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Sample records for meningocele

  1. Meningocele repair

    Science.gov (United States)

    ... is surgery to repair birth defects of the spine and spinal membranes. Meningocele and myelomeningocele ... is covered by a sterile dressing. Your child may then be transferred to a neonatal intensive ...

  2. Optic Nerve Sheath Meningocele: A Case Report

    Science.gov (United States)

    Halimi, E.; Wavreille, O.; Rosenberg, R.; Bouacha, I.; Lejeune, J.-P.; Defoort-Dhellemmes, S.

    2013-01-01

    Abstract Isolated optic nerve sheath meningocele is a rare affection defined as the cystic enlargement of the optic nerve sheath filled with cerebrospinal fluid. We report the case of a 39-year-old woman presenting with bilateral meningocele uncovered during a routine examination for headache complaints. A 5-year follow-up validated the lesion’s clinical and imaging stability. Magnetic resonance imaging (MRI) is an essential tool in the diagnosis of this pathology, alongside characteristic symptoms indicating that the meningocele might have progressively expanded into the orbit. In this case we present a therapeutic approach based on pathophysiological hypotheses and review of the literature. PMID:28163760

  3. Surgical and pathological findings of meningocele manque

    International Nuclear Information System (INIS)

    Nishimoto, Hiroshi; Kurihara, Jun; Kishimoto, Hiroshi; Takezawa, Youko

    2006-01-01

    We reviewed the neuroradiological presentation and long-term outcomes in patients with meningocele manque. From April 1983 through March 2005, 15 children (7 boys and 8 girls) aged 12 days to 1 year underwent surgical exploration for suspected occult spinal dysraphism and were found to have manifestations of meningocele manque and other anomalies. All patients had cutaneous manifestations (small skin defects, hemangioma, and vestigial tails). No patient had abnormal neurological or urological findings other than skin stigmata. Spinal ultrasonography or magnetic resonance performed before surgery showed low-lying spinal conus, intrathecal dorsal bands, or subcutaneous tracts. These bands were confirmed at surgery in all patients. Histologic and immunohistochemical studies of these bands showed peripheral nerves, ganglia, collagen fibers, muscle fibers, and small vessels. These findings suggest that the bands were of hamartomatous origin. Sectioning of dorsal tethering bands of meningocele manque provided good long-term results. These dorsal tethering bands should be sought in the evaluation of patients with occult spinal dysraphism and surgically transected. (author)

  4. Surgical and pathological findings of meningocele manque

    International Nuclear Information System (INIS)

    Nishimoto, Hiroshi; Kurihara, Jun

    2006-01-01

    We review and describe the neuroradiological presentation and long-term outcomes in patients with meningocele manque. From April 1983 through March 2005, 15 children (7 boys and 8 girls) aged 12 days to 1 year underwent surgical exploration for suspected occult spinal dysraphism and were found to have manifestations of meningocele manque and other anomalies. All patients had cutaneous manifestations (small skin defect, hemangioma and human tails). No patient had abnormal neurological or urological findings other than skin stigmata. Spinal ultrasonography or MRI performed preoperatively showed low-lying spinal conus, intrathecal dorsal bands or subcutaneous tracts. These bands were confirmed at surgery in all patients. Histology and immunohistochemistry of these bands shows peripheral nerves, ganglions, collagen fibers, muscle fibers and small vessels. These findings suggested that these bands had hamartomatous origins. Sectioning of dorsal tethering bands of meningocele manque had good long-term results. These dorsal tethering bands should be sought in the evaluation of patients with occult spinal dysraphism and surgically transected. (author)

  5. Thoracic meningocele, non-associated with neurofibromatosis: a case report

    International Nuclear Information System (INIS)

    Abdala, N.; Nalli, D.R.; Carrete Junior, H.; Rodrigues, W.M.; Nogueira, R.G.; Carri, J.M.

    1993-01-01

    A case of thoracic meningocele, not associated with neurofibromatosis, in a 30 year-old woman is reported. The importance of imaging diagnostic methods in the differential diagnosis of posterior mediastinal masses is discussed. (author)

  6. Eyelid liquoric fistula secondary to orbital meningocele

    Directory of Open Access Journals (Sweden)

    Renato Antunes Schiave Germano

    2015-02-01

    Full Text Available Liquoric fistula (LF is defined as the communication of the subarachnoid space with the external environment, which main complication is the development of infection in the central nervous system. We reported the case of a patient with non-traumatic eyelid liquoric fistula secondary to orbital meningocele (congenital lesion, which main clinical manifestation was unilateral eyelid edema. Her symptoms and clinical signs appeared in adulthood, which is uncommon. The patient received surgical treatment, with complete resolution of the eyelid swelling. In conclusion, eyelid cerebrospinal fluid (CSF fistula is a rare condition but with great potential deleterious to the patient. It should be considered in the differential diagnosis of unilateral eyelid edema, and surgical treatment is almost always mandatory.

  7. Meningocele Cervical. Presentación de un caso Cervical meningocele. A case report

    Directory of Open Access Journals (Sweden)

    Mabel Rita Camejo Macías

    2012-06-01

    Full Text Available El meningocele cervical es una forma rara de disrafismo espinal. La placa neural pasa por diversas transformaciones hasta convertirse en el tubo neural y cualquier alteración durante su cierre conllevará la aparición de la espina bífida. Durante el embarazo las necesidades maternas de folatos aumentan debido a la síntesis de ácidos nucleicos y proteínas durante la embriogénesis, velocidad de crecimiento y desarrollo fetal de los primeros meses de la gestación. Se presenta el caso de paciente masculino de 12 meses de edad, nacido de un parto eutócico institucional ocurrido el día 5 de diciembre del 2008, con una tumoración en la región posterior del cuello al nacer; es remitido al servicio de Neurocirugía del Hospital Mario Catarino Rivas. Se observó como dato positivo en la región cervical un aumento de volumen redondeado, adherido al plano profundo, renitente, movible y no doloroso cubierto de piel en su totalidad. Se le diagnostica un meningocele cervical y confirma mediante resonancia magnética nuclear cervicodorsal. Se realizó la resección del mismo en el mes de febrero del año 2010 y ha sido evaluado periódicamente en las consultas de neurocirugía y en su área de salud con evolución satisfactoria.Cervical meningocele is a rare form of spinal dysrhahism. Neural plate undergoes various transformations to become into the neural tube, and any modifications occurring during the closure, will lead to the onset of spina bifida. During pregnancy the maternal needs of folates increase due to the synthesis of nucleic acids and proteins, all through the embryogenesis, velocity of growth and fetal development in the first months of pregnancy. A 12-months age, male patient, born from a normal delivery, December 5, 2008 at “Santa Barbara” Hospital, presented a tumor at birth in the posterior region of the neck; the patient was referred to the Neurosurgery Service at “Mario Catarino Rivas” Hospital. As positive information

  8. Progressive bilateral anterior sacral meningoceles in Marfan syndrome

    International Nuclear Information System (INIS)

    Scheck, R.J.; Schramm, T.; Gloning, K.P.; Vogl, T.; Ostermayer, E.

    1995-01-01

    Anterior sacral meningoceles (ASM) in Marfan syndrome are rare. They may cause constipation, urinary frequency, dysmenorrhoea, and low back pain or numbness. This report describes bilateral ASM at the level of S1, S2 and S3 in a woman with Marfan syndrome who was admitted to the gynaecology department for evaluation of left lower abdominal pain. The magnetic resonance appearance of the meningoceles is discussed and compared with findings from transvaginal ultrasound and CT. As MRI offers excellent delineation of spinal and pelvic structures, it is the most useful technique available in establishing the diagnosis and planning the treatment of ASM. (orig.)

  9. Progressive bilateral anterior sacral meningoceles in Marfan syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Scheck, R J [Dept. of Radiology, Univ. Muenchen (Germany); Schramm, T [Dept. of Gynaecology and Obstetrics, 1. Frauenklinik, Univ. Muenchen (Germany); Gloning, K P [Dept. of Gynaecology and Obstetrics, 1. Frauenklinik, Univ. Muenchen (Germany); Vogl, T [Dept. of Radiology, Univ. Muenchen (Germany); Ostermayer, E [Dept. of Gynaecology and Obstetrics, 1. Frauenklinik, Univ. Muenchen (Germany)

    1995-08-01

    Anterior sacral meningoceles (ASM) in Marfan syndrome are rare. They may cause constipation, urinary frequency, dysmenorrhoea, and low back pain or numbness. This report describes bilateral ASM at the level of S1, S2 and S3 in a woman with Marfan syndrome who was admitted to the gynaecology department for evaluation of left lower abdominal pain. The magnetic resonance appearance of the meningoceles is discussed and compared with findings from transvaginal ultrasound and CT. As MRI offers excellent delineation of spinal and pelvic structures, it is the most useful technique available in establishing the diagnosis and planning the treatment of ASM. (orig.)

  10. Analysis of the distribution pattern of spinal meningoceles and ...

    African Journals Online (AJOL)

    Background: Spinal meningoceles and meningomyeloceles are part of a group of developmental abnormalities. They are usually midline and could be found throughout the length of the spinal column. Their preferential occurrence along the spine is not clear and their aetiological factors could be numerous. Aim: To ...

  11. Myelo-meningocele: A multi-disciplinary problem | Ibe | Nigerian ...

    African Journals Online (AJOL)

    Background: Myelo-meningoceles are part of congenital afflictions of the spinal column. They arise from the failure of the neural tube to fuse properly during early embryonic growth. The causes and sequalae are multiple and, therefore, require multiple disciplines, to handle them. This study assessed the role of ...

  12. Encephalocele development from a congenital meningocele: case report.

    Science.gov (United States)

    Gandhoke, Gurpreet S; Goldschmidt, Ezequiel; Kellogg, Robert; Greene, Stephanie

    2017-11-01

    A fetal MRI study obtained at 21 weeks' gestation revealed a suboccipital meningocele without hydrocephalus. One day after term birth, MRI demonstrated an acquired cerebellar encephalocele, and MRI obtained 5 months later showed progressive enlargement of the encephalocele, still without obvious hydrocephalus. The patient underwent an operation in which an external ventricular drain was placed, the grossly normal cerebellum was reduced into the posterior fossa without resection, and the dural defect was closed. The drain was weaned out over 5 days, and no ventriculoperitoneal shunt was placed. Postoperative MR images revealed normal cerebellum and no hydrocephalus. The patient is developmentally normal. Meningocele and encephalocele are embryologically distinct. An acquired encephalocele could develop from hydrocephalus (which was not present in this case), or secondary to the lower resistance to expansion into the dural defect of the meningocele relative to the resistance to expansion of the fetal skull. The cerebellar tissue was normal in this case, and was thus preserved. The developmental prognosis is excellent. To the authors' knowledge, this is the first reported case of this occurrence. It is important to differentiate between congenital and acquired encephalocele etiologies, because resection of the cerebellar tissue in an acquired encephalocele (as is routinely done in cases of congenital encephalocele) would be expected to result in neurological deficits.

  13. Myelo-meningocele: A multi-disciplinary problem.

    Science.gov (United States)

    Nnamdi, Ibe Michael Onwuzuruike

    2014-01-01

    Myelo-meningoceles are part of congenital afflictions of the spinal column. They arise from the failure of the neural tube to fuse properly during early embryonic growth. The causes and sequalae are multiple and, therefore, require multiple disciplines, to handle them. This study assessed the role of inter-disciplinary approach in the management of myelo-meningoceles. From 1975 to 2007, the author repaired 20 midline lumbar and lumbo-sacral myelo-meningoceles; 5 in Jamaica and 15 in Nigeria. There were 11 males and 9 females. Their ages, at operation, ranged from 1 to 168 days. All had urine and faecal incontinence and severe paraparesis to paraplegia. Skeletal deformities were present in 16 cases. The operations were carried out under routine general anaesthesia and in prone position. All cases were followed-up for up to 60 months, apart from one who died 4 days at home after discharge. There were no deaths within the period of hospitalisation, usually about 14 days. Those followed-up have not made much improvement, though they were able to sit up without support and move around by shifting on their buttocks on the floor. We must continue to help these patients, but under the umbrella of specialised rehabilitation centres with the different specialists working together to make these patients attain a meaningful life and be useful to themselves and the society.

  14. Endoscopic endonasal repair of spontaneous sphenoid sinus lateral wall meningocele presenting with cerebrospinal fluid leak

    Directory of Open Access Journals (Sweden)

    Ali Erdem Yildirim

    2014-01-01

    Full Text Available Spontaneous sphenoid sinus lateral wall meningoceles are rare lesions with an unknown etiology. Endoscopic endonasal technique is a considerable route in the treatment of this condition. The aim of this paper is to report the etiology, surgical technique, and outcome in a patient repaired via endoscopic endonasal approach. A 51-year-old male patient applied with rhinorrhea started three months ago after an upper respiratory infection. There were no history of trauma or sinus operation. Biochemical analysis of the fluid was positive for beta-2-transferrin. This asypthomatic patient had undergone for repairment of lateral sphenoid sinus meningocele with endoscopic endonasal transsphenoidal approach. After endoscopic endonasal meningocele closure procedure no complications occured and a quick recovery was observed. Endoscopic endonasal approach is an effective and safe treatment modality of spontaneous lateral sphenoid sinus meningoceles and efficient in anterior skull base reconstruction.

  15. Is repair of the protruded meninges sufficient for treatment of meningocele?

    Science.gov (United States)

    Yun-Hai, Song; Nan, Bao; Ping-Ping, Gao; Bo, Yang; Cheng, Chen

    2015-11-01

    The present study aimed to investigate the relationship between meningocele and tethered cord syndrome, diagnosis of meningocele associated with tethered cord syndrome, and when to perform surgery and the best surgical procedure. Sixty-nine children with meningocele who were admitted to Shanghai Children's Medical Center were analyzed. The relationship between meningocele and other lesions causing tethered cord syndrome was studied by combining magnetic resonance imaging (MRI) and intraoperative findings. The MRI results and intraoperative findings showed that 67 children (97%) had associated lesions such as tight filum terminale, fibrous band tethering, spinal cord or cauda equina adhesion, diastematomyelia, arachnoid cyst, and epidermoid cyst. The protruded meninges were repaired, and the intraspinal lesions were treated at the same time. Also, the tethered spinal cord was released. No neurological injuries were observed after surgery. The rate of meningocele associated with tethered cord syndrome is very high. MRI is necessary for the diagnosis of meningocele. Active surgical treatment is recommended immediately after definite diagnosis. During surgery, the surgeon should not only repair the protruded meninges but also explore the spinal canal and release the tethered cord.

  16. Transillumination test: A bedside aid for differentiating meningocele from myelomeningocele: Point of care testing

    Directory of Open Access Journals (Sweden)

    Aakash Pandita

    2015-01-01

    Full Text Available Transillumination test is a bedside and simple technique to illuminate the body cavity by transmission of light through the cavity. Transillumination test is used in a variety of conditions like hydrocele, Hydrocephalus, pneumoperitonium and pneumothorax in neonatology. We describe use of transillumination for differentiating meningocele and myelomeningocele.

  17. Thoracic meningocele in lumbo-costo-vertebral syndrome in a child: possible enlargement with repeated motion by anchoring to the diaphragm.

