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Sample records for ear malformation cardiospondylocarpofacial

  1. Congenital malformation of inner ear, single cavity

    International Nuclear Information System (INIS)

    Torres Pazmino, Julio Cesar; Marrugo Pardo, Gilberto Eduardo

    2010-01-01

    Congenital malformations of the inner ear are rare conditions, but their detection requires high diagnostic accuracy. In this report we describe the case of a patient with single or common cavity, discuss the corresponding radiological images, describe the treatment of this patient with a cochlear implant, and review the classification and differential diagnosis of the other anomalies of the inner ear.

  2. Multislice spiral computed tomography imaging in congenital inner ear malformations.

    Science.gov (United States)

    Ma, Hui; Han, Ping; Liang, Bo; Tian, Zhi-liang; Lei, Zi-qiao; Kong, Wei-jia; Feng, Gan-sheng

    2008-01-01

    The purpose of this study is to evaluate the usefulness of multislice spiral computed tomography (CT) in the diagnosis of congenital inner ear malformations. Forty-four patients with sensorineural hearing loss were examined on a Somatom Sensation 16 (Siemens) CT scanner. The 3-dimensional reconstructions and multiplanar reformation (MPR) were performed using the volume-rendering technique (VRT) on the workstation. Of the 44 patients examined for this study, 25 patients were found to be normal and 19 patients (36 ears) were diagnosed with congenital inner ear malformations. Of the malformations, the axial, MPR, and VRT images can all display the site and degree in 33 of the ears. Volume-rendering technique images were superior to the axial images in displaying the malformations in 3 ears with small lateral semicircular canal malformations. The common malformations were Michel deformity (1 ear), common cavity deformity (3 ears), incomplete partition I (3 ears), incomplete partition II (Mondini deformity) (5 ears), vestibular and semicircular canal malformations (14 ears), enlarged vestibular aqueduct (16 ears, 6 of which had other malformations), and internal auditory canal malformation (8 ears, all accompanied by other malformations). Multislice spiral CT allows a comprehensively assessment of various congenital inner ear malformations through high-quality MPR and VRT reconstructions. Volume-rendering technique images can display the site and degree of the malformation 3-dimensionally and intuitionisticly. This is very useful to the cochlear implantation.

  3. Clinical review of inner ear malformation

    International Nuclear Information System (INIS)

    Kokai, Hiromi; Oohashi, Masami; Ishikawa, Kazuo; Harada, Kouji; Hiratsuka, Hitoshi; Ogasawara, Makoto; Miyashita, Souji; Terayama, Yoshihiko

    2003-01-01

    We had 126 patients with inner ear malformation diagnosed with temporal bone computed tomography (CT) scans at Azabu Triology Hospital between 1996 and 2002. We classified cases of inner ear malformation according to Jackler et al. The incidence of inner ear malformation in our series was as follows; labyrinthine anomalies 61% (isolated lateral semicircular canal dysplasia 56%, compound semicircular canal dysplasia 4%, semicircular canal aplasia 1%), cochlear anomalies 24%, enlargement of the vestibular aqueduct 12%, narrow internal auditory canal 2%, complete labyrinthine aplasia 1%, enlargement of the cochlear aqueduct 0%. The most frequent anomaly was isolated lateral semicircular canal dysplasia. We did not detect any significant clinical features in this anomaly. There were 2 patients with cochlear anomalies who had past histories of meningitis. Some patients with enlargement of the vestibular aqueduct had frequent attacks of fluctuating hearing. Clinically it is important to detect patients with inner ear malformation such as cochlear anomalies and enlargement of the vestibular aqueduct usually accompanied by congenital sensorineural hearing loss. For patients with congenital sensorineural hearing loss, we recommend temporal bone CT scan. (author)

  4. [Diagnostic value of high-resolution computed tomography imaging in congenital inner ear malformations].

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    Sun, Xiaowei; Ding, Yuanping; Zhang, Jianji; Chen, Ying; Xu, Anting; Dou, Fenfen; Zhang, Zihe

    2007-02-01

    To observe the inner ear structure with volume rendering (VR) reconstruction and to evaluate the role of high-resolution computed tomography (HRCT) in congenital inner ear malformations. HRCT scanning was performed in 10 patients (20 ears) without ear disease (control group) and 7 patients (11 ears) with inner ear malformations (IEM group) and the original data was processed with VR reconstruction. The inner ear osseous labyrinth structure in the images generated by these techniques was observed respectively in the normal ears and malformation ears. The inner ear osseous labyrinth structure and the relationship was displayed clearly in VR imaging in the control group,meanwhile, characters and degree of malformed structure were also displayed clearly in the IEA group. Of seven patients (11 ears) with congenital inner ear malformations, the axial, MPR and VR images can display the site and degree in 9 ears. VR images were superior to the axial images in displaying the malformations in 2 ears with the small lateral semicircular canal malformations. The malformations included Mondini deformity (7 ears), vestibular and semicircular canal malformations (3 ears), vestibular aqueduct dilate (7 ears, of which 6 ears accompanied by other malformations) , the internal auditory canal malformation (2 ears, all accompanied by other malformations). HRCT can display the normal structure of bone inner ear through high quality VR reconstructions. VR images can also display the site and degree of the malformations three-dimensionally and intuitively. HRCT is valuable in diagnosing the inner ear malformation.

  5. Congenital malformations of the external and middle ear

    International Nuclear Information System (INIS)

    Koesling, S.; Omenzetter, M.; Bartel-Friedrich, S.

    2009-01-01

    With the focus on imaging, this paper gives a summarized view of the present knowledge on fields, which are necessary to know for a profound understanding of congenital malformations of the external and middle ear. Typical and less typical combinations of malformed parts of the ear can be derived from the embryogenesis. Clinical signs and audiometric findings lead to diagnosis in congenital aural atresia. Isolated middle ear malformations can be clinically mixed up especially with otosclerosis and tympanosclerosis. Imaging is needed for exact morphological information. In malformations of the external and middle ear, CT is the imaging modality of choice. Requirements on CT-technique as well as radiological findings including classification and pre-surgical rating are described. Morphological CT-correlates of congenital malformations and their differential diagnoses are enlisted and illustrated. The impact of CT-results on therapy is explained and actual therapeutic concepts are briefly presented

  6. [Clinical analysis of 102 patients with congenital inner ear malformation].

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    Zhu, X; Lian, N; Cai, Z

    1995-01-01

    Hearing loss and CT findings of 200 ears from 102 cases with congenital malformation of inner ear were included in our study. Hearing loss was typically bilateral severe, or total deafness. 75 percent of them were found deaf within one-year-old. In addition, 47 patients' (46%) mothers were noted to have caught a cold in first trimester of pregnancy. Temporal bone abnormalties were described as five types: 1. Michel malformation, 2. Mondini malformation, 3. enlargement of the vestibular aqueducts, 4. developmental deformity of cochlear aqueduct, 5. developmental deformity of internal acoustic meatus. Most cases showed malformations of vestibule or vestibular aqueducts.

  7. CT analysis of 333 cases of congenital malformations of the external and middle ear

    International Nuclear Information System (INIS)

    Zou Xin; Li Qiang; Wang Zhenchang; Xian Junfang; Lan Baosen

    1997-01-01

    To analyze the different CT findings of congenital malformations of the external and middle ear, 333 cases including 404 ears with external and middle ear malformations diagnosed by high resolution CT (HRCT) were analysed according to the location and type of the malformation. In 404 ears, there were 364 ears with atresia of external auditory meatus, 40 ears with stenosis of external auditory meatus, 377 ears with malformation of the ossicles, 382 ears with stenosis of tympanum and 333 ears with anterior position of the mastoid segment of the facial canal. HRCT can show the location and type of external and middle ear malformation and provide valuable information for surgery

  8. The role of MRI in suspected inner ear malformations

    International Nuclear Information System (INIS)

    Koesling, S.; Juettemann, S.; Amaya, B.; Rasinski, C.; Bloching, M.; Koenig, E.

    2003-01-01

    Purpose: This is a prospective analysis of the value of MRI in suspected inner ear malformations. Materials and Methods: In 50 patients (43 children and young adults, 7 adults) with suspected inner ear malformation MRI (1.5 T) was performed. In addition, 42 of these patients underwent CT. For the analysis of the inner ear structures, the constructive interference in steady state (CISS) sequence with 0.7 mm slice thickness was used. Functional tests revealed a sensorineural hearing loss or deafness in 82 temporal bones (TB) and a combined hearing loss in 4 TB. The hearing loss was unilateral in 14 patients. MRI and CT findings were compared. Results: Imaging findings were normal in 58 TB. The pathological findings included inner ear malformations (35 TB), inflammatory changes (4 TB), partial obliteration of labyrinth (2 TB) and congenital aural atresia (1 TB). An isolated absence of the cochlear nerve (1 TB) could only be found by MRI. In the remaining cases, an inner ear malformation was diagnosed by MRI and CT with the same confidence but MRI was superior in displaying the fine details. Conclusions: MRI will become the method of choice in the diagnosis of inner ear malformations. (orig.) [de

  9. Congenital inner ear malformations without sensorineural hearing loss.

    Science.gov (United States)

    Yukawa, Kumiko; Horiguchi, Satoshi; Suzuki, Mamoru

    2008-03-01

    It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations [Kokai H, Oohashi M, Ishikawa K, Harada K, Hiratsuka H, Ogasawara M et al. Clinical review of inner ear malformation. J Otolaryngol Jpn 2003;106(10):1038-44; Schuknecht HF. Mondini dysplasia. A clinical pathological study. Ann Otol Rhinol Laryngol 1980;89(Suppl. 65):1-23; Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987;97:2-14; Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography. Arch Otolaryngol 1974;100:11-8]. A 37-year-old woman had combined dysplasia of the posterior and lateral semicircular canals (PSCC, LSCC) with normal cochlear development and normal hearing in both ears. She had complained of dizziness for 8 months. High resolution computed tomography (CT) showed hypogenesis of the bony labyrinth in both ears. Bilateral PSCC and LSCC dysplasia and dilatation of the vestibule were detected. Magnetic resonant imaging (MRI) revealed that the deformity of the PSCC was more severe than the LSCC. Although the caloric test of the left ear elicited no nystagmus and there was reduced response in the right ear, the horizontal vestibulo-occular reflex (VOR) was present. Her dizzy sensation disappeared within 3 months without special treatment. The dizziness attack might have been caused by a temporary breakdown of her peripheral vestibular system.

  10. [Cochlear implant in patients with congenital malformation of inner ear].

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    Han, Dong-yi; Wu, Wen-ming; Xi, Xin; Huang, De-liang; Yang, Wei-yan

    2004-02-01

    To study surgical difficulty and key of the cochlear implant in patients with congenital malformation of inner ear. The cochlear implantations were performed in our department from Jan. 2001 to Apr. 2003 for 18 patients with the malformation of inner ear. In this series, there were 11 cases of large vestibular aqueduct syndrome (LVAS), 3 cases of Waardenberg syndrome, 3 cases of Mondini malformation, and 1 case of Usher syndrome. All 18 patients accepted the Nucleus 24-channel cochlear implantations, including Nucleus straight electrode in 13 cases but Contour implantation in 5 cases of LVAS. During operations, leakage of perilymph but not cerebrospinal fluid (CSF) from the open of scala tympani occurred in 11 cases of LVAS, however, the electrode was inserted successfully. The abnormalities of round window occurred in one of 3 cases of Waardenberg syndrome and 3 cases of Mondini malformation, respectively. The cochlear implant could be conducted successfully for the LVAS, and the postoperative effect was same as the ones for the deafness persons with normal development of inner ear. However, for the patients with Mondini syndrome and common cavity, it is important to accurately assess the extent of abnormalities in the inner ear and accompanied malformation before operation, and to evaluate the full extent of difficulties of the operation in order to minimize the risk of CSF leakage and meningitis.

  11. Cochlear implantation in the Mondini inner ear malformation.

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    Miyamoto, R T; Robbins, A J; Myres, W A; Pope, M L

    1986-07-01

    We report the case of a profoundly deaf 4-year-old boy with congenital deafness as a result of Mondini's dysplasia. The Mondini inner ear malformation is the result of arrested labyrinthine development during embryogenesis and is characterized by both bony and membranous anomalies of the inner ear. The dysplastic cochlear anatomy does not preclude successful cochlear implantation, and electrical threshold measurements are similar to those recorded in pediatric subjects deafened as a result of other causes.

  12. Congenital ossicular malformation. A study of 27 ears

    International Nuclear Information System (INIS)

    Morioka, Shigefumi; Sakaguchi, Hirofumi; Taki, Masakatsu; Hyogo, Misako; Suzuki, Toshihiro; Hisa, Yasuo

    2010-01-01

    Despite otological surgerical progress improving clinical congenital ossicular malformation management, some cases remain inadequately treated. We report 27 cases of congenital ossicular malformation, focusing on reasons for remaining or delayed postoperative hearing loss evaluated in 27 congenital ossicular malformation cases in Kyoto Prefecture from 2002 to 2008. Overall success was 93% (25/27) 6 months postoperatively. Two ears had no hearing improvement and three delayed hearing loss 8 to 48 months postoperatively. The first two ears underwent small fenestration stapedotomy with malleus attachment piston, and the other three tympanoplasty type III using an autologous ossicle or total ossicular replacement prosthesis (TORP) as a columella. We discuss problems and solutions using a malleus attachment piston or prosthesis, preoperative audio- and radiological findings, and operative findings including facial nerve anomaly and congenital cholesteatoma. (author)

  13. Classification and Current Management of Inner Ear Malformations

    Directory of Open Access Journals (Sweden)

    Levent Sennaroğlu

    2017-10-01

    Full Text Available Morphologically congenital sensorineural hearing loss can be investigated under two categories. Majority of the congenital hearing loss (80% are membranous malformations. Here the pathology involves inner ear hair cells. There is no gross bony abnormality and therefore, in these cases, high resolution computerized tomography and MRI of the temporal bone reveal normal findings. Remaining 20% have various malformations involving the bony labyrinth and therefore, can be radiologically demonstrated by CT and MRI. The latter group involves surgical challenges as well as problems in decision making. Some cases may be managed by hearing aid, some need cochlear implantation while some cases are candidates for an auditory brainstem implantation. During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During the surgery for inner ear malformations, surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcome after various implantation methods is closely related to the status of cochlear nerve and a practical classification of the cochlear nerve deficiency is also provided

  14. "COCHLEAR IMPLANTATION IN PATIENTS WITH INNER EAR MALFORMATIONS"

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    P. Borghei S. Abdi

    2004-08-01

    Full Text Available Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. This study was designed to analyze the operative findings,complications, and postoperative performance of patients with inner ear anomalies who underwent cochlear implantation. Six patients with inner ear malformations underwent implantation in our academic tertiary referral center from 1997 to 2002. The average follow-up period was 27 months. Malformations included one incomplete partition, one common cavity, one narrow internal acoustic canal (IAC in a patient with Riley-Day syndrome and 3 cases of large vestibular aqueduct. All received multi-channel implants either Nucleus 22 or Clarion device. Facial nerve was anomalous in 2 cases. CSF gusher occurred in 4 patients, which was controlled with packing the cochleostomy site. In all cases, the full length of electrode array was inserted, except one with Mondini's dysplasia where insertion failed in the first operation and was referred to another center for a successful surgery on the opposite ear. No other surgical complications were encountered. In 4 cases, all the 22 electrodes could be activated. All patients showed improved hearing performance after implantation. Four showed open-set speech recognition. The one with narrow IAC showed improved awareness to environmental sounds. In the other case (common cavity, the perception tests could not be performed because of very young age. Cochlear implantation in patients with inner ear malformations is a successful way of rehabilitation, although complications should be expected and auditory responses may be highly variable and relatively moderate.

  15. [Diagnostic significance of multi-slice computed tomography imaging in congenital inner ear malformations].

    Science.gov (United States)

    Ma, Hui; Han, Ping; Liang, Bo; Liu, Fang; Tian, Zhi-Liang; Lei, Zi-Qiao; Li, You-Lin; Kong, Wei-Jia

    2005-04-01

    To evaluate the feasibility and usability of multi-slice computed tomography (MSCT) in congenital inner ear malformations. Fourty-four patients with sensorineural hearing loss (SNHL) were examined by a Somatom Sensation 16 (siemens, Germany) CT scanner with following parameters: 120 kV, 100 mAs, 0.75 mm collimation, 1 mm reconstruction increment, a pitch factor of 1 and a field of view of 100 mm. The axial images of interested ears were reconstructed with 0.1 mm reconstruction increment, and a field of view of 50 mm. The 3D reconstructions were done with volume rendering technique (VRT) on the workstation (3D Virtuoso and Wizard,siemens). Twenty-five patients were normal and 19 patients (36 ears) were congenital inner ear malformations among 44 patients scanned with MSCT. Of the malformations, all the axial, MPR and VRT images can display the site and degree in 33 ears. VRT images were superior to the axial images in displaying the malformations in 3 ears with the small lateral semicircular canal malformations. The malformations were Michel deformity (1 ear), common cavity deformity (3 ears), incomplete partition I (3 ears), incomplete partition II (Mondini deformity, 5 ears), vestibular and semicircular canal malformations( 14 ears), vestibular aqueduct dilate( 16 ears, of which 6 ears accompanied by other malformations), the internal auditory canal malformation(8 ears, all accompanied by other malformations). MSCT allows a comprehensively assessing various congenital ear malformations through high quality MPR and VRT reconstructions. VRT images can display the site and degree of the malformations three-dimensionally and intuitionisticly. It is very useful to the cochlear implantation.

  16. Preoperative diagnosis and surgical strategy in congenital auditory ossicular malformation of 26 ears

    International Nuclear Information System (INIS)

    Kanazawa, Yuji; Naito, Yasushi; Shinohara, Shogo; Fujiwara, Keizo; Kikuchi, Masahiro; Yamazaki, Hiroshi; Kurihara, Risa; Kishimoto, Ippei

    2012-01-01

    We retrospectively analyzed 26 ears of 21 subjects having auditory ossicular malformation and who had undergone auditory reconstruction between April 2004 and December 2010 at our clinic. We checked preoperative condition, pathological classification, surgical procedure, and hearing improvement. We could predict pathological conditions precisely from preoperative computed tomography (CT), including incudostapedial disconnection (9/12, 75%) and malleus and/or incus fixation (7/12, 58%), which tended to be present in external ear malformation, and stapes footplate fixation (0/12, 0%). We could not, however, predict complex malformation (0/8, 0%). Overall success was 90% (18/20) in the 20 ears observed for at least 1 year. In the 2 ears without improved hearing, the first had congenital cholesteatoma and no stapes superstructure, was treated with type IV tympanoplasty. The second had malleus, incus, and stapes fixation and discontinuity between the incus and stapes, and was treated with type III tympanoplasty and stapes mobilization. Preoperative diagnosis is difficult in mixed congenital auditory ossicular malformation, especially stapes footplate fixation, possibly requiring unexpected procedures, with a poor hearing outcome. Preoperative status must thus be evaluated precisely using hearing, tympanometry, acoustic reflex test, and CT. Temporal bone CT and external ear findings are useful in diagnosing middle-ear malformation. Subjects' informed consent should also be obtained due to the possible need for changing procedure based on findings during surgery. (author)

  17. [Meta-analysis of the efficacy of cochlear implantation in deaf patients with inner ear malformation].

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    Xu, Qingqing; Zhai, Suoqiang; Han, Dongyi; Yang, Shiming; Shen, Weidong

    2014-04-01

    Assess and compare the clinical efficacy and safety of cochlear implantation in deaf patients with inner ear malformation and in the ones with normal inner ear structure, so that to clarify whether it is effective to restore hearing for the deaf patients with inner ear malformation. The literature with relevant key words were retrieved in the databases including PubMed, YZ365. com, WANFANG data, CMJD, CHKD and CNKI with language limited to Chinese and English. Extracted data included author, year of publication, diagnosis, treatment, et al. The clinical efficacy of cochlear implantation was assessed by the complications, electrode impedance, behavior T-level, hearing abilities and speech discrimination. Meta-analysis was performed using random or fixed effects model according to the heterogeneity of data. There were 11 randomized control studies involving 655 patients included in this study. There was no statistically significant difference among the deaf patients in mixed inner ear-malformation group, Mondini group and large vestibular aqueduct syndrome group in the aspects of postoperative complications, electrode impedance, behavior T-level, hearing abilities and speech discrimination. Cochlear implantation could be the way of treatment and rehabilitation for deaf patients with inner ear malformation. Further controlled studies with longer follow-up periods and more multiracial cases included may help to evaluate the efficacy of cochlear implantation for deaf patients with inner ear malformation more reliably.

  18. Inner ear malformations in siblings presenting with vestibular ...

    African Journals Online (AJOL)

    Although the association between inner ear abnormalities and progressive sensorineural hearing loss is well known, vestibular signs or loss of vestibular function in these ... We provide a brief overview of the latest classification of these inner ear defects as well as a review of the literature pertaining to children with inner ear ...

  19. Speech perception and production in children with inner ear malformations after cochlear implantation.

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    Rachovitsas, Dimitrios; Psillas, George; Chatzigiannakidou, Vasiliki; Triaridis, Stefanos; Constantinidis, Jiannis; Vital, Victor

    2012-09-01

    The aim of this study was to assess the speech perception and speech intelligibility outcome after cochlear implantation in children with malformed inner ear and to compare them with a group of congenitally deaf children implantees without inner ear malformation. Six deaf children (five boys and one girl) with inner ear malformations who were implanted and followed in our clinic were included. These children were matched with six implanted children with normal cochlea for age at implantation and duration of cochlear implant use. All subjects were tested with the internationally used battery tests of listening progress profile (LiP), capacity of auditory performance (CAP), and speech intelligibility rating (SIR). A closed and open set word perception test adapted to the Modern Greek language was also used. In the dysplastic group, two children suffered from CHARGE syndrome, another two from mental retardation, and two children grew up in bilingual homes. At least two years after switch-on, the dysplastic group scored mean LiP 62%, CAP 3.8, SIR 2.1, closed-set 61%, and open-set 49%. The children without inner ear dysplasia achieved significantly better scores, except for CAP which this difference was marginally statistically significant (p=0.009 for LiP, p=0.080 for CAP, p=0.041 for SIR, p=0.011 for closed-set, and p=0.006 for open-set tests). All of the implanted children with malformed inner ear showed benefit of auditory perception and speech production. However, the children with inner ear malformation performed less well compared with the children without inner ear dysplasia. This was possibly due to the high proportion of disabilities detected in the dysplastic group, such as CHARGE syndrome and mental retardation. Bilingualism could also be considered as a factor which possibly affects the outcome of implanted children. Therefore, children with malformed inner ear should be preoperatively evaluated for cognitive and developmental delay. In this case

  20. [Constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss].

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    Li, Youjin; Yang, Jun; Li, Yun

    2011-01-01

    To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss(SNHL). Imaging and auditory data of 125 cases (225 ears) in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied. Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases (78.4%), unilateral in 27 cases (21.6%). One hundred and sixty-seven ears (74.2%) were identified as profound deafness, 36 ears (16%) severe deafness and 22 ears (9.8%) moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct (75.6%) was the most common and next was vestibular deformity (32%), then cochleovestibular deformity (23.1%). Mondini deformity (55.8%) was the most common and common cavity deformity (28.9%) next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations. The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.

  1. Arteriovenous malformation of the external ear: a clinical assessment with a scoping review of the literature

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    Shin Hye Kim

    Full Text Available Abstract Introduction: Auricular Arteriovenous Malformation of the external ear is a rarely encountered disease; in particular, arteriovenous malformation arising from the auricle, with spontaneous bleeding, has seldom been reported. Objective: In the current study, we report an unusual case of late-onset auricular arteriovenous malformation originating from the posterior auricular artery that was confirmed by computed tomographic angiography. The case was successfully managed by pre-surgical intravascular embolization followed by total lesion excision. Prompted by this case, we also present a scoping review of the literature. Methods: A case of a 60 year-old man with right auricular arteriovenous malformation treated in our tertiary care center, and 52 patients with auricular arteriovenous malformation described in 10 case reports and a retrospective review are presented. Auricular arteriovenous malformation can manifest as swelling of the ear, pulsatile tinnitus, pain, and/or bleeding. On physical examination, a pulsatile swelling and/or a tender mass is evident. When arteriovenous malformation is suspected, the lesions should be visualized using imaging modalities that optimally detect vascular lesions, and managed via embolization, mass excision, or auricular resection. Effectiveness of the various diagnostic methods used and the treatment outcomes were analyzed. Results: Various imaging modalities including Doppler sonography, computed tomographic angiography, magnetic resonance angiography, and/or transfemoral cerebral angiography were used to diagnose 38 cases reported in the literature. In another 15 cases, no imaging was performed; treatment was determined solely by physical examination and auscultation. Of the total of 53 cases, 12 were not treated (their symptoms were merely observed whereas 20 underwent therapeutic embolization. In total, 32 patients, including 1 patient who was not treated and 10 with persistent or aggravated

  2. A FGF3 mutation associated with differential inner ear malformation, microtia, and microdontia.

    Science.gov (United States)

    Ramsebner, Reinhard; Ludwig, Martin; Parzefall, Thomas; Lucas, Trevor; Baumgartner, Wolf-Dieter; Bodamer, Olaf; Cengiz, Filiz Basak; Schoefer, Christian; Tekin, Mustafa; Frei, Klemens

    2010-02-01

    Analysis of association between genotype and phenotype. Prospective genetic study in a family. Auditory investigations, computer tomography, and genetic sequencing of the fibroblast growth factor 3 (FGF3) gene were performed on a Somali family presenting with autosomal recessive, hearing impairment, microdontia, and outer ear morphologies ranging from normal auricle development to microtia assessed as type 1 Weerda dysplasia in affected individuals. Computed tomography imaging identified differential inter- and intraindividual malformations of the inner ear including labyrinth aplasia, development of a common cavity to the presence of a cochlear with 1.5 windings (Mondini malformation) in affected individuals, symptoms similar to those described as labyrinth aplasia, microtia, and microdontia (LAMM) syndrome, caused by mutations in FGF3. Genetic sequencing revealed the presence of a novel p.R95W missense mutation in FGF3 segregating with pathology. The p.R95W mutation substitutes a positively charged arginine for a polar tryptophan in the highly conserved RYLAM consensus of the beta 6 sheet of FGF3 that interacts with FGFR2. These findings describe, for the first time, variable inner ear malformations and outer ear dysplasia in the presence of constant microdontia, associated with homozygous inheritance of the p.R95W mutation in FGF3, mirroring phenotypes observed in mouse models ablating FGF3/FGFR2 signaling.

  3. Ear malformations, hearing loss and hearing rehabilitation in children with Treacher Collins syndrome.

    Science.gov (United States)

    Rosa, Francisco; Coutinho, Miguel Bebiano; Ferreira, João Pinto; Sousa, Cecilia Almeida

    2016-01-01

    The aim of this study was to assess the main ear malformations, hearing loss and auditory rehabilitation in children with Treacher Collins syndrome. We performed a retrospective study of 9 children with Treacher Collins syndrome treated in a central hospital between January 2003 and January 2013. This study showed a high incidence of malformations of the outer and middle ear, such as microtia, atresia or stenosis of the external auditory canal, hypoplastic middle ear cavity, dysmorphic or missing ossicular chain. Most patients had bilateral hearing loss of moderate or high degree. In the individuals studied, there was functional improvement in patients with bone-anchored hearing aids in relation to conventional hearing aids by bone conduction. Treacher Collins syndrome is characterized by bilateral malformations of the outer and middle ear. Hearing rehabilitation in these children is of utmost importance, and bone-anchored hearing aids is the method of choice. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  4. [Cochlear implant in patients with congenital malformation of the inner ear].

    Science.gov (United States)

    Wan, Liang-cai; Guo, Meng-he; Qian, Yu-hong; Liu, Shuang-xiu; Zhang, Hong-zheng; Chen, Shuai-jun; Chen, Hao; Gong, Jian

    2009-10-01

    To summarize the clinical experience with multi-channel cochlear implantation in patients with inner ear malformations and evaluate and the outcomes of speech rehabilitation. A retrospective study was conducted in 295 patients receiving cochlear implantation from 1998 to 2007, including 25 patients with large vestibular aqueduct syndrome (LVAS), 9 with Modini malformation, and 5 with common cavity deformity. All the patients received the Nucleus24 cochlear implants. In LVAS cases, 4 had Nucleus 24R (ST) implants, 8 had Contuor implants, 10 had Contuor Advance, and the remaining cases used Nucleus24(M) straight-electrode implants. Severe gusher appeared in 3 cases of LVAS, and perilymph fluctuation were seen in other 15 cases. Four patients with Mondini malformation and 2 with common cavity malformation also experienced severe gusher, but the electrodes were inserted smoothly in all the patients without postoperative facial paralysis or cerebrospinal fluid leakage. The hearing threshold in these patients was similar to that in patients with normal cochlear structure. After speech rehabilitation for over 6 months, the abilities of speech discrimination and spoken language improved in all the cases in comparison with the preoperative lingual functions. Multi-channel cochlear implantation can be performed in patients with inner ear malformation, but should not be attempted in patients with poor cochlear and cochlear nerve development. A comprehensive pre-operative radiographic and audiological evaluation is essential.

  5. A new cochlear implant electrode with a "cork"-type stopper for inner ear malformations.

    Science.gov (United States)

    Sennaroğlu, Levent; Atay, Gamze; Bajin, Münir Demir

    2014-08-01

    Gusher in inner ear malformations is common in patients with incomplete partition type I and type III. It is also common in less severe form as oozing in incomplete partition type II and large vestibular aqueduct. It is important to prevent cerebrospinal fluid (CSF) escape around the electrode to prevent meningitis. The custom-made device was produced by Med-El Company. It has a "cork"-like stopper instead of the usual silicon ring to prevent gusher. There are two types of electrodes of different lengths. The standard one is 25mm (contact space 1.7mm) and the short one is 20mm (contact space 1.3mm). It was used in 50 patients with different inner ear malformations. Thirteen patients had gusher, and 11 patients oozing during cochleostomy. One patient with initial prototype of the cork electrode had to be revised because of persistent oozing around the electrode. Another patient had slow extrusion of the electrode most probably due to CSF pulsation and had to be revised. Both patients had no more CSF fistula. CSF fistula in inner ear malformations is a serious situation which may lead to recurrent meningitis. The new electrode with "cork" stopper looks promising in preventing the postoperative CSF leak around the electrode. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  6. Vibrant Soundbridge and Bone Conduction Hearing Aid in Patients with Bilateral Malformation of External Ear

    Directory of Open Access Journals (Sweden)

    Mondelli, Maria Fernanda Capoani Garcia

    2015-10-01

    Full Text Available Introduction Hearing loss is the most common clinical finding in patients with malformation of the external ear canal. Among the possibilities of treatment, there is the adaptation of hearing aids by bone conduction and the adaptation of implantable hearing aids. Objective To assess speech perception with the use of Vibrant Soundbridge (VBS - MED-EL, Innsbruck, Austria associated with additional amplification in patients with bilateral craniofacial malformation. Method We evaluated 11 patients with bilateral malformation over 12 years with mixed hearing loss or bilateral conductive. They were using the Softband (Oticon Medical, Sweden and bone conduction hearing aid in the ear opposite the one with the VSB. We performed the evaluation of speech perception using the Hearing in Noise Test. Results Participants were eight men and three women with a mean of 19.5 years. The signal / noise ratio presented significant results in patients fitted with VSB and bone conduction hearing aid. Conclusion The results of speech perception were significantly better with use of VBS combined with bone conduction hearing aids.

  7. Four variations of the Mondini inner ear malformations as seen in microdissections.

    Science.gov (United States)

    Johnsson, L G; Hawkins, J E; Rouse, R C; Kingsley, T C

    1984-01-01

    Four variations and degrees of severity of the Mondini malformation were found in the temporal bones from two neonates, one with congenital heart disease and the other with trisomy D, and from one teenager with leukemia: 1) short cochlea and normal vestibular organs; 2) short cochlea and persistent horizontal canal anlage; 3) markedly shortened cochlea with no modiolus, wide internal auditory meatus, and persistent horizontal canal anlage; 4) same as variation 3, but with persistent anlagen in all semicircular canals. Variations 3 and 4 were from the case of trisomy D, in which the left cochlea had a normal hair cell population but few nerve fibers, and the intraganglionic spiral bundle was displaced from Rosenthal's canal to the osseous spiral lamina. The right ear had no cochlear nerve fibers; the organ of Corti was present, but hair cells were unusually small. In the case of trisomy D, both ears showed subtotal loss of vestibular nerve fibers. Although the rudimentary cristae of the right ear had numerous hair cells, the macular hair cells were fewer and malformed. No hydrops was present.

  8. Inner ear malformations in patients with sensorineural heating loss: detection with gradient-echo (3DFT-CISS) MRI

    Energy Technology Data Exchange (ETDEWEB)

    Casselman, J.W. [Dept. of Radiology, A.Z. St.-Jan Brugge, Bruges (Belgium); Kuhweide, R. [Dept. of Otorhinolaryngology, A.Z. St.-Jan Brugge, Bruges (Belgium); Ampe, W. [Dept. of Otorhinolaryngology, A.Z. St.-Jan Brugge, Bruges (Belgium); D`Hont, G.D. [Dept. of Otorhinolaryngology, A.Z. St.-Jan Brugge, Bruges (Belgium); Offeciers, E.F. [ENT Dept., Sint-Augustinus Medical Inst., Univ. of Antwerp (Belgium); Faes, W.K. [Dept. of Radiology, A.Z. St.-Jan Brugge, Bruges (Belgium); Pattyn, G. [Dept. of Radiology, A.Z. St.-Jan Brugge, Bruges (Belgium)

    1996-04-01

    The sensitivity of different MRI sequences in the detection of inner ear malformations in patients presenting with sensorineural hearing loss (SNHL) and/or vertigo was evaluated. We studied 650 patients presenting with SNHL and/or vertigo, clinically not suspected of having inner ear malformations. The sensitivity of T1-weigted, Gd-enhanced T1-weighted and (when available) T2-weighted spin-echo images, and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) gradient-echo images, to unexpected malformations was assessed. Inner ear malformations were found in 15 (2.3%) of these patients. Enlargement of the endolymphatic duct and sac was the most frequent malformation, found in 11 patients. The 3DFT-CISS images showed all lesions; the other sequences were less sensitive and the pathology was missed, partially or only retrospectively seen in 11 of the 15 patients. Therefore, in addition to the routine unenhanced and Gd-enhanced T1-weighted and T2-weighted images, thin gradient-echo (3DFT-CISS) images are necessary to detect all clinically unexpected inner ear malformations in patients presenting with vertigo and/or SNHL. (orig.)

  9. Inner ear malformations in patients with sensorineural heating loss: detection with gradient-echo (3DFT-CISS) MRI

    International Nuclear Information System (INIS)

    Casselman, J.W.; Kuhweide, R.; Ampe, W.; D'Hont, G.D.; Offeciers, E.F.; Faes, W.K.; Pattyn, G.

    1996-01-01

    The sensitivity of different MRI sequences in the detection of inner ear malformations in patients presenting with sensorineural hearing loss (SNHL) and/or vertigo was evaluated. We studied 650 patients presenting with SNHL and/or vertigo, clinically not suspected of having inner ear malformations. The sensitivity of T1-weigted, Gd-enhanced T1-weighted and (when available) T2-weighted spin-echo images, and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) gradient-echo images, to unexpected malformations was assessed. Inner ear malformations were found in 15 (2.3%) of these patients. Enlargement of the endolymphatic duct and sac was the most frequent malformation, found in 11 patients. The 3DFT-CISS images showed all lesions; the other sequences were less sensitive and the pathology was missed, partially or only retrospectively seen in 11 of the 15 patients. Therefore, in addition to the routine unenhanced and Gd-enhanced T1-weighted and T2-weighted images, thin gradient-echo (3DFT-CISS) images are necessary to detect all clinically unexpected inner ear malformations in patients presenting with vertigo and/or SNHL. (orig.)

  10. Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity.

    Science.gov (United States)

    Graham, J M; Phelps, P D; Michaels, L

    2000-01-01

    The objective of this review is to analyze aspects of congenital malformation of the ear in relation to cochlear implantation in children. Having briefly described the in utero development of the ear and the classification of types of external, middle and inner ear malformation, five practical aspects of these malformations are discussed. It seems likely that the combination of bilateral profound sensorineural deafness with bilateral microtia severe enough to make a surgical approach to the cochlea difficult will be extremely uncommon. No such cases have been reported, although Klippel-Feil deformity seems the syndrome most likely to produce this set of circumstances. Abnormalities in the intratympanic course of the facial nerve have been associated with cochlear malformation, emphasizing the benefit of intra-operative facial nerve monitoring, and a technique suggested for safely avoiding an abnormally placed nerve. Fistulae of cerebrospinal fluid (CSF) and perilymph can complicate surgery and are relatively common in common cavity and Mondini malformations. Strategies for facilitating surgery in the presence of 'gushers', for measuring the pressure of a gusher and for placement of the cochlear implant electrode array are reviewed, with reports of fluctuating levels of electric current when implants lie in dysplastic cochleas. The relationship of implant performance to VIIIth nerve tissue in malformed cochleas is discussed, with a description of the histological findings in a common cavity cochlea. Techniques for identifying the absence of the cochlear nerve are reviewed. Stimulation of the facial nerve by cochlear implants has been described in cases of congenital malformation of the labyrinth but is relatively uncommon. Case reports of the benefit received by implanted children with congenital cochlear malformation have appeared since 1988. Most cases reported have not yet been followed for long enough to establish a clear picture of the outcome following

  11. Question mark ears, temporo-mandibular joint malformation and hypotonia: auriculo-condylar syndrome or a distinct entity?

    Science.gov (United States)

    Priolo, M; Lerone, M; Rosaia, L; Calcagno, E P; Sadeghi, A K; Ghezzi, F; Ravazzolo, R; Silengo, M

    2000-10-01

    We report a boy with prominent, peculiarly malformed ears, abnormality of the ramus of the mandible and hypotonia. An isolated peculiar bilateral ear deformity named 'question mark ear' has been delineated in plastic reconstruction surgery reviews [Cosman et al., 1970 Plast Reconstr Surg 46:454-457; Cosman (1984) Plast Reconstr Surg 73:572-576; Takato et al. (1989) Ann Plast Surg 22:69-73; Brodovsky (1997) Plast Reconstr Surg 100:1254-1257; Park (1998) Plast Reconstr Surg 101:1620-1623; Al-Quattan (1998) Plast Reconstr Surg 102:439-441] and a similar deformity of the ear and changes in the temporo-mandibular joint and condyle has been described by Jampol et al. [(1998) Am J Med Genet 75:449-452] and by Guion-Almeida et al. [(1999) Am J Med Genet 86:130-133]. The present case may be the third description of this malformation complex with additional clinical features characterized by hypotonia and mild developmental delay, or possibly a new distinct entity.

  12. Screening of SLC26A4, FOXI1, KCNJ10, and GJB2 in bilateral deafness patients with inner ear malformation.

    Science.gov (United States)

    Chen, Kaitian; Wang, Xianren; Sun, Liang; Jiang, Hongyan

    2012-06-01

    Bilateral nonsyndromic sensorineural hearing loss associated with inner ear malformation is closely related to genetics. SLC26A4 is considered to be the major involved gene. Recently, FOXI1 and KCNJ10 mutations have been linked to enlarged vestibular aqueducts and GJB2 mutations linked to temporal bone malformation. The authors aimed to investigate the mutation spectrums of these genes in Chinese patients with bilateral hearing impairment associated with inner ear malformation. Cross-sectional study. Affiliated hospital of the university. The authors analyzed the GJB2, SLC26A4, FOXI1, and KCNJ10 gene sequences in 43 patients presenting with bilateral hearing impairment associated with inner ear malformation using pyrosequencing and direct DNA sequencing. In total, 74.4% (32/43) of patients carried at least 1 of 14 pathogenic SLC26A4 mutations, including 6 novel mutations and 4 polymorphisms. Patients with enlarged vestibular aqueducts had a higher rate of SLC26A4 mutation than Mondini dysplasia patients. No FOXI1 or KCNJ10 potential pathogenic mutation was present, and GJB2 biallelic pathogenic mutations were uncommon (2.3%; 1/43). No significant correlation was observed between the genotype and phenotype of SLC26A4 mutations. SLC26A4 accounts for 74.4% of inner ear malformations in our cohort, whereas FOXI1, KCNJ10, and GJB2 mutations are not common. Other possible genes or external factors may contribute to this multibranch abnormality.

  13. [SOX10 mutation is relevant to inner ear malformation in patients with Waardenburg syndrome].

