cyclical lower abdominal pain. On clinical and radiological evaluation, she was diagnosed with complete cervicovaginal agenesis with right unicornuate uterus (hematometra and hematosalpinx. She underwent vaginoplasty (McIndoes method along with uterovaginal anastomosis by neocervix formation, in order to preserve her uterus. On follow-up, her vagina was completely healed, and she was menstruating normally.

  2. Hereditary motor and sensory neuropathy with agenesis of the corpus callosum.

    Science.gov (United States)

    Dupré, Nicolas; Howard, Heidi C; Mathieu, Jean; Karpati, George; Vanasse, Michel; Bouchard, Jean-Pierre; Carpenter, Stirling; Rouleau, Guy A

    2003-07-01

    Hereditary motor and sensory neuropathy associated with agenesis of the corpus callosum (OMIM 218000) is an autosomal recessive disease of early onset characterized by a delay in developmental milestones, a severe sensory-motor polyneuropathy with areflexia, a variable degree of agenesis of the corpus callosum, amyotrophy, hypotonia, and cognitive impairment. Although this disorder has rarely been reported worldwide, it has a high prevalence in the Saguenay-Lac-St-Jean region of the province of Quebec (Canada) predominantly because of a founder effect. The gene defect responsible for this disorder recently has been identified, and it is a protein-truncating mutation in the SLC12A6 gene, which codes for a cotransporter protein known as KCC3. Herein, we provide the first extensive review of this disorder, covering epidemiological, clinical, and molecular genetic studies.

  3. A rare case of Turner′s syndrome presenting with Mullerian agenesis

    Directory of Open Access Journals (Sweden)

    Suresh Vaddadi

    2013-01-01

    Full Text Available Turner′s syndrome also called as Ullrich Turner′s syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner′s syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina.

  4. Dental root agenesis following radiation and antineoplastic therapy: A Case Report

    Directory of Open Access Journals (Sweden)

    Abdul Hafiz

    2016-01-01

    Full Text Available The survival rates of patients suffering from various childhood neoplasms have improved dramatically with the advent of chemo-radiation therapy. The harmful effects of chemo-radiation therapy in the oro-facial region such as root agenesis, short roots, impaired amelogenesis, dentinogenesis, radiation caries, and other soft tissue pathologies are well recognized. In spite of these documented risks, the antineoplastic treatment modalities continue to serve the patient for overall improvement in survival and quality of life. However, a thorough understanding of the growth and development process and its relation with the complex antineoplastic treatment is very important for all clinicians. Such awareness could significantly improve the status of patients in the posttreatment period with the implementation of proper preventive and interceptive measures. This article intends to document a case of root agenesis that developed secondary to chemo-radiation therapy in a 12-year-old girl.

  5. Agenesis of the dorsal pancreas: systematic review of a clinical challenge

    Directory of Open Access Journals (Sweden)

    Javier A. Cienfuegos

    Full Text Available Background: Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015. Methods: A systematic review of the Medline and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults. Conclusions: Although dorsal pancreatic agenesis is a rare malformation, given its association with non-alcoholic pancreatitis and neoplasia of the residual pancreas, physicians should maintain an expectant attitude.

  6. A case of Klippel-Feil syndrome with renal agenesis

    Directory of Open Access Journals (Sweden)

    Mehmet Karakoç

    2010-05-01

    Full Text Available Klippel-Feil syndrome (KFS is is a rare syndrome that characterized by fusion of at least two congenital vertebrae in the cervical region. The most common characteristics of KFS are short neck, low nuchal hair line and restricted joint mobility of the cervical region.Addititonal features are scoliosis, sipina bifida, cervical rib, Sprengel deformity and facial asymmetry, and renal and cardiac anomalies. In this report a 10 years old girl was admitted with complaints of short stature and abnormal neck curvature. Physical examination of the patient revealed short neck, low nuchal hair line, and Sprengel’s deformity, height hard palate, facial asymmetry and torticollis. Cervical paravertebral muscle spasm was found on the left as (+ / right (+ +. Muscle tone, muscle strength, reflexes and sensory examination were found to be normal. Radiological examination of the C4-5 and C6-7 cervical region revealed the loss of inteverebral disc spaces and block vertebra. Also there was hemivertebra anomaly and aplasia of odontoid process. Abdominal and pelvic ultrasound examination revelaed absence of right kidney. Echocardiographical examination gave no abnormality. The case was presented in order to draw attention to KFS with renal agenesia in the light of current literature.

  7. Caudal Regression Syndrome with Partial Agenesis of the Corpus callosum and Partial Lobar Holoprosencephaly

    Science.gov (United States)

    Hashami, Hilal Al; Bataclan, Maria F; Mathew, Mariam; Krishnan, Lalitha

    2010-01-01

    Caudal regression syndrome is a rare fetal condition of diabetic pregnancy. Although the exact mechanism is not known, hyperglycaemia during embryogenesis seems to act as a teratogen. Independently, caudal regression syndrome (CRS), agenesis of the corpus callosum (ACC) and partial lobar holoprosencephaly (HPE) have been reported in infants of diabetic mothers. To our knowledge, a combination of all these three conditions has not been reported so far. PMID:21509087

  8. Caudal Regression Syndrome with Partial Agenesis of the Corpus callosum and Partial Lobar Holoprosencephaly: Case report.

    Science.gov (United States)

    Hashami, Hilal Al; Bataclan, Maria F; Mathew, Mariam; Krishnan, Lalitha

    2010-04-01

    Caudal regression syndrome is a rare fetal condition of diabetic pregnancy. Although the exact mechanism is not known, hyperglycaemia during embryogenesis seems to act as a teratogen. Independently, caudal regression syndrome (CRS), agenesis of the corpus callosum (ACC) and partial lobar holoprosencephaly (HPE) have been reported in infants of diabetic mothers. To our knowledge, a combination of all these three conditions has not been reported so far.

  9. Learning and memory in individuals with agenesis of the corpus callosum

    OpenAIRE

    Paul, Lynn K.; Erickson, Roger L.; Hartman, Jo Ann; Brown, Warren S.

    2016-01-01

    Damage to long white matter pathways in the cerebral cortex is known to affect memory capacity. However, the specific contribution of interhemispheric connectivity in memory functioning is only beginning to become understood. The present study examined verbal and visual memory processing in individuals with agenesis of the corpus callosum (AgCC) using the Wechsler Memory Scale-Third Edition (WMS-III; Wechsler, 1997b). Thirty participants with AgCC (FSIQ >78) were compared against 30 healthy a...

  10. Uterus didelphys with partial vaginal septum and distal vaginal agenesis: an unusual anomaly

    International Nuclear Information System (INIS)

    Singhal, S.R.; Lakra, P.; Bishnoi, P.; Rohilla, S.; Dahiya, P.

    2013-01-01

    Complex malformations of female genital tract are not so common. Their correct identification is of paramount importance for appropriate management. A thorough knowledge of embryology, pre-operative imaging with MRI and examination under anaesthesia is essential to identify accurately the constellation of anomalies and to plan appropriate management. This case reports the coexistence of Mullerian abnormality with vaginal agenesis in an 18 years old girl which was managed by dissecting the lower half of vagina and pull-through vaginoplasty. (author)

  11. Tracheal palpation to assess endotracheal tube depth: an exploratory study.

    Science.gov (United States)

    McKay, William P; Klonarakis, Jim; Pelivanov, Vladko; O'Brien, Jennifer M; Plewes, Chris

    2014-03-01

    Correct placement of the endotracheal tube (ETT) occurs when the distal tip is in mid-trachea. This study compares two techniques used to place the ETT at the correct depth during intubation: tracheal palpation vs placement at a fixed depth at the patient's teeth. With approval of the Research Ethics Board, we recruited American Society of Anesthesiologists physical status I-II patients scheduled for elective surgery with tracheal intubation. Clinicians performing the tracheal intubations were asked to "advance the tube slowly once the tip is through the cords". An investigator palpated the patient's trachea with three fingers spread over the trachea from the larynx to the sternal notch. When the ETT tip was felt in the sternal notch, the ETT was immobilized and its position was determined by fibreoptic bronchoscopy. The position of the ETT tip was compared with our hospital standard, which is a depth at the incisors or gums of 23 cm for men and 21 cm for women. The primary outcome was the incidence of correct placement. Correct placement of the ETT was defined as a tip > 2.5 cm from the carina and > 3.5 cm below the vocal cords. Movement of the ETT tip was readily palpable in 77 of 92 patients studied, and bronchoscopy was performed in 85 patients. Placement by tracheal palpation resulted in more correct placements (71 [77%]; 95% confidence interval [CI] 74 to 81) than hospital standard depth at the incisors or gums (57 [61%]; 95% CI 58 to 66) (P = 0.037). The mean (SD) placement of the ETT tip in palpable subjects was 4.1 (1.7) cm above the carina, 1.9 cm (1.5-2.3 cm) below the ideal mid-tracheal position. Tracheal palpation requires no special equipment, takes only a few seconds to perform, and may improve ETT placement at the correct depth. Further studies are warranted.

  12. Two-piece cryopreserved tracheal allotransplantation: an experimental study.

    Science.gov (United States)

    Iyikesici, Tuncel; Tuncozgur, Bulent; Sanli, Maruf; Isik, Ahmet Feridun; Meteroglu, Fatih; Elbeyli, Levent

    2009-10-01

    For successful reconstruction with tracheal allotransplants following long tracheal resections, problems related to the preservation and vascularisation of the tracheal graft have to be solved. In this study, instead of using a long-segment single-piece graft, we used a graft that has been split into two. The aim was to use this graft after cryopreservation in order to ease neo-vascularisation and to maintain tracheal integrity by transplanting it to two separate regions of the dog cervical trachea. This experimental study was conducted in animal laboratories of the medical school on 11 half-blood dogs. The trachea obtained from the first dog was 8 cm in length; it was split into two pieces of 4 cm each and stored in the preservation solution at -80 degrees C for 4 weeks. Following this, the dog was sacrificed. Two 2 cm portions of cervical trachea were excised from the second dog. These parts were then reconstructed with two tracheal grafts of the same length as the cryopreserved ones. Ten dogs that were grouped into five groups of two dogs each underwent the same procedure. The subjects had a bronchoscopic evaluation on the third postoperative week. Anastomosis regions of the test tracheas were resected to be examined histopathologically. Seven subjects were found to have third-degree obstructions during bronchoscopy; two had close to fourth-degree obstructions. In the histopathological examination, contrary to the findings of the bronchoscopies, 75% of the anastomoses had intact epithelium. The cartilage was seen to have well-preserved structural characteristics in all the anastomoses. Twelve anastomoses had moderate, seven mild and one had severe inflammation. All anastomoses had either good or very good level of vascularisation. The integrity of the tracheal epithelium can be maintained with cryopreservation and split anastomosis technique. The cartilage preserves its structural characteristics despite losing its viability, thereby offering an advantage to

  13. Stent placement for tracheal stenosis in patients with esophageal cancer

    International Nuclear Information System (INIS)

    Takagi, Keigo; Hata, Yoshinobu; Sasamoto, Shuichi; Takahashi, Shoji; Sato, Fumitomo; Tamaki, Kazuyoshi; Goto, Hidenori; Yuasa, Rena

    2011-01-01

    Tracheal invasion including tracheal bifurcation due to esophageal cancer can sometimes cause serious complications of the airway, but such cases sometimes improve quickly following chemoradiation treatment. The absolute indications for stent replacement in the airway for this disorder and the optimal choice of stent are herein discussed. Between 1992 and 2010, 28 patients with airway stenosis, including 7 patients with esophago-tracheal fistula, were treated by placement of various stents; namely, 12 patients received Dumon stents, 3 patients had Dynamic stents, 10 patients were given Ultraflex stents, while 3 other patients were treated without the use of stents. Severe dyspnea in the supine position was observed, which mainly originated from invasion to the membranous portion of the trachea. Airway patency was maintained after stent replacement, although the median survival time of such cases was only 4 months. Three patients with severe dyspnea who could lie in a supine position recovered after undergoing chemoradiation treatment without stent replacement. No cases of stent removal were observed after chemoradiation treatment. Chemoradiation treatment for esophageal cancer was found to be effective for the management of airway disturbances, and thus the absolute indications for stent replacement are restricted to patients who cannot lie in a supine position due to severe impairment of ventilation during radiation therapy, as well as patients presenting with tracheobroncho-esophageal fistula. Concerning stent selection, a metal stent should be the first choice for tracheal stenosis due to its ease of insertion, because there is no substantial difference between silicone and metal stents regarding the treatment of tracheal stenosis. However, it is important to note that a silicone Y stent is useful for the treatment of tracheal bifurcation. (author)

  14. Surgery for postintubation tracheal and tracheosubglottic stenotic lesions

    International Nuclear Information System (INIS)

    Ashour, M.; Al-Kattan, K.; Rafay, M.A.; El-Bakry, A.K.; El-Dawlatly, A.; Naguib, M.; Seraj, M.; Joharjy, I.; Al-Serhani, A.

    1996-01-01

    Postintubation tracheal stenosis is a recognized problem. Although its incidence has recently decreased, it is still a difficult complication to treat. We have reviewed our experience with 10 patients with tracheal stenosis over the last five years between 1990 and 1995. There were seven male and three female patients with an average age of 14.2+-4 years (range 6 to 48 years). Resection and reconstruction with primary anastomosis was performed in seven patients, while conservative treatment with dilation was performed in two patients. One patient refused surgery. Operations performed included resection of tracheocricoid segment with tracheothyroid anastomosis (N=3) and tracheal resection with end-to-end anastomosis (N=4). The resected airway ranged from 3 cm to 6 cm. In view of the intense inflammatory and fibrotic process in and around stenotic segment, the practice of tracheostomy for the relief of postintubation acute tracheal obstruction should not be taken lightly, as it adds not only to the severity of the inflammatory process, but also increases the length of the tracheal segment to be resected. Postoperatively, all patients were extubated; this was accomplished by the end of surgery in six patients, while the seventh patient was extubated three weeks later. There was no mortality in this series. When normal functional activity and airway patency were taken as two parameters to judge the outcome of the surgery, results were good in six (86%) patients and satisfactory in one. These results support the validity of the one-stage reconstruction approach as one alternative for the treatment of postintubation tracheal and tracheosubglottic stenotic lesions. (author)

  15. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.

    Science.gov (United States)

    Fedele, L; Motta, F; Frontino, G; Restelli, E; Bianchi, S

    2013-06-01

    What are the anatomic variants (and their frequencies) of double uterus, obstructed hemivagina and ipsilateral renal agenesis? Most cases examined (72.4%) were of the classic anatomic variant of the Herlyn-Werner-Wunderlich syndrome (with didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis) but the 27.6% of cases are of a rare variant of the syndrome (with uterus septum or cervical agenesis), showing relevant clinical and surgical implications. The extreme variability of anatomic structures involved in this syndrome (both uterus, cervico-vaginal and renal anomalies) is well known, even if a complete and uniform analysis of all its heterogeneous presentations in a large series is lacking. This is a retrospective study with 87 patients referred to our third level referral center between 1981 and 2011. We analyzed the laparoscopic and chart records of 87 women, who referred to our institute with double uterus, unilateral cervico-vaginal obstruction and ipsilateral renal anomalies. Sixty-three of 87 patients had the more classic variant of didelphys uterus with obstructed hemivagina; 10/87 patients had septate bicollis uterus with obstructed hemivagina; 9/87 patients had bicornuate bicollis uterus with obstructed hemivagina; 4/87 patients had didelphys uterus with unilateral cervical atresia; 1/87 patients had bicornuate uterus with one septate cervix and unilateral obstructed hemivagina. This is a retrospective study with a long enrolling period (30 years). New insights in the anatomic variants of this rare syndrome with their relevant surgical implications.

  16. Effect of Vestibulo-Proprioceptive Stimulations in a Child with Agenesis of the Corpus Callosum

    Directory of Open Access Journals (Sweden)

    Hamid Dalvand

    2010-06-01

    Full Text Available Background and Aim: The purpose of the present study was to investigate the effect of vestibulo-proprioceptive stimulations of sensory integration theory on the development of gross and fine motor, language and personal-social functions in a child with agenesis of the corpus callosum.Case: We report a 10.5 month old boy with agenesis of the corpus callosum. The intervention was administered based on sensory integration theory an hour a week for 20 weeks. The exercise intervention consisted of proprioceptive and linear, sustained and low frequency vestibular stimulations on suspension device and physio roll. A Denver Developmental Screening- II and milestones skill testing was completed pre-intervention and monthly. Post-intervention, age of gross motor, fine motor adaptive, language, and personal-social functions significantly improved. Based on milestones skills, maintenance of gross motor functions (e.g. sitting and quadruped position improved. The child could roll from side to side and released objects voluntarily. The reaction time to auditory stimulations became less than 2 seconds.Conclusion: vestibulo-proprioceptive stimulations using the neuroplasticity ability of the central nervous system is effective for development of gross and fine motor, language, and personal-social functions. These exercises can be administered for a child with agenesis of the corpus callosum.

  17. Use of physical therapy to augment dilator treatment for vaginal agenesis.

    Science.gov (United States)

    McVearry, Mary E; Warner, William B

    2011-05-01

    : Dilator therapy has been used successfully for many years to treat vaginal agenesis. Our objective was to show how established physical therapy techniques can be used to augment dilator therapy. : A 36-year-old woman desiring nonsurgical management of vaginal agenesis was instructed in the use of vaginal dilators by a pelvic-floor physical therapist. Manual stretching of the vaginal tissues was performed during office visits after application of heat and therapeutic ultrasound to the perineum. In addition, the patient's husband was taught how to perform the vaginal stretching at home in conjunction with dilator therapy. : The patient was able to attempt intercourse after 6 weeks of treatment and achieved full penetration by 9 weeks. At the completion of treatment, she scored 31.9 on the Female Sexual Function Index. The patient and her husband were very satisfied with the treatment approach, especially the encouragement and guidance received in physical therapy. : By using established physical therapy techniques in conjunction with dilator therapy, a faster time to intercourse may be achieved with high patient and spouse satisfaction. We recommend the involvement of a physical therapist specializing in the pelvic floor as an adjunct to standard dilator therapy in the treatment of vaginal agenesis.

  18. [Congenital hypothyroidism].

    Science.gov (United States)

    Castilla Peón, María Fernanda

    Congenital hypothyroidism (CH) is a cause of preventable mental retardation; therefore, timely diagnosis and treatment by the primary care physician is very important. CH screening must be performed between the second and fifth days of life with capillary blood done with a heel prick and must be confirmed by measurement of thyroid hormones in venous blood. The most common cause of CH is thyroid dysgenesis, which may be identified by a thyroid scan carried out before initiating treatment. Treatment should be with levothyroxine (10-15μg/kg/day) and should not be delayed or suspended during the first 3 years of life due to the deleterious effect on neurodevelopment in case of low thyroid hormones during this time. Preterm or sick infants or those with Down syndrome require special consideration. This article provides diagnostic and therapeutic algorithms for CH. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  19. Tracheal dimness as a sign of mediastinal pathology

    International Nuclear Information System (INIS)

    Schiavon, F.; Nardini, S.; Giannico, S.

    1987-01-01

    Some cases of mediastinal pathology in which the only pathological pattern was a dimness of the tracheal transparency are described. This sign is not described in previous report and is described as a short break in the aerial tracheogram as seen on the frontal roentgenograph. This sign is produced by an increase in the structures outside the trachea or by a decrease in the air column inside the trachea. Conventional and CT anatomic findings which account for the sign are discussed. A short review of the normal causes of tracheal dimness is presented. This sign may be useful expecially in emergency radiology, since it provides additional information to a simple routine chest roentgenograph

  20. A new retrograde transillumination technique for videolaryngoscopic tracheal intubation

    DEFF Research Database (Denmark)

    Biro, P; Fried, E; Schlaepfer, M

    2018-01-01

    This single-centre, prospective trial was designed to assess the efficacy of a new retrograde transillumination device called the 'Infrared Red Intubation System' (IRRIS) to aid videolaryngoscopic tracheal intubation. We included 40 adult patients, who were undergoing elective urological surgery......-10])), credibility (10 (8-10 [5-10])) and ease of use (10 (9-10 [8-10])). Tracheal intubation with the system lasted 26 (16-32 [6-89]) s. No alternative technique of securing the airway was necessary. The lowest SpO2 during intubation was 98 (97-99 [91-100])%. We conclude that this method of retrograde...

  1. Tracheal adenoid cystic carcinoma masquerading asthma: A case report

    Directory of Open Access Journals (Sweden)

    Kurul Cuneyt

    2004-10-01

    Full Text Available Abstract Background Tracheal tumors are often misdiagnosed as asthma and are treated with inhaled steroids and bronchodilators without resolution. Case Presentation Here, a patient with tracheal adenoid cystic carcinoma who had been previously diagnosed with difficult asthma was reported. The possibility of the presence of localized airway obstruction was raised when the flow-volume curve suggesting fixed airway obstruction, was obtained. Conclusion The presenting case report emphasizes the fact that not all wheezes are asthma. It is critical to bear in mind that if a patient does not respond to appropriate anti-asthma therapy, localized obstructions should be ruled out before establishing the diagnosis of asthma.

  2. [Congenital abnormalities of the aorta in children and adolescents].

    Science.gov (United States)

    Eichhorn, J G; Ley, S

    2007-11-01

    Aortic abnormalities are common cardiovascular malformations accounting for 15-20% of all congenital heart disease. Ultrafast CT and MR imaging are noninvasive, accurate and robust techniques that can be used in the diagnosis of aortic malformations. While their sensitivity in detecting vascular abnormalities seems to be as good as that of conventional catheter angiocardiography, at over 90%, they are superior in the diagnosis of potentially life-threatening complications, such as tracheal, bronchial, or esophageal compression. It has been shown that more than 80% of small children with aortic abnormalities benefit directly from the use of noninvasive imaging: either cardiac catheterization is no longer necessary or radiation doses and periods of general anesthesia for interventional catheterization procedures can be much reduced. The most important congenital abnormalities of the aorta in children and adolescents are presented with reference to examples, and the value of CT and MR angiography is documented.

  3. Incidence of Congenital Spinal Abnormalities Among Pediatric Patients and Their Association With Scoliosis and Systemic Anomalies.

    Science.gov (United States)

    Passias, Peter G; Poorman, Gregory W; Jalai, Cyrus M; Diebo, Bassel G; Vira, Shaleen; Horn, Samantha R; Baker, Joseph F; Shenoy, Kartik; Hasan, Saqib; Buza, John; Bronson, Wesley; Paul, Justin C; Kaye, Ian; Foster, Norah A; Cassilly, Ryan T; Oren, Jonathan H; Moskovich, Ronald; Line, Breton; Oh, Cheongeun; Bess, Shay; LaFage, Virginie; Errico, Thomas J

    2017-10-09

    Congenital abnormalities when present, according to VACTERL theory, occur nonrandomly with other congenital anomalies. This study estimates the prevalence of congenital spinal anomalies, and their concurrence with other systemic anomalies. A retrospective cohort analysis on Health care Cost and Utilization Project's Kids Inpatient Database (KID), years 2000, 2003, 2006, 2009 was performed. ICD-9 coding identified congenital anomalies of the spine and other body systems. Overall incidence of congenital spinal abnormalities in pediatric patients, and the concurrence of spinal anomaly diagnoses with other organ system anomalies. Frequencies of congenital spine anomalies were estimated using KID hospital-and-year-adjusted weights. Poisson distribution in contingency tables tabulated concurrence of other congenital anomalies, grouped by body system. Of 12,039,432 patients, rates per 100,000 cases were: 9.1 hemivertebra, 4.3 Klippel-Fiel, 56.3 Chiari malformation, 52.6 tethered cord, 83.4 spina bifida, 1.2 absence of vertebra, and 6.2 diastematomyelia. Diastematomyelia had the highest concurrence of other anomalies: 70.1% of diastematomyelia patients had at least one other congenital anomaly. Next, 63.2% of hemivertebra, and 35.2% of Klippel-Fiel patients had concurrent anomalies. Of the other systems deformities cooccuring, cardiac system had the highest concurrent incidence (6.5% overall). In light of VACTERL's definition of a patient being diagnosed with at least 3 VACTERL anomalies, hemivertebra patients had the highest cooccurrence of ≥3 anomalies (31.3%). With detailed analysis of hemivertebra patients, secundum ASD (14.49%), atresia of large intestine (10.2%), renal agenesis (7.43%) frequently cooccured. Congenital abnormalities of the spine are associated with serious systemic anomalies that may have delayed presentations. These patients continue to be at a very high, and maybe higher than previously thought, risk for comorbidities that can cause devastating

  4. Congenital platelet function defects

    Science.gov (United States)

    ... pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital ... Congenital platelet function defects are bleeding disorders that cause reduced platelet function. Most of the time, people with these disorders have ...

  5. Congenital Heart Information Network

    Science.gov (United States)

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  6. Congenital heart disease

    Science.gov (United States)

    Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth. ... Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, ... Sabiston Textbook of Surgery: The Biological Basis of Modern ...

  7. Tracheoesophageal Fistula due to a Damaged Tracheal Stent

    Directory of Open Access Journals (Sweden)

    Masahiro Kimura

    2014-01-01

    Full Text Available We describe the management of a tracheoesophageal fistula due to a damaged tracheal stent, which was first inserted to treat tracheal stenosis. A 29-year-old woman with a history of treated epilepsy had a seizure and suffered from smoke inhalation during a fire. Breathing difficulties appeared and gradually worsened; consultation was obtained two years afterward. After undergoing a thorough examination, the patient was diagnosed with tracheal strangulation. A noncovered, metallic stent was inserted. When the patient was 37 years old, she was admitted to our hospital for the treatment of a tracheoesophageal fistula. We diagnosed it as a tracheoesophageal fistula due to the collapse of the damaged tracheal stent toward the esophageal side, and we decided to perform a mediastinal tracheostomy. Granulation may be formed in the circumference of a stent that has been present for a prolonged period, and removal of the stent may become difficult. This case suggests that insertion of a noncovered, metallic stent is contraindicated for a benign disease.

  8. Tubeless tracheal resection and reconstruction for management of benign stenosis.

    Science.gov (United States)

    Caronia, Francesco Paolo; Loizzi, Domenico; Nicolosi, Tommaso; Castorina, Sergio; Fiorelli, Alfonso

    2017-12-01

    We reported a tubeless tracheal resection and reconstruction for the management of benign posttracheostomy tracheal stenosis. A 34-year-old man with stridor, severe respiratory distress, and recurrent pneumonia was referred to our attention for treatment of benign posttracheostomy tracheal stenosis. As he refused general anesthesia, the procedure was performed while he was under local anesthesia and spontaneous ventilation. Sedation was started with infusion of dexmedetomidine 0.7 mg/kg/min and of remifentanil 0.5 mg/kg/h; also, 40%-50% oxygen was delivered using a laryngeal mask at a rate of 3.5 mL/min. An additional dose of 2% lidocaine was injected into the surgical site during the operation to achieve an adequate level of anesthesia. A standard resection and reconstruction of trachea was carried out and no recurrence was found in the follow-up of 41 months. Tubeless tracheal surgery seems to be a feasible and safe procedure. Larger prospective series should validate our results. © 2017 Wiley Periodicals, Inc.

  9. Laryngeal Preservation in Managing Advanced Tracheal Adenoid Cystic Carcinoma

    Directory of Open Access Journals (Sweden)

    Thavakumar Subramaniam

    2015-01-01

    Full Text Available A 37-year-old male athlete was diagnosed with primary tracheal adenoid cystic carcinoma following investigation for dyspnea, wheeze, and eventual stridor. Preoperative bronchoscopy revealed a highly vascular tumor 4 cm distal to the cricoid with no gross disease extending to the carina. Imaging revealed circumferential tracheal irregularity immediately inferior to the cricoid, with no definite cricoid invasion. Locoregional extension of disease was noted invading the thyroid and abutment of the carotid approximately 180°. Intraoperative findings identified tracheal mucosal disease extending distal to the carina and proximally at the cricothyroid joints where bilateral functional recurrent nerves were preserved. A decision made to preserve the larynx given the inability to fully resect distal tracheal disease. A 5 cm sleeve resection of the trachea was made with a cricotracheal anastomosis following suprahyoidal muscle release and laryngeal drop-down. The patient was treated with adjuvant radiotherapy including platinum based chemotherapy in an effort to maximise local control. PET scanning three months after therapy revealed no FDG uptake locally or distally.

  10. Congenital Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Woong Hee; Kim, Young Tong; Jou, Sung Shick; Shin, Hyeong Cheol [Soonchunhyang University, Asan (Korea, Republic of)

    2008-12-15

    Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

  11. [Circular tracheal resection for cicatrical stenosis and functioning tracheostomy].

    Science.gov (United States)

    Parshin, V D; Titov, V A; Parshin, V V; Parshin, A V; Berikkhanov, Z; Amangeldiev, D M

    To analyze the results of tracheal resection for cicatricial stenosis depending on the presence of tracheostomy. 1128 patients with tracheal cicatricial stenosis were treated for the period 1963-2015. The first group consisted of 297 patients for the period 1963-2000, the second group - 831 patients for the period 2001-2015. Most of them 684 (60.6%) were young and able-bodied (age from 21 to 50 years). In the first group 139 (46.8%) out of 297 patients had functioning tracheostomy. For the period 2001-2015 tracheostomy was made in 430 (51.7%) out of 831 patients with cicatricial stenosis. Time of cannulation varied from a few weeks to 21 years. Re-tracheostomy within various terms after decanulation was performed in 68 (15.8%) patients. Tracheal resection with anastomosis was performed in 59 and 330 in both groups respectively. At present time these operations are performed more often in view of their standard fashion in everyday practice. In the second group tracheal resection followed by anastomosis was observed in 110 (25.6%) out of 430 patients with tracheostomy that is 4.4 times more often than in previous years. In total 2 patients died after 330 circular tracheal resections within 2001-2015 including one patient with and one patient without tracheostomy. Mortality was 0.6%. Moreover, this value was slightly higher in patients operated with a functioning tracheostomy compared with those without it - 0.9 vs. 0.5% respectively. The causes of death were bleeding into tracheobronchial lumen and pulmonary embolism. The source of bleeding after tracheal resection was innominate artery. Overall incidence of postoperative complications was 2 times higher in tracheostomy patients compared with those without it - 22 (20%) vs. 26 (11.8%) cases respectively. Convalescence may be achieved in 89.8% patients after circular tracheal resection. Adverse long-term results are associated with postoperative complications. So their prevention and treatment will improve the

  12. An ovine tracheal explant culture model for allergic airway inflammation

    Directory of Open Access Journals (Sweden)

    Abeynaike Latasha

    2010-08-01

    Full Text Available Abstract Background The airway epithelium is thought to play an important role in the pathogenesis of asthmatic disease. However, much of our understanding of airway epithelial cell function in asthma has been derived from in vitro studies that may not accurately reflect the interactive cellular and molecular pathways active between different tissue constituents in vivo. Methods Using a sheep model of allergic asthma, tracheal explants from normal sheep and allergic sheep exposed to house dust mite (HDM allergen were established to investigate airway mucosal responses ex vivo. Explants were cultured for up to 48 h and tissues were stained to identify apoptotic cells, goblet cells, mast cells and eosinophils. The release of cytokines (IL-1α, IL-6 and TNF-α by cultured tracheal explants, was assessed by ELISA. Results The general morphology and epithelial structure of the tracheal explants was well maintained in culture although evidence of advanced apoptosis within the mucosal layer was noted after culture for 48 h. The number of alcian blue/PAS positive mucus-secreting cells within the epithelial layer was reduced in all cultured explants compared with pre-cultured (0 h explants, but the loss of staining was most evident in allergic tissues. Mast cell and eosinophil numbers were elevated in the allergic tracheal tissues compared to naïve controls, and in the allergic tissues there was a significant decline in mast cells after 24 h culture in the presence or absence of HDM allergen. IL-6 was released by allergic tracheal explants in culture but was undetected in cultured control explants. Conclusions Sheep tracheal explants maintain characteristics of the airway mucosa that may not be replicated when studying isolated cell populations in vitro. There were key differences identified in explants from allergic compared to control airways and in their responses in culture for 24 h. Importantly, this study establishes the potential for the

  13. Implantation of Induced Pluripotent Stem Cell-Derived Tracheal Epithelial Cells.

    Science.gov (United States)

    Ikeda, Masakazu; Imaizumi, Mitsuyoshi; Yoshie, Susumu; Nakamura, Ryosuke; Otsuki, Koshi; Murono, Shigeyuki; Omori, Koichi

    2017-07-01

    Compared with using autologous tissue, the use of artificial materials in the regeneration of tracheal defects is minimally invasive. However, this technique requires early epithelialization on the inner side of the artificial trachea. After differentiation from induced pluripotent stem cells (iPSCs), tracheal epithelial tissues may be used to produce artificial tracheas. Herein, we aimed to demonstrate that after differentiation from fluorescent protein-labeled iPSCs, tracheal epithelial tissues survived in nude rats with tracheal defects. Red fluorescent tdTomato protein was electroporated into mouse iPSCs to produce tdTomato-labeled iPSCs. Embryoid bodies derived from these iPSCs were then cultured in differentiation medium supplemented with growth factors, followed by culture on air-liquid interfaces for further differentiation into tracheal epithelium. The cells were implanted with artificial tracheas into nude rats with tracheal defects on day 26 of cultivation. On day 7 after implantation, the tracheas were exposed and examined histologically. Tracheal epithelial tissue derived from tdTomato-labeled iPSCs survived in the tracheal defects. Moreover, immunochemical analyses showed that differentiated tissues had epithelial structures similar to those of proximal tracheal tissues. After differentiation from iPSCs, tracheal epithelial tissues survived in rat bodies, warranting the use of iPSCs for epithelial regeneration in tracheal defects.

  14. Novel Insights into the Pathogenesis of Monogenic Congenital Anomalies of the Kidney and Urinary Tract.

    Science.gov (United States)

    van der Ven, Amelie T; Vivante, Asaf; Hildebrandt, Friedhelm

    2018-01-01

    Congenital anomalies of the kidneys and urinary tract (CAKUT) comprise a large spectrum of congenital malformations ranging from severe manifestations, such as renal agenesis, to potentially milder conditions, such as vesicoureteral reflux. CAKUT causes approximately 40% of ESRD that manifests within the first three decades of life. Several lines of evidence indicate that CAKUT is often caused by recessive or dominant mutations in single (monogenic) genes. To date, approximately 40 monogenic genes are known to cause CAKUT if mutated, explaining 5%-20% of patients. However, hundreds of different monogenic CAKUT genes probably exist. The discovery of novel CAKUT-causing genes remains challenging because of this pronounced heterogeneity, variable expressivity, and incomplete penetrance. We here give an overview of known genetic causes for human CAKUT and shed light on distinct renal morphogenetic pathways that were identified as relevant for CAKUT in mice and humans. Copyright © 2018 by the American Society of Nephrology.

  15. Position and Eruption of Permanent Maxillary Canines in Cases of Maxillary Lateral Incisor Agenesis in Mixed Dentition.