    Science.gov (United States)

    Wataya, Takafumi; Horikawa, Kyohei; Kitagawa, Masashi; Tashiro, Yuzuru

    2016-08-01

    Lumbo-costo-vertebral syndrome (LCVS) is a rare disorder in children that is characterized by hemivertebrae, congenital absence of ribs, meningocele, and hypoplasia of the truncal and abdominal wall presenting as a congenital lumbar hernia. An otherwise healthy 12-month-old girl was referred to the authors' hospital with soft swelling on her left middle back; scoliosis had been present since birth. Imaging revealed a thoracic meningocele, ectopia of the spleen suggesting lumbar hernia, multiple anomalies of the thoracic vertebral columns, and defects of the ribs; thus, LCVS was diagnosed. Surgical observation revealed that the meningocele was firmly anchored to part of the diaphragm, which created stretching tension in the meningocele continuously with exhalation. Once detached, the meningocele shrank spontaneously and never developed again after cauterization. In this case, continuous or pulsatile pressure in the presence of a vertebral defect was thus considered to be an important factor for formation of the thoracic meningocele.

  18. 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.

  19. Cerebellar dermoid tumor and occipital meningocele in a monozygotic twin : clues to the embryogenesis of craniospinal dysraphism

    NARCIS (Netherlands)

    Groen, R J; van Ouwerkerk, W J

    A case of monochorionic/monoamnionic twin with discordant occipital developmental malformations is presented. One female twin appeared to have an occipital meningocele with cerebellar aplasia and died immediately after birth. The other twin presented with signs and symptoms of raised intracranial

  20. Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1

    International Nuclear Information System (INIS)

    Reis, C.; Carneiro, E.; Fonseca, J.; Salgado, A.; Pereira, P.; Vaz, R.; Pinto, R.; Capelinha, A.F.; Lopes, J.M.

    2005-01-01

    Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles. (orig.)

  1. Analysis of petrous apex meningocele associated with meningioma. Is there any relation with chronic intracranial hypertension?

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Wan-Qun; Huang, Biao; Liang, Chang-Hong [Southern Medical University, The Second School of Clinical Medicine, Guangzhou (China); Guangdong Academy of Medical Sciences, Department of Radiology, Guangdong General Hospital, Guangzhou, Guangdong (China); Feng, Jie-Ying [Nanhai Hospital Affiliated Southern Medical University, Department of Radiology, Foshan, Guangdong (China); Liu, Hong-Jun [Guangdong Academy of Medical Sciences, Department of Radiology, Guangdong General Hospital, Guangzhou, Guangdong (China)

    2018-02-15

    Petrous apex meningocele (PAM) is an uncommon cystic lesion involving the petrous apex. The underlying cause of PAM may be related to chronic elevated intracranial pressure. The aim of the study was to explore the relationship between PAM and meningioma and between PAM and other intracranial hypertension findings. Two hundred seventy-eight consecutive patients with meningiomas were retrospectively studied. Fifty age- and gender-matched controls were also enrolled in this study. The incidence of PAM, empty sella, tortuosity of the optic nerve, and hydrops of optic nerve sheath was evaluated. The maximum width, area, volume of each PAM, or Meckel's cave and volume of meningioma were measured in controls and patients, separately. One hundred fifty-nine (57.19%) patients were detected with coexistent PAMs. One hundred twenty-five patients had bilateral PAMs, 34 had unilateral lesions, and the remaining 119 did not have PAM. Two subjects (4/50) had unilateral PAMs in normal controls. The maximum width, area, volume of PAM, or Meckel's cave were significantly larger in the patients with bilateral PAM group than those in the unilateral PAM group, in the group without PAM, and those in control group (p = 0.000). The volume of meningioma was positively correlated with the PAM volume (r = 0.48). There was a positive correlation for the incidence between PAM and (1) empty sella (r = 0.901) and (2) tortuosity of the optic nerves and hydrops of the optic sheath (r = 0.825). Coexistence of PAMs with meningiomas is not rare in incidence, and it suggests a potential role for chronically elevated intracranial pressure and disturbance of CSF circulation in their pathophysiology. (orig.)

  2. Analysis of petrous apex meningocele associated with meningioma. Is there any relation with chronic intracranial hypertension?

    International Nuclear Information System (INIS)

    Yang, Wan-Qun; Huang, Biao; Liang, Chang-Hong; Feng, Jie-Ying; Liu, Hong-Jun

    2018-01-01

    Petrous apex meningocele (PAM) is an uncommon cystic lesion involving the petrous apex. The underlying cause of PAM may be related to chronic elevated intracranial pressure. The aim of the study was to explore the relationship between PAM and meningioma and between PAM and other intracranial hypertension findings. Two hundred seventy-eight consecutive patients with meningiomas were retrospectively studied. Fifty age- and gender-matched controls were also enrolled in this study. The incidence of PAM, empty sella, tortuosity of the optic nerve, and hydrops of optic nerve sheath was evaluated. The maximum width, area, volume of each PAM, or Meckel's cave and volume of meningioma were measured in controls and patients, separately. One hundred fifty-nine (57.19%) patients were detected with coexistent PAMs. One hundred twenty-five patients had bilateral PAMs, 34 had unilateral lesions, and the remaining 119 did not have PAM. Two subjects (4/50) had unilateral PAMs in normal controls. The maximum width, area, volume of PAM, or Meckel's cave were significantly larger in the patients with bilateral PAM group than those in the unilateral PAM group, in the group without PAM, and those in control group (p = 0.000). The volume of meningioma was positively correlated with the PAM volume (r = 0.48). There was a positive correlation for the incidence between PAM and (1) empty sella (r = 0.901) and (2) tortuosity of the optic nerves and hydrops of the optic sheath (r = 0.825). Coexistence of PAMs with meningiomas is not rare in incidence, and it suggests a potential role for chronically elevated intracranial pressure and disturbance of CSF circulation in their pathophysiology. (orig.)

  3. Meningocele repair - series (image)

    Science.gov (United States)

    ... containing a portion of the spinal cord membrane (meninges), spinal fluid, and a portion of spinal cord ... The spinal cord is covered with the membranes (meninges) and the skin is closed over the protruding ...

  4. Genetics Home Reference: lateral meningocele syndrome

    Science.gov (United States)

    ... back pain can also occur. Delayed development of motor skills in infancy, such as sitting and crawling, ... dysphagia), and backflow of stomach acids into the esophagus (called gastroesophageal reflux or GERD). Related Information What does it mean if a disorder seems to run in my family? What is ...

  5. Fístula de líquido cefalorraquídeo recidivante postraumática asociada a meningocele esfenoidal: Técnica abierta-endoscópica Cerebrospinal fluid fistula associated with posttraumatic recurrent sphenoidal meningocele: Open-endoscopic technique

    Directory of Open Access Journals (Sweden)

    Ignacio Zubillaga Rodríguez

    2012-12-01

    Full Text Available Introducción: Las fístulas de líquido cefalorraquídeo surgen tras la ruptura de las barreras que separan la cavidad nasal y senos paranasales de los espacios subaracnoideos: base craneal, duramadre y membrana aracnoidea. Aproximadamente el 80% surgen en el contexto de traumatismos craneofaciales con fracturas de la base craneal. La elección del abordaje y técnica quirúrgica más adecuada en cada caso es esencial para la obtención de resultados quirúrgicos globales satisfactorios. El desarrollo de la cirugía endoscópica endonasal ha supuesto un arma terapéutica menos invasiva y eficaz, siendo las fístulas de líquido cefalorraquídeo una indicación bien establecida para su tratamiento definitivo. Caso clínico: Se presenta el caso de una paciente con fístula de líquido cefalorraquídeo recurrente con meningoencefalocele asociado tratada vía endoscópica. Discusión: Se discute el tratamiento conservador versus quirúrgico de las fístulas de líquido cefalorraquídeo. Ventajas y desventajas de los distintos tipos de abordajes relacionados con el manejo definitivo.Introduction: Cerebrospinal fluid fistulas arise after the breakdown of the barriers that separate the nasal cavity and paranasal sinuses of the subarachnoid space, skull base, dura and arachnoid membrane. Approximately 80% arise in the context of craniofacial trauma with fractures of the skull base. The choice of approach, appropriate surgical technique in each case is essential to achieve a good overall surgical outcome. Development of endoscopic endonasal surgery has become a less invasive and effective therapeutic tool, with cerebrospinal fluid fistulas being a well-established indication for definitive treatment. Case report: A case of a patient with cerebrospinal fluid fistula associated with recurrent meningoencephalocele, treated endoscopically. Discussion: We discuss the surgical versus conservative treatment of spinal fluid fistulas, and the advantages and disadvantages of different types of approaches related to definitive management.

  6. EAMJ Feb. Editorial.indd

    African Journals Online (AJOL)

    2009-02-02

    Feb 2, 2009 ... Design:a retrospective study reviewing the age and sex of the patients, type and contents ... meninges alone (meningoceles), may contain brain ... difference between sincipital and occipital types in terms of sex distribution ...

  7. Myelography for nerve root avulsion in birth palsy

    Energy Technology Data Exchange (ETDEWEB)

    Hashimoto, Tsutomu; Mitomo, Masanori; Hirabuki, Norio; Miura, Takashi; Kawai, Ryuji; Imakita, Satoshi; Harada, Koshi; Nakamura, Hironobu; Kozuka, Takahiro (Osaka Univ. (Japan). Faculty of Medicine)

    1990-04-01

    Myelography and CT myelography (CMT) were reviewed in 18 cases of birth palsy with clinically suspected avulsion injury. Root-somatosensory evoked potential (root-SEP) was also reviewed for myelographic evaluation of the nerve root avolusion in birth palsy. Root-SEP is not induced in case of avulsed nerve roots, but is induced in case of both normal and incompletely avulsed roots. Myelography demonstrated 58 abnormal nerve roots in 18 cases (19 limbs); 45 (78%) complete and 13 (22%) incomplete nerve root avulsions. Each of complete and incomplete avulsions was defined as total absence and partial presence of rootlets on myelography, respectively. Traumatic meningoceles were detected at 46 roots (79%) on myelography and/or CTM; 35 roots on myelography and 45 roots on CTM. CTM could not detect only a very small meningocele at one root. At 11 roots CTM was superior to myelography in delineating a meningocele because CTM is sensitive to a poorly enhanced meningocele. CTM, however, could not diagnose nerve root avulsions so accurately as myelography, since myelography detected 12 (7 completely and 5 incompletely) avulsed roots without meningocele, whereas CTM could not delineate the nerve roots clearly. Thus, myelography is indispensable to evaluate nerve root avulsions without meningocele. Root-SEP was examined in 9 patients who underwent branchial plexus exploration. SEP was negative at 22/25 roots with complete avulsion and was positive at 7/7 roots with myelographically incomplete avulsion, regardless of presence or absence of any traumatic meningocele. Myelography and root-SEP correlated well at 29 (92%) out of 32 roots in evaluating complete and incomplete avulsion injuries. Myelography and root-SEP were not considered in 3 roots. Though myelography demonstrated complete avulsions with traumatic meningocele, SEP was positive in these three roots, which were interpreted as partially avulsed roots. (J.P.N.).

  8. Myelography for nerve root avulsion in birth palsy

    International Nuclear Information System (INIS)

    Hashimoto, Tsutomu; Mitomo, Masanori; Hirabuki, Norio; Miura, Takashi; Kawai, Ryuji; Imakita, Satoshi; Harada, Koshi; Nakamura, Hironobu; Kozuka, Takahiro

    1990-01-01

    Myelography and CT myelography (CMT) were reviewed in 18 cases of birth palsy with clinically suspected avulsion injury. Root-somatosensory evoked potential (root-SEP) was also reviewed for myelographic evaluation of the nerve root avolusion in birth palsy. Root-SEP is not induced in case of avulsed nerve roots, but is induced in case of both normal and incompletely avulsed roots. Myelography demonstrated 58 abnormal nerve roots in 18 cases (19 limbs); 45 (78%) complete and 13 (22%) incomplete nerve root avulsions. Each of complete and incomplete avulsions was defined as total absence and partial presence of rootlets on myelography, respectively. Traumatic meningoceles were detected at 46 roots (79%) on myelography and/or CTM; 35 roots on myelography and 45 roots on CTM. CTM could not detect only a very small meningocele at one root. At 11 roots CTM was superior to myelography in delineating a meningocele because CTM is sensitive to a poorly enhanced meningocele. CTM, however, could not diagnose nerve root avulsions so accurately as myelography, since myelography detected 12 (7 completely and 5 incompletely) avulsed roots without meningocele, whereas CTM could not delineate the nerve roots clearly. Thus, myelography is indispensable to evaluate nerve root avulsions without meningocele. Root-SEP was examined in 9 patients who underwent branchial plexus exploration. SEP was negative at 22/25 roots with complete avulsion and was positive at 7/7 roots with myelographically incomplete avulsion, regardless of presence or absence of any traumatic meningocele. Myelography and root-SEP correlated well at 29 (92%) out of 32 roots in evaluating complete and incomplete avulsion injuries. Myelography and root-SEP were not considered in 3 roots. Though myelography demonstrated complete avulsions with traumatic meningocele, SEP was positive in these three roots, which were interpreted as partially avulsed roots. (J.P.N.)

  9. Basal sphenoethmoidal encephalocele in association with midline cleft lip and palate: case report

    International Nuclear Information System (INIS)

    Holanda, Maurus Marques de Almeida; Rocha, Artur Bastos; Santos, Rayan Haquim Pinheiro; Furtado, Paulo Germano Cavalcanti

    2011-01-01

    Association of basal sphenoethmoidal encephalocele with midline cleft lip and palate is extremely rare. The authors report the case of a nine-year-old girl presenting a midline facial cleft with meningocele that was noticeable through the palatine defect as a medial intranasal pulsatile mass. An analysis of clinical and radiological findings of the present case of cranial dysraphism is carried out. (author)

  10. Basal sphenoethmoidal encephalocele in association with midline cleft lip and palate: case report

    Energy Technology Data Exchange (ETDEWEB)

    Holanda, Maurus Marques de Almeida; Rocha, Artur Bastos; Santos, Rayan Haquim Pinheiro [Universidade Federal da Paraiba (UFPB), Joao Pessoa, PB (Brazil); Furtado, Paulo Germano Cavalcanti [Universidade Federal da Paraiba (UFPB), Joao Pessoa, PB (Brazil). Dept. de Pediatria e Genetica

    2011-11-15

    Association of basal sphenoethmoidal encephalocele with midline cleft lip and palate is extremely rare. The authors report the case of a nine-year-old girl presenting a midline facial cleft with meningocele that was noticeable through the palatine defect as a medial intranasal pulsatile mass. An analysis of clinical and radiological findings of the present case of cranial dysraphism is carried out. (author)

  11. Case report

    African Journals Online (AJOL)

    abp

    2015-02-04

    Feb 4, 2015 ... Unlike the more common traumatic or post-surgical leaks, pneumocephalus and smell impairment is rare. The non-traumatic group is associated with brain tumors which are known to erode skull like cholestatoma or tuberculoma, skull base congenital defects and meningoceles or meningoencephalocele.

  12. Spina Bifida. Fact Sheet = Espina Bifida. Hojas Informativas Sobre Discapacidades.

    Science.gov (United States)

    National Information Center for Children and Youth with Disabilities, Washington, DC.