    Science.gov (United States)

    Xu, G Y; Hao, Q Q; Zhong, L L; Ren, W; Yan, Y; Liu, R Y; Li, J N; Guo, W W; Zhao, H; Yang, S M

    2016-11-07

    Objective: To determine the relevance between the SOX 10 mutation and Waardenburg syndrome (WS) accompanied with inner ear abnormality by analyzing the inner ear imaging results and molecular and genetic results of the WS patients with the SOX 10 mutation. Methods: This study included 36 WS in patients during 2001 and 2015 in the department of otorhinolaryngology head and neck surgery, Chinese Peoples's Liberation Army General Hospital. The condition of the inner ear of each patient was assessed by analyzing HRCT scans of the temporal bone and MRI scans of the brain and internal auditory canal. Meanwhile, the possible pathogenic genes of WS, including SOX10, MITF , and PAX 3, were also screened. Patients were divided into two groups according to SOX 10 mutation.The Fisher accuracy test was used to determine statistical difference of inner ear deformation incidence between the two groups. Results: Among all 36 patients, 12 were found to have inner ear abnormality. Most abnormalities were posterior semicircular canal deformations, some accompanied with cochlear deformation and an enlarged vestibule. Among all patients, 9 patients were SOX 10 heterozygous mutation carriers, among which six showed bilateral inner ear abnormality. Fisher accuracy test results suggested a significant correlation between the SOX 10 mutation and inner ear abnormality in WS patients ( P =0.036). Conclusion: This study found that WS patients with the SOX 10 mutation are more likely to have deformed inner ears when compared to WS patients without the SOX 10 mutation.

  14. Severe Klippel-Feil syndrome with Mondini malformation of inner ear.

    Science.gov (United States)

    Alaqeel, Aqeel Abdullah

    2014-01-01

    Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal bone showed choclear dysplasia with incomplete number of turns that is compatible with Mondini Malformation.

  15. SLC26A4 mutations are associated with a specific inner ear malformation.

    Science.gov (United States)

    Fitoz, Suat; Sennaroğlu, Levent; Incesulu, Armağan; Cengiz, Filiz Başak; Koç, Yasemin; Tekin, Mustafa

    2007-03-01

    Inner ear anomalies have been reported in approximately 30% of children with early onset deafness. Identification of causative genetic factors in a large proportion of these patients was not successful. Mutations in the SLC26A4 gene have been detected in individuals with enlarged vestibular aqueduct (EVA) or Mondini dysplasia. We aimed to characterize the inner ear anomalies associated with SLC26A4 mutations. The SLC26A4 gene has been screened for mutations in 16 subjects from 14 unrelated Turkish families with a variety of inner ear anomalies ranging from Michel aplasia to incomplete partition-II and EVA. None of the patients was diagnosed to have a recognizable genetic syndrome. Additional four patients with Pendred syndrome from three families were included. Only one patient with EVA was found to have a heterozygous mutation (c.1586delT) in SLC26A4. All patients with Pendred syndrome had homozygous mutations and were noted to have either EVA or EVA associated with incomplete partition-II on the computed tomography of the temporal bone. SLC26A4 mutations are not associated with a large spectrum of inner ear anomalies. They, instead, result in a specific morphological appearance consistent with EVA or incomplete partition-II.

  16. Severe Klippel-Feil syndrome with Mondini malformation of inner ear

    OpenAIRE

    Alaqeel, Aqeel Abdullah

    2014-01-01

    Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal bone showed choclear dysplasia with incomplete number of turns that is compatible with Mondini Malfor...

  17. [Analysis the relationship between SLC26A4 mutation and current diagnosis of inner ear malformation in children with sensorineural hearing loss].

    Science.gov (United States)

    Sun, Baochun; Zhou, Chengyong; Dai, Zhiyao

    2014-11-01

    Explore the relationship between the pathogenic mutations of SLC26A4 gene and inner ear malformation, and analyze the feasibility of genetic testing to help current diagnosis in part of children with sensorineural hearing loss. 2094 cases of children were detected by SLC26A4 with the method of DNA sequence. CT phenotypes of those children were classified according to the method proposed by Sennaroglu. We analyzed the relationship between the pathogenic mutations of gene and the CT phenotypes. (1) 685 cases of inner ear malformations were found in 2094 cases of children with sensorineural hearing loss by CT examination (371 cases of cochlea malformation were consisted of the follow types of malformation. Michel deformity was 6 cases, cochlea aplasia was 8 cases, common cavity deformity was 12 cases, incomplete partition type I was 27 cases, cochlea hypoplasia was 30 cases and Mondini malformation was 288 cases); Vestibular aqueduct was 265 cases; Vestibular/semicircular canal/internal auditory canal were 49 cases, normal was 1409 cases. (2) The DNA sequence results revealed that 465 cases carried pathogenic mutations (Bi-allelic mutations) of SLC26A4 gene, among which 135 cases were homozygous, 330 cases were compound heterozygous. (3) Pathogenic mutations of SLC26A4 gene detected 100% (465/465) in the group related to vestibular aqueduct malformation. The results suggest that pathogenic mutation of SLC26A4 gene is closely related to the CT phenotype of vestibular aqueduct malformation. Detecting of pathogenic mutations for hearing loss is binging the possibility to identify children with inner malformations at an early stage. As a consequence, it will improve the current diagnosis and therapeutical option.

  18. Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

    Science.gov (United States)

    Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

    2001-01-01

    The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

  19. Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import

    International Nuclear Information System (INIS)

    Swartz, J.D.; Faerber, E.N.

    1985-01-01

    The external auditory canal, middle ear, and bulk of the ossicular chain develop from the first branchial groove, first and second branchial arches, and first pharyngeal pouch. Embryologic development of these structures is complex and only rarely are two anomalies identical. This study includes 11 cases of unilateral external auditory canal atresia and two cases of bilateral atresia. Eight cases (four bilateral) of isolated congenital ossicular anomalies are also included. Emphasis is placed on findings of surgical import. All patients were studied with computed tomography only, because it was believed that the bony and soft-tissue detail achieved is superior to that with conventional multidirectional tomography

  20. JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

    Science.gov (United States)

    Hoeve, Hans L J; Brooks, Alice S; Smit, Liesbeth S

    2015-07-01

    We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  1. Middle and inner ear malformations in mutation-proven branchio-oculo-facial (BOF) syndrome: case series and review of the literature.

    Science.gov (United States)

    Carter, Melissa T; Blaser, Susan; Papsin, Blake; Meschino, Wendy; Reardon, Willie; Klatt, Regan; Babul-Hirji, Riyana; Milunsky, Jeff; Chitayat, David

    2012-08-01

    Hearing impairment is common in individuals with branchio-oculo-facial (BOF) syndrome. The majority of described individuals have conductive hearing impairment due to malformed ossicles and/or external canal stenosis or atresia, although a sensorineural component to the hearing impairment in BOF syndrome is increasingly being reported. Sophisticated computed tomography (CT) of the temporal bone has revealed middle and inner ear malformations in three previous reports. We present middle and inner ear abnormalities in three additional individuals with mutation-proven BOF syndrome. We suggest that temporal bone CT imaging be included in the medical workup of a child with BOF syndrome, in order to guide management. Copyright © 2012 Wiley Periodicals, Inc.

  2. Long-term outcomes of a transmastoid lateral semicircular canal approach to congenital CSF otorrhea in children associated with recurrent meningitis and severe inner ear malformation.

    Science.gov (United States)

    Wang, Jie; Li, Yongxin; Chen, Shubin; Hao, Xinping

    2016-08-01

    To investigate the long-term effectiveness of transmastoid lateral semicircular canal approach (TMLSCCA) to repair cerebrospinal fluid (CSF) leakage in children associated with recurrent meningitis and severe congenital inner malformation. A retrospective study was conducted in a university hospital, academic medical center. Fifteen children with recurrent meningitis, secondary to severe congenital inner ear malformation, were included in the study. All of them had CSF associated otorrhea and treated using TLSCCA to repair CSF otorrhea by packing the vestibule with muscle and fascia. Observation of the status of postoperative CSF leakage, recurrence of meningitis and complication were conducted. None of the cases had recurrent meningitis and CSF leakage after their TLSCCA procedure in the follow-up period of 1-8.5 years. One case presented with transient facial nerve paralysis and completely recovered 3 months later. TLSCCA for CSF otorrhea in children with recurrent meningitis secondary to congenital inner ear malformation is an alternative approach that offers some advantages. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  3. Deletion of a conserved regulatory element required for Hmx1 expression in craniofacial mesenchyme in the dumbo rat: a newly identified cause of congenital ear malformation

    Directory of Open Access Journals (Sweden)

    Lely A. Quina

    2012-11-01

    Hmx1 is a homeodomain transcription factor expressed in the developing eye, peripheral ganglia, and branchial arches of avian and mammalian embryos. Recent studies have identified a loss-of-function allele at the HMX1 locus as the causative mutation in the oculo-auricular syndrome (OAS in humans, characterized by ear and eye malformations. The mouse dumbo (dmbo mutation, with similar effects on ear and eye development, also results from a loss-of-function mutation in the Hmx1 gene. A recessive dmbo mutation causing ear malformation in rats has been mapped to the chromosomal region containing the Hmx1 gene, but the nature of the causative allele is unknown. Here we show that dumbo rats and mice exhibit similar neonatal ear and eye phenotypes. In midgestation embryos, dumbo rats show a specific loss of Hmx1 expression in neural-crest-derived craniofacial mesenchyme (CM, whereas Hmx1 is expressed normally in retinal progenitors, sensory ganglia and in CM, which is derived from mesoderm. High-throughput resequencing of 1 Mb of rat chromosome 14 from dmbo/dmbo rats, encompassing the Hmx1 locus, reveals numerous divergences from the rat genomic reference sequence, but no coding changes in Hmx1. Fine genetic mapping narrows the dmbo critical region to an interval of ∼410 kb immediately downstream of the Hmx1 transcription unit. Further sequence analysis of this region reveals a 5777-bp deletion located ∼80 kb downstream in dmbo/dmbo rats that is not apparent in 137 other rat strains. The dmbo deletion region contains a highly conserved domain of ∼500 bp, which is a candidate distal enhancer and which exhibits a similar relationship to Hmx genes in all vertebrate species for which data are available. We conclude that the rat dumbo phenotype is likely to result from loss of function of an ultraconserved enhancer specifically regulating Hmx1 expression in neural-crest-derived CM. Dysregulation of Hmx1 expression is thus a candidate mechanism for congenital ear

  4. Ear Infection (Middle Ear)

    Science.gov (United States)

    ... secretions from the middle ear Swelling, inflammation and mucus in the eustachian tubes from an upper respiratory ... your baby for at least six months. Breast milk contains antibodies that may offer protection from ear ...

  5. Ear Injury

    Science.gov (United States)

    ... of different injuries can affect the outer ear. Cauliflower ear (subperichondrial hematoma) A blunt blow to the ... to a deformed ear. This deformity, called a cauliflower ear, is common among wrestlers, boxers, and rugby ...

  6. Characteristics and application of inner ear CT in 20 cases of sensorineural hearing loss in children.

    Science.gov (United States)

    Huo, Liang; Wang, Hua

    2012-12-01

    This study shows that a number of children with sensorineural hearing loss (SNHL) have inner ear malformations demonstrated by multi-slice spiral computed tomography (MSCT). MSCT allows a comprehensive assessment of various congenital inner ear malformations through high quality multiplanar reformation (MPR) and can display the site and degree of the malformation three-dimensionally and intuitively. This is very useful for cochlear implantation. To evaluate the feasibility and usability of MSCT in pediatric SNHL with inner ear malformations. Sixty-five patients were diagnosed with SNHL by brainstem auditory evoked response (BAER). Inner ear MSCT scan and coronal MPR reconstruction were performed in all cases. This study demonstrated 20 cases (33 ears) with inner ear malformations, which included 10 ears with cochlear malformations, 7 with vestibular malformations, 5 with semicircular canal malformations, 8 with internal auditory canal (IAC) malformations, and 15 with vestibular malformations. Cochlear malformations included one ear with Michel deformity, two ears with common cavity deformity, one with cochlear aplasia, one with hypoplastic cochlea, two with incomplete partition type I, and three with incomplete partition type II (Mondini deformity).

  7. Swimmer's Ear

    Science.gov (United States)

    ... Eardrum Taking Care of Your Ears Can Loud Music Hurt My Ears? Your Ears What's Earwax? How Do Pain Relievers Work? View more About Us Contact Us Partners Editorial Policy Permissions Guidelines Privacy Policy & Terms of Use Notice ...

  8. A new classification for cochleovestibular malformations.

    Science.gov (United States)

    Sennaroglu, Levent; Saatci, Isil

    2002-12-01

    The report proposes a new classification system for inner ear malformations, based on radiological features of inner ear malformations reviewed in 23 patients. The investigation took the form of a retrospective review of computerized tomography findings relating to the temporal bone in 23 patients (13 male and 10 female patients) with inner ear malformations. The subjects were patients with profound bilateral sensorineural hearing loss who had all had high-resolution computed tomography (CT) with contiguous 1-mm-thick images obtained through the petrous bone in axial sections. The CT results were reviewed for malformations of bony otic capsule under the following subgroups: cochlear, vestibular, semicircular canal, internal auditory canal (IAC), and vestibular and cochlear aqueduct malformations. Cochlear malformations were classified as Michel deformity, common cavity deformity, cochlear aplasia, hypoplastic cochlea, incomplete partition types I (IP-I) and II (IP-II) (Mondini deformity). Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct. Four patients demonstrated anomalies involving only one inner ear component. All the remaining patients had diseases or conditions affecting more than one inner ear component. Eight ears had IP-I, and 10 patients had IP-II. Ears with IP-I had large cystic vestibules, whereas the amount of dilation was minimal in patients with IP-II. The majority of the semicircular canals (67%) were normal. Semicircular canal aplasia accompanied cases of Michel deformity, cochlear hypoplasia, and common cavity. In 14 ears, the IAC had a

  9. Ear wax

    Science.gov (United States)

    See your provider if your ears are blocked with wax and you are unable to remove the wax. Also call if you have an ear wax blockage and you develop new symptoms, such as: Drainage from the ear Ear pain Fever Hearing loss that continues after you clean the wax

  10. Ear Pieces

    Science.gov (United States)

    DiJulio, Betsy

    2011-01-01

    In this article, the author describes an art project wherein students make fanciful connections between art and medicine. This project challenges students to interpret "ear idioms" (e.g. "blow it out your ear," "in one ear and out the other") by relying almost entirely on realistic ear drawings, the placement of them, marks, and values. In that…

  11. Chiari Malformation

    Science.gov (United States)

    ... the back of the skull, just above the spine. When a child has a Chiari malformation, the space for the ... a portion of the top vertebra in the spine to reduce pressure in that area. If a child with a Chiari malformation also has hydrocephalus, the ...

  12. Imaging of the inner ear

    International Nuclear Information System (INIS)

    Casselman, J.W.; Bensimon, J.L.

    1997-01-01

    New computed tomography (CT) and magnetic resonance (MR) techniques allow more detailed anatomic studies of the inner ear. CT is still the best technique to study patients with fractures, congenital malformations and otodystrophies involving the inner ear. During recent years MR imaging has emerged as an excellent method to detect pathology in the internal auditory canal, membranous labyrinth and bony labyrinth and to characterize petrous apex lesions. MR has even proved its value in patients with fractures and congenital malformations making the diagnosis of, for instance, labyrinthine concussion and absence of the vestibulocochlear nerve possible. The diagnosis of acute/chronic labyrinthitis and intralabyrinthine tumors has also became possible. However, MR and CT are often complementary, as is the case in patients with mixed hearing loss, congenital malformations and petrous apex lesions. (orig.) [de

  13. Brain Malformations

    Science.gov (United States)

    Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

  14. Ear examination

    Science.gov (United States)

    ... may be present. The eardrum is a light-gray color or a shiny pearly-white. Light should ... discharge or bleeding Alternative Names Otoscopy Images Ear anatomy Medical findings based on ear anatomy Otoscopic exam ...

  15. Ear Tubes

    Science.gov (United States)

    ... of the ear drum or eustachian tube, Down Syndrome, cleft palate, and barotrauma (injury to the middle ear caused by a reduction of air pressure, ... specialist) may be warranted if you or your child has experienced repeated ... fluid in the middle ear, barotrauma, or have an anatomic abnormality that ...

  16. Arteriovenous Malformations

    Science.gov (United States)

    Arteriovenous malformations (AVMs) are defects in your vascular system. The vascular system includes arteries, veins, and capillaries. Arteries carry blood away from the heart to other organs; veins carry blood back to the heart. Capillaries connect the arteries and veins. An ...

  17. Congenital Auricular Malformations: Description of Anomalies and Syndromes.

    Science.gov (United States)

    Bartel-Friedrich, Sylva

    2015-12-01

    Half of the malformations in the ear, nose, and throat region affect the ear. Malformations of the external ear (pinna or auricle with external auditory canal [EAC]) are collectively termed microtia. Microtia is a congenital anomaly that ranges in severity from mild structural abnormalities to complete absence of the external ear (anotia). Microtia occurs more frequently in males (∼2 or 3:1), is predominantly unilateral (∼70-90%), and more often involves the right ear (∼60%). The reported prevalence varies geographically from 0.83 to 17.4 per 10,000 births. Microtia may be genetic (with family history, spontaneous mutations) or acquired. Malformations of the external ear can also involve the middle ear and/or inner ear. Microtia may be an isolated birth defect, but associated anomalies or syndromes are described in 20 to 60% of cases, depending on study design. These generally fit within the oculo-auriculo-vertebral spectrum; defects are located most frequently in the facial skeleton, facial soft tissues, heart, and vertebral column, or comprise a syndrome (e.g., Treacher Collins syndrome). Diagnostic investigation of microtia includes clinical examination, audiologic testing, genetic analysis and, especially in higher grade malformations with EAC deformities, computed tomography (CT) or cone-beam CT for the planning of surgery and rehabilitation procedures, including implantation of hearing aids. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  18. [HRCT imaging characterized of congenital abnormalities of the inner ear in 45 cases].

    Science.gov (United States)

    Wang, Jinling; Meng, Meijuan; Huan, Yi; Zhang, Jinsong

    2003-10-01

    To explore the high resolution CT (HRCT) image characterized of congenital abnormalities of the inner ear(CAIE), and its value in the diagnosis and treatment of CAIE. The clinic data and axial HRCT scans of CAIE in 45 cases were analyzed. In 45 CAIE patients, most of them were frequently associated with slowly progressive sensorineural hearing loss in childhood, 15 ears were fluctuating hearing loss. Seventeen ears were unilateral semicircular canal paralysis. HRCT showed that Michel type 3 cases(4 ears), Mondini type 25 cases(39 ears). Large vestibular aqueduct malformation not associated with anomalies of inner ears 13 cases(23 ears), anomalies of internal auditory canal 4 cases (5 ears). Thirteen ears were associated with outer and middle ear malformation. HRCT image has the important value in the diagnosis and treatment of CAIE, especially for the excerpt of indication of cochlear implantation.

  19. Your Ears

    Science.gov (United States)

    ... More on this topic for: Kids Can Loud Music Hurt My Ears? What Is an Ear Infection? Senses Experiment: Model Eardrum Going to the Audiologist What's Earwax? View more About Us Contact Us Partners Editorial Policy Permissions Guidelines Privacy Policy & Terms of Use Notice ...

  20. Temporal bone CT analysis of congenital ear anomalies

    International Nuclear Information System (INIS)

    Hwang, Jung Won; Moon, Min Joo; Sung, Kyu Bo

    1988-01-01

    Authors analysed the CT findings of the congenital ear anomalies of twenty-nine patients for 2 years and 3 months. The results were as follows: 1. Most of the patients were under the age of 20 (82.7%) and prevalent in male (72.4%). 2. Clinically, congenital ear anomalies were detected in 20 patients (68.9%), conductive hearing loss in 4, sensorineural hearing loss in 1, and the remained 4 patients were detected incidentally without clinical symptom. 3. In the cases of unilateral involvement of 20 patients, right ear was more common (12/20). Eight of 9 bilateral involvement showed similar degree. 4. The middle ear malformations were found in 22 patients (75.9%) and bilateral in 4 patients. 26 cases of middle ear malformations had been classified by Frey into 4 groups; Group I in 5, Group II in 9, Graoup III in 9 and Group IV in 3. 5. Incidentally found ear anomaly was lateral semicircular canal formed a single cavity with the vestibule in all patients (5 pts.). 6. Inner ear malformations accompanying sensorineural hearing loss were found in 3 patients with bilateral involvement and middle ear malformations were accompanied in 2 patients. The degree of involvement of labyrinth was variable.

  1. CT and MR imaging of congenital abnormalities of the inner ear and internal auditory canal

    International Nuclear Information System (INIS)

    Casselman, Jan W.; Offeciers, Erwin F.; Foer, Bert de; Govaerts, Paul; Kuhweide, Rudy; Somers, Thomas

    2001-01-01

    The embryology of the inner ear must be known as many of the inner ear malformations present as a result of the arrest during the various stages of embryology. These malformations are described in this 'embryologic' perspective and specific names for certain malformations are no longer used. Both CT and MR can be used to look at inner ear malformations but often both techniques are complementary. However, CT is preferred when associated middle- or external ear malformations must be excluded. Magnetic resonance is preferred when subtle changes in the membranous labyrinth or abnormalities of the nerves in the internal auditory canal must be visualised. The CT and MR technique must however be adapted as more and more subtle congenital malformations can only be seen when the right technique is used. The heavily T2-weighted gradient-echo or fast spin-echo MR techniques are mandatory if malformations of the inner ear must be excluded. The purpose of this paper is to describe the techniques used to study these patients and to give an overview of the most frequent and important congenital malformations which can be found in the inner ear and internal auditory canal/cerebellopontine angle

  2. Genetics Home Reference: capillary malformation-arteriovenous malformation syndrome

    Science.gov (United States)

    ... Institute: How the Heart Works National Institute of Neurological Disorders and Stroke: Arteriovenous Malformation Educational Resources (7 links) Boston Children's Hospital: Arteriovenous Malformation Boston Children's Hospital: Capillary Malformation ...

  3. Cosmetic ear surgery

    Science.gov (United States)

    Otoplasty; Ear pinning; Ear surgery - cosmetic; Ear reshaping; Pinnaplasty ... Cosmetic ear surgery may be done in the surgeon's office, an outpatient clinic, or a hospital. It can be performed under ...

  4. Meningitis after cochlear implantation in Mondini malformation.

    Science.gov (United States)

    Page, E L; Eby, T L

    1997-01-01

    Although the potential for CSF leakage and subsequent meningitis after cochlear implantation in the malformed cochlea has been recognized, this complication has not been previously reported. We report a case of CSF otorhinorrhea and meningitis after minor head trauma developing 2 years after cochlear implantation in a child with Mondini malformation. Leakage of CSF was identified from the cochleostomy around the electrode of the implant, and this leak was sealed with a temporalis fascia and muscle plug. Although this complication appears to be rare, care must be taken to seal the cochleostomy in children with inner ear malformations at the initial surgery, and any episode of meningitis after surgery must be thoroughly investigated to rule out CSF leakage from the labyrinth.

  5. [Diagnosis and management of Mondini malformation].

    Science.gov (United States)

    Yang, W; Fang, Y; Yang, S

    1997-02-01

    Owing to the development of imaging technology and audiology, some of the sensorineural hearing loss cases that were previcusly considered to be of unknown cause have been found to be inner ear malformation. Five cases of Mondini malformation are reviewed in this paper. CSF otorhinorrhea occurred in four cases, Klippel-Feil syndrome in three, and concurrent otosclerosis in one patient. In discussion, the authors point out that: 1) CT scanning is an supplement to audiologic tests for such patients; 2) perilymph fistula in Mondini malformation is often found at the oval window and its vicinity, the foot plate and the round window; 3) the fistula can be treated by plugging with fascial tissue via tympanoplastic approach with endaural incision. The mucosa around the fistula should be stripped away and the plugging tissue should be of dumb-bell shape.

  6. Vascular malformations in pediatrics

    International Nuclear Information System (INIS)

    Reith, W.; Shamdeen, M.G.

    2003-01-01

    Vascular malformations are the cause of nearly all non-traumatic intracranial hemorrhage in children beyond the neonatal stage. Therefore, any child presenting with spontaneous intracranial hemorrhage should be evaluated for child abuse and for vascular malformations. Intracerebral malformations of the cerebral vasculature include vein of Galen malformations, arteriovenous malformation (AVM), cavernomas, dural arteriovenous fistulas, venous anomalies (DVA), and capillary teleangiectasies. Although a few familial vascular malformation have been reported, the majority are sporadic. Clinical symptoms, diagnostic and therapeutic options are discussed. (orig.) [de

  7. Vitamin D receptor deficiency impairs inner ear development in zebrafish

    International Nuclear Information System (INIS)

    Kwon, Hye-Joo

    2016-01-01

    The biological actions of vitamin D are largely mediated through binding to the vitamin D receptor (VDR), a member of the nuclear hormone receptor family, which regulates gene expression in a wide variety of tissues and cells. Mutations in VDR gene have been implicated in ear disorders (hearing loss and balance disorder) but the mechanisms are not well established. In this study, to investigate the role of VDR in inner ear development, morpholino-mediated gene knockdown approaches were used in zebrafish model system. Two paralogs for VDR, vdra and vdrb, have been identified in zebrafish. Knockdown of vdra had no effect on ear development, whereas knockdown of vdrb displayed morphological ear defects including smaller otic vesicles with malformed semicircular canals and abnormal otoliths. Loss-of-vdrb resulted in down-regulation of pre-otic markers, pax8 and pax2a, indicating impairment of otic induction. Furthermore, zebrafish embryos lacking vdrb produced fewer sensory hair cells in the ears and showed disruption of balance and motor coordination. These data reveal that VDR signaling plays an important role in ear development. - Highlights: • VDR signaling is involved in ear development. • Knockdown of vdrb causes inner ear malformations during embryogenesis. • Knockdown of vdrb affects otic placode induction. • Knockdown of vdrb reduces the number of sensory hair cells in the inner ear. • Knockdown of vdrb disrupts balance and motor coordination.

  8. Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI

    Energy Technology Data Exchange (ETDEWEB)

    Katorza, Eldad; Nahama-Allouche, Catherine; Ducou le Pointe, Hubert; Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Service de Radiologie, Paris (France); Castaigne, Vanina [Hopital Saint-Antoine, Service de Gynecologie-Obstetrique, Paris (France); Gonzales, Marie; Marlin, Sandrine [Hopital d' Enfants Armand-Trousseau, Service de Genetique et Embryologie medicales, Paris (France); Galliani, Eva [Hopital d' Enfants Armand-Trousseau, Service de Chirurgie maxillo-faciale, Paris (France); Jouannic, Jean-Marie; Rosenblatt, Jonathan [Hopital d' Enfants Armand-Trousseau, Service de Gynecologie-Obstetrique, Centre pluridisciplinaire de diagnostic prenatal, Paris (France)

    2011-05-15

    Analysis of the middle ear with fetal MRI has not been previously reported. To show the contribution of fetal MRI to middle ear imaging. The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia. (orig.)

  9. Anthropometric growth study of the ear in a Chinese population.

    Science.gov (United States)

    Zhao, Shichun; Li, Dianguo; Liu, Zhenzhong; Wang, Yibiao; Liu, Lei; Jiang, Duyin; Pan, Bo

    2018-04-01

    A large number of anthropometric studies of the auricle have been reported in different nations, but little data were available in the Chinese population. The aim of this study was to analyze growth changes in the ear by measuring the width and length of ears in a Chinese population. A total of 480 participants were enrolled and classified into 1-, 3-, 5-, 7-, 9-, 12-, 14-, and 18-year groups (half were boys and half were girls in each group). Ear length, ear width, body weight, and body length were measured and recorded; ear index was calculated according to ear length and ear width. The growth of auricle and differences between genders were analyzed. Growth of ear in relation to body height and weight and the degree of emphasis on the length and width of the auricle were also analyzed. Ear length and width increased with age. Ear length achieved its mature size in both 14-year-old males and females. Ear width reached its mature size in males at 7 years and in females at 5 years. Different trends of ear index were shown between males and females. People in this population paid more attention to the length than the width of the auricle. The data indicated that ear development followed increase in age. There were gender and ethnic difference in the development of ear. These results may have potential implications for the diagnosis of congenital malformations, syndromes, and planning of ear reconstruction surgery. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  10. Inner ear anatomy in Waardenburg syndrome: radiological assessment and comparison with normative data.

    Science.gov (United States)

    Kontorinis, Georgios; Goetz, Friedrich; Lanfermann, Heinrich; Luytenski, Stefan; Giesemann, Anja M

    2014-08-01

    As patients with Waardenburg syndrome (WS) represent potential candidates for cochlear implantation, their inner ear anatomy is of high significance. There is an ongoing debate whether WS is related to any inner ear dysplasias. Our objective was to evaluate radiologically the inner ear anatomy in patients with WS and identify any temporal bone malformations. A retrospective case review was carried out in a tertiary, referral center. The high resolution computed tomography (HRCT) scans of the temporal bone from 20 patients (40 ears) with WS who were managed for deafness in a tertiary referral center from 1995 to 2012 were retrospectively examined. Measurements of 15 different inner ear dimensions, involving the cochlea, the vestibule, the semicircular canals and the internal auditory meatus, as well as measurements of the vestibular aqueduct, were performed independently by two neuroradiologists. Finally, we compared the results from the WS group with a control group consisting of 50 normal hearing subjects (100 ears) and with previously reported normative values. Inner ear malformations were not found in any of the patients with WS. All measured inner ear dimensions were within the normative values compiled by our study group as well as by others. Inner ear malformations are not characteristic for all types of WS; however, certain rare subtypes might be related to inner ear deformities. Normative cochleovestibular dimensions that can help in assessing the temporal bone anatomy are provided. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  11. Play it by Ear

    DEFF Research Database (Denmark)

    Kammersgaard, Nikolaj Peter Iversen; Kvist, Søren Helstrup; Thaysen, Jesper

    2014-01-01

    The first antenna for ear-to-ear communication with a standard Bluetooth chip has the potential to improve hearing aid technology.......The first antenna for ear-to-ear communication with a standard Bluetooth chip has the potential to improve hearing aid technology....

  12. Diagnosis of hearing impairment by high resolution CT scanning of inner ear anomalies

    International Nuclear Information System (INIS)

    Murata, Kiyotaka; Isono, Michio; Ohta, Fumihiko

    1988-01-01

    High resolution CT scanning of the temporal bone in our clinic has provided a more detailed radiological classification of inner ear anomalies than before. The statistical analysis of inner ear malformations based on the theory of quantification II has produced discriminant equations for the measurable diagnosis of hearing impairment and development of the inner ear. This analysis may make it possible to diagnose total and partial deafness on ipsi- and contralateral sides. (author)

  13. Aortic arch malformations

    Energy Technology Data Exchange (ETDEWEB)

    Kellenberger, Christian J. [University Children' s Hospital, Department of Diagnostic Imaging, Zuerich (Switzerland)

    2010-06-15

    Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

  14. Dandy-Walker Malformation

    African Journals Online (AJOL)

    rme

    Dandy-Walker malformation is a rare congenital malformation and involves the cerebellum and fourth ventricle. The condition is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. A large number of concomitant problems may be ...

  15. Aortic arch malformations

    International Nuclear Information System (INIS)

    Kellenberger, Christian J.

    2010-01-01

    Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

  16. Congenital spinal malformations

    International Nuclear Information System (INIS)

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

    2001-01-01

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

  17. Ear Infection and Vaccines

    Science.gov (United States)

    ... an ENT Doctor Near You Ear Infection and Vaccines Ear Infection and Vaccines Patient Health Information News ... or may need reinsertion over time. What about vaccines? A vaccine is a preparation administered to stimulate ...

  18. Swimmer's Ear (For Parents)

    Science.gov (United States)

    ... Development Infections Diseases & Conditions Pregnancy & Baby Nutrition & Fitness Emotions & Behavior School & Family Life First Aid & Safety Doctors & ... can lead to an infection. Dry skin or eczema , scratching the ear canal, vigorous ear cleaning with ...

  19. Taking Care of Your Ears

    Science.gov (United States)

    ... Audiologist Perforated Eardrum What's Hearing Loss? Can Loud Music Hurt My Ears? What Is an Ear Infection? Swimmer's Ear Your Ears What's Earwax? View more About Us Contact Us Partners Editorial Policy Permissions Guidelines Privacy Policy & Terms of Use Notice ...

  20. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  1. Cardiac and vascular malformations

    International Nuclear Information System (INIS)

    Ley, S.; Ley-Zaporozhan, J.

    2015-01-01

    Malformations of the heart and great vessels show a high degree of variation. There are numerous variants and defects with only few clinical manifestations and are only detected by chance, such as a persistent left superior vena cava or a partial anomalous pulmonary venous connection. Other cardiovascular malformations are manifested directly after birth and need prompt mostly surgical interventions. At this point in time echocardiography is the diagnostic modality of choice for morphological and functional characterization of malformations. Additional imaging using computed tomography (CT) or magnetic resonance imaging (MRI) is only required in a minority of cases. If so, the small anatomical structures, the physiological tachycardia and tachypnea are a challenge for imaging modalities and strategies. This review article presents the most frequent vascular, cardiac and complex cardiovascular malformations independent of the first line diagnostic imaging modality. (orig.) [de

  2. Congenital Vascular Malformation

    Science.gov (United States)

    ... also be effective for small, localized birthmarks (port wine stains). Patients with a rare venous malformation (Kleppel–Trenaunay Syndrome) of the limbs, frequently benefit from elastic garments and bandages used for com- ...

  3. Visualization of normal and abnormal inner ear with volume rendering technique using multislice spiral CT

    International Nuclear Information System (INIS)

    Ma Hui; Han Ping; Liang Bo; Lei Ziqiao; Liu Fang; Tian Zhiliang

    2006-01-01

    Objective: To evaluate the ability of the volume rendering technique to display the normal and abnormal inner ear structures. Methods: Forty normal earand 61 abnormal inner ears (40 congenital inner ear malformations, 7 labyrinthitis ossificans, and 14 inner ear erosion caused by cholesteatomas) were examined with a MSCT scanner. Axial imaging were performed using the following parameters: 120 kV, 100 mAs, 0.75 mm slice thickness, a pitch factor of 1. The axial images of interested ears were reconstructed with 0.1 mm reconstruction increment and a FOV of 50 mm. The 3D reconstructions were done with volume rendering technique on the workstation. Results: In the subjects without ear disorders a high quality 3D visualization of the inner ear could be achieved. In the patients with inner ear' disorders all inner ear malformations could be clearly displayed on 3D images as follows: (1) Michel deformity (one ear): There was complete absence of all cochlear and vestibular structures. (2) common cavity deformity (3 ears): The cochlea and vestibule were represented by a cystic cavity and couldn't be differentiated from each other. (3)incomplete partition type I (3 ears): The cochlea lacked the entire modiolus and cribriform area, resulting in a cystic appearance. (4) incomplete partition type II (Mondini deformity) (5 ears): The cochlea consisted of 1.5 turns, in which the middle and apical turns coalesced to form a cystic apex. (5) vestibular and semicircular canal malformations (14 ears): Cochlea was normal, vestibule dilated, semicircular canals were absent, hypoplastic or enlarged. (6) dilated vestibular aqueduct (14 ears): The vestibular aqueduct was bell-mouthed. In 7 patients with labyrinthifis ossificans, 3D images failed to clearly show the completeinner ears in 4 ears because of too high ossifications in the membranous labyrinth. In the other 3 ears volume rendering could display the thin cochlea basal turn and the intermittent semicircular canals. In the patients

  4. Supratentorial CNS malformations

    International Nuclear Information System (INIS)

    Zlatareva, D.

    2012-01-01

    Full text: Clinical suspicion of a developmental anomaly of the central nervous system (CNS) is a frequent indication for performing and magnetic resonance imaging (MRI) examination of the brain. Classification systems for malformation of the CNS are constantly revised according to newer scientific research. Developmental abnormalities can be classified in two main types. The first category consists of disorders of organogenesis in which genetic defects or any ischemic, metabolic, toxic or infectious insult to the developing brain can cause malformation. These malformations result from abnormal neuronal and glial proliferation and from anomalies of neuronal migration and or cortical organization. They are divided into supra- and infratentorial and may involve grey or white matter or both. The second category of congenital brain abnormalities is disorders of histogenesis which result from abnormal cell differentiation with a relatively normal brain appearance. Supratentorial CNS malformations could be divided into anomalies in telencephalic commissure, holoprosencephalies and malformations in cortical development. There are three main telencephalic commissures: the anterior commissure, the hippocampal commissure and the corpus callosum. Their morphology (hypoplasia, hyperplasia, agenesis, dysgenesis, even atrophy) reflects the development of the brain. Their agenesis, complete or partial, is one of the most commonly observed features in the malformations of the brain and is a part of many syndromes. Malformations of cortical development (MCD) are heterogeneous group of disease which result from disruption of 3 main stages of cortical development. The common clinical presentation is refractory epilepsy and or developmental delay. The most common MCD are heterotopias, focal cortical dysplasia, polymicrogyria, schizencephaly, pachygyria and lizencephaly. The exact knowledge of the brain anatomy and embryology is mandatory to provide a better apprehension of the

  5. Inner ear malformations in siblings presenting with vestibular ...

    African Journals Online (AJOL)

    Any child presenting with vestibular symptoms should be referred for an audiological assessment. I R T Butler, MMed ... rhythmically to music. The patient was ... was enrolled in an intensive speech therapy programme at age 2 years 7 months.

  6. Congenital Malformations in Neonates after irradiation of Rats During Pregnancy

    International Nuclear Information System (INIS)

    Abdel-Gawad, I.I.; Mohammad, M.H.M.

    2000-01-01

    Radiation is considered a teratogen during the whole period of embryonic development and fetal growth. However, the time of gestation at which irradiation takes place will affect the type of congenital malformation Induced. A study was carried out to observe various forms of congenital malformations induced after irradiation of pregnant rats to 1,2 and 3 Gy on the 9 th , 12 th and 15 th days of gestation. Various types of congenital malformations were observed in the neonates of irradiated animals as compared to controls. Most of the malformations were observed in neonates of animals irradiated with 2 and 3 Gy on the 12 th and 15 th days of gestation. This confirms that developmental anomalies occur mostly during the period of organ development. Other periods of gestation are less vulnerable to, induction of malformation after irradiation. Some representative photographs of the malformations induced such as penguin shape, absence of tail, low set ears, growth retardation and others are illustrated in the text

  7. Computer tomography in children and adolescents with suspected malformation of the petrous portion of the temporal bone

    International Nuclear Information System (INIS)

    Koesling, S.; Schneider-Moebius, C.; Koenig, E.; Meister, E.F.

    1997-01-01

    Purpose: To demonstrate HRCT findings and their therapeutic relevance in suspected congenital hearing disorders. Material and Methods: It was checked in 96 young patients if HRCT findings of the temporal bone could explain functional findings. Furthermore, the therapeutic consequences were noted. Results: Normal CT and normal functional findings were obtained in 49 temporal bones (TB). In conductive hearing loss (41 TB), dysplasias of the conducting apparatus (37 TB) and inflammatory changes (3 TB) were found. Combined hearing loss (18 TB) was clarified completely or partially in half the cases. There were 22 dysplasias of the inner ear, 3 dysplasias of the middle ear, 1 adandoned examination (2 TB), and 55 normal CT findings in senorineural hearing disorders (82 TB). 1 retardate had a malformation of the inner ear and, contralaterally, inflammatory middle ear. In cases of vestibular disorders (24 TB), 14 malformations of the inner ear were detected. (orig./AJ) [de

  8. Rare malformation of glans penis: arteriovenous malformation.

    Science.gov (United States)

    Akin, Y; Sarac, M; Yucel, S

    2013-01-01

    Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children.

  9. Hairy ears; Revisited

    Directory of Open Access Journals (Sweden)

    Daifullah Al Aboud

    2014-04-01

    Full Text Available Hair can grow in areas which are not usually hairy in human skin. The Online Mendelian Inheritance in Man (OMIM (http:// www.ncbi.nlm.nih.gov/omimhave some entries in this regards. These include ( %139600 – HAIRY ELBOWS, #605130 – HAIRY ELBOWS, SHORT STATURE, FACIAL DYSMORPHISM, AND DEVELOPMENTAL DELAY, 139630 – HAIRY NOSE TIP, 139500 – HAIRY EARS, and 425500 – HAIRY EARS, Y-LINKED. Hairy ears, (Fig. 1, are uncommon trait and it is rare to see a person with very long hair on the ears.

  10. MRI of the brain: malformations

    International Nuclear Information System (INIS)

    Treguier, C.; Heautot, J.F.; Gandon, Y.; Carsin, M.; Buhe, T.; Weppe, V.