    Science.gov (United States)

    Lamas, Cátia; Lavall, Asela; Pinho, Teresa

    2018-01-01

    Assess whether the permanent maxillary canine (MC) has a natural tendency to erupt mesially in children with maxillary lateral incisors agenesis (MLIA), compared to children without agenesis. This retrospective, observational, cross-sectional study consisted of children between 5 and 12 years old divided into three groups: the first group with unilateral MLIA, in which an intraindividual analysis was performed, the second group presented bilateral MLIA, and the third group with patients without agenesis. These last two groups were matched for comparison interindividual, being pared by sex and maturation of the MC. The canine position in the horizontal sector showed a clear mesial positioning of the MC on the agenesis side in individuals with unilateral MLIA (group 1) when compared with the counter lateral side; and in individuals with bilateral MLIA (Group 2) compared with control Individuals without agenesis (group 3). Even with the maintenance of this deciduous tooth in the dental arch, the MC keeps its tendency to mesial eruption. There is a greater tendency for mesial angulation of the maxillary canine in patients with MLIA, regardless of the presence or absence of deciduous lateral incisor.

  16. Tracheal and Crico-Tracheal Resection and Anastomosis for Malignancies Involving the Thyroid Gland and the Airway.

    Science.gov (United States)

    Piazza, Cesare; Del Bon, Francesca; Barbieri, Diego; Grazioli, Paola; Paderno, Alberto; Perotti, Pietro; Lombardi, Davide; Peretti, Giorgio; Nicolai, Piero

    2016-02-01

    To evaluate outcomes in different malignancies involving the thyroid and infiltrating the airway submitted to tracheal (TRA) or crico-tracheal resection and anastomosis (CTRA). Retrospective charts review of 27 patients affected by thyroid malignancies involving the airway treated by TRA/CTRA in a single academic institution. Kaplan-Meier curves were used to evaluate the overall (OS) and disease-specific (DSS) survivals and local (LC) and loco-regional control (LRC). Impact on survival of age, comorbidities, previous radiotherapy, types of TRA/CTRA, Shin's stage (II, III, IV), grading (well vs poorly differentiated), and length of airway resected was calculated by the log-rank test. Overall survival and DSS at 3 and 5 years were 82.3% and 71.6%, respectively. Local control and LRC in the entire group were 82.3% at 3 and 5 years. Crico-tracheal resection and anastomosis involving the cricoid arch and plate (type C) and tumor differentiation significantly affected OS and DSS (both P < .001). Type C CTRA and tumor differentiation significantly impacted on LC (P = .002 and P = .009, respectively). Grading and extension of CTRA to the cricoid plate are the most important factors for oncologic outcomes in thyroid malignancies infiltrating the airway. Except for poorly differentiated tumors, TRA/CTRA allows adequate LC even in advanced stage lesions involving the crico-tracheal junction. © The Author(s) 2015.

  17. Lung hypoplasia and its associated major congenital abnormalities in perinatal death: an autopsy study of 850 cases.

    Science.gov (United States)

    Aghabiklooei, A; Goodarzi, P; Kariminejad, Mohammad H

    2009-11-01

    To determine the relative frequency of causes of lung hypoplasia (LH) and its associated congenital malformations among perinatal deaths. 850 medical reports of perinatal autopsies, in a 25-year period, assessed for LH as a cause of death. LH found in 96 (11.3%) cases, 89 (92.7%) were associated with major congenital malformation (secondary type) and primary type was seen in 7 cases (7.3%). Fourteen cases were associated with multiple congenital anomalies. 32 cases (33.3%) with Genito-urinary anomalies were the most common associated major malformations, followed by 19 cases (19.8%) of diaphragmatic impairment, 15 cases (15.6%) of musculoskeletal abnormalities and 11 cases (11.4%) of kidney agenesis. The most common musculoskletal abnormality was thanatophoric dwarfism in 10 cases (10.4%). Meckle-Gruber syndrome with 7 affected fetuses (7.3%) was the most common malformation syndrome associated with LH. More than ninety percent of LH was secondary to pathology outside the respiratory tract. Renal agenesis is the most common association observed in LH, followed by diaphragmatic hernia and thanatophoric dysplasia.

  18. Functional aspects of treatment with implant-supported single crowns: a quality control study in subjects with tooth agenesis

    DEFF Research Database (Denmark)

    Goshima, Kenichi; Lexner, Michala O; Thomsen, Carsten Eckhart

    2010-01-01

    -Related Quality of Life (OHRQoL) in subjects with tooth agenesis. MATERIAL AND METHODS: In nine females and nine males (32 + or - 10 years) with agenesis treated with one to four ISSC (68% in the premolar region), the treatment effect and masticatory function were assessed. The evaluation was performed first...... after implant placement shortly before crown cementation, and again 1 month after cementation. It consisted of questionnaires [including Oral Health Impact Profile (OHIP-49)] and functional examination with plastic strips, the Dental Prescale Film and the Occluzer system, Xylitol color-changeable gum...... with ISSCs in subjects with tooth agenesis significantly increased masticatory function subjectively and clinically as well as OHRQoL. However, as the functional parameters before replacement of the teeth corresponded to values in subjects with complete dentitions, the functional importance of the increase...

  19. Tooth agenesis in patients referred to an Irish tertiary care clinic for the developmental dental disorders.

    LENUS (Irish Health Repository)

    Hashem, Atef A

    2010-01-01

    PURPOSE: This study was carried out to determine the prevalence, severity and pattern of hypodontia in Irish patients referred to a tertiary care clinic for developmental dental disorders. MATERIALS AND METHODS: Details of 168 patients with hypodontia referred during the period 2002-2006 were entered in a database designed as a national record. Tooth charting was completed using clinical and radiographic examinations. The age of patients ranged from 7-50 years, with a median age of 20 years (Mean: 21.79; SD: 8.005). RESULTS: Hypodontia referrals constituted 65.5% of the total referrals. Females were more commonly affected than males with a ratio of 1.3:1. The number of referrals reflected the population density in this area; the majority were referrals from the public dental service. Mandibular second premolars were the most commonly missing teeth, followed by maxillary second premolars and maxillary lateral incisors; maxillary central incisors were the least affected. Symmetry of tooth agenesis between the right and left sides was an evident feature. Slightly more teeth were missing on the left side (n = 725) than on the right side (n = 706) and in the maxillary arch (n = 768) as compared to the mandibular arch (n = 663). Some 54% of patients had severe hypodontia with more than six teeth missing; 32% had moderate hypodontia, with four to six teeth missing. The most common pattern of tooth agenesis was four missing teeth. CONCLUSION: Hypodontia was a common presentation in a population referred to this tertiary care clinic. The pattern and distribution of tooth agenesis in Irish patients appears to follow the patterns reported in the literature.

  20. Prosthetic replacement vs space closure for maxillary lateral incisor agenesis: A systematic review.

    Science.gov (United States)

    Silveira, Giordani Santos; de Almeida, Natália Valli; Pereira, Daniele Masterson Tavares; Mattos, Cláudia Trindade; Mucha, José Nelson

    2016-08-01

    Defining the best treatment for maxillary lateral incisor agenesis is a challenge. Our aim in this study was to determine, with the evidence available in the literature, the best treatment for maxillary lateral incisor agenesis in the permanent dentition, evaluating the esthetic, occlusal (functional), and periodontal results between prosthetic replacement and orthodontic space closure. Electronic databases (CENTRAL, PubMed, Web of Science, Scopus, and LILACS) were searched in September 2014 and updated in January 2015, with no restriction on language or initial date. A manual search of the reference lists of the potential studies was performed. Risk of bias was assessed by the Newcastle Ottawa Scale. The search identified 2174 articles, of which 1196 were excluded because they were duplicates. Titles and abstracts of 978 articles were accessed, and 957 were excluded. In total, 21 articles were read in full, and 9 case-control studies were included after applying the inclusion and exclusion criteria. Data were extracted from the articles selected, and a table was compiled for comparison and analysis of the results. There were no randomization and blinding, and the risk of bias evaluation found gaps in compatibility and outcome domains in almost all selected studies. Tooth-supported dental prostheses of maxillary lateral incisor agenesis had worse scores in the periodontal indexes than did orthodontic space closure. Space closure is evaluated better esthetically than prosthetic replacements, and the presence or absence of a Class I relationship of the canines showed no relationship with occlusal function or with signs and symptoms of temporomandibular disorders. Copyright © 2016 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

  1. Fetal bilateral renal agenesis, phocomelia, and single umbilical artery associated with cocaine abuse in early pregnancy.

    Science.gov (United States)

    Kashiwagi, Maki; Chaoui, Rabih; Stallmach, Thomas; Hürlimann, Sandra; Lauper, Urs; Hebisch, Gundula

    2003-11-01

    Maternal cocaine abuse in pregnancy is associated with complications such as intrauterine growth retardation, abruptio placentae, and preterm delivery. We report what is, to our knowledge, the first published observation of fetal bilateral renal agenesis associated with a vascular disruption syndrome comprising upper limb reduction defect and a single umbilical artery following maternal cocaine abuse in early pregnancy. This constellation in a fetus aborted at 18 weeks extends the spectrum of complications possibly associated with cocaine abuse in pregnancy. Copyright 2003 Wiley-Liss, Inc.

  2. Agenesis of mandibular second premolar in patient with dental bimaxillary protrusion

    Directory of Open Access Journals (Sweden)

    Carlos Alberto Estevanell Tavares

    Full Text Available ABSTRACT The present study reports the treatment carried out in a patient with mandibular second premolar agenesis associated with early loss of a deciduous second molar, deep overbite, severe overjet and dentoalveolar bimaxillary protrusion, which led to lip incompetence and a convex facial profile. The main objectives of this treatment were: to eliminate the spaces in mandibular arch, correct overbite, as well as eliminate bimaxillary protrusion and lip incompetence, thus leading to a balanced profile. The case was presented to the Brazilian Board of Orthodontics and Dentofacial Orthopedics (BBO as part of the requirements to obtain the title of BBO diplomate.

  3. A "blind" vascular ring in association with congenital cystic adenomatoid malformation: A case report.

    Science.gov (United States)

    Xia, Bo; Hong, Chun; Tang, Jing; Liu, Cuifen; Yu, Gang

    2017-12-01

    The occurrence of congenital cystic adenomatoid malformation (CCAM) and vascular ring (VR) is extremely rare. We present a case of left CCAM with VR consisting of a left aortic arch and right descending aorta with left tracheal compression causing atelectasis. A high-risk male neonate with the diagnosis of left CCAM was diagnosed at 20 weeks gestational age by antenatal ultrasound. Chest CT revealed multiple cysts in the left inferior lung. Cardiac CT showed VR consisting of a left aortic arch and right descending aorta with left tracheal compression causing atelectasis. left inferior lobectomy was performed. Cardiac CT showed VR consisting of a left aortic arch and right descending aorta with left tracheal compression causing atelectasis. Descending aorta transposition was performed. The patient recovered smoothly and remained asymptomatic during the 12-months of postoperative follow-up period. We report this rare case of CCAM with VR consisting of left aortic arch and right descending aorta with left tracheal compression causing atelectasis. From the findings of this report, early surgical treatment is recommended. Although the prognosis after surgery remained good, second surgery can be avoided if VR was detected early. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  4. Complex malformations of the urogenital tract in a female dog: Gartner duct cyst, ipsilateral renal agenesis, and ipsilateral hydrometra.

    Science.gov (United States)

    Fujita, Atsushi; Tsuboi, Masaya; Uchida, Kazuyuki; Nishimura, Ryohei

    2016-05-01

    A 10-month-old female toy poodle was referred to the University of Tokyo Veterinary Medical Center with a urogenital anomaly found during sterilization. An exploratory laparotomy revealed a cyst adhering to the cervix and a unilateral renal agenesis. Histopathology and immunohistochemical analysis of the cyst was consistent with remnants of the Wolffian duct or a Gartner duct cyst. This is a rare case of a canine Gartner duct cyst with renal agenesis and uterine anomaly. We discuss the similarity of this case to that of humans and introduce a classification in the literature for these complex urogenital malformations for further clinical research into the precise diagnosis and appropriate surgical planning.

  5. Orthodontic management of bilateral maxillary canine-first premolar transposition and bilateral agenesis of maxillary lateral incisors: a case report.

    Science.gov (United States)

    Di Palma, Elena; Di Giuseppe, Biagio; Tepedino, Michele; Chimenti, Claudio

    2015-01-01

    Maxillary canine-first premolar transposition (Mx.C.P1) is an uncommon dental positional anomaly that may create many orthodontic problems from both esthetic and functional points of view. In this report we show the orthodontic management of a case of Mx.C.P1 associated with bilateral maxillary lateral incisor agenesis and unilateral mandibular second premolar agenesis The patient was treated with a multibracket appliance and the extraction of the lower premolar. treatment was completed without the need for any prosthetic replacement.

  6. Herlyn-Werner-Wunderlich Syndrome Consisting of Uterine Didelphys, Obstructed Hemivagina and Ipsilateral Renal Agenesis in a Newborn

    Directory of Open Access Journals (Sweden)

    Tsung-Hsin Wu

    2012-02-01

    Full Text Available Herlyn-Werner-Wunderlich (HWW syndrome is a rare variant of Müllerian duct anomalies consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome are usually asymptomatic until menarche, when they present with acute lower abdominal pain. Here we report a case of a female newborn with right renal agenesis diagnosed during the pregnancy. The patient presented with a protruding mass over the vaginal introitus that was associated with an obstructed hemivagina and uterine didelphys.

  7. Unilateral Complete Agenesis of Mesonephric Duct Derivatives in an 82-year-Old Male Cadaver: Embryology, Anatomy and Clinical Considerations

    Directory of Open Access Journals (Sweden)

    David G. Darcy

    2017-11-01

    Full Text Available Development of urogenital anatomy in the human fetus is the result of a complex interplay between multiple different tissues. The time course of development is well documented and the morphologic outcomes of insults at various time points during development are predictable. We present a cadaveric case of unilateral agenesis of the left kidney, ureter, bladder hemitrigone, ureteric opening, seminal vesicle, vas deferens, and epididymis. Failure of development of the mesonephric duct early during embryogenesis, likely between the third and fifth week, caused ipsilateral urogenital organ agenesis.

  8. Effect of aging on tracheal mucociliary clearance in Beagle dogs

    International Nuclear Information System (INIS)

    Whaley, S.L.; Muggenburg, B.A.; Wolff, R.K.

    1985-01-01

    Tracheal mucous velocity measurements were made in 24 Beagle dogs, in 5 age groups, using a gamma camera to detect movement on instilled radiolabeled material. Age groups were defined as immature, young adult, middle-aged, mature, and aged dogs. Mean velocities (+/- SE) were 3.6 +/- 0.4 mm/min in the immature dogs, 9.8 +/- 0.7 mm/min in the young adults, 6.9 +/- 0.5 mm/min in the middle-aged dogs, 3.6 +/- 1.1 mm/min in the mature dogs, and 2.8 +/- 0.6 mm/min in the aged dogs. Tracheal mucous velocity was significantly faster in the young adult and middle-aged groups than in the immature, mature, and aged dog groups. 4 references, 1 figure, 1 table

  9. Transoral tracheal intubation of rodents using a fiberoptic laryngoscope.

    Science.gov (United States)

    Costa, D L; Lehmann, J R; Harold, W M; Drew, R T

    1986-06-01

    A fiberoptic laryngoscope which allows direct visualization of the deep pharynx and epiglottis has been developed for transoral tracheal intubation of small laboratory mammals. The device has been employed in the intubation and instillation of a variety of substances into the lungs of rats, and with minor modification, has had similar application in mice, hamsters, and guinea pigs. The simplicity and ease of handling of the laryngoscope permits one person to intubate large numbers of enflurane anesthetized animals either on an open counter top or in a glove-box, as may be required for administration of carcinogenic materials. Instillation of 7Be-labeled carbon particles into the lungs of mice, hamsters, rats, and guinea pigs resulted in reasonably consistent interlobal distribution of particles for each test animal species with minimal tracheal deposition. However, actual lung tissue doses of carbon exhibited some species dependence.

  10. Difficult Tracheal Intubation in Obese Gastric Bypass patients

    DEFF Research Database (Denmark)

    Dohrn, Niclas; Sommer, Thorbjørn; Bisgaard, J.

    2016-01-01

    Endotracheal intubation is commonly perceived to be more difficult in obese patients than in lean patients. Primarily, we investigated the association between difficult tracheal intubation (DTI) and obesity, and secondarily, the association between DTI and validated scoring systems used to assess...... the airways, the association between DTI and quantities of anesthetics used to induce general anesthesia, and the association between DTI and difficulties with venous and arterial cannulation. This is a monocentric prospective observational clinical study of a consecutive series of 539 obese patients...... was 3.5 % and the patients with DTI were more frequently males, had higher CLC, higher American Society of Anesthesiologists physical status classification (ASA), and noticeably, a lower BMI compared to the patients with easy tracheal intubation. After adjustment with multivariable analyses body mass...

  11. Treatment of Benign Tracheal Stenosis Using Endoluminal Spray Cryotherapy.

    Science.gov (United States)

    Bhora, Faiz Y; Ayub, Adil; Forleiter, Craig M; Huang, Chyun-Yin; Alshehri, Khalid; Rehmani, Sadiq; Al-Ayoubi, Adnan M; Raad, Wissam; Lebovics, Robert S

    2016-11-01

    Tracheal stenosis is a debilitating disorder with heterogeneity in terms of disease characteristics and management. Repeated recurrences substantially alter patients' quality of life. There is limited evidence for the use of spray cryotherapy (SCT) in the management of benign airway disease. To report our early results for the use of SCT in patients with benign tracheal stenosis. Data were extracted from the medical records of a consecutive series of patients with benign airway stenosis secondary to granulomatosis with polyangiitis (GPA) (n = 13), prior tracheotomy or tracheal intubation (n = 8), and idiopathic strictures (n = 5) treated from September 1, 2013, to September 30, 2015, at a tertiary care hospital. Airway narrowing was quantified on a standard quartile grading scale. Response to treatment was assessed by improvement in airway caliber and the time interval for reintervention. Delivery of 4 5-second SCT cycles and 2 balloon dilatations. Twenty-six patients (median [range] age, 53 [16-83] years; 20 [77%] female) underwent 48 SCT sessions. Spray cryotherapy was successfully used without any substantial intraoperative or postoperative complications in all patients. In a median (range) follow-up of 11 (1-26) months, all patients had improvement in symptoms. Before the institution of SCT, 23 patients (88%) had grade III or IV stenosis. At the last evaluation after induction of SCT, 4 (15%) had grade III or IV stenosis, with a mean (SD) change of 1.39 (0.51) (P benign tracheal stenosis. Although efficacy evidence is limited for SCT, it may be useful for patients who have experienced treatment failure with conventional modalities. Further analysis of this cohort will determine the physiologic durability of the reported short-term changes. Additional trials are warranted for further evaluation of this modality.

  12. Mucus glycoprotein secretion by tracheal explants: effects of pollutants

    International Nuclear Information System (INIS)

    Last, J.A.; Kaizu, T.

    1980-01-01

    Tracheal slices incubated with radioactive precursors in tissue culture medium secrete labeled mucus glycoproteins into the culture medium. We have used an in vivtro approach, a combined method utilizing exposure to pneumotoxins in vivo coupled with quantitation of mucus secretion rates in vitro, to study the effects of inhaled pollutants on mucus biosynthesis by rat airways. In addition, we have purified the mucus glycoproteins secreted by rat tracheal explants in order to determine putative structural changes that might by the basis for the observed augmented secretion rates after exposure of rats to H2SO4 aerosols in combination with high ambient levels of ozone. After digestion with papain, mucus glycoproteins secreted by tracheal explants may be separated into five fractions by ion-exchange chromatography, with recovery in high yield, on columns of DEAE-cellulose. Each of these five fractions, one neutral and four acidic, migrates as a single unique spot upon cellulose acetate electrophoresis at pH values of 8.6 and 1.2. The neutral fraction, which is labeled with [3H] glucosamine, does not contain radioactivity when Na2 35SO4 is used as the precursor. Acidic fractions I to IV are all labeled with either 3H-glucosamine or Na2 35SO4 as precursor. Acidic fraction II contains sialic acid as the terminal sugar on its oligosaccharide side chains, based upon its chromatographic behavior on columns of wheat-germ agglutinin-Agarose. Treatment of this fraction with neuraminidase shifts its elution position in the gradient to a lower salt concentration, coincident with acidic fraction I. After removal of terminal sialic acid residues with either neuraminidase or low pH treatment, the resultant terminal sugar on the oligosaccharide side chains is fucose. These results are identical with those observed with mucus glycoproteins secreted by cultured human tracheal explants and purified by these same techniques

  13. Sonographic Findings of Primary Tracheal Lymphoma: Case Report

    International Nuclear Information System (INIS)

    Kim, Min Sung; Ahn, Kook Jin; Kim, Jee Young; Kim, Hyo Lim; Park, Jeong Mi

    2010-01-01

    Primary lymphoma of the trachea is extremely rare and clinical presentation is nonspecific. CT findings are focal tracheal narrowing caused by a solitary mass or polypoid thickening of the tracheobronchial wall caused by diffuse infiltration of the submucosa. However, US finding of primary lymphoma of the trachea has been not reported. We experienced a case of primary lymphoma of the trachea presenting as a homogenous hypoechoic mass, and discuss ultrasonographic and CT findings of the case

  14. Congenital orbital teratoma

    OpenAIRE

    Aiyub, Shereen; Chan, Weng Onn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-01-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The p...

  15. Regeneration of tracheal epithelium using mouse induced pluripotent stem cells.

    Science.gov (United States)

    Ikeda, Masakazu; Imaizumi, Mitsuyoshi; Yoshie, Susumu; Otsuki, Koshi; Miyake, Masao; Hazama, Akihiro; Wada, Ikuo; Omori, Koichi

    2016-01-01

    Conclusion The findings demonstrated the potential use of induced pluripotent stem cells for regeneration of tracheal epithelium. Objective Autologous tissue implantation techniques using skin or cartilage are often applied in cases of tracheal defects with laryngeal inflammatory lesions and malignant tumor invasion. However, these techniques are invasive with an unstable clinical outcome. The purpose of this study was to investigate regeneration in a tracheal defect site of nude rats after implantation of ciliated epithelium that was differentiated from induced pluripotent stem cells. Method Embryoid bodies were formed from mouse induced pluripotent stem cells. They were cultured with growth factors for 5 days, and then cultured at the air-liquid interface. The degree of differentiation achieved prior to implantation was determined by histological findings and the results of real-time polymerase chain reaction. Embryoid bodies including ciliated epithelium were embedded into collagen gel that served as an artificial scaffold, and then implanted into nude rats, creating an 'air-liquid interface model'. Histological evaluation was performed 7 days after implantation. Results The ciliated epithelial structure survived on the lumen side of regenerated tissue. It was demonstrated histologically that the structure was composed of ciliated epithelial cells.

  16. Low tracheal tumor and airway management: An anesthetic challenge

    Directory of Open Access Journals (Sweden)

    Richa Saroa

    2015-01-01

    Full Text Available We describe a case presenting with tracheal tumor wherein a Microlaryngeal tube was advanced into the trachea distal to the tumor for primary airway control followed by cannulation of both endobronchial lumen with 5.5 mm endotracheal tubes to provide independent lung ventilation post tracheal transection using Y- connector attached to anesthesia machine. The plan was formulated to provide maximal surgical access to the trachea while providing adequate ventilation at the same time. A 32 yrs non smoker male, complaining of cough, progressive dyspnea and hemoptysis was diagnosed to have a broad based mass in the trachea on computed tomography of chest. Bronchoscopy of the upper airway confirmed presence of the mass at a distance of 9 cms from the vocal cords, obstructing the tracheal lumen by three fourth of the diameter. The patient was scheduled to undergo the resection of the mass through anterolateral thoracotomy. We recommend the use of extralong, soft, small sized microlaryngeal surgery tube in tumors proximal to carina, for securing the airway before the transection of trachea and bilateral endobronchial intubation with small sized cuffed endotracheal tubes for maintenance of ventilation after the transection of trachea in patients with mass in the lower trachea.

  17. Myosin light chain kinase phosphorylation in tracheal smooth muscle

    International Nuclear Information System (INIS)

    Stull, J.T.; Hsu, L.C.; Tansey, M.G.; Kamm, K.E.

    1990-01-01

    Purified myosin light chain kinase from smooth muscle is phosphorylated by cyclic AMP-dependent protein kinase, protein kinase C, and the multifunctional calmodulin-dependent protein kinase II. Because phosphorylation in a specific site (site A) by any one of these kinases desensitizes myosin light chain kinase to activation by Ca2+/calmodulin, kinase phosphorylation could play an important role in regulating smooth muscle contractility. This possibility was investigated in 32 P-labeled bovine tracheal smooth muscle. Treatment of tissues with carbachol, KCl, isoproterenol, or phorbol 12,13-dibutyrate increased the extent of kinase phosphorylation. Six primary phosphopeptides (A-F) of myosin light chain kinase were identified. Site A was phosphorylated to an appreciable extent only with carbachol or KCl, agents which contract tracheal smooth muscle. The extent of site A phosphorylation correlated to increases in the concentration of Ca2+/calmodulin required for activation. These results show that cyclic AMP-dependent protein kinase and protein kinase C do not affect smooth muscle contractility by phosphorylating site A in myosin light chain kinase. It is proposed that phosphorylation of myosin light chain kinase in site A in contracting tracheal smooth muscle may play a role in the reported desensitization of contractile elements to activation by Ca2+

  18. Efficacy of transoral intraluminal Wallstents for tracheal stenosis or tracheomalacia.

    Science.gov (United States)

    Casiano, R R; Numa, W A; Nurko, Y J

    2000-10-01

    The efficacy and safety of intraluminal Wallstent Endoprosthesis (Boston Scientific/ Medi-Tech, Quincy, MA) placement to restore airway patency in patients with tracheal stenosis or tracheomalacia are unknown. Retrospective review in setting of tertiary, referral, and academic center. A retrospective review of 13 consecutive patients over a 2-year period who underwent transoral resection of tracheal stenosis and immediate transoral Wallstent placement. One patient had tracheomalacia. All of the patients were considered at high risk for transcervical surgery or had failed prior traditional open procedures. The average patient age was 54.2 years, with nine male and four female patients. All had Cotton/Myer stenoses (grades II to IV) with moderate to severe degrees of inspiratory stridor. Four patients were tracheotomy dependent. The length of stenosis varied from 1 to 4 cm. One patient had a 10-cm segment of tracheomalacia. At the time of writing, none of the patients has had a problem with significant migration or extrusion and most of the patients have incorporated the stent well without any short-term obstructive granulation tissue. After a mean follow-up of 15 months (range, 4-24 mo). 10 of the 12 patients with stenosis (83%) have remained free of any inspiratory noise during breathing. The one patient with tracheomalacia also has remained free of symptoms. Transoral Wallstents appear to be safe and may be a reasonable alternative in the restoration of airway patency in select patients with tracheal stenosis or tracheomalacia.

  19. Multimodal non-surgical treatment of a feline tracheal adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Carolina Azevedo

    2017-02-01

    Full Text Available Case summary A 10-year-old, castrated male domestic shorthair cat presented with a 2–3 month history of weight loss, lethargy and coughing. Thoracic radiographs revealed a soft tissue opacity overlying the dorsal trachea from the first rib to second rib and the ventral aspect of the trachea extending from the second rib to approximately the fourth rib. CT confirmed a mass involving the dorsal, right lateral and ventral aspects of the trachea narrowing the lumen and extending from vertebra C7 through T4. Bronchoscopy revealed a partially circumferential irregular and multilobulated tracheal mass, which was biopsied. The histopathological diagnosis was tracheal adenocarcinoma. The cat was treated with a definitive course of external beam radiation therapy (RT; 3 Gy × 18, cytotoxic chemotherapy, a tyrosine kinase inhibitor and palliative RT. The cat remained asymptomatic for 2 months and the mass remained stable radiographically for 11 months after RT. Relevance and novel information With multimodal treatment the cat had a survival time of 755 days. Initial treatment included definitive RT, carboplatin and piroxicam, followed by toceranib phosphate and palliative RT when the mass recurred. This case report describes the first documented use of non-surgical treatment and long-term outcome of tracheal adenocarcinoma in a cat. This case report is an indication that prolonged survival can be achieved with multimodal therapy.

  20. Agenesis of the gallbladder with hypoplastic cystic duct diagnosed at laparoscopy.

    Science.gov (United States)

    Kwon, A-Hon; Yanagimoto, Hiroaki; Matsui, Yoichi; Imamura, Atsushi

    2006-08-01

    An 86-year-old man was admitted to our department with complaints of intermittent upper abdominal pain. Ultrasonography of the abdomen showed dilated extrahepatic bile ducts containing stones; however, the gallbladder was not clearly identified. Magnetic resonance cholangiopancreatography showed dilated extrahepatic ducts and choledocholithiasis without gallbladder visualization. The stone extraction was performed with endoscopic sphincterotomy. Three-dimensional images using spiral-computed tomography after intravenous-infusion cholangiography clearly demonstrated an obstruction of the cystic duct. The patient was scheduled for laparoscopic cholecystectomy. At laparoscopy, the gallbladder fossa was not identified on the undersurface of the liver. Despite a thorough examination of the intrahepatic (left-sided within the lesser omentum), retroperitoneal, retrohepatic (within the falciform ligament), retroduodenal, and retropancreatic areas using laparoscopic ultrasonography, the gallbladder was not found. After careful dissection of the hepatoduodenal ligament, the dilated extrahepatic bile duct and a 1-cm length of hypoplastic cystic duct were found. Gallbladder agenesis is usually accompanied by the lack of the cystic duct. The present case is the third report of gallbladder agenesis with a patent or hypoplastic cystic duct.

  1. Nonsyndromic tooth agenesis patterns and associated developmental dental anomalies: a literature review with radiographic illustrations.

    Science.gov (United States)

    Agarwal, P; Vinuth, D P; Dube, G; Dube, P

    2013-01-01

    Tooth agenesis is one of the most intriguing phenomena, because it is frequently associated with other oral anomalies, structural variations and malformations of other teeth, late eruption, transposition and crowding. The diagnosis can be quite challenging as radiographic examination is critical for the diagnosis but not always possible and the late developing teeth may be sometimes scored developmentally missing. Accurate diagnosis therefore requires radiographic, clinical, and dental cast examinations. It is an important clinical and public health problem. Patients with missing permanent teeth may suffer from a reduced chewing ability, inarticulate pronunciation, and an unfavorable aesthetic appearance. Clinically, early diagnosis of a dental anomaly can alert the clinician to the possible development of other associated dental anomalies in the same patient or family, and avoid the possible sequelae. Understanding of tooth agenesis patterns and their impact on diagnosis, prevention, and eventually therapeutics are becoming integral parts of comprehensive dental care. Dental examination with radiographic screening of hypodontia in early childhood should be emphasized as part of public oral health policy to allow early diagnosis and timely intervention.

  2. Prevalence and distribution of dental anomalies: a comparison between maxillary and mandibular tooth agenesis.

    Science.gov (United States)

    Al-Abdallah, Mariam; AlHadidi, Abeer; Hammad, Mohammad; Al-Ahmad, Hazem; Saleh, Raja'

    2015-11-01

    The aim of this study was to compare the pattern and prevalence of associated dental anomalies between maxillary and mandibular tooth agenesis (hypodontia). A sample of 3315 dental patients, aged 8.6 to 25.4 years, was surveyed for tooth agenesis (excluding third molars): 106 subjects were diagnosed with maxillary hypodontia (group 1) and 70 with mandibular hypodontia (group 2). Both groups were examined for the following dental anomalies: retained deciduous molars, infraocclusion of deciduous molars, impaction, microdontia of maxillary lateral incisors, supernumerary teeth, transposition, transmigration, and ectopic eruption of the permanent molars. For statistical testing, the chi-square test (P anomalies among the groups. We found that 77.5% of the patients in the mandibular hypodontia group had at least 1 dental anomaly compared with 49.5% in the maxillary hypodontia group (P dental anomaly with a significantly increased prevalence in the maxillary hypodontia group compared with the mandibular hypodontia group was microdontia of the maxillary lateral incisors (groups 1, 46.7%; group 2, 12.9%; P dental development in both jaws. Copyright © 2015 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

  3. [Tracheal Intubation by Paramedics in a Local Community: Current Situation and Future Challenges].

    Science.gov (United States)

    Takinami, Yoshikazu

    2016-03-01

    As of April 2013, 164 paramedics are certified to perform tracheal intubation in Fukui Prefecture. This study investigated the current situation surrounding tracheal intubation performed by paramedics in prehospital care. Subjects were 58 paramedics who completed practical training at our hospital. Post-training duration, number of tracheal intubation cases, number of attempts before successful tracheal intubation, disease involved, rate of return of spontaneous circulation, and prognosis were examined. Tracheal intubation was successful on the first attempt in 92% of cases. Rate of return of spontaneous circulation was high in paramedics whose post-training duration was short. No return of spontaneous circulation occurred after a second attempt. Four patients survived asphyxia or aspiration. It is important to perform successful tracheal intubation on the first attempt, to recognize the probability of successful resuscitation in patients with exogenous disease, and to strengthen the medical control system.

  4. Cytomegalovirus Congenital Cataract

    Directory of Open Access Journals (Sweden)

    Ridha Wahyutomo

    2011-06-01

    Full Text Available Cytomegalovirus congenital infection is an infection caused by the the subfamily â Herpesviridae, during pregnancy. The incidence of infections among newborn infants is 1 %. One of the effects of congenitally acquired infection is the congenital cataract. A 6-year-old child complained to have a blurred vision diagnosed with cytomegalovirus congenital cataract. The diagnosis was confirmed by a positive serology testing for Ig M and Ig G CMV. The laboratory test using Giemsa staining to find inclusion bodies and a faster PCR could not be carried out (Sains Medika, 3(1:84-88.

  5. Genetics Home Reference: congenital hypothyroidism

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Congenital hypothyroidism Congenital hypothyroidism Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Congenital hypothyroidism is a partial or complete loss of function ...

  6. Sphingolipids in Congenital Diaphragmatic Hernia; Results from an International Multicenter Study.

    Directory of Open Access Journals (Sweden)

    Kitty G Snoek

    Full Text Available Congenital diaphragmatic hernia is a severe congenital anomaly with significant mortality and morbidity, for instance chronic lung disease. Sphingolipids have shown to be involved in lung injury, but their role in the pathophysiology of chronic lung disease has not been explored. We hypothesized that sphingolipid profiles in tracheal aspirates could play a role in predicting the mortality/ development of chronic lung disease in congenital diaphragmatic hernia patients. Furthermore, we hypothesized that sphingolipid profiles differ between ventilation modes; conventional mechanical ventilation versus high-frequency oscillation.Sphingolipid levels in tracheal aspirates were determined at days 1, 3, 7 and 14 in 72 neonates with congenital diaphragmatic hernia, born after > 34 weeks gestation at four high-volume congenital diaphragmatic hernia centers. Data were collected within a multicenter trial of initial ventilation strategy (NTR 1310.36 patients (50.0% died or developed chronic lung disease, 34 patients (47.2% by stratification were initially ventilated by conventional mechanical ventilation and 38 patients (52.8% by high-frequency oscillation. Multivariable logistic regression analysis with correction for side of the defect, liver position and observed-to-expected lung-to-head ratio, showed that none of the changes in sphingolipid levels were significantly associated with mortality /development of chronic lung disease. At day 14, long-chain ceramides 18:1 and 24:0 were significantly elevated in patients initially ventilated by conventional mechanical ventilation compared to high-frequency oscillation.We could not detect significant differences in temporal sphingolipid levels in congenital diaphragmatic hernia infants with mortality/development of chronic lung disease versus survivors without development of CLD. Elevated levels of ceramides 18:1 and 24:0 in the conventional mechanical ventilation group when compared to high

  7. Mitochondrial disorders in congenital myopathies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

  8. Tracheal Penetration and Tracheoesophageal Fistula Caused by an Esophageal Self-Expanding Metallic Stent

    Directory of Open Access Journals (Sweden)

    Karan Madan

    2014-01-01

    Full Text Available Tracheal penetration of esophageal self-expanding metallic stents (SEMS with/without tracheoesophageal fistula (TEF formation is a rare occurrence. We report the case of a 66-year-old female patient with advanced esophageal squamous cell carcinoma who had undergone palliative esophageal stenting on three occasions for recurrent esophageal stent obstruction. On evaluation of symptoms of breathing difficulty and aspiration following third esophageal stent placement, tracheal erosion and TEF formation due to the tracheal penetration by esophageal stent were diagnosed. The patient was successfully managed by covered tracheal SEMS placement under flexible bronchoscopy.