    This fact sheet offers definitions of the three types of spina bifida (spina bifida occulta, meningocele, and myelomeningocele), outlines their incidence, describes characteristics of individuals with spina bifida, and reviews educational implications. The fact sheet discusses the need for many children with myelomeningocele to learn to manage…

  13. Spina Bifida: General Information. Fact Sheet Number 12 = La Espina Bifida: Informacion General. Fact Sheet Number 21.

    Science.gov (United States)

    Interstate Research Associates, McLean, VA.

    This fact sheet on spina bifida is offered in both English and Spanish. It provides definitions of the three types of spina bifida (spina bifida occulta, meningocele, and myelomeningocele). Incidence figures are given as are typical characteristics of children with spina bifida. Educational implications are briefly noted, including the need to…

  14. Comparative Studies of Spinal Celes in Switzerland, Jamaica, and ...

    African Journals Online (AJOL)

    Clinical Methods and Materials: From 1975 to 2008,17 spinal celes (including 2 meningoceles) were routinely repaired in Imo and Ebonyi States of Nigeria, and 5 in Jamaica,the West Indies; none in Basel, Switzerland. All 20 meningomyeloceles were incontinent of urine and faeces, had severe paraparesis to paraplegia, ...

  15. Comparative Studies of Spinal Celes in Switzerland, Jamaica, and ...

    African Journals Online (AJOL)

    Objective and Background: The need to sanitize our environment from elements hazardous to life on earth is hereby high-lighted, as well as a properly organized and efficiently run prenatal health care system. Clinical Methods and Materials: From 1975 to 2008,17 spinal celes (including 2 meningoceles) were routinely ...

  16. Syndromes, Disorders and Maternal Risk Factors Associated with Neural Tube Defects (II

    Directory of Open Access Journals (Sweden)

    Chih-Ping Chen

    2008-03-01

    Full Text Available Fetuses with neural tube defects (NTDs maybe associated with syndromes, disorders, and maternal risk factors. This article provides a comprehensive review of syndromes, disorders, and maternal risk factors associated with NTDs, such as Currarino syndrome, sacral defect with anterior meningocele, Jarcho-Levin syndrome (spondylo-costal dysostosis, lateral meningocele syndrome, neurofibromatosis type I, Marfan syndrome, and hyperthermia. The recurrence risk and the preventive effect of maternal folic acid intake in NTDs associated with syndromes, disorders, and maternal risk factors may be different from those of non-syndromic multifactorial NTDs. Perinatal identification of NTDs should alert one to the syndromes, disorders, and maternal risk factors associated with NTDs, and prompt a thorough etiologic investigation and genetic counseling.

  17. Encefalocele basal esfenoetmoidal associada a fissura labiopalatina mediana: relato de caso

    Directory of Open Access Journals (Sweden)

    Maurus Marques de Almeida Holanda

    2011-12-01

    Full Text Available Associação de encefalocele basal esfenoetmoidal com fissura labiopalatina é extremamente rara. Relatamos um caso de uma criança de nove anos de idade apresentando uma fissura facial mediana com meningocele, que era evidente através da falha do palato como uma massa mediana intranasal pulsátil. Uma análise dos aspectos clínicos e radiológicos deste caso de disrafia craniana foi realizada.

  18. Unusual causes of spinal foraminal widening

    Energy Technology Data Exchange (ETDEWEB)

    Zibis, A.H.; Markonis, A.; Karantanas, A.H. [Dept. of CT and MRI, Larissa General Hospital (Greece)

    2000-01-01

    Spinal neural foraminal widening is usually caused by benign lesions, most commonly neurofibromas. Rare lesions can also cause spinal neural foraminal widening. Computed tomography and/or MRI are the modalities of choice for studying the spinal foraminal widening. The present pictorial review describes six rare lesions, namely a lateral thoracic meningocele, a malignant fibrous histiocytoma, a tuberculous abscess, an osteoblastoma, a chondrosarcoma and a malignant tumour of the lung which caused spinal neural foraminal widening. (orig.)

  19. Currarino triad associated with malrotation of the colon

    International Nuclear Information System (INIS)

    Daoud, Fajez S.; AbuRub, Mohammad A.; Hadidy, Azmy M.

    2007-01-01

    Currarino triad is a rare congenital condition characterized by a sacral bony defect, presacral mass, and anorectal malformations. We describe an unusual case of complete Currarino triad in a 22-year-old female with sacral bony defect, anal stenosis, recto-vaginal fistula, and dual pathology meningocele and teratoma in the pre sacral mass associated with malrotation of the colon. This combination has not been reported previously. The clinical presentation, surgical management and review of literature are discussed. (author)

  20. Currarino triad associated with malrotation of the colon

    Energy Technology Data Exchange (ETDEWEB)

    Daoud, Fajez S [Dept. of General Surgery, Jordan University Hospital, Amman (Jordan); AbuRub, Mohammad A [Dept. of Neurosurgery, Jordan University Hospital, Amman (Jordan); Hadidy, Azmy M [Dept. of Radiology (Hadidy), Jordan University Hospital, Amman (Jordan)

    2007-07-01

    Currarino triad is a rare congenital condition characterized by a sacral bony defect, presacral mass, and anorectal malformations. We describe an unusual case of complete Currarino triad in a 22-year-old female with sacral bony defect, anal stenosis, recto-vaginal fistula, and dual pathology meningocele and teratoma in the pre sacral mass associated with malrotation of the colon. This combination has not been reported previously. The clinical presentation, surgical management and review of literature are discussed. (author)

  1. MR imaging of the spine in children

    Energy Technology Data Exchange (ETDEWEB)

    Duthoy, M.J.; Lund, G.

    1988-08-01

    MR imaging was reviewed in 66 pediatric spinal cord patients with diagnoses of posterior fossa tumor, primary spinal cord tumor, metastatic disease, neuroectodermal disorder, congenital malformation, trauma, and demyelinating, neurodegenerative, or metabolic disorders. MR proved to be useful in delineating the extent of posterior fossa and cord tumor including metastasis to the cord. MR was of limited value in demyelinating and metabolic disorders. Arnold Chiari malformations, syringomyelia, tethered cord and meningoceles were all easily evaluated using MR.

  2. Terminal hemimyelocystocele associated with Chiari II malformation

    Directory of Open Access Journals (Sweden)

    Umamaheswara Reddy V.

    2014-06-01

    Full Text Available Terminal myelocystocele (TMC results from failure of embryonic CSF to drain outside the neural tube creating CSF reservoir within a dorsal meningocele. Association of Chiari II malformation with diastematomyelia and myelocystocele is extremely rare. Myelocystoceles do not have neural tissue so they have good prognosis after treatment, however when associated with hydromelia and Chiari malformation they present with neurological deficits. We present details of a 2 year old female who presented to us with this rare anomaly.

  3. Currarino triad with dual pathology in the presacral mass: report of a case.

    Science.gov (United States)

    Thambidorai, C R; Muin, I; Razman, J; Zulfiqar, A

    2003-07-01

    Currarino triad, which comprises anorectal stenosis, anterior sacral defect, and a presacral mass, is an uncommon cause of constipation in children and adults. The presacral mass in this triad is most often caused by an anterior sacral meningocele, a teratoma, or an enterogenous cyst, but rarely may be caused by dual pathology. A neonate with Currarino triad and dual pathology in the presacral mass is described in this report. A male Chinese neonate, who presented with abdominal distention and constipation on the second day of life, was found to have features of Currarino triad. Colostomy was done in the neonatal period, and the presacral mass was excised by posterior sagittal perineal approach at the age of six months. The excised presacral mass consisted of an anterior meningocele and a teratoma. The patient continued to have constipation during follow-up and required anorectoplasty to correct residual anorectal stenosis. At the time of this report the patient was three years old and growing normally with normal anorectal function. Of a total of about 200 cases of complete Currarino triad found in the literature, in only 22 patients did the presacral mass contain both meningocele and teratoma. The features of these 22 patients and the current views on the surgical management of Currarino triad are discussed.

  4. MR evaluation of brachial plexus injuries

    International Nuclear Information System (INIS)

    Gupta, R.K.; Jain, R.K.; Mehta, V.S.; Banerji, A.K.

    1989-01-01

    Ten cases of brachial plexus injury were subjected to magnetic resonance (MR) to demonstrate the roots, trunks, divisions or cord abnormalities. Both normal and abnormal brachial plexuses were imaged in sagittal, axial, coronal and axial oblique planes. Myelography, using water soluble contrast agents, was performed in seven cases. MR demonstrated one traumatic meningocele, one extradural cerebrospinal fluid (CSF) collection, trunk and/or root neuromas in four, focal root fibrosis in two and diffuse fibrosis in the remaining two cases. Results of MR were confirmed at surgery in four cases with neuromas, while myelography was normal in two and was not carried out in the remaining two. In two cases, where MR demonstrated diffuse fibrosis of the brachial plexus, myelography showed C7 and T1 traumatic meningocele in one and was normal in the other. Both these patients showed excellent clinical and electrophysiological correlation with MR findings and in one of them surgical confirmation was also obtained. In the other two cases with focal nerve root fibrosis, myelography was normal in one and showed a traumatic meningocele in another. Operative findings in these cases confirmed focal root fibrosis but no root avulsion was observed although seen on one myelogram. Focal fibrosis, however, was noted at operation in more roots than was observed with MR. Initial experience suggests that MR may be the diagnostic procedure of choice for complete evaluation of brachial plexus injuries. (orig.)

  5. Currarino triad: surgical management and follow-up results of four [correction of three] cases.

    Science.gov (United States)

    Isik, Nejat; Elmaci, Ilhan; Gokben, Bekir; Balak, Naci; Tosyali, Nadir

    2010-08-01

    The Currarino syndrome is a rare triad that is a combination of a presacral mass, a congenital sacral bony abnormality and an anorectal malformation. We present 4 children with complete Currarino triad who were diagnosed using MRI. Our aim was to report the neurosurgical management of Currarino syndrome in children. All of the patients had chronic constipation and pain in the lumbosacral region. In the plain radiograph, 3 patients had a sacral scimitar-shaped bony abnormality, and 1 patient had total sacral agenesis. There was a narrow anal canal or narrow ventrally displaced anus in all patients. Their anorectal malformations were characterized as anal stenoses (4 patients), associated with Hirschsprung's disease in 2 cases. In 3 patients, MRI showed tethered cord syndrome in addition to the presacral mass. There was hydrocephalus in 1 patient. Anal stenosis was treated by anal dilatation. In 2 patients, rectal biopsy and temporary colostomy (2 patients) had been performed previously due to Hirschsprung's disease. We performed a posterior procedure via lumbar and sacral partial laminectomy-laminoplasty and transdural ligation of the neck of the meningocele for anterior sacral meningoceles, or alternatively, tumor excision for other types of presacral lesions. Histopathologically, 3 were cases of anterior sacral meningoceles and 1 was a teratoma. One of them also had a spinal abscess. He required reoperation (twice) and appeared at the time to have improved with medical therapy. All patients improved and stabilized. There were no additional neurological deficits and no recurrence of the presacral mass over the follow-up period (6 years, on average). The family pedigree did not reveal any familial transmission pattern. In cases of Currarino triad, MRI can allow the characterization of the presacral masses. If it is an anterior sacral meningocele or a solid tumor without severe anorectal malformation, it can be managed with posterior lumbar and sacral procedures

  6. Comparison of hydrocephalus appearance at spinaldysraphia.

    Science.gov (United States)

    Elshani, Besnik; Lenjani, Basri

    2013-01-01

    Congenital malformation of spinal dysraphism followed by hydrocephalus are phenomenon reveals during intrauterine child growth. Prime objective of this work was to present Comparison of hydrocephalus appearance at spinal dysraphism respectively at its meningocele and myelomeningocele forms in Neurosurgery Clinic in UCC in Prishtina. It is perfected with retrospective and prospective method precisely of its epidemiologic part summarizing notices from patients' histories which in 2000-2006 are hospitalized in Neurosurgery Clinic from (QFLPK)--Pediatric Clinic and Children Box (Department)--Gynecology Clinic and from Sanitary Regional Center throughout Kosova. Our study objects were two groups, as the first group 90 patients with spinal dysraphism where neurosurgery operations were done and classified types of dysraphism. At myelomeningocele hydrocephalus has dominated and in a percent of appearance and as acute of its active form was 97% of hydrocephalus form where subjected to cerebrospinal liquid derivation with ventriculo -peritoneal shunt in comparison with meningocele we do not have involvation of spinal nerve element, hydrocephalus takes active form with intervention indication in 60% of cases. Reflection in shown deficit aspect is totally different at myelomeningoceles where lower paraplegia dominate more than paraparesis. The second patient operative technique developed by hydrocephalus with neurosurgical intervention indication has to do with placing of (VP) ventriculo- peritoneal system (shant) at myelomeningoceles with hydrocephalus 58 cases and 12 cases meningoceles with hydrocephalus. Post operative meningitis (shant meningitis): from 70 operated cases of hydrocephalus with spinal dysraphism shunts complications from all types are just cases. Finally that appearance of hydrocephalus compared at spinal dysraphism dominate at myellomeningoceles as in notice time aspect, it is persisting and further acute, with vital motivation for neurosurgical

  7. Brain Herniation in Neurofibromatosis with Dysplasia of Occipital Bone and Posterior Skull Base

    Directory of Open Access Journals (Sweden)

    Vithal Rangarajan

    2015-01-01

    Full Text Available A 22-year-old female, a known case of neurofibromatosis 1 (NF1, presented with a congenital swelling in the left occipital region. She had developed recent onset dysphagia and localized occipital headache. Neuroradiology revealed a left occipital meningoencephalocele and a left parapharyngeal meningocele. This was associated with ventriculomegaly. She was advised on cranioplasty along with duraplasty which she denied. She agreed to a lumbar-peritoneal shunt. She described a dramatic improvement in her symptoms following the lumbar-peritoneal shunt. Occipital dysplasias, though uncommon, have been reported in the literature. We review this case and its management and discuss relevant literature on occipital dysplasias in NF1.

  8. Congenital high airway obstruction syndrome (CHAOS) associated with cervical myelomeningocele.

    Science.gov (United States)

    Adin, Mehmet Emin

    2017-10-01

    Congenital high airway obstruction syndrome (CHAOS) is a rare and potentially fatal entity resulting from complete or near complete developmental airway obstruction. Although most reported cases of CHAOS are sporadic, the condition may also be associated with certain syndromes and a variety of cervical masses. Meningocele and myelomeningocele have not yet been reported in association with CHAOS. We describe the typical constellation of sonographic findings in a case of early diagnosis of CHAOS associated with cervical myelomeningocele. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:507-510, 2017. © 2016 Wiley Periodicals, Inc.

  9. Lumbar nerve root avulsions with secondary ipsilateral hip dysplasia in a child

    Energy Technology Data Exchange (ETDEWEB)

    Polyzoidis, Konstandinos; Vranos, Georgios [Department of Neurosurgery, Medical School, University of Ioannina, 45110, Ioannina (Greece); Petropoulou, Calliope; Argyropoulou, Paraskevi I.; Argyropoulou, Maria I. [Department of Radiology, Medical School, University of Ioannina, 45110, Ioannina (Greece); Sarmas, Ioannis [Department of Neurology, Medical School, University of Ioannina, 45110, Ioannina (Greece)

    2002-09-01

    We report on an 8-year-old child with avulsions of the left L3, L4 and L5 nerve roots and traumatic meningoceles that were not associated with lumbar spine or pelvic girdle fractures. The patient had a history of a road traffic accident. Plain radiographs of the pelvis revealed left hip dysplasia. The magnetic resonance imaging findings of the lumbar spine are illustrated. The pathogenesis of lumbar nerve root avulsions and their association with ipsilateral hip dysplasia are discussed. (orig.)