    1990-01-01

    Brain malformations include occlusion, diverticula, neuronal migration abnormalities, cystic malformations, and histogenetic disorders in neurocutaneous syndromes. The multiple planes of section, excellent white/gray matter differentiation and accurate delineation of the brain cortex available with magnetic resonance imaging make it a tool of choice for the diagnosis of malformations, as well as for prognosis and genetic counseling [fr

  11. Congenital cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Chaudhry, A.K.; Azam, M.; Maqsood, R.; Naz, B.; Salam, A.

    2003-01-01

    This case report presents the clinical picture, diagnostic methodology and surgical treatment of a female child who presented with chronic cough and dyspnoea due to congenital malformation of lung. A discussion of diagnosis and management is presented at the end. (author)

  12. 'Outrunning' the running ear

    African Journals Online (AJOL)

    Chantel

    In even the most experienced hands, an adequate physical examination of the ears can be difficult to perform because of common problems such as cerumen blockage of the auditory canal, an unco- operative toddler or an exasperated parent. The most common cause for a running ear in a child is acute purulent otitis.

  13. "Swimmer's Ear" (Otitis Externa) Prevention

    Science.gov (United States)

    ... infections, swimmer’s ear, and healthy swimming. "Swimmer's Ear" (Otitis Externa) What are the symptoms of swimmer's ear? ... Healthy page. Reference CDC. Estimated burden of acute otitis externa —United States, 2003–2007 . MMWR Morb Mortal ...

  14. Radiosurgery for brain arteriovenous malformations

    International Nuclear Information System (INIS)

    Latorzeff, I.; Schlienger, M.; Sabatier, J.; Borius, P.Y.; Lotterie, J.A.; Bourdin, S.; Menegalli, D.; Cognard, C.; Januel, A.C.; Desal, H.; Debono, B.; Blond, S.

    2012-01-01

    Radiosurgery as treatment for arteriovenous malformations has shown a good efficacy in reducing intracranial bleeding due to rupture. The choice of therapeutic modalities is based on evolutive risk and arteriovenous malformations volume, patient profile and risks stratification following therapeutic techniques (microsurgery, radiosurgery, embolization). Nidus size, arteriovenous malformations anatomical localization, prior embolization or bleeding, distributed dose are predictive factors for radio-surgery's good results and tolerance. This review article will highlight arteriovenous malformations radiosurgery indications and discuss recent irradiation alternatives for large arteriovenous malformation volumes. (authors)

  15. Ear Problems in Swimmers

    Directory of Open Access Journals (Sweden)

    Mao-Che Wang

    2005-08-01

    Full Text Available Acute diffuse otitis externa (swimmer's ear, otomycosis, exostoses, traumatic eardrum perforation, middle ear infection, and barotraumas of the inner ear are common problems in swimmers and people engaged in aqua activities. The most common ear problem in swimmers is acute diffuse otitis externa, with Pseudomonas aeruginosa being the most common pathogen. The symptoms are itching, otalgia, otorrhea, and conductive hearing loss. The treatment includes frequent cleansing of the ear canal, pain control, oral or topical medications, acidification of the ear canal, and control of predisposing factors. Swimming in polluted waters and ear-canal cleaning with cotton-tip applicators should be avoided. Exostoses are usually seen in people who swim in cold water and present with symptoms of accumulated debris, otorrhea and conductive hearing loss. The treatment for exostoses is transmeatal surgical removal of the tumors. Traumatic eardrum perforations may occur during water skiing or scuba diving and present with symptoms of hearing loss, otalgia, otorrhea, tinnitus and vertigo. Tympanoplasty might be needed if the perforations do not heal spontaneously. Patients with chronic otitis media with active drainage should avoid swimming, while patients who have undergone mastoidectomy and who have no cavity problems may swim. For children with ventilation tubes, surface swimming is safe in a clean, chlorinated swimming pool. Sudden sensorineural hearing loss and some degree of vertigo may occur after diving because of rupture of the round or oval window membrane.

  16. The ear in fetal MRI: what can we really see?

    Energy Technology Data Exchange (ETDEWEB)

    Moreira, Nuno Canto [Neuroradiology Section C., Campos Costa, Fragosela, Viseu (Portugal); Uppsala University, Department of Radiology, Uppsala (Sweden); Teixeira, Joao [Department of Neuroradiology, Porto (Portugal); Raininko, Raili; Wikstrom, Johan [Uppsala University, Department of Radiology, Uppsala (Sweden)

    2011-12-15

    The aim of this study was to investigate the ability to depict the components of the ear on brain-oriented fetal MRI studies. Retrospective evaluation of the ear in MRI studies was performed post-mortem in 16 fetuses ranging from 15 to 22 gestation weeks (GW), and in 122 examinations in vivo of fetuses ranging from 20 to 38 GW. The cochlea, vestibular apparatus, middle ear, and external auditory canal were separately graded according to the components that were delineated. The components of the inner and middle ear were fully delineated in 100% of the post-mortem examinations, but the external auditory canals were only seen in only 25%. In the in vivo group, the imaging detail was much lower. Cochlear turns could be identified in 75% of the fetuses, the vestibule and the lateral semicircular canals in 72% andossicles in 70%. Before 25 GW, the ability to identify these individual parts was 50%, 30%, and 33%, respectively, and above it was 89%, 93%, and 90%. In most cases, the external auditory canals could only be seen after 29 GW. In fetal MRI studies in vivo, it is possible to depict the components of the ear in the majority of the fetuses, in such a manner as to exclude major malformations. However, MRI might not provide enough detail to rule out pathology of the ear before 25 GW, this being a critical age for pregnancy management in many countries. (orig.)

  17. Congenital cystic lung malformations

    International Nuclear Information System (INIS)

    Stoever, B.; Scheer, I.; Bassir, C.; Chaoui, R.; Henrich, W.; Schwabe, M.; Wauer, R.

    2006-01-01

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  18. Arteriovenous malformation of face

    Directory of Open Access Journals (Sweden)

    Ashok Kumar

    2017-01-01

    Full Text Available Arteriovenous malformations (AVMs are rare congenital vascular malformations accounting only 1.5% of all vascular anomalies with 50% occurrence in the oral and maxillofacial region. It usually results from birth defects of the vasculature. A literature search revealed only few case reports of AVMs in the facial region. Lack of meticulous diagnosis, scarcity of knowledge, and paucity of literature can result in their exsanguinations leading to fatal hemorrhagic incidents after various dental procedures such as tooth extraction, surgical intervention, puncture wound, or blunt injury in involved area. The present case describes the accidental diagnosis of asymptomatic high-flow AVMs in the facial region of pediatric patient reported primarily for the treatment of periapical abscess. This case report is unique because although there was no history of bleeding episodes, thorough examination and investigations diagnosed it as high-flow vascular malformation. It is important for the dental practitioner to be aware of AVM which may be present in the head and neck region that can produce fatal bleeding episodes during various dental procedures. Proper diagnosis of AVMs through complete history, precise clinical examination, and advanced imaging modalities can help in preventing serious life-threatening complications.

  19. External Otitis (Swimmer's Ear)

    Science.gov (United States)

    ... otitis. Fungal external otitis (otomycosis), typically caused by Aspergillus niger or Candida albicans, is less common. Boils are ... in the ear. Fungal external otitis caused by Aspergillus niger usually causes grayish black or yellow dots (called ...

  20. Ear Infections in Children

    Science.gov (United States)

    ... ear infections may get better without antibiotics. Using antibiotics cautiously and with good reason helps prevent the development of bacteria that become resistant to antibiotics. If your doctor prescribes an antibiotic, it’s important ...

  1. Cerebral malformations without antenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Girard, Nadine J. [Diagnostic Neuroradiology, Hopital Timone, Marseille (France)

    2010-06-15

    Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms), or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital hemiparesis, epilepsy and mental or psychomotor retardation. MRI is the imaging method of choice to assess cerebral malformations. (orig.)

  2. [Management of occult malformations at the lateral skull base].

    Science.gov (United States)

    Bryson, E; Draf, W; Hofmann, E; Bockmühl, U

    2005-12-01

    Occult malformations of the lateral skull base are rare anomalies, but can cause severe complications such as recurrent meningitis. Therefore, they need to be precisely delineated and sufficient surgical closure is mandatory. Between 1986 and 2004 twenty patients (10 children and 10 adults) with occult malformations at the lateral skull base were treated surgically at the ENT-Department of the Hospital Fulda gAG. Of these 3 Mondini-malformations, 11 defects of the tegmen tympani or the mastoidal roof, 2 dural lesions to the posterior fossa and 4 malformations within the pyramidal apex have been found. Four patients have had multiple anomalies. Routing symptom was in all cases at least one previous meningitis. Radiological diagnostics included high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) as well as CT- or MR-cisternography. Depending on type and localisation of the defect the following surgical algorithm was carried out: The trans-mastoidal approach was used in all cases of Mondini-malformation (including obliteration of the ear), in case of lesions to the posterior fossa as well as partly in anomalies at the tegmen tympani and mastoidal roof, respectively. Defects of the pyramidal apex should be explored via the trans-mastoidal way if the lesion is located caudally to the inner auditory canal (IAC), whereas the trans-temporal approach should be used if the lesion is situated ventral to the IAC and dorso-medially to the internal carotid artery (ICA). The trans-temporal approach was also performed in large defects of the tegmen tympani and mastoidal roof as well as in recurrences. In all cases of recurrent meningitis caused by agents of the upper airway tract the basic principle should be to search for occult skull base malformations radiologically as well as by sodium fluorescein endoscopy as long as the anomaly is detected.

  3. Inner ear anomalies seen on CT images in people with Down syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Intrapiromkul, Jarunee; Aygun, Nafi; Yousem, David M. [The Johns Hopkins Medical Institutions, The Russell H. Morgan Department of Radiology and Radiological Sciences, Baltimore, MD (United States); Tunkel, David E. [The Johns Hopkins Medical Institutions, Department of Otolaryngology-Head and Neck Surgery, Baltimore, MD (United States); Carone, Marco [University of California, Division of Biostatistics, School of Public Health, Berkeley, CA (United States)

    2012-12-15

    Although dysplasia of inner ear structures in Down syndrome has been reported in several histopathological studies, the imaging findings have not been widely studied. To evaluate the prevalence and clinical significance of inner ear anomalies detected on CT images in patients with Down syndrome. The temporal bone CT images of patients with Down syndrome were assessed for inner ear anomalies; clinical notes and audiograms were reviewed for hearing loss. Logistic regression models were employed to identify which CT findings were associated with sensorineural hearing loss (SNHL). Inner ear anomalies were observed in 74.5% (38/51) of patients. Malformed bone islands of lateral semicircular canal (LSCC), narrow internal auditory canals (IACs), cochlear nerve canal stenoses, semicircular canal dehiscence (SCCD), and enlarged vestibular aqueducts were detected in 52.5% (53/101), 24.5% (25/102), 21.4% (21/98), 8.8% (9/102) and 2% (2/101) of patients' ears, respectively. IAC stenosis had the highest odds ratio (OR = 5.37, 95% CI: 1.0-28.9, P = 0.05) for SNHL. Inner ear anomalies occurred in 74.5% of our population, with malformed (<3 mm) bone island of LSCC being the most common (52.5%) anomaly. Narrow IAC was seen in 24.5% of patients with Down syndrome and in 57.1% of ears with SNHL. High-resolution CT is a valuable for assessing the cause of hearing loss in people with Down syndrome. (orig.)

  4. Inner ear anomalies seen on CT images in people with Down syndrome

    International Nuclear Information System (INIS)

    Intrapiromkul, Jarunee; Aygun, Nafi; Yousem, David M.; Tunkel, David E.; Carone, Marco

    2012-01-01

    Although dysplasia of inner ear structures in Down syndrome has been reported in several histopathological studies, the imaging findings have not been widely studied. To evaluate the prevalence and clinical significance of inner ear anomalies detected on CT images in patients with Down syndrome. The temporal bone CT images of patients with Down syndrome were assessed for inner ear anomalies; clinical notes and audiograms were reviewed for hearing loss. Logistic regression models were employed to identify which CT findings were associated with sensorineural hearing loss (SNHL). Inner ear anomalies were observed in 74.5% (38/51) of patients. Malformed bone islands of lateral semicircular canal (LSCC), narrow internal auditory canals (IACs), cochlear nerve canal stenoses, semicircular canal dehiscence (SCCD), and enlarged vestibular aqueducts were detected in 52.5% (53/101), 24.5% (25/102), 21.4% (21/98), 8.8% (9/102) and 2% (2/101) of patients' ears, respectively. IAC stenosis had the highest odds ratio (OR = 5.37, 95% CI: 1.0-28.9, P = 0.05) for SNHL. Inner ear anomalies occurred in 74.5% of our population, with malformed (<3 mm) bone island of LSCC being the most common (52.5%) anomaly. Narrow IAC was seen in 24.5% of patients with Down syndrome and in 57.1% of ears with SNHL. High-resolution CT is a valuable for assessing the cause of hearing loss in people with Down syndrome. (orig.)

  5. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  6. Genetics Home Reference: Dandy-Walker malformation

    Science.gov (United States)

    ... Twitter Home Health Conditions Dandy-Walker malformation Dandy-Walker malformation Printable PDF Open All Close All Enable ... to view the expand/collapse boxes. Description Dandy-Walker malformation affects brain development, primarily development of the ...

  7. Advances tomographic in evaluation of middle ear

    International Nuclear Information System (INIS)

    Anjos, Mayara Alves Pinheiro dos; Ledo, Mirelle D'arc Frota; Ribeiro, Marcio Duarte

    2011-01-01

    Computed tomography has a key role in the study of hearing, since through it can be evaluated structures not seen by otoscope. In many clinical situations the diagnosis through this test proves limited, being fundamental examination of the associated image reconstructions: multiplanar reconstruction, maximum intensity projection, and volume-rendering technique. The ossicular chain is a complex formed by the ossicles malleus, incus and stapes, situated in the middle ear; it is difficult to view them in orthogonals planes. This review article intends to demonstrate the importance of post-processing the image of the ossicular chain for a better representation of the anatomy and possible diseases. Reformatting of images helps, significantly, to a better visualization of these structures as related congenital malformations, vascular abnormalities, inflammatory conditions, neoplasia and traumas. (author)

  8. Genetics Home Reference: cerebral cavernous malformation

    Science.gov (United States)

    ... Additional NIH Resources (1 link) National Institute of Neurological Disorders and Stroke Educational Resources (7 links) Boston Children's Hospital: Cavernous Malformations Disease InfoSearch: Cerebral Cavernous Malformation ...

  9. Listening to the ear

    Science.gov (United States)

    Shera, Christopher A.

    Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics-termed "tapering symmetry" after its geometric interpretation in simple models-that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical

  10. Ear, Hearing and Speech

    DEFF Research Database (Denmark)

    Poulsen, Torben

    2000-01-01

    An introduction is given to the the anatomy and the function of the ear, basic psychoacoustic matters (hearing threshold, loudness, masking), the speech signal and speech intelligibility. The lecture note is written for the course: Fundamentals of Acoustics and Noise Control (51001)......An introduction is given to the the anatomy and the function of the ear, basic psychoacoustic matters (hearing threshold, loudness, masking), the speech signal and speech intelligibility. The lecture note is written for the course: Fundamentals of Acoustics and Noise Control (51001)...

  11. Extrasinusal dural arteriovenous malformations

    International Nuclear Information System (INIS)

    Piske, R.L.; Lasjaunias, P.

    1988-01-01

    Of 60 cases of dural arteriovenous malformations (DAVM) explored and treated in the Vascular Neuroradiology Unit of the Hospital of Bicetre between 1980 and 1986, 3 presented in an extrasinusal location: intraorbital, middle cerebral fossa and the region of the superior orbital fissure. We have not found any prior description of DAVMs in these regions in the literature. The classic concept that DAVMs rise in direct relationship with the dural sinuses is limited. We believe that these lesions may also developed in relationship with the venous drainage system of the sinuses and the venous drainage of the peripheral nervous system. We note that the superior ophthalmic vein which drains DAVMs of the orbit and the olfactory vein which drains DAVMs of the anterior cranial fossa and other veins that accompany the cranial nerves through the skull base have the characteristics of emissary veins. It may be that DAVMs can develop wherever veins follow a transosseous trajectory. (orig.)

  12. Surgical management of venous malformations.

    Science.gov (United States)

    Loose, D A

    2007-01-01

    Among vascular malformations, the predominantly venous malformations represent the majority of cases. They form a clinical entity and therefore need clear concepts concerning diagnosis and treatment. This paper presents an overview of contemporary classification as well as tactics and techniques of treatment. According to the Hamburg Classification, predominantly venous malformations are categorized into truncular and extratruncular forms, with truncular forms distinguished as obstructions and dilations, and extratruncular forms as limited or infiltrating. The tactics of treatment represent surgical and non-surgical methods or combined techniques. Surgical approaches utilize different tactics and techniques that are adopted based on the pathologic form and type of the malformation: (I) operation to reduce the haemodynamic activity of the malformation; (II) operation to eliminate the malformation; and (III) reconstructive operation. As for (I), a type of a tactic is the operation to derive the venous flow. In (II), the total or partial removal of the venous malformation is demonstrated subdivided into three different techniques. In this way, the infiltrating as well as the limited forms can be treated. An additional technique is dedicated to the treatment of a marginal vein. Approach (III) involves the treatment of venous aneurysms, where a variety of techniques have been successful. Long-term follow-up demonstrates positive results in 91% of the cases. Congenital predominantly venous malformations should be treated according to the principles developed during the past decades in vascular surgery, interventional treatment and multidisciplinary treatment. The days of predominantly conservative treatment should be relegated to the past. Special skills and experiences are necessary to carry out appropriate surgical strategy, and the required operative techniques should be dictated by the location and type of malformation and associated findings.

  13. From Ear to Brain

    Science.gov (United States)

    Kimura, Doreen

    2011-01-01

    In this paper Doreen Kimura gives a personal history of the "right-ear effect" in dichotic listening. The focus is on the early ground-breaking papers, describing how she did the first dichotic listening studies relating the effects to brain asymmetry. The paper also gives a description of the visual half-field technique for lateralized stimulus…

  14. Middle ear implants

    Directory of Open Access Journals (Sweden)

    K S Gangadhara Somayaji

    2013-01-01

    Full Text Available Hearing loss is becoming more common in the society living in cities with lot of background noise around, and frequent use of gadgets like mobile phones, MP3s, and IPods are adding to the problem. The loss may involve the conductive or perceptive pathway. Majority of the patients with conductive hearing loss will revert back to normal hearing levels with medical and/or surgical treatment. However, in sensorineural hearing loss, many factors are involved in the management. Though traditionally hearing aids in various forms are the most commonly used modality in managing these patients, there are some drawbacks associated with them. Implantable middle ear amplifiers represent the most recent breakthrough in the management of hearing loss. Middle ear implants are surgically implanted electronic devices that aim to correct hearing loss by stimulating the ossicular chain or middle ear. Of late, they are also being used in the management of congenital conductive hearing loss and certain cases of chronic otitis media with residual hearing loss. The article aims to provide general information about the technology, indications and contraindications, selection of candidates, available systems, and advantages of middle ear implants. (MEI

  15. Seeing With the Ears

    NARCIS (Netherlands)

    Koenderink, Jan

    2015-01-01

    In recent talks, I mentioned how my artist friends often complain that their clients see with their ears. It recently dawned on me that nobody understood what I said, or—worse—got the wrong idea. The audience thinks of bionic devices (Proulx, Stoerig, Ludowig, & Knoll, 2008) or bat echo location

  16. Ear infection - acute

    Science.gov (United States)

    ... to the back of the throat. Normally, this tube drains fluid that is made in the middle ear. ... allows air to get in so fluids can drain more easily. Usually the tubes fall out by themselves. Those that don't ...

  17. Low-set ears and pinna abnormalities

    Science.gov (United States)

    Low-set ears; Microtia; "Lop" ear; Pinna abnormalities; Genetic defect - pinna; Congenital defect - pinna ... conditions: Abnormal folds or location of the pinna Low-set ears No opening to the ear canal ...

  18. Imaging of the cloacal malformation

    International Nuclear Information System (INIS)

    Jaramillo, D.; Lebowitz, R.L.; Hendren, W.H.

    1989-01-01

    Sixty-two newborns with the cloacal malformation were evaluated to define variation in cloacal configuration, associated anomalies, and optimal approach to imaging. Cloacal malformations were classified according to level of urethrovaginal confluence (47 low, 15 high); level of rectal communication (47 vaginal, seven cloacal, eight other); and cloacal appearance (34 urethral, 28 vaginal, 10 transistional). Lower urinary abnormalities included reflux, ureteral ectopia, bladder diverticula, bladder duplication (five), and urachal remnants (five). Uterovaginal abnormalities included duplication and partial agenesis. Anomalies were common in the upper urinary tract, spine (sacral agenesis, 20), and bony pelvis (pubic diastasis, 10). Gastrointestinal, cardiovascular, neurologic, and limb (seven) malformations were seen. Fluoroscopically monitored opacification of the cloacal components and voiding cystourethrogram were essential. Although sonography of the malformation was of limited usefulness, MR imaging seemed to be useful for evaluation of the spinal cord and pelvic musculature

  19. Classification of Cortical Brain Malformations

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-03-01

    Full Text Available Clinical, radiological, and genetic classifications of 113 cases of malformations of cortical development (MCD were evaluated at the Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands.

  20. Facial vascular malformations in children

    International Nuclear Information System (INIS)

    Brunelle, F.O.; Lallemand, D.; Chaumont, P.; Teillac, D.; Manach, Y.

    1988-01-01

    The authors present their experience with conventional and digital angiography of vascular malformations of the head and neck in children. 22 hemangioendotheliomas, 8 venous angiomas, and 3 arteriovenous fistula were studied. 22 patients were embolised. DSA offers many advantages during the diagnostic as well as during the therapeutic phase of angiography. Embolization appears to have a major role in treatment of such vascular malformations. (orig.)

  1. Radiosurgery of arteriovenous malformations

    International Nuclear Information System (INIS)

    Kawamoto, Shunsuke

    1994-01-01

    Steiner has first published a report of a case of cerebral arteriovenous malformation (AVM) treated with gamma knife radiosurgery in 1972. Since the mid-1980s, radiosurgery has increasingly received much attention. There have already been more than 6,000 radiosurgically treated AVM cases. This article reviews radiosurgery in the context of the following: (1) radiosurgery according to gamma knife, linear accelerator, heavy-charged particle Bragg-peak radiation and neutron; (2) therapeutic outcome in view of angiographically proven complete obliteration (CO) of AVM, including the time required for CO and factors regulating CO (such as the volume of AVM, radiation doses, correlation between the volume of AVM and radiation doses, age, and site of AVM), histopathological findings, and clinical complaints (such as headache and epilepsy) after radiosurgery; (3) bleeding after radiosurgery; (4) complications (including angiography-related complications, those immediately after radiosurgery, delayed complications, factors influencing delayed complications, and endocrine or growth retardation); (5) other problems (such as radiation doses, follow-up strategy, approaches to giant AVMs, and repeat irradiation); (6) indication of radiosurgery for lesions other than brain stem, and lesions of the mid-brain, pons and bulb. (N.K.) 134 refs

  2. Rehabilitation with ear prosthesis linked to osseointegrated implants.

    Science.gov (United States)

    Goiato, Marcelo C; dos Santos, Daniela M; Haddad, Marcela F; Moreno, Amália

    2012-06-01

    The absence of an ear, which can be the result of a congenital malformation, surgical tumour resection or traumatic injury, is a significant aesthetic problem. Attachment of ear prostheses with adhesives can cause local irritation for the wearer and affect the colour of the prostheses. Use of implants in craniofacial reconstruction can improve the retention and stability of prostheses giving to patient greater comfort and security relative to adhesive attachment. The aim of this report was to present a clinical case of a mutilated patient who was rehabilitated by means of installing an ear prosthesis fixed through osseointegrated implants. The patient had two implants installed in the mastoid region that were linked by a bar, and a clip-type system was used. The ear prosthesis was constructed from medical-use silicone, pigmented to match the patient's skin colour and linked to the retention system. The patient's rehabilitation was satisfactory from both a functional and an aesthetic point of view, making it possible for the patient to return to a normal social life and regain lost self-esteem. © 2012 The Gerodontology Society and John Wiley & Sons A/S.

  3. Prevalence of inner ear anomalies among cochlear implant candidates.

    Science.gov (United States)

    Aldhafeeri, Ahmad M; Alsanosi, Abdulrahman A

    2016-10-01

    To determine the prevalence of inner ear anomalies and the frequency of different anomaly types among cochlear implant recipients. This study included a retrospective chart review of all patients who received cochlear implants between January 2009 and January 2013 in King Abdulaziz University Hospital cochlear implant program in Riyadh, Saudi Arabia. All subjects underwent thin-cut CT of the temporal bone and MRI. The collected data included age, gender, and CT and MRI findings regarding temporal bone anomalies. Patients with any identified congenital inner ear anomalies were included in the study.  In total, 316 patients' cases were reviewed. Inner ear malformations were identified in 24 patients, which represented a prevalence of 7.5%. Among these 24 patients, 8 (33.3%) presented with a large vestibular aqueduct (LVA), 8 (33.3%) semicircular canal (SCC) dysplasia, 7 (29.1%) classical Mondini deformity, and one (4.1%) cochlear hypoplasia. The prevalence of inner ear anomalies among cochlear implant recipients was 7.5%. This result is consistent with findings worldwide. The most common anomalies were LVA and SCC hypoplasia; by contrast, in other regions, the most common anomaly is either the Mondini deformity, or LVA.

  4. Activity patterns in malformed fetuses.

    Science.gov (United States)

    Rayburn, W F; Barr, M

    1982-04-15

    Knowledge of a malformed fetus before the onset of labor would assist the physician in preparing the expectant parents, managing the timing and method of delivery, and preparing for the immediate care of a salvageable infant. This 3-year prospective investigation compared the activity patterns of fetuses who were later found to have major malformation with those of fetuses who had no apparent defects. Fetal motion over prolonged periods was determined by daily charting of fetal movement by the mother. Although not a reliable predictor for all malformations, evidence of fetal inactivity was found to be more common (p less than 0.0001) among fetuses with anomalies (16 of 58 cases, 28%) than among those with no defects (39 of 1,098 cases, 4%). All malformations associated with fetal inactivity were strongly suspected ultrasonographically and included hydrocephalus, gastroschisis, nonimmune hydrops, bilateral renal agenesis, and bilateral dislocation of the hips. Documentation of fetal inactivity is helpful in recognizing certain major malformations and constitutes grounds for more detailed study by ultrasonography.

  5. Arteriovenous malformation of the mandible

    International Nuclear Information System (INIS)

    Kim, Dong Youn; Lee, Kang Sook; Choi, Karp Shik

    1995-01-01

    The arteriovenous malformation, an uncommon lesion, is a direct communication between an artery and a vein that bypasses the intervening capillary bed. The authors experienced two cases of arteriovenous malformation in 17-year-old and 18-year-old female patients who had suffered from mandibular pain and gingival bleeding. As a result of careful analysis of clinical and angiographic radiological examinations, we diagnosed them as arteriovenous malformations. The following result were obtained; 1. Main clinical symptoms were dull pain and gingival bleeding on the mandibular body area and redness, pain on palpitation in the lesional sites were also observed. 2. Radiographically, well-defined radiolucent lesions with multiple osteolytic defects were observed. In radionuclide scan, increased uptakes of radioisotope were observed in the lesional sites. 3. Increased venous shunt supplied by the facial, lingual and inferior alveolar arteries was observed in angiography. After embolotherapy was performed, no recurrence was reported during 3 to 6 months follow up.

  6. Pediatric central nervous system vascular malformations

    International Nuclear Information System (INIS)

    Burch, Ezra A.; Orbach, Darren B.

    2015-01-01

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

  7. Pediatric central nervous system vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Burch, Ezra A. [Brigham and Women' s Hospital, Department of Radiology, Boston, MA (United States); Orbach, Darren B. [Boston Children' s Hospital, Neurointerventional Radiology, Boston, MA (United States)

    2015-09-15

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

  8. Arteriovenous Malformation of the Pancreas

    Directory of Open Access Journals (Sweden)

    Alexandros Charalabopoulos

    2011-01-01

    Full Text Available Pancreatic arteriovenous malformation (PAVM is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented.

  9. RISK FACTORS IN CHILD CONGENITAL MALFORMATIONS

    OpenAIRE

    Alina-Costina LUCA; Mirabela SUBOTNICU

    2015-01-01

    Congenital heart malformations are among the most common congenital malformations. Congenital heart malformations occur due to genetic and environmental factors during embryonic morphogenesis period of the heart. About 25% of these malformations are severe, requiring intervention immediately after birth or in infancy. Abnormalities of structure and function of the heart and great vessels are the consequence of teratogenic factors occurring between day 19 and 45 of gestation. (Yagel et al...

  10. Abernethy malformation: a case report

    Directory of Open Access Journals (Sweden)

    Pathak Ashish

    2012-05-01

    Full Text Available Abstract Background Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. Case presentation A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4 weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder. Conclusion The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients.

  11. Congenital Pulmonary Malformation in Children

    Directory of Open Access Journals (Sweden)

    Montasser Nadeem

    2012-01-01

    Full Text Available Congenital Pulmonary Malformations (CPMs are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life.

  12. Congenital Pulmonary Malformation in Children

    OpenAIRE

    Nadeem, Montasser; Elnazir, Basil; Greally, Peter

    2012-01-01

    Congenital Pulmonary Malformations (CPMs) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life.

  13. Reconstruction of partially amputated external ear with costal cartilage graft: case report.

    Science.gov (United States)

    Brunelli, A; Bottini, D J; Cervelli, V; Cervelli, G; Grimaldi, M

    2004-06-01

    Many causes are responsible for secondary anomalies of the outer ear, such as: car accidents, sport- or work-related accidents, assaults, bites from animals or humans, benign or malignant tumours, burns and the effects of surgical interventions of the ear (plastic surgery on the ear or attempts at correction of primary malformations of the ear). The anatomical complexity of the ear makes its reconstruction particularly complicated with post-operative results that are often disappointing. The Authors describe their experience in the reconstruction of a partially amputated outer ear following a dog bite. The therapeutic protocol required various surgical stages. Initially, a cutaneous expander was applied at the level of the mastoid in order to ensure a sufficient quantity of local skin. The second stage was to remove cartilage from the ribs, followed by construction of a cartilaginous model of the ear and its insertion into the subcutaneous mastoid region after removal of the cutaneous expander and any residual ear cartilage. The last stage was to separate the neo-formed outer ear from the mastoid skin with the insertion of a cartilage graft to the posterior region of the reconstructed ear. This graft was covered by the occipital fascia rotated at 180 degrees and by a skin graft removed from the pubis. The postoperative result was satisfactory with recuperation of a good aesthetic appearance of the ear. Aim of the present report is to describe the surgical technique employed in the reconstruction of secondary anomalies of the ear and to highlight errors committed during this procedure. These considerations have allowed us to stress some fundamental elements in the reconstruction of the ear. In particular, the watershed was the awareness that we had to create a cartilaginous model that respected, as far as possible, the anatomy of the outer ear with all its ridges, trenches and cavities. This as well as ensuring a sufficient quantity of local skin in order to cover

  14. Pressure difference receiving ears

    DEFF Research Database (Denmark)

    Michelsen, Axel; Larsen, Ole Næsbye

    2007-01-01

    Directional sound receivers are useful for locating sound sources, and they can also partly compensate for the signal degradations caused by noise and reverberations. Ears may become inherently directional if sound can reach both surfaces of the eardrum. Attempts to understand the physics...... of the eardrum. The mere existence of sound transmission to the inner surface does not ensure a useful directional hearing, since a proper amplitude and phase relationship must exist between the sounds acting on the two surfaces of the eardrum. The gain of the sound pathway must match the amplitude and phase...... of the sounds at the outer surfaces of the eardrums, which are determined by diffraction and by the arrival time of the sound, that is by the size and shape of the animal and by the frequency of sound. Many users of hearing aids do not obtain a satisfactory improvement of their ability to localize sound sources...

  15. Twelve years of chiari-like malformation and syringomyelia scanning in Cavalier King Charles Spaniels in the Netherlands : Towards a more precise phenotype

    NARCIS (Netherlands)

    Wijnrocx, Katrien; Van Bruggen, Leonie W L; Eggelmeijer, Wieteke; Noorman, Erik; Jacques, Arnold; Buys, Nadine; Janssens, Steven; Mandigers, Paul J J

    2017-01-01

    Chiari-like malformation (CM), syringomyelia (SM) and middle ear effusion (also called PSOM) are three conditions that frequently occur in Cavalier King Charles Spaniels (CKCS). Both CM and SM are currently screened in the Netherlands prior to breeding and are graded according to the British

  16. Human ear recognition by computer

    CERN Document Server

    Bhanu, Bir; Chen, Hui

    2010-01-01

    Biometrics deals with recognition of individuals based on their physiological or behavioral characteristics. The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. Unlike the fingerprint and iris, it can be easily captured from a distance without a fully cooperative subject, although sometimes it may be hidden with hair, scarf and jewellery. Also, unlike a face, the ear is a relatively stable structure that does not change much with the age and facial expressions. ""Human Ear Recognition by Computer"" is the first book o

  17. Screening for pulmonary arteriovenous malformations

    DEFF Research Database (Denmark)

    Oxhøj, H; Kjeldsen, A D; Nielsen, G

    2000-01-01

    Pulmonary arteriovenous malformations (PAVM) cause right-to-left shunt and imply risk of paradoxical embolism and cerebral abscess. These complications can be prevented by appropriate treatment. Detection of PAVMs is therefore important, so simple and reliable screening methods are needed...... for this purpose. The aim of this investigation was to compare pulse oximetry and contrast echocardiography as screening tools for detection of pulmonary arteriovenous malformations. Eighty-five hereditary haemorrhagic telangiectasia (HHT) patients and first-degree relatives identified in a comprehensive study...... echocardiography with intravenous injection of echo contrast was performed in all subjects. Outcome measures were oxygen saturation change >2% units on changing body position and echo contrast observed in the left-sided heart chambers. Positive contrast echocardiography indicating the presence of PAVM was found...

  18. Perinatal risk factors including malformation

    International Nuclear Information System (INIS)

    Brachner, A.; Grosche, B.

    1991-10-01

    The study gives a survey of the factors most frequently mentioned in the literature as factors likely to adversely affect a pregnancy. One essential aspect is the discussion of those factors that can be counted among the causes of malformations, as among others, prenatal radiation exposure. The study prepared within the framework of the research project 'Radiobiological environmental monitoring in Bavaria' is intended to serve as a basis for a retrospective and prospective evaluation of infant mortality, perinatal conditions and occurrence of malformations in Bavaria, with the principal idea of drawing up an environment - related health survey. The study therefore, in addition to ionizing radiation also takes into account other detectable risks within the ecologic context, as e.g. industrial installations, refuse incineration plants or waste dumps, or urbanity. (orig./MG) [de

  19. [Identification of risk factors for congenital malformations].

    Science.gov (United States)

    Canals C, Andrea; Cavada C, Gabriel; Nazer H, Julio

    2014-11-01

    The relative importance of congenital malformations as a cause of death in the first year of life is increasing along with the control of preventable causes of perinatal mortality. To identify risk factors for congenital malformations. Retrospective case-control study of births registered in the database of The Latin American Collaborative Study of Congenital Malformations (ECLAMC), in the period 2001-2010. Birth weight and gestational age were significantly lower in cases than controls, behaving as risk factors and associated with a greater severity of congenital malformations. The risk and severity of congenital malformations increased along with mother's age. Fetal growth retardation, a history of congenital malformations in the family, physical factors and acute illnesses of the mother in the first trimester of pregnancy were also significant risk factors for congenital malformations and their severity. The educational level of the mother was a protective factor for congenital malformations and their severity. Variables previously identified as risk factors for congenital malformations, were significantly related with the occurrence of congenital malformations and their severity.

  20. Headache in children with Chiari I malformation.

    Science.gov (United States)

    Toldo, Irene; Tangari, Marta; Mardari, Rodica; Perissinotto, Egle; Sartori, Stefano; Gatta, Michela; Calderone, Milena; Battistella, Pier Antonio

    2014-05-01

    Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children. The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up. Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders. Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation. In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia. © 2014 American Headache Society.

  1. Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

    Directory of Open Access Journals (Sweden)

    Micol Busi

    2015-01-01

    Full Text Available Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT and magnetic resonance imaging (MRI in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years. Among these, 143 patients (64 females and 79 males presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children ranged from 9 months and 16 years (average = 4.4; median = 3.0. Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities

  2. Global Ear. Werke 2001 - 2006

    Index Scriptorium Estoniae

    2006-01-01

    Dresdenis muusikafestivalil "Global Ear" 23.3.03 esitusel Eesti heliloojate muusika: Helena Tulve "lumineux/opaque", Jaan Rääts "Meditation", Mirjam Tally "Aura", Mati Kuulberg "Sonate Nr.4", Mari Vihmand "Seitsmele"

  3. COMMON INFECTIONS OF THE EAR

    African Journals Online (AJOL)

    Enrique

    tion of the skin covering the tympanic membrane and deep exter- nal canal, and the production of ... of combination steroid/antifungal/antibiotic ointment (e.g.. Kenacomb) with a cotton bud ..... protection to the delicate middle ear structures.

  4. Spontaneous ileal perforation complicating low anorectal malformation

    Directory of Open Access Journals (Sweden)

    TiJesuni Olatunji

    2015-01-01

    Full Text Available Anorectal malformation is a common anomaly in neonates. Although colorectal perforations have been reported as a complication, ileal perforation is rarely encountered. This is a report of a 2-day-old boy presenting with a low anorectal malformation, complicated with ileal perforation, necessitating laparotomy and ileal repair. Anoplasty was done for the low anomaly. Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication.

  5. Congenital malformations of the skull and meninges.

    Science.gov (United States)

    Kanev, Paul M

    2007-02-01

    The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

  6. Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Hannah E. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Moore, Kevin R. [University of Utah School of Medicine, Department of Radiology, Salt Lake City, UT (United States); Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

    2014-11-15

    Complex Chiari malformation is a subgroup of Chiari 1 malformation with distinct imaging features. Children with complex Chiari malformation are reported to have a more severe clinical phenotype and sometimes require more extensive surgical treatment than those with uncomplicated Chiari 1 malformation. We describe reported MR imaging features of complex Chiari malformation and evaluate the utility of craniometric parameters and qualitative anatomical observations for distinguishing complex Chiari malformation from uncomplicated Chiari 1 malformation. We conducted a retrospective search of the institutional imaging database using the keywords ''Chiari'' and ''Chiari 1'' to identify children imaged during the 2006-2011 time period. Children with Chiari 2 malformation were excluded after imaging review. We used the first available diagnostic brain or cervical spine MR study for data measurement. Standard measurements and observations were made of obex level (mm), cerebellar tonsillar descent (mm), perpendicular distance to basion-C2 line (pB-C2, mm), craniocervical angle (degrees), clivus length, and presence or absence of syringohydromyelia, basilar invagination and congenital craniovertebral junction osseous anomalies. After imaging review, we accessed the institutional health care clinical database to determine whether each subject clinically met criteria for Chiari 1 malformation or complex Chiari malformation. Obex level and craniocervical angle measurements showed statistically significant differences between the populations with complex Chiari malformation and uncomplicated Chiari 1 malformation. Cerebellar tonsillar descent and perpendicular distance to basion-C2 line measurements trended toward but did not meet statistical significance. Odontoid retroflexion, craniovertebral junction osseous anomalies, and syringohydromyelia were all observed proportionally more often in children with complex Chiari malformation than in

  7. Congenital malformations in embryos of female mice exposed to alcohol and nicotinamide

    Directory of Open Access Journals (Sweden)

    Natasha Soares Simões dos Santos

    2009-03-01

    Full Text Available Objective: To compare the incidence of congenital malformations among the offspring of female mice exposed to alcohol or alcohol plus nicotinamide. Methods: Three groups of pregnant C57BL/6J mice were studied; G1 received alcohol (5 g/kg in saline solution (20% - vol/vol; G2 received nicotinamide, 50 mg/ml associated to alcohol; and G3, only saline solution; all by intraperitoneal injection on the seventh day of pregnancy. The animals were killed in a CO2 chamber on day 18 of pregnancy. The intrauterine content was assessed and the number of complete and reabsorbed fetuses was counted. The complete fetuses had their weight and crown-rump length measured and malformations were identified. Rresults: G1 showed the highest number of malformations: micrognathia, low set ears, hypertrophic nose, scoliosis, and atrophy of the lower and upper limbs. Weight was significantly different among the groups (p = 0.0139, and in G1 it was below average as compared to G3 (p = 0.3133. As for length, the lowest values were found in G2 and G3 showed the highest ones. There was a significant difference among the groups (p = 0.0145. Cconclusions: Ethanol, when administered to pregnant mice was teratogenic. However, length of G1 fetuses was, in average, higher than that of other groups. Nicotinamide decreased the number of malformations and may be a possible protector against alcohol effects.