  9. Two cases of severe tracheal stenosis due to advanced adenoid cystic carcinoma of the trachea

    Energy Technology Data Exchange (ETDEWEB)

    Yuasa, Hiroshi; Akaogi, Eiichi; Morita, Riichiro; Onizuka, Masataka; Mitsui, Kiyofumi [Tsukuba Univ., Ibaraki (Japan). Inst. of Clinical Medicine; Tsuji, Hirohiko

    1991-12-01

    Two cases of severe tracheal stenosis due to advanced nonresectable adenoid cystic carcinoma of the trachea were reported. Case 1 was a 38-year-old male who underwent emergency {sup 60}Co irradiation following endoscopic YAG laser irradiation. This approach was quite effective. Proton irradiation was effective against tumor recurrence. Case 2 was a 28-year-old female in whom emergency treatment for tracheal obstruction due to edema after laser therapy necessitated transient tracheal intubation. The tracheal stenosis due to recurrent tumor after {sup 60}Co irradiation was improved by insertion of an intraluminal permanent stent. (author).

  10. Fatal complication from a balloon-expandable tracheal stent in a child: a case report.

    Science.gov (United States)

    Stotz, William H; Berkowitz, Ivor D; Hoehner, Jeff C; Tunkel, David E

    2003-01-01

    The use of airway stents in the pediatric population is uncommon, reflected in the few patient series reported in the literature. We describe a fatal complication of tracheal stent placement in an 18-month-old child with spondylothoracic dysplasia. Case report. Intensive care unit of a tertiary academic pediatric center. An 18-month-old child with spondylothoracic dysplasia who underwent tracheal stent placement for tracheomalacia. Management of an acute upper-airway hemorrhage. The patient died, despite aggressive interventions. Use of tracheal stents in pediatric patients with tracheomalacia is not without risks; tracheal erosion with severe hemoptysis is an infrequent but devastating complication of this intervention.

  11. Static end-expiratory and dynamic forced expiratory tracheal collapse in COPD

    International Nuclear Information System (INIS)

    O'Donnell, C.R.; Bankier, A.A.; O'Donnell, D.H.; Loring, S.H.; Boiselle, P.M.

    2014-01-01

    Aim: To determine the range of tracheal collapse at end-expiration among chronic obstructive pulmonary disease (COPD) patients and to compare the extent of tracheal collapse between static end-expiratory and dynamic forced-expiratory multidetector-row computed tomography (MDCT). Materials and methods: After institutional review board approval and obtaining informed consent, 67 patients meeting the National Heart, Lung, and Blood Institute (NHLBI)/World Health Organization (WHO) Global Initiative for Chronic Obstructive Lung Disease (GOLD) criteria for COPD were sequentially imaged using a 64-detector-row CT machine at end-inspiration, during forced expiration, and at end-expiration. Standardized respiratory coaching and spirometric monitoring were employed. Mean percentage tracheal collapse at end-expiration and forced expiration were compared using correlation analysis, and the power of end-expiratory cross-sectional area to predict excessive forced-expiratory tracheal collapse was computed following construction of receiver operating characteristic (ROC) curves. Results: Mean percentage expiratory collapse among COPD patients was 17 ± 18% at end-expiration compared to 62 ± 16% during forced expiration. Over the observed range of end-expiratory tracheal collapse (approximately 10–50%), the positive predictive value of end-expiratory collapse to predict excessive (≥80%) forced expiratory tracheal collapse was <0.3. Conclusion: COPD patients demonstrate a wide range of end-expiratory tracheal collapse. The magnitude of static end-expiratory tracheal collapse does not predict excessive dynamic expiratory tracheal collapse

  12. [Is there either agenesis or regression of the Mullerian duct in female bird embryos under the influence of male hormone?].

    Science.gov (United States)

    Lutz-Ostertag, Y; Lutz, H

    1976-01-01

    The natural occurence of "Free-Martinism" in Birds and the chorio-allantoïc grafting experiments of testis fragments on female chick host-embryos allow to the authors to define the manner provoking the entire or partial disappearance of the müllerian ducts and to state exactly if the phenomenon is a agenesis or a regression.

  13. Radiographic assessment of agenesis, impaction, and pararadicular radiolucencies in relation with third molar in Nashik City of Maharashtra

    Directory of Open Access Journals (Sweden)

    Bhushan Sukdeo Ahire

    2016-01-01

    Full Text Available Introduction: The third molar develops entirely after birth and also it is the last tooth to erupt in all ethnic groups despite racial variations in the eruption sequence. Tooth development with effects on tooth size, shape, position, and total absence is affected due to environmental factors, systemic diseases, genetic polymorphisms, and teratogens. Aims and Objectives: The aim of this study was to assess the prevalence of agenesis, impaction, and pararadicular radiolucencies in relation with third molars. Materials and Methods: One hundred digital orthopantomograph (OPG scans of patients of age ranging from 18 to 25 years were selected randomly from the digital OPG database of the Department of Oral Medicine and Radiology, and then assessment and analysis were carried out. Results: Overall agenesis rate was about 14.5%. Bilateral agenesis of maxillary and mandibular third molars was about 14% and 13%, respectively. Overall impaction rate was about 21.92%. Mesioangular impaction rate was about 57.33% whereas distoangular impaction rate was about 26.66%. Prevalence of pararadicular radiolucencies was only about 0.8% with mandibular predilection. Conclusion: As the agenesis of the third molars is increasing, they can be considered as vestigial. Prevalence of impaction of the third molars is increasing. Pararadicular radiolucencies are mostly seen with mandibular third molar.

  14. Immediate, short-, and long-term changes in tracheal stent diameter, length, and positioning after placement in dogs with tracheal collapse syndrome.

    Science.gov (United States)

    Raske, Matthew; Weisse, Chick; Berent, Allyson C; McDougall, Renee; Lamb, Kenneth

    2018-03-01

    Intraluminal tracheal stenting is a minimally invasive procedure shown to have variable degrees of success in managing clinical signs associated with tracheal collapse syndrome (CTCS) in dogs. Identify immediate post-stent changes in tracheal diameter, determine the extent of stent migration, and stent shortening after stent placement in the immediate-, short-, and long-term periods, and evaluate inter-observer reliability of radiographic measurements. Fifty client-owned dogs. Retrospective study in which medical records were reviewed in dogs with CTCS treated with an intraluminal tracheal stent. Data collected included signalment, location, and type of collapse, stent diameter and length, and post-stent placement radiographic follow-up times. Radiographs were used to obtain pre-stent tracheal measurements and post-stent placement measurements. Immediate mean percentage change was 5.14%, 5.49%, and 21.64% for cervical, thoracic inlet, and intra-thoracic tracheal diameters, respectively. Ultimate mean follow-up time was 446 days, with mean percentage change of 2.55%, 15.09%, and 8.65% for cervical, thoracic inlet, and intra-thoracic tracheal diameters, respectively. Initial mean stent length was 26.72% higher than nominal length and ultimate long-term tracheal mean stent shortening was only 9.90%. No significant stent migration was identified in the immediate, short-, or long-term periods. Good inter-observer agreement of radiographic measurements was found among observers of variable experience level. Use of an intraluminal tracheal stent for CTCS is associated with minimal stent shortening with no clinically relevant stent migration after fluoroscopic placement. Precise stent sizing and placement techniques likely play important roles in avoiding these reported complications. Copyright © 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  15. Immediate, short‐, and long‐term changes in tracheal stent diameter, length, and positioning after placement in dogs with tracheal collapse syndrome

    Science.gov (United States)

    Raske, Matthew; Berent, Allyson C.; McDougall, Renee; Lamb, Kenneth

    2018-01-01

    Background Intraluminal tracheal stenting is a minimally invasive procedure shown to have variable degrees of success in managing clinical signs associated with tracheal collapse syndrome (CTCS) in dogs. Objectives Identify immediate post‐stent changes in tracheal diameter, determine the extent of stent migration, and stent shortening after stent placement in the immediate‐, short‐, and long‐term periods, and evaluate inter‐observer reliability of radiographic measurements. Animals Fifty client‐owned dogs. Methods Retrospective study in which medical records were reviewed in dogs with CTCS treated with an intraluminal tracheal stent. Data collected included signalment, location, and type of collapse, stent diameter and length, and post‐stent placement radiographic follow‐up times. Radiographs were used to obtain pre‐stent tracheal measurements and post‐stent placement measurements. Results Immediate mean percentage change was 5.14%, 5.49%, and 21.64% for cervical, thoracic inlet, and intra‐thoracic tracheal diameters, respectively. Ultimate mean follow‐up time was 446 days, with mean percentage change of 2.55%, 15.09%, and 8.65% for cervical, thoracic inlet, and intra‐thoracic tracheal diameters, respectively. Initial mean stent length was 26.72% higher than nominal length and ultimate long‐term tracheal mean stent shortening was only 9.90%. No significant stent migration was identified in the immediate, short‐, or long‐term periods. Good inter‐observer agreement of radiographic measurements was found among observers of variable experience level. Conclusions and Clinical Importance Use of an intraluminal tracheal stent for CTCS is associated with minimal stent shortening with no clinically relevant stent migration after fluoroscopic placement. Precise stent sizing and placement techniques likely play important roles in avoiding these reported complications. PMID:29460368

  16. Congenital heat disease

    International Nuclear Information System (INIS)

    Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

    1990-01-01

    The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations

  17. Analysis of the golden proportion and width/height ratios of maxillary anterior dentition in patients with lateral incisor agenesis.

    Science.gov (United States)

    Pini, Núbia Pavesi; de-Marchi, Luciana Manzotti; Gribel, Bruno Frazão; Ubaldini, Adriana Lemos Mori; Pascotto, Renata Corrêa

    2012-12-01

      The purpose of this study was to assess the presence of the golden proportion (GP) in the facial view tooth-to-tooth width proportion of the six maxillary anterior teeth and to evaluate the width/height (W/H) ratios of the incisors of patients with maxillary lateral incisor (LI) agenesis treated either with implants or orthodontically (by moving canines into the position of the laterals, recontouring them, and placing composite restorations over the repositioned teeth).   Forty-eight patients with LI agenesis were divided into four experimental groups: unilateral recontouring group (N = 10), bilateral recontouring group (BRG, N = 18), unilateral implant group (UIG, N = 10), bilateral implant group (N = 10), and a control group (CG, N = 25) of patients without agenesis. GP ratios were determined on patients' dental casts placed over Levin's grids, whereas W/H ratios were measured directly on the casts and a millimeter ruler to determine these distances. Statistical analysis was performed with Shapiro-Wilk, Kruskal-Wallis, Mann-Whitney, Friedman, and Wilcoxon tests (p mean W/H ratios of the laterals ranged between 0.75 and 0.90.   Although the GP may be a useful diagnostic guide, it was not observed in the majority of esthetic outcomes of patients treated with maxillary LI agenesis in this study. The assessment of the golden proportion and width/height ratio of upper anterior teeth in patients with upper lateral incisor agenesis treated with either implants or tooth re-contouring may assist dentists and patients in deciding the best treatment option based on the peculiarities of each case. © 2012 Wiley Periodicals, Inc.

  18. Key aspects congenital infection

    Directory of Open Access Journals (Sweden)

    Yu. V. Lobzin

    2014-01-01

    Full Text Available The key questions to solve the problem of congenital infection in the Russian Federation are: using in national practice over world accepted terminology adapted to the recommendations of the World Health Organization; representation of the modern concepts of an infectious process in the classification of congenital infections; scientific development and introducing in clinical practice the «standard case definitions», applied to different congenital infections; optimization of protocols and clinical guidelines for diagnosis, treatment and prevention of congenital infections; improvement a knowledge in the infectious disease for all  pecialists involved in the risk assessment of congenital infections, manage pregnancy and children. Based on our experience and analysis of publications, the authors suggest possible solutions.

  19. Novel EDA or EDAR Mutations Identified in Patients with X-Linked Hypohidrotic Ectodermal Dysplasia or Non-Syndromic Tooth Agenesis

    Directory of Open Access Journals (Sweden)

    Binghui Zeng

    2017-10-01

    Full Text Available Abstract: Both X-linked hypohidrotic ectodermal dysplasia (XLHED and non-syndromic tooth agenesis (NSTA result in symptoms of congenital tooth loss. This study investigated genetic causes in two families with XLHED and four families with NSTA. We screened for mutations of WNT10A, EDA, EDAR, EDARADD, PAX9, MSX1, AXIN2, LRP6, and WNT10B through Sanger sequencing. Whole exome sequencing was performed for the proband of NSTA Family 4. Novel mutation c.1051G>T (p.Val351Phe and the known mutation c.467G>A (p.Arg156His of Ectodysplasin A (EDA were identified in families with XLHED. Novel EDA receptor (EDAR mutation c.73C>T (p.Arg25*, known EDA mutation c.491A>C (p.Glu164Ala, and known Wnt family member 10A (WNT10A mutations c.511C>T (p.Arg171Cys and c.742C>T (p.Arg248* were identified in families with NSTA. The novel EDA and EDAR mutations were predicted as being pathogenic through bioinformatics analyses and structural modeling. Two variants of WNT10A, c.374G>A (p.Arg125Lys and c.125A>G (p.Asn42Ser, were found in patients with NSTA. The two WNT10A variants were predicted to affect the splicing of message RNA, but minigene experiments showed normal splicing of mutated minigenes. This study uncovered the genetic foundations with respect to six families with XLHED or NSTA. We identified six mutations, of which two were novel mutations of EDA and EDAR. This is the first report of a nonsense EDAR mutation leading to NSTA.

  20. A Neuropsychological Profile for Agenesis of the Corpus Callosum? Cognitive, Academic, Executive, Social, and Behavioral Functioning in School-Age Children.

    Science.gov (United States)

    Siffredi, Vanessa; Anderson, Vicki; McIlroy, Alissandra; Wood, Amanda G; Leventer, Richard J; Spencer-Smith, Megan M

    2018-05-01

    Agenesis of the corpus callosum (AgCC), characterized by developmental absence of the corpus callosum, is one of the most common congenital brain malformations. To date, there are limited data on the neuropsychological consequences of AgCC and factors that modulate different outcomes, especially in children. This study aimed to describe general intellectual, academic, executive, social and behavioral functioning in a cohort of school-aged children presenting for clinical services to a hospital and diagnosed with AgCC. The influences of age, social risk and neurological factors were examined. Twenty-eight school-aged children (8 to 17 years) diagnosed with AgCC completed tests of general intelligence (IQ) and academic functioning. Executive, social and behavioral functioning in daily life, and social risk, were estimated from parent and teacher rated questionnaires. MRI findings reviewed by a pediatric neurologist confirmed diagnosis and identified brain characteristics. Clinical details including the presence of epilepsy and diagnosed genetic condition were obtained from medical records. In our cohort, ~50% of children experienced general intellectual, academic, executive, social and/or behavioral difficulties and ~20% were functioning at a level comparable to typically developing children. Social risk was important for understanding variability in neuropsychological outcomes. Brain anomalies and complete AgCC were associated with lower mathematics performance and poorer executive functioning. This is the first comprehensive report of general intellectual, academic, executive social and behavioral consequences of AgCC in school-aged children. The findings have important clinical implications, suggesting that support to families and targeted intervention could promote positive neuropsychological functioning in children with AgCC who come to clinical attention. (JINS, 2018, 24, 445-455).

  1. Intubação traqueal Tracheal intubation

    Directory of Open Access Journals (Sweden)

    Toshio Matsumoto

    2007-05-01

    Full Text Available OBJETIVO: Revisar os conceitos atuais relacionados ao procedimento de intubação traqueal na criança. FONTES DOS DADOS: Seleção dos principais artigos nas bases de dados MEDLINE, LILACS e SciELO, utilizando as palavras-chave intubation, tracheal intubation, child, rapid sequence intubation, pediatric airway, durante o período de 1968 a 2006. SÍNTESE DOS DADOS: O manuseio da via aérea na criança está relacionado à sua fisiologia e anatomia, além de fatores específicos (condições patológicas inerentes, como malformações e condições adquiridas que influenciam decisivamente no seu sucesso. As principais indicações são manter permeável a aérea e controlar a ventilação. A laringoscopia e intubação traqueal determinam alterações cardiovasculares e reatividade de vias aéreas. O uso de tubos com balonete não é proibitivo, desde que respeitado o tamanho adequado para a criança. A via aérea difícil pode ser reconhecida pela escala de Mallampati e na laringoscopia direta. A utilização da seqüência rápida de intubação tem sido recomendada cada vez mais em pediatria, por facilitar o procedimento e apresentar menores complicações. A intubação traqueal deve ser realizada de modo adequado em circunstâncias especiais (alimentação prévia, disfunção neurológica, instabilidade de coluna espinal, obstrução de vias aéreas superiores, lesões laringotraqueais, lesão de globo ocular. A extubação deve ser meticulosamente planejada, pois pode falhar e necessitar de reintubação. CONCLUSÕES: A intubação traqueal de crianças necessita conhecimento, aprendizado e experiência, pois o procedimento realizado por pediatras inexperientes pode resultar em complicações ameaçadoras da vida.OBJECTIVE: To review current concepts related to the procedure of tracheal intubation in children. SOURCES: Relevant articles published from 1968 to 2006 were selected from the MEDLINE, LILACS and SciELO databases, using the

  2. Difficulty with cuff deflation of reinforced tracheal tube caused by inflation line occlusion with silk thread ligation and fixation.

    Science.gov (United States)

    Gotoh, Sayoko; Sugiyama, Daisuke; Imai, Eriko; Kawamata, Mikito

    2015-03-05

    A reinforced tracheal tube, ligated with silk threads, was inserted into a tracheostomy orifice and fixed to the skin. The cuff inflation line of the reinforced tracheal tube became occluded. Reinforced 'armoured' tracheal tubes have a spiral of wire embedded into the wall of the tube to give strength and flexibility, and may be sharply bent without compromising the tube lumen. The tracheal cuff attached to the tube is inflated by injecting air through a narrow-diameter tube welded to the outside of the tracheal tube. When a reinforced tracheal tube is ligated and fixed with silk threads, it should be confirmed whether the tracheal tube cuff can be deflated and inflated after fixation. Moreover, because occlusion can be eliminated by removing all silk threads used to ligate a tracheal tube, they should be removed before extubation. 2015 BMJ Publishing Group Ltd.

  3. Mullerian agenesis associated with in-utero thalidomide exposure: A case report

    Directory of Open Access Journals (Sweden)

    Sarah Dotters-Katz

    2013-09-01

    Full Text Available Thalidomide is a well-known teratogen, which is experiencing resurgence as new uses are identified. Exposure is classically associated with limb deformities, such as: dysmelia, phocomelia, preaxial hypoplasia and polydactyly, in addition to visceral anomalies that have been documented as well. We report a case of a 38 year-old nulligravid female, who was previously evaluated for primary amenorrhea, and given the presumptive false diagnosis of an imperforate hymen. On magnetic resonance imaging (MRI exam, she was noted to have uterovaginal agenesis. The implications of thalidomide on women’s health extend beyond external birth defects. Although, most commonly associated with limb deformities, there may also be gynecologic implications of in utero exposure. As this medication is increasingly used for various medical conditions, obstetricians/gynecologists need to remain aware of this potential mullerian teratogenic effect.

  4. EARS2 mutations cause fatal neonatal lactic acidosis, recurrent hypoglycemia and agenesis of corpus callosum.

    Science.gov (United States)

    Danhauser, Katharina; Haack, Tobias B; Alhaddad, Bader; Melcher, Marlen; Seibt, Annette; Strom, Tim M; Meitinger, Thomas; Klee, Dirk; Mayatepek, Ertan; Prokisch, Holger; Distelmaier, Felix

    2016-06-01

    Mitochondrial aminoacyl tRNA synthetases are essential for organelle protein synthesis. Genetic defects affecting the function of these enzymes may cause pediatric mitochondrial disease. Here, we report on a child with fatal neonatal lactic acidosis and recurrent hypoglycemia caused by mutations in EARS2, encoding mitochondrial glutamyl-tRNA synthetase 2. Brain ultrasound revealed agenesis of corpus callosum. Studies on patient-derived skin fibroblasts showed severely decreased EARS2 protein levels, elevated reactive oxygen species (ROS) production, and altered mitochondrial morphology. Our report further illustrates the clinical spectrum of the severe neonatal-onset form of EARS2 mutations. Moreover, in this case the live-cell parameters appeared to be more sensitive to mitochondrial dysfunction compared to standard diagnostics, which indicates the potential relevance of fibroblast studies in children with mitochondrial diseases.

  5. Tracheal ceramic rings for tracheomalacia: a review after 17 years.

    Science.gov (United States)

    Göbel, Gyula; Karaiskaki, Niki; Gerlinger, Imre; Mann, Wolf J

    2007-10-01

    Despite different support techniques, the surgical management of tracheomalacia is still a challenging problem. Satisfactory results after internal stenting are above 80%, whereas, when performing external stenting using biocompatible ceramic rings, results are reported at over 90%. The purpose of this study was to examine the efficiency of surgical treatment in patients with segmentary tracheomalacia using external ceramic ring grafts. In this retrospective study, we collected data from 12 patients who underwent surgery during the last 17 years for symptomatic segmentary tracheomalacia by use of biocompatible aluminum-oxide ceramic rings. All except one patient had undergone previous tracheostomy, six had a history of long-term intubation, two had previous trauma, and two patients had previous cancer treatment including radiotherapy. One of the patients still had an existing tracheostoma, which was closed when a ceramic ring was implanted. Tracheal wall collapse with pseudoglottis formation or flattened anterior-posterior tracheal diameter was documented with fiberoscopy at rest, and both pre- and postoperative airway resistance measurements were performed in all 12 patients using a spirometer. After malacic segments were found to be expandable using rigid tracheoscopy while the patient was under general anesthesia, preparation of the trachea was performed using a midline vertical incision in the neck. Subsequently, the malacic trachea was expanded by placing and suturing proper-sized ceramic ring(s) around it. In all patients, surgical expansion of the malacic segment using ceramic rings was successfully carried out without major complications while inspiratory stridor was resolved. Airway resistance decreased significantly from an average of 0.62 to 0.385 kPascal. Although the results of applying internal tracheal stents are encouraging, complications such as stent migration, granulation tissue and fistula formation, and mucociliary transport arrest are possible

  6. Accessory mammary tissue associated with congenital and hereditary nephrourinary malformations.

    Science.gov (United States)

    Urbani, C E; Betti, R

    1996-05-01

    The association between polythelia (supernumerary nipple) and kidney and urinary tract malformations (KUTM) is controversial. Some authors reported this association in newborns and infants. Case-control studies dealing with adult subjects are not found in the literature. The purpose of this study is to determine the frequency of the association between accessory mammary tissue (AMT) and congenital and hereditary nephrourinary defects in an adult population compared to a control group. The study was performed in 146 white patients (123 men, 23 women) with AMT out of 2645 subjects consecutively referred to us for physical examination. The following investigations were undertaken: ultrasonographic examination of the abdomen and the kidneys, ECG, echocardiogram, roentgenogram of the vertebral column, urinalysis, and other laboratory tests. A sex- and age-matched control group without any evidence of AMT or lateral displacement of the nipples underwent the same examinations. Kidney and urinary tract malformations were detected in 11 patients with AMT (nine men, two women) and in one control. These data indicate a significantly higher frequency of KUTM in the AMT-affected patients compared to controls (7.53% vs. 0.68%, P < 0.001). A broad spectrum of KUTM was discovered in association with AMT: adult dominant polycystic kidney disease, unilateral renal agenesis, cystic renal dysplasia, familial renal cysts, and congenital stenosis of the pyeloureteral joint. Accessory mammary tissue offers an important clue for congenital and hereditary anomalies of the kidneys and urinary collecting systems. Patients with AMT should, therefore, be extensively examined for the presence of occult nephrouropathies.

  7. Retrovesical cystic lesions in female patients with unilateral renal agenesis or dysplasia

    International Nuclear Information System (INIS)

    Shimada, Kenji; Matsumoto, Fumi; Matsui, Futosi; Obara, Takasi

    2010-01-01

    The objective of this study was to review our experience with female children who have unilateral renal agenesis or renal dysplasia associated with cystic lesions in the reproductive system. Between 1991 and 2007, we treated 26 patients with unilateral renal agenesis or renal dysplasia associated with pelvic, retrovesical or interlabial cystic lesions. In 16 patients, an abnormality either of the kidney or a cystic lesion was detected during the perinatal period. Another 10 patients presented clinical symptoms, including urinary incontinence in five, urinary tract infection in one, and vaginal discharge in four. Based on clinical features and imaging findings, the patients were divided into four groups: those with Gartner's duct cyst (Group 1, n=9); those with uterovaginal duplication with obstructed hemivagina (Herlyn-Werner-Wunderlich syndrome [HWW]; Group 2, n=3); those with both Gartner's duct cyst and HWW syndrome (Group 3, n=3); and those without definitive diagnosis (Group 4, n=11). In Group 1, leakage completely stopped after nephrectomy in three patients, whereas six patients continued to be incontinent despite the removal of dysgenetic kidneys. In Group 2, after the excision of a vaginal septum, no patient presented urinary incontinence. In patients in Group 3, both the mesonephric remnant and mullerian structures were confirmed on imaging or through endoscopy. Because of the high coincidence of genital and renal anomalies, it is recommended that genital systems in female patients with renal anomalies associated with cystic lesions behind the lower urinary tract be investigated. The best imaging modality to use remains under scrutiny. It is important to follow the patients until the age of puberty. The importance of a long-term follow-up in these patients needs to be emphasized. (author)

  8. Tracheal obstructions in two horses and a donkey

    International Nuclear Information System (INIS)

    Mair, T.S.; Lane, J.G.

    1990-01-01

    The clinical signs associated with intramural tracheal obstructions in two horses and a donkey were respiratory distress and coughing, which were aggravated by exercise and excitement. The obstructions were at the level of the thoracic inlet and consisted either of flattening of the cartilage rings in the dorsoventral plane or of cartilage rings having a scroll-like conformation. They appeared to be developmental in origin and to have been present for a considerable time before the onset of clinical signs. Endoscopy and radiography were helpful in the diagnosis of the condition

  9. Repopulation of denuded tracheal grafts with alveolar type II cells

    International Nuclear Information System (INIS)

    Johnson, N.F.

    1988-01-01

    Repopulation of denuded heterotopic tracheal grafts with populations of specific epithelial cell types is one approach to study the differentiation potential of various cell types. This technique has been adopted to delineate the differentiation pathways of alveolar type II cells isolated from rat lungs. Under the conditions of this experiment, the reestablished epithelial lining was alveolar-like, however, ultrastructural analysis of the cells showed them to be like Clara cells. These preliminary results suggest that the secretary cells of the lung parenchyma and terminal airways may share a common ancestry. (author)

  10. Evaluation of Tracheal and Main Bronchial Diverticula Using Thin-Section MDCT

    Energy Technology Data Exchange (ETDEWEB)

    Jou, Sung Shick; Kim, Young Tong; Bae, Won Kyung; Kim, Il Yung; Kim, Hyung Hwan; Han, Jong Kyu [Soonchunhyang University Cheonan Hospital, Cheonan (Korea, Republic of)

    2010-02-15

    To evaluate the characteristics of tracheal and main bronchial diverticula in relation with emphysema. A total of 967 CT images were reconstructed with 1.25 mm axial images over 2 months. The incidence, size, number, and location of the tracheal and main bronchial diverticula were analyzed using 3D medical software (Seoul, Korea). The incidence of emphysema and the relationship between emphysema and the size of the diverticula were analyzed. In total, 50 patients (5.1%) showed tracheal diverticula in the right posterolateral wall. In addition, 51 patients (5.2%) showed 89 (9.4%) main bronchial diverticula in the inferior wall, while 68 (72%) showed diverticula in the left posterolateral wall. Tracheal diverticula (6.4 {+-} 5.0 mm, 1.0 {+-} 0.2) were larger and fewer than the main bronchial diverticula (2.1 {+-} 2.0 mm, 1.8 {+-} 1.6) (p<0.05). Moreover, tracheal diverticula (10.3 {+-} 7.4 mm) with emphysema in 13 patients (26%), were larger than those without emphysema (5.1 {+-} 3.0 mm) (p<0.05). On thin-section MDCT, the rates of incidence for tracheal and main bronchial diverticula are about 5%, respectively. Tracheal diverticula in the right posterolateral wall are smaller and fewer than the main bronchial diverticula, which are located primarily in the inferior wall of the left bronchus. Tracheal diverticula with emphysema are larger than those without emphysema.

  11. Tracheal involvement of bronchus-associated lymphoid tissue lymphoma: a case report

    International Nuclear Information System (INIS)

    Sohn, Kyung Sik; Jeon, Kyung Neough; Kang, Duk Sik

    2002-01-01

    Primary malignant tumors of the trachea are rare, the most prevalent histologies beeing squamous cell and adenoid cystic carcinoma. A review of the literature revealed only ten cases of primary tracheal or bronchial non-Hodgkin's lymphoma. We describe a case in which tracheal involvement of bronchus-associated lymphoid tissue lymphoma, a subtype of non-Hodgkin's lymphoma, occurred

  12. Both rare and de novo copy number variants are prevalent in agenesis of the corpus callosum but not in cerebellar hypoplasia or polymicrogyria.

    Directory of Open Access Journals (Sweden)

    Samin A Sajan

    Full Text Available Agenesis of the corpus callosum (ACC, cerebellar hypoplasia (CBLH, and polymicrogyria (PMG are severe congenital brain malformations with largely undiscovered causes. We conducted a large-scale chromosomal copy number variation (CNV discovery effort in 255 ACC, 220 CBLH, and 147 PMG patients, and 2,349 controls. Compared to controls, significantly more ACC, but unexpectedly not CBLH or PMG patients, had rare genic CNVs over one megabase (p = 1.48×10⁻³; odds ratio [OR] = 3.19; 95% confidence interval [CI] = 1.89-5.39. Rare genic CNVs were those that impacted at least one gene in less than 1% of the combined population of patients and controls. Compared to controls, significantly more ACC but not CBLH or PMG patients had rare CNVs impacting over 20 genes (p = 0.01; OR = 2.95; 95% CI = 1.69-5.18. Independent qPCR confirmation showed that 9.4% of ACC patients had de novo CNVs. These, in comparison to inherited CNVs, preferentially overlapped de novo CNVs previously observed in patients with autism spectrum disorders (p = 3.06×10⁻⁴; OR = 7.55; 95% CI = 2.40-23.72. Interestingly, numerous reports have shown a reduced corpus callosum area in autistic patients, and diminished social and executive function in many ACC patients. We also confirmed and refined previously known CNVs, including significantly narrowing the 8p23.1-p11.1 duplication present in 2% of our current ACC cohort. We found six novel CNVs, each in a single patient, that are likely deleterious: deletions of 1p31.3-p31.1, 1q31.2-q31.3, 5q23.1, and 15q11.2-q13.1; and duplications of 2q11.2-q13 and 11p14.3-p14.2. One ACC patient with microcephaly had a paternally inherited deletion of 16p13.11 that included NDE1. Exome sequencing identified a recessive maternally inherited nonsense mutation in the non-deleted allele of NDE1, revealing the complexity of ACC genetics. This is the first systematic study of CNVs in congenital brain malformations, and

  13. Congenital symmastia revisited

    DEFF Research Database (Denmark)

    Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik

    2012-01-01

    Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia...... is a rare condition in which web-like soft tissue traverses the sternum to connect the breasts medially. The literature on congenital symmastia is limited, few cases have been published, and knowledge about ideal treatment is still insufficient....

  14. An embryological point of view on associated congenital anomalies of children with Hirschsprung disease.

    Science.gov (United States)

    Slavikova, T; Zabojnikova, L; Babala, J; Varga, I

    2015-01-01

    The most common congenital gut motility disorder is the Hirschsprung disease (HSCR). This anomaly is characterized by absence of neural crest-derived enteric neuronal ganglia. The aim of our study was to analyze the relationship between HSCR and other congenital anomalies or malfunctions. We examined 130 patients with Hirschsprung disease from Slovakia for last 10 years. During patients examination we focused not only on morphological abnormalities, but also functional anomalies. The incidence of associated congenital anomalies in our patients with HSCR was 26.1 %. But if we add functional defects (hypothyroidism, malfunction in cellular immunity, neurological deficit) to the morphological congenital abnormalities, the rate of the patients with HSCR with additional defects achieves 50.1 %. Nine of our patients (6.9 %) had syndromic HSCR. The most frequent disorder (13.6 % of patients) was primary deficiency in cellular immunity. More than 12.3 % of patients with HSCR had genitourinary abnormalities, in 10.0 % of patients variable degree of psychomotor retardation was observed, and skeletal, muscle and limb anomalies involved 7.7 % of patients. In 7.6 % cases of patients we found congenital hypothyroidism (including 2 cases of agenesis of thyroid gland). More than 6.1 % of patients presented with an associated anomaly in gastrointestinal tract (mostly anorectal malformations). Up to 5.5 % patients had congenital anomaly of heart, 3.8 % had ophthalmic and 3.1 % had craniofacial anomalies. Down syndrome was the main diagnosis in 3.8 % patients. We discussed  the relationship between HSCR and other anomalies, which are probably caused by abnormal migration, proliferation, or differentiation, of neural crest cells during embryogenesis (Tab. 1, Fig. 2, Ref. 75).

  15. Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract.

    Science.gov (United States)

    Ramanathan, Subramaniyan; Kumar, Devendra; Khanna, Maneesh; Al Heidous, Mahmoud; Sheikh, Adnan; Virmani, Vivek; Palaniappan, Yegu

    2016-02-28

    Congenital abnormalities of the kidney and urinary tract (CAKUT) include a wide range of abnormalities ranging from asymptomatic ectopic kidneys to life threatening renal agenesis (bilateral). Many of them are detected in the antenatal or immediate postnatal with a significant proportion identified in the adult population with varying degree of severity. CAKUT can be classified on embryological basis in to abnormalities in the renal parenchymal development, aberrant embryonic migration and abnormalities of the collecting system. Renal parenchymal abnormalities include multi cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), shape and cystic renal diseases. Aberrant embryonic migration encompasses abnormal location and fusion anomalies. Collecting system abnormalities include duplex kidneys and Pelvi ureteric junction obstruction. Ultrasonography (US) is typically the first imaging performed as it is easily available, non-invasive and radiation free used both antenatally and postnatally. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful to confirm the ultrasound detected abnormality, detection of complex malformations, demonstration of collecting system and vascular anatomy and more importantly for early detection of complications like renal calculi, infection and malignancies. As CAKUT are one of the leading causes of end stage renal disease, it is important for the radiologists to be familiar with the varying imaging appearances of CAKUT on US, CT and MRI, thereby helping in prompt diagnosis and optimal management.