  10. SPHENOCHOANAL POLYP: A CASE STUDY

    Directory of Open Access Journals (Sweden)

    Gaurav

    2015-09-01

    Full Text Available Sphenochoanal polyp is a rare clinical occurrence as compared to the much common antro - choanal polyp. It originates from the sphenoid sinus and extends into the choana via the sphenoid ostium. We present a case of spheno - choanal polyp and its clinical features and surgical management is discussed. Our aim in this case was to properly d elineate the origin of the polyp and differentiate it from other lesions such as the antro - choanal polyp and meningocele, followed by meticulous endoscopic excision of the polyp

  11. Roentgenographic features of neurofibromatosis von Recklinghausen

    Energy Technology Data Exchange (ETDEWEB)

    Galanski, M.; Vogelsang, H.; Cramer, B.M.; Thun, F.; Peters, P.E.

    1983-10-01

    The basic abnormality of neurofibromatosis consists not only of a maldevelopment of the neuroectoderm but also of the mesoderm. Therefore any organ or system of the body may be involved. Following a short clinical review of the central (acoustic) and peripheral type the well known and the more unusual radiographic findings are summarized. They include cranial and intracranial manifestations (orbitosphenoid dysplasia, bone defects of the skull, acoustic neuroma, glioma of the optic nerve and chiasm, meningioma), spinal lesions (scoliosis, vetebral scalloping, meningocele, neuroma, ependymoma), skeletal abnormalities (pseudarthrosis), cardiovascular manifestations, pulmonary fibrosis, tumors of the gastrointestinal and urinary tract and different endocrinopathies. Some of the roentgeologic symptoms are very characteristic and allow definitive diagnosis.

  12. Antenatal sonographic appearance of a large orbital encephalocele: a case report and differential diagnosis of orbital cystic mass.

    Science.gov (United States)

    Ahmed, Ahmed; Noureldin, Rehab; Gendy, Mohamed; Sakr, Sharif; Abdel Naby, Mahmoud

    2013-06-01

    Orbital meningoceles and encephaloceles are rare extracranial extensions of the brain and meninges with or without direct communication between the central nervous system and the abnormal mass. We reported a rare case of large fetal orbital encephalocele; the diagnosis was suspected initially by prenatal ultrasound and confirmed by postnatal MRI and CT scans. The differential diagnosis of an intrauterine fetal cystic orbital mass includes orbital teratoma, epidermoid inclusion cysts, hemangioma or lymphangioma, congenital cystic eye, dacryocystocele, and orbital cephalocele. Copyright © 2012 Wiley Periodicals, Inc.

  13. Lumbar nerve root avulsions with secondary ipsilateral hip dysplasia in a child

    International Nuclear Information System (INIS)

    Polyzoidis, Konstandinos; Vranos, Georgios; Petropoulou, Calliope; Argyropoulou, Paraskevi I.; Argyropoulou, Maria I.; Sarmas, Ioannis

    2002-01-01

    We report on an 8-year-old child with avulsions of the left L3, L4 and L5 nerve roots and traumatic meningoceles that were not associated with lumbar spine or pelvic girdle fractures. The patient had a history of a road traffic accident. Plain radiographs of the pelvis revealed left hip dysplasia. The magnetic resonance imaging findings of the lumbar spine are illustrated. The pathogenesis of lumbar nerve root avulsions and their association with ipsilateral hip dysplasia are discussed. (orig.)

  14. MR imaging evaluation of congenital malformation of the spine

    International Nuclear Information System (INIS)

    Byrd, S.E.; Radkowski, M.A.; McLone, D.G.; Storrs, B.B.

    1988-01-01

    One hundred fifty children with congenital malformations of the spine were studied with MR imaging, US, and water-soluble myelography with CT. The malformations encountered included myelomeningoceles and Chiari II malformations, diastematomyelia, hydromyelia, arachnoid cyst, dermal sinus and dermoid, spinal lipomas and teratomas, tight filum terminale, anterior sacral meningoceles, lipomyelomeningoceles, myelocystocele, and severe scoliosis. MR imaging was the most effective modality in delineating these abnormalities. At times, real-time US and water-soluble CT myelography were required as adjuncts to MR imaging in delineating subtle spinal cord tethering, arachnoid cyst, diastematomyelia, and severe scoliosis

  15. A tale of two tails: Not just skin deep

    Directory of Open Access Journals (Sweden)

    Abhijit Dutta

    2015-01-01

    Full Text Available The dorsal cutaneous appendage or the so called human tail is a rare congenital anomaly protruding from the lumbo-sacro-coccygeal area. These caudal appendages are divided into true-tails and pseudo-tails. We report here two cases of congenital pseudo-tail with underlying spina bifida and lipo-meningocele. In this article we seek to emphasize that, as the skin and nervous systems are intimately linked by their similar ectodermal origin, a dorsal appendage may be regarded as a cutaneous marker of the underlying spinal dysraphism.

  16. Diagnosis of fetal neural tube defects by MRI

    International Nuclear Information System (INIS)

    Dong Suzhen; Zhu Ming; Zhong Yumin; Zhang Hong; Pan Huihong

    2010-01-01

    Objective: To explore the diagnostic value of MRI on fetal neural tube defects. Methods: Ten pregnant women, aged from 25 to 35 years (average 28 years) and with gestation from 20-39 weeks (average 33 weeks) were studied with a 1.5 T superconductive MR unit within 24 to 48 hours after ultrasound (US) studies. The imaging protocol included fast-imaging employing steady-state acquisition, single-shot FSE and T 1 -weighted fast inversion recovery motion insensitive sequences in the axial, fromtal, and sagittal planes relative to the fetal brain, thorax, abdomen, and spines. Prenatal US and MRI findings were compared with postnatal MRI diagnoses (3 fetuses) or autopsy (7 fetuses). Results: Ten pregnant women (9 with a single fetus and 1 with twin fetuses) were examined. For all cases, the diagnoses established by MRI were correct when compared with postnatal diagnosis or autopsy. In 7 cases, US and MRI findings were in complete agreement with postnatal diagnoses. US missed the diagnosis in 1 cases and misdiagnosed in 2 cases. Ten neural tube defects in this study included anencephaly (1 case), exencephaly (1 case), meningoencephalocele associated with amniotic band sequence (1 case), meningocele (1 case), thoracic myelomeningocele (1 case), lumbar spinal bifida (1 case), sacroiliac myelomeningocele (2 cases), sacroiliac large cystic spinal meningocele (1 case), sacroiliac spinal bifida (1 case). Conclusions: Prenatal MRI is effective in the assessment of fetal neural tube defects. It can exactly discriminate herniated contents and locate the spinal lesion level. (authors)

  17. Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida Sacrococcygeal agenesis association and anal atresia in mixed breed cats

    Directory of Open Access Journals (Sweden)

    Felipe Purcell de Araújo

    2009-09-01

    Full Text Available O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

  18. Endoscopic transnasal repair of cerebrospinal fluid leaks with and without an encephalocele in pediatric patients: from infants to children.

    Science.gov (United States)

    Ma, Jingying; Huang, Qian; Li, Xiaokui; Huang, Dongsheng; Xian, Junfang; Cui, Shunjiu; Li, Yunchuan; Zhou, Bing

    2015-09-01

    The diagnosis and management of pediatric cerebrospinal fluid (CSF) leak and encephalocele are challenging. The current study aimed to identify patient characteristics, review operative techniques, and evaluate the efficacy and safety of endoscopic endonasal repair in a pediatric population. We retrospectively reviewed the records of pediatric patients who underwent transnasal endoscopic repair of CSF leak with or without a meningocele or an encephalocele at Beijing Tongren Hospital, Capital Medical University, between July 2003 and May 2014. All patients had preoperative radiological evaluations and underwent endoscopic endonasal repair of their skull base defects. Altogether, 23 children (mean age 7.0 years) underwent the procedures. Sixteen cases were congenital, and 7 patients had trauma history. The herniations or defects included meningoencephaloceles in 15 cases, meningoceles in 4 cases, and CSF leak in 4 cases (2 patients had bilateral leaks). The leak or herniation sites were ethmoid roof in 10 patients (one was bilateral), cribriform plate in 5, lateral to the foramen cecum in 3, posterior wall of the frontal sinus in 1, sphenoid sinus in 2, lateral recess of the sphenoid sinus in 1, and sella turcica base in 2. All subjects had favorable clinical outcomes without recurrence during a follow-up of 6-123 months (mean 61.1 months). The endoscopic endonasal approach was the preferred method for repairing CSF leaks with or without an encephalocele in pediatric patients. Compared to traditional operations, this endoscopic procedure is minimally invasive, efficient, and safe.

  19. Innovation in the planning of V-Y rotation advancement flaps: A template for flap design

    Directory of Open Access Journals (Sweden)

    Utku Can Dölen

    2018-01-01

    Full Text Available Local flaps exhibit excellent color matching that no other type of flap can compete with. Moreover, surgery using a local flap is easier and faster than surgery using a distant or free flap. However, local flaps can be much more difficult to design. We designed 2 templates to plan a V-Y rotation advancement flap. The template for a unilateral V-Y rotation advancement flap was used on the face (n=5, anterior tibia (n=1, posterior axilla (n=1, ischium (n=1, and trochanter (n=2. The template for a bilateral flap was used on the sacrum (n=8, arm (n=1, and anterior tibia (n=1. The causes of the defects were meningocele (n=3, a decubitus ulcer (n=5, pilonidal sinus (n=3, and skin tumor excision (n=10. The meningocele patients were younger than 8 days. The mean age of the adult patients was 50.4 years (range, 19–80 years. All the donor areas of the flaps were closed primarily. None of the patients experienced wound dehiscence or partial/total flap necrosis. The templates guided surgeons regarding the length and the placement of the incision for a V-Y rotation advancement flap according to the size of the wound. In addition, they could be used for the training of residents.

  20. Case of Joubert syndrome. CT findings of brainstem and review of literature

    Energy Technology Data Exchange (ETDEWEB)

    Suzuki, Hisaharu; Nakazato, Akihiko; Ikota, Hiroko; Koide, Hiroyoshi (Saitama Medical School (Japan)); Yasaka, Atsushi; Nakada, Yoshitaka

    1983-01-01

    Joubert et al. first reported a familial syndrome which showed episodic tachypena, abnormal eye movement, mental and motor retardation. Since then eighteen cases have been reported in the world. In this paper, we reported a male baby with Joubert syndrome who was observed since the newborn period. He showed episodic tachypnea (respiratory rate over 100/min), apnea, severe mental and motor retardation, no normal eye movements, occipital meningocele, high arched palate and poor sucking. CT scan revealed vermian agenesis, hypoplasia and deformity of brainstem, enlarged fourth ventricle and cisterna magna. EEG showed episodic discharges. Laboratory test investigations including amino acids, lactate, pyruvate, ammonia, chromosomal analysis, IVP and renogram showed no abnormal findings. He showed poor development and at eleventh month of age he died at home because of respiratory arrest. Similar syndromes were reported by Koya et al., Dekaban, Gardner et al., D'Agostino et al. and Friede. They reported syndromes consisting of abnormal respiration, abnormal eye movements, mental and motor retardation, occipital meningocele, retinal degeneration and polycystic kidney. Some causative events may have occurred at 6 to 7 weeks of gestation affecting central nervous system as well as other organs.

  1. Cancer of the breast and anterior sacral meningeal in a patient with Marfan syndrome

    International Nuclear Information System (INIS)

    Cataldi, S.; Laureiro, E.; Musetti, C.; Vázquez, A.; Cabovianco, A.

    2004-01-01

    Introduction. Breast cancer is the most common malignancy of women in the world Western. It is rare below 30 years. Marfan syndrome (MS) is an entity clinically characterized by cardiovascular, ocular and skeletal genetic base. Its prevalence is estimated at 4-6 per 100,000 births. In the literature there are few reports of cancers diagnosed in association with SM, and after a thorough review, we found only two communications association with malformations such as dural sac meningocele. Objective. The aim of this study was to review the literature from communication A case report of a patient with SM in which the age of 24 he diagnosed with breast cancer and a previous sacral meningocele. Case. Female patient 24 years old, with SM, who consulted a tumor of right breast. Was studied with mammography and cytological puncture were positive for malignancy. Local treatment consisted of modified radical mastectomy and chest wall radiotherapy. The pathology corresponded to ductal carcinoma Infiltrating (CDI) NOS 27mm diameter greater final histologic grade II carcinoma in situ solid and cribriform intermediate grade without necrosis greater than 30%; 10 axillary lymph resected, all free of metastases. The dosage of hormone receptors was frankly positive for both estrogen and progesterone. In sum CDI NOS stage IIA. the chest radiograph and bone scan showed no abnormalities and abdominal ultrasound He requested postoperatively revealed an abdominopelvic image 13 x 16 cm. positron abdomen and pelvis confirmed a predominantly cystic mass in the pelvis and abdomen lower. Exploratory laparotomy revealed that the tumor corresponded to a meningocele before and proceeded to peritoneal cyst resection and closure of the sacral gap. At the time of writing, the patient is free and without neurological deficit disease, low adjuvant Tamoxifen for 3 years. Conclusions. The SM as breast cancer in younger women is uncommon. Few cancers have been reported in association with SM. Some of

  2. Drainage or Packing of the Sella? Transsphenoidal Surgery for Primary Pituitary Abscess: Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Soichi Oya

    2009-01-01

    Full Text Available The detailed surgical procedure of the transsphenoidal surgery for pituitary abscess has scarcely been described previously because it is a very rare clinical entity. The authors reported two cases of primary pituitary abscess. In case 1, the anterior wall of the sella turcica was reconstructed with the vomer bone after irrigating the abscess cavity, but the sella was not packed by fat for fear of the persistent infection by devascularized tissues. This led to the postoperative meningocele, the cerebrospinal fluid leak, and bacterial meningitis despite the successful abscess drainage. In case 2, tight sellar packing and reconstruction of the sellar wall were performed to avoid these postoperative complications, which resulted in complete drainage and uneventful postoperative course. Although accumulation of more cases is obviously needed to establish the definitive surgical technique in pituitary abscess surgery, our experience might suggest that packing of the sella is not impeditive for postoperative sufficient drainage.