  8. Embolization of spinal arteriovenous malformations

    International Nuclear Information System (INIS)

    Son, Mi Young; Kim, Sun Yong; Park, Bok Hwan

    1990-01-01

    Recently, therapeutic embolization has been advocated as the treatment of choice for spinal AVM(arteriovenous malformations). The authors review our experience with two cases of spinal AVM treated by embolization using coaxial Tracker-18 microcatheter with Latvian. The patients included a 10 year old male with glomus type and a 14 year old female with juvenile type spinal AVM revealed recanalization 5 month later. Embolization provides curative or temporary treatment for spinal AVM. After embolic occlusion, delayed reassessment with arteriography is indicated, particularly if symptoms persist or recur

  9. 3D Printed Bionic Ears

    Science.gov (United States)

    Mannoor, Manu S.; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A.; Soboyejo, Winston O.; Verma, Naveen; Gracias, David H.; McAlpine, Michael C.

    2013-01-01

    The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the precise anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing. PMID:23635097

  10. 3D printed bionic ears.

    Science.gov (United States)

    Mannoor, Manu S; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A; Soboyejo, Winston O; Verma, Naveen; Gracias, David H; McAlpine, Michael C

    2013-06-12

    The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing.

  11. Ear Disorders in Scuba Divers

    Directory of Open Access Journals (Sweden)

    MH Azizi

    2010-12-01

    Full Text Available History of underwater diving dates back to antiquity. Breath-hold technique in diving was known to the ancient nations. However, deep diving progressed only in the early decades of the 19th century as the result of advancements in efficient underwater technologies which subsequently led to invention of sophisticated sets of scuba diving in the 20th century. Currently, diving is performed for various purposes including commercial, recreational, military, underwater construction, oil industry, underwater archeology and scientific assessment of marine life. By increasing popularity of underwater diving, dive-related medical conditions gradually became more evident and created a new challenge for the health care professionals, so that eventually, a specialty the so-called “diving medicine” was established. Most of the diving-associated disorders appear in the head and neck. The most common of all occupational disorders associated with diving are otologic diseases. External otitis has been reported as the most common otolaryngologic problem in underwater divers. Exostosis of the external ear canal may be formed in divers as the result of prolonged diving in cold waters. Other disorders of the ear and paranasal sinuses in underwater divers are caused by barometric pressure change (i.e., barotraumas, and to a lesser extent by decompression sickness. Barotrauma of the middle ear is the most prevalent barotrauma in divers. The inner ear barotraumas, though important, is less common. The present paper is a brief overview of diving-related ear disorders particularly in scuba divers.

  12. Genetics Home Reference: megalencephaly-capillary malformation syndrome

    Science.gov (United States)

    ... Additional NIH Resources (1 link) National Institute of Neurological Disorders and Stroke: Megalencephaly Educational Resources (5 links) Boston Children's Hospital: Capillary Malformation Cincinnati Children's Hospital: Capillary Malformations ...

  13. Fetal chromosome abnormalities and congenital malformations: an ...

    African Journals Online (AJOL)

    The results also showed that Multiple congenital anomalies (MCA) represented among 42.2%, congenital malformation of CNS represents 26.6%, congenital malformation of the skeletal system 20%, congenital polycystic kidney 8.8% and pyloric stenosis in 2.2%. Among the 21 women with abnormal karyotype of amniotic ...

  14. Congenital malformations among newborns in Kenya | Muga ...

    African Journals Online (AJOL)

    Therefore, a study was conducted to determine the patterns and incidence of congenital malformations at birth in newborns in Kenya and thereby analyze associated predisposing factors in their mothers. This single cross-sectional ... followed by malformations of the central nervous system (28.6%). Polydactyl was the most ...

  15. Skeletal malformations in fetuses with Meckel syndrome

    DEFF Research Database (Denmark)

    Kjaer, K W; Fischer Hansen, B; Keeling, J W

    1999-01-01

    one foot was normal. Malformations of the cranial base (the basilar part of the occipital bone or the postsphenoid bone) occurred in five cases, and the vertebral bodies in the lumbar region of the spine were malformed (cleft) in three cases. It is proposed that a skeletal analysis be included...

  16. Cerebellar arteriovenous malformations in children

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, P.D. [Sheffield Univ. (United Kingdom). Acad. Dept. of Radiol.; Blaser, S.; Armstrong, D.; Chuang, S.; Harwood-Nash, D. [Division of Neuroradiology, The Hospital for Sick Children and University of Toronto, Toronto (Canada); Humphreys, R.P. [Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto (Canada)

    1998-05-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.) With 4 figs., 4 tabs., 23 refs.

  17. Subfascial involvement in glomuvenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Shaikh, Raja; Alomari, Ahmad I.; Chaudry, Gulraiz [Boston Children' s Hospital, Division of Interventional Radiology, Boston, MA (United States); Mulliken, John B. [Boston Children' s Hospital, Division of Plastic Surgery, Boston, MA (United States); Fishman, Steven J. [Boston Children' s Hospital, Department of Surgery, Boston, MA (United States); Kozakewich, Harry P.W. [Boston Children' s Hospital, Department of Pathology, Boston, MA (United States)

    2014-07-15

    Glomuvenous malformation (GVM) is an inherited autosomal dominant trait. The lesions, which appear as bluish nodules or plaque-like cutaneous elevations, are usually tender and more firm than sporadic venous malformations. Conventionally, the lesions are thought to be limited to the cutaneous and subcutaneous tissue planes. The objective was to characterize the depth of involvement of GVM lesions. Magnetic resonance imaging (MRI) findings in GVM were retrospectively evaluated by two radiologists. The signal characteristics, tissue distribution, pattern of contrast enhancement of the lesions in GVM were documented. Thirty patients (19 female) aged 1-35 years (mean 18 years) were diagnosed with GVM based on clinical features (n = 20) and/or histopathological findings (n = 10). The lesions were present in the lower extremity (n = 15), upper extremity (n = 6), cervico-facial region (n = 6), pelvis (n = 2), and chest wall (n = 1). All patients had skin and subcutaneous lesions. Fifty percent of the patients (n = 15) demonstrated subfascial intramuscular (n = 15), intra-osseous (n = 1), and intra-articular involvement (n = 1). Contrary to the conventional belief that GVMs are generally limited to the skin and subcutaneous tissue, deep subfascial extension of the lesions is common. (orig.)

  18. Cerebellar arteriovenous malformations in children

    International Nuclear Information System (INIS)

    Griffiths, P.D.; Humphreys, R.P.

    1998-01-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.)

  19. The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear.

    Directory of Open Access Journals (Sweden)

    Amy E Kiernan

    2006-01-01

    Full Text Available In mammals, six separate sensory regions in the inner ear are essential for hearing and balance function. Each sensory region is made up of hair cells, which are the sensory cells, and their associated supporting cells, both arising from a common progenitor. Little is known about the molecular mechanisms that govern the development of these sensory organs. Notch signaling plays a pivotal role in the differentiation of hair cells and supporting cells by mediating lateral inhibition via the ligands Delta-like 1 and Jagged (JAG 2. However, another Notch ligand, JAG1, is expressed early in the sensory patches prior to cell differentiation, indicating that there may be an earlier role for Notch signaling in sensory development in the ear. Here, using conditional gene targeting, we show that the Jag1 gene is required for the normal development of all six sensory organs within the inner ear. Cristae are completely lacking in Jag1-conditional knockout (cko mutant inner ears, whereas the cochlea and utricle show partial sensory development. The saccular macula is present but malformed. Using SOX2 and p27kip1 as molecular markers of the prosensory domain, we show that JAG1 is initially expressed in all the prosensory regions of the ear, but becomes down-regulated in the nascent organ of Corti by embryonic day 14.5, when the cells exit the cell cycle and differentiate. We also show that both SOX2 and p27kip1 are down-regulated in Jag1-cko inner ears. Taken together, these data demonstrate that JAG1 is expressed early in the prosensory domains of both the cochlear and vestibular regions, and is required to maintain the normal expression levels of both SOX2 and p27kip1. These data demonstrate that JAG1-mediated Notch signaling is essential during early development for establishing the prosensory regions of the inner ear.

  20. Mutation of Rubie, a novel long non-coding RNA located upstream of Bmp4, causes vestibular malformation in mice.

    Directory of Open Access Journals (Sweden)

    Kristina A Roberts

    Full Text Available The vestibular apparatus of the vertebrate inner ear uses three fluid-filled semicircular canals to sense angular acceleration of the head. Malformation of these canals disrupts the sense of balance and frequently causes circling behavior in mice. The Epistatic circler (Ecl is a complex mutant derived from wildtype SWR/J and C57L/J mice. Ecl circling has been shown to result from the epistatic interaction of an SWR-derived locus on chromosome 14 and a C57L-derived locus on chromosome 4, but the causative genes have not been previously identified.We developed a mouse chromosome substitution strain (CSS-14 that carries an SWR/J chromosome 14 on a C57BL/10J genetic background and, like Ecl, exhibits circling behavior due to lateral semicircular canal malformation. We utilized CSS-14 to identify the chromosome 14 Ecl gene by positional cloning. Our candidate interval is located upstream of bone morphogenetic protein 4 (Bmp4 and contains an inner ear-specific, long non-coding RNA that we have designated Rubie (RNA upstream of Bmp4 expressed in inner ear. Rubie is spliced and polyadenylated, and is expressed in developing semicircular canals. However, we discovered that the SWR/J allele of Rubie is disrupted by an intronic endogenous retrovirus that causes aberrant splicing and premature polyadenylation of the transcript. Rubie lies in the conserved gene desert upstream of Bmp4, within a region previously shown to be important for inner ear expression of Bmp4. We found that the expression patterns of Bmp4 and Rubie are nearly identical in developing inner ears.Based on these results and previous studies showing that Bmp4 is essential for proper vestibular development, we propose that Rubie is the gene mutated in Ecl mice, that it is involved in regulating inner ear expression of Bmp4, and that aberrant Bmp4 expression contributes to the Ecl phenotype.

  1. In-the-Ear Hearing-Instrument Antenna for ISM-Band Body-Centric Ear-to-Ear Communications

    DEFF Research Database (Denmark)

    Yatman, William H.; Larsen, Lauge K; Kvist, Søren Helstrup

    2012-01-01

    A compact 2.45 GHz slot-loop antenna is implemented for the use in the outer shell of an in-the-ear (ITE) hearing instrument (HI). The antenna is optimized for high ear-to-ear path gain (jS21j). The antenna simulation results are presented for two identical antennas, one placed in the center of e...

  2. Digital Active Noise Reduction Ear Plugs

    National Research Council Canada - National Science Library

    Harley, Thomas

    1994-01-01

    .... In contrast to available ANR headsets that implement fixed analog filters, the prototype defines a digital filter that is optimally defined for the user's current acoustical environment. An above-the-ear (ATE) and an in-the-ear (ITE...

  3. Middle ear infection (otitis media) (image)

    Science.gov (United States)

    Otitis media is an inflammation or infection of the middle ear. Acute otitis media (acute ear infection) occurs when there is ... which causes production of fluid or pus. Chronic otitis media occurs when the eustachian tube becomes blocked ...

  4. External ear: An analysis of its uniqueness

    Directory of Open Access Journals (Sweden)

    Ruma Purkait

    2016-06-01

    Hence, the individuality of every ear has been confirmed which may find use in personal identification studies. The study is a step towards providing scientific support for admitting ear evidence in the Court of Law.

  5. Proteomics and the Inner Ear

    Directory of Open Access Journals (Sweden)

    Isolde Thalmann

    2001-01-01

    Full Text Available The inner ear, one of the most complex organs, contains within its bony shell three sensory systems, the evolutionary oldest gravity receptor system, the three semicircular canals for the detection of angular acceleration, and the auditory system - unrivaled in sensitivity and frequency discrimination. All three systems are susceptible to a host of afflictions affecting the quality of life for all of us. In the first part of this review we present an introduction to the milestones of inner ear research to pave the way for understanding the complexities of a proteomics approach to the ear. Minute sensory structures, surrounded by large fluid spaces and a hard bony shell, pose extreme challenges to the ear researcher. In spite of these obstacles, a powerful preparatory technique was developed, whereby precisely defined microscopic tissue elements can be isolated and analyzed, while maintaining the biochemical state representative of the in vivo conditions. The second part consists of a discussion of proteomics as a tool in the elucidation of basic and pathologic mechanisms, diagnosis of disease, as well as treatment. Examples are the organ of Corti proteins OCP1 and OCP2, oncomodulin, a highly specific calcium-binding protein, and several disease entities, Meniere's disease, benign paroxysmal positional vertigo, and perilymphatic fistula.

  6. Arteriovenous malformations of the uterus.

    Science.gov (United States)

    Cura, M; Martinez, N; Cura, A; Dalsaso, T J; Elmerhi, F

    2009-09-01

    Arterial venous malformations (AVM) of the uterus are uncommon entities and should be considered in patients who present with profuse genital bleeding. There are two types of uterine AVM: acquired and congenital. Acquired uterine AVMs are conformed by communications between the uterine arteries and the myometrial veins, and are caused by an iatrogenic event or a pathological condition. Congenital AVMs are the result of abnormal development of primitive vessels that result in connections between pelvic arteries and veins in the uterus without an interconnecting capillary bed. Ultrasonography is a noninvasive diagnostic method able to demonstrate and characterize AVMs of the uterus. AVM in the pelvis may be noted incidentally by computed tomography (CT) of the pelvis, and magnetic resonance imaging (MRI) is frequently used to confirm and further characterize the sonographic findings of uterine AVM. Catheter angiography and embolization are very effective in defining the vascular anatomy and treating uterine vascular abnormalities.

  7. Can Loud Music Hurt My Ears?

    Science.gov (United States)

    ... Videos for Educators Search English Español Can Loud Music Hurt My Ears? KidsHealth / For Kids / Can Loud Music Hurt My Ears? Print en español La música ... up? Oh! You want to know if loud music can hurt your ears . Are you asking because ...

  8. Congenital heart defects and extracardiac malformations.

    Science.gov (United States)

    Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

    2013-06-01

    To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

  9. Chiari malformations: diagnosis, treatments and failures.

    Science.gov (United States)

    Abd-El-Barr, M M; Strong, C I; Groff, M W

    2014-12-01

    Chiari malformations refer to abnormalities of the hindbrain originally described by the Austrian pathologist Hans Chiari in the early 1890s. These malformations range from herniation of the cerebellar tonsils through the foramen magnum to complete agenesis of the cerebellum. In this review, we review the different classification schemes of Chiari malformations. We discuss the different signs and symptoms that the two most common malformations present with and diagnostic criteria. We next discuss current treatment paradigms, including the new measure of possible in utero surgery to help decrease the incidence of Chiari type II malformations. There is also a small discussion of treatment failures and salvage procedures in these difficult cases. Chiari malformations are a difficult clinical entity to treat. As more is learned about the genetic and environmental factors relating to their characteristics, it will be interesting if we are able to predict which treatments are better suited for different patients. Similarly, with the evolution of in utero techniques especially for Chiari II malformations, it will be interesting to see if the incidence and practice of treating these difficult patients will change.

  10. Pattern and outcome of gross congenital malformations at birth ...

    African Journals Online (AJOL)

    Background: Congenital malformation(s) do occur in newborns and are thought to be often responsible for a significant proportion of perinatal morbidity and mortality worldwide. Objective: This prospective study was designed to determine the pattern and outcome of congenital malformation(s) among newborn deliveries ...

  11. Unusual association of congenital middle ear cholesteatoma and first branchial cleft anomaly: management and embryological concepts.

    Science.gov (United States)

    Nicollas, R; Tardivet, L; Bourlière-Najean, B; Sudre-Levillain, I; Triglia, J M

    2005-02-01

    To report two cases of an undescribed association of first branchial cleft fistula and middle ear congenital cholesteatoma and to discuss management and embryological hypothesis. Retrospective study and review of the literature Both patients were young girls free of past medical or surgical history. Surgical removal of the first cleft anomaly found in the two cases a fistula routing underneath the facial nerve. Both cholesteatomas were located in the hypotympanum, mesotympanum. In one case, an anatomical link between the two malformations was clearly identified with CT scan. The main embryological theories and classification are reviewed. A connection between Aimi's and Michaels' theories (congenital cholesteatoma) and Work classification might explain the reported clinical association.

  12. Carcinoid tumour of the middle ear

    LENUS (Irish Health Repository)

    Baig, Salman

    2012-09-01

    A case of middle ear mass in a young female from Ireland is described, who presented with left ear hearing loss and intermittent bloody discharge from the same ear. Examination under microscope revealed occlusive polyp in the left ear and a biopsy had been taken under general anaesthesia. Histopathology report described an adenoma \\/ carcinoid tumour of the middle ear confirmed by positive immunohistochemical staining. CT temporal bones revealed the extension of the disease. The patient underwent left tympanotomy and excision of the tumour. In general, these tumours are regarded as benign but may be mistaken for adenocarcinomas because of their histological heterogenecity.

  13. Bilateral absence of fifth ray in feet, cleft palate, malformed ears, and ...

    African Journals Online (AJOL)

    Rabah M. Shawky

    2014-01-04

    Jan 4, 2014 ... Abstract Background: Miller syndrome is one of the acrofacial dysostosis syndromes, which are characterized by ... scar of cleft lip operation, thin upper lip, and high arched pal- ... rise of serum testosterone from baseline level.

  14. Malformations and abnormalities of the petrous portion of the temporal bone

    International Nuclear Information System (INIS)

    Reith, W.; Yilmaz, U.; Heumueller, I.

    2014-01-01

    High-resolution computed tomography (HRCT) is the procedure of choice in the diagnostics of abnormalities of the middle and inner ear. It allows a detailed presentation of anatomical features and achieves the prerequisites for selection of the various therapeutic options. The highly diverse abnormalities can be described using detailed imaging analyses. Malformations with an abnormally developed modiolus are assumed to be early embryological defects, such as the classical Mondini dysplasia. The essential therapeutic option for middle ear deformities is still a cochlear implant. The domain of magnetic resonance imaging (MRI) is not only in the analysis of the cochlear nerve and for exclusion of fibrosis or ossification of the labyrinth but is also able to visualize details of isolated malformations, such as an extended vestibular aqueduct or subtle alterations to the vestibule or can visualize them better in comparison to CT. Radiological diagnostics are used not only for classification but also to recognize typical clinical problem situations and play a key role in the diagnostics of hearing disorders and selection of the optimal therapeutic procedure. (orig.) [de

  15. Importance of bony analysis for interpreting ear CT scans: part three; ORL - tomodensitometrie de l'oreille. Interet de l'analyse osseuse dans l'interpretation des scanners de l'oreille (troisieme partie)

    Energy Technology Data Exchange (ETDEWEB)

    Serhal, M.; Dordea, M.; Cymbalista, M. [Hopital de Montfermeil, Service de Radiologie, 93 - Montfermeil (France); Halimi, P. [Hopital Europeen Georges-Pompidou, Service de Radiologie, 75 - Paris (France); Iffenecker, C. [Clinique Radiologique, 62 - Boulogne sur Mer (France); Bensimon, J.L

    2003-02-01

    The accurate description of bony changes in ear CT scans has a great diagnostic and therapeutic impact. The third part shows the way to analyze bone remodeling when CT scan is performed for tumors in the vicinity of the temporal bone, for intra temporal lesions of the facial nerve and for external auditory canal malformations. It demonstrates how bony analysis should be included in postoperative report of ear CT scan. The importance of bony signs in tumors and pseudo tumors of the inner ear are outlined. (authors)

  16. Cardiovascular malformations in infants of diabetic mothers

    OpenAIRE

    Wren, C; Birrell, G; Hawthorne, G

    2003-01-01

    Objective: To compare the prevalence at live birth and the spectrum of cardiovascular malformations in infants born to diabetic mothers with pre-existing diabetes with that in infants of non-diabetic mothers.

  17. Human malformations induced by environmental noxae

    International Nuclear Information System (INIS)

    Hecker, W.C.; Angerpointner, T.A.

    1980-01-01

    The paper reviews congenital malformations in humans and presents possible causes. 60% of all malformations are a result of environmental and other factors; i.e. not hereditary or caused by a disease of the mother. The teratogenic effects of ionizing radiation, drugs, alcohol, polyvinyl chloride and trichlorophenol are discussed as well as the effect of the mother's working in certain fields, e.g. clinical laboratories or printing offices; in the latter case the teratogenic noxae are still unknown. Efficient research requires centralized storage of all data on children born with malformations and on the mother's health situation during pregnancy, and the legislator is asked to do so while observing the law on data protection. Foundation of a German Institute of Teratology is recommended. In order to intensify research, it is suggested to set up groups or departments for research on malformations in some major paediatric hospitals. (MG) [de

  18. Chiari I malformation associated with turner syndrome

    Directory of Open Access Journals (Sweden)

    Kamble Jayaprakash Harsha

    2017-01-01

    Full Text Available Turner syndrome (TS is a rare genetic disease due to the absence of one X chromosome. Patients with TS have more subtle neurological/neuropsychiatric problems, while headache is an uncommon clinical presentation which needs attention. We report a 12-year-old child presenting with typical cough headache. Her magnetic resonance imaging revealed Chiari I malformation associated with TS. To the best of our knowledge, Chiari I malformation associated with TS is not described in literature. We report the first case of TS associated with Chiari I malformation. Interestingly, Chiari I malformation is also associated with Noonan's syndrome, which is a close morphological mimicker of TS, raising the possibility of sharing similar pathogenesis in both conditions.

  19. Syringomyelia associated with Chiari malformation in children

    International Nuclear Information System (INIS)

    Sakamoto, Hiroaki; Kitano, Shouhei; Nishikawa, Misao; Yasui, Toshihiro; Fujitani, Ken; Hakuba, Akira; Nakanishi, Naruhiko

    1997-01-01

    Among 28 patients with myelomeningocele (MMC group), the myelomeningocele in all patients was repaired shortly after birth, and a shunt was implanted for the associated hydrocephalus in 18 patients. MRI of the group of 28 indicated 20 were afflicted with Chiari II malformation, and the remaining 8 by Chiari I malformation. Among 8 patients lacking myelomeningocele (non-MMC group), seven demonstrated a large syrinx at the cervical and cervico-thoracic level; only one had a syrinx extending from the cervical level down to the lumbar level. None of these patients had hydrocephalus. Surgical decompression to improve cerebrospinal fluid (CSF) flow at the major cistern improved neurological signs in 7 patients. MRI indicated 4 patients were afflicted with Chiari I malformation, and the remaining 4 with Chiari II malformation. In the MMC group, the initial development of the syrinx at the lumbar level may be the result of a combination of occlusion of the caudal end of the central canal brought about by repair of the myelomeningocele and CSF flow into the hydromyelic cavity via the patent proximal portion of the central canal. In the non-MMC group, the syringomyelia may be considered an early onset type of syringomyelia associated with adult type Chiari malformation because the location of the syrinx was quite similar to that found in adult type Chiari malformation, and decompressive surgery was quite effective. In the non-MMC group, turbulence of the CSF now at the major cistern caused by the herniated cerebellum plays an important role in the enlargement of the syringomyelia. To offer greater appropriate management of pediatric Chiari malformation accompanied by syringomyelia, the malformation should be classified not by degree of the herniated brain tissue but by its association with neural tube defect (myelomeningocele). (K.H.)

  20. Congenital pseudoarthrosis associated with venous malformation

    International Nuclear Information System (INIS)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N.; Hamamy, H.; Al-Hadidi, S.

    2007-01-01

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  1. Common congenital malformations of the brain

    International Nuclear Information System (INIS)

    Naidich, T.P.; Zimmerman, R.A.

    1987-01-01

    In nearly all cases, congenital malformations are characterized most easily by their anatomic features and are best images with T1-weighted short TR/short TE pulse sequences. T2-weighted, long TR/long TE images are used primarily for the phakomatoses that are commonly associated with brain tumors. This chapter reviews the features of the most common congenital malformations and illustrates their typical magnetic resonance imaging (MRI) appearance

  2. Congenital bronchopulmonary foregut malformations: concepts and controversies

    International Nuclear Information System (INIS)

    Newman, Beverley

    2006-01-01

    This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

  3. Congenital pseudoarthrosis associated with venous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N. [Jordan University Hospital, Radiology Department, P.O. Box 340621, Amman (Jordan); Hamamy, H. [Endocrinology and Genetics, National Center for Diabetes, Amman (Jordan); Al-Hadidi, S. [Jordan University Hospital, Departments of Orthopedics, Amman (Jordan)

    2007-06-15

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  4. Congenital bronchopulmonary foregut malformations: concepts and controversies

    Energy Technology Data Exchange (ETDEWEB)

    Newman, Beverley [University of Pittsburgh School of Medicine and Children' s Hospital of Pittsburgh, Pittsburgh, PA (United States)

    2006-08-15

    This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

  5. 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.

  6. [Neonatal tumours and congenital malformations].

    Science.gov (United States)

    Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

    2008-06-01

    The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

  7. Characteristics and treatment of cavernous malformations

    International Nuclear Information System (INIS)

    Takagi, Kenji; Ishida, Mitsuaki; Okabe, Hidetoshi; Nozaki, Kazuhiko

    2013-01-01

    Cavernous malformations are clusters of dilated sinusoidal channels with thin walls devoid of elastin and smooth muscle. They have no intervening brain tissue. They occur both in sporadic and familial forms. The genes responsible for cavernous malformations have been identified. Recent reports show that mutations of these responsible genes are involved not only in familial but also in sporadic forms. Germline and somatic mutations may occur before cavernous malformations develop (two-hit mechanism). Two patterns, with mulberry-like and hematoma-like appearances, are seen intraoperatively, and from histological findings, mulberry-like appearance may change into hematoma-like one by intralesional hemorrhage. Cavernous malformation treatments include open surgery and radiosurgery. Open surgery is usually chosen for the treatment of symptomatic hemorrhagic cavernous malformations because post-radiosurgical annual bleeding risks at the early phase seem to be higher than those of open surgery. If open surgery has a high risk because of lesion location, radiosurgery becomes an effective alternative treatment. Brainstem cavernous malformations have high annual bleeding and re-bleeding incidence, so if the lesion is accessible with low risk, open surgery is recommended. (author)

  8. Giant pediatric cervicofacial lymphatic malformations.

    Science.gov (United States)

    Benazzou, Salma; Boulaadas, Malik; Essakalli, Leila

    2013-07-01

    Lymphatic malformations (LMs) are benign lesions. Most of them are found in head and neck regions as asymptomatic mass, but giant lymphangiomas may affect breathing or swallowing and constitute a major therapeutic challenge. A retrospective analysis of giant head and neck LMs with impairment of respiration or swallow for the past 11 years was performed in the Department of Maxillofacial Surgery and ENT of the Avicenne Medical University Center. Seven patients with large and extensive LMs of the head and neck were identified. There were 3 males and 4 females with a mean age of 6 years. The predominant reason for referral was airway compromise necessitating tracheostomy (57%) and dysphagia (43%). Three patients had macrocystic lesions; others were considered mixed or microcystic. All the patients underwent surgical excision as a primary treatment modality. Complete surgical resection was realized in 4 patients, and subtotal resection in 3 patients. Of 7 patients, 4 patients had complications including nerve damage and recurrence of the disease. The majority of the patients underwent only a single surgical procedure. Cervicofacial LMs in children should be managed in multidisciplinary setting. Surgery remains the first treatment for managing giant, life-threatening lesions.

  9. Management of intracranial arteriovenous malformations

    International Nuclear Information System (INIS)

    Miyamoto, Susumu; Takahashi, Jun C.

    2008-01-01

    Intracranial arteriovenous malformations (AVMs) are congenital lesions that can cause serious neurological deficits or even death. They can manifest as intracranial hemorrhage, epileptic seizure, or other symptoms such as headache or tinnitus. They are detected by computed tomography or magnetic resonance imaging. Recently there have been significant developments in the management of AVMs. In this paper, the authors represent an overview of the epidemiology of AVMs and the existing treatment strategies. AVMs are ideally excised by standard microsurgical techniques. The grading scale which was proposed by Spetzler and Martin is widely used to estimate the risk of direct surgery. Stereotactic radiosurgery such as that using a gamma knife is very useful for small lesions located in eloquent areas. Technological advances in endovascular surgery have provided new alternatives in the treatment of AVMs. Currently indications for embolization can be divided into presurgical embolization in large AVMs to occlude deep arterial feeding vessels and embolization before stereotactic radiosurgery to reduce the size of the nidus. Palliative embolization can be also applied for patients with large, inoperable AVMs who are suffering from progressive neurological deficits secondary to venous hypertension and/or arterial steal phenomenon. (author)

  10. Management of intracranial arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Miyamoto, Susumu; Takahashi, Jun C [National Cardiovascular Center, Suita, Osaka (Japan)

    2008-10-15

    Intracranial arteriovenous malformations (AVMs) are congenital lesions that can cause serious neurological deficits or even death. They can manifest as intracranial hemorrhage, epileptic seizure, or other symptoms such as headache or tinnitus. They are detected by computed tomography or magnetic resonance imaging. Recently there have been significant developments in the management of AVMs. In this paper, the authors represent an overview of the epidemiology of AVMs and the existing treatment strategies. AVMs are ideally excised by standard microsurgical techniques. The grading scale which was proposed by Spetzler and Martin is widely used to estimate the risk of direct surgery. Stereotactic radiosurgery such as that using a gamma knife is very useful for small lesions located in eloquent areas. Technological advances in endovascular surgery have provided new alternatives in the treatment of AVMs. Currently indications for embolization can be divided into presurgical embolization in large AVMs to occlude deep arterial feeding vessels and embolization before stereotactic radiosurgery to reduce the size of the nidus. Palliative embolization can be also applied for patients with large, inoperable AVMs who are suffering from progressive neurological deficits secondary to venous hypertension and/or arterial steal phenomenon. (author)

  11. Anomalous facial nerve canal with cochlear malformations.

    Science.gov (United States)

    Romo, L V; Curtin, H D

    2001-05-01

    Anteromedial "migration" of the first segment of the facial nerve canal has been previously identified in a patient with a non-Mondini-type cochlear malformation. In this study, several patients with the same facial nerve canal anomaly were reviewed to assess for the association and type of cochlear malformation. CT scans of the temporal bone of 15 patients with anteromedial migration of the first segment of the facial nerve canal were collected from routine departmental examinations. In seven patients, the anomalous course was bilateral, for a total of 22 cases. The migration was graded relative to normal as either mild/moderate or pronounced. The cochlea in each of these cases was examined for the presence and size of the basilar, second, and apical turns. The turns were either absent, small, normal, or enlarged. The CT scans of five patients with eight Mondini malformations were examined for comparison. The degree of the facial nerve migration was pronounced in nine cases and mild/moderate in 13. All 22 of these cases had associated cochlear abnormalities of the non-Mondini variety. These included common cavity anomalies with lack of definition between the cochlea and vestibule (five cases), cochleae with enlarged basilar turns and absent second or third turns (five cases), and cochleae with small or normal basilar turns with small or absent second or third turns (12 cases). None of the patients with Mondini-type cochlear malformations had anteromedial migration of the facial nerve canal. Anteromedial migration of the facial nerve canal occurs in association with some cochlear malformations. It did not occur in association with the Mondini malformations. A cochlea with a Mondini malformation, being similar in size to a normal cochlea, may physically prohibit such a deviation in course.

  12. Internally Coupled Ears in Living Mammals

    OpenAIRE

    Mason, Matthew James

    2015-01-01

    It is generally held that the right and left middle ears of mammals are acoustically isolated from each other, such that mammals must rely on neural computation to derive sound localisation cues. There are, however, some unusual species in which the middle ear cavities intercommunicate, in which case each ear might be able to act as a pressure-difference receiver. This could improve sound localisation at lower frequencies. The platypus Ornithorhynchus is apparently unique among mammals in tha...

  13. Multiple pregnancies achieved with IVF/ICSI and risk of specific congenital malformations: a meta-analysis of cohort studies.

    Science.gov (United States)

    Zheng, Zan; Chen, Letao; Yang, Tubao; Yu, Hong; Wang, Hua; Qin, Jiabi

    2018-04-01

    Studies comparing risk of specific congenital malformations (CM) between multiple pregnancies resulting from IVF/intracytoplasmic sperm injection (ICSI) and those conceived naturally report conflicting results; furthermore, there is a lack of a complete overview. This meta-analysis aimed to address which types of CM are increased in IVF/ICSI multiple pregnancies compared with those conceived naturally. All studies testing the association between IVF/ICSI multiple pregnancies and specific CM identified in various databases were considered. The literature search yielded 856 records, of which 21 cohort studies were included for analysis. Overall, multiple pregnancies achieved with IVF/ICSI experienced a significantly higher risk of chromosomal defects (relative risk [RR] = 1.36; 95% confidence interval [CI]: 1.04-1.77), urogenital (RR = 1.18; 95% CI: 1.03-1.36) and circulatory (RR = 1.22; 95% CI: 1.01-1.47) system malformations. However, the remaining specific CM, such as cleft lip and/or palate, eye, ear, face and neck, respiratory, musculoskeletal, nervous and digestive system malformations, were similar in the two groups. No substantial heterogeneity was observed for most outcomes except for digestive (P = 0.094; I 2 = 38.3%) and circulatory (P = 0.070; I 2 = 35.2%) system malformations. These findings provide additional information on risks of IVF/ICSI for use when counselling patients. Copyright © 2018 Reproductive Healthcare Ltd. Published by Elsevier Ltd. All rights reserved.

  14. An Effective 3D Ear Acquisition System.

    Directory of Open Access Journals (Sweden)

    Yahui Liu

    Full Text Available The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. It can be easily captured from a distance without a fully cooperative subject. Also, the ear has a relatively stable structure that does not change much with the age and facial expressions. In this paper, we present a novel method of 3D ear acquisition system by using triangulation imaging principle, and the experiment results show that this design is efficient and can be used for ear recognition.

  15. Ethanol sclerotherapy of peripheral venous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Rimon, U. E-mail: rimonu@sheba.health.gov.il; Garniek, A.; Galili, Y.; Golan, G.; Bensaid, P.; Morag, B

    2004-12-01

    Background: venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. Objectives: to report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. Patients and methods: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. Results: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. Conclusion: sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur.

  16. Ethanol sclerotherapy of peripheral venous malformations

    International Nuclear Information System (INIS)

    Rimon, U.; Garniek, A.; Galili, Y.; Golan, G.; Bensaid, P.; Morag, B.

    2004-01-01

    Background: venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. Objectives: to report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. Patients and methods: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. Results: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. Conclusion: sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur

  17. Subdural hematoma from a cavernous malformation.

    Science.gov (United States)

    Schmitt, Anne J; Mitha, Alim P; Germain, Rasha; Eschbacher, Jennifer; Spetzler, Robert F

    2014-01-01

    To present a case of a cavernous malformation presenting with a subdural hematoma. A 27-year-old woman was admitted with progressively worsening headache, vomiting, weakness, and word-finding difficulties 1 week after she was discharged from an outside hospital, where she was managed conservatively for a presumed traumatic subdural hematoma. Computed tomography revealed an enlarging subacute left hemispheric subdural hematoma for which she underwent drill craniostomy. Postprocedural magnetic resonance imaging showed a posterior left temporal lobe mass consistent with a cavernous malformation juxtaposed with the subdural hematoma. Craniotomy for resection of the lesion was performed. She had an uncomplicated postoperative course and experienced a good recovery. The signs and symptoms, diagnostic imaging, and intraoperative findings suggest that the subdural hematoma was caused by extralesional hemorrhage of the cavernous malformation, which is a rare finding associated with these malformations. The clinical course, radiologic, and intraoperative findings suggest that the subdural hemorrhage was caused by extralesional hemorrhage of the cavernous malformation. Copyright © 2014 Elsevier Inc. All rights reserved.

  18. Prevalence and pattern of congenital malformations among neonates in the neonatal unit of a teaching hospital

    International Nuclear Information System (INIS)

    Hussain, S.; Sabir, M.; Tarar, S. H.; Mushtaq, R.; Asghar, I.; Chattha, M. N.

    2014-01-01

    Objective: To determine the prevalence and pattern of congenital malformations among neonates in a teaching hospital. Methods: The prospective hospital-based study was conducted over a period of 18 months in the neonatal unit of Combined Military Hospital, Kharian, from September 2011 to February 2013. All neonates from newborn to 28 days of age admitted to the unit irrespective of their condition comprised the study population. Neonatal examination was done by the Registrar at the time of admission followed by neonatologist/paediatrician. Information regarding gender, weight, gestational age, mode of delivery, consanguinity, maternal age, antenatal visit record and family history were recorded on a predesigned proforma. After clinical examination, if required, relevant investigations like ultrasonography, radiology, echocardiography, laboratory and genetic studies were done to confirm diagnosis. Data was statistically analysed by using SPSS 20. Results: Out of 3,210 total admissions, 226 (7%) neonates were congenitally malformed. Of them, 130 (57.52 %) were male and 96 (42.47 %) females. Among different body systems affected, anomalies related to the central nervous system were 46(20.35%) musculoskeletal 42(18.58%), genitourinary 34 (15.04%), cardiovascular system 30 (13.27%), ear, eye, face, neck 27(11.94%), digestive system 19 (8.40%), syndromes and skin 14 (6.19%) each. Conclusion: Congenital Malformations are not rare in our community and central nervous system is the most commonly affected system. Healthcare managers must stress upon primary prevention in the form of vaccination, nutrition and drugs to decrease preventable share of congenital malformations. (author)

  19. Imaging of head and neck venous malformations

    International Nuclear Information System (INIS)

    Flis, Christine M.; Connor, Stephen E.

    2005-01-01

    Venous malformations (VMs) are non proliferative lesions that consist of dysplastic venous channels. The aim of imaging is to characterise the lesion and define its anatomic extent. We will describe the plain film, ultrasound (US) (including colour and duplex Doppler), computed tomography (CT), magnetic resonance imaging (MRI), conventional angiographic and direct phlebographic appearances of venous malformations. They will be illustrated at a number of head and neck locations, including orbit, oral cavity, superficial and deep facial space, supraglottic and intramuscular. An understanding of the classification of such vascular anomalies is required to define the correct therapeutic procedure to employ. Image-guided sclerotherapy alone or in combination with surgery is now the first line treatment option in many cases of head and neck venous malformations, so the radiologist is now an integral part of the multidisciplinary management team. (orig.)

  20. Interventional treatment of pulmonary arteriovenous malformations

    DEFF Research Database (Denmark)

    Andersen, Poul Erik; Kjeldsen, Anette Drøhse

    2010-01-01

    Pulmonary arteriovenous malformations (PAVM) are congenital vascular communications in the lungs. They act as right to left shunts so that the blood running through these malformations is not oxygenated or filtered. These patients are typically hypoxaemic with exercise intolerance and are at high...... risk of paradoxical emboli to the brain and other organs. These malformations are most commonly seen in hereditary haemorrhagic telangiectasia (HHT) (Mb. Osler-Weber-Rendu syndrome). Nowadays, the generally accepted treatment strategy of first choice is embolization of the afferent arteries...... the functional level. Embolization is a well-established method of treating PAVM, with a significant effect on oxygenation of the blood. Screening for PAVM in patients at risk is recommended, especially in patients with HHT....

  1. Neurodevelopmental malformations of the cerebellar vermis in genetically engineered rats

    Science.gov (United States)

    The cerebellar vermis is particularly vulnerable to neurodevelopmental malformations in humans and rodents. Sprague-Dawley, and Long-Evans rats exhibit spontaneous cerebellar malformations consisting of heterotopic neurons and glia in the molecular layer of the vermis. Malformati...

  2. Surgical excision of a cerebral arteriovenous malformation in a dog

    International Nuclear Information System (INIS)

    Thomas, W.B.; Schueler, R.O.; Kornegay, J.N.

    1995-01-01

    A 7-year-old dog was presented with seizures and epistaxis. Computed tomography showed a contrast-enhancing lesion in the cerebrum. An arteriovenous malformation was identified at surgery and resected. A discussion of intracranial arteriovenous malformation is presented

  3. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    Energy Technology Data Exchange (ETDEWEB)

    Cornelis, F., E-mail: francoiscornelis@hotmail.com [Institut Bergonie, Department of Radiology (France); Neuville, A. [Institut Bergonie, Department of Pathology (France); Labreze, C. [Pellegrin Hospital, Department of Pediatric Dermatology (France); Kind, M. [Institut Bergonie, Department of Radiology (France); Bui, B. [Institut Bergonie, Department of Oncology (France); Midy, D. [Pellegrin Hospital, Department of Vascular Surgery (France); Palussiere, J. [Institut Bergonie, Department of Radiology (France); Grenier, N. [Pellegrin Hospital, Department of Radiology (France)

    2013-06-15

    The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations.