  16. A cross-sectional analysis of the prevalence of tooth agenesis and structural dental anomalies in association with cleft type in non-syndromic oral cleft patients.

    Science.gov (United States)

    Konstantonis, Dimitrios; Alexandropoulos, Alexandros; Konstantoni, Nikoleta; Nassika, Maria

    2017-12-01

    The aim of this study was to investigate the prevalence of tooth agenesis, microdontia, and tooth malformation among non-syndromic oral cleft patients and their potential association with cleft type and gender. Intraoral records and radiographs of 154 patients (97 males and 57 females) were examined. The variables assessed were tooth agenesis, microdontia, dental malformations, and cleft types. The statistics included chi-square and Fisher's exact tests as well as logistic regression to assess any mutual effects of gender and cleft type on the dental variables. Tooth agenesis occurred in 50% of the sample and microdontia in 18%. Non-statistically significant odds ratios for the association of gender and cleft type with tooth agenesis were obtained. Tooth agenesis was substantially higher at the unilateral right CL + P and the bilateral CL + P in quadrant 1 and at the unilateral left CL + P and bilateral CL + P in quadrant 2. It was also higher, at the isolated cleft palate (CP) in quadrants 3 and 4. These results were attributed to teeth 22 (31.8%) and 12 (21.6%) in the maxilla and to teeth 35 (6.1%) and 45 (5.4%) in the mandible. In unilateral CL + P patients, the cleft quadrant that presented tooth agenesis was associated with the side of the cleft. Interdisciplinary treatment of the oral cleft patients should take into consideration the high prevalence of tooth agenesis and their association with the different cleft types. The most frequently affected teeth by cleft are by far the upper lateral incisors. Results indicate that tooth agenesis appears to be a genetically controlled anomaly related to the orofacial cleft development through various genetic links and not caused by the cleft disruptive process.

  17. Congenital renal anomalies in cloacal exstrophy: Is there a difference?

    Science.gov (United States)

    Suson, K D; Inouye, B; Carl, A; Gearhart, J P

    2016-08-01

    . There were also clinical implications. Women with renal agenesis and uterine anomalies were more likely to have endometriosis than those with isolated uterine anomalies, but were also more likely to have successful pregnancies. Males may have had an analogous condition with renal agenesis and seminal vesicle cysts. Future research into long-term kidney function in this population, uterine function, and possible male sexual duct malformation is warranted. Congenital renal anomalies occurred frequently in children with CE. They were more common in boys than in girls. Girls with abnormal Müllerian anatomy were more likely to have anomalous renal development. Mesonephric duct dysfunction may be embyologically responsible for both renal and Müllerian maldevelopment. Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

  18. Management of tracheal chondrosarcoma almost completely obstructing the airway: a case report.

    Science.gov (United States)

    Andolfi, Marco; Vaccarili, Maurizio; Crisci, Roberto; Puma, Francesco

    2016-07-11

    Primary malignant tracheal tumors account for only 0.2 % of all malignancies of the respiratory tract. Tracheal chondrosarcoma is a rare condition and only 17 cases have been described in the literature from 1965 to date. Herein we report the very unusual case of a patient with a tracheal chondrosarcoma, electively treated by curative surgery despite the virtually complete obstruction of the airway. We present the case of a 79-year old Caucasian man with long-lasting wheezing misdiagnosed as asthma and affected by a tracheal chondrosarcoma almost completely obstructing the airway. Videobronchoscopy and imaging investigations revealed a well-circumscribed mass arising from the cartilaginous rings of the cervical trachea with a posterior residual respiratory space of about 1 mm. Because of the mobility and flaccidity of the uninvolved pars membranacea, the tiny respiratory space slightly expanded during inspiration and expiration allowing the patient to be treated without an essential emergency procedure. Standard tracheal intubation was impossible. Rigid bronchoscopy enabled placement of a small tracheal tube distally to the tumor. Successful cervical tracheal resection and reconstruction was then performed, achieving complete tumor excision. Histologically, the mass was characterized as a low-grade tracheal chondrosarcoma. Videobronchoscopy performed 9 months after surgery showed a wide, well healed tracheal anastomosis. Ten months after surgery, the patient is alive and disease free. Complete surgical resection is the treatment of choice for tracheal chondrosarcoma. Rigid bronchoscopy is an essential tool for diagnostic and therapeutic purposes. It allows the palliative maneuvers for obstruction relief but also, in resectable patients, the intraoperative safe and straightforward management of the obstructed airway.

  19. Effects of hydration and physical therapy on tracheal transport velocity

    International Nuclear Information System (INIS)

    Chopra, S.K.; Taplin, G.V.; Simmons, D.H.; Coulson, A.; Elam, D.

    1976-01-01

    A new tracer method for quantitative measurements of tracheal transport velocity (TTV) in mm/min in dogs has been described recently. Using the same technique, the effects of dehydration, hydration, postural drainage and physical therapy on TTV were studied. There was a significant (p < 0.05) reduction in TTV following dehydration and these values reverted to normal with hydration in all ten dogs. Tracheal transport velocity increased on the average of 39.7 percent with a mean change of 7.7 mm/min (p < 0.01) following postural drainage in seven dogs. On the other hand, TTV increased on the average of 51 percent with a mean change of 8.2 mm/min (p <0.01) following chest percussion in six dogs. Postural drainage and chest percussion each increased TTV significantly beyond the base-line values. However, hydration only restored TTV to base-line values when applied to dogs in the hydropenic state. These therapeutic measures have been used empirically in the past. The present study gives objective evidence of their beneficial effects in dogs and suggests that such treatments may have a definite scientific basis for clinical application in chronic obstructive airways disease

  20. A case of tracheal leiomyoma misdiagnosed as asthma.

    Science.gov (United States)

    Öztürk, Ayperi; Aktaş, Zafer; Yılmaz, Aydın; Yeşildağlı, Havva; Memiş, Leyla

    2016-12-01

    Primary benign tumors of trachea are rare. Of them, tracheal leiomyoma, constitutes only 1% of all benign lower respiratory tract tumors. Here, we present a case of tracheal leiomyoma who has been receiving high doses of inhaled corticosteroids and bronchodilators for a year with a misdiagnosis of asthma. As the symptoms did not resolve with an overtreatment, she has been undergone radiologic study to find a possible alternative diagnosis. The chest roentgenogram revealed an opacity in the upper mediastinum. In computed tomography, a lesion has been detected in proximal trachea, arising from the posterior wall and protruding through the lumen and almost obliterating the air column. Rigid bronchoscopy has been performed under general anesthesia due to a high risk of bleeding and the endobronchial lesion, freely moving with respiration, has been removed and cryotherapy was applied to the base of the lesion. Receiving the histopathological diagnosis of leiomyoma, the patient is now on 12th month of the follow-up without any recurrence.

  1. Congenital orbital teratoma.

    Science.gov (United States)

    Aiyub, Shereen; Chan, Wengonn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-12-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  2. Congenital orbital teratoma

    Directory of Open Access Journals (Sweden)

    Shereen Aiyub

    2013-01-01

    Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  3. Congenital Constriction Band Syndrome

    OpenAIRE

    Rajesh Gupta, Fareed Malik, Rishabh Gupta, M.A.Basit, Dara Singh

    2008-01-01

    Congenital constriction bands are anomalous bands that encircle a digit or an extremity. Congenitalconstriction band syndrome is rare condition and is mostly associated with other musculoskeletaldisorders.We report such a rare experience.

  4. Congenital anterior urethral diverticulum

    International Nuclear Information System (INIS)

    Jung, Hyun Sub; Chung, Young Sun; Suh, Chee Jang; Won, Jong Jin

    1985-01-01

    Two cases of congenital anterior urethral diverticular which have occurred in a 4 year old and one month old boy are presented. Etiology, diagnostic procedures, and its clinical results are briefly reviewed

  5. Congenital chylous ascites

    International Nuclear Information System (INIS)

    Romanska-Kita, J.; Borszewska-Kornacka, M. K.; Rudzinska, I.; Wawrzoniak, T.; Dobrzanska, A.; Czech-Kowalska, J.

    2011-01-01

    Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity. (authors)

  6. Multifocal Congenital Hemangiopericytoma.

    Science.gov (United States)

    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

  7. Association of tracheal mucus or blood and airway neutrophilia with racing performance in Thoroughbred horses in an Australian racing yard.

    Science.gov (United States)

    Salz, R O; Ahern, B J; Boston, R; Begg, L M

    2016-04-01

    To determine the variation of tracheal mucus scores, tracheal blood scores and transendoscopic tracheal wash (TW) cytology in a population of Thoroughbred (TB) racehorses and assess their association with racing performance. A total of 220 endoscopic examinations were performed and TWs obtained from 155 TB racehorses. Samples were collected 60-120 min following gallop work. Tracheal mucus score, tracheal blood score and TW cytology were analysed and their association with racing performance assessed. Of the total examinations and samples, 194 from 135 horses fitted the criteria for inclusion. The overall prevalence of visible tracheal mucus was 2.5% (5/194) and of increased tracheal mucus was 0%. The prevalence of visible tracheal blood was 8.8% (17/194) and of increased tracheal blood was 4.6% (9/194). A total of 36% (70/194) of TWs contained elevated percentages of neutrophils and of these, 96% (67/70) occurred in the absence of any visible tracheal mucus. There was no significant association between tracheal mucus score or TW cytology and subsequent racing performance. There was a statistically significant association (P = 0.004) between increased tracheal blood scores and poor racing performance. Visible tracheal blood seen after strenuous exercise in clinically normal TB racehorses was a risk factor for poor racing performance, but the presence of airway neutrophilia was not. No horses in this study were found to have increased tracheal mucus, so the association of increased tracheal mucus with racing performance could not be assessed. © 2016 Australian Veterinary Association.

  8. Ciliated cells in vitamin A-deprived cultured hamster tracheal epithelium do divide

    International Nuclear Information System (INIS)

    Rutten, A.A.; Beems, R.B.; Wilmer, J.W.; Feron, V.J.

    1988-01-01

    The pseudostratified tracheal epithelium, composed of a heterogeneous phenotypically varying cell population, was studied with respect to the in vitro cell proliferative activity of differentiated epithelial cells. Ciliated tracheal epithelial cells so far have been considered to be terminally differentiated, nonproliferating cells. Tracheal organ cultures obtained from vitamin A-deprived Syrian Golden hamsters were cultured in a vitamin A-deficient, serum-free, hormone-supplemented medium. In vitamin A-deprived tracheal epithelium treated with physiologically active all-trans retinol and low cigarette-smoke condensate concentrations it is possible to stimulate the cell proliferation of both basal and columnar cells. Therefore, the probability of finding proliferating columnar cells was increased compared with the in vivo and the vitamin A-deprived situation in which cell proliferative activity is relatively low. In the presence of cigarette-smoke condensate in a noncytotoxic concentration, basal, small mucous granule, ciliated, and indifferent tracheal epithelial cells incorporated [methyl-3H]-thymidine into the DNA during the S phase. The finding that ciliated cells were labeled was supported by serial sections showing the same labeled ciliated cell in two section planes separated by 2 to 3 micron, without labeled epithelial cells next to the ciliated cell. Furthermore, a ciliated tracheal epithelial cell incorporating [methyl- 3 H]thymidine into DNA was also seen in tracheal cultures of vitamin A-deprived hamsters treated with all-trans retinol in a physiologic concentration

  9. Tracheal CT morphology: correlation with distribution and extent of thoracic adipose tissue

    Energy Technology Data Exchange (ETDEWEB)

    Ap Dafydd, Derfel [Imperial College Healthcare NHS Trust, Department of Radiology, Charing Cross Hospital, London (United Kingdom); Desai, Sujal R. [King' s College Hospital NHS Foundation Trust, King' s College London, King' s Health Partners, London (United Kingdom); Gordon, Fabiana; Copley, Susan J. [Imperial College, London (United Kingdom)

    2016-10-15

    To evaluate the relationship between adipose tissue measurements and anterior bowing of the posterior tracheal wall in a large nonselected group of patients undergoing CT pulmonary angiography (CTPA). Consecutive patients undergoing CTPA over a 4-month period were analyzed retrospectively. Using an adapted scoring system (posterior bowing, flattening, mild/moderate or severe anterior bowing of the posterior tracheal membrane), the axial morphology and cross-sectional area of the trachea at the narrowest point and 1 cm above the aortic arch were evaluated. Measurements of adipose tissue were taken (anterior mediastinal fat width, sagittal upper abdominal diameter and subcutaneous fat thickness at the level of the costophrenic angle). Relationships between tracheal morphology and measurements of adipose tissue were analyzed. 296 patients were included (120 males, 176 females, mean age 59 years, range 19-90). Severe anterior bowing of the posterior tracheal wall correlated with increasing sagittal upper abdominal diameter (p = 0.002). Mild/moderate and severe anterior bowing of the posterior tracheal wall correlated with increasing mediastinal fat width (p = 0.000 and p = 0.031, respectively). Tracheal cross-sectional area was inversely correlated with increasing subcutaneous fat thickness (p = 0.022). The findings demonstrate a statistically significant relationship between CT tracheal morphology and adipose tissue measurements in a large nonselected population. (orig.)

  10. Tracheal rupture caused by blunt chest trauma: radiological and clinical features

    Energy Technology Data Exchange (ETDEWEB)

    Kunisch-Hoppe, M.; Rauber, K.; Rau, W.S. [Dept. of Diagnostic Radiology, Justus Liebig Univ., Giessen (Germany); Hoppe, M. [Dept. of Diagnostic Radiology, University Hospital, Philipps University, Marburg (Germany); Popella, C. [Dept. of ENT, Justus Liebig University, Giessen (Germany)

    2000-03-01

    The aim of this study was to assess radiomorphologic and clinical features of tracheal rupture due to blunt chest trauma. From 1992 until 1998 the radiomorphologic and clinical key findings of all consecutive tracheal ruptures were retrospectively analyzed. The study included ten patients (7 men and 3 women; mean age 35 years); all had pneumothoraces which were persistent despite suction drainage. Seven patients developed a pneumomediastinum as well as a subcutaneous emphysema on conventional chest X-rays. In five patients, one major hint leading to the diagnosis was a cervical emphysema, discovered on the lateral cervical spine view. Contrast-media-enhanced thoracic CT was obtained in all ten cases and showed additional injuries (atelectasis n = 5; lung contusion n = 4; lung laceration n = 2; hematothorax n = 2 and hematomediastinum n = 4). The definite diagnosis of tracheal rupture was made by bronchoscopy, which was obtained in all patients. Tracheal rupture due to blunt chest trauma occurs rarely. Key findings were all provided by conventional chest X-ray. Tracheal rupture is suspected in front of a pneumothorax, a pneumomediastinum, or a subcutaneous emphysema on lateral cervical spine and chest films. Routine thoracic CT could also demonstrate these findings but could not confirm the definite diagnosis of an tracheal rupture except in one case; in the other 9 cases this was done by bronchoscopy. Thus, bronchoscopy should be mandatory in all suspicious cases of tracheal rupture and remains the gold standard. (orig.)

  11. Tracheal rupture caused by blunt chest trauma: radiological and clinical features

    International Nuclear Information System (INIS)

    Kunisch-Hoppe, M.; Rauber, K.; Rau, W.S.; Hoppe, M.; Popella, C.

    2000-01-01

    The aim of this study was to assess radiomorphologic and clinical features of tracheal rupture due to blunt chest trauma. From 1992 until 1998 the radiomorphologic and clinical key findings of all consecutive tracheal ruptures were retrospectively analyzed. The study included ten patients (7 men and 3 women; mean age 35 years); all had pneumothoraces which were persistent despite suction drainage. Seven patients developed a pneumomediastinum as well as a subcutaneous emphysema on conventional chest X-rays. In five patients, one major hint leading to the diagnosis was a cervical emphysema, discovered on the lateral cervical spine view. Contrast-media-enhanced thoracic CT was obtained in all ten cases and showed additional injuries (atelectasis n = 5; lung contusion n = 4; lung laceration n = 2; hematothorax n = 2 and hematomediastinum n = 4). The definite diagnosis of tracheal rupture was made by bronchoscopy, which was obtained in all patients. Tracheal rupture due to blunt chest trauma occurs rarely. Key findings were all provided by conventional chest X-ray. Tracheal rupture is suspected in front of a pneumothorax, a pneumomediastinum, or a subcutaneous emphysema on lateral cervical spine and chest films. Routine thoracic CT could also demonstrate these findings but could not confirm the definite diagnosis of an tracheal rupture except in one case; in the other 9 cases this was done by bronchoscopy. Thus, bronchoscopy should be mandatory in all suspicious cases of tracheal rupture and remains the gold standard. (orig.)

  12. Awake tracheal intubation using Pentax airway scope in 30 patients: A Case series

    Directory of Open Access Journals (Sweden)

    Payal Kajekar

    2014-01-01

    Full Text Available Background and Aims: Pentax airway scope (AWS has been successfully used for managing difficult intubations. In this case series, we aimed to evaluate the success rate and time taken to complete intubation, when AWS was used for awake tracheal intubation. Methods: We prospectively evaluated the use of AWS for awake tracheal intubation in 30 patients. Indication for awake intubation, intubation time, total time to complete tracheal intubation, laryngoscopic view (Cormack and Lehane grade, total dose of local anaesthetic used, anaesthetists rating and patient′s tolerance of the procedure were recorded. Results: The procedure was successful in 25 out of the 30 patients (83%. The mean (standard deviation intubation time and total time to complete the tracheal intubation was 5.4 (2.4 and 13.9 (3.7 min, respectively in successful cases. The laryngeal view was grade 1 in 24 and grade 2 in one of 25 successful intubations. In three out of the five patients where the AWS failed, awake tracheal intubation was successfully completed with the assistance of flexible fibre optic scope (FOS. Conclusion: Awake tracheal intubation using AWS was successful in 83% of patients. Success rate can be further improved using a combination of AWS and FOS. Anaesthesiologists who do not routinely use FOS may find AWS easier to use for awake tracheal intubation using an oral route.

  13. Clinical application of self-expandable metallic stents in the treatment of malignant tracheal stenosis under general anesthesia

    International Nuclear Information System (INIS)

    Wang Weitao; Shi Haibin; Yang Zhengqiang; Liu Sheng; Zhou Chungao; Zhao Linbo; Xia Jinguo; Li Linsun

    2009-01-01

    Objective: To evaluate the safety and efficacy of self-expandable metallic stent placement for the treatment of malignant tracheal stenosis under general anesthesia and fluoroscopic guidance. Methods: Under general anesthesia the placement of self-expandable metallic stent was performed in 10 patients with malignant tracheal stenosis, the procedure was completed under fluoroscopic guidance in all patients. Results: Successful tracheal stenting was achieved in all 10 patients. In one patient, a Y-shaped stent was used as the tracheal carina was involved in the airway stenosis. The symptoms of dyspnea and asthma were markedly improved immediately after the implantation of stent in all patients. Conclusion: Tracheal implantation of self-expandable metallic stent under general anesthesia and fluoroscopic guidance is a safe and effective treatment for malignant tracheal stenosis, it can promptly relieve various symptoms caused by malignant tracheal stenosis and obviously improve patient's living quality, therefore,t his technique is of great value in clinical practice. (authors)

  14. Use of the peritracheal fold in the dog tracheal transplantation model.

    Science.gov (United States)

    Gannon, P J; Costantino, P D; Lueg, E A; Chaplin, J M; Brandwein, M S; Passalaqua, P J; Fliegelman, L J; Laitman, J T; Marquez, S; Urken, M L

    1999-09-01

    To investigate the technical aspects of the canine model of human tracheal transplantation for potential application to reconstruction of extremely long tracheal defects (> 10 cm). In phase 1, long tracheal segments were skeletonized and pedicled with the thyroid glands, cranial thyroid arteries and veins, and internal jugular vein branches. The segments were elevated completely, attached to the vascular pedicle only, and replaced with primary tracheal anastomoses. In phase 2, long segments were elevated along with a diffuse soft tissue "blanket" that envelops the trachea and thyroid glands. Because this study was designed to primarily address, in situ, tracheal perfusion territories of a cranially located vascular pedicle, microvascular anastomoses were not conducted. Two small-bodied beagles (10-15 kg) and 5 large-bodied mixed-breed dogs (20-30 kg) were humanely killed 2 to 41 days after surgery, and anatomic and histological analyses were conducted. Unlike that of humans, the thyroid gland complex of dogs is not intimately associated with the trachea but is conjoined with a peritracheal soft tissue "fold." Within this fold, blood is transmitted to the trachea via a diffuse, segmental vascular plexus. In phase 1, pronounced tracheal necrosis occurred within 2 to 5 days. In phase 2, extremely long tracheal segments (10-12 cm), based only on a cranially located pedicle, were still viable at 2 to 6 weeks. Preservation of the "peritracheal fold" in the dog model of tracheal transplantation is critical to the onset and maintenance of vascular perfusion in a long tracheal segment. Furthermore, the use of large-bodied dogs is necessary to provide for a usable venous efflux component.

  15. Variations of CITED2 are associated with congenital heart disease (CHD in Chinese population.

    Directory of Open Access Journals (Sweden)

    Yan Liu

    Full Text Available CITED2 was identified as a cardiac transcription factor which is essential to the heart development. Cited2-deficient mice showed cardiac malformations, adrenal agenesis and neural crest defects. To explore the potential impact of mutations in CITED2 on congenital heart disease (CHD in humans, we screened the coding region of CITED2 in a total of 700 Chinese people with congenital heart disease and 250 healthy individuals as controls. We found five potential disease-causing mutations, p.P140S, p.S183L, p.S196G, p.Ser161delAGC and p. Ser192_Gly193delAGCGGC. Two mammalian two-hybrid assays showed that the last four mutations significantly affected the interaction between p300CH1 and CITED2 or HIF1A. Further studies showed that four CITED2 mutations recovered the promoter activity of VEGF by decreasing its competitiveness with HIF1A for binding to p300CH1 and three mutations decreased the consociation of TFAP2C and CITED2 in the transactivation of PITX2C. Both VEGF and PITX2C play very important roles in cardiac development. In conclusion, we demonstrated that CITED2 has a potential causative impact on congenital heart disease.

  16. Clinical and neuroimaging profile of congenital brain malformations in children with spastic cerebral palsy

    International Nuclear Information System (INIS)

    Kulak, W.; Okurowska-Zawada, B.; Sobaniec, W.; Goscik, M.; Olenski, J.

    2008-01-01

    Purpose: Analysis of the incidence of congenital brain malformations in children with spastic cerebral palsy (CP) in a hospital based study. Material and Methods: The present study included 74 boys and 56 girls with spastic tetraplegia, diplegia, and hemiplegia CP. Magnetic resonance imaging MRI findings were analyzed in children with CP. Results: Significant abnormalities relevant to the CP were evident on MRI in 124 (95.3%) subjects. Periventicular leukomalacia (PVL) was detected more frequently in children with spastic diplegia than in patients with tetraplegia or hemiplegia. Cerebral atrophy was found more often in the tetraplegic group compared to the diplegic patients. Porencephalic cysts were detected more often in children with spastic hemiplegia. Congenital brain anomalies were evident in 15 (10.7%) children with spastic CP. Brain malformations included: schizencephaly (5), agenesis corpus callosum (4), polymicrogyria (2), holoprosencephaly (2) and lissencephaly (2). Intractable epilepsy and mental retardation were observed more often in children with brain anomalies. Twelve patients with congenital brain malformations were born at term and three born at preterm. Conclusions: Neuroimaging results in children with CP may help determine the etiology and make better prognosis of CP. (authors)

  17. The association of congenital neuroblastoma and congenital heart disease

    International Nuclear Information System (INIS)

    Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

    1989-01-01

    Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

  18. Congenital malaria in China.

    Directory of Open Access Journals (Sweden)

    Zhi-Yong Tao

    2014-03-01

    Full Text Available BACKGROUND: Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum-endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax-endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. METHODS/PRINCIPAL FINDINGS: Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%, reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients

  19. Diagnosis of tracheomalacia using tracheal dynamic respiratory-scan

    International Nuclear Information System (INIS)

    Matsui, Teruo; Watabe, Hideki; Ikeda, Sadao

    1995-01-01

    We performed tracheal-dynamic respiratory scan (T-DRS) in 54 cases which were thought to be tracheomalacia clinically. T-DRS was performed by TOSHIBA TCT-900S at the level of the aortic arch under the condition of 120 kV (140 kV), 100 mA, 5 mm of slice width for 20 seconds when the patient was directed to breath rapidly or slowly. In 19 cases, which underwent both T-DRS and bronchoscopy, could evaluate the results. Only one case was overestimated by T-DRS than bronchoscopic findings, while 9 cases were underestimated and 9 cases were equal. Though T-DRS tended to underestimate the degree of the stenosis of tracheomalacia than bronchoscopic finding. T-DRS can do more objective evaluation than bronchoscopy non-invasively and thought to be useful for the evaluation of tracheomalalcia. (author)

  20. Effects of preoperative irradiation on primary tracheal anastomosis

    International Nuclear Information System (INIS)

    Tsubota, N.; Simpson, W.J.; Van Nostrand, A.W.P.; Pearson, F.G.

    1975-01-01

    Preoperative radiotherapy was used in the management of selected patients with cancer of the lung or trachea who might subsequently require segmental resection of bronchus or trachea and reconstruction by primary anastomosis. This study was designed to determine the effects of varying dosages of preoperative irradiation on anastomotic healing. Two rings were resected from the cervical trachea of 20 dogs following irradiation with varying doses of cesium. There were no important adverse effects on healing of the trachea or adjacent organs in dogs receiving up to 3,500 rads. All dogs receiving a higher dose than this developed some anastomotic stenosis, which was severe in 6 of 8 animals. Similar adverse effects were observed in 2 patients managed by preoperative radiotherapy (4,000 rads in three weeks) and tracheal resection with primary anastomosis

  1. Management of Tracheal Deformity During Intubation: A Case Report

    Directory of Open Access Journals (Sweden)

    Sheng-Hua Wu

    2007-06-01

    Full Text Available Failure or difficulty in intubating the trachea can be either due to inability to visualize the glottis or some pathology at the level of or below the cords. This report describes a case of difficult intubation suspected of being related to neck scarring from previous surgery. Computed tomography (CT was used to evaluate the patient's airway and revealed upper tracheal angulation. We describe a method to secure the airway in this patient with a two-person technique by rotating an oral endotracheal tube 180° counterclockwise to adjust to the curvature of the trachea. Problems with intubation should be anticipated in patients with scarring of the neck, and equipment for aiding intubation should be on hand. Furthermore, we found that CT contributed to the assessment of the difficulty of intubation in this kind of patient.

  2. Effects of preoperative irradiation on primary tracheal anastomosis

    Energy Technology Data Exchange (ETDEWEB)

    Tsubota, N.; Simpson, W.J.; Van Nostrand, A.W.P.; Pearson, F.G.

    1975-08-01

    Preoperative radiotherapy was used in the management of selected patients with cancer of the lung or trachea who might subsequently require segmental resection of bronchus or trachea and reconstruction by primary anastomosis. This study was designed to determine the effects of varying dosages of preoperative irradiation on anastomotic healing. Two rings were resected from the cervical trachea of 20 dogs following irradiation with varying doses of cesium. There were no important adverse effects on healing of the trachea or adjacent organs in dogs receiving up to 3,500 rads. All dogs receiving a higher dose than this developed some anastomotic stenosis, which was severe in 6 of 8 animals. Similar adverse effects were observed in 2 patients managed by preoperative radiotherapy (4,000 rads in three weeks) and tracheal resection with primary anastomosis.

  3. Congenital cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Katarina Rednak-Paradiž

    2006-11-01

    Full Text Available Background: CMV is the most common agent that causes congenital virus infection. Only 10 % of infected children have symptomatic infection immediately after birth. Signs of central nervous system damage, neurosensory deafness and delayed psychomotor development may manifest as a result of asimptomatic congenital infection later in childhood. In the article we present basic properties of CMV; we describe clinical picture of the congenital infection and possibilities of diagnose and its treatment. We present five children with symptomatic congenital CMV infection that were hospitalized for the period 1992–2002 at the Neonatal department in the University Children’s Hospital in Ljubljana.Conclusions: Identification of infected neonates, especially those with asimptomatic congenital CMV infection, is difficult. Latest incidence of infection in Slovenia is unknown. With new investigations the efficiency of antiviral therapy was discovered but exact indications for therapy are not yet known. CMV vaccine, once available, may ultimately be the best control strategy for this important public health problem. Proper educating women in childbearing age about the risks of CMV and how to avoid disease transmission during pregnancy (hand washing, avoiding mouth-to-mouth contact with preschool children, usage of gloves especially when handling dipers or respiratory secretions are the only control strategies available.

  4. Prevalence of congenital amusia.

    Science.gov (United States)

    Peretz, Isabelle; Vuvan, Dominique T

    2017-05-01

    Congenital amusia (commonly known as tone deafness) is a lifelong musical disorder that affects 4% of the population according to a single estimate based on a single test from 1980. Here we present the first large-based measure of prevalence with a sample of 20 000 participants, which does not rely on self-referral. On the basis of three objective tests and a questionnaire, we show that (a) the prevalence of congenital amusia is only 1.5%, with slightly more females than males, unlike other developmental disorders where males often predominate; (b) self-disclosure is a reliable index of congenital amusia, which suggests that congenital amusia is hereditary, with 46% first-degree relatives similarly affected; (c) the deficit is not attenuated by musical training and (d) it emerges in relative isolation from other cognitive disorder, except for spatial orientation problems. Hence, we suggest that congenital amusia is likely to result from genetic variations that affect musical abilities specifically.

  5. Surgical treatment of benign tracheo-oesophageal fistulas with tracheal resection and oesophageal primary closure: is the muscle flap really necessary?

    Science.gov (United States)

    Camargo, José Jesus; Machuca, Tiago Noguchi; Camargo, Spencer Marcantônio; Lobato, Vivalde F; Medina, Carlos Remolina

    2010-03-01

    Nowadays, despite the advances of the low-pressure high-volume cuffs, post-intubation tracheo-oesophageal fistula (TEF) still poses a major challenge to thoracic surgeons. The original technique includes interposition of muscle flaps between suture lines to avoid recurrence. It is not clear if this manoeuvre is indispensable and, in fact, we and others have faced problems with it. Our aim is to present our experience with TEF management in a consecutive group with no muscle interposition. From June 1992 to November 2007, we evaluated 14 patients presenting with TEF, with a mean age of 44 years (from 18 to 79 years). Thirteen patients had a prolonged intubation history. The remaining case was a 40-year-old male with congenital TEF. Three patients had been previously submitted to failed repairs in other institutions. Ten patients had associated tracheal stenosis, which was subglottic in three of them. Regarding surgical technique, in all cases, we performed a single-staged procedure, which consisted of tracheal resection and anastomosis with double-layer oesophageal closure. In none of our cases was a muscle flap interposed between suture lines. All operations were performed through a cervical incision; however, in one case, an extension with partial sternotomy was required. There was no operative mortality. Thirteen patients were extubated in the first 24h after the procedure, while one patient required 48 h of mechanical ventilation. Four complications were recorded: one each of pneumonia and left vocal cord paralysis and two small tracheal dehiscences managed with a T-tube and a tracheostomy tube. After discharge, three patients returned to their native cities and were lost to follow-up. The remaining 11 patients have been followed up by a mean of 32 months (from three to 108 months), with 10 presenting excellent and one good anatomic and functional results. The single-staged repair with tracheal resection and anastomosis with oesophageal closure provides good

  6. Ethical issues in laryngology: tracheal stenting as palliative care.

    Science.gov (United States)

    Kazi, Aasif A; Flowers, W Jeffrey; Barrett, Jeanna M; O'Rourke, Ashli K; Postma, Gregory N; Weinberger, Paul M

    2014-07-01

    To improve understanding of aspects of end-of-life care that may not be intuitive to the otolaryngology community. A comprehensive review of the literature was performed by searching Medline, Embase, and Google Scholar databases. Primary manuscripts' bibliographies were reviewed to identify any nonindexed references. Prospective consultation by means of one-on-one interviews was sought from nonotolaryngology key stakeholders in the areas of hospice nursing care and patient advocacy in order to identify pertinent issues. We identified over 1,000 articles published from 1965 to 2013 on the topic of tracheal stents, as well as over 40,000 on hospice/end-of-life care. Three articles focusing specifically on palliative care and airway stenting were identified, of which three were case reports and none were definitive reviews. There are a number of significant issues and concepts unique to hospice care. These are likely unfamiliar to all except for head and neck oncology-specialized otolaryngologists. An example is that hospice care focuses on quality of life rather than prolongation of life (such as curative surgery). Patients with nonoperable tracheal obstruction from malignancy face an unpleasant demise from suffocation. For those patients, stenting can relieve suffering by restoring airway patency. Airway stenting can be a valid palliative care option, even for terminal patients receiving hospice care, when performed to relieve airway obstruction and improve quality of life. End-of-life ethics is an underdeveloped area of otolaryngology that should be explored. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  7. Management of postintubation tracheal stenosis: appropriate indications make outcome differences.

    Science.gov (United States)

    Melkane, Antoine E; Matar, Nayla E; Haddad, Amine C; Nassar, Michel N; Almoutran, Homère G; Rohayem, Ziad; Daher, Mohammad; Chalouhy, Georges; Dabar, George

    2010-01-01

    Laryngotracheal stenosis is difficult to treat and its etiologies are multiple; nowadays, the most common ones are postintubation or posttracheostomy stenoses. To provide an algorithm for the management of postintubation laryngotracheal stenoses (PILTS) based on the experience of a tertiary care referral center. A retrospective study was conducted on all patients treated for PILTS over a 10-year period. Patients were divided into a surgically and an endoscopically treated group according to predefined criteria. The characteristics of the two groups were analyzed and the outcomes compared. Thirty-three consecutive patients were included in the study: 14 in the surgically treated group and 19 in the endoscopically treated group. Our candidates for airway surgery were healthy patients presenting with complex tracheal stenoses, subglottic involvement or associated tracheomalacia. The endoscopic candidates were chronically ill patients presenting with simple, strictly tracheal stenoses not exceeding 4 cm in length. Stents were placed if the stenosis was associated with tracheomalacia or exceeded 2 cm in total length. In the surgically treated group, 2/14 patients needed more than one procedure versus 8/19 patients in the endoscopically treated group. At the end of the intervention, 50% of the patients were decannulated in the surgically treated group versus 84.2% in the endoscopically treated group (p = 0.03). However, the decannulation rates at 6 months and the symptomatology at rest and on exertion on the last follow-up visit were comparable in the two groups. Our experience in the management of PILTS demonstrates that both surgery and endoscopy yield excellent functional outcomes if the treatment strategy is based on clear, predefined objective criteria. Copyright 2010 S. Karger AG, Basel.