  3. Ultrasonography of Midline Scalp Masses : A Preliminary Report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Hyo Kyeong; Lee, Ho Kyu; Choi, Choong Gon; Kim, Kyeong Sook; Jung, Seung Mun; Suh, Dae Chul [Asan Medical Center University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    1995-06-15

    We report our ultrasonographic experiences in the evaluation of small midline scalp masses. Ultrasonography was performed in four patients with midline scalp mass less than 3cm and its connecting tract into the cranialcavity. Ultrasonographic findings were correlated with pathologic results. There were three cephaloceles and one cystic lymph angioma. Two encephalomenin-goceles, located in occipital region, were mixed cystic and solid lesions and one atretic meningocele in parietal region was a solid lesion. Ultrasonogram of all three cases showed calvarial defect and connecting tract into the cranial cavity. On the contrary, a cystic lymph angioma in occipital region was a purely cystic mass without an associated calvarial defect. We suppose that ultrasonography could be a useful screening tool in the evaluation of midline scalp masses

  4. Efficacies of various diagnostic modalities in acute trauma of the cervical spine

    Energy Technology Data Exchange (ETDEWEB)

    Tani, Ichiro (St. Marianna Univ., Kawasaki (Japan). School of Medicine)

    1993-12-01

    The author reviewed 71 consecutive cases of acute trauma of the cervical spine to define efficacies of various modalities such as plain film, CT and MRI. Pathologies on CT and/or MRI additionally found to plain films were analyzed and correlated to three groups divided according to neurological deficit. The following conclusions were obtained. The usefulness of plain films as the first modality of choice was confirmed. Both CT and MRI are necessary in addition to abnormal plain films in this group if patients have neurological deficit. MRI may be a modality of choice following plain films if they are negative. In the group of brachial plexus palsy MRI should be performed before CT to demonstrate traumatic meningocele. CT myelography is also useful although it is invasive. It is warranted to say that in the asymptomatic group CT and MRI are not indicated, because additionally found abnormalities are clinically insignificant. (author).

  5. Dural ectasia of the optic nerve sheath: is it always benign?

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    Berker Bakbak

    2009-11-01

    Full Text Available Berker Bakbak1, Hava Dönmez2, Tülay Kansu3, Hayyam Kiratli41Hacettepe University Institute of Neurological Sciences and Psychiatry, Neuro-ophthalmology Unit, Ankara, Turkey; 2Diskapi Yildirim Beyazid Education and Research Hospital Neurology Clinic, Ankara, Turkey; 3Hacettepe University Medical Faculty, Department of Neurology, Neuro-Ophthalmology Unit, Ankara, Turkey; 4Hacettepe University Medical Faculty, Department of Ophthalmology, Ocular Oncology Unit, Ankara, TurkeyAbstract: A 36-year-old woman with a 3-month history of progressive visual loss had papilledema, dilatation of the optic nerve sheaths and normal cerebrospinal fluid pressures. She was diagnosed as dural ectasia of the optic nerve sheaths and surgical decompression was performed. In this case report, severe visual loss is described as a serious complication of this rare disease and the importance of early surgical intervention is emphasized.Keywords: optic nerve, dural ectasia, meningocele

  6. Neural Tube Defects and Pregnancy

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    Emine Çoşar

    2009-09-01

    Full Text Available OBJECTIVE: Neural tube defects are congenital malformations those mostly causing life-long morbidities. They are prevented by the periconseptional folic acid usage and prenatal diagnostic methods. MATERIALS-METHODS: Pregnants from Afyonkarahisar and neighbourhood cities applied to our hospital and determined NTD, were investigated. RESULTS: In our obstetrics clinic 1403 delivery were made and 43 of them had fetus with NTD. Among these fetuses 41.3% had meningomyelocel, 17.4% had meningocel, 21.7% had encephalocel, 8.7% had unencephali and 4.3% had iniencephali. CONCLUSION: Incidence of NTD is high in our region and geographic region, nutrition and other socioeconomic factors may be related to the high incidence. Education of the mother and periconceptional folic acid usage may reduce teh incidence of NTD.

  7. A case of double meningomyelocoele with hydrocephalous in a four months′ infant

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    Anuja A Goyal

    2013-01-01

    Full Text Available Meningomyelocoele is a developmental congenital disorder caused by failure of the neural tube to close during the first month of embryonic development thus presenting with herniation of neural elements along with meninges. Myelomeningocoeles most commonly occur in the lumbosacral region, but can also occur at any level in the neuraxis, including occipital/suboccipital areas or nasally (encephalocoele.We report a rare case of double meningomyelocoele in a 4-month-old infant (cervical as well as thoracic meningocele with weakness in the left lower limb (power grade 0 posted for resection with difficult intubation because of obstructive hydrocephalus, fear of rupture of neuroplaque, positioning and care of blood loss and temperature. The infant was discharged within 15 days, and his hospital stay was uneventful.

  8. Massa paranasal: dacrioestenose congênita? Relato de caso Paranasal mass: congenital dacryostenosis? Case report

    Directory of Open Access Journals (Sweden)

    Claudia Akemi Shiratori

    2004-10-01

    Full Text Available O objetivo deste é descrever uma criança portadora de massa paranasal, atentando para a importância dos diagnósticos diferenciais. RELATO DO CASO: ACS, 6 meses, sexo feminino, desde o nascimento apresentando abaulamento não inflamatório, no canto medial do olho esquerdo, lacrimejamento e hiperemia no olho direito. Ao exame apresentava fenômeno de Bell negativo bilateral, lagoftalmo à direita, ulceração e opacidade corneana à direita; presença de lesão arredondada, de superfície lisa no canto medial do olho esquerdo, sem sinais inflamatórios, medindo aproximadamente 2 cm de diâmetro, não pulsátil. À palpação, a lesão era elevada, de consistência fibroelástica, imóvel, indolor, irredutível. À propedêutica das vias lacrimais, não havia refluxo à compressão, o teste de Milder foi negativo em ambos olhos e as vias apresentavam-se pérvias à dacriocistografia. O exame tomográfico revelou tratar-se de meningocele fronto-etmoidal. COMENTÁRIOS: Os autores chamam a atenção para a adequada semiologia para a investigação das massas paranasais, a fim de se instituir o adequado tratamento.Report of a child presenting a paranasal mass, and discussion of the importance of the differential diagnosis. CASE REPORT: ACS, 6 months old, female, presenting a non inflammatory nodulation on the left medial canthus; tearing and redness in the right eye since birth. On examination, there were bilateral lagophthalmos and corneal ulceration and opacity at the right side; on the left medial canthus there was a rounded lesion with a smooth surface, without inflammation, with an approximately 2-cm diameter. On palpation, the lesion was elevated, fibroelastic, non-mobile, painless, and irreductible. Tear or discharge reflux was absent on lacrimal pathway compression, Milder's test was negative on both sides. Dacryocystographic examination showed normal lacrimal drainage of the paranasal sinus system. Computadorized tomography revealed a

  9. Partial craniofacial duplication: a review of the literature and case report.

    Science.gov (United States)

    Costa, Melinda A; Borzabadi-Farahani, Ali; Lara-Sanchez, Pedro A; Schweitzer, Daniela; Jacobson, Lia; Clarke, Noreen; Hammoudeh, Jeffery; Urata, Mark M; Magee, William P

    2014-06-01

    Diprosopus (Greek; di-, "two" + prosopon, "face"), or craniofacial duplication, is a rare craniofacial anomaly referring to the complete duplication of facial structures. Partial craniofacial duplication describes a broad spectrum of congenital anomalies, including duplications of the oral cavity. This paper describes a 15 month-old female with a duplicated oral cavity, mandible, and maxilla. A Tessier type 7 cleft, midline meningocele, and duplicated hypophysis were also present. The preoperative evaluation, surgical approach, postoperative results, and a review of the literature are presented. The surgical approach was designed to preserve facial nerve innervation to the reconstructed cheek and mouth. The duplicated mandible and maxilla were excised and the remaining left maxilla was bone grafted. Soft tissue repair included closure of the Tessier type VII cleft. Craniofacial duplication remains a rare entity that is more common in females. The pathophysiology remains incompletely characterized, but is postulated to be due to duplication of the notochord, as well as duplication of mandibular growth centres. While diprosopus is a severe deformity often associated with anencephaly, patients with partial duplication typically benefit from surgical treatment. Managing craniofacial duplication requires a detailed preoperative evaluation as well as a comprehensive, staged treatment plan. Long-term follow up is needed appropriately to address ongoing craniofacial deformity. Published by Elsevier Ltd.

  10. Imaging review of cerebrospinal fluid leaks.

    Science.gov (United States)

    Vemuri, Naga V; Karanam, Lakshmi S P; Manchikanti, Venkatesh; Dandamudi, Srinivas; Puvvada, Sampath K; Vemuri, Vineet K

    2017-01-01

    Cerebrospinal fluid (CSF) leak occurs due to a defect in the dura and skull base. Trauma remains the most common cause of CSF leak; however, a significant number of cases are iatrogenic, and result from a complication of functional endoscopic sinus surgery (FESS). Early diagnosis of CSF leak is of paramount importance to prevent life-threatening complications such as brain abscess and meningitis. Imaging plays a crucial role in the detection and characterization of CSF leaks. Three-dimensional, isotropic, high resolution computed tomography (HRCT) accurately detects the site and size of the bony defect. CT cisternography, though invasive, helps accurately identify the site of CSF leak, especially in the presence of multiple bony defects. Magnetic resonance imaging (MRI) accurately detects CSF leaks and associated complications such as the encephaloceles and meningoceles. In this review, we emphasize the importance and usefulness of 3D T2 DRIVE MR cisternography in localizing CSF leaks. This sequence has the advantages of effective bone and fat suppression, decreased artefacts, faster acquisition times, three-dimensional capability, y and high spatial resolution in addition to providing very bright signal from the CSF.

  11. Imaging review of cerebrospinal fluid leaks

    Directory of Open Access Journals (Sweden)

    Naga V Vemuri

    2017-01-01

    Full Text Available Cerebrospinal fluid (CSF leak occurs due to a defect in the dura and skull base. Trauma remains the most common cause of CSF leak; however, a significant number of cases are iatrogenic, and result from a complication of functional endoscopic sinus surgery (FESS. Early diagnosis of CSF leak is of paramount importance to prevent life-threatening complications such as brain abscess and meningitis. Imaging plays a crucial role in the detection and characterization of CSF leaks. Three-dimensional, isotropic, high resolution computed tomography (HRCT accurately detects the site and size of the bony defect. CT cisternography, though invasive, helps accurately identify the site of CSF leak, especially in the presence of multiple bony defects. Magnetic resonance imaging (MRI accurately detects CSF leaks and associated complications such as the encephaloceles and meningoceles. In this review, we emphasize the importance and usefulness of 3D T2 DRIVE MR cisternography in localizing CSF leaks. This sequence has the advantages of effective bone and fat suppression, decreased artefacts, faster acquisition times, three-dimensional capability, y and high spatial resolution in addition to providing very bright signal from the CSF.

  12. [Bath Plug Closure Method for Cerebrospinal Fluid Leakage by Endoscopic Endonasal Approach:Cooperative Treatment by Neurosurgeons and Otolaryngologists].

    Science.gov (United States)

    Kawaguchi, Tomohiro; Arakawa, Kazuya; Nomura, Kazuhiro; Ogawa, Yoshikazu; Katori, Yukio; Tominaga, Teiji

    2017-12-01

    Endoscopic endonasal surgery, an innovative surgical technique, is used to approach sinus lesions, lesions of the skull base, and intradural tumors. The cooperation of experienced otolaryngologists and neurosurgeons is important to achieve safe and reliable surgical results. The bath plug closure method is a treatment option for patients with cerebrospinal fluid(CSF)leakage. Although it includes dural and/or intradural procedures, surgery tends to be performed by otolaryngologists because its indications, detailed maneuvers, and pitfalls are not well recognized by neurosurgeons. We reviewed the cases of patients with CSF leakage treated by using the bath plug closure method with an endoscopic endonasal approach at our institution. Three patients were treated using the bath plug closure method. CSF leakage was caused by a meningocele in two cases and trauma in one case. No postoperative intracranial complications or recurrence of CSF leakage were observed. The bath plug closure method is an effective treatment strategy and allows neurosurgeons to gain in-depth knowledge of the treatment options for CSF leakage by using an endoscopic endonasal approach.

  13. Neural tube defects in Waardenburg syndrome: A case report and review of the literature.

    Science.gov (United States)

    Hart, Joseph; Miriyala, Kalpana

    2017-09-01

    Waardenburg syndrome type 1 (WS1) is an autosomal dominant genetic condition characterized by sensorineural deafness and pigment abnormalities, and is caused by variants in the PAX3 homeodomain. PAX3 variants have been associated with severe neural tube defects in mice and humans, but the frequency and clinical manifestations of this symptom remain largely unexplored in humans. Consequently, the role of PAX3 in human neural tube formation remains a study of interest, for clinical as well as research purposes. Though the association between spina bifida and WS1 is now well-documented, no study has attempted to characterize the range of spina bifida phenotypes seen in WS. Spina bifida encompasses several diagnoses with a wide scope of clinical severity, ranging from spina bifida occulta to myelomeningocele. We present a patient with Waardenburg syndrome type 1 caused by a novel missense variant in PAX3, presenting with myelomeningocele, Arnold-Chiari malformation, and hydrocephalus at birth. Additionally, we review 32 total cases of neural tube defects associated with WS. Including this report, there have been 15 published cases of myelomeningocele, 10 cases of unspecified spina bifida, 3 cases of sacral dimples, 0 cases of meningocele, and 4 cases of miscellaneous other neural tube defects. Though the true frequency of each phenotype cannot be determined from this collection of cases, these results demonstrate that Waardenburg syndrome type 1 carries a notable risk of severe neural tube defects, which has implications in prenatal and genetic counseling. © 2017 Wiley Periodicals, Inc.

  14. Limb/pelvis hypoplasia/aplasia with skull defect (Schinzel phocomelia): distinctive features and prenatal detection.

    Science.gov (United States)

    Olney, R S; Hoyme, H E; Roche, F; Ferguson, K; Hintz, S; Madan, A

    2001-11-01

    Schinzel phocomelia syndrome is characterized by limb/pelvis hypoplasia/aplasia: specifically, intercalary limb deficiencies and absent or hypoplastic pelvic bones. The phenotype is similar to that described in a related multiple malformation syndrome known as Al-Awadi/Raas-Rothschild syndrome. The additional important feature of large parietooccipital skull defects without meningocele, encephalocele, or other brain malformation has thus far been reported only in children with Schinzel phocomelia syndrome. We recently evaluated a boy affected with Schinzel phocomelia born to nonconsanguineous healthy parents of Mexican origin. A third-trimester fetal ultrasound scan showed severe limb deficiencies and an absent pelvis. The infant died shortly after birth. Dysmorphology examination, radiographs, and autopsy revealed quadrilateral intercalary limb deficiencies with preaxial toe polydactyly; an absent pelvis and a 7 x 3-cm skull defect; and extraskeletal anomalies including microtia, telecanthus, micropenis with cryptorchidism, renal cysts, stenosis of the colon, and a cleft alveolar ridge. A normal 46,XY karyotype was demonstrated, and autosomal recessive inheritance was presumed on the basis of previously reported families. This case report emphasizes the importance of recognizing severe pelvic and skull deficiencies (either post- or prenatally) in differentiating infants with Schinzel phocomelia from other multiple malformation syndromes that feature intercalary limb defects, including thalidomide embryopathy and Roberts-SC phocomelia. Copyright 2001 Wiley-Liss, Inc.

  15. [Our experience about the use of resorbable plates in the treatment of craniostenosis].