  4. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    International Nuclear Information System (INIS)

    Cornelis, F.; Neuville, A.; Labrèze, C.; Kind, M.; Bui, B.; Midy, D.; Palussière, J.; Grenier, N.

    2013-01-01

    The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations.

  5. Recurrent meningitis due to pneumococci and non-typable Haemophilus influenzae in a child with a Mondini malformation.

    Science.gov (United States)

    Valmari, P; Palva, A

    1986-01-01

    An eight-year-old boy with a congenital inner ear malformation and recurrent otitis media had three episodes of bacteriologically confirmed meningitis within seven months. The first episode was caused by pneumococci, the other two by non-typable Haemophilus influenzae. All episodes were characterized by an abrupt onset. The CSF cultures were positive within 0.5 to 12 hours after the onset of symptoms. Despite misleading laboratory studies, surgical exploration revealed a CSF fistula associated to the inner ear anomaly. No further episodes occurred after the fistula was closed. Careful roentgenographic evaluation, including recently introduced special computed tomography (CT) methods, is indicated in recurrent meningitis. In addition, such evaluations should be considered even after the first episode, when special clinical features suggest a CSF fistula. Such features include an extremely rapid onset and detection of common non-invasive bacteria as causative agents, as illustrated by the present case.

  6. Objective Audiometry using Ear-EEG

    DEFF Research Database (Denmark)

    Christensen, Christian Bech; Kidmose, Preben

    Recently, a novel electroencephalographic (EEG) method called ear-EEG [1], that enable recording of auditory evoked potentials (AEPs) from a personalized earpiece was introduced. Initial investigations show that well established AEPs, such as ASSR and P1-N1-P2 complex can be observed from ear-EEG...

  7. Interconnections between the Ears in Nonmammalian Vertebrates

    DEFF Research Database (Denmark)

    Feng, Albert S.; Christensen-Dalsgaard, J.

    2010-01-01

    Many of the nonmammalian vertebrates (anurans, lizards, crocodiles, and some bird species) have large, continuous air spaces connecting the middle ears and acoustically coupling the eardrums. Acoustical coupling leads to strongly enhanced directionality of the ear at frequencies where diffraction...... cues are negligible in small-sized animals. The chapter reviews the peripheral basis of directionality in these animal groups....

  8. Coupled ears in lizards and crocodilians

    DEFF Research Database (Denmark)

    Carr, Catherine E; Christensen-Dalsgaard, Jakob; Bierman, Hilary

    2016-01-01

    Lizard ears are coupled across the pharynx, and are very directional. In consequence all auditory responses should be directional, without a requirement for computation of sound source location. Crocodilian ears are connected through sinuses, and thus less tightly coupled. Coupling may improve th...... range is reviewed in the light of current theories of sound localization....

  9. Perinatal outcome of obvious congenital malformation as seen at the ...

    African Journals Online (AJOL)

    Gastrointestinal, central nervous and musculo-skeletal system malformations were the commonest seen with individual incidences of 3.9, 3.5 and 2.1 per 1000 total births, respectively. Unclassified congenital malformations had the highest case fatality while central nervous system malformations constituted the commonest ...

  10. Microbiomes of the normal middle ear and ears with chronic otitis media.

    Science.gov (United States)

    Minami, Shujiro B; Mutai, Hideki; Suzuki, Tomoko; Horii, Arata; Oishi, Naoki; Wasano, Koichiro; Katsura, Motoyasu; Tanaka, Fujinobu; Takiguchi, Tetsuya; Fujii, Masato; Kaga, Kimitaka

    2017-10-01

    The aim of this study was to profile and compare the middle ear microbiomes of human subjects with and without chronic otitis media. Prospective multicenter cohort study. All consecutive patients undergoing tympanoplasty surgery for chronic otitis media or ear surgery for conditions other than otitis media were recruited. Sterile swab samples were collected from the middle ear mucosa during surgery. The variable region 4 of the 16S rRNA gene in each sample were amplified using region-specific primers adapted for the Illumina MiSeq sequencer (Illumina, CA, USA)). The sequences were subjected to local blast and classified using Metagenome@KIN (World Fusion, Tokyo, Japan). In total, 155 participants were recruited from seven medical centers. Of these, 88 and 67 had chronic otitis media and normal middle ears, respectively. The most abundant bacterial phyla on the mucosal surfaces of the normal middle ears were Proteobacteria, followed by Actinobacteria, Firmicutes, and Bacteroidetes. The children and adults with normal middle ears differed significantly in terms of middle ear microbiomes. Subjects with chronic otitis media without active inflammation (dry ear) had similar middle ear microbiomes as the normal middle ears group. Subjects with chronic otitis media with active inflammation (wet ear) had a lower prevalence of Proteobacteria and a higher prevalence of Firmicutes than the normal middle ears. The human middle ear is inhabited by more diverse microbial communities than was previously thought. Alteration of the middle ear microbiome may contribute to the pathogenesis of chronic otitis media with active inflammation. 2b. Laryngoscope, 127:E371-E377, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  11. Lymphatic malformations: a proposed management algorithm.

    LENUS (Irish Health Repository)

    Oosthuizen, J C

    2012-02-01

    OBJECTIVE: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors\\' experience in managing these lesions as well as current literature on the subject. STUDY DESIGN AND METHODS: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. DATA COLLECTED: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. RESULTS: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. CONCLUSION: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

  12. Cystic malformations of the neck in children

    Energy Technology Data Exchange (ETDEWEB)

    Koch, Bernadette L. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States)

    2005-05-01

    The most common cystic malformations of the neck are the result of abnormal embryogenesis involving the thyroglossal duct (TGD), lymphatic primordia and the branchial apparatus. When the basic embryology of these structures is considered, a reasonable differential diagnosis - and in some cases a definitive diagnosis - can be achieved based on the location and the imaging characteristics of the cystic mass. (orig.)

  13. Cystic malformations of the neck in children

    International Nuclear Information System (INIS)

    Koch, Bernadette L.

    2005-01-01

    The most common cystic malformations of the neck are the result of abnormal embryogenesis involving the thyroglossal duct (TGD), lymphatic primordia and the branchial apparatus. When the basic embryology of these structures is considered, a reasonable differential diagnosis - and in some cases a definitive diagnosis - can be achieved based on the location and the imaging characteristics of the cystic mass. (orig.)

  14. Fetal MRI clues to diagnose cloacal malformations

    Energy Technology Data Exchange (ETDEWEB)

    Calvo-Garcia, Maria A.; Kline-Fath, Beth M.; Patel, Manish N.; Kraus, Steven [Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States); Levitt, Marc A.; Pena, Alberto [Cincinnati Children' s Hospital Medical Center, Colorectal Center for Children, Pediatric Surgery, Cincinnati, OH (United States); Lim, Foong-Yen; Crombleholme, Timothy M. [Cincinnati Children' s Hospital Medical Center, Fetal Care Center of Cincinnati, Pediatric Surgery, Cincinnati, OH (United States); Linam, Leann E. [Arkansas Children' s Hospital, Department of Radiology, Little Rock, AR (United States)

    2011-09-15

    Prenatal US detection of cloacal malformations is challenging and rarely confirms this diagnosis. To define the prenatal MRI findings in cloacal malformations. We performed a retrospective study of patients with cloacal malformations who had pre- and post-natal assessment at our institution. Fetal MRI was obtained in six singleton pregnancies between 26 and 32 weeks of gestation. Imaging analysis was focused on the distal bowel, the urinary system and the genital tract and compared with postnatal clinical, radiological and surgical diagnoses. The distal bowel was dilated and did not extend below the bladder in five fetuses. They had a long common cloacal channel (3.5-6 cm) and a rectum located over the bladder base. Only one fetus with a posterior cloacal variant had a normal rectum. Three fetuses had increased T2 signal in the bowel and two increased T1/decreased T2 signal bladder content. All had renal anomalies, four had abnormal bladders and two had hydrocolpos. Assessment of the anorectal signal and pelvic anatomy during the third trimester helps to detect cloacal malformations in the fetus. The specificity for this diagnosis was highly increased when bowel fluid or bladder meconium content was identified. (orig.)

  15. MR findings of congenital anorectal malformation

    Energy Technology Data Exchange (ETDEWEB)

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

    1995-05-15

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

  16. CT imaging of cervical spinal vascular malformation

    International Nuclear Information System (INIS)

    Ueda, Takashi; Iwamoto, Munehisa; Miyamoto, Etsuo; Kuriyama, Tsuyoshi; Hayama, Tsuneto

    1982-01-01

    The patient had a history of the onset of motor paralysis of the right upper and lower extremities. Eight years later, numbness of the right upper extremity and a severe neck pain developed, and transverse paralysis of the lower extremities appeared in about 10 hours. CT demonstrated the presence of spinal vascular abnormality. Angiography suggested arteriovenous malformation of glomus type. (Chiba, N.)

  17. Vascular permeability in cerebral cavernous malformations

    DEFF Research Database (Denmark)

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao

    2015-01-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observ...

  18. The posterior urethra in anorectal malformations.

    Science.gov (United States)

    Mickelson, Jennifer J; MacNeily, Andrew E; Blair, Geoffrey K

    2007-03-01

    Anorectal malformations are commonly associated with recto-urethral fistulas. Definitive repair of these anomalies may result in injury to the bladder and urethra. Accurate preoperative assessment of the anatomy is imperative to identify the structures and avoid unnecessary injury. This brief report provides radiographic images that demonstrate these anomalies.

  19. Congenital lung malformations: correlation between prenatal and ...

    African Journals Online (AJOL)

    Aim: Congenital lung malformations are a common finding during prenatal ultrasonography (US). Investigations were completed by means of prenatal MRI and postnatal computed tomographic (CT) scan. The purpose of this study was to compare these prenatal findings with postnatal findings and pathological findings after ...

  20. MR findings of congenital anorectal malformation

    International Nuclear Information System (INIS)

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

    1995-01-01

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

  1. Underdiagnosis of Mild Congenital Anorectal Malformations

    NARCIS (Netherlands)

    Jonker, Jara E.; Trzpis, Monika; Broens, Paul M. A.

    Objective: To determine whether the frequency and severity of congenital anorectal malformations (CARMs) differs by sex. Study design: We included 129 patients (0-319 weeks old) diagnosed with CARMs, who had been referred to our Department of Pediatric Surgery between 2004 and 2013. Rectoperineal

  2. Congenital spinal malformations; Kongenitale spinale Malformationen

    Energy Technology Data Exchange (ETDEWEB)

    Ertl-Wagner, B.B.; Reiser, M.F. [Klinikum Grosshadern, Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

    2001-12-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [German] Kongenitale spinale Malformationen stellen eine komplexe Gruppe an Stoerungen dar, deren Genese sich am einfachsten aus der Embryologie heraus erklaeren laesst. Bei der klinisch-radiologischen Begutachtung ist zunaechst ihre korrekte Klassifikation im Rahmen der Erstdiagnose wichtig. Im weiteren Verlauf ist es jedoch zudem entscheidend, moegliche Komplikationen wie beispielsweise eine Hydromyelie oder ein Wiederanheften des Myelons nach Operation einer Spina bifida aperta zu erkennen. Zudem sollte bei der Diagnosestellung einer kongenitalen spinalen Malformation immer auch auf assoziierte Fehlbildungen, wie z.B. die Diastematomyelie oder das intraspinale Lipom bei der Spina bifida aperta, sowie auf eine moegliche syndromale Einordnung wie beispielsweise beim OEIS-oder VACTERL-Syndrom geachtet werden. (orig.)

  3. Congenital Malformations in River Buffalo (Bubalus bubalis

    Directory of Open Access Journals (Sweden)

    Sara Albarella

    2017-02-01

    Full Text Available The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital malformations most frequently reported in literature or signaled by breeders to the Department of Veterinary Medicine and Animal Production of the University Federico II (Naples, Italy in river buffalo are: musculoskeletal defects (transverse hemimelia, arthrogryposis, umbilical hernia and disorders of sexual development. In conclusion this review put in evidence that river buffalo have a great variety of malformations due to genetic causes, and TH and omphalocele are the most frequent and that several cases are still not reported, leading to an underestimation of the real weight of genetic diseases in this species.

  4. Congenital malformations in paediatric and neurosurgical practices ...

    African Journals Online (AJOL)

    Congenital malformations in paediatric and neurosurgical practices: problems and pattern (A preliminary report) ... Open Access DOWNLOAD FULL TEXT ... over a 5-year period (1998 to 2002) with congenital anomalies to the Paediatric Surgery and Neurosurgery units of the University Teaching Hospital, Ilorin, Nigeria.

  5. Challenges of congenital malformations: an African perspective ...

    African Journals Online (AJOL)

    Background: Congenital malformations are defects of morphogenesis of organs or body regions identifiable during the intrauterine life or after birth. The etiological factors proposed have varied in history based on prevailing understanding, culture, and religion. Worldwide historically, the role of the supernatural had been in ...

  6. Sex and congenital malformations: An international perspective

    NARCIS (Netherlands)

    Lisi, A.; Botto, L.D.; Rittler, M.; Castilla, E.; Botting, B.; De Walle, H.; Erickson, J.D.; Gatt, M.; De Vigan, C.; Irgens, L.; Johnson, W.; Lancaster, P.; Merlob, P.; Mutchinick, O.M.; Ritvanen, A.; Robert, 28033; Scarano, G.; Stoll, C.; Mastroiacovo, P.

    2005-01-01

    The study evaluated the sex distribution of major isolated malformations and common trisomies among a large and geographically varied sample. Eighteen registries from 24 countries contributed cases, which were centrally reviewed and classified in three clinical types as isolated, associated, or

  7. Sex and congenital malformations : An international perspective

    NARCIS (Netherlands)

    Lisi, A; Botto, LD; Rittler, M; Castilla, E; Bianchi, F; Botting, B; De Walle, H; Erickson, JD; Gatt, M; De Vigan, C; Irgens, L; Johnson, W; Lancaster, P; Merlob, P; Mutchinick, OM; Ritvanen, A; Robert, E; Scarano, G; Stoll, C; Mastroiacovo, P

    2005-01-01

    The study evaluated the sex distribution of major isolated malformations and common trisomies among a large and geographically varied sample. Eighteen registries from 24 countries contributed cases, which were centrally reviewed and classified in three clinical types as isolated, associated, or

  8. Lamb congenital lymphatic malformation - a case report

    Directory of Open Access Journals (Sweden)

    Neria Santos

    2014-01-01

    Full Text Available Lymphatic malformations have been rarely reported in literature either in humans or in animals. However, in recent years, concern about these malformations in humans has increased. A five-month-old Rasa Aragonesa male lamb was received at the Ovine Clinical Service of the Veterinary Faculty of Zaragoza, Spain, with a history of cervical protuberances coming from birth. The lamb showed three round swollen lumps (5–12 cm in diameter parallel to the trachea on the left side of the neck. Clinical examination, haematology, ultrasonography, fluid examination and histopathology were performed. No abnormalities were found in blood samples and ultrasound confirmed a multicystic lesion with internal separations. Histological evaluation of the tissue revealed dilated lymphatic vessels and channels in the dermis and hypodermis; some lymphatic vessels were filled with amorphous proteinaceous material and occasional lymphocytes and macrophages. These protuberances were diagnosed as congenital lymphatic malformations. Most of the gross and microscopical lesions were very similar to those described in humans. To the authors’ knowledge, this is the first time that a congenital lymphatic malformation is described in sheep.

  9. CT imaging of cervical spinal vascular malformation

    Energy Technology Data Exchange (ETDEWEB)

    Ueda, Takashi; Iwamoto, Munehisa; Miyamoto, Etsuo; Kuriyama, Tsuyoshi; Hayama, Tsuneto [Wakayama Red Cross Hospital, Wakayama (Japan)

    1982-05-01

    The patient had a history of the onset of motor paralysis of the right upper and lower extremities. Eight years later, numbness of the right upper extremity and a severe neck pain developed, and transverse paralysis of the lower extremities appeared in about 10 hours. CT demonstrated the presence of spinal vascular abnormality. Angiography suggested arteriovenous malformation of glomus type.

  10. Congenital Malformations in River Buffalo (Bubalus bubalis)

    Science.gov (United States)

    Albarella, Sara; Ciotola, Francesca; D’Anza, Emanuele; Coletta, Angelo; Zicarelli, Luigi; Peretti, Vincenzo

    2017-01-01

    Simple Summary Congenital malformations (due to genetic causes) represent a hidden danger for animal production, above all when genetic selection is undertaken for production improvements. These malformations are responsible for economic losses either because they reduce the productivity of the farm, or because their spread in the population would decrease the total productivity of that species/breed. River buffalo is a species of increasing interest all over the world for its production abilities, as proved by the buffalo genome project and the genetic selection plans that are currently performed in different countries. The aim of this review is to provide a general view of different models of congenital malformations in buffalo and their world distribution. This would be useful either for those who performed buffalo genetic selection or for researchers in genetic diseases, which would be an advantage to their studies with respect to the knowledge of gene mutations and interactions in this species. Abstract The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis) world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital

  11. Congenital vascular malformations in scintigraphic evaluation

    International Nuclear Information System (INIS)

    Pilecki, Stanisław; Gierach, Marcin; Gierach, Joanna; Świętaszczyk, Cyprian; Junik, Roman; Lasek, Władysław

    2014-01-01

    Congenital vascular malformations are tumour-like, non-neoplastic lesions caused by disorders of vascular tissue morphogenesis. They are characterised by a normal cell replacement cycle throughout all growth phases and do not undergo spontaneous involution. Here we present a scintigraphic image of familial congenital vascular malformations in two sisters. A 17-years-old young woman with a history of multiple hospitalisations for foci of vascular anomalies appearing progressively in the upper and lower right limbs, chest wall and spleen. A Parkes Weber syndrome was diagnosed based on the clinical picture. Due to the occurrence of new foci of malformations, a whole-body scintigraphic examination was performed. A 12-years-old girl reported a lump in the right lower limb present for approximately 2 years, which was clinically identified as a vascular lesion in the area of calcaneus and talus. Phleboscintigraphy visualized normal radiomarker outflow from the feet via the deep venous system, also observed in the superficial venous system once the tourniquets were released. In static and whole-body examinations vascular malformations were visualised in the area of the medial cuneiform, navicular and talus bones of the left foot, as well as in the projection of right calcaneus and above the right talocrural joint. People with undiagnosed disorders related to the presence of vascular malformations should undergo periodic follow-up to identify lesions that may be the cause of potentially serious complications and to assess the results of treatment. Presented scintigraphic methods may be used for both diagnosing and monitoring of disease progression

  12. A case of pancreatic AV malformation in an elderly man.

    Science.gov (United States)

    Gupta, Vipin; Kedia, Saurabh; Sonika, Ujjwal; Madhusudhan, Kumble Seetharama; Pal, Sujoy; Garg, Pramod

    2018-06-01

    A 60-year-old man presented with recurrent abdominal pain and weight loss for 6 months. Abdominal imaging showed a large vascular lesion in the head and neck of pancreas suggestive of arteriovenous malformation (AV malformation). Endoscopic ultrasound was done which showed features of AV malformation with no evidence of pancreatic malignancy. Surgery was planned for definitive treatment of malformation. Digital subtraction angiography with angioembolization was done prior to surgery to reduce vascularity of the lesion. He recovered after a pylorus preserving pancreaticoduodenectomy. Histopathology of the resected specimen confirmed the pancreatic AV malformation. There has been no recurrence at 2 years of follow-up.

  13. Acceleration induced water removal from ear canals.

    Science.gov (United States)

    Kang, Hosung; Averett, Katelee; Jung, Sunghwan

    2017-11-01

    Children and adults commonly experience having water trapped in the ear canals after swimming. To remove the water, individuals will shake their head sideways. Since a child's ear canal has a smaller diameter, it requires more acceleration of the head to remove the trapped water. In this study, we theoretically and experimentally investigated the acceleration required to break the surface meniscus of the water in artificial ear canals and hydrophobic-coated glass tubes. In experiments, ear canal models were 3D-printed from a CT-scanned human head. Also, glass tubes were coated with silane to match the hydrophobicity in ear canals. Then, using a linear stage, we measured the acceleration values required to forcefully eject the water from the artificial ear canals and glass tubes. A theoretical model was developed to predict the critical acceleration at a given tube diameter and water volume by using a modified Rayleigh-Taylor instability. Furthermore, this research can shed light on the potential of long-term brain injury and damage by shaking the head to push the water out of the ear canal. This research was supported by National Science Foundation Grant CBET-1604424.

  14. Prognostic factors of congenital diaphragmatic hernia accompanied by cardiovascular malformation.

    Science.gov (United States)

    Takahashi, Shigehiro; Sago, Haruhiko; Kanamori, Yutaka; Hayakawa, Masahiro; Okuyama, Hiroomi; Inamura, Noboru; Fujino, Yuji; Usui, Noriaki; Taguchi, Tomoaki

    2013-08-01

    Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified. This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity. Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95%CI 1.96-30.27; adjusted OR 7.93, 95%CI 1.76-35.79, respectively) than with severity of congenital diaphragmatic hernia. The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

  15. Imaging of the postoperative middle ear

    Energy Technology Data Exchange (ETDEWEB)

    Williams, Marc T. [Department of Medical Imaging, Fondation Ophtalmologique Adolphe de Rothschild, 25 rue Manin, 75940, Paris (France); Ayache, Denis [Department of Otorhinolaryngology, Fondation Ophtalmologique Adolphe de Rothschild, Paris (France)

    2004-03-01

    The aim of this article is twofold: (a) to present the principles and the indications of surgical treatment of middle ear pathologies; and (b) to review the imaging findings after middle ear surgery, including the normal postoperative aspects and imaging findings in patients presenting with unsatisfactory surgical results or with suspicion of postoperative complications. This review is intentionally restricted to the most common diseases involving the middle ear: chronic otitis media and otosclerosis. In these specific fields of interest, CT and MR imaging play a very important role in the postoperative follow-up and in the work-up of surgical failures and complications. (orig.)

  16. EARS: Electronic Access to Reference Service.

    Science.gov (United States)

    Weise, F O; Borgendale, M

    1986-10-01

    Electronic Access to Reference Service (EARS) is a front end to the Health Sciences Library's electronic mail system, with links to the online public catalog. EARS, which became operational in September 1984, is accessed by users at remote sites with either a terminal or microcomputer. It is menu-driven, allowing users to request: a computerized literature search, reference information, a photocopy of a journal article, or a book. This paper traces the history of EARS and discusses its use, its impact on library staff and services, and factors that influence the diffusion of new technology.

  17. Malformations and abnormalities of the petrous portion of the temporal bone; Fehlbildungen und Missbildungen des Felsenbeins

    Energy Technology Data Exchange (ETDEWEB)

    Reith, W.; Yilmaz, U. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany); Heumueller, I. [Westpfalzklinikum Kaiserslautern, Institut fuer Radiologie, Kaiserslautern (Germany)

    2014-04-15

    High-resolution computed tomography (HRCT) is the procedure of choice in the diagnostics of abnormalities of the middle and inner ear. It allows a detailed presentation of anatomical features and achieves the prerequisites for selection of the various therapeutic options. The highly diverse abnormalities can be described using detailed imaging analyses. Malformations with an abnormally developed modiolus are assumed to be early embryological defects, such as the classical Mondini dysplasia. The essential therapeutic option for middle ear deformities is still a cochlear implant. The domain of magnetic resonance imaging (MRI) is not only in the analysis of the cochlear nerve and for exclusion of fibrosis or ossification of the labyrinth but is also able to visualize details of isolated malformations, such as an extended vestibular aqueduct or subtle alterations to the vestibule or can visualize them better in comparison to CT. Radiological diagnostics are used not only for classification but also to recognize typical clinical problem situations and play a key role in the diagnostics of hearing disorders and selection of the optimal therapeutic procedure. (orig.) [German] Die hochaufloesende CT ist das Verfahren der Wahl in der Diagnostik von Fehlbildungen des Mittel- und Innenohrs. Sie erlaubt die detaillierte Darstellung anatomischer Details und schafft die Voraussetzung fuer die unterschiedlichen Therapieoptionen. Durch detaillierte bildgebenden Analysen koennen die unterschiedlichsten Fehlbildungen beschrieben werden. Fehlbildungen mit einem nicht normal ausgebildeten Modiolus werden als fruehere embryologische Schaedigungen als die klassische Mondini-Dysplasie angesehen. Die wesentliche therapeutische Option bei Mittelohrfehlbildung bleibt das Kochleaimplantat. Die Domaene der MRT liegt nicht nur in der Analyse des N. cochlearis und im Ausschluss von Fibrosierungen oder Ossifikationen des Labyrinths, sondern darueber hinaus ist sie in der Lage, Details einzelner

  18. RASA1 analysis guides management in a family with capillary malformation-arteriovenous malformation.

    Science.gov (United States)

    Flore, Leigh Anne; Leon, Eyby; Maher, Tom A; Milunsky, Jeff M

    2012-06-01

    Capillary malformation-arteriovenous malformation (CM-AVM; MIM 60354) is an autosomal dominant disorder characterized by multifocal cutaneous capillary malformations, often in association with fast-flow vascular lesions, which may be cutaneous, subcutaneous, intramuscular, intraosseus, or cerebral arteriovenous malformations or arteriovenous fistulas. CM-AVM results from heterozygous mutations in the RASA1 gene. Capillary malformations of the skin are common, and clinical examination alone may not be able to definitively diagnose-or exclude- CM-AVM. We report a family in which the proband was initially referred for a genetic evaluation in the neonatal period because of the presence of a cardiac murmur and minor dysmorphic features. Both he and his mother were noted to have multiple capillary malformations on the face, head, and extremities. Echocardiography revealed dilated head and neck vessels and magnetic resonance imaging and angiography of the brain revealed a large infratentorial arteriovenous fistula, for which he has had two embolization procedures. RASA1 sequence analysis revealed a heterozygous mutation, confirming his diagnosis of CM-AVM. We established targeted mutation analysis for the proband's mother and sister, the latter of whom is a healthy 3-year-old whose only cutaneous finding is a facial capillary malformation. This revealed that the proband's mother is also heterozygous for the RASA1 mutation, but his sister is negative. Consequently, his mother will undergo magnetic resonance imaging and angiography screening for intracranial and spinal fast-flow lesions, while his sister will require no imaging or serial evaluations. Targeted mutation analysis has been offered to additional maternal family members. This case illustrates the benefit of molecular testing in diagnosis and making screening recommendations for families with CM-AVM.

  19. Structural Metadata Research in the Ears Program

    National Research Council Canada - National Science Library

    Liu, Yang; Shriberg, Elizabeth; Stolcke, Andreas; Peskin, Barbara; Ang, Jeremy; Hillard, Dustin; Ostendorf, Mari; Tomalin, Marcus; Woodland, Phil; Harper, Mary

    2005-01-01

    Both human and automatic processing of speech require recognition of more than just words. In this paper we provide a brief overview of research on structural metadata extraction in the DARPA EARS rich transcription program...

  20. Mozart ear: diagnosis, treatment, and literature review.

    Science.gov (United States)

    Yamashita, Ken; Yotsuyanagi, Takatoshi; Saito, Tamotsu; Isogai, Noritaka; Mori, Hiromasa; Itani, Yoshihito

    2011-11-01

    Mozart ear is a congenital auricular deformity, which is mainly characterized by a bulging appearance of the anterosuperior portion of the auricle, a convexly protruded cavum conchae, and a slit-like narrowing of the orifice of the external auditory meatus. It is said to be uncommon, and because no one has yet fully described neither the disease nor the treatment, the concept of Mozart ear has not been unified. This report describes a case of a 13-year-old girl presented with an unusual congenital deformity which showed the features of Mozart ear. It is an extremely rare deformity that only about 4 clinical cases have been reported in medical literature thereby a treatment method has not been fully discussed. For surgical correction of our cases, we excised deformed conchal cartilage, turned it over, regrafted, and maintained a cosmetically positive result. We also reviewed and described the origin, current concept, and treatment method of Mozart ear.

  1. Environment for Auditory Research Facility (EAR)

    Data.gov (United States)

    Federal Laboratory Consortium — EAR is an auditory perception and communication research center enabling state-of-the-art simulation of various indoor and outdoor acoustic environments. The heart...

  2. Profound microcephaly, primordial dwarfism with developmental brain malformations: a new syndrome.

    Science.gov (United States)

    Abdel-Salam, Ghada M H; Abdel-Hamid, Mohamed S; Saleem, Sahar N; Ahmed, Mahmoud K H; Issa, Mahmoud; Effat, Laila K; Kayed, Hisham F; Zaki, Maha S; Gaber, Khaled R

    2012-08-01

    We describe two sibs with a lethal form of profound congenital microcephaly, intrauterine and postnatal growth retardation, subtle skeletal changes, and poorly developed brain. The sibs had striking absent cranial vault with sloping of the forehead, large beaked nose, relatively large ears, and mandibular micro-retrognathia. Brain magnetic resonance imaging (MRI) revealed extremely simplified gyral pattern, large interhemispheric cyst and agenesis of corpus callosum, abnormally shaped hippocampus, and proportionately affected cerebellum and brainstem. In addition, fundus examination showed foveal hypoplasia with optic nerve atrophy. No abnormalities of the internal organs were found. This profound form of microcephaly was identified at 17 weeks gestation by ultrasound and fetal brain MRI helped in characterizing the developmental brain malformations in the second sib. Molecular analysis excluded mutations in potentially related genes such as RNU4ATAC, SLC25A19, and ASPM. These clinical and imaging findings are unlike that of any recognized severe forms of microcephaly which is believed to be a new microcephalic primordial dwarfism (MPD) with developmental brain malformations with most probably autosomal recessive inheritance based on consanguinity and similarly affected male and female sibs. Copyright © 2012 Wiley Periodicals, Inc.

  3. Verrucous carcinoma of the middle ear.

    Science.gov (United States)

    Woodson, G E; Jurco, S; Alford, B R; McGavran, M H

    1981-01-01

    A case of a highly destructive, cytologically nondysplastic squamous epithelial lesion of the middle ear is presented. The cranial nerve involvement and bone destruction are more extensive than has been seen in cholesteatoma. Cultures are negative for Pseudomonas, and the patient does not have the reported diathesis for malignant otitis externa. The gross and microscopic features are those of verrucous carcinoma. To our knowledge, the middle ear has not been previously reported as a site of involvement by verrucous carcinoma.

  4. Osteoma of the middle ear: case report

    International Nuclear Information System (INIS)

    Ryu, Ji Hwa

    2005-01-01

    Osteomas of the middle ear are exceedingly rare benign neoplasms. To date, only 21 cases have been reported in the literature. They arise from the promontory, the pyramidal process and the ossicles, and they are usually asymptomatic or cause some conductive hearing loss. We report here the CT and pathologic findings in a 38-year-old woman with a benign osteoma of the middle ear along with chronic otitis media

  5. Commissioning of n_TOF EAR2

    CERN Multimedia

    The construction of the second beam line and experiment area (EAR2) of the n_TOF facility is currently ongoing and scheduled to be completed by July 2014. An extensive series of measurements is planned in order to determine the beam characteristics like the neutron flux, the spatial beam profile and the resolution function, as well as the response of several detectors considered for use in future measurements at EAR2. A rigorous study of backgrounds will be undertaken in various conditions.

  6. Osteoma of the middle ear: case report

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, Ji Hwa [College of Medicine, Inje University, Dongrae Paik Hospital, Busan (Korea, Republic of)

    2005-07-15

    Osteomas of the middle ear are exceedingly rare benign neoplasms. To date, only 21 cases have been reported in the literature. They arise from the promontory, the pyramidal process and the ossicles, and they are usually asymptomatic or cause some conductive hearing loss. We report here the CT and pathologic findings in a 38-year-old woman with a benign osteoma of the middle ear along with chronic otitis media.

  7. Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele

    Energy Technology Data Exchange (ETDEWEB)

    Weon, Y.C.; Roh, H.G.; Byun, H.S. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology, Seoul (Korea); Chung, J.I. [Medimoa Hospital, Department of Radiology, Seoul (Korea); Eoh, W. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea)

    2005-10-01

    Combined spinal arteriovenous malformation and lipomyelomeningocele are extremely rare. We present a rare combined case of a lipomyelomeningocele with an intramedullary arteriovenous malformation (AVM) occurred at the L3-L4 level in a 30-year-old man who suffered from low back pain radiating to the lower extremities, dysuria, and frequency for 5 years. The MR studies showed an intradural mass with high-signal intensity on both T1-weighted and T2-weighted images, intermingled with multiple signal-void structures. The mass extended extradurally toward a subcutaneously forming fatty mass on the patient's back. Spinal angiography showed an AVM supplied by the radiculopial branches of the lumbar arteries and drained by tortuous, dilated, perimedullary veins. Endovascular embolization and surgical resection were performed. (orig.)

  8. Arteriovenous Malformation Detected by Small Bowel Endoscopy

    Directory of Open Access Journals (Sweden)

    Takaaki Fujii

    2014-10-01

    Full Text Available Gastrointestinal bleeding that originates in the small intestine is often difficult to diagnose. When successful diagnosis reveals a lesion that can be localized preoperatively, the laparoscopic approach is an appropriate and beneficial treatment modality for small bowel resection. A 69-year-old man presented with a 6-month history of gastrointestinal bleeding and symptomatic transfusion-dependent anemia. Upper and lower endoscopy were normal. Double-balloon endoscopy established the source of the bleeding as a 0.5-cm polypoid mass appearing as a submucosal tumor with redness and pulsation in the lower ileum, suggesting a vascular lesion. Laparoscopic small bowel resection was successful in removing the mass in the ileum. Histological evaluation of the mass revealed an arteriovenous malformation. Preoperative small bowel endoscopy can be useful for diagnosing the cause and localization of arteriovenous malformation in the small intestine.

  9. Bronchopulmonary foregut malformations: embryology, radiology and quandary

    International Nuclear Information System (INIS)

    Barnes, N.A.; Pilling, D.W.

    2003-01-01

    Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM. (orig.)

  10. Chiari Type II malformation: Prenatal sonographic findings

    Directory of Open Access Journals (Sweden)

    Sadhanandham Shrinuvasan

    2015-01-01

    Full Text Available Chiari malformations (CM are a group of defects associated with the congenital caudal displacement of the cerebellum and brainstem. A thorough understanding of the sonographic findings is necessary for the diagnosis of CM in the developing fetus. Here, we present the classical imaging findings of CM Type II detected in a 25-year-old primigravida at 26 weeks of gestation by routine sonographic screening.

  11. UTERINE ARTERIOVENOUS MALFORMATION: A CASE REPORT

    OpenAIRE

    Chandrashekar Murthy; Kiran

    2014-01-01

    Uterine arteriovenous malformation (AVM) is a rare condition, with less than 100 cases reported in the literature. Despite it being rare, it is a potentially life threatening disease. This case report describes 31- year-old women who presented with abnormal uterine bleeding. Trans abdominal sonography, colour and spectral Doppler imaging was performed, diagnosis was confirmed by non- invasive MRI scan. Laparoscopic bilateral uterine artery ligation was done successfully.

  12. NAD Deficiency, Congenital Malformations, and Niacin Supplementation.

    Science.gov (United States)

    Shi, Hongjun; Enriquez, Annabelle; Rapadas, Melissa; Martin, Ella M M A; Wang, Roni; Moreau, Julie; Lim, Chai K; Szot, Justin O; Ip, Eddie; Hughes, James N; Sugimoto, Kotaro; Humphreys, David T; McInerney-Leo, Aideen M; Leo, Paul J; Maghzal, Ghassan J; Halliday, Jake; Smith, Janine; Colley, Alison; Mark, Paul R; Collins, Felicity; Sillence, David O; Winlaw, David S; Ho, Joshua W K; Guillemin, Gilles J; Brown, Matthew A; Kikuchi, Kazu; Thomas, Paul Q; Stocker, Roland; Giannoulatou, Eleni; Chapman, Gavin; Duncan, Emma L; Sparrow, Duncan B; Dunwoodie, Sally L

    2017-08-10

    Congenital malformations can be manifested as combinations of phenotypes that co-occur more often than expected by chance. In many such cases, it has proved difficult to identify a genetic cause. We sought the genetic cause of cardiac, vertebral, and renal defects, among others, in unrelated patients. We used genomic sequencing to identify potentially pathogenic gene variants in families in which a person had multiple congenital malformations. We tested the function of the variant by using assays of in vitro enzyme activity and by quantifying metabolites in patient plasma. We engineered mouse models with similar variants using the CRISPR (clustered regularly interspaced short palindromic repeats)-Cas9 system. Variants were identified in two genes that encode enzymes of the kynurenine pathway, 3-hydroxyanthranilic acid 3,4-dioxygenase (HAAO) and kynureninase (KYNU). Three patients carried homozygous variants predicting loss-of-function changes in the HAAO or KYNU proteins (HAAO p.D162*, HAAO p.W186*, or KYNU p.V57Efs*21). Another patient carried heterozygous KYNU variants (p.Y156* and p.F349Kfs*4). The mutant enzymes had greatly reduced activity in vitro. Nicotinamide adenine dinucleotide (NAD) is synthesized de novo from tryptophan through the kynurenine pathway. The patients had reduced levels of circulating NAD. Defects similar to those in the patients developed in the embryos of Haao-null or Kynu-null mice owing to NAD deficiency. In null mice, the prevention of NAD deficiency during gestation averted defects. Disruption of NAD synthesis caused a deficiency of NAD and congenital malformations in humans and mice. Niacin supplementation during gestation prevented the malformations in mice. (Funded by the National Health and Medical Research Council of Australia and others.).

  13. Congenital cystic adenomatoid lung malformation of newborn

    International Nuclear Information System (INIS)

    Reither, M.; Peltner, H.U.; Weigel, W.; Braune, M.; Heiming, E.

    1980-01-01

    The congenital cystic adenomatoid malformation (CCAM) of the newborn is a particular form among the cystic disorders of the lung. The clinical findings, illustrated by four cases, and especially the roentgenographic symptoms are typical. Different radiologic examinations, including the computertomography, are discussed. The differential diagnosis of the disease is various, and therefore a correct and on time diagnosis is necessary, because the prognosis of the patient depends on an adequate therapy. (orig.) [de

  14. Cerebral arteriovenous malformation in Noonan's syndrome.

    OpenAIRE

    Schon, F.; Bowler, J.; Baraitser, M.

    1992-01-01

    Noonan's syndrome involves the association of multiple congenital abnormalities including neck webbing, pectus excavatum, facial anomalies with a variety of cardiac defects. In this paper the association of Noonan's syndrome with a large cerebral arteriovenous malformation is reported. Congenital cerebrovascular abnormalities are not a recognized feature of the syndrome. The paper also reviews previous reports of neurological associations with Noonan's syndrome, the commonest being mild intel...

  15. Radiological features of childhood giant cavernous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Ozgen, Burce; Senocak, Efsun; Oguz, Kader K. [Hacettepe University, Department of Radiology, Faculty of Medicine, Ankara (Turkey); Soylemezoglu, Figen [Hacettepe University, Department of Pathology, School of Medicine, Ankara (Turkey); Akalan, Nejat [Hacettepe University, Department of Neurosurgery, School of Medicine, Ankara (Turkey)

    2011-04-15

    Giant cavernous malformations (GCM) are very large, low-flow vascular malformations, which usually have atypical imaging features and are commonly misdiagnosed preoperatively as neoplasms or vascular malformations. These lesions have mostly been reported in children. As cavernomas show different features in children compared to adults, we evaluated the imaging features of pediatric GCMs in order to help in the preoperative diagnosis of these malformations. Brain MR studies of nine children (mean age of 4 years; 8 months-9 years) with biopsy-proven GCM were retrospectively evaluated. We defined GCMs as cavernomas of {>=}4 cm. Lesions were evaluated regarding their size, location, signal characteristics, general appearance (uni/multilocular) as well as regarding the presence of mass effect, edema, and fluid-fluid levels and were classified according to the Mottolese classification of pediatric cavernomas. Lesion locations were parietal (n = 5), frontal (n = 2), temporal, and intraventricular. Seven lesions were in the periventricular region (with five in the periatrial region). Six patients had T1 hyperintense multilobulated lesions with ''bubbles of blood'' appearance and three patients had heterogeneous lesions with reticular core. All lesions had mass effect, edema (marked in four cases), and peripheral hemosiderin rim. Fluid-fluid levels were also common (n = 7). Most of our lesions (six of nine) were classified as type IIIA, two as type IIIC, and one as type IA. In children, a GCM should be considered in case of very large hemorrhagic intra-axial mass with ''bubbles of blood'' multicystic appearance, surrounding hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect, especially in the periatrial location. (orig.)