  8. A comparison of simple continuous versus simple interrupted suture patterns for tracheal anastomosis after large-segment tracheal resection in dogs.

    Science.gov (United States)

    Fingland, R B; Layton, C I; Kennedy, G A; Galland, J C

    1995-01-01

    Two suture techniques for tracheal anastomosis after large-segment tracheal resection were compared. Eight cartilages were resected from the trachea of each of 12 dogs; anastomoses with 4-0 polydioxanone suture were created using a simple continuous suture technique in six dogs and a simple interrupted suture technique in six dogs. Surgical time was shorter but apposition of tracheal segments at the time of surgery was less precise with the simple continuous suture technique. The dogs were evaluated for 150 days after surgery. Clinical abnormalities after tracheal resection and anastomosis were not observed. Percent dorsoventral luminal stenosis was calculated by measuring the tracheal lumen diameter on lateral cervical radiographs. Percent luminal stenosis was calculated planimetrically using a computerized digitizing tablet. Anastomotic stenosis was mild in all dogs; however, the mean percent.luminal stenosis determined planimetrically was significantly greater for dogs that had the simple continuous suture technique. Planimetric measurements of cross-sectional area made before and after formalin fixation were not significantly different. Radiographic determination of percent dorsoventral luminal stenosis was a poor predictor of diminution of cross-sectional area determined planimetrically.

  9. MR imaging features of the congenital uterine anomalies

    International Nuclear Information System (INIS)

    Hamcan, S.; Akgun, V.; Battal, B.; Kocaoglu, M.

    2012-01-01

    Full text: Introduction: Congenital uterine anomalies are common and usually asymptomatic. The agenesis, malfusion or deficient resorption of the Mullerian canals during embryogenesis may lead to these anomalies. Although ultrasonography (US) is the first step imaging technique in assessment of the uterine pathologies, it can be insufficient in differentiation of them. Magnetic resonance (MR) imaging is an adequate imaging technique in depicting pelvic anatomy and different types of uterine anomalies. Objectives and tasks: In this article, we aimed to present imaging features of the uterine anomalies. Material and methods: Pelvic MR scans of the cases who were referred to our radiology department for suspicious uterine anomaly were evaluated retrospectively. Results: We determined uniconuate uterus (type II), uterus didelphys (type III), bicornuate uterus (type IV), uterine septum (type V) and arcuate uterus (type VI) anomalies according to ASRM (American Society of Reproductive Medicine) classification. Conclusion: In cases with such pathologies leading to obstruction, dysmenorrhea or palpable pelvic mass in the puberty are the main clinical presentations. In cases without obstruction, infertility or multiple abortions can be encountered in reproductive ages. The identification of the subtype of the uterine anomalies is important for the preoperative planning of the management. MR that has multiplanar imaging capability and high soft tissue resolution is a non-invasive and the most important imaging modality for the detection and classification of the uterine anomalies

  10. Usefulness of fetal MR imaging for congenital urological anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Akasaka, Yoshinobu; Sugimura, Kazuro [Kobe Univ. (Japan). Graduate School of Medicine; Kanegawa, Kimio [Kobe Children' s Hospital (Japan)

    2002-04-01

    Despite the fact that congenital urological anomalies are not rare, the role of fetal MRI in these disorders has not been well defined. We evaluated the usefulness of MRI in the prenatal diagnosis of patients with such anomalies. A total of 23 cases were included in this study. The 23 cases were divided as follows: 7 cases of bilateral renal agenesis or severe hypogenesis (Potter sequence: PS), 8 cases of multicystic dysplastic kidney (2 cases were bilateral: MCDK), 5 cases of hydronephrosis (HN), one case of hydroureteronephrosis (HUN) and 2 cases of HN or HUN with duplication (DUP). In this study the scan time for fetal MRI was approximately 30 seconds for one sequence. Overall, the diagnostic accuracy was 65.2%; 85.2% for PS, 87.5% for MCDK, 60.0% for HN, 50.0% for HUN and 0% for DUP. Even though imaging quality was relatively poor for motion artifact in this series, we were able to diagnose PS and MCDK because of associated lung hypoplasia and its characteristic shape. The diagnosis of HN, HUN and DUP was difficult. HN was sometimes misdiagnosed as a retroperitoneal cystic mass because the dilation of calices was obscured in severe cases. In HUN and DUP cases dilation of the ureter was unclear. However, using HASTE or true FISP sequence may solve this problem. Based on this data, we conclude that fetal MRI is useful for prenatal diagnosis of urological anomalies. (author)

  11. Bilateral Congenital Absence of the Stapes Superstructure in Two Siblings

    Directory of Open Access Journals (Sweden)

    Jose Ignacio Undabeitia

    2014-01-01

    Full Text Available Middle ear ossicle malformations are an uncommon event. Among them, the congenital absence of the stapes is a very rare condition that is seldom described in the literature. We report the cases of two women, aged 19 and 22 , who presented with a long history of conductive deafness. An exploratory tympanotomy was performed and the absence of the stapes superstructure and an abnormal position of the facial nerve could be observed. A bone anchored hearing aid (BAHA was implanted in both patients with good results. It is believed that stapes agenesis is related to an abnormal development of the facial nerve, which by the 5th to 6th week of gestation would interpose between the otic capsule and the stapes blastema, preventing these structures from contacting. A long history of nonprogressive hearing loss from birth or early childhood is the key to reach a diagnosis. Several treatment options have been described. The authors opted for a hearing aid due to the high risk of facial nerve lesion, with good functional results.

  12. Nitrofurantoin and congenital abnormalities

    DEFF Research Database (Denmark)

    Czeizel, A.E.; Rockenbauer, M.; Sørensen, Henrik Toft

    2001-01-01

    or fetuses with Down’s syndrome (patient controls), 23 (2.8%) pregnant women were treated with nitrofurantoin. The above differences between population controls and cases may be connected with recall bias, because the case-control pair analysis did not indicate a teratogenic potential of nitrofurantoin use......Objective: To study human teratogenic potential of oral nitrofurantoin treatment during pregnancy. Materials and Methods: Pair analysis of cases with congenital abnormalities and matched population controls in the population-based dataset of the Hungarian Case-Control Surveillance of Congenital...... during the second and the third months of gestation, i.e. in the critical period for major congenital abnormalities. Conclusion: Treatment with nitrofurantoin during pregnancy does not present detectable teratogenic risk to the fetus....

  13. Congenital syphilis: literature review

    Directory of Open Access Journals (Sweden)

    Eduardo Chaida Sonda

    2013-01-01

    Full Text Available Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher than the ones spent with a baby without this infection. The treatment is generally carried out with penicillin and must be extended to sexual partners. Inadequate or lack of treatment of congenital syphilis can result in miscarriage, premature birth, acute complications and other fetal sequelae. KEYWORDS: Congenital syphilis. Treponema pallidum. Vertical transmission.

  14. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  15. A Case of a Newborn with Agenesis of the Corpus Callosum Complicated with Ocular Albinism

    Directory of Open Access Journals (Sweden)

    Michiko Miki

    2016-05-01

    Full Text Available Purpose: To report a case of ocular albinism found in a newborn infant in whom agenesis of the corpus callosum (ACC was indicated in utero. Case Report: This study involved a female newborn who was delivered after a gestational period of 41 weeks. The patient was referred to the Obstetrics Department at Takatsuki Hospital, Takatsuki City, Japan, after the indication of ACC by magnetic resonance imaging (MRI at a nearby clinic during the fetal period. At birth, the baby’s weight was 2,590 g, and ACC and ventricular enlargement were found by cranial sonography and cranial MRI. While initial ophthalmic findings noted partial loss of pigmentation of the iris and hypopigmentation of broad areas of the fundus in both eyes, nystagmus was not observed. The patient’s hair pigment was slightly diluted, and the color of her skin was slightly off-white. At 2 years after birth, obvious mental retardation was observed. With regard to other systemic findings, no apparent heart, kidney, or immune system abnormalities were found. Conclusion: Although the patient in question is presently growing without any major systemic problems, it will be necessary in the future to pay attention to any changes in systemic and ophthalmic findings.

  16. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

    Directory of Open Access Journals (Sweden)

    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  17. Anaesthetic management of emergency pacemaker implantation in a case of neonatal lupus erythematosus with complete congenital heart block & severe respiratory distress

    Directory of Open Access Journals (Sweden)

    Usha Kiran

    2007-01-01

    Full Text Available An 8-week old 3-kilogram male baby was brought to this tertiary care hospital with respiratory distress, marked tracheal tug, poor feeding and a heart rate of 46/minute. The child had been referred from a peripheral hospital as a case of neonatal lupus with complete congenital heart block. The mother was seropositive for systemic lupus erythematosus with a history of two abortions. Evaluation on admission revealed a heart rate between 40-60/ minute, respiratory rate 40-50/ minute, inspiratory stridor, bilateral crepitations, chest retrac-tion and a marked tracheal tug that improved with prone positioning. Electrocardiography and echocardiography confirmed complete congenital heart block with cardiomegaly and mild left ventricular dysfunction. Keeping in view the impending congestive heart failure, possible early cardiomyopathy and the bad obstetric history ur-gent pacemaker implantation was planned to allow early recovery of the child. The anaesthetic risk was high due to the heart block, ventricular dysfunction, laryngomalacia, severe tracheal tug and anticipated difficult weaning from controlled ventilation. General anaesthesia was administered with endotracheal tube and con-trolled ventilation using ketamine, rocuronium and sufentanil. For patient safety invasive monitoring was pro-vided and external pacing was kept standby. Epicardial pacemaker leads were implanted onto the left ventricu-lar wall through a left anterior 6th intercostal space thoracotomy. The child was electively ventilated for two post operative days. The tracheal tug and secretions gradually subsided over 2 weeks with oxygen, antibiotics, steroids, bronchodilators and physiotherapy. At the time of discharge from hospital 2 weeks after the implant the child was feeding well, tracheal tug was minimal and the lungs were clear.

  18. Congenital maxillary double lip

    Directory of Open Access Journals (Sweden)

    Dinesh Singh Chauhan

    2012-01-01

    Full Text Available Double lip, also referred to as "macrocheilia," is a rare anomaly which affects the upper lip more commonly than the lower lip. It consists of a fold of excess or redundant hypertrophic tissue on the mucosal side of the lip. The congenital double lip is believed to be present at birth and becomes more prominent after eruption of teeth. It affects esthetics and also interferes with speech and mastication. Simple surgical excision produces good functional and cosmetic results. We report a case of a non-syndromic congenital maxillary double lip in a 21-year-old male patient.

  19. Congenital hearing impairment

    Energy Technology Data Exchange (ETDEWEB)

    Robson, Caroline D. [Children' s Hospital and Harvard Medical School, Division of Neuroradiology, Department of Radiology, Boston, MA (United States)

    2006-04-15

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  20. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Reiser, M.F.

    2001-01-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [de

  1. Congenital hearing impairment

    International Nuclear Information System (INIS)

    Robson, Caroline D.

    2006-01-01

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  2. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  3. Congenital Heart Defects and CCHD

    Science.gov (United States)

    ... and more. Stony Point, NY 10980 Close X Home > Complications & Loss > Birth defects & other health conditions > Congenital heart defects and ... in congenital heart defects. You have a family history of congenital heart ... syndrome or VCF. After birth Your baby may be tested for CCHD as ...

  4. [Congenital anomalies of cerebral artery and intracranial aneurysm].

    Science.gov (United States)

    Nakajima, K; Ito, Z; Hen, R; Uemura, K; Matsuoka, S

    1976-02-01

    It is well known that congenital anomalies such as polycystic kidney, aortic coarctation, Marfan syndrome, Ehler-Danlos syndrome are apt to be complicated by intracranial aneurysms. In this report we attempt to reveal the relation and incidence between cerebrovascular anomalies and intracranial aneurysms. The etiology of aneurysms has been discussed, too. 12 cases of persistent trigeminl artery, 2 cases of persistent hypoglossal artery and 11 cases of fenestration were obtained from 3841 patients who were angiographically examined in our clinic for 5 years. The incidence is 0.31%, 0.05% and 0.29%, respectively. Persistent trigeminal arteries were complicated by 2 cases of intracranial aneurysms and one case of arterivenous malformations (AVM), persistent hypoglossal arteries were complicated by one case of aneurysm, and fenestrations were complicated by 2 cases of aneurysms and one case of AVM. One case of congenital agenesis of right internal carotid artery was obtained which was complicated by aneurysm of anterior communicating artery. Totally, 8 cases of aneurysms and AVM were obtained from 26 cases of cerebrovascular anomalies (incidence 30.8%). On the other hand, thalamic or caudate hemorrhage revealed the highest incidence of complication of intracranial aneurysms among intracerebral hematomas (10.7%). Compared with the incidence of aneurysms between cerebro vascular anomalies (30.8%) and thalamic or caudate hemorrhage (10.7%), the difference is statistically signigicant (P less than 0.05). The cause of intracranial aneurysm has not yet been clarified. But it is well accepted that the defect of tunica media vasorum is most responsible factor as to the occurrence of intracranial aneurysms. We concluded that the genetic error of cerebral vessels including defect of media caused intracranial aneurysms, and this result was supported from the evidence that cerebrovascular anomalies showed statistically high incidence of complication of intracranial aneurysms.

  5. Diagnosis of tracheal instability: inspiratory and expiratory spiral CT and cine CT

    International Nuclear Information System (INIS)

    Heussel, C.P.; Thelen, M.; Kauczor, H.U.; Hafner, B.; Lill, J.

    2000-01-01

    Purpose: In tracheo- and bronchomalacia, localization and determination of collapse is necessary for planning a surgical procedure. We compared inspiratory and spiral CT, cine CT, and bronchoscopy and evaluated the relevance of each method. Methods: Seventeen patients with suspected or verified tracheal stonosis or collapse underwent paired inspiratory and exspiratory spiral CT and cine CT during continuous respiration (temporal increment 100 ms). The tracheal cross-sectional area was calculated and compared. Results: In addition to bronchoscopy, further information concerning localization, extent, collapse, stability of the tracheal wall, distal portions of the stenosis, and extraluminal compressions was obtained. A significantly higher degree of tracheal collapse was seen using cine CT compared to paired spiral CT (p [de

  6. In vitro pharmacokinetics of sirolimus-coated stent for tracheal stenosis

    African Journals Online (AJOL)

    Further increases in sirolimus: PLGA ratio did not improve stent drug loading. A slow release of ... tracheal stent can damage the airway mucosa, and cause .... Figure 3: Cumulative drug release from sirolimus-coated stents. DISCUSSION.

  7. Long-term outcome of conventional endotracheal tube balloon dilation of tracheal stenosis in a dog

    OpenAIRE

    Kahane, Nili; Segev, Gilad

    2014-01-01

    This report describes a successful dilation of tracheal stenosis in a 16-year-old dog using a conventional endotracheal tube balloon. This technique should be considered as palliative treatment when owners decline other therapeutic options.

  8. Long-term outcome of conventional endotracheal tube balloon dilation of tracheal stenosis in a dog.

    Science.gov (United States)

    Kahane, Nili; Segev, Gilad

    2014-01-01

    This report describes a successful dilation of tracheal stenosis in a 16-year-old dog using a conventional endotracheal tube balloon. This technique should be considered as palliative treatment when owners decline other therapeutic options.

  9. Tracheal obstruction caused by an expandable metallic stent: a case of successful removal of the stent.

    Science.gov (United States)

    Okuyama, Hiroomi; Kubota, Akio; Kawahara, Hisayoshi; Oue, Takaharu; Nose, Satoko; Ihara, Toshiyuki

    2005-07-01

    We report a case of tracheal obstruction caused by an expandable metallic stent. A 3-month-old girl with severe tracheomalacia had a placement of a Palmaz stent. At 3 years of age, she developed progressive dyspnea. The CT scan showed tracheal obstruction caused by granulation tissue over the stent. At operation, the stent was found to have penetrated the posterior tracheal wall. Under partial cardiopulmonary bypass, the stent was removed along with the membranous wall of the trachea, and the trachea was reconstructed using slide tracheoplasty. Tracheal obstruction is one of the serious complications caused by an expandable metallic stent. Direct open approach to the trachea under cardiopulmonary bypass is thought to be a safe way to manage this problem.

  10. Penatalaksanaan Kasus Maloklusi dengan Agenesis Insisif Lateral Atas dan Premolar Dua Bawah pada Periode Gigi Bercampur

    Directory of Open Access Journals (Sweden)

    Ratri Anandita

    2012-10-01

    Full Text Available In orthodontic practice, orthodontist frequently face the patients with congenitally missing maxillary lateral incisors and mandibular second premolar. There are some treatment options for patients with congenitally missing second premolar such as keeping the deciduous second molar, extracting the molars and allowing space to close spontaneously, prosthetic replacement, autotransplantation and orthodontic space closure. Instead of treatment option for congenitally maxillary lateral incisor are orthodontic space opening for future restoration or orthodontic space closure using canines to replace missing maxillary lateral incisors. There are some factors must be consider before making a decision, such as the facial profile, dentoalveolar protrusion, the tooth size-arch length discrepancy, inclination and position of canine, dental esthetic of canines, occlusion, the dental relationship, patient’s age, the stage of development of the adjacent teeth and the condition of the deciduous predecessors with regard to root resorption and infracclusion. In this paper it will be discussed about the indications, advantages and disadvantages of both treatment modalities to help clinicians to cope with the malocclusion cases with congenital missing teeth. In this regard it needed teamwork among the orthodontist, prosthodontist, dental surgeon and restorative dentist to analyzing some factors related to individual patients and establishing overall treatment plans.DOI: 10.14693/jdi.v16i1.18

  11. Management of benign dynamic "A-shape" tracheal stenosis: a retrospective study of 60 patients.

    Science.gov (United States)

    Plojoux, Jérôme; Laroumagne, Sophie; Vandemoortele, Thomas; Astoul, Philippe J; Thomas, Pascal A; Dutau, Hervé

    2015-02-01

    Benign tracheal stenosis complicates tracheal intubation or tracheostomy in 0.6% to 65% of cases. Surgical resection is the standard treatment. Endoscopic management is used for inoperable patients with 17% to 69% success. Dynamic "A-shape" tracheal stenosis (DATS) results in a dynamic stenosis with anterior fracture of tracheal cartilage and frequently associated posterior malacia. We report the results of our multidisciplinary management. Sixty patients with DATS were included. Management decision was made during initial bronchoscopy. When suitable, patients were referred to thoracic surgery for tracheal resection. Posterior localized tracheomalacia was treated with laser photocoagulation of the posterior tracheal wall. Tracheal stents were placed if the stenosis persisted after laser treatment. The choice of stent (straight silicone, hour-glass shaped silicone, T-tube, or fully-covered self-expandable metallic stent) was based on operator's judgment. After 12 to 18 months, stents were removed. If the stenosis persisted after stent removal, surgery was reconsidered. If surgery was not possible, a stent was replaced. In case of satisfactory result, a stent was replaced only after recurrence. Stable patients after treatment were considered as success, requirement of long-term tracheostomy or T tube as failure, and long-term stent as partial success. All patients developed DATS after tracheostomy. Thirty-three patients had posterior tracheomalacia. In 13 patients, mild stenosis required only endoscopic surveillance. Two patients were referred to thoracic surgery for tracheal resection surgery. Endoscopic management was the initial therapy in 45 patients (75%) and was considered successful in 23 patients (51%), partially successful in 10 (22%), and failed in 12 (27%). Five patients with successful outcomes required only laser therapy. Overall 70 stents were placed in 35 patients, with a migration rate of 31%. The DATS management was successful in 63%. Stent migration

  12. Heart Sound Localization and Reduction in Tracheal Sounds by Gabor Time-Frequency Masking

    OpenAIRE

    SAATCI, Esra; Akan, Aydın

    2018-01-01

    Background and aim: Respiratorysounds, i.e. tracheal and lung sounds, have been of great interest due to theirdiagnostic values as well as the potential of their use in the estimation ofthe respiratory dynamics (mainly airflow). Thus the aim of the study is topresent a new method to filter the heart sound interference from the trachealsounds. Materials and methods: Trachealsounds and airflow signals were collected by using an accelerometer from 10 healthysubjects. Tracheal sounds were then pr...

  13. A comparison of skeletal maturation in patients with tooth agenesis and unaffected controls assessed by the cervical vertebral maturation (CVM) index.

    Science.gov (United States)

    Casey, Christine; Gill, Daljit S; Jones, Steven P

    2013-12-01

    The aims of this study were to (1) investigate if there is a difference in skeletal maturation between tooth agenesis and control patients and (2) whether skeletal maturation is affected by the severity of tooth agenesis. The cervical vertebral maturation (CVM) index can be used to assess skeletal maturation. A retrospective cross-sectional study. Eastman Dental Hospital, London, UK. A total of 360 cephalograms of patients aged 9-17 years (164 males and 196 females) allocated to four subgroups (mild, moderate and severe tooth agenesis patients, and controls) were assessed retrospectively. There were 90 patients in each of the four subgroups. The skeletal maturation of each subject was assessed both quantitatively and qualitatively using the CVM index. All patients in the study were either currently receiving treatment or had been discharged from the hospital. There was no statistically significant relationship between skeletal maturation and the presence of tooth agenesis. Furthermore, there was no statistically significant relationship between the skeletal maturity of patients and different severities of tooth agenesis. The data obtained from this group of patients and using this measurement tool alone does not supply sufficient reason to reject the null hypothesis. However, it suggests that it is possible that no difference exists between the groups.

  14. Idiopathic tracheal stenosis: a clinicopathologic study of 63 cases and comparison of the pathology with chondromalacia.

    Science.gov (United States)

    Mark, Eugene J; Meng, Fanqing; Kradin, Richard L; Mathisen, Douglas J; Matsubara, Osamu

    2008-08-01

    Tracheal stenosis in adults usually is the result of mechanical injuries either from direct trauma or intubation. Rarely do cases develop in patients without such a precedent history, and there are few reports of the pathology of idiopathic tracheal stenosis (ITS). We reviewed clinicopathologically, 63 tracheal resections for tracheal stenosis in patients who had no antecedent explanation for their stenosis. We contrasted these 63 cases with 34 cases of tracheal stenosis owing to chondromalacia (CM) after mechanical injury. All 63 cases occurred in females, with a mean age of 49 years. The most common symptom was dyspnea on exertion. The average duration of symptoms was greater than 2 years. One-third of the patients gave a history of gastroesophageal reflux. All but one of the cases occurred in the subglottic region and/or upper one-third of the trachea. Pathologically, most cases showed extensive keloidal fibrosis and dilation of mucus glands, a finding that was not obvious in most cases of CM. ITS has relatively normal cartilage with smooth inner and outer perichondrium, whereas CM has extensive degeneration of cartilage with irregular border of inner perichondrium observable at shirt sleeve magnification. Immunohistochemical staining for estrogen receptor and progesterone receptor was positive in fibroblasts cells in most cases. ITS is a rare disease and restricted to females. It may represent some form of fibromatosis. ITS can be distinguished histologically from CM in tracheal resection specimens in most cases.

  15. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  16. Giant Congenital Melanocytic Nevus

    DEFF Research Database (Denmark)

    Rasmussen, Bo Sonnich; Henriksen, Trine Foged; Kølle, Stig-Frederik Trojahn

    2015-01-01

    Giant congenital melanocytic nevi (GCMN) occur in 1:20,000 livebirths and are associated with increased risk of malignant transformation. The treatment of GCMN from 1981 to 2010 in a tertiary referral center was reviewed evaluating the modalities used, cosmetic results, associated complications...

  17. Congenital neck masses.

    Science.gov (United States)

    Rosa, Peter A; Hirsch, David L; Dierks, Eric J

    2008-08-01

    Congenital neck lesions reflect abnormal embryogenesis in head and neck development. A thorough knowledge of embryology and anatomy is critical in the diagnosis and treatment of these lesions. The appropriate diagnosis of these lesions is necessary to provide appropriate treatment and long-term follow up, because some of these lesions may undergo malignant transformation or be harbingers of malignant disease.

  18. Congenital cystic eyeball

    Directory of Open Access Journals (Sweden)

    Gupta V

    1990-01-01

    Full Text Available A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye′s predilection for the left eye has been pointed out for the first time in this article.

  19. Congenital extracranial meningioma

    International Nuclear Information System (INIS)

    Wong, H.F.; Ng, S.H.; Wai, Y.Y.; Wan, Y.L.; Kong, M.S.

    1995-01-01

    The authors report a case of congenital meningioma in a newborn. This tumour is extremely rare and only six cases have been reported in the literature. Those reported cases were mainly intracranial. This is the first case of a neonatal extracranial meningioma that was evaluated preoperatively by computed tomography and magnetic resonance imaging. (orig.)

  20. Congenital cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Chaudhry, A.K.; Azam, M.; Maqsood, R.; Naz, B.; Salam, A.

    2003-01-01

    This case report presents the clinical picture, diagnostic methodology and surgical treatment of a female child who presented with chronic cough and dyspnoea due to congenital malformation of lung. A discussion of diagnosis and management is presented at the end. (author)

  1. Pseudoamblyopia in Congenital Cyclotropia

    Directory of Open Access Journals (Sweden)

    Antonio Frattolillo

    2017-01-01

    Full Text Available Purpose. To study the effect of surgery on amblyopia and suppression associated with congenital cyclovertical strabismus. Methods. The fixation pattern was investigated with microperimetry before and soon after surgery in ten consecutive children operated for congenital superior oblique palsy at the S. Martino Hospital, Belluno, Italy, between September 2014 and December 2015. Changes in visual performance in terms of best-corrected visual acuity (BCVA and stereopsis between the day before and one week after surgery were also evaluated. No other amblyopia treatment has been administered during the time study. Results. Surgical correction of the excyclodeviation in congenital SO palsy determined monocular and binocular sensory consequences: monocularly, in the cyclodeviated amblyopic eye, BCVA (0.46–0.03 LogMAR; p<0.0001 and the fixation pattern improved, as demonstrated by microperimetry examination. Binocularly, stereopsis improved or emerged while suppression at the Worth four-dot test disappeared. Conclusions. In the absence of further amblyopic factors such as coexisting constant vertical and/or horizontal deviation and anisometropia, the amblyopia encountered in congenital SO palsy may resolve soon after the surgical alignment. Therefore, it may be considered and defined “pseudoamblyopia.”

  2. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    . The study evaluated the assessment procedure of 190 children and adults found to be congenitally deafblind. Among the 190 individuals 76 percent were determined using functional assessment in addition to medical examination. A case example involving a 12-year-old child is also presented to illustrate...

  3. Providers' Experiences with Vaginal Dilator Training for Patients with Vaginal Agenesis.

    Science.gov (United States)

    Patel, Vrunda; Hakim, Julie; Gomez-Lobo, Veronica; Amies Oelschlager, Anne-Marie

    2018-02-01

    To examine providers' experiences with vaginal dilator training for patients with vaginal agenesis. Anonymous electronic survey. Members of the North American Society for Pediatric and Adolescent Gynecology. How providers learn about vaginal dilator training, common techniques, and methods used for patient training, assessment of patient readiness, common patient complaints, issues leading to early discontinuation. There were a total of 55 completed survey responses of which 31 respondents (56%) had been in practice for more than 10 years. Forty-nine were gynecologists (89%), 20 had completed a fellowship in pediatric and adolescent gynecology (36%), and 6 were reproductive endocrinologists (11%). Thirty-one respondents had first learned about vaginal dilator training through lectures (56%) whereas only 9 through mentorship and fellowship (16%). According to respondents, the most common issue leading to early discontinuation was lack of patient motivation and readiness (n = 42; 76%). The most common complication was pain or discomfort (n = 45; 82%). More than half of respondents determined dilator therapy was successful when patients reported comfortable sexual intercourse (n = 30; 55%) and 65% (n = 35) did not delineate any restrictions to initiation of sexual intercourse. Most respondents (87%) requested further vaginal dilator training at either a clinical meeting (n = 26; 47%) or with a training video (n = 22; 40%). Our study in an experienced cohort of pediatric gynecology providers highlights the need for further research and training on vaginal dilation education. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  4. Neurodevelopmental outcome in prenatally diagnosed isolated agenesis of the corpus callosum.

    Science.gov (United States)

    Folliot-Le Doussal, Lise; Chadie, Alexandra; Brasseur-Daudruy, Marie; Verspyck, Eric; Saugier-Veber, Pascale; Marret, Stéphane

    2018-01-01

    Neurodevelopmental outcome in children with agenesis of the corpus callosum (ACC) is correlated with the presence or absence of associated brain abnormalities. Indeed, neurodevelopmental outcome shows severe disabilities when the ACC is not isolated whereas in isolated forms, the neurologic development is mainly normal. Contrary to data in several published studies, the prognosis remains uncertain even in isolated forms, which may lead in France to medical termination of pregnancy. To evaluate long-term neurodevelopmental outcome in children with prenatally diagnosed isolated ACC. This is a follow-up study conducted in Normandy (France). It included a cohort of 25 children born between January 1991 and June 2016, with a prenatal diagnosis of isolated ACC and who were followed for at least two years. The average follow-up was 8±5years. ACC was complete in 17 patients (68%), partial in 5 (20%) and hypoplastic in 3 (12%). Whereas global motor development was normal in each case, normal neurodevelopmental outcome or mild disabilities occurred in 88% children and moderate/severe neuro-disabilities were present in 12% of children. Wechsler Intelligence Scale for Children-IV evaluations and Intellectual Total Quotients were within normal range, but we observed lower scores in verbal comprehension, social judgment, executive functions. A lower score in morphosyntax was observed among 52% of children with oral language disorders. Neurodevelopmental outcome was favorable in most of our patients with isolated ACC, but mild learning disabilities emerged in older children. Long-term follow-up until school age is essential to provide early diagnosis and appropriate care support. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Recurrent airway obstructions in a patient with benign tracheal stenosis and a silicone airway stent: a case report

    OpenAIRE

    Sriram, KB; Robinson, PC

    2008-01-01

    Airway stents (silicone and metal stents) are used to treat patients with benign tracheal stenosis, who are symptomatic and in whom tracheal surgical reconstruction has failed or is not appropriate. However airway stents are often associated with complications such as migration, granuloma formation and mucous hypersecretion, which cause significant morbidity, especially in patients with benign tracheal stenosis and relatively normal life expectancy. We report a patient who had frequent critic...

  6. Traumatic tracheal diverticulum corrected with resection and anastomosis during one-lung ventilation and total intravenous anesthesia in a cat.

    Science.gov (United States)

    Sayre, Rebecca S; Lepiz, Mauricio; Wall, Corey; Thieman-Mankin, Kelley; Dobbin, Jennifer

    2016-11-01

    This report describes the clinical findings and diagnostic images of a traumatic intrathoracic tracheal avulsion with a tracheal diverticulum in a cat. Furthermore, a complete description of the tracheal resection and anastomosis using one-lung ventilation (OLV) with total and partial intravenous anesthesia is made. A 3-year-old neutered male domestic shorthair cat weighing 6.8 kg was presented to the University Teaching Hospital for evaluation of increased respiratory noise 3 months following unknown trauma. Approximately 12 weeks prior to presentation, the cat had been seen by the primary care veterinarian for respiratory distress. At that time, the cat had undergone a tracheal ballooning procedure for a distal tracheal stricture diagnosed by tracheoscopy. The tracheal ballooning had provided only temporary relief. At presentation to our institution, the cat had increased respiratory effort with harsh upper airway noise auscultated during thoracic examination. The remainder of the physical examination was normal. Diagnostics included a tracheoscopy and a thoracic computed tomographic examination. The cat was diagnosed with tracheal avulsion, pseudotrachea with a tracheal diverticulum, and stenosis of the avulsed tracheal ends. Surgical correction of the tracheal stricture via a thoracotomy was performed using OLV with total and partial intravenous anesthesia. The cat recovered uneventfully and at last follow-up was active and doing well. This case report describes OLV using standard anesthesia equipment that is available at most private practices. Furthermore, this case describes the computed tomographic images of the intrathoracic tracheal avulsion and offers a positive outcome for tracheal resection and anastomosis. © Veterinary Emergency and Critical Care Society 2015.

  7. Tracheal intubation in the ICU: Life saving or life threatening?

    Directory of Open Access Journals (Sweden)

    Jigeeshu V Divatia

    2011-01-01

    Full Text Available Tracheal intubation (TI is a routine procedure in the intensive care unit (ICU, and is often life saving. However, life-threatening complications occur in a significant proportion of procedures, making TI perhaps one the most common but underappreciated airway emergencies in the ICU. In contrast to the controlled conditions in the operating room (OR, the unstable physiologic state of critically ill patients along with underevaluation of the airways and suboptimal response to pre-oxygenation are the major factors for the high incidence of life-threatening complications like severe hypoxaemia and cardiovascular collapse in the ICU. Studies have shown that strategies planned for TI in the OR can be adapted and extrapolated for use in the ICU. Non-invasive positive-pressure ventilation for pre-oxygenation provides adequate oxygen stores during TI for patients with precarious respiratory pathology. The intubation procedure should include not only airway management but also haemodynamic, gas exchange and neurologic care, which are often crucial in critically ill patients. Hence, there is a necessity for the implementation of an Intubation Bundle during routine airway management in the ICU. Adherence to a plan for difficult airway management incorporating the use of intubation aids and airway rescue devices and strategies is useful.

  8. Large-segment tracheal resection and interannular anastomosis with a tension-release technique in the dog.

    Science.gov (United States)

    Dallman, M J; Bojrab, M J

    1982-02-01

    Tracheal resection and anastomosis were done on 7 dogs, and a modified procedure to provide optimum first-intention healing, minimum connective tissue proliferation, and optimum epithelialization was used. To test the integrity of the technique at high incision-line tensile forces, 15 to 17 tracheal rings were excised. Excessive tracheal manipulation resulted in an increased amount of lamina propria and submucosa collagenous tissue, increased size and number of blood vessels, and increased leukocytic invasion. Criteria for a successful anastomosis were minimum postoperative coughing, good mucosal apposition and epithelialization, and tracheal lumen diameters that were at least 80% of the preoperative measurement.

  9. Successful radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with dextrocardia due to unilateral pulmonary agenesis: a case report

    Directory of Open Access Journals (Sweden)

    Aksu T

    2015-02-01

    Full Text Available Tolga Aksu, Tumer Erdem Guler, Ebru Golcuk, Ismail Erden, Kazim Serhan Ozcan Department of Cardiology, Kocaeli Derince Education and Research Hospital, Derince, Kocaeli, Turkey Abstract: Radiofrequency catheter ablation of the slow pathway is considered to be the treatment of choice for patients with atrioventricular nodal reentrant tachycardia. We report a 34-year-old female with mirror image dextrocardia due to unilateral pulmonary agenesis who underwent successful slow pathway ablation for typical atrioventricular nodal reentrant tachycardia. Using contrast injection, cardiac anatomy was identified in a short time and successfully ablated. Keywords: dextrocardia, AVNRT, ablation, pulmonary agenesis

  10. Large Sphenoethmoidal Encephalocele Associated with Agenesis of Corpus Callosum and Cleft Palate

    Directory of Open Access Journals (Sweden)

    Basir Hashemi

    2010-06-01

    Full Text Available AbstractBasal encephalocele is a rare craniofacial anomaly. In the presentpaper we report a 10-year-old boy presented with cleftpalate, congenital nystagmus, and hypertelorism. During preoperativeevaluation for cleft palate repair, a pulsatile masswas detected in the pharynx. Magnetic resonance imagingshowed sphenoethmoidal type of basal encephalocele andagenesis of corpus callosum. Neurosurgical consultation wasperformed for further evaluation and management.Iran J Med Sci 2010; 35(2: 154-156.