    Science.gov (United States)

    Grassiot, B; Delabar, V; Szathmari, A; Beuriat, P A; Paulus, C; Mottolese, C

    2015-09-01

    The use of resorbable plates increases for craniosynostosis surgery. This material, based on polymere (PLA, PGA) can replace steel wire and non resorbable plates. A few studies present surgical results about the use of this material with a long follow-up. We present our ten years experience of using resorbable material for craniosynostosis treatment in children. Between 2002 and 2012, we operated 283 craniosynostosis (98 scaphocephalies, 55 trigonocephalies, 79 plagiocephalies et 51 craniofaciostenoses). Among these surgeries, 211 were realized with resorbable material (plates and screws). Different criteria were observed: the esthetic result, the infection rate, the re-intervention, the bone defects and the inflammatory granuloma. Among the 211 craniosynostosis, we found 62 plagiocephalies, 66 scaphocephalies, 50 trigonocephalies, 33 craniofaciostenoses. All the reconstructions were realized with the same resorbable material (Macropore by Medtronic). The rate of complications was low: one scar infection without participation of material for two patients (0.9%), a pseudo-meningocele for two patients (0.9%), epilepsy for four children (1.8%) and bone defect for 15 (7%). We observed no granuloma for these patients. Our experience of ten years using resorbable material is very satisfactory. This material permits to realize solid and esthetic reconstructions with a low rate of infection without dangerous reaction for children in young age. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  16. Giant pseudomeningocele causing urinary obstruction in a patient with Marfan syndrome.

    Science.gov (United States)

    Stone, Jeremy G; Bergmann, Liisa L; Takamori, Ryan; Donovan, Daniel J

    2015-07-01

    Defective collagen biosynthesis in Marfan syndrome predisposes to dural defects such as dural ectasia, meningocele, and pseudomeningocele; thus, an increased index of suspicion for these conditions should be present in the clinical setting of Marfan syndrome. The authors describe a young woman with Marfan syndrome who was being treated with anticoagulants for a prosthetic heart valve and who presented with a spontaneous retroperitoneal hemorrhage requiring surgical evacuation. No CSF leak was encountered at surgery, but she developed progressively more severe positional headaches over the following year. She then experienced the sudden onset of acute urinary obstruction, at which time CT revealed a 17 × 15 × 13-cm presacral pseudomeningocele communicating with the thecal sac through a sacral bone defect. An anterior surgical approach was used for drainage of the pseudomeningocele as well as for primary closure of the dural defect with a bovine pericardial patch and autologous subcutaneous fat graft. After a short period of lumbar subarachnoid drainage of the CSF, the patient was able to resume normal activity without recurrent symptoms. To the authors' knowledge, such a pseudomeningocele in a patient with Marfan syndrome has been reported only twice, and this case features the largest pseudomeningocele to date. They also review the pertinent literature regarding presentation, diagnosis, and management of these lesions.

  17. Lumbar CT findings of patients with low back pain

    International Nuclear Information System (INIS)

    Lee, Yong Chul; Kim, Yang Soo; Kim, Kyun Sang

    1986-01-01

    Low back pain is probably the second most common disease entity to upper respiratory infection in developed country. We were missing at least 50% of the pathologic conditions by using conventional diagnostic modalities in low back pain. They did tell us nothing or little about facet abnormalities, lateral recesses, vertebral canal and soft tissue surrounding lumbar spines. High resolutional CT has been the biggest turning point in the diagnosis and management of low back pain. CT make a contribution to reducing the morbidity and probably the cost of evaluating patients with low back pain, and to increasing diagnostic accuracy. We observed 100 cases of lumbar CT using TCT 80A scanner for the evaluation of low back pain during the period from Apr. 1985 to Sept. 1985 at Chung-Ang University Hospital. Lumbar CT scan reveals high-positive findings (98%) in low back pain patients. Common low back disorders in CT are disc bulging (53%), herniated nucleus pulposus (32%), degenerative arthritis in posterior facet joints (27%), spinal stenosis (20%) and postoperative spines (15%). Uncommon low back disorders in CT are compression fracture of vertebral bodies, spondylolysis or spondylolisthesis, tropism, transitional vertebra, Scheueman's disease, limbic fracture, transverse process or articular process fracture, sacroiliac joint subluxation, conjoined nerve root and meningocele.

  18. Fetal magnetic resonance imaging in prenatal diagnosis of central nervous system abnormalities

    International Nuclear Information System (INIS)

    Morioka, Takato; Hashiguchi, Kimiaki; Kawamura, Tadao; Mihara, Futoshi; Hikino, Shunji; Nagata, Hideaki; Iwaki, Toru; Sasaki, Tomio

    2005-01-01

    The diagnostic value of fetal magnetic resonance imaging (MRI), performed in 42 pregnant women whose fetuses had structural abnormalities of the central nervous system identified with transabdominal ultrasonography from 1995 through 2002, was analyzed retrospectively. Half-Fourier acquisition single-shot turbo spin-echo (HASTE) T 2 -weighted imaging clearly delineated the cerebrospinal fluid (CSF) space and the malformed brain and spinal cord and provided valuable information for the diagnosis of structural abnormalities related to the CSF space, such as spina bifida with Chiari type II malformation (7 cases), colpocephaly with agenesis of the corpus callosum (7 cases), holoprosencephaly (6 cases), porencephaly (2 cases), lissencephaly with hydrocephalus (2 cases), and middle fossa arachnoid cyst (1 case). However, some difficulty was encountered in the diagnosis of rare pathologic conditions that were not related to the CSF space, such as epignathus, multiple arteriovenous fistulae, trapped suboccipital meningocele, and Turner syndrome. We conclude that HASTE T 2 -weighted imaging, which provides useful diagnostic images in a reasonable time, is a useful adjunct to ultrasonography to confirm or exclude certain abnormalities related to the CSF space. (author)

  19. Lumbar CT findings of patients with low back pain

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yong Chul; Kim, Yang Soo; Kim, Kyun Sang [Chung-Ang University, Seoul (Korea, Republic of)

    1986-04-15

    Low back pain is probably the second most common disease entity to upper respiratory infection in developed country. We were missing at least 50% of the pathologic conditions by using conventional diagnostic modalities in low back pain. They did tell us nothing or little about facet abnormalities, lateral recesses, vertebral canal and soft tissue surrounding lumbar spines. High resolutional CT has been the biggest turning point in the diagnosis and management of low back pain. CT make a contribution to reducing the morbidity and probably the cost of evaluating patients with low back pain, and to increasing diagnostic accuracy. We observed 100 cases of lumbar CT using TCT 80A scanner for the evaluation of low back pain during the period from Apr. 1985 to Sept. 1985 at Chung-Ang University Hospital. Lumbar CT scan reveals high-positive findings (98%) in low back pain patients. Common low back disorders in CT are disc bulging (53%), herniated nucleus pulposus (32%), degenerative arthritis in posterior facet joints (27%), spinal stenosis (20%) and postoperative spines (15%). Uncommon low back disorders in CT are compression fracture of vertebral bodies, spondylolysis or spondylolisthesis, tropism, transitional vertebra, Scheueman's disease, limbic fracture, transverse process or articular process fracture, sacroiliac joint subluxation, conjoined nerve root and meningocele.

  20. Poorly understood and often miscategorized congenital umbilical cord hernia: an alternative repair method.

    Science.gov (United States)

    İnce, E; Temiz, A; Ezer, S S; Gezer, H Ö; Hiçsönmez, A

    2017-06-01

    Umbilical cord hernia is poorly understood and often miscategorized as "omphalocele minor". Careless clamping of the cord leads to iatrogenic gut injury in the situation of umbilical cord hernia. This study aimed to determine the characteristics and outcomes of umbilical cord hernias. We also highlight an alternative repair method for umbilical cord hernias. We recorded 15 cases of umbilical cord hernias over 10 years. The patients' data were retrospectively reviewed, and preoperative preparation of the newborn, gestational age, birth weight, other associated malformations, surgical technique used, enteral nutrition, and length of hospitalization were recorded. This study included 15 neonates with umbilical cord hernias. The mean gestational age at the time of referral was 38.2 ± 2.1 meningocele, club foot, and choanal atresia. Surgical exploration was carried out in all of the neonates and they underwent corrective surgery with good outcomes. In an umbilical cord hernia, the body folds develop normally and form the umbilical ring. The double purse-string technique is easy to apply and produces satisfactory cosmetic results in neonates with umbilical cord hernias.

  1. Imaging in spine and spinal cord malformations

    International Nuclear Information System (INIS)

    Rossi, Andrea; Biancheri, Roberta; Cama, Armando; Piatelli, Gianluca; Ravegnani, Marcello; Tortori-Donati, Paolo

    2004-01-01

    Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis

  2. Co-infusion of autologous adipose tissue derived neuronal differentiated mesenchymal stem cells and bone marrow derived hematopoietic stem cells, a viable therapy for post-traumatic brachial plexus injury: A case report

    Directory of Open Access Journals (Sweden)

    Umang G Thakkar

    2014-08-01

    Full Text Available Stem cell therapy is emerging as a viable approach in regenerative medicine. A 31-year-old male with brachial plexus injury had complete sensory-motor loss since 16 years with right pseudo-meningocele at C5-D1 levels and extra-spinal extension up to C7-D1, with avulsion on magnetic resonance imaging and irreversible damage. We generated adipose tissue derived neuronal differentiated mesenchymal stem cells (N-AD-MSC and bone marrow derived hematopoietic stem cells (HSC-BM. Neuronal stem cells expressed β-3 tubulin and glial fibrillary acid protein which was confirmed on immunofluorescence. On day 14, 2.8 ml stem cell inoculum was infused under local anesthesia in right brachial plexus sheath by brachial block technique under ultrasonography guidance with a 1.5-inch-long 23 gauge needle. Nucleated cell count was 2 × 10 4 /μl, CD34+ was 0.06%, and CD45-/90+ and CD45-/73+ were 41.63% and 20.36%, respectively. No untoward effects were noted. He has sustained recovery with re-innervation over a follow-up of 4 years documented on electromyography-nerve conduction velocity study.

  3. Nuclear scintigraphy of the lower genitourinary tract in children

    International Nuclear Information System (INIS)

    Leonard, J.C.

    1987-01-01

    A primary goal of this review is to try to increase the reader's understanding of several clinical problems that present primarily in children. A second goal is to increase the reader's understanding of the applicability of nuclear cystography with manometry and the practical as well as potential aspects of testicular and varicocele imaging, while providing sufficient reference material to facilitate further study in specific areas of interest. If these goals are achieved, then the authors final goal, an increased awareness and utilization of these techniques by clinicians, can be accomplished by interested and convinced colleagues. A major portion of this review focuses on nuclear cystography, in particular, its role in the evaluation of patients with known urinary tract infections, patients with known or suspected vesicoureteral reflux who are siblings of patients with vesicoureteral reflux, and patients with neurogenic bladder in general and myelo-meningocele in particular. Attention is given to the association of GU tract anomalies with gastrointestinal tract anomalies, focusing on imperforate anus as a common example. Testicular imaging, in its conventional mode for evaluating the acute, painful scrotum, is presented, but, in addition, radionuclide venography to detect varicoceles are reviewed. Whereas this technique has been used in the investigation of male infertility, it appears to have considerable potential for assessment of adolescent males with asymptomatic, swollen testicals or those with evidence of testicular atrophy

  4. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification

    International Nuclear Information System (INIS)

    Tortori-Donati, P.; Rossi, A.; Cama, A.

    2000-01-01

    Our purpose was to review the neuroradiological features of spinal dysraphism and to correlate them with clinical findings and up-to-date embryological theory. We also aimed to formulate a working classification which might prove useful in clinical practice. We reviewed series of 986 children referred to our Spina Bifida Centre in the past 24 years. There were 353 children with open spinal (OSD) and 633 with closed (skin-covered) spinal (CSD) dysraphism. By far the most common open abnormality was myelomeningocele, and all patients with OSD had a Chiari II malformation. CSD was categorised clinically, depending on the presence of a subcutaneous mass in the back. CSD with a mass mainly consisted of lipomas with dural defects and meningoceles, and accounted for 18.8 % of CSD. CSD without a mass were simple (tight filum terminale, intradural lipoma) or complex (split cord malformations, caudal regression). Our suggested classification is easy to use and to remember and takes into account clinical and MRI features; we have found it useful and reliable when making a preoperative neuroradiological diagnosis in clinical practice. (orig.)

  5. An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain

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    Shotaro Kanda

    2015-01-01

    Full Text Available Adult tethered cord syndrome (ATCS is a rare entity that usually presents with multiple neurological symptoms, including lower extremity pain, backache, lower extremity muscle weakness, and bowel/bladder disturbances. Prompt surgical treatment is often necessary to avoid permanent sequelae. We report a 63-year-old man with sudden-onset severe right chest and upper back pain, followed by urinary retention. His initial workup included computed tomography of the abdomen and pelvis, which showed a presacral mass. His symptom-driven neurological workup focused on the cervical and thoracic spine, the results of which were normal. Pelvic radiographs and magnetic resonance imaging of the lumbosacral spine showed spina bifida occulta, meningocele, and presacral masses consistent with a teratomatous tumor. His symptoms, except for urinary retention, improved dramatically with surgical treatment. The excised specimen contained a teratomatous lesion plus an organized hematoma. Hematoma formation was suspected as the trigger of his sudden-onset right chest and upper back pain.

  6. 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

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    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

  7. Neurofibromatose tipo 1: aspectos radiológicos do tórax Type 1 neurofibromatosis: radiological findings of the chest

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    Marcos Pontes Muniz

    2010-06-01

    Full Text Available OBJETIVO: Identificar alterações e frequências nas radiografias simples do tórax sugestivas de neurofibromatose tipo 1 e avaliar a possibilidade de inclusão de massa no mediastino posterior como critério de diagnóstico de neurofibromatose tipo 1. MATERIAIS E MÉTODOS: Foram realizadas radiografias com técnica padrão de tórax em póstero-anterior e em perfil de 141 pacientes com neurofibromatose tipo 1, atendidos no Serviço de Radiologia do Hospital de Base e Faculdade de Medicina de São José do Rio Preto, SP. Os resultados obtidos foram avaliados por métodos não paramétricos ao nível de 0,05 de significância (p = 0,05. RESULTADOS: No presente estudo, 141 pacientes com neurofibromatose tipo 1 realizaram radiografia de tórax, sendo as alterações mais frequentes: erosão óssea das costelas (19,8%, peito escavado (12,0%, cifoescoliose (3,5% e massas no mediastino posterior (7,1%. Esses resultados sugerem que as massas (neurofibroma e meningocele devem ser incluídas como critério diagnóstico para neurofibromatose tipo 1, juntamente com displasia do osso esfenoide, pseudoartrose e afinamento do córtex de ossos longos, conforme definido pelo National Institutes of Health. CONCLUSÃO: A presença das massas no mediastino posterior associada às alterações ósseas características definidas pelo National Institutes of Health indicam ser um achado consistente para se considerar como critério diagnóstico da doença.OBJECTIVE: To identify chest radiography findings suggestive of type 1 neurofibromatosis, establishing their frequency and evaluating the possibility of including the presence of posterior mediastinal masses as a criterion for the diagnosis of type 1 neurofibromatosis. MATERIALS AND METHODS: The present study included 141 patients with type 1 neurofibromatosis assisted at the Service of Radiology of Hospital de Base and Faculdade de Medicina de São José do Rio Preto, SP, Brazil, and submitted to standard chest

  8. Disostose espôndilo-costal associada a defeitos de fechamento do tubo neural Spondylocostal dysostosis associated with neural tube defects