  16. Complex arteriovenous malformation - a case report

    International Nuclear Information System (INIS)

    Sirakov, S.; Penkov, M.; Marinov, M.; Kamenov, B.

    2014-01-01

    AVMs are composed of a network of channels interposed between feeding arteries and draining veins, without any direct shunt. Two different anatomic types of nidus may be more or less differentiated: The most frequent clinical presentations of brain AVMs are hemorrhage, seizure, chronic headache, and focal deficits not related to hemorrhage. We show a case of 27 years old female came to the hospital in heavy condition with subarachnoid hemorrhage Fisher 4. She had a history of 4 surgical operation of brain AVM in the last 10 years, without significant reduction of the malformation. After discussion of multidisciplinary team, of neuroradiologist and neurosurgeon was decided to be perform endovascular embolization, because of the better outcome for the patient. We performed endovascular treatment of the AVM with achieving subtotal embolization of malformation (90%). The patient recovery completely after 22 days and went home. For the next 18 months follow up there is no data of accidents for the patient. Key words: Arteriovenous Malformation. Subarachnoid hemorrhage. Endovascular treatment. Embolization

  17. Neuroradiological evaluation of dorsal cyst malformations

    International Nuclear Information System (INIS)

    Utsunomiya, Hidetsuna; Hayashi, Takashi; Hashimoto, Takeo; Matsuishi, Toyojiro; Okudera, Toshio.

    1988-01-01

    We discussed six cases with dorsal cyst malformations listing their neuroradiological observations and proposed to differentiate between the holosphere and hemisphere as defined by Yokota (1984). The cases were divided into holospheric and hemispheric groups depending on the continuity of their frontal lobe midlines. Cases 1, 2 and 3 were placed in the holospheric group because of their unseparated frontal lobe sbeneath the partially formed anterior interhemispheric fissures. Cases 4, 5 and 6 were grouped in the hemisphere due to the completion of the interhemispheric fissures. There has been a tendency in recent years for most cases of cerebral malformations having an endogenous dorsal cyst with monoventricular configuration to be diagnosed as holoprosencephaly. However, we believe that only patients who have a dorsal cyst in the holospheric brain should be included, and the others in the hemispheric brain, which is capable of completing hemispheric cleavage, should not. Therefore, we emphasize the importance of correctly identifying the holospheric state in the dorsal cyst malformations for diagnosing holoprosencephaly. (author)

  18. Congenital Malformations Associated with Maternal Diabetes

    Directory of Open Access Journals (Sweden)

    Chih-Ping Chen

    2005-03-01

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

  19. Congenital cystic lung malformations; Konnatale zystische Lungenfehlbildungen

    Energy Technology Data Exchange (ETDEWEB)

    Stoever, B.; Scheer, I.; Bassir, C. [Klinik fuer Strahlenheilkunde, Berlin (Germany). Abt. Paediatrische Radiologie, Charite; Mau, H. [Campus Virchow-Klinikum, Klinik fuer Kinderchirurgie, Berlin (Germany); Chaoui, R. [Campus Mitte, Klinik fuer Geburtsmedizin, Berlin (Germany); Henrich, W. [Campus Virchow-Klinikum, Klinik fuer Geburtsmedizin, Berlin (Germany); Schwabe, M. [Campus Mitte, Inst. fuer Pathologie, Berlin (Germany); Wauer, R. [Campus Mitte, Klinik fuer Neonatologie, Berlin (Germany)

    2006-04-15

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  20. CT findings in Arnold-Chiari malformation

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Y.; Nakamura, S. (Aichi Prefecture Colony, Kasugai (Japan). Central Hospital); Yamada, H.; Kageyama, N.

    1981-12-01

    CT scans of Arnold-Chiari malformations demonstrate many abnormal findings that are considered to be specific for this type of malformation. Fifty patients with Arnold-Chiari malformations were studied, and their CT findings were analyzed. This number included 14 preshunted neonatal cases and 36 post-shunted infantile cases. Craniolacunia was recognized only in the neonatal cases, but other skull changes, including scaphocephaly and petrous scalloping, were found more frequently in the infantile cases. The posterior fossa abnormality was composed of several specific changes, such as a non-visualized fourth ventricle, a lateral or upward growth of the cerebellum, or a beaking deformity of the midbrain. These findings were far more common in the infantile cases. However, in 7 neonatal cases on which CT cisternography was performed, these posterior fossa changes were well recognized in the majority of cases. Hydrocephalus was found in all cases. In half of the post-shunted infantile cases, the lateral ventricles were markedly collapsed. Characteristic features of the lateral ventricles, such as a protruding of the caudate nuclei, a pointing of the frontal and/or occipital horns, an absent septum pellucidum, or dominently dilated occipital horns, were commonly found in both neonatal and infantile cases. The subarachnoid space was remarkably widened at the retrothalamic cistern and/or the interhemispheric fissure in many cases.

  1. Abortion, premature delivery, stillborn, and malformations

    International Nuclear Information System (INIS)

    Sato, Yukio

    1992-01-01

    Since A-bomb disaster in Hiroshima and Nagasaki, genetic effects of A-bomb radiation have been investigated in the offspring of A-bomb survivors. This paper outlines the results of the previous studies in the context of the historical backgrounds. An earlier survey using a cohort of 71,280 children of Hiroshima and Nagasaki A-bomb survivors and a suitable control population of non-exposed 55,870 persons have dealt with the stillborn, neonate death, 9-month-old infant death, malformations at birth and 9 months after birth, and sex ratio in F 1 offspring; it was found that there was no significant difference in these items between the exposed and non-exposed groups. The other survey using fetal and neonatal autopsy cases has revealed that the incidence of malformations was significantly higher in children born to A-bomb survivors than those of the control population (18.5% vs 11.0%); however, there was no evidence of genetic abnormalities specific to the group of A-bomb survivors. Until now, no definitive conclusions of the sex ratio at birth have been drawn. Regarding height in F 1 offspring, no significant difference existed between the exposed and non-exposed groups. Nor was there significant difference in malformations in F 1 and F 2 offspring between the group of A-bomb survivors and the suitable control group. (N.K.)

  2. Developmental malformations of the cerebral cortex

    International Nuclear Information System (INIS)

    Reiss-Zimmermann, Martin; Weber, D.; Sorge, I.; Hirsch, W.; Merkenschlager, A.

    2010-01-01

    Migration disorders (MD) are increasingly recognized as an important cause of epilepsy and developmental delay. Up to 25% of children with refractory epilepsy have a cortical malformation. MD encompass a wide spectrum with underlying genetic etiologies and clinical manifestations. Research regarding the delineation of the genetic and molecular basis of these disorders has provided greater insight into the pathogenesis of not only the malformation but also the process involved in normal cortical development. Diagnosis of MD is important since patients who fail three antiepileptic medications are less likely to have their seizures controlled with additional trials of medications and therefore epilepsy surgery should be considered. Recent improvements in neuroimaging have resulted in a significant increase in the recognition of MD. Findings can be subdivided in disorders due to abnormal neurogenesis, neuronal migration, neuronal migration arrest and neuronal organization resulting in different malformations like microcephaly, lissencephaly, schizencephaly and heterotopia. The examination protocol should include T1-w and T2-w sequences in adequate slice orientation. T1-w turbo-inversion recovery sequences (TIR) can be helpful to diagnose heterotopia. Contrast agent is needed only to exclude other differential diagnoses. (orig.)

  3. Developmental biology and the study of malformations.

    Science.gov (United States)

    Hughes, A F

    1976-05-01

    Experimental work on abnormal conditions of incubation in the chick has been undertaken to acquire a scientific approach to malformations. More precise experiments on causing abnormalities had a common origin with experimental embryology. Progress in experimental teratology during the last 50 years is reviewed in a commentary on the 4 principles formulated by Stockard in 1921. The results of cytogenetical studies in man and in other organisms have led to the tracing of some relationships between them. Present knowledge concerning malformations of the neural tube, originating either experimentally, spontaneously, or phenotypically, has been presented and the teratological implications of some recent theories on the expression of the genotype are discussed in particular reference to problems of hormones as teratogens, the implication of carbohydrate metabolism, and teratogenesis. It is speculated that teratogenesis is possibly related to cationic balance in early development and that 1 factor retarding progress in the understanding of malformations is the tendency toward the development of teratology in an adequately close relationship with other branches of cell biology.

  4. Serous otitis media (S.O.M.). A bacteriological study of the ear canal and the middle ear

    NARCIS (Netherlands)

    Cabenda, S. I.; Peerbooms, P. G.; van Asselt, G. J.; Feenstra, L.; van der Baan, S.

    1988-01-01

    A bacteriological study of the middle-ear effusions and the ear canals in children with chronic serous otitis media (S.O.M.) was performed. Sixty-eight children (127 ears) were investigated. From this study it appeared that cleansing of the ear canal with 0.5% chlorhexidine in 70% ethanol for 30 s

  5. Ear-to-Ear On-Body Channel Fading in the ISM-band for Tangentially-Polarized Antennas

    DEFF Research Database (Denmark)

    Kvist, Søren Helstrup; Thaysen, Jesper; Jakobsen, Kaj Bjarne

    2011-01-01

    The ear-to-ear on-body channel fading has been studied in the ISM-band. The ear-to-ear path gain was measured on six persons in an indoor environment for a duration of 200 s. The channel fading has been characterized in terms of empirical cumulative distribution functions (CDF), average fade...

  6. Statistical Shape Analysis of the Human Ear Canal with Application to In-the-Ear Hearing Aid Design

    DEFF Research Database (Denmark)

    Paulsen, Rasmus Reinhold

    2004-01-01

    This thesis is about the statistical shape analysis of the human ear canal with application to the mechanical design of in-the-ear hearing aids. Initially, it is described how a statistical shape model of the human ear canal is built based on a training set of laser-scanned ear impressions. A thin...

  7. MRI findings of intracranial cavernous malformations

    International Nuclear Information System (INIS)

    Han, Byoung Hee; Kim, Dong Ik; Cho, Yong Kuk

    1995-01-01

    To analyze the variable MRI features and clinical significance of intracranial cavernous malformations. Forty patients(mean age 35.4) with cavernous malformation were evaluated by MRI. Eleven patients were surgically confirmed. Cavernous malformations were divided into four categories on the basis of the MR imaging characteristics, especially on T2-weighted image. Type I lesion was defined as an extralesional subacute hemorrhage outside the low signal rim, type II as an intralesional hemorrhage surrounded by low signal rim, type III lesion as an intralesional thrombosis with variegated central core surrounded by low signal rim, and type IV lesion as a focal old hemorrhagic core with small low signal intensity. Type IV was further divided into IVa and IVb, whether the lesion has small iso-or hypersignal central core (IVa) or not (IVb). Follow-up MRI was evaluated in 12 patients who were managed conservatively. Follow-up intervals ranged from 2 weeks to 29 months (mean 6 months). Total 80 lesions were detected in 40 patients. Multiple lesions were noted in 10 patients. The topography of the cavernous malformations was supratentorial in 75% and infratentorial in 23%. There were 10 lesions in type I, 15 in type II, 21 in type III, 14 in type IVa, and 20 in type IVb. Type I lesions mainly showed mass effect and edema. Type III lesions showed minimal contrast enhancements in 7 lesions on delayed images. Type II lesions showed the characteristics of both type I and type III lesions. On follow up images, decrease in size in 5, change of type in 7, rebleeding in 2 and no change in 12 lesions were demonstrated. Hemorrhage, edema and mass effect were combined in the cases of rebleeding. On follow-up study, the estimated risk of bleeding was 32.3%/person-year and 13.7%/lesion-year. Cavernous malformations show as variable appearance, on MR imaging suggesting variable stages of evolution. The MR morphologic classification and evaluation of secondary findings are helpful to

  8. Fever in pregnancy and the risk of congenital malformations

    DEFF Research Database (Denmark)

    Sass, L.; Urhoj, S. K.; Kjærgaard, J.

    2017-01-01

    fetal malformations or death. Fever during pregnancy, especially during embryogenesis, has also been associated with congenital malformations in human offspring. The purpose of this large cohort study of clinically recognized pregnancies was to investigate whether fever during first trimester...... was associated with an increased risk of congenital malformations in the offspring. Methods: The Danish National Birth Cohort is a population-based cohort of 100,418 pregnant women and their offspring recruited in 1996 to 2002. Information on fever during pregnancy was collected prospectively by means of two....... Congenital malformations within the first three and a half years of life were categorized according to EUROCAT's classification criteria. Logistic regression models were used to estimate the associations between fever in first trimester and overall congenital malformations and congenital malformations...

  9. Maternal smoking in pregnancy and risk for congenital malformations

    DEFF Research Database (Denmark)

    Leite, Mimmi; Albieri, Vanna; Kjaer, Susanne K.

    2014-01-01

    and registered in the Danish Medical Birth Register containing detailed information on smoking during pregnancy and congenital malformations. METHODS: Associations [odds ratios (OR) with 95% CI] between maternal smoking and risk for various groups of congenital malformations, investigated using the generalized.......e. when two or more malformations are diagnosed in a child) (odds ratio 1.06, 95% confidence interval 1.01-1.10) and various main groups of congenital malformations including the cardiovascular system (odds ratio 1.13, 95% confidence interval 1.07-1.19), the respiratory system (odds ratio 1.25, 95......% confidence interval 1.11-1.41), the digestive system (odds ratio 1.15, 95% confidence interval 1.07-1.24) and oral clefts (odds ratio 1.29, 95% confidence interval 1.14-1.46), as well as for some specific congenital malformations including cardiac septal defects, malformations of the pulmonary and tricuspid...

  10. Body lift, drag and power are relatively higher in large-eared than in small-eared bat species

    DEFF Research Database (Denmark)

    Håkansson, Jonas; Jakobsen, Lasse; Hedenström, Anders

    2017-01-01

    Bats navigate the dark using echolocation. Echolocation is enhanced by external ears, but external ears increase the projected frontal area and reduce the streamlining of the animal. External ears are thus expected to compromise flight efficiency, but research suggests that very large ears may mi....... The result of this trade-off would be the eco-morphological correlation in bat flight, with large-eared bats generally adopting slow-flight feeding strategies....

  11. Structure and function of the mammalian middle ear. I: Large middle ears in small desert mammals.

    Science.gov (United States)

    Mason, Matthew J

    2016-02-01

    Many species of small desert mammals are known to have expanded auditory bullae. The ears of gerbils and heteromyids have been well described, but much less is known about the middle ear anatomy of other desert mammals. In this study, the middle ears of three gerbils (Meriones, Desmodillus and Gerbillurus), two jerboas (Jaculus) and two sengis (elephant-shrews: Macroscelides and Elephantulus) were examined and compared, using micro-computed tomography and light microscopy. Middle ear cavity expansion has occurred in members of all three groups, apparently in association with an essentially 'freely mobile' ossicular morphology and the development of bony tubes for the middle ear arteries. Cavity expansion can occur in different ways, resulting in different subcavity patterns even between different species of gerbils. Having enlarged middle ear cavities aids low-frequency audition, and several adaptive advantages of low-frequency hearing to small desert mammals have been proposed. However, while Macroscelides was found here to have middle ear cavities so large that together they exceed brain volume, the bullae of Elephantulus are considerably smaller. Why middle ear cavities are enlarged in some desert species but not others remains unclear, but it may relate to microhabitat. © 2015 Anatomical Society.

  12. Cranial CT signs of the Chiari II malformation

    International Nuclear Information System (INIS)

    Naidich, T.P.; Pudlowski, R.M.

    1980-01-01

    Serial CT scans of 32 patients with proved Chiari II malformations and 19 patients with hydrocephalus and meningomyelocele (presumed to have Chiari II malformation) were reviewed and compared with CT scans from 30 patients with non-chiari aqueductal stenosis to develop criteria for identifying the Chiari II malformation and for differentiating it from other forms of hydrocephalus. Correlation with post-mortem specimens of Chiari II brains provided a pathologic basis for the CT signs observed. (orig.) [de

  13. Clinico-roentgenological atlas of congenital malformations in human limbs

    International Nuclear Information System (INIS)

    Luzina, E.V.; Shakirov, Eh.A.

    1990-01-01

    The objective of the present atlas is to familiarize a wide range of physicians with localizations of congenital malformations in human limbs which are little studied clinically and roentgenologically. The atlas illustrates different variants of malformations of upper and lower limbs systematized by nosological principle; multiple and some, rarely occuring system deformations of the skeleton. Malformation features are described and their names are presented in compliance with the international classification taking into account the vocabulary of medical terms. 102 refs.; 121 figs

  14. Fever in pregnancy and the risk of congenital malformations

    DEFF Research Database (Denmark)

    Sass, L; Urhoj, S K; Kjærgaard, J

    2017-01-01

    Background: In a variety of animal species, hyperthermia in pregnancy has been recognized as teratogenic. Hyperthermia interferes with protein synthesis via heat-shock proteins, which can entail membrane disruption, cell death, vascular disruption, and placental infarction. This can induce severe....... Congenital malformations within the first three and a half years of life were categorized according to EUROCAT's classification criteria. Logistic regression models were used to estimate the associations between fever in first trimester and overall congenital malformations and congenital malformations...

  15. Anaesthetic management of a child with massive extracranial arteriovenous malformation

    Directory of Open Access Journals (Sweden)

    Faisal Shamim

    2012-01-01

    Full Text Available Vascular tumors affect the head and neck commonly but arteriovenous malformations are rare. Vascular malformations are often present at birth and grow with the patient, usually only becoming significant later in childhood. Embolization has been the mainstay of treatment in massive and complex arteriovenous malformations. We present a case of massive extracranial arteriovenous malformation in a 7-year-old boy causing significant workload on right heart and respiratory distress. The management of angioembolization under general anaesthesia and anaesthetic concerns are presented.

  16. Endometriosis and uterine malformations: infertility may increase severity of endometriosis.

    Science.gov (United States)

    Boujenah, Jeremy; Salakos, Eleonora; Pinto, Mélodie; Shore, Joanna; Sifer, Christophe; Poncelet, Christophe; Bricou, Alexandre

    2017-06-01

    The aim of our study was to compare the stage and severity of endometriosis in fertile and infertile women with congenital uterine malformations. We performed an observational study from September 2007 to December 2015 in a tertiary care university hospital and assisted reproductive technology center. A total of 52 patients with surgically proven uterine malformations were included. We compared 41 infertile patients with uterine malformations with 11 fertile patients with uterine malformation. The main outcome was the stage, score and type of endometriosis in regard to infertility and class of uterine malformation. The rate of endometriosis did not differ between the two groups (43.9 vs. 36.4%). The mean revised American Fertility Society score was higher in infertile patients with uterine malformations (19.02 vs. 6, p endometriosis (43.9 vs. 37.5%). Endometrioma and deep infiltrating endometriosis were associated with uterine malformations in infertile women, respectively 14.6 and 0%. No difference in the characteristics of endometriosis was found regarding the class of malformation. The association of uterine malformations and infertility may increase the severity of endometriosis and raise the issue of their diagnosis and management. © 2016 Nordic Federation of Societies of Obstetrics and Gynecology.

  17. Associated malformations among infants with anophthalmia and microphthalmia.

    Science.gov (United States)

    Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

    2012-03-01

    Infants with anophthalmia and microphthalmia frequently have other associated congenital anomalies. The reported frequency and types of associated malformations vary among different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with anophthalmia and microphthalmia in a geographically well defined population from 1979 to 2004 of 346,831 consecutive births. Of the 87 infants with anophthalmia and microphthalmia born during this period (prevalence at birth, 2.5 per 10,000), 90% had associated malformations. Infants with associated malformation were divided into recognizable conditions (22 infants [25%] with chromosomal and 15 infants [17%] with nonchromosomal conditions), and nonrecognizable conditions (41 infants [47%] with multiple malformations). Trisomies 13 and 18 were the most frequent chromosomal abnormalities. Amniotic bands sequence, CHARGE syndrome, Meckel-Gruber syndrome, and VACTERL association were most often present in recognizable nonchromosomal conditions. Malformations in the musculoskeletal, cardiovascular, and central nervous systems were the most common other anomalies in infants with multiple malformations and nonrecognizable conditions. The frequency of associated malformations in infants with anophthalmia or microphthalmia emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations-especially musculoskeletal, cardiac, and central nervous system anomalies-may need to be considered in infants with anophthalmia or microphthalmia, and referral of these infants for genetics evaluation and counseling seems warranted. Copyright © 2012 Wiley Periodicals, Inc.

  18. MR imaging of anorectal malformations and associated anomalies

    International Nuclear Information System (INIS)

    Nievelstein, R.A.J.; Valk, J.; Vos, A.

    1998-01-01

    Congenital anorectal malformations are found in many forms, and are frequently associated with other anomalies, especially of the spinal cord, spine, and urogenital system. Decisions concerning initial management of children with anorectal malformations can be made only after accurate determination of (a) the level and type of malformation, (b) the type of fistula, (c) the developmental state of the sphincter muscle complex, and (d) the presence of associated anomalies. Magnetic resonance imaging has proven to be the only modality to answer all these crucial questions, and has contributed to a better insight in the morphology and pathogenesis of such complex congenital malformations. (orig.)

  19. Genetics Home Reference: microcephaly-capillary malformation syndrome

    Science.gov (United States)

    ... and Stroke: Epilepsy Information Page National Institute of Neurological Disorders and Stroke: Microcephaly Educational Resources (7 links) Boston Children's Hospital: Capillary Malformation Boston Children's Hospital: Microcephaly Centers ...

  20. Study of placenta of children born with congenital malformations.

    Science.gov (United States)

    Stoll, Claude; Alembik, Yves; Dott, Béatrice; Roth, Marie-Paule

    2003-01-01

    The malformations in this study were observed in a series of 279,642 consecutive births of known outcome registered in our Registry of congenital anomalies. For each case, more than 50 factors included in the registration forms were studied. One of the factors studied was the placenta. For each malformed child, a control was chosen. Cases with maternal known factors impairing placenta function, i.e. vasculopathy and diabetes, were excluded. In each category of malformations studied, the malformed children were divided into isolated and non-isolated (multiple malformed) cases. The weight of placenta of isolated cases was not lower than the weight of placenta of the controls. In contrast, the weight of placenta of the cases with non-isolated malformations was lower than the weight of placenta of the controls and of the isolated cases, for all categories of malformations but gastroschisis and omphalocele. The mean weights at birth of the cases with multiple malformations were also lower than those of the controls. The human placenta discounts a principal functional part, the maternal blood in the intervillous space. Congenital malformations may interact with this function.

  1. CT of temporal bone - IV. inner ear

    International Nuclear Information System (INIS)

    Kwon, Jae Yoon; Sung, Kyu Bo; Youn, Eun Kyoung; Park, Youn Kyeung; Lee, Young Uk

    1990-01-01

    Temporal bone CT was done in 697 patients from April 1985 to October 1989. The abnormal findings were seen in 453 patients, which were chronic otitis media in 355 patients, fracture in 49 patients and congenital anomaly in 44 patients, etc. The abnormal findings of inner ear were observed on 46 patients. The results were summarized as follows : 1. The incidence of inner ear involvement by chronic otitis media was 7.3% (26/355 : labyrinthine fistula in 17 patients, labyrinthitis ossificans in 9 patients). Labyrinthine fistula was most commonly located on lateral semicircular canal (15/17, 88.2%). 2. Fusion of vestibule with lateral semicircular canal and formation of common cavity was demonstrated incidentally in 5 patients (0.7% of total number of temporal bone CT), and bilateral in 3 patients. 3. The incidence of inner ear anomaly in congenital ear anomaly was 11.4% (5/44). All cases were bilateral and three patients showed associated middle ear anomaly. 4. The incidence of involvement of bony labyrinth in temporal bone fracture was 10.2% (5/49). Labyrinthine fracture was seen all patients of transverse(3) and mixed fracture(1). In longitudinal fracture, labyrinthine fracture was seen in 2.2% (1/45). 5. Others were traumatic labyrinthitis ossificans(1), intracanalicular acoustic neuroma(3) and facial nerve neuroma(1)

  2. Evolution and development of the vertebrate ear

    Science.gov (United States)

    Fritzsch, B.; Beisel, K. W.

    2001-01-01

    This review outlines major aspects of development and evolution of the ear, specifically addressing issues of cell fate commitment and the emerging molecular governance of these decisions. Available data support the notion of homology of subsets of mechanosensors across phyla (proprioreceptive mechanosensory neurons in insects, hair cells in vertebrates). It is argued that this conservation is primarily related to the specific transducing environment needed to achieve mechanosensation. Achieving this requires highly conserved transcription factors that regulate the expression of the relevant structural genes for mechanosensory transduction. While conserved at the level of some cell fate assignment genes (atonal and its mammalian homologue), the ear has also radically reorganized its development by implementing genes used for cell fate assignment in other parts of the developing nervous systems (e.g., neurogenin 1) and by evolving novel sets of genes specifically associated with the novel formation of sensory neurons that contact hair cells (neurotrophins and their receptors). Numerous genes have been identified that regulate morphogenesis, but there is only one common feature that emerges at the moment: the ear appears to have co-opted genes from a large variety of other parts of the developing body (forebrain, limbs, kidneys) and establishes, in combination with existing transcription factors, an environment in which those genes govern novel, ear-related morphogenetic aspects. The ear thus represents a unique mix of highly conserved developmental elements combined with co-opted and newly evolved developmental elements.

  3. Ear Acupuncture in European Traditional Medicine

    Directory of Open Access Journals (Sweden)

    Luigi Gori

    2007-01-01

    Full Text Available Auricular acupuncture is a diagnostic and treatment system based on normalizing the body's dysfunction through stimulation of definite points on the ear. Rudimentary forms of acupuncture which probably arose during the Stone Age have survived in many parts of the world right down to present day. It was used in the ancient Egypt, Rome, Greece and all the Mediterranean area. It is a microacupuncture technique similar to reflexology, and was first described in France in 1950 by Paul Nogier who is considered the Father of modern ear acupuncture. It was speculated that the technique works because groups of pluripotent cells contain information from the whole organism and create regional organization centers representing different parts of the body. Nevertheless stimulation of a reflex point in the ear seems relieve symptoms of distant pathologies. Modern research is confirming the efficacy of ear acupuncture for analgesia and anxiety related disease, while tobacco dependence and other substance abuse still need confirmation. Actually main methodological problems with auricular acupuncture are that exist too many maps with little agreement regarding point location in the ear, and that the correspondence or reflex systems does not correlated with modern knowledge of anatomy and physiology.

  4. Laser treatment of oral vascular malformations

    Science.gov (United States)

    Romeo, U.; Gaimari, G.; Mohsen, M.; Tenore, G.; Palaia, G.

    2014-01-01

    Oral Vascular Malformations (OVM) are congenital anomalies characterized by morph-structural and/or functional changes of nature in severity and extension. OVM can affect any type of vessels arterial, venous or lymphatic and any capillary or anatomical. They are divided into two categories: low and high flow. In this study were treated 40 patients with OVM with a range size from 2 mm to 44 mm; they were subjected to clinical examination supported by Colour-Doppler Ultrasound instrumental examination and only for doubt cases the Magnetic Resonance Imaging (MRI) was prescribed. Only low flow venous and capillary malformations were treated by GaAlAs laser (Wiser®, Lambda, Brindole,Italy, 980nm) and KTP laser (SmartLite®, DEKA, Florence, Italy, 532nm) with two different techniques: the Transmucosal Thermophotocoagulation (TMT) and the Intralesional Photocoagulation (ILP). These techniques permitted a good control of haemostasis, avoiding bleeding both during surgery and in the postoperative. It is obtained an excellent and good healing respectively in 10% and 60% of cases, a moderate and poor resolution respectively in 22.5% and 7.5% of cases. A clear diagnosis allowed the management of Venous malformations (VM) by laser devices with wavelengths highly absorbed in haemoglobin in safety and efficacy and according to the principles of minimal invasive surgery. The aim of this study was to verify if the laser is effective in the treatment of OVM for the purpose of the clinical findings and the postoperative course. The Authors concluded that the laser can be considered the "gold standard" for treating OVM.

  5. A Newborn with Icthyosis, Corpus Callosum Hypoplasia, Microcephaly, Atrichia and Intra Uterine Growth Retardation (IUGR: AVariant of Icthyosis Follicularis Atrichia Photophobia (IFAP or Brain Anomalies, Retardation, Ectodermal Dysplasia, Skeletal Deformities, Hirschsprung Disease, Ear/Eye Anomalies, Cleft Palate, Cryptorchidism (BRESHECK?

    Directory of Open Access Journals (Sweden)

    Gurudutt S. Joshi

    2017-10-01

    Full Text Available A full term newborn small for gestational age Intra Uterine Growth Retardation (IUGR admitted with congenital dysmorphic features with icthyosis, atrichia, microcephaly and eye abnormalities, when explored further for other congenital malformations, revealed Corpus callosum hypoplasia and closely related features with two rare syndromes Icthyosis Follicularis Atrichia Photophobia (IFAP or Brain Anomalies Retardation, Ectodermal Dysplasia, Skeletal Deformities, Hirschsprung Disease, Hemivertebrae, Ear/Eye Anomalies, and Kidney Dysplasia (BRESHECK.

  6. Are two ears not better than one?

    Science.gov (United States)

    McArdle, Rachel A; Killion, Mead; Mennite, Monica A; Chisolm, Theresa H

    2012-03-01

    The decision to fit one or two hearing aids in individuals with binaural hearing loss has been debated for years. Although some 78% of U.S. hearing aid fittings are binaural (Kochkin , 2010), Walden and Walden (2005) presented data showing that 82% (23 of 28 patients) of their sample obtained significantly better speech recognition in noise scores when wearing one hearing aid as opposed to two. To conduct two new experiments to fuel the monaural/binaural debate. The first experiment was a replication of Walden and Walden (2005), whereas the second experiment examined the use of binaural cues to improve speech recognition in noise. A repeated measures experimental design. Twenty veterans (aged 59-85 yr), with mild to moderately severe binaurally symmetrical hearing loss who wore binaural hearing aids were recruited from the Audiology Department at the Bay Pines VA Healthcare System. Experiment 1 followed the procedures of the Walden and Walden study, where signal-to-noise ratio (SNR) loss was measured using the Quick Speech-in-Noise (QuickSIN) test on participants who were aided with their current hearing aids. Signal and noise were presented in the sound booth at 0° azimuth under five test conditions: (1) right ear aided, (2) left ear aided, (3) both ears aided, (4) right ear aided, left ear plugged, and (5) unaided. The opposite ear in (1) and (2) was left open. In Experiment 2, binaural Knowles Electronics Manikin for Acoustic Research (KEMAR) manikin recordings made in Lou Malnati's pizza restaurant during a busy period provided a typical real-world noise, while prerecorded target sentences were presented through a small loudspeaker located in front of the KEMAR manikin. Subjects listened to the resulting binaural recordings through insert earphones under the following four conditions: (1) binaural, (2) diotic, (3) monaural left, and (4) monaural right. Results of repeated measures ANOVAs demonstrated that the best speech recognition in noise performance was

  7. MRI measurement for inner ear structures

    International Nuclear Information System (INIS)

    Li Shuling; Liu Huaijun; Chi Chen; Qin Ruiping; Shi Zhaoxia

    2003-01-01

    Objective: To reconstruct the image of inner ear by using 3D-FASE heavily T 2 WI, and to establish MRI measurement criterion of inner ear structures. Methods: One hundred and six inner ears of 53 healthy volunteers underwent MRI heavily T2-weighted axial scanning by using 3D fast advanced spin echo sequence. All the original images were transferred to an online workstation. Analyze AVW software was used for image post-processing. All the structures of inner ear were reconstructed, rotated from various angles and measured by using maximum intensity projection (MIP). Results: (1) All the structures of inner ear and internal auditory channel (IAC) could be visualized clearly by using 3D-FASE heavily T 2 WI. (2) Using analysis of variance, there was no age, side or race-related difference in inner ear volume, but it was bigger in male than in female [(0.242 ± 0.0236) mm 3 (male) versus (0.226 ± 0.021) mm 3 (female)]. There was no age, side-related differences in three semicircular canal height and vestibule vertical diameter, but, again, they were bigger in male than in female. The height of upper, lateral and posterior semicircular canal were (5.511 ± 0.626) mm (male) versus (5.167 ± 0.357) mm (female); (3.763 ± 0.495) mm (male) versus (3.446 ± 0.405) mm (female); (5.227 ± 0.547) mm (male) versus (4.786 ± 0.500) mm (female). There was no age, sex or side-related differences in three semicircular canal diameter and cochlea. The diameter of upper, lateral and posterior semicircular canal were (1.06 ± 0.119) mm, (1.14 ± 0.181) mm, and (1.22 ± 0.196)mm; the external diameter of cochlea basal turn was (6.520 ± 0.475) mm, the diameter of cochlea basal turn was (1.413 ± 0.144) mm, and cochlea height was (4.100 ± 0.405) mm. Conclusion: (1) For the first time, the MRI measurement criterion of inner ear structures is established. (2) Vestibule and three semicircular canal of inner ear are bigger in male than in female

  8. Clinical and Genetic Studies in Inherited Cardiovascular Malformations

    NARCIS (Netherlands)

    I.M.B.H. van de Laar (Ingrid)

    2012-01-01

    textabstractCardiovascular malformations comprise a broad spectrum of anomalies of the heart and blood vessels, including congenital heart malformations (CHM) and aortic aneurysms, the two main topics of this thesis. These conditions lead to significant morbidity and mortality both in infancy and

  9. NPHP4 Variants Are Associated With Pleiotropic Heart Malformations

    NARCIS (Netherlands)

    French, Vanessa M.; van de Laar, Ingrid M. B. H.; Wessels, Marja W.; Rohe, Christan; Roos-Hesselink, Jolien W.; Wang, Guangliang; Frohn-Mulder, Ingrid M. E.; Severijnen, Lies-Anne; de Graaf, Bianca M.; Schot, Rachel; Breedveld, Guido; Mientjes, Edwin; van Tienhoven, Marianne; Jadot, Elodie; Jiang, Zhengxin; Verkerk, Annemieke; Swagemakers, Sigrid; Venselaar, Hanka; Rahimi, Zohreh; Najmabadi, Hossein; Meijers-Heijboer, Hanne; de Graaff, Esther; Helbing, Wim A.; Willemsen, Rob; Devriendt, Koen; Belmont, John W.; Oostra, Ben A.; Amack, Jeffrey D.; Bertoli-Avella, Aida M.

    2012-01-01

    Rationale: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. Objective: To identify genetic

  10. NPHP4 variants are associated with pleiotropic heart malformations.

    NARCIS (Netherlands)

    French, V.M.; Laar, I.M. van de; Wessels, M.W.; Rohe, C.; Roos-Hesselink, J.W.; Wang, G.; Frohn-Mulder, I.M.; Severijnen, L.A.; Graaf, B.M. de; Schot, R.; Breedveld, G.; Mientjes, E.; Tienhoven, M. van; Jadot, E.; Jiang, Z.; Verkerk, A.; Swagemakers, S.; Venselaar, H.; Rahimi, Z.; Najmabadi, H.; Meijers-Heijboer, H.; Graaff, E. de; Helbing, W.A.; Willemsen, R.; Devriendt, K.; Belmont, J.W.; Oostra, B.A.; Amack, J.D.; Bertoli-Avella, A.M.

    2012-01-01

    RATIONALE: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. OBJECTIVE: To identify genetic

  11. Epizootic of ovine congenital malformations associated with Schmallenberg virus infection

    NARCIS (Netherlands)

    Brom, van der R.; Luttikholt, S.J.; Lievaart-Peterson, K.; Peperkamp, N.H.M.T.; Mars, M.H.; Poel, van der W.H.M.; Vellema, P.

    2012-01-01

    Epizootic outbreaks of congenital malformations in sheep are rare and have, to the best of our knowledge, never been reported before in Europe. This paper describes relevant preliminary findings from the first epizootic outbreak of ovine congenital malformations in the Netherlands. Between 25

  12. Urogenital tract anomalies in children with congenital anorectal malformation

    NARCIS (Netherlands)

    J.W. Hoekstra

    1991-01-01

    textabstractThe term 'imperforate anus' covers a variety of congenital anorectal malformations ranging in severity from anal stenosis to cloacal exstrophy. The clinical picture of the anorectal malformation has been known for thousands of years, during which many attempts have been made to find

  13. Multiple congenital skeletal malformations in a lamb associated with ...

    African Journals Online (AJOL)

    Other malformations included patella absence, resulting in bowing of both fore and hind limbs with poorly developed muscles associated with these skeletal structure. Dystocia was believed to be a result of fetal monstrosity resulting in abnormal posture. The cause of the congenital malformations was not obvious ...

  14. Angiographic findings of congenital vascular malformation in soft tissue

    International Nuclear Information System (INIS)

    Choi, Dae Seob; Park, Jae Hyung; Han, Joon Koo; Chung, Jin Wook; Moon, Woo Kyung; Han, Man Chung

    1994-01-01

    We evaluated the clinical, plain radiographic, and angiographic findings of congenital vascular malformation of the soft tissue. Retrospective analysis was performed in 36 patients. Pathological diagnosis was done in 25 patients by surgery and the others were clinically and angiographically diagnosed. On the basis of angiographic findings, we classified the lesions to three groups as arteriovenous malformation (AVM), hemangioma, and venous malformation. In pathologically proven 25 cases, we compared the angiographic diagnosis with the pathologic diagnosis. By angiographic classification, AVM was 13 cases, hemangioma 16 cases, and venous malformation 7 cases. The locations of the lesions were upper extremities in 14 cases, lower extremities in 20 cases, both extremities in 1 case, and back in 1 case. Clinical findings were bruit and thrill in 13 cases(12 AVMs,1 hemangioma) and varicosities in 16 cases(11 AVMs, 3 hemangiomas and 2 venous malformations). The varicosities in AVM were pulsating nature, but not in hemangioma and venous malformation. The concordance rate of the angiographic and pathologic diagnosis was 100%(6/6) in AVM, 71%(10/14) in hemangioma and 60% (3/5) in venous malformation. We think that angiography is an essential study for accurate diagnosis and appropriate treatment of congenital vascular malformation

  15. Dandy-Walker malformation | Hamid | Egyptian Journal of Medical ...

    African Journals Online (AJOL)

    Dandy-Walker malformation is a rare congenital malformation and involves the cerebellum and fourth ventricle. The condition is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. A large number of concomitant problems may be ...

  16. Congenital malformation of the systemic heart of Sepia officinalis l ...

    African Journals Online (AJOL)

    Numerous preparations of the circulatory system of Sepia officinalis L. caught from the Bay of Arcachon (Atlantic Coast of France) in 1989 and 1996 showed an obvious congenital malformation of the systemic heart complex. The malformation consisted of a cord- or truncus-like structure at the left cranio-apical ventricle.

  17. Uterine arteriovenous malformation as a rare cause of genital bleeding

    International Nuclear Information System (INIS)

    Rodriguez, A.; Escartin, I.; Riazuelo, G.; Zaragozano, R.

    2002-01-01

    Uterine arteriovenous malformation is a rarely described entity, the presenting sign of which is usually genital bleeding. We report a case of this malformation in a woman of child-bearing age with a history of traumatic delivery and repeated subsequent curettage, describing the ultrasound, computed tomography and magnetic resonance findings, as well as the results of arteriography. (Author) 5 refs

  18. Valproic acid monotherapy in pregnancy and major congenital malformations

    DEFF Research Database (Denmark)

    Jentink, Janneke; Loane, Maria A; Dolk, Helen

    2010-01-01

    The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited.......The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited....

  19. First report of microcephaly-capillary malformations syndrome in ...

    African Journals Online (AJOL)

    Background: Microcephaly-capillary malformation (MIC-CAP) syndrome is a newly described autosomal recessive syndrome characterized by microcephaly, multiple cutaneous capillary malformations, intractable epilepsy and profound developmental delay. We present the first description of MIC-CAP syndrome in Russia.