  11. Unilateral Pulmonary Artery Agenesis with Ipsilateral Pulmonary Hypoplasia as Incidental Finding in an Asthmatic Patient

    Directory of Open Access Journals (Sweden)

    Catalina Contreras-Arias

    2014-01-01

    Full Text Available Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unsual nding in some of these patients. This paper reports the case of a 4-year-old male with recurrent respiratory infections and asthma symptoms, in who further studies found agenesia of right pulmonary artery with pulmonary hypoplasia of the same side.

  12. Imperfuração anal associada à agenesia parcial do sacro e lipoma pré-sacral: síndrome de Currarino Imperforate anus associated with partial sacral agenesis and presacral lipoma: Currarino syndrome

    Directory of Open Access Journals (Sweden)

    Paulo Ricardo G. Zen

    2010-09-01

    Full Text Available OBJETIVO: Relatar o caso de uma criança com síndrome de Currarino diagnosticada após avaliação por episódios recorrentes de infecção urinária. DESCRIÇÃO DE CASO: Menina branca de dois anos, única filha de pais hígidos e sem história familiar de defeitos congênitos. A criança nasceu com imperfuração anal e com fístula retovestibular diagnosticadas no primeiro dia de vida. Por volta dos sete meses, começou a apresentar episódios recorrentes de infecção urinária, estabelecendo-se o diagnóstico de bexiga neurogênica. Na mesma ocasião, foi constatada a presença de agenesia parcial do sacro. A avaliação pela tomografia computadorizada e ressonância nuclear magnética de coluna identificou presença de fístula coincidente com a fosseta da transição lombo-sacral, observada ao exame físico; amputação da porção inferior da medula, com diminuição do número de raízes nervosas da cauda equina e massa pré-sacral de aspecto lipomatoso. Esta foi confirmada durante a cirurgia de correção do ânus imperfurado. A criança não apresentava outras dismorfias e a avaliação radiológica dos pais não identificou anormalidades sacrais. COMENTÁRIOS: A síndrome de Currarino é uma doença genética autossômica, dominante e rara caracterizada pela tríade formada por atresia anal, agenesia parcial do sacro e tumoração pré-sacral. Inclui teratomas, meningoceles, cistos entéricos e lipomas, como observado em nossa paciente. Crianças apresentando anormalidades anorretais deveriam ser sempre cuidadosamente avaliadas quanto à presença da síndrome de Currarino. A agenesia parcial do sacro é um forte indicativo da doença.OBJECTIVE: To report a patient with Currarino syndrome diagnosed after evaluation for recurrent urinary infections. CASE DESCRIPTION: This is a Caucasian two-year-old girl, the only daughter of healthy unrelated parents with no family history of congenital defects. The patient was born with imperforate

  13. Tracheal reconstruction by re-inforced Gore-Tex in esophageal submuscular tunneling: An experimental study

    Directory of Open Access Journals (Sweden)

    Hodjati Hossein

    2011-01-01

    Full Text Available Background: Tracheal reconstructions are aimed at rearranging or replacing parts of the tracheal tissue by different techniques. Here we introduce a new technique for tracheal reconstruction. Methods: In 10 adult dogs, after intubation with an endotracheal tube, a segment of trachea including seven tracheal rings was resected circumferentially. A submuscular tunnel was induced between mucosal and muscular layers of the adjacent esophagus lying right next to the trachea. The esophageal submuscular tunnel starts and ends exactly at the level of distal and proximal ends of tracheal resection, respectively. Inforced Gore-Tex passed through the esophageal submuscular tunnel the distal segment of trachea and end-to-end anastomosis were made between distal ends of Gore-Tex and trachea, then endotracheal tube removed and the same procedure was made for proximal ends of Gore-Tex and trachea. Afterward, the proximal and distal ends of the esophageal tunnel were approximated to proximal and distal tracheal parts over the anastomosis. Results: All dogs, except one due to anesthetic problem, survived and tolerated the operation; the first two dogs experienced postoperative fever, aspiration pneumonia, and died due to tracheoesophageal fistula. All survived animals were eating and barking well. We started to scarify dogs at least 6 and 12 weeks after operation for microscopy and pathologic examination. The Gore-Texes were patent and supported externally with fibrous connective tissue in esophageal tunneling, with in growth of respiratory epithelium on inner surfaces. Conclusion: Air tightness, good re-epithelialization, and relatively no limitation of esophageal length and no risk of luminal collapse are advantages of tracheal reconstruction by submuscular esophageal tunneling. This new method is worthy of further investigation, as it is technically feasible and easy to implement.

  14. Experimental study of different nickel-titanium memory tracheal stents in dogs

    International Nuclear Information System (INIS)

    Lang Xu; He Nengshu; Fan Hailun

    2009-01-01

    Objective: Since membrane -covered metal tracheal stent was applied successfully to treat the airway stenosis, it has been widely used and obtained satisfactory result during the past years. The purpose of our study was provide theoretical rational for treatment with membrane-covered tracheal stent by using animal experiment. Methods: The nickel- titanium memory stents of 22 mm in diameter and 6 cm in length were deployed in 18 dogs. The dogs were grouped into full- length membrane-covered group (n=6), partial-length membrane-covered group (membrane was covered for 4 cm in the medial part of the stent, n=6), and naked stent group (n=6). After the stent placement dysphagia or dysphonia was monitored daily. Twenty-four weeks later, the dogs were executed. Speiments were taken from the cephalic, medial, and caudal trachea under the stent. HE stain and proliferating cell nuclear antigen (PCNA) were evaluated. Results: After the stent placement, no dyspnea, dysphagia or dysphonia but cough was present in each dog. There was no significant difference in symptom between the three groups. Inflammation reaction, metaplasia from low columnar epithelium to spuamous epithelium, and mild granulation tissue hyperplasia in the underlying mucosa were present in each group. However, fibrosis and tracheal straitness were present only in the non-membrane-covered area, but not in the membrane-covered area. More PCNA expression was observed in the non-membrane -covered area than in the membrane-covered area. Conclusion: Both membrane-covered and non-membrane-covered stents can cause similar symptoms. However, the membrane -covered tracheal stent has good biological compatibility and would not induce tracheal straitness. The non-membrane -covered tracheal stent could induce a severe pathologic reaction and tracheal straitness. (authors)

  15. Ex vivo tracheomalacia model with 3D-printed external tracheal splint.

    Science.gov (United States)

    Kaye, Rachel; Goldstein, Todd; Aronowitz, Danielle; Grande, Daniel A; Zeltsman, David; Smith, Lee P

    2017-04-01

    To design and evaluate an ex vivo model of tracheomalacia with and without a three-dimensional (3D)-printed external tracheal splint. Prospective, ex vivo animal trial. Three groups of ex vivo porcine tracheas were used: 1) control (unmanipulated trachea), 2) tracheomalacia (tracheal rings partially incised and crushed), and 3) splinted tracheomalacia (external custom tracheal splint fitted onto group 2 trachea). Each end of an ex vivo trachea was sealed with a custom-designed and 3D-printed cap; a transducer was placed through one end to measure the pressure inside the trachea. Although the negative pressure was applied to the tracheal lumen, the tracheal wall collapse was measured externally and internally using a bronchoscope. Each group had at least three recorded trials. Tracheal diameter was evaluated using ImageJ software (National Institutes of Health, Bethesda, MD) and was averaged between two raters. Average tracheal occlusion percentage was compared using Student t test. The average occlusion was 31% for group 1, 87.4% for group 2, and 20% for group 3. Significant differences were found between the control and tracheomalacia groups (P tracheomalacia and splinted tracheomalacia groups (P tracheomalacia groups (P = 0.13). Applied pressure was plotted against occlusion and regression line slope differed between the tracheomalacia (0.91) and control (0.12) or splinted tracheomalacia (0.39) groups. We demonstrate the potential for an ex vivo tracheomalacia model to reproduce airway collapse and show that this collapse can be treated successfully with a 3D-printed external splint. These results are promising and justify further studies. N/A. Laryngoscope, 127:950-955, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  16. Congenital imprinting disorders

    DEFF Research Database (Denmark)

    Eggermann, Thomas; Netchine, Irène; Temple, I Karen

    2015-01-01

    Imprinting disorders (IDs) are a group of eight rare but probably underdiagnosed congenital diseases affecting growth, development and metabolism. They are caused by similar molecular changes affecting regulation, dosage or the genomic sequence of imprinted genes. Each ID is characterised...... by specific clinical features, and, as each appeared to be associated with specific imprinting defects, they have been widely regarded as separate entities. However, they share clinical characteristics and can show overlapping molecular alterations. Nevertheless, IDs are usually studied separately despite...... EUCID.net (European network of congenital imprinting disorders) now aims to promote better clinical care and scientific investigation of imprinting disorders by establishing a concerted multidisciplinary alliance of clinicians, researchers, patients and families. By encompassing all IDs and establishing...

  17. Congenital intestinal lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Popović Dušan Đ.

    2011-01-01

    Full Text Available Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  18. [Congenital intestinal lymphangiectasia].

    Science.gov (United States)

    Popović, Dugan D j; Spuran, Milan; Alempijević, Tamara; Krstić, Miodrag; Djuranović, Srdjan; Kovacević, Nada; Damnjanović, Svetozar; Micev, Marjan

    2011-03-01

    Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and supportive therapy. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  19. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  20. Congenital Hepatic Cyst

    Directory of Open Access Journals (Sweden)

    Aldo Recinos

    2017-04-01

    Full Text Available Congenital hepatic cyst is a rare and nonsymptomatic condition in infants and children. Its incidence is 2.5% in the postnatal life with a much lower incidence in the prenatal period. Incidental finding on antenatal imaging is the most common presentation. We present a case of a newborn in whom fetal ultrasound detected a cyst within the fetal liver. Postnatal imaging revealed a liver cyst in the right lobe of the liver, with no other intrahepatic structure affected. Liver function tests were abnormal, but the patient was asymptomatic. Posterior follow-up imaging showed a minor decrease in size. Management of congenital hepatic cyst is usually conservative, done with periodic ultrasound monitoring. However, surgical treatment is the mainstay of treatment when hydrops, progressive enlargement, hemorrhage, torsion, or compression of adjacent structures occurs. Malignant transformation can occur, but it is extremely rare. Partial or total removal of the cyst is the preferred treatment in neonates with a large lesion.

  1. Congenital diaphramatic hernia

    International Nuclear Information System (INIS)

    Kline-Fath, Beth M.

    2012-01-01

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  2. Characterizing Congenital Amusia

    OpenAIRE

    Stewart, Lauren

    2011-01-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of ...

  3. Management of complex benign post-tracheostomy tracheal stenosis with bronchoscopic insertion of silicon tracheal stents, in patients with failed or contraindicated surgical reconstruction of trachea.

    Science.gov (United States)

    Tsakiridis, Kosmas; Darwiche, Kaid; Visouli, Aikaterini N; Zarogoulidis, Paul; Machairiotis, Nikolaos; Christofis, Christos; Stylianaki, Aikaterini; Katsikogiannis, Nikolaos; Mpakas, Andreas; Courcoutsakis, Nicolaos; Zarogoulidis, Konstantinos

    2012-11-01

    Tracheal stenosis is a potentially life-threatening condition. Tracheostomy and endotracheal intubation remain the commonest causes of benign stenosis, despite improvements in design and management of tubes. Post-tracheostomy stenosis is more frequently encountered due to earlier performance of tracheostomy in the intensive care units, while the incidence of post-intubation stenosis has decreased with application of high-volume, low-pressure cuffs. In symptomatic benign tracheal stenosis the gold standard is surgical reconstruction (often after interventional bronchoscopy). Stenting is reserved for symptomatic tracheal narrowing deemed inoperable, due to local or general reasons: long strictures, inflammation, poor respiratory, cardiac or neurological status. When stenting is decided, silicone stent insertion is considered the treatment of choice in the presence of inflammation and/or when removal is desirable. We inserted tracheal silicone stents (Dumon) under general anaesthesia through rigid bronchoscopy in two patients with benign post-tracheostomy stenosis: a 39-year old woman with failed initial operation, and continuous relapses with proliferation after multiple bronchscopic interventions, and a 20-year old man in a poor neurological status, with a long tracheal stricture involving the subglottic larynx (lower posterior part), and inflamed tracheostomy site tissues (positive for methicillin resistant staphylococcus aureus). The airway was immediately re-establish, without complications. At 15- and 10-month follow-up (respectively) there was no stent migration, luminal patency was maintained without: adjacent structure erosion, secretion adherence inside the stents, granulation at the ends. Tracheostomy tissue inflammation was resolved (2(nd) patient), new infection was not noted. The patients maintain good respiratory function and will be evaluated for scheduled stent removal. Silicone stents are removable, resistant to microbial colonization and are

  4. The heart: Congenital disease

    International Nuclear Information System (INIS)

    Higgins, C.B.

    1987-01-01

    The most important diagnostic requirement in congenital heart disease (CHD) is definition of cardiovascular pathoanatomy. The considerable success in operative correction of even the most complex anomalies in recent years compels ever increasing precision in preoperative demonstration of these anomalies. Early experience with magnetic resonance imaging (MRI) at several institutions indicated that this modality is an effective noninvasive technique for evaluation of CHD. Indeed, MRI seems to have some advantage over other techniques, including angiography, for definitive diagnosis of congenital anomalies of the heart and great arteries and veins. The absence of ionizing radiation and contrast medium in MRI is an additional advantage; the former is particularly important for children, who, up to this time, have frequently been subjected to enormous radiation burdens from multiple cineangiograms during initial diagnosis and follow-up. This chapter describes the MRI appearance of cardiovascular anatomy im the segmental fashion proposed for analysis of complex CHD. Likewise, MRI demonstration of congenital cardiovascular lesions is organized into abnormalities situated at the four segmental cardiovascular levels: great vessels, atria, ventricles, and visceroatrial relationship. The role of MRI in evaluation of complex ventricular anomalies such as single ventricle and thoracic aortic abnormalities is specifically described

  5. Congenital aplasia of the semicircular canals.

    Science.gov (United States)

    Satar, Bulent; Mukherji, Suresh K; Telian, Steven A

    2003-05-01

    To describe the underrecognized inner ear malformation characterized by complete aplasia of the labyrinthine semicircular canals associated with a relatively well-formed cochlea, to investigate its relationship with known syndromic forms of hearing loss, and to hypothesize regarding the potential embryopathogenesis of this anomaly. A retrospective case review consisting of cases of sensorineural hearing loss with radiographic evidence demonstrating agenesis of the semicircular canals associated with a cochlea that was either morphologically normal or sufficiently well developed to accommodate the full insertion of a cochlear implant electrode. Cases were identified by computerized tomography findings that identified the anomaly under study. Departments of otolaryngology and radiology in a tertiary referral center, with a large cochlear implant program serving over 800 patients, more than half of whom are children. Fifteen patients with the anomaly under study were identified. Each patient underwent a complete otologic examination, audiometric studies, and high resolution computerized tomography of the temporal bone in axial and coronal planes. The bony morphology of the cochlea, round and oval windows, vestibule, semicircular canals, and vestibular aqueduct, and the course of the facial nerve were examined. Auditory findings and otologic treatment are presented. Of the 15 identified patients, 4 were nonsyndromic, 9 had CHARGE association (Coloboma of the eye, congenital Heart defects, choanal Atresia, mental and/or growth Retardation, Genital hypoplasia, and Ear anomalies and/or deafness), 1 met criteria for Noonan's syndrome, and one had features of both these syndromes. Although the cochlea was present in all cases, the cochlear morphology was usually abnormal in the CHARGE association patients. Of the 20 ears in the CHARGE subjects, only 3 ears (15%) were seen to have completely normal development of the cochlea in both the basal and upper turns. The others

  6. Radiology of congenital heart disease

    International Nuclear Information System (INIS)

    Amplatz, K.

    1986-01-01

    This is a text on the radiologic diagnosis of congenital heart disease and its clinical manifestations. The main thrust of the book is the logical approach which allows an understanding of the complex theory of congenital heart disease. The atlas gives a concise overview of the entire field of congenital heart disease. Emphasis is placed on the understanding of the pathophysiology and its clinical and radiological consequences. Surgical treatment is included since it provides a different viewpoint of the anatomy

  7. Anaesthetic Management for Cataract Surgery in VACTERL Syndrome Case Report

    Directory of Open Access Journals (Sweden)

    Sonal S Khatavkar

    2009-01-01

    Full Text Available Eight year old girl, weighing 14 kg with VACTERL syndrome V: Vertebral anomalies, A: Anal malformation, C: Cardiovascular defect, TE: Tracheal and esophageal malformation, R:Renal agenesis, L: Limb anomalies., underwent cataract surgery under general anaesthesia. She had multiple congenital anomalies like esophageal atresia, imperfo-rate anus (corrected, single kidney& radial aplasia. Anticipating problems of gastro-esophageal reflux& chronic renal failure, successful management was done.

  8. The relationship between smile attractiveness and esthetic parameters of patients with lateral agenesis treated with tooth recontouring or implants

    Science.gov (United States)

    De-Marchi, Luciana Manzotti; Pini, Núbia Inocencya Pavesi; Pascotto, Renata Corrêa

    2012-01-01

    Background The purpose of this study was to associate smile esthetic judgment with dentofacial attributes of patients with unilateral and bilateral agenesis of maxillary lateral incisors treated with recontouring of canines or implants and patients with no agenesis (control). Material and methods Forty-six participants were divided into two groups: those treated with recontouring (N = 26) and those treated with implants (N = 20). The participants in the control group (N = 22) were selected among dentistry students at the State University of Maringá, Brazil. Photographs of posed smiles (17 cm × 10 cm) were evaluated with a 100-mm Visual Analog Scale. Smile attractiveness was judged by two groups: laypersons and dentists (N = 20 in each group). Judgment was classified into Unpleasant and Pleasant. Measurements of 11 smile attributes were done with ImageTool Version 3.0. These measurements were correlated with the type of judgment using the Pearson correlation coefficient. Results The two groups of evaluators showed no rating difference (analysis of variance, P = 0.64), thus they were placed into a single group. No significant correlation was found between esthetic judgment and six smile attributes (incisor exposure, interlabial gap, width 3 to 3, smile index, right buccal corridor, and buccal corridor ratio). The control group showed more correlations with the unpleasant judgment type than the other groups. Conclusion Some correlations between smile attributes and esthetic judgment were found, but other features of smiles not evaluated in this study may interfere in smile attractiveness. PMID:23674925

  9. Association between pathogens from tracheal aspirate and oral biofilm of patients on mechanical ventilation

    Directory of Open Access Journals (Sweden)

    Luana Carneiro Diniz SOUZA

    2017-06-01

    Full Text Available Abstract The aim of this study was to detect possible associations between respiratory pathogens from tracheal aspirate and oral biofilm samples in intubated patients in an intensive care unit (ICU, and to identify the most common respiratory pathogens in oral biofilm, particularly in patients that developed ventilator-associated pneumonia (VAP. Two oral biofilm samples were collected from the tongue of intubated patients (at admission and after 48 hours and analyzed by culture with the Antibiotic Sensitivity Test. The results from the tongue biofilm samples were compared with the tracheal secretions samples. A total of 59.37% of patients exhibited the same species of pathogens in their tracheal aspirate and oral biofilm, of which 8 (42.1% developed VAP, 10 (52.63% did not develop pneumonia and one (5.26% had aspiration pneumonia. There was a statistically significant association between presence of microorganisms in the tracheal and mouth samples for the following pathogens: Klebsiella pneumoniae, Candida albicans, Pseudomonas aeruginosa, Enterobacter gergoviae, Streptococcus spp and Serratia marcescens (p < 0.05. Pathogens that are present in tracheal aspirates of intubated patients can be detected in their oral cavity, especially in those who developed VAP or aspiration pneumonia. Thus, the results indicate that an improved oral care in these patients could decrease ICU pneumonia rates.

  10. Fracture of an endoluminal nitinol stent used in the treatment of tracheal collapse in a dog.

    Science.gov (United States)

    Mittleman, Elise; Weisse, Chick; Mehler, Stephen J; Lee, Justine A

    2004-10-15

    A 5-year-old castrated male Pomeranian was evaluated because of severe dyspnea and coughing, and a diagnosis of complete, static collapse of the trachea at the thoracic inlet was made. After failure to improve with medical management alone, an endoluminal tracheal stent was placed, which resulted in resolution of signs. Ten weeks after stent placement, the dog underwent tracheal resection and anastomosis because the stent had fractured at the level of the thoracic inlet. One year after surgery, the dog was doing well and required treatment with hydrocodone infrequently. Compared with other surgical treatment options, placement of an endoluminal tracheal stent is a relatively noninvasive intervention that can provide effective relief from the clinical signs associated with tracheal collapse in dogs. Implantation of endoluminal tracheal stents may be associated with complications; therefore, the procedure may best be regarded as a salvage procedure for dogs with end-stage disease that are refractory to appropriate medical management, have extensive collapse of the intrathoracic portion of the trachea, or are poor candidates for surgery.

  11. Evaluation of the Airtraq and Macintosh laryngoscopes in patients at increased risk for difficult tracheal intubation.

    LENUS (Irish Health Repository)

    Maharaj, C H

    2008-02-01

    The Airtraq, a novel single use indirect laryngoscope, has demonstrated promise in the normal and simulated difficult airway. We compared the ease of intubation using the Airtraq with the Macintosh laryngoscope, in patients at increased risk for difficult tracheal intubation, in a randomised, controlled clinical trial. Forty consenting patients presenting for surgery requiring tracheal intubation, who were deemed to possess at least three characteristics indicating an increased risk for difficulty in tracheal intubation, were randomly assigned to undergo tracheal intubation using a Macintosh (n = 20) or Airtraq (n = 20) laryngoscope. All patients were intubated by one of three anaesthetists experienced in the use of both laryngoscopes. Four patients were not successfully intubated with the Macintosh laryngoscope, but were intubated successfully with the Airtraq. The Airtraq reduced the duration of intubation attempts (mean (SD); 13.4 (6.3) vs 47.7 (8.5) s), the need for additional manoeuvres, and the intubation difficulty score (0.4 (0.8) vs 7.7 (3.0)). Tracheal intubation with the Airtraq also reduced the degree of haemodynamic stimulation and minor trauma compared to the Macintosh laryngoscope.

  12. Early detection of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Nagalakshmi Chowdhary

    2014-01-01

    Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

  13. Genetics Home Reference: congenital mirror movement disorder

    Science.gov (United States)

    ... Health Conditions Congenital mirror movement disorder Congenital mirror movement disorder Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Congenital mirror movement disorder is a condition in which intentional movements ...

  14. Relation between agenesis and shape anomaly of maxillary lateral incisors and canine impaction

    Directory of Open Access Journals (Sweden)

    Anísio Bueno de Carvalho

    2012-12-01

    Full Text Available OBJECTIVE: The purpose was to obtain information about the relation between agenesis and shape anomaly of maxillary lateral incisors and canine impaction. METHODS: Seventy-three patients with canine impaction and 73 control patients, without canine impaction, were evaluated. The mesiodistal distances of the maxillary lateral incisors adjacent to the impacted canines and the correspondent mandibular lateral incisors were measured. The adjacent lateral incisors were classified in: 1 - absent, 2 - small, 3 - peg-shaped, 4 - standard. RESULTS: The results showed that among the patients with impacted canines, there were 21 anomalous teeth (small and peg-shaped and among the control patients there were only three small and peg-shaped teeth, with a statistically significant difference (p = 0.001. No patients were found with impacted canines and absent lateral incisors. CONCLUSION: It was concluded that in patients with anomalous lateral incisors (small and peg-shaped there is a probability to present impacted canines and this must be considered.OBJETIVO: o objetivo foi obter informação sobre a relação existente entre a agenesia e/ou anomalia de forma de incisivos laterais superiores e impacção de caninos. MÉTODOS: foram avaliados 73 pacientes com impacção de caninos e 73 pacientes controle, sem impacção de caninos. Foram medidas as distâncias mesiodistais dos incisivos laterais superiores adjacentes aos caninos impactados e os incisivos laterais correspondentes inferiores. Os incisivos laterais adjacentes foram classificados em: 1 - ausentes; 2 - pequenos; 3 - conoides; 4 - normais. RESULTADOS: os resultados mostraram que no grupo de pacientes com caninos impactados foram encontrados 22 dentes anômalos (pequenos e conoides, e no grupo controle apenas três dentes pequenos e conoides, sendo uma diferença estatisticamente significativa (p=0,001. Não foram encontrados pacientes com canino impactado e incisivo lateral ausente. CONCLUS

  15. Deep Venous Thrombosis of the Leg, Associated with Agenesis of the Infrarenal Inferior Vena Cava and Hypoplastic Left Kidney (KILT Syndrome in a 14-Year-Old Child

    Directory of Open Access Journals (Sweden)

    Sakshi Bami

    2015-01-01

    Full Text Available Agenesis of the inferior vena cava (IVC is a rare anomaly which can be identified as incidental finding or can be associated with iliofemoral vein thrombosis. IVC agenesis has a known association with renal anomalies which are mainly confined to the right kidney. We describe a case of a 14-year-old male who presented with left leg swelling and pain. Ultrasonography confirmed the presence of left leg deep vein thrombosis (DVT. No underlying hematologic risk factors were identified. A CT scan was obtained which demonstrated absent infrarenal IVC and extensive thrombosis in the left deep venous system and development of collateral venous flow into the azygous/hemiazygous system, with extension of thrombus into paraspinal collaterals. An additional finding in the patient was an atrophic left kidney and stenosis of an accessory left renal artery. Agenesis of the IVC should be considered in a young patient presenting with lower extremity DVT, especially in patients with no risk factors for thrombosis. As agenesis of the IVC cannot be corrected, one should be aware that there is a lifelong risk of lower extremity DVT.

  16. Molecular and Genetic Studies of Congenital Myopathies

    Science.gov (United States)

    2018-03-21

    Central Core Disease; Centronuclear Myopathy; Congenital Fiber Type Disproportion; Multiminicore Disease; Myotubular Myopathy; Nemaline Myopathy; Rigid Spine Muscular Dystrophy; Undefined Congenital Myopathy

  17. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... with mental retardation Muscular dystrophy, congenital, Fukuyama type Muscular dystrophy, congenital, with central nervous system involvement Polymicrogyria with muscular dystrophy Related Information How ...

  18. Tracheal stenosis after treatment with metallic stent: a situation worse than the initial problem

    International Nuclear Information System (INIS)

    Barreto, Jaime; Mejia, Bibiana; Nino, Federico; Garavito, Carlos

    2008-01-01

    Stens are a tubular device for use in trachea, carina or main bronchus to provide a support and maintain the permeability of these structures. There are two groups: Metallic and Silicone stent. Either metallic and silicone stents has a special properties. Metallic stent are very attractive option due to relative easy in their placement. The accumulated experience in this therapy has allowed to recognize several complications to take into consideration mainly in patients with tracheal benign stenosis. FDA has recommended don't use metallic stents, covered and not covered in benign diseases. We report a case of a tracheal stenosis secondary to prolonged ortho tracheal intubation that required extensive interventions to control symptoms of central airway obstruction, between April and July of 2005.

  19. Management of a case of left tracheal sleeve pneumonectomy under cardiopulmonary bypass: Anesthesia perspectives

    Directory of Open Access Journals (Sweden)

    Aman Jyoti

    2014-01-01

    Full Text Available The lung tumors with carinal involvement are frequently managed with tracheal sleeve pneumonectomy and tracheobronchial anastomosis without use of cardiopulmonary bypass (CPB. Various modes of ventilation have been described during tracheal resection and anastomosis. Use of CPB during this period allows the procedure to be conducted in a more controlled way. We performed tracheal sleeve pneumonectomy for adenoid cystic carcinoma of left lung involving carina. The surgery was performed in two stages. In the first stage, left pneumonectomy was performed and in the second stage after 48 h, tracheobronchial resection and anastomosis was performed under CPB. Second stage was delayed to avoid excessive bleeding (due to heparinization from the extensive vascular raw area left after pneumonectomy. Meticulous peri-operative planning and optimal post-operative care helped in successful management of a complex case, which is associated with high morbidity and mortality.

  20. Tracheal epithelial-myoepithelial carcinoma associated with sarcoid-like reaction: A case report

    Science.gov (United States)

    Dong, Huawei; Tatsuno, Brent K.; Betancourt, Jaime; Oh, Scott S.

    2014-01-01

    Epithelial-myoepithelial carcinomas are rare tumors that primarily originate in the salivary glands but have also been found in the tracheobronchial tree. We report the first case of epithelial-myoepithelial carcinoma associated with sarcoidosis. A 61 year old Hispanic man presented with altered mental status and hypercalcemia. Imaging revealed diffuse intra-thoracic and intra-abdominal lymphadenopathy. A diagnostic bronchoscopy was performed where an incidental tracheal nodule was discovered and biopsied. Pathology was consistent with epithelial-myoepithelial carcinoma. Lymph node biopsy demonstrated non-caseating granulomas consistent with sarcoidosis. Patient underwent tracheal resection of the primary tumor with primary tracheal reconstruction. Hypercalcemia subsequently normalized with clinical improvement. Repeat CT imaging demonstrated complete resolution of lymphadenopathy. Our findings are suggestive of a possible paraneoplastic sarcoid-like reaction to the epithelial-myoepithelial carcinoma with associated lymphadenopathy and symptomatic hypercalcemia. PMID:26029574

  1. Thyroidectomy improves tracheal anatomy and airflow in patients with nodular goiter

    DEFF Research Database (Denmark)

    Sørensen, Jesper Roed; Lauridsen, Jeppe Killerich; Døssing, Helle

    . Effect sizes (ES) were calculated as mean change divided by standard deviation at baseline. ES of 0.2-0.5 were defined as small, 0.5-0.8 as moderate, and values >0.8 as large. Results: Sixty-five patients completed all examinations. Median goiter volume was 58 mL (range, 14-642 mL) before surgery...... resonance images (MRI) of the neck and respiratory flow-volume curves, including both in- and expiration, were performed prior to and six months following surgery. The evaluated tracheal dimensions included the smallest cross-sectional area of the trachea (SCAT), tracheal narrowing, and tracheal deviation...... with surgical removal of median 46 mL (range, 5-642 mL) (pdeviation were diminished by median 26% (ES=0.67), and 33% (ES=0.73), respectively, while SCAT increased by 17% (ES=0.61). Correspondingly, each 10% decrease in goiter volume resulted...

  2. The use of scaning electron microscopy in postvaccinal evaluation of tracheal epithelium of Coturnix coturnix japonica

    Directory of Open Access Journals (Sweden)

    Santin Elizabeth

    2003-01-01

    Full Text Available This study aimed at evaluating the use of scanning electron microscopy in the study of the post-vaccinal respiratory reaction of the tracheal epithelium of quails (Coturnix coturnix japonica immunized against Newcastle disease. A number of 36 quails were distributed into four groups: T1 fraction three-quarters control birds (non-vaccinated; T2 fraction three-quarters birds vaccinated with Ulster 2C strain; t3 fraction three-quarters birds vaccinated with B1 strain; t4 fraction three-quarters birds vaccinated with LaSota strain. Regardless the experimental group, birds did not show detectable clinical signs of post-vaccinal respiratory reaction. However, the analysis of tracheal fragments by scanning electron microscopy showed that birds vaccinated with B1 and LaSota strains developed epithelial sloughing of the trachea, whereas those vaccinated Ulster 2C strain did not develop this change, demonstrating intact tracheal epithelium, similar to the control group.

  3. Endoscopic Resection of Tracheal Tumor in an Elderly Woman Under Extracorporeal Membrane Oxygenation

    Directory of Open Access Journals (Sweden)

    Yi-Ying Chiang

    2011-03-01

    Full Text Available Anesthetic management of patients with difficult airway is challenging, especially in patients who present with near total occlusion of the airway. Tracheal tumors occur more frequently in elderly patients who are more prone to hypoxic injury. Reliable ventilation and oxygenation are mandatory for a safe and sound intervention. Herein, we report on a 71-year-old woman with a large tracheal tumor occluding approximately 90% of the tracheal lumen. Extracorporeal membrane oxygenation under local anesthesia was used during electrocautery resection of the tumor because of the possibility of fatal airway collapse due to the degree of occlusion and location of the tumor. After the tumor had been successfully resected by means of bronchoscopy, an endotracheal tube was inserted, and the patient was weaned from extracorporeal membrane oxygenation.

  4. Development of a 3D bellows tracheal graft: mechanical behavior analysis, fabrication and an in vivo feasibility study

    International Nuclear Information System (INIS)

    Park, Jeong Hun; Jung, Jin Woo; Lee, Jung-Seob; Cho, Dong-Woo; Kang, Hyun-Wook; Joo, Young Hoon

    2012-01-01

    Artificial tracheal grafts should have not only enough compressive strength to maintain an open tracheal lumen, but also sufficient flexibility for stable mechanical behavior, similar to the native trachea at the implant site. In this study, we developed a new 3D artificial tracheal graft using a bellows design for considering its mechanical behavior. To investigate the mechanical behavior of the bellows structure, finite element method (FEM) analysis in terms of longitudinal tension/compression, bending and radial compression was conducted. The bellows structure was then compared with the cylinder structure generally used for artificial tracheal grafts. The FEM analysis showed that the bellows had outstanding flexibility in longitudinal tension/compression and bending. Moreover, the bellows kept the lumen open without severe luminal deformation in comparison with the cylinder structure. A three-dimensional artificial tracheal graft with a bellows design was fabricated using indirect solid freeform fabrication technology, and the actual mechanical test was conducted to investigate the actual mechanical behavior of the bellows graft. The fabricated bellows graft was then applied to segmental tracheal reconstruction in a rabbit model to assess its applicability. The bellows graft was completely incorporated into newly regenerated connective tissue and no obstruction at the implanted site was observed for up to 8 weeks after implantation. The data suggested that the developed bellows tracheal graft could be a promising alternative for tracheal reconstruction. (paper)

  5. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  6. Real-time tracheal ultrasonography for confirmation of endotracheal tube placement during cardiopulmonary resuscitation.