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    Rafael Fabiano M. Rosa

    2009-09-01

    Full Text Available OBJETIVO: Salientar a relação dos defeitos de fechamento do tubo neural com a disostose espôndilo-costal (DEC por meio da descrição de três pacientes. DESCRIÇÃO DOS CASOS: Paciente 1: menina branca, 22 meses, nascida com mielomeningocele lombar. Na avaliação, apresentava hipotonia, baixa estatura, dolicocefalia, fendas palpebrais oblíquas para cima, pregas epicânticas e tronco curto com tórax assimétrico. A avaliação radiográfica revelou hemivértebras múltiplas, vértebras em borboleta e fusão e ausência de algumas costelas. Paciente 2: menina branca, 22 meses, com moderado atraso do desenvolvimento neuropsicomotor, baixa estatura, olhos profundos, pregas epicânticas, pescoço e tronco curtos com assimetria do tórax, abdome protruso, hemangioma plano na altura da transição lombossacra e fosseta sacral profunda no dorso. A avaliação radiográfica identificou hemivértebras, fusão incompleta de vértebras e vértebras em borboleta, malformações de costelas e espinha bífida oculta em L5/S1. Paciente 3: menina branca, 9 dias de vida, com fendas palpebrais oblíquas para cima, ponte nasal alargada, orelhas baixo implantadas e rotadas posteriormente, tronco curto, tórax assimétrico e meningocele tóraco-lombar. A avaliação radiográfica evidenciou hemivértebras, malformação e ausência de algumas costelas e agenesia diafragmática à esquerda. A tomografia computadorizada de encéfalo mostrou estenose de aqueduto. COMENTÁRIOS: Vários defeitos de fechamento do tubo neural, de espinha bífida oculta a grandes mielomeningoceles, são observados em pacientes com DEC, indicando que tais pacientes devem ser cuidadosamente avaliados quanto à possível presença desses defeitos.OBJECTIVE: To highlight the relationship between neural tube defects and spondylocostal dysostosis (SCD through the description of three patients. CASES DESCRIPTION: Patient 1: white girl, 22 months old, born with a lumbar meningomyelocele. At

  9. Computed tomography (CT) findings in 88 neurofibromatosis 1 (NF1) patients: Prevalence rates and correlations of thoracic findings

    Energy Technology Data Exchange (ETDEWEB)

    Ueda, Ken, E-mail: k-ueda@radiol.med.osaka-u.ac.jp [Department of Radiology, Osaka University Graduate School of Medicine (Japan); Honda, Osamu [Department of Radiology, Osaka University Graduate School of Medicine (Japan); Satoh, Yukihisa [Department of Diagnostic Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases (Japan); Kawai, Misa; Gyobu, Tomoko; Kanazawa, Toru; Hidaka, Shojiro; Yanagawa, Masahiro [Department of Radiology, Osaka University Graduate School of Medicine (Japan); Sumikawa, Hiromitsu [Department of Diagnostic Radiology, Osaka Rosai Hospital (Japan); Tomiyama, Noriyuki [Department of Radiology, Osaka University Graduate School of Medicine (Japan)

    2015-06-15

    Highlights: • Various thoracic CT findings, including cysts, mediastinal masses, etc. were found. • Cysts show upper and peripheral dominant distribution. • The number, size, and distribution of the pulmonary cysts in NF-1 revealed significant correlation. • It is suspected that thoracic CT findings in NF-1 occur independently. - Abstract: Purpose: To evaluate the prevalence rates and the correlations of thoracic computed tomography (CT) findings of neurofibromatosis 1 (NF1) in 88 patients. Materials and methods: Chest CT images of 88 NF1 patients were independently reviewed by three observers, and the CT findings were evaluated. If abnormal findings were present, their number, size, and distribution were recorded. The prevalence rate of each CT finding was calculated, and the correlations between CT findings were analyzed. Results: Of the 88 cases, 13 were positive for cysts, 16 for emphysema, 8 for nodules, 8 for GGNs (ground glass nodules), 13 for mediastinal masses, 20 for scoliosis, 44 for subcutaneous nodules, and 34 for skin nodules. Cysts showed upper and peripheral dominant distributions. Regarding 13 mediastinal masses, 2 were diagnosed as malignant peripheral nerve sheath tumors (MPNSTs), 1 was diagnosed as primary lung cancer, 2 were diagnosed as lateral meningocele, 3 were diagnosed as neurofibromas, and the remaining 7 were considered neurofibromas. There was a significant correlation between the prevalence of subcutaneous nodules and that of skin nodules. Significant positive correlations were also seen between size and number, size and rate of central distribution, and number and rate of central distribution of cysts. Conclusion: Various CT findings were found in NF-1 patients, and the prevalence rates of subcutaneous and skin nodules were higher than other findings. Though the prevalence rates of subcutaneous nodules and skin nodules were significantly correlated, the other CT findings in NF-1 occurred independently. The number, size, and

  10. Clinical outcomes of temporary shunting for infants with cerebral pseudomeningocele.

    Science.gov (United States)

    Mattei, Tobias A; Sambhara, Deepak; Bond, Brandon J; Lin, Julian

    2014-02-01

    Although in the case of subdural collections temporary shunting has been suggested as a viable alternative for definitive drainage of the accumulated fluid until restoration of the normal CSF dynamics, there is no agreement on the best management strategy for pseudomeningocele. The authors performed a retrospective chart review in order to evaluate the clinical outcomes of infants temporarily shunted for pseudomeningocele without encephalocele at our institution (The University of Illinois at Peoria/Illinois Neurological Institute) in the period from 2004 to 2012. The epidemiological characteristics, clinical management, and final outcomes of such subpopulation were compared with a control group which received temporary shunting for subdural hematomas (SDH) during the same period. Four patients (100% male) ranging in age from 8.9 to 27.1 months (mean = 13.88) with pseudomeningocele and 17 patients (64.7% male) ranging in age from 1.9 to 11.8 months (mean = 4.15) with SDH were identified. Although the initial management included sequential percutaneous subdural tapping in 82% of the patients, all children ultimately failed such strategy, requiring either subdural-peritoneal (81% of the cases) or subgaleal-peritoneal (19% of the cases) shunting. The mean implant duration was 201 days for the pseudomeningocele group and 384 days for the SDH one. Mean post-shunt hospitalization was 2 days for patients with pseudomeningocele and 4 days for patients with SDH. There was no statistical difference in terms of complications, length of hospitalization post-shunting, or clinical outcomes between the patients with pseudomeningocele and those with SDH. Temporary shunting of infants with pseudo-meningocele constitutes a viable therapeutic alternative with favorable clinical outcomes and a low risk of shunt dependency similar to those of children with SDH.

  11. Differential diagnosis and diagnostic flow chart of joint hypermobility syndrome/ehlers-danlos syndrome hypermobility type compared to other heritable connective tissue disorders.

    Science.gov (United States)

    Colombi, Marina; Dordoni, Chiara; Chiarelli, Nicola; Ritelli, Marco

    2015-03-01

    Joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT) is an evolving and protean disorder mostly recognized by generalized joint hypermobility and without a defined molecular basis. JHS/EDS-HT also presents with other connective tissue features affecting a variety of structures and organs, such as skin, eye, bone, and internal organs. However, most of these signs are present in variable combinations and severity in many other heritable connective tissue disorders. Accordingly, JHS/EDS-HT is an "exclusion" diagnosis which needs the absence of any consistent feature indicative of other partially overlapping connective tissue disorders. While both Villefranche and Brighton criteria include such an exclusion as a mandatory item, a systematic approach for reaching a stringent clinical diagnosis of JHS/EDS-HT is still lacking. The absence of a consensus on the diagnostic approach to JHS/EDS-HT concerning its clinical boundaries with similar conditions contribute to limit our actual understanding of the pathologic and molecular bases of this disorder. In this review, we revise the differential diagnosis of JHS/EDS-HT with those heritable connective tissue disorders which show a significant overlap with the former and mostly include EDS classic, vascular and kyphoscoliotic types, osteogenesis imperfecta, Marfan syndrome, Loeys-Dietz syndrome, arterial tortuosity syndrome, and lateral meningocele syndrome. A diagnostic flow chart is also offered with the attempt to support the less experienced clinician in stringently recognizing JHS/EDS-HT and stimulate the debate in the scientific community for both management and research purposes. © 2015 Wiley Periodicals, Inc.

  12. Computed tomography (CT) findings in 88 neurofibromatosis 1 (NF1) patients: Prevalence rates and correlations of thoracic findings

    International Nuclear Information System (INIS)

    Ueda, Ken; Honda, Osamu; Satoh, Yukihisa; Kawai, Misa; Gyobu, Tomoko; Kanazawa, Toru; Hidaka, Shojiro; Yanagawa, Masahiro; Sumikawa, Hiromitsu; Tomiyama, Noriyuki

    2015-01-01

    Highlights: • Various thoracic CT findings, including cysts, mediastinal masses, etc. were found. • Cysts show upper and peripheral dominant distribution. • The number, size, and distribution of the pulmonary cysts in NF-1 revealed significant correlation. • It is suspected that thoracic CT findings in NF-1 occur independently. - Abstract: Purpose: To evaluate the prevalence rates and the correlations of thoracic computed tomography (CT) findings of neurofibromatosis 1 (NF1) in 88 patients. Materials and methods: Chest CT images of 88 NF1 patients were independently reviewed by three observers, and the CT findings were evaluated. If abnormal findings were present, their number, size, and distribution were recorded. The prevalence rate of each CT finding was calculated, and the correlations between CT findings were analyzed. Results: Of the 88 cases, 13 were positive for cysts, 16 for emphysema, 8 for nodules, 8 for GGNs (ground glass nodules), 13 for mediastinal masses, 20 for scoliosis, 44 for subcutaneous nodules, and 34 for skin nodules. Cysts showed upper and peripheral dominant distributions. Regarding 13 mediastinal masses, 2 were diagnosed as malignant peripheral nerve sheath tumors (MPNSTs), 1 was diagnosed as primary lung cancer, 2 were diagnosed as lateral meningocele, 3 were diagnosed as neurofibromas, and the remaining 7 were considered neurofibromas. There was a significant correlation between the prevalence of subcutaneous nodules and that of skin nodules. Significant positive correlations were also seen between size and number, size and rate of central distribution, and number and rate of central distribution of cysts. Conclusion: Various CT findings were found in NF-1 patients, and the prevalence rates of subcutaneous and skin nodules were higher than other findings. Though the prevalence rates of subcutaneous nodules and skin nodules were significantly correlated, the other CT findings in NF-1 occurred independently. The number, size, and

  13. Magnetic resonance imaging of spinal tumors. A study using a 0.3 T vertical magnetic field

    International Nuclear Information System (INIS)

    Li Ming Hua.

    1992-12-01

    A total of 168 patients with spinal tumors were evaluated with MRI. The study shows that MRI is a sensitive method for demonstration of spinal tumors. MRI also provides a possibility to separate different histological types of tumors based on their morphology and signal characteristics. Intramedullary tumors (25 cases): Ependymomas (6 cases) and astrocytomas (7 cases) were most common. Ependymomas have a more irregular signal pattern than astrocytomas. Astrocytomas are more common in the upper spine and are more often completely cystic. Contrast enhancement is important for separation of cyst, edema and solid tumor. Intradural extramedullary tumors (31 cases): Neuromas (14 cases) and meningeomas (11 cases) were most common. Neuromas always had markedly increased signal intensity on T2-weighted images. Meningeomas were only hyperintense occasionally. Neuromas were more inhomogeneous than meningeomas on T1-weighted images. Contrast enhancement is valuable for delineation of small tumors. Extradural tumors (91 cases): 76 patients had metastases, 7 primary spinal tumors and 8 multiple myelomas. T1-weighted images are almost always superior to other sequences because tumor invasion in the fatty bone marrow is seen as a low signal area in contrast to the high signal from the fat. Spinal lymphomas (14 cases): May be divided into vertebral, paraspinal and epidural tumors. Most cases have all locations. Spinal neurofibromatosis (7 cases): Most patients had multiple, often bilateral neurofibromas. One patient had a meningeomas on one spinal dysplasia with meningoceles. MRI is superior to other modalities for evaluation of the full extent of the disease. The coronal view is often valuable because of the arrangement of the tumors. In addition to providing diagnosis, MRI is of great value in treatment follow-up

  14. Agnathia-holoprosencephaly associated with a 46,XY,-21,+t(21q;21q) karyotype

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    Niedermeyer, K.K.; McCorquodale, M.M.; Burton, B.K. [Univ. of Illinois, Chicago, IL (United States)

    1994-09-01

    We report an unusual case of agnathia-holoprosencephaly associated with Down syndrome due to a 21/21 translocation. The patient presented prenatally at 21 wks gestation. A fetal ultrasound revealed multiple CNS anomalies including hydrocephalus, compressed cerebellum, absent septum pellucidum and possible cranial meningocele or encephalocele. High resolution ultrasound & fetal karyotype were recommended. The patient refused & elected to have a pregnancy termination. Chromosomal analysis performed on products of conception revealed a 46,XY,-21,+t(21q;21q) karyotype. Fluorescence in situ hybridization was performed and confirmed the 21/21 translocation chromosome. An autopsy revealed agnathia and multiple CNS anomalies including absence of the septum pellucidum, absence of the corpus callosum, arhinencephaly, an occiptal meningoencephalocele, dilation of the lateral ventricles, and extensive dysgenesis & heterotopias of the central cerebrum & mid-brain. Additional abnormalities included a persistent left superior vena cava, atrial & ventricular septal defects, irregular length of the fingers with absence of the middle phalanges of the right 2nd and 5th & left 5th digits and bilateral simian creases. Agnathia can be an isolated abnormality but often is associated with holoprosencephaly and/or situs inversus. The majority of familial case of agnathis-holoprosencephaly was caused by an inherited unbalanced translocation resulting in duplication of 6p and monosomy of 18p. Our patient had a translocation form of trisomy 21 but did not have a phenotype consistent with Down syndrome. Trisomy 21 has not been previously reported in other cases of agnathia-holoprosencephaly. Whether the chromosomal abnormality caused the phenotypic abnormalities or if it is a coincidental finding cannot be determined.

  15. Diagnostic value of multiplanar reconstruction in CT recognition of lumbar spinal disorders

    International Nuclear Information System (INIS)

    Im, S. K.; Choi, J. H.; Kim, C. H.; Sohn, M. H.; Lim, K. Y.; Choi, K. C.

    1984-01-01

    The computer tomography is useful in evaluation of bony structures and adjacent soft tissues of the lumbar spine. Recently, the multiplanar reconstruction of lumbar spine of CT of significant value for the anatomical localization and for the myelographic and surgical correlation. We observed 177 cases of lumbar spine CT, who complains of spinal symptom, during the period from Dec. 1982 to Aug. 1984. The results were as follows: 1. The sex distribution of cases were 113 males and 44 females. The CT diagnosis showed 152 cases of herniated lumbar disc, 15 cases of degenerative disease, 5 cases of spine tbc., 3 cases of spine trauma and 2 cases of meningocele. 2. CT findings of herniated disc were as follows: focal protrusion of posterior disc margin and obliteration of anterior epidural fat in all cases, indentation on dural sac in 92 cases (60.5%) soft tissue mass in epidural fat in 85 cases (55.9%), compression or displacement of nerve root sheath in 22 cases(14.4%). 3. Sites of herniated lumbar disc were at L4-L5 level in 100 cases(59.1%) and at L5-S1 level in 65 cases (38.4%). Location of it were central type in 70 cases(41.1%), left-central type in 46 cases (27.2%), right-central type in 44 cases(26.0%) and lateral type in 9 cases (5.1%). 4. The sagittal reconstruction images were helpful in evaluating neural foramina, size of disc bluge into spinal canal, especially at L5-S1, and patients with spondylolisthesis. The coronal reconstruction images were the least informative, although they contributed to the evaluation of lumbar nerve roots of course, the axial CT scans were the most sensitive and specific.