  20. Imaging of cardiovascular malformations in Williams syndrome

    International Nuclear Information System (INIS)

    Li Shiguo; Zhao Shihua; Jiang Shiliang; Huang Lianjun; Xu Zhongying; Ling Jian; Zheng Hong; Yan Chaowu; Lu Jinguo

    2008-01-01

    Objective: To evaluate the imaging methods for cardiovascular malformations in Williams syndrome(WS). Methods: Thirteen cases of WS (7 males and 6 females) aged 10 months to 13 years were involved in this study. All patients underwent chest X-ray radiography, electrocardiography, echocardiography and physical examination. 3 cases underwent electronic beam computed tomography (EBCT), cardiac catheterization and angiography were performed in 8 cases. Results: Twelve patients were referred to our hospital for cardiac murmur and 1 case for cyanosis after birth. 7 patients were found with 'elfin-like' facial features, 6 patients with pulmonary arterial stenosis, 2 cases with patent ductus arteriosus, 2 cases with severe pulmonary hypertension and 1 case with total endocardial cushion defect. Sudden death occurred in 2 patients during and after catheterization, respectively. Conclusions: Conventional angiography is the golden standard for the diagnosis of cardiovascular malformations in WS. Noninvasive methods such as MSCT and MRI should be suggested because of the risk of sudden death in conventional angiography. (authors)

  1. Evolutionary hypothesis for Chiari type I malformation.

    Science.gov (United States)

    Fernandes, Yvens Barbosa; Ramina, Ricardo; Campos-Herrera, Cynthia Resende; Borges, Guilherme

    2013-10-01

    Chiari I malformation (CM-I) is classically defined as a cerebellar tonsillar herniation (≥5 mm) through the foramen magnum. A decreased posterior fossa volume, mainly due to basioccipital hypoplasia and sometimes platybasia, leads to posterior fossa overcrowding and consequently cerebellar herniation. Regardless of radiological findings, embryological genetic hypothesis or any other postulations, the real cause behind this malformation is yet not well-elucidated and remains largely unknown. The aim of this paper is to approach CM-I under a broader and new perspective, conjoining anthropology, genetics and neurosurgery, with special focus on the substantial changes that have occurred in the posterior cranial base through human evolution. Important evolutionary allometric changes occurred during brain expansion and genetics studies of human evolution demonstrated an unexpected high rate of gene flow interchange and possibly interbreeding during this process. Based upon this review we hypothesize that CM-I may be the result of an evolutionary anthropological imprint, caused by evolving species populations that eventually met each other and mingled in the last 1.7 million years. Copyright © 2013 Elsevier Ltd. All rights reserved.

  2. Sirenomelia and caudal malformations in two families.

    Science.gov (United States)

    Gerard, Marion; Layet, Valérie; Costa, Teresa; Roumazeilles, Yves; Chenal, Pierre; Cailliez, Daniel; Gerard, Bénédicte

    2012-07-01

    We report on two families with co-occurrence of sirenomelia and caudal malformations. In the first family, the mother had undergone surgery for a short form of imperforate anus. Her first pregnancy was terminated because of bilateral renal agenesis with oligohydramnios. Her second pregnancy was interrupted because of sirenomelia. The second family was referred to us because of caudal malformation in their two children. The parents' spinal radiographs were normal. The first pregnancy resulted in a girl with imperforate anus, absence of S3-S5 and coccyx, abnormal pelvic floor, and an almost bifid anteriorly located bladder. The second pregnancy resulted in a baby girl with sirenomelia. No diabetes was present during the pregnancies in either of these two families. These families confirm the hypothesis that major genes are responsible for the embryogenesis of the caudal part of the embryo, with variable expression, as has been already described in sirenomelia mouse models (CYP26A1, BMP7/tsg). Molecular studies are underway in these families and in sporadic cases in our laboratory to explore the genetic basis of sirenomelia in humans. Copyright © 2012 Wiley Periodicals, Inc.

  3. 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

  4. Three dimensional animated images of anorectal malformations

    International Nuclear Information System (INIS)

    Ueno, Shigeru; Yanagimachi, Noriharu; Muro, Isao; Komiya, Taizo; Yokoyama, Seishichi; Hirakawa, Hitoshi; Tajima, Tomoo; Mitomi, Toshio; Suto, Yasuzo.

    1996-01-01

    Accurate reconstruction of the pelvic structures is a most important factor in obtaining a desirable result after anorectoplasty for a patient with anorectal malformation. Preoperative evaluation of the anatomy is indispensable for choosing an appropriate operative method in each case. To facilitate preoperative evaluation, three dimensional animated images of the pelvic structure of patients with anorectal malformations were constructed by computer graphics based upon tomographic images obtained from magnetic resonance imaging. Axial 1-mm thick images of the pelvic portion were generated with spoiling pulse gradient echo sequences using short repetition times (13 msec TR) and short echo times (6 msec TE) with a flip angle of 25 degrees with the patient in the jack-knife position. Graphic data from MR images were transferred to a graphic work station and processed on it. The skin surface, the ano-rectum, the lower urinary tract and the sphincter musculature were segmented by thresholding images by the signal intensity. Three dimensional images were displayed by surface rendering method using the segmented data of each organ and then animation images of these organs were obtained. The anatomy of each type of anomaly was easily recognized by 3-D visualization, and animation of the pelvic viscera and the sphincter musculature made the images more realistic. Animated images of the musculature were especially useful for simulating surgical procedures and could be helpful for reviewing surgical results. (author)

  5. Morphologic-echocardiographic correlates of Ebstein's malformation.

    Science.gov (United States)

    Rusconi, P G; Zuberbuhler, J R; Anderson, R H; Rigby, M L

    1991-07-01

    The cross-sectional echocardiographic findings were analysed retrospectively in 26 patients with Ebstein's malformation in the light of studies of autopsied specimens from different patients showing this lesion. The salient anatomical feature in diagnosis is the finding of the hinge point of the septal and mural leaflets of the valve within the inlet component of the right ventricle rather than at the atrioventricular junction. The other important feature is the nature of the distal attachment of the leaflets, particularly the anterosuperior one, which can either be in focal or linear fashion. The hinge point of the septal leaflet was noted echocardiographically to be displaced in 19 patients but, significantly, the leaflet was absent in the other seven. Also significant was that the hinge point of the mural leaflet at the crux had been visualized in only 15 of the patients. The anterosuperior leaflet had a distal linear attachment in 20 of the patients, with the anteroseptal commissure becoming a keyhole in six of these through which blood passed to the functional right ventricle. The valve remained a competent structure, even though closing at the junction of atrialized and functional components of the right ventricle rather than at the atrioventricular junction. Cross-sectional echocardiography is the technique of choice with which to display the salient morphological features of Ebstein's malformation.

  6. Amplatzer vascular plugs in congenital cardiovascular malformations

    International Nuclear Information System (INIS)

    Barwad, Parag; Ramakrishnan, Sivasubramanian; Kothari, Shyam S; Saxena, Anita; Gupta, Saurabh K; Juneja, Rajnish; Gulati, Gurpreet Singh; Jagia, Priya; Sharma, Sanjiv

    2013-01-01

    Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 – 7F Judkin's right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow

  7. Application of three-dimensional fast spin-echo T2-weighted image in lesions of the inner ear

    International Nuclear Information System (INIS)

    Xian Junfang; Wang Zhenchang; Yan Fei; Niu Yantao; Zhu Ye; Wang Yan; Tian Qichang; Lan Baosen

    1999-01-01

    Objective: To investigate the advantage of three-dimensional fast spin-echo T 2 -weighted image (3D FSE T 2 WI) in depicting normal structures and lesions of the inner ear. Methods: 3D FSE T 2 WI and 2D FSE T 2 WI were performed in 10 healthy volunteers and 20 cases with inner ear diseases. Advantages and disadvantages of the two techniques were compared. CT was performed in 6 cases with enlarged endo-lymphatic sac and 1 cases of Mondini malformation. Results: 3D FSE T 2 WI enabled visualization of detailed anatomic structures. Enlarged endo-lymphatic sacs were clearly revealed in 9 cases on 16 sides by 3D FSE T 2 WI, while only a part but not the whole of the enlarged endo-lymphatic sac could be shown on 2D FSE T 2 WI. In 6 cases, 3D FSE T 2 WI displayed enlarged endo-lymphatic sac on 11 sides and normal on 1 side; however, CT revealed enlarged vestibular aqueduct on all 12 sides. One case with small acoustic neuroma (only 4 mm in diameter) was clearly demonstrated on 3D FSE T 2 WI but not well shown on 2D FSE T 2 WI. One case with cochlear Mondini malformation associated with dysplasia of vestibule and semicircular canals was displayed more clearly on 3D FSE T 2 WI than on 2D FSE T 2 WI. Conclusions: 3D FSE T 2 WI can clearly display normal structures and lesions of the inner ear

  8. Analysis of Lethality and Malformations During Zebrafish (Danio rerio) Development.

    Science.gov (United States)

    Raghunath, Azhwar; Perumal, Ekambaram

    2018-01-01

    The versatility offered by zebrafish (Danio rerio) makes it a powerful and an attractive vertebrate model in developmental toxicity and teratogenicity assays. Apart from the newly introduced chemicals as drugs, xenobiotics also induce abnormal developmental abnormalities and congenital malformations in living organisms. Over the recent decades, zebrafish embryo/larva has emerged as a potential tool to test teratogenicity potential of these chemicals. Zebrafish responds to compounds as mammals do as they share similarities in their development, metabolism, physiology, and signaling pathways with that of mammals. The methodology used by the different scientists varies enormously in the zebrafish embryotoxicity test. In this chapter, we present methods to assess lethality and malformations during zebrafish development. We propose two major malformations scoring systems: binomial and relative morphological scoring systems to assess the malformations in zebrafish embryos/larvae. Based on the scoring of the malformations, the test compound can be classified as a teratogen or a nonteratogen and its teratogenic potential is evaluated.

  9. Transcatheter arterial embolization for congenital renal arteriovenous malformation

    International Nuclear Information System (INIS)

    Zhou Jun; Hu Tingyang; Yuan Jianhua; Yu Wenqiang

    2008-01-01

    Objective: To evaluate the effectiveness of transcatheter arterial embolization for congenital renal arteriovenous malformation. Methods: Seven cases of congenital renal arteriovenous malformation causing gross hematuria were retrospectively studied. All of 7 cases were demonstrated by means of angiography and then the catheter was placed superselectively into the involved arterial end of the malformation undertaking embolization with gelfoam, dehydrated ethanol, coils, etc. Results: All the malformations of the 7 cases were successfully embolized with stoppage of gross hematuria within 24 hours. No serious complications occurred except lumbago, fever, gastrointestinal reaction for one week. There was no recurrence of haematuria and the renal function was also normal in all cases during the follow-up for 36 to 98 months. Conclusions: Transcatheter renal arterial angiography and embolization are the important and effective management for the diagnosis and treatment of congenital renal arteriovenous malformation. (authors)

  10. Ear tube surgery - what to ask your doctor

    Science.gov (United States)

    What to ask your doctor about ear tube surgery; Tympanostomy - what to ask your doctor; Myringotomy - what ... other treatments? What are the risks of the surgery? Is it safe to wait before getting ear ...

  11. Pediatric Obesity and Ear, Nose, and Throat Disorders

    Science.gov (United States)

    ... Marketplace Find an ENT Doctor Near You Pediatric Obesity and Ear, Nose, and Throat Disorders Pediatric Obesity ... self-esteem, and isolation from their peers. Pediatric obesity and otolaryngic problems Otolaryngologists, or ear, nose, and ...

  12. Middle Ear Infection (Chronic Otitis Media) and Hearing Loss

    Science.gov (United States)

    ... Marketplace Find an ENT Doctor Near You Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic Otitis ... relations staff at newsroom@entnet.org . What is otitis media? Otitis media refers to inflammation of the middle ...

  13. Sub-clinical middle ear malfunctions in elderly patients; prevalence ...

    African Journals Online (AJOL)

    ... absent acoustic reflex. Keywords: Middle ear malfunctions, elderly patients. ... hearing loss, had audiometric changes suggestive of such. We regarded such .... predictors of silent middle ear malfunction (control for Age and Sex). Variable.

  14. Pediatric Obesity and Ear, Nose, and Throat Disorders

    Science.gov (United States)

    ... ENTCareers Marketplace Find an ENT Doctor Near You Pediatric Obesity and Ear, Nose, and Throat Disorders Pediatric ... of self-esteem, and isolation from their peers. Pediatric obesity and otolaryngic problems Otolaryngologists, or ear, nose, ...

  15. Middle Ear Infection (Chronic Otitis Media) and Hearing Loss

    Science.gov (United States)

    ... ENT Doctor Near You Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic ... relations staff at newsroom@entnet.org . What is otitis media? Otitis media refers to inflammation of the ...

  16. Body lift, drag and power are relatively higher in large-eared than in small-eared bat species.

    Science.gov (United States)

    Håkansson, Jonas; Jakobsen, Lasse; Hedenström, Anders; Johansson, L Christoffer

    2017-10-01

    Bats navigate the dark using echolocation. Echolocation is enhanced by external ears, but external ears increase the projected frontal area and reduce the streamlining of the animal. External ears are thus expected to compromise flight efficiency, but research suggests that very large ears may mitigate the cost by producing aerodynamic lift. Here we compare quantitative aerodynamic measures of flight efficiency of two bat species, one large-eared ( Plecotus auritus ) and one small-eared ( Glossophaga soricina ), flying freely in a wind tunnel. We find that the body drag of both species is higher than previously assumed and that the large-eared species has a higher body drag coefficient, but also produces relatively more ear/body lift than the small-eared species, in line with prior studies on model bats. The measured aerodynamic power of P. auritus was higher than predicted from the aerodynamic model, while the small-eared species aligned with predictions. The relatively higher power of the large-eared species results in lower optimal flight speeds and our findings support the notion of a trade-off between the acoustic benefits of large external ears and aerodynamic performance. The result of this trade-off would be the eco-morphological correlation in bat flight, with large-eared bats generally adopting slow-flight feeding strategies. © 2017 The Author(s).

  17. Headaches from ear, nose and throat diseases

    International Nuclear Information System (INIS)

    Reck, R.

    1984-01-01

    Headaches are a frequent symptom in ENT-patients. The complex sensory innervation of the ear, nose and paranasal sinuses is demonstrated. Heterotopic or referred pain must be differentiated from homotopic pain that is experienced at the point of injury. The nervous pathways of heterotopic otalgia are shown. The quality of pain of the most common rhinological and otological diseases is reported. (orig.) [de

  18. DNA isolation from rat tail or ear

    NARCIS (Netherlands)

    Cuppen, E.

    2010-01-01

    This protocol describes a rapid procedure for isolating DNA from rat tail or ear punches. The simplest version of the protocol can be scaled for use in 96-well (deep-well) plates. The quality of the DNA is sufficient for any polymerase chain reaction (PCR)-based genotyping approach.

  19. The first neutron beam hits EAR2

    CERN Multimedia

    Antonella Del Rosso

    2014-01-01

    On 25 July 2014, about a year after construction work began, the Experimental Area 2 (EAR2) of CERN’s neutron facility n_TOF recorded its first beam. Unique in many aspects, EAR2 will start its rich programme of experimental physics this autumn.   The last part of the EAR2 beamline: the neutrons come from the underground target and reach the top of the beamline, where they hit the samples. Built about 20 metres above the neutron production target, EAR2 is in fact a bunker connected to the n_TOF underground facilities via a duct 80 cm in diameter, where the beamline is installed. The feet of the bunker support pillars are located on the concrete structure of the n_TOF tunnel and part of the structure lies above the old ISR building. A beam dump located on the roof of the building completes the structure. Neutrons are used by physicists to study neutron-induced reactions with applications in a number of fields, including nuclear waste transmutation, nuclear technology, nuclear astrop...

  20. "Hot Tub Rash" and "Swimmer's Ear" (Pseudomonas)

    Science.gov (United States)

    Facts About “Hot Tub Rash” and “Swimmer’s Ear” (Pseudomonas) What is Pseudomonas and how can it affect me? Pseudomonas (sue-doh- ... a major cause of infections commonly known as “hot tub rash” and “swimmer’s ear.” This germ is ...

  1. Cholesteatoma of the external ear canal

    DEFF Research Database (Denmark)

    Owen, Hanne H; Rosborg, Jørn; Gaihede, Michael

    2006-01-01

    (n = 2), and otorrhea (n = 1). Similar symptoms were found in secondary EECC, but less pronounced. In total the temporomandibular joint was exposed in 11 cases, while the mastoid and middle ear was invaded in six and three cases, respectively. In one primary case the facial nerve was exposed...

  2. The inner ear produces a natriuretic hormone

    DEFF Research Database (Denmark)

    Qvortrup, K; Rostgaard, J; Holstein-Rathlou, N H

    1996-01-01

    Cytoplasmic granules have been demonstrated in epithelial cells from the endolymphatic sac, an extraosseus part of the inner ear located in the posterior cranial fossa. Intravenously infused extracts from endolymphatic sacs in anesthetized rats elicited a potent natriuresis and diuresis without e...

  3. Bacteriology of chronic discharging ears in Port Harcourt, Nigeria ...

    African Journals Online (AJOL)

    Methods: Ear swabs of discharging ears aseptically collected from 102 patients of various age groups attending Ear, Nose, and Throat out-patient clinic at University of Port Harcourt Teaching Hospital were cultured for bacterial agents using blood agar, chocolate agar and MacConkey agar. Culture plates were incubated ...

  4. Influence of Ear Surface Area on Heat Tolerance of Composite ...

    African Journals Online (AJOL)

    Relative importance of ear surface area on heat tolerance of composite rabbit population was evaluated. The study was conducted during the dry and rainy seasons, climatic data were recorded to obtain categorical heat stress index. Physiological parameters, growth performance, ear length and ear width of the rabbits ...

  5. Role of skeletal muscle in ear development.

    Science.gov (United States)

    Rot, Irena; Baguma-Nibasheka, Mark; Costain, Willard J; Hong, Paul; Tafra, Robert; Mardesic-Brakus, Snjezana; Mrduljas-Djujic, Natasa; Saraga-Babic, Mirna; Kablar, Boris

    2017-10-01

    The current paper is a continuation of our work described in Rot and Kablar, 2010. Here, we show lists of 10 up- and 87 down-regulated genes obtained by a cDNA microarray analysis that compared developing Myf5-/-:Myod-/- (and Mrf4-/-) petrous part of the temporal bone, containing middle and inner ear, to the control, at embryonic day 18.5. Myf5-/-:Myod-/- fetuses entirely lack skeletal myoblasts and muscles. They are unable to move their head, which interferes with the perception of angular acceleration. Previously, we showed that the inner ear areas most affected in Myf5-/-:Myod-/- fetuses were the vestibular cristae ampullaris, sensitive to angular acceleration. Our finding that the type I hair cells were absent in the mutants' cristae was further used here to identify a profile of genes specific to the lacking cell type. Microarrays followed by a detailed consultation of web-accessible mouse databases allowed us to identify 6 candidate genes with a possible role in the development of the inner ear sensory organs: Actc1, Pgam2, Ldb3, Eno3, Hspb7 and Smpx. Additionally, we searched for human homologues of the candidate genes since a number of syndromes in humans have associated inner ear abnormalities. Mutations in one of our candidate genes, Smpx, have been reported as the cause of X-linked deafness in humans. Our current study suggests an epigenetic role that mechanical, and potentially other, stimuli originating from muscle, play in organogenesis, and offers an approach to finding novel genes responsible for altered inner ear phenotypes.

  6. Diagnosis of ossicular deformities and inner ear anomalies by high resolution CT scanning of the temporal bone

    International Nuclear Information System (INIS)

    Isono, Michio

    1988-01-01

    A comparative study was undertaken to determine the optimum slice thickness and slice gaps that can define the fine contour and continuity of the ossicles and the inner ear. Horizontal tomography was proved to be contributory enough to obtain the whole ossicular chains and the whole labyrinthine capsule. Comparison of 1.5 mm slice thickness / 1.5 mm slice gaps, 1.0 mm slice thickness / 1.0 mm slice gaps and 1.5 mm slice thickness / 1.0 mm slice gaps revealed that the last condition was optimal for demonstrating the ossicular chains, the first for the inner ear. A significant relationship was found between CT findings and surgical findings of ossicular chains in chronic otitis media and cholesteatoma. Radiological classification of the inner ear malformation, which was obtained by this method, were agenesis of the whole labyrinthine capsule, agenesis of the cochlea, slight protrusion of the cochlear growth, poor cochlear turning, agenesis of all the semicircular canals, anterior semicircular canals (s.c.) without posterior and lateral s.c., anterior s.c. without hypoplasia of posterior s.c. and aplasia of lateral s.c., and smallness of the vestibule. (author) 59 refs

  7. Diagnosis of ossicular deformities and inner ear anomalies by high resolution CT scanning of the temporal bone

    Energy Technology Data Exchange (ETDEWEB)

    Isono, Michio

    1988-04-01

    A comparative study was undertaken to determine the optimum slice thickness and slice gaps that can define the fine contour and continuity of the ossicles and the inner ear. Horizontal tomography was proved to be contributory enough to obtain the whole ossicular chains and the whole labyrinthine capsule. Comparison of 1.5 mm slice thickness / 1.5 mm slice gaps, 1.0 mm slice thickness / 1.0 mm slice gaps and 1.5 mm slice thickness / 1.0 mm slice gaps revealed that the last condition was optimal for demonstrating the ossicular chains, the first for the inner ear. A significant relationship was found between CT findings and surgical findings of ossicular chains in chronic otitis media and cholesteatoma. Radiological classification of the inner ear malformation, which was obtained by this method, were agenesis of the whole labyrinthine capsule, agenesis of the cochlea, slight protrusion of the cochlear growth, poor cochlear turning, agenesis of all the semicircular canals, anterior semicircular canals (s.c.) without posterior and lateral s.c., anterior s.c. without hypoplasia of posterior s.c. and aplasia of lateral s.c., and smallness of the vestibule. (author) 59 refs.

  8. Naturopathic treatment for ear pain in children.

    Science.gov (United States)

    Sarrell, E Michael; Cohen, Herman Avner; Kahan, Ernesto

    2003-05-01

    Otitis media is 1 of the most frequent diseases of early infancy and childhood and 1 of the most common reasons for children to visit a physician. In the past 2 decades, there has been a substantial increase in the diagnosis of otitis media worldwide. In the United States, 93% of all children have had at least 1 episode of acute otitis media (AOM) by 7 years of age. Otalgia is the hallmark of AOM. Most affected children either complain of earache or manifest behavior that the parents interpret as indicating ear pain. Treatment of the ear pain early in the course of AOM decreases both parental anxiety and the child's discomfort and accelerates the healing process. The objective of this study was to determine the efficacy and tolerability of naturopathic versus traditional treatment for the management of otalgia commonly associated with AOM in children. The study was designed as a double-blind trial in an outpatient community clinic. A total of 171 children who were aged 5 to 18 years and had otalgia and clinical findings associated with middle-ear infection were studied. The children were randomly assigned to receive treatment with Naturopathic Herbal Extract Ear Drops (NHED) or anesthetic ear drops, with or without amoxicillin. On enrollment, the children were assigned by computer-numbered randomization to receive NHED (contents: allium sativum, verbascum thapsus, calendula flores, hypericum perfoliatum, lavender, and vitamin E in olive oil) 5 drops 3 times daily, alone (group A) or together with a topical anesthetic (amethocaine and phenazone in glycerin) 5 drops 3 times daily (group B), or oral amoxicillin 80 mg/kg/d (maximum 500 mg/dose) divided into 3 doses with either NHED 5 drops 3 times daily (group C) or topical anesthetic 5 drops 3 times daily (group D). A double-blind design was used, and all ear drops were placed in identical bottles. Treatment was initiated by the nurse in all cases. A single physician (M.S.) evaluated and treated all of the patients

  9. PET in malformations of cortical development

    International Nuclear Information System (INIS)

    Bouilleret, V.; O'Brien, T.J.; Bouilleret, V.; Bouilleret, V.; Chiron, C.; Chiron, C.

    2009-01-01

    Within the group of malformations of cortical development, focal cortical dysplasia (FCD) are an increasingly recognized cause of intractable epilepsy that can be cured by surgery. The success of cortical resection for intractable epilepsy is highly dependent on the accurate pre-surgical delineation of the regions responsible for generating seizures. [ 18 F]-FDG PET, which images cerebral metabolism studying brain glucose uptake, is the most established functional imaging modality in the evaluation of patients with epilepsy. The aim of this article is to review [ 18 F]-FDG PET usefulness as a pre-surgical tool in the evaluation of medically refractory partial epilepsy. It has an established place in assisting in the localisation and definition of FCD in patients with no lesion, or only a subtle abnormality, on MRI. The role of FDG-PET in defining the extent of the surgical resection is still uncertain and needs to be the focus of future research. (authors)

  10. Intracranial arteriovenous malformation. Contralateral steal phenomena

    Energy Technology Data Exchange (ETDEWEB)

    Batjer, H H; Devous, M D; Seibert, G B; Purdy, P D; Ajmani, A K; Delarosa, M; Bonte, F J [Texas Univ., Dallas, TX (USA). Southwestern Medical Center

    1989-05-01

    Sixty-two patients with radiographically proven intracranial arteriovenous malformations underwent preoperative regional cerebral blood flow measurement with {sup 133}Xe signal-photon emission computed tomography. Contralateral regions of hypoperfusion were deteceted in all cases. Steal severity was assessed according to the contralateral steal index (ISteal(c)). ISteal(c) was < 0.7 (severe) in 22 (35%), 0.7-0.8 (intermediate) in 18 (29%), and > 0.8 (mild) in 22 (35%). ISteal(c) was more frequently severe or mild in females and more often intermediate in males in males (p < 0.05). Hyperemic complications were encountered more frequently in patients with intermediate ISteal(c) (p = 0.086). An unfavorable outcome was associated with less severe contralateral steal (p = 0.12). A detailed clinical, radiographic, and hemodynamic profile may help to preperatively identify patients at high risk for a poor surgical outcome. (author).

  11. Management of patients with brain arteriovenous malformations

    International Nuclear Information System (INIS)

    Soederman, Michael; Andersson, Tommy; Karlsson, Bengt; Wallace, M. Christopher; Edner, Goeran

    2003-01-01

    Arteriovenous malformations (AVMs) of the brain, which are probably genetically determined, are errors in the development of the vasculature that, together with the effects of blood flow, may lead to a focal arteriovenous shunt. Clinically, the adult patient may present with acute or chronic neurological symptoms--fixed or unstable--such as deficits, seizures or headache. Sometimes the lesion is an incidental finding. In about half of the patients, the revealing event is an intracranial haemorrhage. The prevalence of AVM in the western world is probably 10 ml could benefit from targeted partial embolisation followed by radiosurgery or surgery, depending on the angioarchitecture; and (IV) AVMs >20 ml nidus volume usually have a high treatment risk with any treatment modality and are not obvious targets for treatment at all

  12. Contemporary Management of Pulmonary Arteriovenous Malformations.

    Science.gov (United States)

    Rauh, Nicholas; Gurley, John; Saha, Sibu

    2017-12-01

    Pulmonary arteriovenous malformations (PAVMs) are atypical vascular structures involving a direct connection between the pulmonary arterial and venous circulations. While PAVMs are a relatively uncommon disorder, unmanaged cases are at risk for the development of serious complications including embolization and infection. Since their first description in 1897, PAVMs have been identified and treated in a variety of ways. Advancements in diagnostic methods and operative techniques have allowed for more effective treatment of the disease. Most recently, the use of vascular plug transcatheter embolization has been described as an effective therapeutic procedure in the management of PAVMs. In this report, we present our experience with nine cases of PAVMs treated at the University of Kentucky, including special consideration of an exemplary case that illustrates the typical course of treatment for the disease.

  13. Symptomatic Chiari malformation in infancy and adolescence

    International Nuclear Information System (INIS)

    Fukushima, Takeo; Matsuda, Toshihiro; Tsugu, Hitoshi; Sakamoto, Seisaburou; Tomonaga, Masamichi; Asakawa, Koji; Mitsudome, Akihisa; Hashimoto, Takahisa.

    1994-01-01

    Of the 7 children and adolescents with Chiari malformation reported here 5 had Chiari type 1 and 2 had Chiari type 2 disease. All 5 Chiari type 1 patients had syringomyelia and scoliosis, but the Chiari type 2 patients did not. All the patients except one with localized syringomyelia (case 3) underwent posterior decompression with suboccipital craniectomy, upper cervical laminectomy and duraplasty, and their clinical signs and symptoms improved. Case 3 received a syringo-subarachnoid shunt and improved clinically. Motor function was restored better than sensory function. Cranial nerve signs were cleared up in the Chiari type 2 patients. In the Chiari type 1 patients postoperative neurological improvement correlated well with the collapse of syringomyelia. Cine MR imaging was useful in the evaluation of CSF dynamics at the cranio-vertebral junction and in the syringomyelic cavity before and after surgery. (author)

  14. Congenital bronchopulmonary malformation: CT histopathological correlation.

    Science.gov (United States)

    Kyncl, Martin; Koci, Martin; Ptackova, Lea; Hornofova, Ludmila; Ondrej, Fabian; Snajdauf, Jiri; Pychova, Marcela

    2016-12-01

    This study evaluated the accuracy of postnatal computed tomography (CT) imaging in the identification of congenital bronchopulmonary malformation (BPM) in comparison with histopathological analysis. CT scans of prenatally diagnosed BPMs from 24 patients with available histology were analysed retrospectively. The CT images were reviewed blinded to histological findings by two radiologists. Specific diagnosis was assigned based on predetermined criteria. The accuracy of CT was evaluated. The agreement rate in CT diagnosis between two radiologists was 100%. In 75% the lesions were located in the lower lobes. An overlap of 71% in CT and histopathological diagnoses was reached. The least matching diagnosis was type 2 CPAM. Contrast enhanced chest CT is very accurate in characterizing the BPM spectrum and provides important information on lesion type and structure.

  15. Inner ear pressure changes following square wave intracranial or ear canal pressure manipulation in the same guinea pig

    NARCIS (Netherlands)

    Thalen, E; Wit, H; Segenhout, H; Albers, F

    Inner ear pressure was measured in scala tympani with a micropipette during square wave pressure manipulation of the intracranial compartment and, subsequently, of the external ear canal (EEC) in the same guinea pig. As expected, the combination of the cochlear aqueduct and the inner ear behaves as

  16. Pulmonary arteriovenous malformations: overview and transcatheter embolotherapy

    International Nuclear Information System (INIS)

    Pugash, R.A.

    2001-01-01

    The majority of pulmonary arteriovenous malformations (pAVMs) are found in people with hereditary hemorrhagic telangiectasia (HHT), a condition also known as Osler-Weber-Rendu syndrome. HHT is a clinically heterogeneous autosomal dominant disorder in which abnormal blood vessels cause bleeding and arteriovenous shunting. The 2 basic lesions of HHT - telangiectasias and arteriovenous malformations (AVMs) - are closely related. Multisystem involvement leads to a staggering array of clinical manifestations, making HHT one of medicine's less familiar 'great pretenders'. Telangiectasias are dilated blood vessels, typically located in mucocutaneous surfaces (i.e., skin, conjunctiva, respiratory tract, gastrointestinal tract, urinary tract). Small telangiectasias are simply dilated post-capillary venules, whereas larger telangiectasias are made up of dilated arterioles and venules, often with no intervening capillary. They are, in essence, diminutive AVMs. These tiny lesions are visible as punctate bright red spots on skin and mucosal surfaces (Fig. 1). Their fragility and superficial location account for the disabling epistaxis and chronic gastrointestinal bleeding, which are so common with HHT. Hematuria (caused by urothelial telangiectasias) occurs occasionally but is not a prominent feature of the disease. Although tracheobronchial telangiectasias do occur and may cause hemoptysis, severe hemoptysis is typically related to pAVM rupture. AVMs are direct artery-to-vein connections. Though larger and far more impressive radiologically than telangiectasias, AVMs are more likely to be clinically silent until they either declare themselves in a catastrophic fashion or are detected by screening tests. In contrast to telangiectasias, which are generally found in epithelial surfaces, AVMs tend to develop within organs, most commonly the lung and brain. As screening methods evolve, liver involvement with both telangiectasias and complex AVMs is being recognized with increasing

  17. Pulmonary arteriovenous malformations: overview and transcatheter embolotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Pugash, R.A. [Univ. of Toronto, St. Michael' s Hospital, Dept. of Medical Imaging, Toronto, Ontario (Canada)

    2001-04-01

    The majority of pulmonary arteriovenous malformations (pAVMs) are found in people with hereditary hemorrhagic telangiectasia (HHT), a condition also known as Osler-Weber-Rendu syndrome. HHT is a clinically heterogeneous autosomal dominant disorder in which abnormal blood vessels cause bleeding and arteriovenous shunting. The 2 basic lesions of HHT - telangiectasias and arteriovenous malformations (AVMs) - are closely related. Multisystem involvement leads to a staggering array of clinical manifestations, making HHT one of medicine's less familiar 'great pretenders'. Telangiectasias are dilated blood vessels, typically located in mucocutaneous surfaces (i.e., skin, conjunctiva, respiratory tract, gastrointestinal tract, urinary tract). Small telangiectasias are simply dilated post-capillary venules, whereas larger telangiectasias are made up of dilated arterioles and venules, often with no intervening capillary. They are, in essence, diminutive AVMs. These tiny lesions are visible as punctate bright red spots on skin and mucosal surfaces (Fig. 1). Their fragility and superficial location account for the disabling epistaxis and chronic gastrointestinal bleeding, which are so common with HHT. Hematuria (caused by urothelial telangiectasias) occurs occasionally but is not a prominent feature of the disease. Although tracheobronchial telangiectasias do occur and may cause hemoptysis, severe hemoptysis is typically related to pAVM rupture. AVMs are direct artery-to-vein connections. Though larger and far more impressive radiologically than telangiectasias, AVMs are more likely to be clinically silent until they either declare themselves in a catastrophic fashion or are detected by screening tests. In contrast to telangiectasias, which are generally found in epithelial surfaces, AVMs tend to develop within organs, most commonly the lung and brain. As screening methods evolve, liver involvement with both telangiectasias and complex AVMs is being recognized

  18. Amplatzer vascular plugs in congenital cardiovascular malformations

    Directory of Open Access Journals (Sweden)

    Parag Barwad

    2013-01-01

    Full Text Available Background: Amplatzer vascular plugs (AVPs are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs. Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33% were AVP type I and 23 (59% were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM (n = 7, aortopulmonary collaterals (n = 7, closure of a patent Blalock-Taussig shunt (n = 5, systemic AVM (n = 5, coronary AVM (n = 4, patent ductus arteriosus (PDA (n = 3, pulmonary artery aneurysms (n = 3, and venovenous collaterals (n = 2. Deployment of the AVP was done predominantly via the 5 - 7F Judkin′s right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow.

  19. Eye malformations in Cameroonian children: a clinical survey

    Directory of Open Access Journals (Sweden)

    Eballé AO

    2012-10-01

    Full Text Available André Omgbwa Eballé,1,2 Augustin Ellong,3 Godefroy Koki,3 Ngoune Chantal Nanfack,3 Viola Andin Dohvoma,3 Côme Ebana Mvogo2,31Yaoundé Gynaecology, Obstetrics and Pediatrics Hospital, Yaoundé, Cameroon; 2Faculty of Medicine and Pharmaceutical Sciences, University of Douala, Cameroon; 3Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, CameroonSummary: The aim of this work was to describe the clinical aspects of eye malformations observed at the ophthalmology unit of the Yaoundé Gynaecology, Obstetrics and Pediatrics Hospital.Patients and methods: We carried out a retrospective study of all malformations of the eye and its adnexae observed among children aged 0–5 years who were seen at the ophthalmology unit from January 2003 to December 2009.Results: Out of the 2254 children who were examined, 150 (6.65% presented eye malformations. The mean age was 14.40 ± 4 months. Eye malformations were diagnosed in 71.66% of cases during the first year of life. The most frequent malformations were congenital lacrimal duct obstruction (66.66%, congenital cataract (10.9%, congenital glaucoma (10.9%, microphthalmos (5.03%, and congenital ptosis (3.77%.Conclusion: Eye malformations among children can lead to visual impairment and are a cause for discomfort to children and parents. Therefore, systematic postnatal screening is recommended to enable early management.Keywords: malformations, ophthalmology, child, Cameroon

  20. Ear care: an update for nurses (part 1)

    OpenAIRE

    Millward, K.

    2017-01-01

    A healthy ear is vital not just for hearing, but for balance and for full engagement with the community. In the first of two articles, Kat Millward reviews the anatomy of the ear, outlines methods of dealing with cerumen, and discusses methods of assessment and diagnosis\\ud \\ud Our ears are essential for both hearing and balance. Up to 4% of the population will have difficulties with their ears relating to impacted cerumen and many of them will present in primary care with ear discomfort or h...

  1. Contribution of Rare Copy Number Variants to Isolated Human Malformations

    Science.gov (United States)

    Serra-Juhé, Clara; Rodríguez-Santiago, Benjamín; Cuscó, Ivon; Vendrell, Teresa; Camats, Núria; Torán, Núria; Pérez-Jurado, Luis A.

    2012-01-01

    Background Congenital malformations are present in approximately 2–3% of liveborn babies and 20% of stillborn fetuses. The mechanisms underlying the majority of sporadic and isolated congenital malformations are poorly understood, although it is hypothesized that the accumulation of rare genetic, genomic and epigenetic variants converge to deregulate developmental networks. Methodology/Principal Findings We selected samples from 95 fetuses with congenital malformations not ascribed to a specific syndrome (68 with isolated malformations, 27 with multiple malformations). Karyotyping and Multiplex Ligation-dependent Probe Amplification (MLPA) discarded recurrent genomic and cytogenetic rearrangements. DNA extracted from the affected tissue (46%) or from lung or liver (54%) was analyzed by molecular karyotyping. Validations and inheritance were obtained by MLPA. We identified 22 rare copy number variants (CNV) [>100 kb, either absent (n = 7) or very uncommon (n = 15, malformations (21%), including 11 deletions and 11 duplications. One of the 9 tested rearrangements was de novo while the remaining were inherited from a healthy parent. The highest frequency was observed in fetuses with heart hypoplasia (8/17, 62.5%), with two events previously related with the phenotype. Double events hitting candidate genes were detected in two samples with brain malformations. Globally, the burden of deletions was significantly higher in fetuses with malformations compared to controls. Conclusions/Significance Our data reveal a significant contribution of rare deletion-type CNV, mostly inherited but also de novo, to human congenital malformations, especially heart hypoplasia, and reinforce the hypothesis of a multifactorial etiology in most cases. PMID:23056206

  2. Prenatal ultrasound findings observed in the Wolf-Hirschhorn syndrome: data from the registry of congenital malformations in Auvergne.

    Science.gov (United States)

    Debost-Legrand, Anne; Goumy, Carole; Laurichesse-Delmas, Hélène; Déchelotte, Pierre; Beaufrère, Anne-Marie; Lémery, Didier; Francannet, Christine; Gallot, Denis

    2013-12-01

    Wolf-Hirschhorn syndrome (WHS) is associated with facial dysmorphism including high forehead, high nasal bridge, hypertelorism and severe mental retardation. WHS results from a 4p16.3 deletion. Only a small number of reports have been made on the prenatal ultrasound findings observed in WHS. Here we report our experience on 10 cases of WHS ascertained prenatally between 1983 and 2009 through the CEMC-Auvergne registry of congenital malformations. The assumption that a "Greek warrior helmet" facies is pathognomonic of WHS could lead to misdiagnosis. Other clinical findings such as severe and early onset intrauterine growth retardation, facial dysmorphism (high forehead, high nasal bridge, low-set ears, micrognathia, hypertelorism), atrial or ventricular septal defect, and renal dysplasia should help obstetricians to suspect the diagnosis of WHS prenatally. Copyright © 2013 Wiley Periodicals, Inc.

  3. Surgical experience with arteriovenous malformations of the brain

    International Nuclear Information System (INIS)

    Stein, B.M.

    1984-01-01

    Personal experience with the treatment of 200 arteriovenous malformations of the brain seen during the past decade is presented. One hundred and twenty of these patients underwent surgery, with a mortality of 1,7%. The morbidity rate of 10% includes cases of hemianopia after occipital malformations had been removed. Significant morbidity, including aphasia, hemiparesis and hemisensory loss, occurred in under 10% of cases. Computerized tomography plays an important role in determining the relationships of the arteriovenous malformations to associated structures such as the ventricular system

  4. ECOLOGICALLY DETERMINED MALFORMATIONS IN CHILDREN IN THE ULYANOVSK REGION

    Directory of Open Access Journals (Sweden)

    Elizaveta Grigoryevna Panchenko

    2018-03-01

    Full Text Available Currently particularly relevant is the interaction between ecology and people. The study had been carried to examine the correlation of congenital malformations from the residence. The analysis showed that increasing concentrations of heavy metals (lead, cadmium, and chromium in soil and air in some districts of the Ulyanovsk region correlates with a large number of congenital malformations in children, in contrast to those areas, where their content does not excees MPC, which allows to consider imbalance of trace elements as a possible factor in the development of congenital malformations.