    Science.gov (United States)

    Chou, Hao-Chang; Chong, Kah-Meng; Sim, Shyh-Shyong; Ma, Matthew Huei-Ming; Liu, Shih-Hung; Chen, Nai-Chuan; Wu, Meng-Che; Fu, Chia-Ming; Wang, Chih-Hung; Lee, Chien-Chang; Lien, Wan-Ching; Chen, Shyr-Chyr

    2013-12-01

    This study aimed to evaluate the accuracy of tracheal ultrasonography for assessing endotracheal tube position during cardiopulmonary resuscitation (CPR). We performed a prospective observational study of patients undergoing emergency intubation during CPR. Real-time tracheal ultrasonography was performed during the intubation with the transducer placed transversely just above the suprasternal notch, to assess for endotracheal tube positioning and exclude esophageal intubation. The position of trachea was identified by a hyperechoic air-mucosa (A-M) interface with posterior reverberation artifact (comet-tail artifact). The endotracheal tube position was defined as endotracheal if single A-M interface with comet-tail artifact was observed. Endotracheal tube position was defined as intraesophageal if a second A-M interface appeared, suggesting a false second airway (double tract sign). The gold standard of correct endotracheal intubation was the combination of clinical auscultation and quantitative waveform capnography. The main outcome was the accuracy of tracheal ultrasonography in assessing endotracheal tube position during CPR. Among the 89 patients enrolled, 7 (7.8%) had esophageal intubations. The sensitivity, specificity, positive predictive value, and negative predictive value of tracheal ultrasonography were 100% (95% confidence interval [CI]: 94.4-100%), 85.7% (95% CI: 42.0-99.2%), 98.8% (95% CI: 92.5-99.0%) and 100% (95% CI: 54.7-100%), respectively. Positive and negative likelihood ratios were 7.0 (95% CI: 1.1-43.0) and 0.0, respectively. Real-time tracheal ultrasonography is an accurate method for identifying endotracheal tube position during CPR without the need for interruption of chest compression. Tracheal ultrasonography in resuscitation management may serve as a powerful adjunct in trained hands. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  7. Congenital Midureteric Stricture

    Directory of Open Access Journals (Sweden)

    Singh Shalinder

    2001-01-01

    Full Text Available Congenital midureteric obstruction is a rare entity which can be caused by either ureteric valves or strictures. We report our experience with four patients with midureteric obstruction due to stricture over a six-year period. The condition needs to be differentiated from obstruction of the pelviureteric and vesicoureteric junctions. Obstruction can be initially screened by ultrasound and confirmed by a radionuclide scan with furosemide challenge. Retrograde ureteropyelography and/or intravenous urography may be required to define the level of the stricture.

  8. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  9. Congenital Syphilis Masquerading as Leukemia

    OpenAIRE

    Lee, Tiffany; Bell, Stephanie; Scimeme, Jason; Maraqa, Nizar

    2017-01-01

    As of late, the incidence of congenital syphilis in the United States is increasing. Each new case represents a failure of preventing, diagnosing, and treating syphilis in pregnant women. Pediatricians should confirm that all women have received adequate screening for and management of syphilis during pregnancy. Congenital syphilis is easily treatable but may be a diagnostic challenge with high morbidity and mortality.

  10. What Are Congenital Heart Defects?

    Science.gov (United States)

    ... a baby with a congenital heart defect. Family history and genetics Congenital heart disease is not usually passed along ... you or your child to a specialist in genetic testing. Cardiac MRI to diagnose a ... Factors to review family history, smoking, and medicines that increase your risk of ...

  11. Tracheal Chondrosarcoma: Systematic Review of Tumor Characteristics, Diagnosis, and Treatment Outcomes with Case Report

    Directory of Open Access Journals (Sweden)

    Emily A. Kutzner

    2017-01-01

    Full Text Available To our knowledge this is the first systematic review of tracheal chondrosarcoma treatment outcomes. Management insights are thoroughly discussed. Men constitute 93.8% of cases, and most of these occur in the distal trachea. The most common symptom, dyspnea, occurs in virtually all patients. Extratracheal extension had occurred in 78.6% of patients. Definitive treatment with tracheal resection showed no recurrences in 10 patients with mean follow-up of 3.1 years. Adjuvant radiotherapy may be utilized for improving local control when open complete resection cannot be performed, but only after endoscopic excision of gross tumor.

  12. Complete subglottic tracheal stenosis managed with rigid bronchoscopy and T-tube placement

    Directory of Open Access Journals (Sweden)

    Kuruswamy Thurai Prasad

    2016-01-01

    Full Text Available Surgery is the preferred treatment modality for benign tracheal stenosis. Interventional bronchoscopy is used as a bridge to surgery or in instances when surgery is not feasible or has failed. Stenosis in the subglottic trachea is particularly a treatment challenge, in view of its proximity to the vocal cords. Herein, we describe a patient with complete tracheal stenosis in the subglottic region, which developed after prolonged intubation and mechanical ventilation. The patient developed recurrent stenosis despite multiple surgical and endoscopic procedures. We were able to manage the patient successfully with rigid bronchoscopy and Montgomery T-tube placement.

  13. Is intrathoracic tracheal collapsibility correlated to clinical phenotypes and sex in patients with COPD?

    Directory of Open Access Journals (Sweden)

    Camiciottoli G

    2015-04-01

    Full Text Available Gianna Camiciottoli,1 Stefano Diciotti,2 Francesca Bigazzi,1 Simone Lombardo,3 Maurizio Bartolucci,4 Matteo Paoletti,1 Mario Mascalchi,3 Massimo Pistolesi1 1Section of Respiratory Medicine, Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy; 2Department of Electrical, Electronic, and Information Engineering “Guglielmo Marconi,” University of Bologna, Cesena, Italy; 3Radiodiagnostic Section, Department of Clinical and Experimental Biomedical Sciences, University of Florence, Florence, Italy; 4Department of Diagnostic Imaging, Careggi University Hospital, Florence, Italy Abstract: A substantial proportion of patients with chronic obstructive pulmonary disease (COPD develops various degree of intrathoracic tracheal collapsibility. We studied whether the magnitude of intrathoracic tracheal collapsibility could be different across clinical phenotypes and sex in COPD. Intrathoracic tracheal collapsibility measured at paired inspiratory–expiratory low dose computed tomography (CT and its correlation with clinical, functional, and CT-densitometric data were investigated in 69 patients with COPD according to their predominant conductive airway or emphysema phenotypes and according to sex. Intrathoracic tracheal collapsibility was higher in patients with predominant conductive airway disease (n=28 and in females (n=27. Women with a predominant conductive airway phenotype (n=10 showed a significantly greater degree of collapsibility than women with predominant emphysema (28.9%±4% versus 11.6%±2%; P<0.001. Intrathoracic tracheal collapsibility was directly correlated with inspiratory–expiratory volume variation at CT and with forced expiratory volume (1 second, and inversely correlated with reduced CT lung density and functional residual capacity. Intrathoracic tracheal collapsibility was not correlated with cough and wheezing; however, intrathoracic tracheal collapsibility and clinical phenotypes of COPD

  14. Bradykinin B2 receptor-mediated phosphoinositide hydrolysis in bovine cultured tracheal smooth muscle cells.

    OpenAIRE

    Marsh, K. A.; Hill, S. J.

    1992-01-01

    1. Bovine tracheal smooth muscle cells were established in culture to study agonist-induced phosphoinositide (PI) hydrolysis in this tissue. 2. Bradykinin (0.1 nM-10 microM) evoked a concentration-dependent increase (log EC50 (M) = -9.4 +/- 0.2; n = 8) in the accumulation of total [3H]-inositol phosphates in cultured tracheal smooth muscle cells whereas the selective B1 receptor agonist des-Arg9-bradykinin (10 microM) was significantly less effective (16% of bradykinin maximal response; relat...

  15. A retropharyngeal-mediastinal hematoma with supraglottic and tracheal obstruction: The role of multidisciplinary airway management

    Directory of Open Access Journals (Sweden)

    Birkholz Torsten

    2010-01-01

    Full Text Available A 77-year-old man suffered hypoxemic cardiac arrest by supraglottic and tracheal airway obstruction in the emergency department. A previously unknown cervical fracture had caused a traumatic retropharyngeal-mediastinal hematoma. A lifesaving surgical emergency tracheostomy succeeded. Supraglottic and tracheal obstruction by a retropharyngeal-mediastinal hematoma with successful resuscitation via emergency tracheostomy after hypoxemic cardiac arrest has never been reported in a context of trauma. This clinically demanding case outlines the need for multidisciplinary airway management systems with continuous training and well-implemented guidelines. Only multidisciplinary staff preparedness and readily available equipments for the unanticipated difficult airway solved the catastrophic clinical situation.

  16. A visual stethoscope to detect the position of the tracheal tube.

    Science.gov (United States)

    Kato, Hiromi; Suzuki, Akira; Nakajima, Yoshiki; Makino, Hiroshi; Sanjo, Yoshimitsu; Nakai, Takayoshi; Shiraishi, Yoshito; Katoh, Takasumi; Sato, Shigehito

    2009-12-01

    Advancing a tracheal tube into the bronchus produces unilateral breath sounds. We created a Visual Stethoscope that allows real-time fast Fourier transformation of the sound signal and 3-dimensional (frequency-amplitude-time) color rendering of the results on a personal computer with simultaneous processing of 2 individual sound signals. The aim of this study was to evaluate whether the Visual Stethoscope can detect bronchial intubation in comparison with auscultation. After induction of general anesthesia, the trachea was intubated with a tracheal tube. The distance from the incisors to the carina was measured using a fiberoptic bronchoscope. While the anesthesiologist advanced the tracheal tube from the trachea to the bronchus, another anesthesiologist auscultated breath sounds to detect changes of the breath sounds and/or disappearance of bilateral breath sounds for every 1 cm that the tracheal tube was advanced. Two precordial stethoscopes placed at the left and right sides of the chest were used to record breath sounds simultaneously. Subsequently, at a later date, we randomly entered the recorded breath sounds into the Visual Stethoscope. The same anesthesiologist observed the visualized breath sounds on the personal computer screen processed by the Visual Stethoscope to examine changes of breath sounds and/or disappearance of bilateral breath sound. We compared the decision made based on auscultation with that made based on the results of the visualized breath sounds using the Visual Stethoscope. Thirty patients were enrolled in the study. When irregular breath sounds were auscultated, the tip of the tracheal tube was located at 0.6 +/- 1.2 cm on the bronchial side of the carina. Using the Visual Stethoscope, when there were any changes of the shape of the visualized breath sound, the tube was located at 0.4 +/- 0.8 cm on the tracheal side of the carina (P Stethoscope (not significant). During advancement of the tracheal tube, alterations of the shape of the

  17. Endobronchial angiofibroma in the aberrant tracheal bronchus presenting as spontaneous pneumomediastinum.

    Science.gov (United States)

    Kim, Kyung Soo; Moon, Young Kyu; Jeon, Hyun Woo; Park, Chan Beom; Ahn, Myeong Im; Lee, Kyo Young; Park, Jae Kil

    2015-07-22

    Spontaneous pneumomediastinum is a self-limiting benign disease but abnormal bronchial lesions can be rarely found incidentally, and in selected cases will require surgical resection. A 38-year-old man presented with a spontaneous pneumomediastinum. Chest computed tomography revealed an incidental linear endobronchial tumour in the aberrant tracheal bronchus. The tumour was removed surgically and diagnosed with a rare benign tumour of endobronchial angiofibroma. We report a rare case of endobronchial angiofibroma in the aberrant tracheal bronchus which was detected during the evaluation of a spontaneous pneumomediastinum.

  18. Laryngeal mask airway guided tracheal intubation in a neonate with the Pierre Robin syndrome

    DEFF Research Database (Denmark)

    Hansen, Tom Giedsing; Joensen, Henning; Henneberg, Steen Winther

    1995-01-01

    Endotracheal intubation in infants with the Pierre Robin syndrome may sometimes be impossible to accomplish by conventional means. To aid difficult tracheal intubation many different techniques have been described. We present a case, in which we successfully intubated a small-for-date newborn boy...... with the Pierre Robin syndrome by using a modified laryngeal mask airway (no. 1) as a guide for the endotracheal tube. The technique is easy to perform, less traumatic and less time-consuming than multiple attempts at laryngoscopy or blind tracheal intubation....

  19. [Neonatal tumours and congenital malformations].

    Science.gov (United States)

    Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

    2008-06-01

    The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

  20. Congenital nystagmus and negative electroretinography

    Directory of Open Access Journals (Sweden)

    Roussi M

    2011-04-01

    Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness

  1. Congenital Leukemia in Down's syndrome

    International Nuclear Information System (INIS)

    Iqbal, W.; Khan, F.; Muzaffar, M.; Khan, U. A.; Rehman, M. U.; Khan, M. A.; Bari, A.

    2006-01-01

    Congenital Leukemia is a condition and often associated with fatal outcome/sup 1/. Most of the neonatal cases reported have acute non-lymphoblastic leukemia, in contrast to the predominance of acute lymphoblastic leukemia found in later childhood. congenital leukemia is occasionally associated with number of congenital anomalies and with chromosomal disorders such as Down's syndrome. Subtle cytogenetic abnormalities may occur more commonly in the affected infants and their parents, when studied with newer cytogenetic techniques/sup 2/. Inherent unstable hematopoieses resulting from chromosomal aberration in children with Downs's syndrome can present with transient myeloproliferative disorder, mimicking leukemia which undergoes spontaneous recovery/sup 3/. Only few cases of congenital leukemia with Downs syndrome, presented as congenital leukemia. (author)

  2. Use of a Nitinol Wire Stent for Management of Severe Tracheal Stenosis in an Eclectus Parrot (Eclectus roratus).

    Science.gov (United States)

    Mejia-Fava, Johanna; Holmes, Shannon P; Radlinsky, MaryAnn; Johnson, Dan; Ellis, Angela E; Mayer, Jörg; Schnellbacher, Rodney; Divers, Stephen J

    2015-09-01

    A 25-year-old, female eclectus parrot (Eclectus roratus) presented for dyspnea 3 weeks after anesthesia and surgery for egg yolk coelomitis. Radiography, computed tomography, and tracheoscopy revealed multiple tracheal strictures spanning a length of 2.6 cm in the mid to distal trachea. Histopathologic examination revealed mild fibrosis, inflammation, and hyperplasia consistent with acquired tracheal strictures. Tracheal resection was not considered possible because of the length of the affected trachea. The strictures were resected endoscopically, and repeated balloon dilation under fluoroscopic guidance over the course of 10 months resulted in immediate but unsustained improvement. Computed tomography was used to measure the stenotic area. A 4 × 36-mm, custom-made, nitinol wire stent was inserted into the trachea under fluoroscopic guidance. After stent placement, intermittent episodes of mild to moderate dyspnea continued, and these responded to nebulization with a combination of saline, acetylcysteine, and dexamethasone. Multiple attempts to wean the patient off nebulization therapy and to switch to a corticosteroid-free combination were unsuccessful. The parrot eventually developed complications, was euthanatized, and necropsy was performed. Histologically, the tracheal mucosa had widespread erosion to ulceration, with accumulation of intraluminal exudate and bacteria, severe degeneration of skeletal muscle and tracheal rings, prominent fibrosis, and mild to moderate, submucosal inflammation. Clinicopathologic findings in this case suggested tracheomalacia, which has not been previously described in birds. Custom-made tracheal stents can be used for severe tracheal stenosis in birds when tracheal resection and anastomosis is not possible. Complications of tracheal stent placement in birds may include tracheitis and tracheomalacia. To our knowledge, this is the first report of tracheal stent placement in an avian species.

  3. Congenital nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Claudia Fanni

    2014-06-01

    Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  4. Congenital extrahepatic portosystemic shunts

    Energy Technology Data Exchange (ETDEWEB)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S. [Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, M5G 1X8, Toronto, Ontario (Canada)

    2003-09-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  5. Congenital extrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S.

    2003-01-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  6. Congenital seminal vesicle cyst accompanying with ipsilateral renal agenesis in an adolescent patient: A pediatric radiologist approach to Zinner’s syndrome

    Directory of Open Access Journals (Sweden)

    Mehmet Burak Özkan

    2015-12-01

    Full Text Available A fifteen-year-old boy who had complaints of left sided pelvic pain with known ipsilateral left renal agenesia was referred to pediatric radiology department. Incidentally, his sonography examination revealed a dilated tubular structure located in the retro-vesicular region from cephalic to prostate. Contrast enhanced pelvic MRI showed a huge seminal vesicle cyst which is over 6 cm without a mass effect near the aspect border of the prostate and bladder. The patient was diagnosed with Zinner syndrome. The patient doesn’t have new complaint with no definite increase in the diameter of the cyst. In this case presentation we are discussing the Zinner syndrome’s imaging findings from a pediatric radiologist approach with a brief review of the literature.

  7. Hippocampal agenesis in an individual who engaged in violent criminal behaviors after discontinuing carbamazepine and paroxetine treatment.

    Science.gov (United States)

    Hanada, Hiroaki; Akiyoshi, Jotaro; Kanehisa, Masayuki; Ishitobi, Yoshinobu; Tsuru, Jusen; Tanaka, Yoshihiro; Shimomura, Tsuyoshi; Kawano, Yoshihisa

    2013-01-01

    Antidepressant discontinuation syndrome (ADS) occurs after abrupt discontinuation of an antidepressant medication. A 23-year-old man with right hippocampal agenesis demonstrated sexual crime (hypersexuality) since the age of eight and had been successfully treated with carbamazepine since the age of 13. He had required increased doses of paroxetine and carbamazepine owing to the development of an unstable affect after quitting his job. He abruptly stopped taking his medication for 3 days and his criminal behaviors re-emerged. We examined changes in brain structure and activity before and after medication cessation, using MRI and functional MRI (fMRI). The image of a girl in a swimsuit increased activity in the thalamus only after medication discontinuation. The alteration in thalamic activity might induce hypersexuality. We conclude that a primary hypersexuality had been suppressed with carbamazepine and paroxetine treatment, and the discontinuation of the medication caused the hypersexuality. © 2012 American Academy of Forensic Sciences.

  8. Discordant tooth agenesis and peg-shaped in a pair of monozygotic twins: Clinical and molecular study

    Directory of Open Access Journals (Sweden)

    Lívia Azeredo Alves Antunes

    2013-01-01

    Full Text Available The present report aimed to study an uncommon case of a pair of twins that presented a discordant dental phenotype. Family investigation, clinical, radiographic examination and molecular analysis were performed in both girls. Molecular analysis confirmed the monozygosity by deoxyribonucleic acid chip technology. One twin presented tooth agenesis in left upper lateral incisor and peg-shaped on the contra lateral side while the other twin had no dental alterations. The dental casts study employed digital caliper to compare morphological dimensions and showed alteration only in peg-shaped tooth. In conclusion, this study provide support that one or more mutated gene could cause discordances in dental phenotype in these monozygotic twins.

  9. Fetal chromosome abnormalities and congenital malformations: an ...

    African Journals Online (AJOL)

    The results also showed that Multiple congenital anomalies (MCA) represented among 42.2%, congenital malformation of CNS represents 26.6%, congenital malformation of the skeletal system 20%, congenital polycystic kidney 8.8% and pyloric stenosis in 2.2%. Among the 21 women with abnormal karyotype of amniotic ...

  10. Genetics Home Reference: critical congenital heart disease

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Critical congenital heart disease Critical congenital heart disease Printable PDF Open All Close All ... for Disease Control and Prevention: Congenital Heart Defects Disease InfoSearch: Congenital Heart Defects KidsHealth from Nemours Lucile Packard Children's ...

  11. General Concepts in Adult Congenital Heart Disease.

    Science.gov (United States)

    Mutluer, Ferit Onur; Çeliker, Alpay

    2018-01-20

    Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

  12. General Concepts in Adult Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Ferit Onur Mutluer

    2018-02-01

    Full Text Available Congenital heart disease in adults (adult congenital heart disease is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face

  13. Groningen Dilatation Tracheoscope in Treatment of Moderate Subglottic and Tracheal Stenosis

    NARCIS (Netherlands)

    Halmos, Gyorgy; van der Laan, Bernard F. A. M.; Dikkers, Frederik G.

    Objectives: We describe our experience with the Groningen Dilatation Tracheoscope (Karl Storz GmbH & Co, Tuttlingen, Germany) in treating benign subglottic and tracheal stenosis. Methods: We performed a retrospective survey from a medical record analysis of 26 patients with different origins of

  14. Expandable metal stents for tracheal obstruction: permanent or temporary? A cautionary tale.

    OpenAIRE

    Hind, C R; Donnelly, R J

    1992-01-01

    An expandable metal stent inserted via a long term tracheostomy successfully relieved life threatening respiratory obstruction due to benign tracheal stenosis. Later the patient's tracheostomy suction catheter became stuck on the stent and dislodged it. The stent was removed electively, without damaging the trachea, with a rigid biopsy forceps.

  15. Intermittent subglottic secretion drainage may cause tracheal damage in patients with few oropharyngeal secretions.

    Science.gov (United States)

    Suys, E; Nieboer, K; Stiers, W; De Regt, J; Huyghens, L; Spapen, H

    2013-12-01

    Injurious prolapse of tracheal mucosa into the suction port has been reported in up to 50% of intubated patients receiving continuous aspiration of subglottic secretions. We investigated whether similar injury could be inflicted by automated intermittent aspiration. Six consecutive patients, intubated with the Mallinckrodt TaperGuard Evac™ endotracheal tube, were studied. A flow sensor was placed between the vacuum regulating system and the mucus collector. Intermittent suctioning was performed at a pressure of -125 mmHg with a 25s interval and duration of 15s. After 24h, a CT scan of the tracheal region was performed. Excessive negative suction pressure, a fast drop in aspiration flow to zero, and important "swinging" movements of secretions in the evacuation line were observed in all patients. Oral instillation of antiseptic mouthwash restored normal aspiration flow and secretion mobility. CT imaging showed marked entrapment of tracheal mucosa into the suction port in all patients. In patients with few oropharyngeal secretions, automated intermittent subglottic aspiration may result in significant and potential harmful invagination of tracheal mucosa into the suction lumen. A critical amount of fluid must be present in the oropharynx to assure adequate and safe aspiration. Copyright © 2013 Elsevier Ltd. All rights reserved.

  16. Awake fiberoptic or awake video laryngoscopic tracheal intubation in patients with anticipated difficult airway management

    DEFF Research Database (Denmark)

    Rosenstock, Charlotte Vallentin; Thøgersen, Bente; Afshari, Arash

    2012-01-01

    Awake flexible fiberoptic intubation (FFI) is the gold standard for management of anticipated difficult tracheal intubation. The purpose of this study was to compare awake FFI to awake McGrath® video laryngoscope, (MVL), (Aircraft Medical, Edinburgh, Scotland, United Kingdom) intubation in patients...... with an anticipated difficult intubation. The authors examined the hypothesis that MVL intubation would be faster than FFI....

  17. Equine tracheal epithelial membrane strips - An alternate method for examining epithelial cell arachidonic acid metabolism

    International Nuclear Information System (INIS)

    Gray, P.R.; Derksen, F.J.; Robinson, N.E.; Peter-Golden, M.L.

    1990-01-01

    Arachidonic acid metabolism by tracheal epithelium can be studied using enzymatically dispersed cell suspensions or cell cultures. Both techniques require considerable tissue disruption and manipulation and may not accurately represent in vivo activity. The authors have developed an alternate method for obtaining strips of equine tracheal epithelium without enzymatic digestion. In the horse, a prominent elastic lamina supports the tracheal epithelium. By physical splitting this lamina, they obtained strips (≤12 x 1.5 cm) of pseudostratified columnar epithelium attached to a layer of elastic tissue 30-100 μm thick. Epithelial strips (1.2 x 0.5 cm) were attached to plexiglass rods and incubated with [ 3 H]arachidonic acid in M199 medium (0.5 μCi/ml) for 24 hours at 37C. The strips incorporated 36±4% (mean ± SEM) of the total radioactivity and released 8.0±1.2% of incorporated radioactivity when stimulated by 5.0 μM calcium ionophore A23187. The extracted supernatant was processed using HPLC, resulting in peaks of radioactivity that co-eluted with authentic PGE 2 , PGF 2 α, and 12-HETE standards. The greatest activity corresponded to the PGE 2 and PGF 2 α standards, which is a similar pattern to that reported for cultured human tracheal epithelium

  18. High frequency components of tracheal sound are emphasized during prolonged flow limitation

    International Nuclear Information System (INIS)

    Tenhunen, M; Huupponen, E; Saastamoinen, A; Kulkas, A; Himanen, S-L; Rauhala, E

    2009-01-01

    A nasal pressure transducer, which is used to study nocturnal airflow, also provides information about the inspiratory flow waveform. A round flow shape is presented during normal breathing. A flattened, non-round shape is found during hypopneas and it can also appear in prolonged episodes. The significance of this prolonged flow limitation is still not established. A tracheal sound spectrum has been analyzed further in order to achieve additional information about breathing during sleep. Increased sound frequencies over 500 Hz have been connected to obstruction of the upper airway. The aim of the present study was to examine the tracheal sound signal content of prolonged flow limitation and to find out whether prolonged flow limitation would consist of abundant high frequency activity. Sleep recordings of 36 consecutive patients were examined. The tracheal sound spectral analysis was performed on 10 min episodes of prolonged flow limitation, normal breathing and periodic apnea-hypopnea breathing. The highest total spectral amplitude, implicating loudest sounds, occurred during flow-limited breathing which also presented loudest sounds in all frequency bands above 100 Hz. In addition, the tracheal sound signal during flow-limited breathing constituted proportionally more high frequency activities compared to normal breathing and even periodic apnea-hypopnea breathing

  19. Clinical evaluation of stethoscope-guided inflation of tracheal tube cuffs.

    Science.gov (United States)

    Kumar, R D C; Hirsch, N P

    2011-11-01

    Tracheal tube cuffs are commonly inflated to pressures exceeding the recommended upper limit of 30 cmH(2)O. We evaluated whether a stethoscope-guided method of cuff inflation results in pressures within the recommended range. Patients were randomly assigned to receive one of two methods of cuff inflation. In the standard 'just seal' group, air was introduced into the tracheal cuff until the audible leak at the mouth disappeared. In the stethoscope-guided group, air was introduced into the cuff until a change from harsh to soft breath sounds occurred, whilst listening with a stethoscope bell placed over the thyroid cartilage. Twenty-five patients were recruited to each group. The median (IQR [range]) cuff pressure in the 'just seal' group was 34 (28-40 [18-49]) cmH(2)O, and in the stethoscope-guided group was 20 (20-26 [16-28]) cmH(2)O, p stethoscope-guided method of tracheal tube cuff inflation is a novel, simple technique that reliably results in acceptable tracheal cuff pressures. © 2011 The Authors. Anaesthesia © 2011 The Association of Anaesthetists of Great Britain and Ireland.

  20. Association between pathogens from tracheal aspirate and oral biofilm of patients on mechanical ventilation.

    Science.gov (United States)

    Souza, Luana Carneiro Diniz; Mota, Vanise Barros Rodrigues da; Carvalho, Alícia Valéria Dos Santos Zaranza de; Corrêa, Rita da Graça Carvalhal Frazão; Libério, Silvana Amado; Lopes, Fernanda Ferreira

    2017-06-05

    The aim of this study was to detect possible associations between respiratory pathogens from tracheal aspirate and oral biofilm samples in intubated patients in an intensive care unit (ICU), and to identify the most common respiratory pathogens in oral biofilm, particularly in patients that developed ventilator-associated pneumonia (VAP). Two oral biofilm samples were collected from the tongue of intubated patients (at admission and after 48 hours) and analyzed by culture with the Antibiotic Sensitivity Test. The results from the tongue biofilm samples were compared with the tracheal secretions samples. A total of 59.37% of patients exhibited the same species of pathogens in their tracheal aspirate and oral biofilm, of which 8 (42.1%) developed VAP, 10 (52.63%) did not develop pneumonia and one (5.26%) had aspiration pneumonia. There was a statistically significant association between presence of microorganisms in the tracheal and mouth samples for the following pathogens: Klebsiella pneumoniae, Candida albicans, Pseudomonas aeruginosa, Enterobacter gergoviae, Streptococcus spp and Serratia marcescens (p aspirates of intubated patients can be detected in their oral cavity, especially in those who developed VAP or aspiration pneumonia. Thus, the results indicate that an improved oral care in these patients could decrease ICU pneumonia rates.

  1. Using tracheal segments for replacement of cervical oesophagus: an experimental study.

    Science.gov (United States)

    Abbasidezfouli, Azizollah; Sharifi, Davood; Sasani, Farhang; Ansari, Damoon; Abarkar, Mohammad; Rahmanijoo, Nasrin; Abbasidezfouli, Golbahar; Sheikhy, Kambiz

    2012-03-01

    Segmental resection and anastomosis of oesophageal lesions are not performed as a routine clinical practice because of complications and associated problems, whereas tracheal resection and anastomosis are a routine clinical practice. In this experimental study, we resected a segment of cervical oesophagus and replaced it with a tracheal segment. In eight dogs (mixed races), weighing 20-30 kg, ageing 1-2 years, under general anaesthesia, through a cervical incision, 5 cm of cervical trachea was separated while preserving its attachments to surrounding fibroareolar tissues. Afterwards, 5 cm of the oesophagus was resected and replaced with a prepared segment of the trachea. Oral liquids were started at the first post-operative day; the animals were kept for 2 months and then euthanized. Quality of swallowing and voice were evaluated. After an autopsy, anastomoses were examined grossly and histopathologically. No complications occurred during surgery. Swallowing function and voice were normal in all eight dogs after the operation. No sign of aspiration was seen in clinical and radiographic examinations after starting oral diet. In autopsy examination, anastomoses were patent without narrowing or abnormal mucosal changes. Remarkable histopathological findings in replaced tracheal segments were squamous metaplasia, atrophy and degeneration of mucosal glands and degeneration of cartilages. Replacement of a segment of the oesophagus with an autogenous tracheal segment is a practical procedure with low complications and can probably be used for the treatment of cervical oesophageal lesions in human beings.

  2. Relaxing action of adrenergic β2-agonists on guinea-pig skinned tracheal muscle

    Directory of Open Access Journals (Sweden)

    Kayo Nemoto

    1999-01-01

    Full Text Available Although adrenergic β2-agonist-induced smooth muscle relaxation has been attributed to increased intracellular cyclic AMP (cAMP, a relaxation response has been observed at low β2-agonist concentrations that do not cause increased cAMP To elucidate the mechanism of tracheal muscle relaxation induced by low concentrations of β2-agonists, we used a guinea-pig skinned tracheal smooth muscle preparation to examine the effects on the contractile protein system. The isotonic contraction of β-escin-treated skinned tracheal muscle from guinea-pig was measured. When the intracellular Ca2+ concentration was maintained at 1 μmol/L in the presence of guanosine 5′-triphosphate (GTP; 100 μmol/L, neither isoproterenol (10nmol/L nor salbutamol (60 nmol/L affected Ca2+ sensitivity, but a significant decrease in Ca2+ sensitivity was observed in the presence of okadaic acid (1 μmol/L. The decrease in Ca2+ sensitivity was a slow response and was blocked by pretreatment with propranolol (1 μmol/L. Forskolin (1 μmol/L did not affect Ca2+ sensitivity. These results suggest that adrenergic b 2-agonists may activate protein phosphatase through an unknown pathway involving the β2-receptor, which enhances dephosphorylation of the myosin light chain and/or thin filament proteins, resulting in relaxation of the tracheal smooth muscle.

  3. Thyroidectomy improves tracheal anatomy and airflow in patients with nodular goiter. A prospective cohort study

    DEFF Research Database (Denmark)

    Sørensen, Jesper Roed; Lauridsen, Jeppe Killerich; Døssing, Helle

    . Effect sizes (ES) were calculated as mean change divided by standard deviation at baseline. ES of 0.2-0.5 were defined as small, 0.5-0.8 as moderate, and values >0.8 as large.Results: Sixty-five patients completed all examinations. Median goiter volume was 58 mL (range, 14-642 mL) before surgery...... resonance images (MRI) of the neck and respiratory flow-volume curves, including both in- and expiration, were performed prior to and six months following surgery. The evaluated tracheal dimensions included the smallest cross-sectional area of the trachea (SCAT), tracheal narrowing, and tracheal deviation...... with surgical removal of median 43 g (range, 8-607 mL). Six months post-surgery, tracheal narrowing and deviation were diminished by median 26% (ES=0.67), and 33% (ES=0.73), respectively, while SCAT increased by 17% (ES=0.61). Correspondingly, each 10% decrease in goiter volume resulted in an increase of 6...

  4. An approach to tracheostomy in a patient with an expandable metallic tracheal stent.

    Science.gov (United States)

    Madden, Brendan P; Sheth, Abhijat

    2005-09-01

    With increasing use of expandable metallic stents to manage patients with a variety of endobronchial pathologies, some will have a subsequent need for tracheostomy insertion. We describe a successful technique to insert a tracheostomy using rigid and fibre-optic bronchoscopy in a patient who had an 8 cm expandable metallic tracheal stent deployed previously on account of tracheomalacia.

  5. Subcellular trafficking of FGF controls tracheal invasion of Drosophila flight muscle.

    Science.gov (United States)

    Peterson, Soren J; Krasnow, Mark A

    2015-01-15

    To meet the extreme oxygen demand of insect flight muscle, tracheal (respiratory) tubes ramify not only on its surface, as in other tissues, but also within T-tubules and ultimately surrounding every mitochondrion. Although this remarkable physiological specialization has long been recognized, its cellular and molecular basis is unknown. Here, we show that Drosophila tracheoles invade flight muscle T-tubules through transient surface openings. Like other tracheal branching events, invasion requires the Branchless FGF pathway. However, localization of the FGF chemoattractant changes from all muscle membranes to T-tubules as invasion begins. Core regulators of epithelial basolateral membrane identity localize to T-tubules, and knockdown of AP-1γ, required for basolateral trafficking, redirects FGF from T-tubules to surface, increasing tracheal surface ramification and preventing invasion. We propose that tracheal invasion is controlled by an AP-1-dependent switch in FGF trafficking. Thus, subcellular targeting of a chemoattractant can direct outgrowth to specific domains, including inside the cell. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Management of dogs and cats with endotracheal tube tracheal foreign bodies

    Science.gov (United States)

    Nutt, Laura K.; Webb, Jinelle A.; Prosser, Kirsten J.; Defarges, Alice

    2014-01-01

    Two cats and 3 dogs were treated for an endotracheal tube tracheal foreign body (ETFB) during recovery from general anesthesia. Bronchoscopy was used to remove the ETFB. Animals were clinically normal at discharge. While rare, ETFB can occur upon recovery from anesthesia. Bronchoscopy is an effective way to remove ETFB. PMID:24891640

  7. Profiles in congenital heart disease

    International Nuclear Information System (INIS)

    Freed, M.D.; Keane, J.F.