  16. Sonographic findings of normal newborn spinal cord

    International Nuclear Information System (INIS)

    Park, Chan Sup; Kim, Dong Gyu

    1988-01-01

    The authors performed spinal cord ultrasonography of 21 healthy newborn infants in Gyeongsang National University Hospital. Normal spinal cord revealed low echogenecity at that of cerebrospinal fluid and was demarcated by intense reflections from its dorsal and ventral surfaces. The central canal was routinely seen as a thin linear reflection in the center of the cord. The nerve roots making up the cauda equina formed a poorly defined collection of intense linear echoes extending from the conus. On real time image, the normal spinal cord exhibited rather slow and rhythmical anteroposterior movement within the subarachnoid fluid. A distinct and rapid vascular pulsation of the spinal cord was usually recognizable. The approximate level of vertebral bodies was determined as follows; most ventrally located vertebral body was thought to be L5 and S1 was seen slightly posterior to the L5 directed inferoposteriorly. According to the above criteria terminal portions of spinal cord were seen around the L2 body in 5 MHz and pointed termination of conus medullaris was clearly seen at L2-3 junction and in upper body of L3 by 7.5 MHz. So it would be better to examine by 5 MHz for spatial orientation and then by 7.5 MHz for more accurate examination. High-resolution, real-time ultrasonography was a safe, rapid screening technique for evaluation of the spinal cord in infants. Additional applications of spinal sonography may be possible in the evaluation of neonatal syringohydromyelia and meningocele as well as intraspinal cyst localization for possible percutaneous puncture by ultrasound guidance

  17. Neural tube defects – disorders of neurulation and related embryonic processes

    Science.gov (United States)

    Copp, Andrew J.; Greene, Nicholas D. E.

    2014-01-01

    Neural tube defects (NTDs) are severe congenital malformations affecting 1 in every 1000 pregnancies. ‘Open’ NTDs result from failure of primary neurulation as seen in anencephaly, myelomeningocele (open spina bifida) and craniorachischisis. Degeneration of the persistently open neural tube in utero leads to loss of neurological function below the lesion level. ‘Closed’ NTDs are skin-covered disorders of spinal cord structure, ranging from asymptomatic spina bifida occulta to severe spinal cord tethering, and usually traceable to disruption of secondary neurulation. ‘Herniation’ NTDs are those in which meninges, with or without brain or spinal cord tissue, become exteriorised through a pathological opening in the skull or vertebral column (e.g. encephalocele and meningocele). NTDs have multifactorial etiology, with genes and environmental factors interacting to determine individual risk of malformation. While over 200 mutant genes cause open NTDs in mice, much less is known about the genetic causation of human NTDs. Recent evidence has implicated genes of the planar cell polarity signalling pathway in a proportion of cases. The embryonic development of NTDs is complex, with diverse cellular and molecular mechanisms operating at different levels of the body axis. Molecular regulatory events include the BMP and Sonic hedgehog pathways which have been implicated in control of neural plate bending. Primary prevention of NTDs has been implemented clinically following the demonstration that folic acid, when taken as a peri-conceptional supplement, can prevent many cases. Not all NTDs respond to folic acid, however, and adjunct therapies are required for prevention of this folic acid-resistant category. PMID:24009034

  18. The cephaloceles: A clinical, epidemiological and therapeutic study of 50 cases.

    Science.gov (United States)

    Kabré, A; Zabsonre, D S; Sanou, A; Bako, Y

    2015-08-01

    Cephaloceles are congenital malformations characterized by externalization of the meninges and/or brain tissue through a congenital skull bone defect. In developed countries, this malformation is rare but in our developing countries, this pathology remains one of our concerns. To describe the epidemiological, clinical and therapeutic aspects of the cephaloceles. A retrospective study was conducted from 2007 to 2013 on all cases of cephalocele supported in the department of neurosurgery of the Yalgado Ouédraogo University Teaching Hospital of Ouagadougou. The malformations were supplemented by CT scan. All patients were operated. The surgical procedure in cephaloceles of the convexity consisted of a direct approach. Sincipital cephaloceles were operated by transcranial approach or combined approach associating transcranial and transnasal approaches. Fifty patients were gathered during the 7-year period. There were 18 boys and 32 girls. The ages ranged from 1 day to 11 years. The cephaloceles were located on the vault of the skull in 78%; the lesion was sincipital in 22%. The malformation was covered with normal skin in 92%; it was pediculated in 78%. CT scan allowed the classification of cases as meningo-encephaloceles in 31 cases (62%) and meningoceles in 18 cases (36%) and one pure encephalocele. The immediate postoperative course was uneventful in 42 cases (84%); 8 postoperative deaths were recorded. At medium and long term, 4 patients (8%) developed complications of psychomotor deficiency in 3 cases and hydrocephalus in 1 case. The main reasons for the poor prognosis were superinfection, hydrocephalus and/or other brain malformations. Cephaloceles remain relatively frequent in our practice. After surgery, mental failure and hydrocephalus can occur impairing the prognosis. The most suitable solution is a policy of prevention with folic acid treatment before and during pregnancy and following up adequate pregnancies. Copyright © 2015 Elsevier Masson SAS. All

  19. Defeitos congênitos em bovinos da Região Central do Rio Grande do Sul

    Directory of Open Access Journals (Sweden)

    J.T.S.A. Macêdo

    2011-04-01

    Full Text Available Foram revisados casos de defeitos congênitos (DCs diagnosticados em bovinos no Laboratório de Patologia da Universidade Federal de Santa Maria em 1964-2010. Durante o período estudado, foram examinados materiais provenientes da necropsia de 7.132 bovinos e foram encontrados 31 bezerros (0,4% com DCs, os quais foram classificados em 34 tipos e alocados nos sistemas orgânicos primariamente afetados. Os DCs ocorriam isoladamente (19 [61,3%] ou afetavam múltiplos sítios anatômicos (15 [28,7%] com frequência semelhante em ambos os sexos. Como vários terneiros mostraram múltiplos DCs, um total de 53 DCs foi computado. Dos 53 DCs diagnosticados, 15 (28,3% afetavam o sistema nervoso central (craniósquise [4], abiotrofia cerebelar [2], degeneração esponjosa [2], hidrocefalia [2], meningocele [2], espinha bífida [1], hipoplasia cerebelar [1] e hipomielinogênese [1]; nove (17,0% afetavam o sistema urogenital (agenesia testicular [1], agenesia vaginal [1], hipoplasia peniana [1], formação de cloaca [1], freemartinismo [1], hamartoma vascular de ovário [1], hipoplasia renal [1], cistos renais [1] e úraco persistente [1]; oito DCs (15,1% eram primários do sistema musculoesquelético (artrogripose [4], escoliose [1], plagiocefalia, [1] schistosomus reflexus [1] e diprosopia [1]; e outros oito (15,1% foram alocados no sistema digestivo (palatosquise [3], atresia anal [1], atresia anorretal [1], atresia - anocolônica [1], fístula reto-vaginal [1] e fístula reto-uretral [1]; em cinco ocasiões (9,4% o DC afetava o sistema cardiovascular (persistência do ducto arterioso [2], persistência do forame oval [2] e defeito do septo ventricular [1]; quatro (7,5% afetavam o sistema linfático e consistiam de hipoplasia ou aplasia de vasos linfáticos e linfonodos associadas a linfedema. Dois casos (3,4%, de hipotricose foram observados afetando o integumento; um caso (1,9% de estenose traqueal foi encontrado no sistema respiratório e um caso (1

  20. Investigation of Quality of Life in Mothers of Children with Spinabifida based on Children\\'s Hoffer Ambulatory Criteria

    Directory of Open Access Journals (Sweden)

    Hamid Dalvand

    2015-07-01

    Full Text Available Objective: Mothers of children with spinabifida spend more time to care for their children. Doing this care, depending on the child's functional mobility level can probably cause damage to physical and mental health of mothers. This study aimed to determine the quality of life (QOL of mothers of children with spinabifida (SB, according to the Hoffer levels using the SF-36 questionnaire. Materials & Methods: This research was a descriptive- analitical, cross-sectional study. The sample of this study was consisted of mothers of children with SB (2/5– 14 years old, in Tehran. The sample were selected by convenience sampling in the Rehabilitation Outpatient Clinics of University of Social Welfare and Rehabilitation Sciences, that 203 mothers were assessed in one clinic. Primery measures were the Hoffer criteria for grouping and determine the levels of ambulation in children with SB and the outcome measure was SF-36 questionnaire. Data were analyzed using, t test, ANOVA and MANOVA in SPSS (version 18. Results: In this study, there were significant differences in mean scores on the physical component summary (PCS (P<0/01 and Mental component summary (MCS of SF36 (P<0/01 in mothers of children with SB in Hoffer levels. The significant difference in PCS and MCS of SF36, was related to the level I with the other levels of Hoffer criteria, there were no Significant difference in mean score for the PCS and MCS the QOL of mothers for sex and child's age levels (P<0/05. There were a significant differences in mean PCS and MCS a QOL of mothers in different types of SB (P<0/01. This significant difference was related to aculta with meningocele and myelomeningocele (P<0/01. Conclusion: The dimensions of PCS and MCS of SF36 of mother with SB were affected with types of SB and increasing the levels of Hoffer. In conclusion, Mothers of children with SB require paying attention to their QOL during treatment of the child. In addition, the services should be

  1. Meningitis in Children: Evaluation of 197 Patients

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    Ali Gunes

    2016-01-01

    Full Text Available Aim: The aim of our study was to evaluate the epidemiologic, clinic and laboratory results and the answers to treatment of meningitis cases. Material and Method: In this study, the epidemiologic, clinic and laboratory results of 197 patients hospitalized with central nervous system infection diagnosis in the Department of Pediatric Health and Diseases of the Faculty of Medicine of Dicle University between 1st of January 2003 and 1st of January 2006 have been studied retrospectively. The files have been studied in details for age, sex, complaints, and results of physical examination, laboratory results, radiological results and treatments applied. Results: 118 of the patients were male, 79 were women and the mean age calculated was 62,2±47,3 months.137, 27 and 33 patients have been respectively considered as ABM, AM and TM. The most frequent complaints of application to hospital were fever (95,4%, vomiting (82,7%, headache (45,6% and change of consciousness (21,3%. The presence of many risks about meningitis has been observed. The most frequent risk factors were head trauma history, parenchymal lung tuberculosis, military tuberculosis, presence of V-P shunt, meningocele, varicella history, having mumps, and the presence of purulent ear discharge. BOS has developed in 7 patients and for five patients, reproduction occurred in blood culture. The most important central nervous system sequels or complications were in order of frequency hydrocephalies requiring the installation of V-P shunt, brain edema, epilepsies, subdural effusions, tuberculoma, retention of head pair, and brain apses. The rate of mortality was (% 13,1. Discussion: During the period of execution of the study, the mortality and morbidity of central nervous system diseases were still at high risk. But this may be associated to the absence of vaccination programs for frequent meningitis factors such as pneumococcus and H. influenza were not in routine vaccination program in our

  2. Malformações do sistema nervoso central e malformações associadas diagnosticadas pela ultrassonografia obstétrica

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    Marcela Leonardo Barros

    2012-12-01

    Full Text Available OBJETIVO: Identificar a prevalência de malformações congênitas do sistema nervoso central (SNC e malformações associadas diagnosticadas pela ultrassonografia obstétrica. MATERIAIS E MÉTODOS: Estudo observacional, transversal, descritivo, em instituição de referência para gestações de alto risco. RESULTADOS: Malformações congênitas do SNC estiveram presentes sem outras malformações associadas em 65,78%, com a distribuição: hidrocefalia (37,5%, mielomeningocele (15%, encefalocele (12,5%, agenesia de corpo caloso (12,5%, anencefalia (12,5%, holoprosencefalia (7,5%, Dandy-Walker (7,5%, Arnold-Chiari (5,0%, hidranencefalia (5,0%, meningocele (5,0%, cisto aracnoideo (2,5%. Malformações congênitas de outros sistemas estiveram associadas às do SNC: craniofacial (73,9%, ortopédica (65,2%, cardiovascular (34,8%, geniturinária (30,4%, gastrintestinal (30,4%, respiratória (8,7%, sindrômica (8,7, oftalmológica (4,3%. A sensibilidade ultrassonográfica no estudo de malformações fetais do SNC foi 79,4%. A taxa de falso-negativos foi 20,5%. Dentre as limitações quantificáveis destaca-se o oligodrâmnio, presente em 25% dos falso-negativos. CONCLUSÃO: A ultrassonografia obstétrica possui boa sensibilidade no rastreio de malformações fetais do SNC, em especial com o aperfeiçoamento constante e domínio na utilização de métodos especializados, como o Doppler e a ultrassonografia volumétrica (3D/4D, contribuindo para firmar-se como modalidade de escolha nesta rotina. Complementar ao método, a ressonância magnética pode vir a fornecer subsídios para uma ainda melhor assistência perinatal.

  3. Malformações congênitas em ruminantes no semiárido do Nordeste Brasileiro Congenital malformations in ruminants in the semiarid of the Brazilian Northeast

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    Antônio Flávio M Dantas

    2010-10-01

    Full Text Available Malformações congênitas causadas pela ingestão de Mimosa tenuiflora têm sido observadas em ruminantes no semiárido do Nordeste Brasileiro. Neste trabalho foram estudadas as malformações congênitas em ruminantes diagnosticadas entre 2000 e 2008, em municípios da Paraíba, Pernambuco e Rio Grande do Norte. Durante o período foram recebidos 1.347 materiais de ruminantes para diagnóstico, desses 47 (3,48% foram dignosticados como malformações congênitas. Com base no tipo de malformação e na procedência do animal as malformações foram divididas em: 1 causadas pelo consumo de M. tenuiflora; e 2 malformações esporádicas, sem causa conhecida. De 418 materiais de ovinos, 21 corresponderam a malformações, sendo 18 (4,3% do total de materiais de malformações causadas por M. tenuiflora e 3 (0,71% de malformações esporádicas. De 434 materiais de bovinos, 14 foram diagnosticados como malformações, sendo 8 (1,84% causadas por M. tenuiflora e 6 (1,38% malformações esporádicas. De 495 materiais de caprinos, 12 apresentaram malformações, sendo 9 (1,81% causadas pela ingestão de M. tenuiflora e 3 (0,6% malformações esporádicas. As principais malformações causadas por M. tenuiflora foram artrogripose, micrognatia, palatosquise, microftalmia e hipoplasia ou aplasia unilateral ou bilateral dos ossos incisivos. As malformações esporádicas incluiram: acefalia e hermafroditismo, dicefalia e malformações de vasos intestinais em ovinos; atresia anal em três caprinos; e hidranencefalia, atresia anal, malformações de costelas com eventração, hipoplasia cerebelar e hidrocefalia, coristoma pulmonar e meningocele, e gêmeos siameses em bovinos. O caso de hipoplasia cerebelar com hidrocefalia foi negativo pela imuno-histoquímica para o vírus da diarreia viral bovina. Malformações congênitas causadas por M. tenuiflora ocorreram durante todo o ano. A maior frequência em ovinos está aparentemente associada ao consumo da