    1986-01-01

    Pediatric cardiology has made great strides in the diagnosis, management, and correction of complex congenital malformations in the past two decades. The foundation of these advances is a more precise understanding of the physiology and anatomy of complex lesions that has been obtained from cardiac catheterization and angiography. The techniques for catheterization of infants and children have been discussed in another paper. This chapter focuses on brief profiles of some of the more important congenital abnormalities. The incidence cited in the discussion of each abnormality pertains to a population comprises of children and adults referred to The Children's Hospital Medical Center and Peter Bent Brigham Hospital, respectively, for evaluation of congenital heart disease

  8. Metallic stent implantation in the treatment of malignant tracheal stenosis under general anaesthesia

    International Nuclear Information System (INIS)

    Bai Xuming; Jin Yong; Xie Hong; Cheng Long; Gu Xingshi; Chang Liuhui

    2012-01-01

    Objective: To evaluate the safety and feasibility of the tracheal stent implantation for treatment of malignant tracheal stenosis under general anaesthesia. Methods: Eighteen patients with malignant tracheal stenosis underwent preoperative 64-slice spiral CT scan and airway reconstruction. The stenotic sites were located in main tracheal trunk (5 patients), in right main bronchus (1 patient), in trachea and left main bronchus (4 patients), in trachea and right main bronchus (3 patients), in main tracheal trunk and bilateral main bronchus (5 patients). The degree of stenosis was rated 51% to 70% in 7 cases, 71% to 90% in 11 cases. All patients, 17 patients of ASA grade Ⅳ and 1 patient of grade Ⅲ, presented with severe dyspnea. Under general anaesthesia, implantation of metallic stent was performed through the sputum aspiration hole of the connecting tubing with DSA guidance. The NBP, ECG, RR, SaO 2 of the patients were recorded and compared with t test during the entire procedure. At the end of the procedure, relief of dyspnea, complications related to anesthesia and operation were recorded. Results: The success rate of stent placement was 18/18, and dyspnea was significantly relived in all patients. Slightly bloody sputum occurred in 7 cases. The stent was obstructed by sputum in 1 case,and the patient was treated with medication. There were no severe complications. The operative course were rated 11 to 9 in 17 cases, and 6 to 8 in 1 case. All 18 patients were cooperative during the procedure. Sixteen patients rated the procedure as very comfortable and 2 rated the procedure as comfortable. Respiratory rate (RR) and heart rate (HR) decreased in all patients after the operation [(37.1 ± 2.8)/min and (106.5 ± 14.2) bpm before the operation respectively, (18.6 ± 1.4)/min and (73.2 ± 7.6) bpm after the operation respectively], t=17.81 and 3.80, P<0.01. Pulse oxygen saturation (SaO 2 ) during the operation [(91.2 ± 1.8)%]increased [(76.3 ± 8.6 )% before the

  9. Congenital pyriform aperture stenosis

    International Nuclear Information System (INIS)

    Osovsky, Micky; Aizer-Danon, Anat; Horev, Gadi; Sirota, Lea

    2007-01-01

    Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis. (orig.)

  10. Characterizing congenital amusia.

    Science.gov (United States)

    Stewart, Lauren

    2011-04-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of music and other domains, such as language. The present article considers findings concerning pitch discrimination, pitch memory, contour processing, experiential aspects of music listening in amusia, and emerging evidence concerning the neurobiology of the disorder. A simplified model of melodic processing is outlined, and possible loci of the cognitive deficit are discussed.

  11. Neurobiology of Congenital Amusia.

    Science.gov (United States)

    Peretz, Isabelle

    2016-11-01

    The past decade of research has provided compelling evidence that musical engagement is a fundamental human trait, and its biological basis is increasingly scrutinized. In this endeavor, the detailed study of individuals who have musical deficiencies is instructive because of likely neurogenetic underpinnings. Such individuals have 'congenital amusia', an umbrella term for lifelong musical disabilities that cannot be attributed to intellectual disability, lack of exposure, or brain damage after birth. Key points are reviewed here that have emerged during recent years regarding the neurobiology of the disorder, focusing on the importance of recurrent processing between the right inferior frontal cortex and the auditory cortex for conscious monitoring of musical pitch, and how this relates to developmental cognitive disorders in general. Copyright © 2016 Elsevier Ltd. All rights reserved.

  12. Congenital sensorineural hearing loss

    International Nuclear Information System (INIS)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-01-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems

  13. Congenital sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  14. An unusual salvage technique for posterior tracheal membranous laceration associated with transhiatal esophagectomy: A transcervical–transsternal approach

    Directory of Open Access Journals (Sweden)

    Seyed Ziaeddin Rasihashemi

    2017-09-01

    Full Text Available Various surgical approaches may be employed for esophageal resection. Major airway injuries due to transhiatal esophagectomy include vertical tears in the membranous trachea. Tracheal injury is an uncommon but potentially fatal complication. This article describes the technique to repair the posterior membranous tracheal tear, extended just over the carina through a transcervical–transsternal approach, thereby avoiding a second thoracotomy. Six patients with posterior membranous tracheal injury underwent this procedure. The laceration ranged from 3 cm to 5 cm in length. Four patients had received neoadjuvant chemoradiation. The management of tracheal laceration added approximately 60 minutes to the total operation time. There was no mortality related to tracheal injury. Patients were followed up for 6 months after surgery, and both posterior tracheal wall and transverse tracheotomy remained intact without stenosis. The transcervical–transsternal approach decreases the need of thoracotomy and its complications in patients with tracheal laceration in any stage, even in cases of an extended tear down to the carina.

  15. Resection and anastomosis for benign tracheal stenosis: Single institution experience of 18 cases.

    Science.gov (United States)

    Kumar, Arvind; Asaf, Belal Bin; Puri, Harsh Vardhan; Abdellateef, Amr

    2017-01-01

    Tracheal stenosis is a complex condition caused by altered inflammatory response to injury and subsequent excessive circumferential scar formation. Surgical resection, wherever possible, offers the best long-term results. Nonsurgical methods provide immediate relief to all can be curative in few but mostly serve as an excellent bridge to surgery in majority. The purpose of this study is to retrospectively evaluate the outcome following surgery for benign tracheal stenosis at our center. This retrospective analysis was conducted on 18 patients who underwent resection and anastomosis for tracheal stenosis at our center between March 2012 and December 2015. Their records were analyzed for demography, history, clinical presentation, computed tomography, bronchoscopy details, preoperative interventions, indications for and details of surgery, the procedure performed, postoperative complications, and course during 6 months follow-up. The patients had a varied list of pathologies for which they were either intubated or tracheostomized. The length of stenosis ranged between 1 cm and 4 cm. The diameter of stenotic segment ranged between 0 mm and 10 mm. Average length of resected segment was 3 cm, and number of tracheal rings resected ranged from 2 to 9. Postoperative complications occurred in four patients (22.22%). All our patients were in the "excellent outcome" category at discharge as well as at 3 months follow-up. Surgical management of tracheal stenosis is challenging and requires multidisciplinary team approach. Thorough preoperative preparation and multidisciplinary planning regarding need for and timing of surgery, meticulous intraoperative technique, and aggressive postoperative care is key to successful surgery, which can provide long-lasting cure to these patients.

  16. Resection and anastomosis for benign tracheal stenosis: Single institution experience of 18 cases

    Directory of Open Access Journals (Sweden)

    Arvind Kumar

    2017-01-01

    Full Text Available Introduction: Tracheal stenosis is a complex condition caused by altered inflammatory response to injury and subsequent excessive circumferential scar formation. Surgical resection, wherever possible, offers the best long-term results. Nonsurgical methods provide immediate relief to all can be curative in few but mostly serve as an excellent bridge to surgery in majority. The purpose of this study is to retrospectively evaluate the outcome following surgery for benign tracheal stenosis at our center. Materials and Methods: This retrospective analysis was conducted on 18 patients who underwent resection and anastomosis for tracheal stenosis at our center between March 2012 and December 2015. Their records were analyzed for demography, history, clinical presentation, computed tomography, bronchoscopy details, preoperative interventions, indications for and details of surgery, the procedure performed, postoperative complications, and course during 6 months follow-up. Results: The patients had a varied list of pathologies for which they were either intubated or tracheostomized. The length of stenosis ranged between 1 cm and 4 cm. The diameter of stenotic segment ranged between 0 mm and 10 mm. Average length of resected segment was 3 cm, and number of tracheal rings resected ranged from 2 to 9. Postoperative complications occurred in four patients (22.22%. All our patients were in the “excellent outcome” category at discharge as well as at 3 months follow-up. Conclusions: Surgical management of tracheal stenosis is challenging and requires multidisciplinary team approach. Thorough preoperative preparation and multidisciplinary planning regarding need for and timing of surgery, meticulous intraoperative technique, and aggressive postoperative care is key to successful surgery, which can provide long-lasting cure to these patients.

  17. Cellular distribution and function of ion channels involved in transport processes in rat tracheal epithelium.

    Science.gov (United States)

    Hahn, Anne; Faulhaber, Johannes; Srisawang, Lalita; Stortz, Andreas; Salomon, Johanna J; Mall, Marcus A; Frings, Stephan; Möhrlen, Frank

    2017-06-01

    Transport of water and electrolytes in airway epithelia involves chloride-selective ion channels, which are controlled either by cytosolic Ca 2+ or by cAMP The contributions of the two pathways to chloride transport differ among vertebrate species. Because rats are becoming more important as animal model for cystic fibrosis, we have examined how Ca 2+ - dependent and cAMP- dependent Cl - secretion is organized in the rat tracheal epithelium. We examined the expression of the Ca 2+ -gated Cl - channel anoctamin 1 (ANO1), the cystic fibrosis transmembrane conductance regulator (CFTR) Cl - channel, the epithelial Na + channel ENaC, and the water channel aquaporin 5 (AQP5) in rat tracheal epithelium. The contribution of ANO1 channels to nucleotide-stimulated Cl - secretion was determined using the channel blocker Ani9 in short-circuit current recordings obtained from primary cultures of rat tracheal epithelial cells in Ussing chambers. We found that ANO1, CFTR and AQP5 proteins were expressed in nonciliated cells of the tracheal epithelium, whereas ENaC was expressed in ciliated cells. Among nonciliated cells, ANO1 occurred together with CFTR and Muc5b and, in addition, in a different cell type without CFTR and Muc5b. Bioelectrical studies with the ANO1-blocker Ani9 indicated that ANO1 mediated the secretory response to the nucleotide uridine-5'-triphosphate. Our data demonstrate that, in rat tracheal epithelium, Cl - secretion and Na + absorption are routed through different cell types, and that ANO1 channels form the molecular basis of Ca 2+ -dependent Cl - secretion in this tissue. These characteristic features of Cl - -dependent secretion reveal similarities and distinct differences to secretory processes in human airways. © 2017 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.

  18. Increase in tracheal investment with beetle size supports hypothesis of oxygen limitation on insect gigantism.

    Science.gov (United States)

    Kaiser, Alexander; Klok, C Jaco; Socha, John J; Lee, Wah-Keat; Quinlan, Michael C; Harrison, Jon F

    2007-08-07

    Recent studies have suggested that Paleozoic hyperoxia enabled animal gigantism, and the subsequent hypoxia drove a reduction in animal size. This evolutionary hypothesis depends on the argument that gas exchange in many invertebrates and skin-breathing vertebrates becomes compromised at large sizes because of distance effects on diffusion. In contrast to vertebrates, which use respiratory and circulatory systems in series, gas exchange in insects is almost exclusively determined by the tracheal system, providing a particularly suitable model to investigate possible limitations of oxygen delivery on size. In this study, we used synchrotron x-ray phase-contrast imaging to visualize the tracheal system and quantify its dimensions in four species of darkling beetles varying in mass by 3 orders of magnitude. We document that, in striking contrast to the pattern observed in vertebrates, larger insects devote a greater fraction of their body to the respiratory system, as tracheal volume scaled with mass1.29. The trend is greatest in the legs; the cross-sectional area of the trachea penetrating the leg orifice scaled with mass1.02, whereas the cross-sectional area of the leg orifice scaled with mass0.77. These trends suggest the space available for tracheae within the leg may ultimately limit the maximum size of extant beetles. Because the size of the tracheal system can be reduced when oxygen supply is increased, hyperoxia, as occurred during late Carboniferous and early Permian, may have facilitated the evolution of giant insects by allowing limbs to reach larger sizes before the tracheal system became limited by spatial constraints.

  19. Use of the silicone tracheal T-tube for tracheostenosis or tracheomalacia.

    Science.gov (United States)

    Liu, H C; Wang, L S; Fahn, H J; Lee, Y C; Lu, C C; Chan, K H; Huang, M H

    1996-09-01

    Tracheobtenosib and tracheomalacia are trivial diseases. The conventional choice of managements with tracheostomy, either temporary or long-term usage, can only partially resolve the problems of airway obstruction. Silicone tracheal T-tube presents a substitute for it. We present 5 patients with tracheostenosis or tracheomalacia managed with nine procedures of long silicone Montgomery T-tube prothesis between 1984 and 1994 in VGH-Taipei. The primary diagnosis included tracheal injury (2), postintubation tracheal stenosis (2), and stenosis due to endotracheal tuberculosis (1). Three patients received a long segmental T-tube for permanent endotracheal stenting and the other two patients used T-tube insertion for temporary stenting of the trachea for 7 and 11 months, respectively, with satisfactory results. All patients got immediate benefit from the prothesis in respiration with simple postoperative care. Two patients with temporary T-tube placement had it successfully removed in 7 and 11 months, respectively. Placement of the T-tube for subglottic stenosis also protected the function of phonation. The tracheal T-tube restored airway patency reliably with good long-term results and could be the preferred management of chronic upper airway obstructive disease not amenable to surgical repair. The most common complication was airway obstruction caused by either granulations or sticky mucoid substance. Three patients and six tubes (60%) developed granulation obstruction and the average duration of granuloma formation was 7.7 months. Laser phototherapy or surgical intervention, such as tracheoplasty, with change of the T-tube was carried out for granuloma obstruction. T-tube is a good endoprothesis for tracheostenosis and tracheomalacia with minimal complication for cases of long tracheostenosis or complex tracheal injury.

  20. Tracheal and laryngeal tumors in the dog and cat: literature review and 13 additional patients

    International Nuclear Information System (INIS)

    Carlisle, C.H.; Biery, D.N.; Thrall, D.E.

    1991-01-01

    Primary tumors of the larynx or trachea are uncommon in the dog and cat. In a review of the English language literature, description of 65 such patients were found. In a search of the Veterinary Teaching Hospitals of the University of Pennsylvania and North Carolina State University, an additional 13 previously unreported patients were identified, bringing the total to at least 78. Of these 78, there have been 16 canine tracheal, 7 feline tracheal, 34 canine laryngeal and 21 feline laryngeal tumors. In the canine and feline trachea, osteochondroma and epithelial malignancies, respectively, appear to be the most common. Epithelial malignancies appear to be the most common tumor of the canine larynx whereas lymphosarcoma appears to be the most common feline laryngeal tumor. In patients described herein, tumors produced clinical signs consistent with airway obstruction. Voice alteration was common in patients with laryngeal tumors. Patients were middle-aged to older, except for dogs with osteochondroma. This compares favorably to historical data. All tumors in this study were readily seen radiographically, with most laryngeal and tracheal tumors appearing as masses within the lumen of the airway. Mineralization was uncommon except for canine osteochondromas. Feline laryngeal tumors in this study appeared as generalized laryngeal thickening rather than as a distinct mass. Response of canine and feline tracheal and laryngeal thickening rather than as a distinct mass. Response of canine and feline tracheal and laryngeal tumors to treatment can not be adequately assessed from available data. Benign tumors of the larynx or trachea may be amenable to complete excision. Neoplastic lesions must be differentiated from polyps or abscesses within the upper airway as these may appear radiographically identical to primary tumors. This can be achieved by endoscopic evaluation and biopsy of airway masses before formulating a prognosis

  1. Tracheal morphology and collapse in COPD: Correlation with CT indices and pulmonary function test

    International Nuclear Information System (INIS)

    Lee, Hyun Joo; Seo, Joon Beom; Chae, Eun Jin; Kim, Namkug; Lee, Choong Wook; Oh, Yeon-Mok; Lee, Sang Do

    2011-01-01

    Objectives: To assess the tracheal morphologic changes using CT in COPD (chronic obstructive lung disease) patients and to assess correlation between them and PFT (pulmonary function test) and CT parameters. Materials and methods: Ninety-two healthy individuals and 115 patients with COPD who underwent volumetric inspiration/expiration CT scanning were included. The Lsag, Lcor, and the tLA were measured. The TI was defined as the ratio of Lcor/Lsag. The tracheal morphologic changes (tLA, TI, and collapsibility) were compared in healthy individuals and COPD. In COPD patients, correlation of the tracheal morphologic change with PFT and CT parameters was assessed. Results: The TIs in the COPD patients were significantly lower than those in the control group (0.80 ± 0.15 vs. 0.88 ± 0.11, Mean ± SD) (p 1 (r = 0.29, p = 1 /FVC (r = 0.26, p < 0.01), and the GOLD stage (r = −0.26, p < 0.01). TI showed significant correlation with EI on both inspiration and expiration CT (r = −0.19, p = 0.04 and r = −0.23, p = 0.02), MLD on expiration CT (r = 0.27, p < 0.01), CT–ATI (r = 0.34, p < 0.01), and with LV on expiration CT (r = −0.25, p < 0.01). Conclusion: Among the tracheal morphologic changes, the most significant change in COPD patients compared with that in the control group, was the TI. In COPD patients, the tracheal morphologic change showed clinically significant correlation with severity of emphysema and CT indices.

  2. Evidence that CFTR is expressed in rat tracheal smooth muscle cells and contributes to bronchodilation

    Directory of Open Access Journals (Sweden)

    Mettey Yvette

    2006-08-01

    Full Text Available Abstract Background The airway functions are profoundly affected in many diseases including asthma, chronic obstructive pulmonary disease (COPD and cystic fibrosis (CF. CF the most common lethal autosomal recessive genetic disease is caused by mutations of the CFTR gene, which normally encodes a multifunctional and integral membrane protein, the CF transmembrane conductance regulator (CFTR expressed in airway epithelial cells. Methods To demonstrate that CFTR is also expressed in tracheal smooth muscle cells (TSMC, we used iodide efflux assay to analyse the chloride transports in organ culture of rat TSMC, immunofluorescence study to localize CFTR proteins and isometric contraction measurement on isolated tracheal rings to observe the implication of CFTR in the bronchodilation. Results We characterized three different pathways stimulated by the cAMP agonist forskolin and the isoflavone agent genistein, by the calcium ionophore A23187 and by hypo-osmotic challenge. The pharmacology of the cAMP-dependent iodide efflux was investigated in detail. We demonstrated in rat TSMC that it is remarkably similar to that of the epithelial CFTR, both for activation (using three benzo [c]quinolizinium derivatives and for inhibition (glibenclamide, DPC and CFTRinh-172. Using rat tracheal rings, we observed that the activation of CFTR by benzoquinolizinium derivatives in TSMC leads to CFTRinh-172-sensitive bronchodilation after constriction with carbachol. An immunolocalisation study confirmed expression of CFTR in tracheal myocytes. Conclusion Altogether, these observations revealed that CFTR in the airways of rat is expressed not only in the epithelial cells but also in tracheal smooth muscle cells leading to the hypothesis that this ionic channel could contribute to bronchodilation.

  3. Penetrating trauma to the kidney and Meckel’s Diverticulum in a patient with unilateral renal agenesis

    Directory of Open Access Journals (Sweden)

    Sanju Sobnach

    2015-01-01

    Conclusion: Although congenital visceral anomalies are spectacular findings at laparotomy, they should not distract the trauma surgeon. Adhering to damage control surgery principles and careful inspection of the peritoneal cavity for further abnormalities remain the mainstay of successful management.

  4. Recurrent airway obstructions in a patient with benign tracheal stenosis and a silicone airway stent: a case report

    Science.gov (United States)

    Sriram, KB; Robinson, PC

    2008-01-01

    Airway stents (silicone and metal stents) are used to treat patients with benign tracheal stenosis, who are symptomatic and in whom tracheal surgical reconstruction has failed or is not appropriate. However airway stents are often associated with complications such as migration, granuloma formation and mucous hypersecretion, which cause significant morbidity, especially in patients with benign tracheal stenosis and relatively normal life expectancy. We report a patient who had frequent critical airway obstructions over 8 years due to granuloma and mucus hypersecretion in a silicone airway stent. The problem was resolved when the silicone stent was removed and replaced with a covered self expanding metal stent. PMID:18840299

  5. Congenital broncho-oesophageal fistula

    African Journals Online (AJOL)

    1983-04-09

    Apr 9, 1983 ... Rigid bronchoscopy performed under general anaesthesia .... Blackburn WR, Armour)' RA. Congenital esophago-pulmonary fistulas without esophageal atresia: an analysis of 260 fistulas in infants, children and adults.

  6. Congenital heart defect corrective surgeries

    Science.gov (United States)

    ... make it bigger with a patch made of Gore-tex, a man-made (synthetic) material. Another way ... 434. Bhatt AB, Foster E, Kuehl K, et al; American Heart Association Council on Clinical Cardiology. Congenital ...

  7. Congenital Heart Defects (For Parents)

    Science.gov (United States)

    ... to be associated with genetic disorders, such as Down syndrome . But the cause of most congenital heart defects isn't known. While they can't be prevented, many treatments are available for the defects and related health ...

  8. Genetics Home Reference: congenital hyperinsulinism

    Science.gov (United States)

    ... Topic: Hypoglycemia Health Topic: Metabolic Disorders Genetic and Rare Diseases Information Center (1 link) Congenital hyperinsulinism Educational Resources (7 links) Boston Children's Hospital: Hypoglycemia and Low Blood Sugar in Children Cook Children's Hospital (PDF) Disease InfoSearch: ...

  9. CHRNE Mutation and Congenital Myasthenia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2009-01-01

    Full Text Available The CHRNE e1293insG mutation was identified in 14 (60% of 23 North African families with an early onset form of congenital myasthenic syndrome studied at centers in France, Tunisia, Algeria, and UK.

  10. Congenital and perinatal cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Chun Soo Kim

    2010-01-01

    Full Text Available Cytomegalovirus (CMV is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID, which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS. Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at 72?#608;for 5 seconds can eliminate CMV completely.

  11. Congenital malformations of the supratentorial brain. Pt. 2. Disorders of the corpus callosum and holoprosencephalies; Kongenitale Malformationen des Grosshirns. Teil 2: Entwicklungsstoerungen des Balkens und Holoprosenzephalien

    Energy Technology Data Exchange (ETDEWEB)

    Rummeny, C. [Institut fuer Klinische Radiologie, Klinikum der Universitaet Muenchen, Grosshadern, Muenchen (Germany); Institut fuer klinische Radiologie, Klinikum der Universitaet Muenchen, Grosshadern, Marchioninistrasse 15, 81377, Muenchen (Germany); Ertl-Wagner, B.; Reiser, M.F. [Institut fuer Klinische Radiologie, Klinikum der Universitaet Muenchen, Grosshadern, Muenchen (Germany)

    2003-11-01

    The corpus callosum is formed between the 7th and the 20th gestational week. If this process is disrupted, partial or complete callosal agenesis may ensue. As large parts of the supra- and infratentorial brain are created druing this critical period, associated anomalies need always to be searched for when callosal agenesis is present. Associations with neuro-genetic syndromes also exist. The corpus callosum is generally formed from front to back (''front-to-back rule''). Therefore, a partial callosal agenesis usually involves the posterior portion of the corpus callosum, while a secondary lesion of the corpus callosum does not follow this rule. Holoprosencephalies are a notable exception to this rule, as the frontal part of the corpus callosum is absent in spite of their classification as congenital malformations. They represent a disturbance of the differentiation and cleavage of the prosencephalon with a disruption of the separation of the cerebral hemispheres. Holoprosencephalies can be due to genetic causes, but also to intrauterine infections or other teratogenic causes. The holoprosencephalies are subdivided into alobar, semilobar and lobar holoprosencephalies. This article aims to describe the most important features of callosal agenesis and holoprosencephalies highlighting the respective imaging characteristics. (orig.) [German] Die Anlage des Balkens findet in der 7.-20. Woche nach Gestation statt. Stoerungen in dieser Entwicklungsphase koennen zu einer Balkenagenesie oder zu einer Balkenhypogenesie fuehren. Da in diesem Entwicklungsstadium grosse Anteile des Gross- und Kleinhirns angelegt werden, muss immer nach assoziierten Anlagestoerungen gesucht werden. Ebenso treten auch Kombinationen mit genetisch bedingten Syndromen auf. Die Entwicklung des Balkens folgt der sog. anterioren-posterioren Sequenz (''front-to-back rule''). Sekundaere Schaedigungen bei der Balkenentwicklung folgen dieser Regel nicht, und lassen

  12. Biallelic PMS2 Mutation and Heterozygous DICER1 Mutation Presenting as Constitutional Mismatch Repair Deficiency With Corpus Callosum Agenesis: Case Report and Review of Literature.

    Science.gov (United States)

    Cheyuo, Cletus; Radwan, Walid; Ahn, Janice; Gyure, Kymberly; Qaiser, Rabia; Tomboc, Patrick

    2017-10-01

    Constitutional mismatch repair deficiency syndrome is a cancer predisposition syndrome caused by autosomal recessive biallelic (homozygous) germline mutations in the mismatch repair genes (MLH1, MSH2, MSH6, and PMS2). The clinical spectrum includes neoplastic and non-neoplastic manifestations. We present the case of a 7-year-old boy who presented with T-lymphoblastic lymphoma and glioblastoma, together with non-neoplastic manifestations including corpus callosum agenesis, arachnoid cyst, developmental venous anomaly, and hydrocephalus. Gene mutation analysis revealed pathogenic biallelic mutations of PMS2 and heterozygous DICER1 variant predicted to be pathogenic. This report is the first to allude to a possible interaction of the mismatch repair system with DICER1 to cause corpus callosum agenesis.

  13. Mayer–Rokitansky–Kuster–Hauser syndrome: Syndrome of Mullerian agenesis – A report of two cases

    Directory of Open Access Journals (Sweden)

    Sushma Yalavarthi

    2017-01-01

    Full Text Available The Mayer–Rokitansky–Kuster–Hauser syndrome (MRKH syndrome, simply called Rokitansky syndrome or vaginal aplasia of the uterus, is a congenital condition that is characterized by the absence of the uterus and vagina, but ovaries are present and the external genitalia are normal. It affects at least 1 out of 4500 women. MRKH may be isolated (Type I, but it is more frequently associated with renal, vertebral, and to a lesser extent, auditory and cardiac defects (MRKH Type II or Mullerian duct aplasia, Renal dysplasia, and Cervical Somite anomalies association - mullerian duct aplasia, renal dysplasia, and cervical somite anomalies. There were very few cases of MRKH syndrome reported in the literature. Here, we report two cases of MRKH syndrome, one in a 20-year-old woman who presented with primary amenorrhea (MRKH Type I and the other in a 65-year-old woman with primary amenorrhea and associated renal malformations and a rare ovarian sertoliform variant of endometrioid tumor (MRKH Type II.

  14. Surgical-orthodontic treatment of Class III malocclusion with agenesis of lateral incisor and unerupted canine

    Directory of Open Access Journals (Sweden)

    Bruno Boaventura Vieira

    2013-06-01

    Full Text Available INTRODUCTION: Orthodontic-surgical treatment was performed in patient with skeletal Class III malocclusion due to exceeding mandibular growth. Patient also presented upper and lower dental protrusion, overjet of -3.0 mm, overbite of -1.0 mm, congenital absence of tooth #22, teeth #13 and supernumerary impaction, tooth #12 with conoid shape and partly erupted in supraversion, prolonged retention of tooth #53, tendency to vertical growth of the face and facial asymmetry. The discrepancy on the upper arch was -2.0 mm and -5.0 mm on the lower arch. METHODS: The pre-surgical orthodontic treatment was performed with extractions of the teeth #35 and #45. On the upper arch, teeth #53, #12 and supernumerary were extracted to accomplish the traction of the impacted canine. The spaces of the lower extractions were closed with mesialization of posterior segment. After aligning and leveling the teeth, extractions spaces closure and correct positioning of teeth on the bone bases, the correct intercuspation of the dental arch, with molars and canines in Angle's Class I, coincident midline, normal overjet and overbite and ideal torques, were evaluated through study models. The patient was submitted to orthognathic surgery and then the post-surgical orthodontic treatment was finished. RESULTS: The Class III malocclusion was treated establishing occlusal and facial normal standards.

  15. Coffin-Siris syndrome with multiple congenital malformations and intrauterine death: towards a better delineation of the severe end of the spectrum.

    Science.gov (United States)

    Coulibaly, Béma; Sigaudy, Sabine; Girard, Nadine; Popovici, Cornel; Missirian, Chantal; Heckenroth, Hélène; Tasei, Anne-Marie; Fernandez, Carla

    2010-01-01

    Coffine-Siris syndrome or "fifth digit" syndrome is a multiple congenital anomaly-mental retardation syndrome with severe developmental delay, coarse facial features, hirsutism and absent fifth fingernails or toenails or fifth distal phalanges. The etiology of this syndrome remains uncertain. Here we report a stillborn male baby born from consanguineous parents who might represent a very severe form of Coffine-Siris syndrome with cardiac defect and multiple brain malformations including corpus callosum agenesis and Dandy Walker malformation. To the best of our knowledge, it is the first case leading to intrauterine death. Karyotype and array comparative genomic hybridization were normal; these results give additional support to mendelian inheritance for this syndrome. In our family, the most likely mode of inheritance is autosomal recessive and the recurrence is probably as high as 25%. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  16. Congenital abnormality of the inner ear and internal auditory canal in a patient with deep sensorineural hearing loss: A case report

    International Nuclear Information System (INIS)

    Rivera, Diego; Saab, Said; Cordoba, Claudia; Montes, Guillermo; Barreto, Tatiana

    2010-01-01

    CT and MRI are complementary studies that have proven to be the best radiological tools in screening of children with unilateral or bilateral sensorineural hearing loss. Only about a 20% of the patients with congenital sensor neural hearing loss have manifestations in images. Due to the fact that most of these manifestations initiate in the bone, the CT is the first line of image study. MRI is indicated in the evaluation of suspected agenesis, neuropathy, aplasia or hypoplasia of the vestibulocochlear nerve, often associated with this type of hearing loss. We present a case of a 6 year old patient with deep bilateral sensor neural hearing loss, with radiological studies to determine a potential candidate for a cochlear or auditory brainstem implant as hearing rehabilitation.

  17. A 54 Mb 11qter duplication and 0.9 Mb 1q44 deletion in a child with laryngomalacia and agenesis of corpus callosum

    Directory of Open Access Journals (Sweden)

    Lall Meena

    2011-09-01

    Full Text Available Abstract Background Partial Trisomy 11q syndrome (or Duplication 11q has defined clinical features and is documented as a rare syndrome by National Organization of Rare Disorders (NORD. Deletion 1q44 (or Monosomy 1q44 is a well-defined syndrome, but there is controversy about the genes lying in 1q44 region, responsible for agenesis of the corpus callosum. We report a female child with the rare Partial Trisomy 11q syndrome and Deletion 1q44 syndrome. The genomic imbalance in the proband was used for molecular characterization of the critical genes in 1q44 region for agenesis of corpus callosum. Some genes in 11q14q25 may be responsible for laryngomalacia. Results We report a female child with dysmorphic features, microcephaly, growth retardation, seizures, acyanotic heart disease, and hand and foot deformities. She had agenesis of corpus callosum, laryngomalacia, anterior ectopic anus, esophageal reflux and respiratory distress. Chromosome analysis revealed a derivative chromosome 1. Her karyotype was 46,XX,der(1t(1;11(q44;q14pat. The mother had a normal karyotype and the karyotype of the father was 46,XY,t(1;11(q44;q14. SNP array analysis showed that the proband had a 54 Mb duplication of 11q14q25 and a 0.9 Mb deletion of the submicroscopic subtelomeric 1q44 region. Fluorescence Insitu Hybridisation confirmed the duplication of 11qter and deletion of 1qter. Conclusion Laryngomalacia or obstruction of the upper airway is the outcome of increased dosage of some genes due to Partial Trisomy 11q Syndrome. In association with other phenotypic features, agenesis of corpus callosum appears to be a landmark phenotype for Deletion 1q44 syndrome, the critical genes lying proximal to SMYD3 in 1q44 region.

  18. Trypsin as enhancement in cyclical tracheal decellularization: Morphological and biophysical characterization

    Energy Technology Data Exchange (ETDEWEB)

    Giraldo-Gomez, D.M., E-mail: davidmauro2008@gmail.com [Posgrado en Ciencia e Ingeniería de Materiales, Universidad Nacional Autónoma de México (UNAM), Unidad de Posgrado Edificio “C” 1er Piso, Circuito de Posgrados, Avenida Universidad 3000, Ciudad Universitaria, Coyoacán, C.P. 04510, México D. F., México (Mexico); Instituto de Investigaciones en Materiales, Universidad Nacional Autónoma de México (UNAM), Circuito Exterior, Avenida Universidad 3000, Ciudad Universitaria, Coyoacán, C.P. 04510, México D.F., México (Mexico); Leon-Mancilla, B. [Departamento de Cirugía, Facultad de Medicina, Universidad Nacional Autónoma de México (UNAM), Edificio “D” Planta Baja, Circuito Interior, Avenida Universidad 3000, Ciudad Universitaria, Coyoacán, C.P. 04510, México D.F., México (Mexico); Del Prado-Audelo, M.L. [Instituto de Investigaciones en Materiales, Universidad Nacional Autónoma de México (UNAM), Circuito Exterior, Avenida Universidad 3000, Ciudad Universitaria, Coyoacán, C.P. 04510, México D.F., México (Mexico); and others

    2016-02-01

    There are different types of tracheal disorders (e.g. cancer, stenosis and fractures). These can cause respiratory failure and lead to death of patients. Several attempts have been made for trachea replacement in order to restore the airway, including anastomosis and implants made from synthetic or natural materials. Tracheal allotransplantation has shown high rejection rates, and decellularization has emerged as a possible solution. Decellularization involves the removal of antigens from cells in the organ or tissue, leaving a matrix that can be used as 3D cell-scaffold. Although this process has been used for tracheal replacement, it usually takes at least two months and time is critical for patients with tracheal disorders. Therefore, there is necessary to develop a tracheal replacement process, which is not only effective, but also quick to prepare. The aim of this research was to develop a faster trachea decellularization protocol using Trypsin enzyme and Ethylenediaminetetraacetic acid (EDTA) as decellularization agents. Three protocols of cyclic trachea decellularization (Protocols A, B, and C) were compared. Following Protocol A (previously described in the literature), 15 consecutive cycles were performed over 32 days. Protocol B (a variation of Protocol A) — EDTA being added — with 15 consecutive cycles performed over 60 days. Finally, Protocol C, with the addition of Trypsin as a decellularization agent, 5 consecutive cycles being performed over 10 days. For the three protocols, hematoxylin–eosin (H&E) staining and DNA residual content quantification were performed to establish the effectiveness of the decellularization process. Scanning Electron Microscopy (SEM) was used to observe the changes in porosity and microarrays. To evaluate the structural matrices integrity, Thermogravimetric Analysis (TGA) and biomechanical test were used. None of the protocols showed significant alteration or degradation in the components of the extracellular matrix

  19. Avoidance versus use of neuromuscular blocking agents for improving conditions during tracheal intubation or direct laryngoscopy in adults and adolescents

    DEFF Research Database (Denmark)

    Lundstrøm, Lars Hyldborg; Duez, Christophe Hv; Nørskov, Anders Kehlet

    2017-01-01

    ) for adults and adolescents allocated to tracheal intubation with direct laryngoscopy. To look at various outcomes, conduct subgroup and sensitivity analyses, examine the role of bias, and apply trial sequential analysis (TSA) to examine the level of available evidence for this intervention. SEARCH METHODS......: We searched CENTRAL, MEDLINE, Embase, BIOSIS, International Web of Science, LILACS, advanced Google, CINAHL, and the following trial registries: Current Controlled Trials; ClinicalTrials.gov; and www.centerwatch.com, up to January 2017. We checked the reference lists of included trials and reviews...... the best conditions for tracheal intubation and may reduce the risk of upper airway discomfort or injury following tracheal intubation. Study results were characterized by indirectness, heterogeneity, and high or uncertain risk of bias concerning our primary outcome describing difficult tracheal intubation...

  20. Congenital Short QT Syndrome

    Directory of Open Access Journals (Sweden)

    Charles Antzelevitch

    2004-04-01

    Full Text Available Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms.2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death.