  5. Fetal magnetic resonance imaging of thoracic and abdominal malformations

    International Nuclear Information System (INIS)

    Woitek, R.; Asenbaum, U.; Furtner, J.; Prayer, D.; Brugger, P.C.

    2013-01-01

    Diagnosis and differential diagnosis of fetal thoracic and abdominal malformations. Ultrasound and magnetic resonance imaging (MRI). In cases of suspected pathologies based on fetal ultrasound MRI can be used for more detailed examinations and can be of assistance in the differential diagnostic process. Improved imaging of anatomical structures and of the composition of different tissues by the use of different MRI sequences. Fetal MRI has become a part of clinical routine in thoracic and abdominal malformations and is the basis for scientific research in this field. In cases of thoracic or abdominal malformations fetal MRI provides important information additional to ultrasound to improve diagnostic accuracy, prognostic evaluation and surgical planning. (orig.) [de

  6. 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

  7. Evaluation of magnetic resonance imaging of Arnold-Chiari malformation

    Energy Technology Data Exchange (ETDEWEB)

    Yoshino, Kimihiro; Suga, Masakazu; Takemoto, Motohisa

    1987-06-01

    We evaluated the usefulness of magnetic resonance imaging (MRI) in the diagnosis of the Arnold-Chiari malformation. The patient, a 52-year-old man, complained of dizziness on walking. He initially refused to undergone myelography, but 4 months later, underwent MRI test, which lead to the diagnosis of Arnold-Chiari malformation (I type). Not all patients with symptoms of Arnold-Chiari malformation, syringomyelia, syringobulbia and cervical spinal tumor, undergone myelography, which is an invasive technique, therefore MRI should be the first examination for the patients with disorders involving the craniocervical junction.

  8. About kinetics of paramagnetic radiation malformations in beryllium ceramics

    International Nuclear Information System (INIS)

    Polyakov, A.I.; Ryabinkin, Yu.A.; Zashkvara, O.V.; Bitenbaev, M.I.; Petukhov, Yu.V.

    1999-01-01

    This paper [1] specifies that γ-radiation of the beryllium-oxide-based ceramics results in development of paramagnetic radiation malformations emerging the ESR spectrum in form of doublet with the splitting rate of oestrasid Δ∼1.6 and g-factor of 2.008. This report presents evaluation outcomes of dependence of paramagnetic radiation malformations concentration in beryllium ceramics on gamma-radiation dose ( 60 Co) within the range of 0-100 Mrad. Total paramagnetic parameters of beryllium ceramics in the range 0-100 Mrad of gamma-radiation dose varied slightly, and were specified by the first type of paramagnetic radiation malformations

  9. A Rare Case of Petrified Ear

    Directory of Open Access Journals (Sweden)

    Kathryn E. Buikema

    2012-01-01

    Full Text Available Calcification or ossification of the auricle, also referred to as petrified ear, is a rare diagnosis in dermatology. In medical literature, it has most often been attributed to trauma, hypothermia and frostbite, or hypercalcemia secondary to a metabolic or endocrine disorder, such as Addison's disease. Here, we report the clinical and radiologic findings of a 79-year-old African American male whose unilateral petrified auricle was an incidental finding. He had a preceding history of hyperparathyroidism and subsequent hypercalcemia treated with a subtotal parathyroidectomy three years prior to presentation. In addition to laboratory analysis, a history and physical examination was performed which revealed no other signs of hypercalcemia. Radiologic studies demonstrated partial ossification of the external auricular cartilage on the left side. The patient was diagnosed with the rare occurrence of a petrified ear. In light of this case, we provide a discussion concerning the possible etiologies of this diagnosis including appropriate patient evaluation and possible treatment recommendations.

  10. Ear embryonic rabdomiosarcoma. A case report

    International Nuclear Information System (INIS)

    Cueto, L.; Canabal, A.; Blanco, A.; Sabate, J.

    2002-01-01

    A case of embryonic rabdomiosarcoma in the ear of a 5-year-old girl who initially shows clinical symptoms of otitis media. The CT reveals a dense lesion of soft tissue which shows up slightly in the right external auditory channel. Also of interest were osteolytic areas in the petrous, clivus and zygomatic arch. A hypointensive lesion with marked enhancement after Gd-DPTA injection is observed. Discussed are the imaging methods used in the diagnosis of this tumor. (Author) 10 refs

  11. ElectroAcoustic Resource Site (EARS)

    OpenAIRE

    Atkinson, Simon; Landy, Leigh

    2002-01-01

    Central to the MTIRC’s electroacoustic music studies focus, EARS is supported by the AHRC’s resource enhancement scheme (ca. £170k) and Unesco (£3k). Beyond its initial goal to create the glossary (~500 entries) and related index (providing a coherent hyperlink structure for the bibliography – ~3000 entries) there are further methodological research imperatives – to offer a web-based resource that: a) Realises an accessible research resource (glossary, keyword index and bibliography) to co...

  12. Structural Metadata Research in the Ears Program

    Science.gov (United States)

    2005-01-01

    detecting structural information in the word stream (the so-called “structural MDE” portion of the EARS program); other MDE efforts on speaker ... diarization are overviewed in [13]. The rest of this paper is organized as follows. We describe the structural MDE tasks, performance measurement, and corpora...tems have only recently been introduced, with NIST reporting re- sults with the Wilcoxon signed rank test for speaker -level average score differences

  13. Pressure equilibration in the penguin middle ear.

    Science.gov (United States)

    Sadé, Jacob; Handrich, Yves; Bernheim, Joelle; Cohen, David

    2008-01-01

    King penguins have a venous structure in the form of a corpus cavernosum (CC) in their middle ear (ME) submucosa. The CC may be viewed as a special organelle that can change ME volume for pressure equilibration during deep-sea diving it is a pressure regulating organelle (PRO). A similar CC and muscles also surround the external ear (EE) and may constrict it, isolating the tympanic membrane from the outside. A CC was previously found also in the ME of marine diving mammals and can be expected to exist in other deep diving animals, such as marine turtles. Marine animals require equalization of middle ear (ME) pressure when diving hundreds or thousands of meters to catch prey. We investigated what mechanism enables king penguins to protect their ME when they dive to great depths. Biopsies and serial sections of the ME and the EE of the deep diving king penguin (Aptenodytes patagonicus) were examined microscopically. It was demonstrated that the penguin ME has an extensive network of small and large submucosal venous sinuses. This venous formation, a corpus cavernosum, can expand and potentially 'flood' the ME almost completely on diving, thus elevating ME pressure and reducing the ME space. The EE has a similar protective mechanism.

  14. Ear recognition from one sample per person.

    Directory of Open Access Journals (Sweden)

    Long Chen

    Full Text Available Biometrics has the advantages of efficiency and convenience in identity authentication. As one of the most promising biometric-based methods, ear recognition has received broad attention and research. Previous studies have achieved remarkable performance with multiple samples per person (MSPP in the gallery. However, most conventional methods are insufficient when there is only one sample per person (OSPP available in the gallery. To solve the OSPP problem by maximizing the use of a single sample, this paper proposes a hybrid multi-keypoint descriptor sparse representation-based classification (MKD-SRC ear recognition approach based on 2D and 3D information. Because most 3D sensors capture 3D data accessorizing the corresponding 2D data, it is sensible to use both types of information. First, the ear region is extracted from the profile. Second, keypoints are detected and described for both the 2D texture image and 3D range image. Then, the hybrid MKD-SRC algorithm is used to complete the recognition with only OSPP in the gallery. Experimental results on a benchmark dataset have demonstrated the feasibility and effectiveness of the proposed method in resolving the OSPP problem. A Rank-one recognition rate of 96.4% is achieved for a gallery of 415 subjects, and the time involved in the computation is satisfactory compared to conventional methods.

  15. Ear recognition from one sample per person.

    Science.gov (United States)

    Chen, Long; Mu, Zhichun; Zhang, Baoqing; Zhang, Yi

    2015-01-01

    Biometrics has the advantages of efficiency and convenience in identity authentication. As one of the most promising biometric-based methods, ear recognition has received broad attention and research. Previous studies have achieved remarkable performance with multiple samples per person (MSPP) in the gallery. However, most conventional methods are insufficient when there is only one sample per person (OSPP) available in the gallery. To solve the OSPP problem by maximizing the use of a single sample, this paper proposes a hybrid multi-keypoint descriptor sparse representation-based classification (MKD-SRC) ear recognition approach based on 2D and 3D information. Because most 3D sensors capture 3D data accessorizing the corresponding 2D data, it is sensible to use both types of information. First, the ear region is extracted from the profile. Second, keypoints are detected and described for both the 2D texture image and 3D range image. Then, the hybrid MKD-SRC algorithm is used to complete the recognition with only OSPP in the gallery. Experimental results on a benchmark dataset have demonstrated the feasibility and effectiveness of the proposed method in resolving the OSPP problem. A Rank-one recognition rate of 96.4% is achieved for a gallery of 415 subjects, and the time involved in the computation is satisfactory compared to conventional methods.

  16. Pathology of ear hematomas in swine.

    Science.gov (United States)

    Drolet, Richard; Hélie, Pierre; D'Allaire, Sylvie

    2016-05-01

    The objectives of our study were to describe the pathology of ear hematomas in swine and to add to the comprehension of the pathogenesis of this condition. The pathogenesis of aural hematomas has been studied mainly in dogs; however, disagreements exist about the precise anatomic location of the hemorrhage. Sixteen pigs with ear hematoma at various stages of development were included in this study. The pigs were submitted for routine autopsy for various and unrelated reasons over a period of several years. Based on gross examination, the 16 cases of aural hematomas were subjectively classified as acute (n = 6), subacute (n = 3), and chronic (n = 7). The age of the animals at the time of autopsy ranged from 2 weeks to adulthood, with all acute cases being hematoma developed predominantly in a subperichondral location on both sides of the cartilaginous plate simultaneously. Within these same cases, there were also some areas in which blood-filled clefts had formed within the cartilage itself. Besides fibroplasia, neoformation of cartilage was found to represent a significant part of the repair process. All chronic cases were characterized on cross-section of the ear by the presence of at least 2 distinct, wavy, focally folded, and roughly parallel plates of cartilage separated from each other by fibrous tissue. © 2016 The Author(s).

  17. Magnetic resonance imaging (MRI) of congenital cardiovascular malformations

    International Nuclear Information System (INIS)

    Sakakibara, Makoto; Kobayashi, Shirou; Imai, Hitoshi; Watanabe, Shigeru; Masuda, Yoshiaki; Inagaki, Yoshiaki; Morita, Huminori; Uematsu, Sadao; Arimizu, Noboru

    1986-01-01

    In order to determine the value of MRI in diagnosing congenital cardiovascular malformations, MR Images were obtained in 25 adult patients with congenital cardiovascular malformations. Gated MRI detected all of 13 atrial septal defects, and all of 4 ventricular septal defects, but ungated MRI detected none of 3 atrial septal defects. Other congenital cardiovascular malformations (2 with Ebstein's disease, 1 with Fallot's pentalogy, and 1 with Pulmonary stenosis) were well visualized. Vascular malformations (1 with Patent ducts arteriosus, 1 with Supravalvelar aortic stenosis, 1 with Coarctation of Aorta, 1 with Right Aortic Arch) were well visualized in all of 7 patients by ungated MRI. MRI was a valuable noninvasive method of diagnosing congenital heart disease. (author)

  18. Prostatic urethra malformation associated with retrograde ejaculation: a case report.

    Science.gov (United States)

    Zhao, Kai; Zhang, Jianzhong; Xu, Aiming; Zhang, Cheng; Wang, Zengjun

    2016-12-21

    Retrograde ejaculation can have anatomical, neurogenic, or pharmacological causes. Among these factors, malformation of the prostatic urethra is an uncommon cause. We describe a 29-year-old Han Chinese man with absence of his verumontanum combined with ejaculatory duct cysts, and no other cause for ejaculatory dysfunction. His verumontanum was replaced by a deep groove adjacent to his bladder neck, which could significantly influence bladder neck contraction. In addition, the large cysts in the ejaculatory duct could obstruct the anterior outlet of his prostatic urethra and prevent seminal fluid flow in an anterograde direction. There are few reports of retrograde ejaculation associated with congenital malformations of the posterior urethra. Malformations associated with bladder neck laxity and increased tone of the prostatic urethral outlet can contribute to retrograde ejaculation. Malformation of the prostatic urethra is an uncommon cause of retrograde ejaculation, and can be difficult to treat.

  19. Screening for congenital heart malformation in child health centres

    NARCIS (Netherlands)

    R.E. Juttmann (Rikard); J. Hess (Jakob); C.W.N. Looman (Caspar); G.J. van Oortmarssen (Gerrit); P.J. van der Maas (Paul)

    1998-01-01

    textabstractBACKGROUND: Although screening for congenital heart malformations is part of the child health care programme in several countries, there are very few published evaluations of these activities. This report is concerned with the evaluation of this screening at

  20. Epidermal Nevus Syndrome Associated with Brain Malformations and Medulloblastoma

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-01-01

    Full Text Available Researchers at Juntendo University and Tokyo Women’s Medical University, Japan; and University of California, San Francisco, Ca, report a male infant with epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma.

  1. Posterior fossa malformations: main features and limits in prenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Department of Radiology, Paris (France)

    2010-06-15

    Posterior fossa (PF) malformations are commonly observed during prenatal screening. Their understanding requires knowledge of the main steps of PF development and knowledge of normal patterns in US and MR imaging. The vast majority of PF malformations can be strongly suspected by acquiring a midline sagittal slice and a transverse slice and by systematically scrutinizing the elements of the PF: cerebellar vermis, hemispheres, brainstem, fourth ventricle, PF fluid spaces and tentorium. Analysis of cerebellar echogenicity and biometry is also useful. This review explains how to approach the diagnosis of the main PF malformations by performing these two slices and answering six key questions about the elements of the PF. The main imaging characteristics of PF malformations are also reviewed. (orig.)

  2. Malformations induced by gamma irradiation combined with vitamin A administration in pregnant female albino rats and their foetuses

    International Nuclear Information System (INIS)

    Ramadan, F.L.

    2007-01-01

    In The Present investigation, oral administration of vitamin A of the therapeutic doses and double therapeutic doses (9.000 lU/kg b.wt and 18.000 IU/ kg body, wt) to female rats starting on day 1 up to day 19 of pregnancy and exposed to 3 Gy (1 Gy/3 times) whole body gamma irradiation on days 7th, 1th and 15th of gestation (dissection was preformed on day 20) caused morphological, histochemical and skeletal changes in pregnant rats and their foetuses. The congenital anomalies occurred in foetuses when pregnant rats were exposed to γ-irradiation including diminution of size and subcutaneous haemorrhage. On the other hand, miscellaneous malformations including kypophysis, exencephally, anophthalmia and deformation of ear region were designated in foetuses maternally treated with excess vitamin A. The malformations were severe when mothers were irradiated during vitamin A administration as manifested by macrocephaly and fusion of digits of the hind limb (Oligosyndactyly). The examination of the endo skeletal system of foetuses obtained from irradiated pregnant rats and treated with low or excess doses of vitamin A showed retardation in of the ossification of the skull bones and lack of ossification at the centre of vertebrae. Moreover, no ossification was observed in sternebra, metacarpals, metatarsals and phalanges. In the present study, the content of DNA exhibited significant decrease in mother irradiated and combined or not with vitamin A. The results are of great importance from the standpoint of radiation protection and drug safety

  3. Echography of congenital malformations of the central nervous system

    International Nuclear Information System (INIS)

    Toirac Romani, Carlos Andres; Salmon Cruzata, Acelia; Musle Acosta, Mirelvis; Rosales Fargie, Yamile; Dosouto Infante, Vivian

    2010-01-01

    A descriptive and prospective study was conducted in 173 pregnant women attended at the Provincial Department of Clinical Genetics of Santiago de Cuba, from January, 2000 to December, 2004, to identify congenital malformations of the central nervous system detected by means of echography. The most frequent malformation was the hydrocephaly, followed by the fusion defects of the spine, associated with the hydrocephaly and the absence of cranial cavity. There was a prevalence of altered alpha fetoprotein and of elevated amniotic fluid

  4. Screening for congenital heart malformations in child health centres

    OpenAIRE

    Juttmann, Rikard

    1999-01-01

    textabstractThe objective of this thesis is to clarify the effectiveness and the efficiency of screening for congenital heart malformations in Dutch child health centres and the possibilities to optimise this prevention programme. To this end the following main questions will be addressed. 1. Does screening for congenital heart malformations, as actually performed in Dutch child health centres, prevent adverse outcomes of these disorders in the short and long run? What would be the answer to ...

  5. Infertility, infertility treatment, and congenital malformations: Danish national birth cohort

    Science.gov (United States)

    Zhu, Jin Liang; Basso, Olga; Obel, Carsten; Bille, Camilla; Olsen, Jørn

    2006-01-01

    Objectives To examine whether infertile couples (with a time to pregnancy of > 12 months), who conceive naturally or after treatment, give birth to children with an increased prevalence of congenital malformations. Design Longitudinal study. Setting Danish national birth cohort. Participants Three groups of liveborn children and their mothers: 50 897 singletons and 1366 twins born of fertile couples (time to pregnancy ≤ 12 months), 5764 singletons and 100 twins born of infertile couples who conceived naturally (time to pregnancy > 12 months), and 4588 singletons and 1690 twins born after infertility treatment. Main outcome measures Prevalence of congenital malformations determined from hospital discharge diagnoses. Results Compared with singletons born of fertile couples, singletons born of infertile couples who conceived naturally or after treatment had a higher prevalence of congenital malformations—hazard ratios 1.20 (95% confidence interval 1.07 to 1.35) and 1.39 (1.23 to 1.57). The overall prevalence of congenital malformations increased with increasing time to pregnancy. When the analysis was restricted to singletons born of infertile couples, babies born after treatment had an increased prevalence of genital organ malformations (hazard ratio 2.32, 1.24 to 4.35) compared with babies conceived naturally. No significant differences existed in the overall prevalence of congenital malformations among twins. Conclusions Hormonal treatment for infertility may be related to the occurrence of malformations of genital organs, but our results suggest that the reported increased prevalence of congenital malformations seen in singletons born after assisted reproductive technology is partly due to the underlying infertility or its determinants. The association between untreated infertility and congenital malformations warrants further examination. PMID:16893903

  6. Aneurysmal malformation of the extrahepatic portal vein: a case report

    International Nuclear Information System (INIS)

    Lee, Jeong Min; Kang, Sung Soo; Kim, Chong Soo; Han, Hyeun Young

    1999-01-01

    Aneurysmal malformation of the portal vein is a rare entity. To our knowledge, only scattered reports of portal vein aneurysms appear in the literature in English, and there is no previously published report in Korean. We describe a case exhibiting aneurysmal malformation of the extrahepatic portal vein at the hepatic hilum ; the findings demonstrated by ultrasound, CT and angiography are discussed, a review of previously described cases is included

  7. Alterations in the Contra lateral Ear in Chronic Otitis Media

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Damghani

    2013-03-01

    Full Text Available Introduction: Chronic otitis media (COM, a persistent and durable inflammation and infection of the middle ear, is a common disorder. Alterations in the contralateral ear in sufferers have been observed in recent years. Because only a few studies have been reported in this area, we performed this study in order to assess alterations in the contralateral ear of patients with COM.   Materials and Methods: Cross-sectional and descriptive methods were used in 100 patients with COM who were selected for surgical treatment and admitted to hospital. An information form was completed for all patients including demographic data, medical history of otoscopy and paraclinical examinations such as pure tone audiometry (PTA, tympanometry, Schuller radiography, and high resolution computed tomography (HRCT. All data were processed using SPSS (version 18 software and descriptive statistical tests.   Results: According to otoscopy, PTA, tympanometry and graphical analysis, 60% of patients experienced disorders of the contralateral ear. Otoscopy analysis showed 54% of patients had a disorder of the contralateral ear, with the most common disorder being perforation of the ear drum. PTA showed a 48% incidence of contralateral ear problems (85% conductive hearing impairment; 12.5% sensorineural hearing impairment; 1.2% mixed. A total of 73.2% of patients with conductive hearing loss had a problem across all frequencies, while half of the patients with sensorineural hearing impairment had problems at frequencies greater than 1000 Hz. According to tympanometry, 38% of patients had problem in the contralateral ear. HRCT and Schuller graphical analyses indicated 31.5% and 36% occurrence of contralateral ear disorders, respectively.   Conclusion:  More than 50% of patients with COM in one ear have a chance of also presenting with the disease in the other ear. Outcomes of this study and previous studies have shown that COM should not be perceived as a disease limited

  8. Angiographic treatment of the arteriovenous malformation occurred after caesarean section

    Directory of Open Access Journals (Sweden)

    Selim Büyükkurt

    2009-03-01

    Full Text Available INTRODUCTION: Uterine arteriovenous malformations are classified as acquired or congenital. Caesarean section which is performed more frequently on nowadays, uterine curettage and other uterine surgeries are the most common causes of the acquired arteriovenous malformations. CASE: Twenty-two years old woman delivered her second child by caesarean section, due to history of caesarean section, was admitted with complaint of profuse vaginal bleeding 15 days after the surgery. She demonstrated the clinical features of the hypovolemic shock. At first she treated with four units of red blood suspension, two units of fresh frozen plasma and rapid infusion of the fluids. On endometrial curettage only blood and coagulum were obtained. The bleeding could only be controlled by the internal pressure of the 18 F Foley catheter. Doppler analyze of the uterus revealed a vascular malformation signifying a turbulent flow pattern with low pressure and high flow rate. A unilateral uterine artery embolization was performed her and her menstruel cycles are resumed at the fifth month of the puerperium. DISCUSSION: The clinical presentation of the uterine arteriovenous malformations depends on the localization and the dimensions of the malformation. The uterine arteriovenous malformation should be kept in mind in cases of late occurrence vaginal bleeding unresponsive to the uterotonic medications, especially in a woman with previous history of uterine damage, such as curettage or caesarean section.

  9. Sincipital Encephaloceles: A Study of Associated Brain Malformations

    Directory of Open Access Journals (Sweden)

    Shashidhar Vedavyas Achar

    2016-01-01

    Full Text Available Objective: The aim of this study was to evaluate the associated intracranial malformations in patients with sincipital encephaloceles. Materials and Methods: A hospital-based cross-sectional study was conducted over 8 years from June 2007 to May 2015 on 28 patients. The patients were evaluated by either computed tomography or magnetic resonance imaging whichever was feasible. Encephaloceles were described with respect to their types, contents, and extensions. A note was made on the associated malformations with sincipital encephaloceles. Results: Fifty percent of the patients presented before the age of 3 years and both the sexes were affected equally. Nasofrontal encephalocele was the most common type seen in 13 patients (46.4%, and corpus callosal agenesis (12 patients was the most common associated malformation. Other malformations noted were arachnoid cyst (10 patients, hydrocephalus (7 patients, and agyria-pachygyria complex (2 patients. Conclusion: Capital Brain malformations are frequently encountered in children with sincipital encephaloceles. Detail radiological evaluation is necessary to plan treatment and also to prognosticate such rare malformations.

  10. Update on neuroimaging phenotypes of mid-hindbrain malformations

    Energy Technology Data Exchange (ETDEWEB)

    Jissendi-Tchofo, Patrice [University Hospital of Lille (CHRU), Department of Neuroradiology, MRI 3T Research, Plateforme Imagerie du vivant, IMPRT-IFR 114, Lille-Cedex (France); CHU Saint-Pierre, Radiology Department, Pediatric Neuroradiology Section, Brussels (Belgium); Severino, Mariasavina [Istituto Giannina Gaslini, Neuroradiology Unit, Genoa (Italy); Nguema-Edzang, Beatrice; Toure, Cisse; Soto Ares, Gustavo [University Hospital of Lille (CHRU), Department of Neuroradiology, MRI 3T Research, Plateforme Imagerie du vivant, IMPRT-IFR 114, Lille-Cedex (France); Barkovich, Anthony James [University of California, Neuroradiology Section, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States)

    2014-10-23

    Neuroimaging techniques including structural magnetic resonance imaging (MRI) and functional positron emission tomography (PET) are useful in categorizing various midbrain-hindbrain (MHB) malformations, both in allowing diagnosis and in helping to understand the developmental processes that were disturbed. Brain imaging phenotypes of numerous malformations are characteristic features that help in guiding the genetic testing in case of direct neuroimaging-genotype correlation or, at least, to differentiate among MHB malformations entities. The present review aims to provide the reader with an update of the use of neuroimaging applications in the fine analysis of MHB malformations, using a comprehensive, recently proposed developmental and genetic classification. We have performed an extensive systematic review of the literature, from the embryology main steps of MHB development through the malformations entities, with regard to their molecular and genetic basis, conventional MRI features, and other neuroimaging characteristics. We discuss disorders in which imaging features are distinctive and how these features reflect the structural and functional impairment of the brain. Recognition of specific MRI phenotypes, including advanced imaging features, is useful to recognize the MHB malformation entities, to suggest genetic investigations, and, eventually, to monitor the disease outcome after supportive therapies. (orig.)

  11. Sheep as a large animal ear model: Middle-ear ossicular velocities and intracochlear sound pressure.

    Science.gov (United States)

    Péus, Dominik; Dobrev, Ivo; Prochazka, Lukas; Thoele, Konrad; Dalbert, Adrian; Boss, Andreas; Newcomb, Nicolas; Probst, Rudolf; Röösli, Christof; Sim, Jae Hoon; Huber, Alexander; Pfiffner, Flurin

    2017-08-01

    Animals are frequently used for the development and testing of new hearing devices. Dimensions of the middle ear and cochlea differ significantly between humans and commonly used animals, such as rodents or cats. The sheep cochlea is anatomically more like the human cochlea in size and number of turns. This study investigated the middle-ear ossicular velocities and intracochlear sound pressure (ICSP) in sheep temporal bones, with the aim of characterizing the sheep as an experimental model for implantable hearing devices. Measurements were made on fresh sheep temporal bones. Velocity responses of the middle ear ossicles at the umbo, long process of the incus and stapes footplate were measured in the frequency range of 0.25-8 kHz using a laser Doppler vibrometer system. Results were normalized by the corresponding sound pressure level in the external ear canal (P EC ). Sequentially, ICSPs at the scala vestibuli and tympani were then recorded with custom MEMS-based hydrophones, while presenting identical acoustic stimuli. The sheep middle ear transmitted most effectively around 4.8 kHz, with a maximum stapes velocity of 0.2 mm/s/Pa. At the same frequency, the ICSP measurements in the scala vestibuli and tympani showed the maximum gain relative to the P EC (24 dB and 5 dB, respectively). The greatest pressure difference across the cochlear partition occurred between 4 and 6 kHz. A comparison between the results of this study and human reference data showed middle-ear resonance and best cochlear sensitivity at higher frequencies in sheep. In summary, sheep can be an appropriate large animal model for research and development of implantable hearing devices. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. The presentation and management of complex female genital malformations.

    Science.gov (United States)

    Acién, Pedro; Acién, Maribel

    2016-01-01

    Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Müllerian malformations are particularly important because they cause serious clinical symptoms and affect woman's quality of life, in addition to creating fertility problems. In these cases of complex female genital tract malformations, a correct diagnosis is essential to avoid inappropriate and/or unnecessary surgery. Therefore, acquiring and applying the appropriate embryological knowledge, management and therapy is a challenge for gynaecologists. Here, we considered complex malformations to be obstructive anomalies and/or those associated with cloacal and urogenital sinus anomalies, urinary and/or extragenital anomalies, or other clinical implications or symptoms creating a difficult differential diagnosis. A diligent and comprehensive search of PubMed and Scopus was performed for all studies published from 1 January 2011 to 15 April 2015 (then updated up to September 2015) using the following search terms: 'management' in combination with either 'female genital malformations' or 'female genital tract anomalies' or 'Müllerian anomalies'. The MeSH terms 'renal agenesis', 'hydrocolpos', 'obstructed hemivagina' 'cervicovaginal agenesis or atresia', 'vaginal agenesis or atresia', 'Herlyn-Werner-Wunderlich syndrome', 'uterine duplication' and 'cloacal anomalies' were also used to compile a list of all publications containing these terms since 2011. The basic embryological considerations for understanding female genitourinary malformations were also revealed. Based on our experience and the updated literature review, we studied the definition and classification of the complex malformations, and we analysed the clinical presentation and different therapeutic strategies for each anomaly, including the embryological and clinical classification of female genitourinary malformations. From 755 search retrieved references, 230 articles were analysed and

  13. Neuroimaging of malformation of cortical development

    International Nuclear Information System (INIS)

    Zlatareva, D.; Hadjidekov, V.; Tournev, I.; Rossi, A.

    2012-01-01

    Malformations of cortical development (MCD) are heterogeneous group of disease which result from disruption of 3 main stages of cortical development.The common clinical presentation is refractory epilepsy and or developmental delay. The aim of this paper is to describe and analyze magnetic resonance (MR) findings and to present protocol for examination. We analyze MR findings in 17 patients with MCD. The average age was 12,1 year (from 2 months - 57 years). The main indications from reference physician are epilepsy and developmental delay. In 12 patients 1.5T MR was performed, and in 5 - 0.5T. Subependymal heterotopias was found in 6 patients, focal cortical dysplasia - 3. polymicrogyria - 3, schizencephaly - 2, hemimegalencephaly -1, lizencephaly -1, tuberous sclerosis -1. The most common MCD are heterotopias, focal cortical dysplasia, polymicrogyria. schizencephaiy, pachygyria and lizencephaly. In our study the number of patients is not big enough to make a conclusion about frequency of the forms of MCD and our goal is to analyze MR findings which are not well studied in our country. MRI is the method of choice for diagnosis of MCD. The protocol should be different from routine brain protocol to interpret the images with good quality and not miss the pathology. Knowledge of MR findings in MCD would help for genetic counselling in some cases or can predict prognosis in some patients. (authors)

  14. Endovascular treatment of intracranial arteriovenous malformations

    International Nuclear Information System (INIS)

    Seruga, T.

    2002-01-01

    Background. The aim of the study was the introduction of endovascular interventional treatment of cerebral arteriovenous malformations (AVM) with superselective embolization with cyanoacrylic polymerisation agent. Case reports. Endovascular embolization was performed in five patients with cerebral AVMs. Three of these patients were presented with intracerebral haemathomas whereas in other two patients, cerebral AVM was an incidental finding. Superselective catheterisation of AVMs was performed and acrylic glue was selectively injected into the nidus. Conclusions. Control cerebral angiography after embolization of AVM showed different results. In one patient, AVM was totally occluded after three sessions and in second case AVM was occluded in a single session. The rate of occlusion in other two cases was estimated between 70% in 80%. Both of these two patients underwent surgery. One patient is still in the process of treatment. Endovascular treatment of cerebral AVMs with superselective embolization with liquid cyanoacrilyc adhesive agent is a safe and effective alternative treatment paths next to microsurgery. Endovascular treatment in combination with radiosurgery could become the method of choice in the therapy of cerebral AVMs in the future. (author)

  15. Sports participation with Chiari I malformation.

    Science.gov (United States)

    Strahle, Jennifer; Geh, Ndi; Selzer, Béla J; Bower, Regina; Himedan, Mai; Strahle, MaryKathryn; Wetjen, Nicholas M; Muraszko, Karin M; Garton, Hugh J L; Maher, Cormac O

    2016-04-01

    OBJECT There is currently no consensus on the safety of sports participation for patients with Chiari I malformation (CM-I). The authors' goal was to define the risk of sports participation for children with the imaging finding of CM-I. METHODS A prospective survey was administered to 503 CM-I patients at 2 sites over a 46-month period. Data were gathered on imaging characteristics, treatment, sports participation, and any sport-related injuries. Additionally, 81 patients completed at least 1 subsequent survey following their initial entry into the registry and were included in a prospective group, with a mean prospective follow-up period of 11 months. RESULTS Of the 503 CM-I patients, 328 participated in sports for a cumulative duration of 4641 seasons; 205 of these patients participated in contact sports. There were no serious or catastrophic neurological injuries. One patient had temporary extremity paresthesias that resolved within hours, and this was not definitely considered to be related to the CM-I. In the prospective cohort, there were no permanent neurological injuries. CONCLUSIONS No permanent or catastrophic neurological injuries were observed in CM-I patients participating in athletic activities. The authors believe that the risk of such injuries is low and that, in most cases, sports participation by children with CM-I is safe.

  16. Transcatheter coil embolisation of pulmonary arteriovenous malformations

    International Nuclear Information System (INIS)

    Wingen, M.; Guenther, R.W.

    2001-01-01

    Evaluation of technical success, complications and long-term results of transcatheter coil embolisation in pulmonary arteriovenous malformations (pAVMs). Material and Methods: Transcatheter embolisations of 46 pAVMs in 14 patients were analysed retrospectively, and, 5 years after treatment, the patients were interviewed by telefone concerning persistent symptoms and complications. Main symptoms before embolisation were dyspnoe (86%), hypoxaemia (100%), cerebral ischemia (21%), and hemoptysis (14%); 11 patients (79%) suffered from hereditary hemorrhagic telangiectasia. Results: Embolisation with an average of 3,9 coils (min. 1, max. 19 coils) per pAVM yielded technical success in all cases. Only two minor complications, transitory pleuritis and a small lung infarction were observed. On follow up examination after 5 years either no residual complaints or substantial improvement of dyspnoe were reported; no patient suffered from neurologic or hemorrhagic complications after the embolisation. Conclusion: Transcatheter embolisation is a safe and minimally invasive therapy for pAVMs and has rightfully replaced surgical resection as the therapy of choice. (orig.) [de

  17. Pulmonary arteriovenous malformation in cryptogenic liver

    International Nuclear Information System (INIS)

    Afzal, N.

    2013-01-01

    The cause in 10 - 20% cases of liver cirrhosis (LC) cannot be elucidated, and are thus termed cryptogenic. Pulmonary arteriovenous malformations (PAVMs) are relatively rare, but the most common anomaly involving the pulmonary tree. Although the rare correlation between LC and PAVM is well-known, there have been no reports of PAVMs occurring in cryptogenic LC. We report a case of PAVM that occurred in cryptogenic liver cirrhosis in a 3 years old male child. The child presented with complaints of malena, hematemesis and variceal bleed. The examination revealed a child with respiratory distress, irritability, tachycardia, clubbing and abdominal distention. He was worked up for recurrent variceal bleeding secondary to portal hypertension but the oxygen saturation during hospital stay kept deteriorating. The diagnosis of hepatopulmonary syndrome as the cause of persistent hypoxemia in the absence of other cardio-pulmonary causes was then made by enhanced echocardiogram using agitated saline. He improved significantly after liver transplantation performed abroad. At a 6 months follow-up, the child was stable with no evidence of intrapulmonary shunting on repeat echo. (author)

  18. Cerebellar malformations alter regional cerebral development.

    Science.gov (United States)

    Bolduc, Marie-Eve; Du Plessis, Adre J; Evans, Alan; Guizard, Nicolas; Zhang, Xun; Robertson, Richard L; Limperopoulos, Catherine

    2011-12-01

    The aim of this study was to compare total and regional cerebral volumes in children with isolated cerebellar malformations (CBMs) with those in typically developing children, and to examine the extent to which cerebellar volumetric reductions are associated with total and regional cerebral volumes. This is a case-control study of children diagnosed with isolated CBMs. Each child was matched on age and sex to two typically developing children. Using advanced three-dimensional volumetric magnetic resonance imaging, the cerebrum was segmented into tissue classes and partitioned into eight regions. Analysis of variance was used to compare cerebral volumes between children with CBMs and control children, and linear regressions to examine the impact of cerebellar volume reduction on cerebral volumes. Magnetic resonance imaging was performed at a mean age of 27 months in 20 children (10 males, 10 females) with CBMs and 40 typically developing children. Children with CBMs showed significantly smaller deep grey matter nuclei (p developing children. Greater cerebellar volumetric reduction in children with CBMs was associated with decreased total cerebral volume and deep grey matter nuclei (p = 0.02), subgenual white/grey matter (p = 0.001), midtemporal white (p = 0.02) and grey matter (p = 0.01), and parieto-occipital grey matter (p = 0.004). CBMs are associated with impaired regional cerebral growth, suggesting deactivation of principal cerebello-cerebral pathways. © The Authors. Developmental Medicine & Child Neurology © 2011 Mac Keith Press.

  19. Arteriovenous malformations of the cervical spinal cord

    International Nuclear Information System (INIS)

    Nagasawa, Shiro; Yoshida, Shinzo; Ishikawa, Masatsune; Yonekawa, Yasuhiro; Handa, Hajime

    1984-01-01

    Arteriovenous malformation (AVM) of the cervical spinal cord has been known to constitute 5-13% of all spinal AVMs. In contrast to the AVMs located in thoracic or thoraco-lumbar regions, cervical AVM has several characteristic features such as preponderance in younger generation, high incidence of subarachnoid hemorrhage, intramedullary location of the nidus usually fed by the anterior spinal arterial system. We reported three cases of cervical AVMs, which located intramedullary at the levels of C 4 -C 6 , C 1 -C 4 and C 1 -C 2 , respectively. Although selective angiography (vertebral artery, thyrocervical artery, costocervical artery) was essential for the diagnosis of these lesions, computerized tomographic (CT) study with both intrathecal injection of metrizamide and intravenous infusion of contrast material (dynamic and static study) was found to be extremely advantageous in detecting the topography of AVMs in the concerned horizontal planes of the spinal cord. Removal of AVM was given up in one case because of its possible involvement of the anterior spinal artery and central artery shown by CT scan. Removal of AVMs were performed in other two cases. A lateral approach was tried in one case with the AVM located in C 1 -C 2 level, in which CT scan revealed not only an intramedullary but the associated extramedullary AVM in ventrolateral surface of the spinal cord. This operative approach was found to involve less bone removal and markedly reduce spinal cord manipulation necessary to deal with ventrally situated high cervical lesions, compared with a posterior approach with laminectomy. (author)

  20. Ethanol embolization of auricular arteriovenous malformations

    International Nuclear Information System (INIS)

    Fan Xindong; Zheng Lianzhou; Yi Hongying; Su Lixin; Zheng Jiawei

    2009-01-01

    Objective: To present the authors' initial experience of treating auricular arteriovenous malformations(AVMs) with ethanol embolization and to assess the clinical effectiveness of this therapeutic method. Methods: Twenty-two patients with AVMs were enrolled in this study. Through local puncturing or super-selective catheterization the absolute ethanol,or diluted alcohol (based on the pattern of the AVMs), was manually injected into the abnormal vascular plexus of the auricular lesion. The clinical results were estimated with physical examination or angiography at intervals of 3-4 month, and telephone questionnaire was made at monthly intervals for all patients. Results: Thirty-eight ethanol embolization procedures were performed, the amount of ethanol used during the procedure ranged from 4 ml to 65 ml. After the treatment the clinical symptoms were improved, which were manifested as healing of the ulceration, stop of bleeding, disappearing or alleviation of tinnitus. Angiographic examination showed that the abnormal vascular lesion was completely vanished in 9 cases, decreased by 50%-75% in 8 cases and decreased less than 50% in remaining 5 cases. The common complications included irreversible local necrosis and vesiculation. Conclusion: For the treatment of auricular AVMs ethanol embolization is an effective and safe method,which might become the therapy of first choice. (authors)

  1. Ethanol embolization of auricular arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Xindong, Fan; Lianzhou, Zheng [Department of Interventional Radiology, the Ninth People' s Hospital, School of Medicine, Shanghai Jiaotong Univ., Shanghai (China); Hongying, Yi; Lixin, Su; Jiawei, Zheng

    2009-11-15

    Objective: To present the authors' initial experience of treating auricular arteriovenous malformations(AVMs) with ethanol embolization and to assess the clinical effectiveness of this therapeutic method. Methods: Twenty-two patients with AVMs were enrolled in this study. Through local puncturing or super-selective catheterization the absolute ethanol,or diluted alcohol (based on the pattern of the AVMs), was manually injected into the abnormal vascular plexus of the auricular lesion. The clinical results were estimated with physical examination or angiography at intervals of 3-4 month, and telephone questionnaire was made at monthly intervals for all patients. Results: Thirty-eight ethanol embolization procedures were performed, the amount of ethanol used during the procedure ranged from 4 ml to 65 ml. After the treatment the clinical symptoms were improved, which were manifested as healing of the ulceration, stop of bleeding, disappearing or alleviation of tinnitus. Angiographic examination showed that the abnormal vascular lesion was completely vanished in 9 cases, decreased by 50%-75% in 8 cases and decreased less than 50% in remaining 5 cases. The common complications included irreversible local necrosis and vesiculation. Conclusion: For the treatment of auricular AVMs ethanol embolization is an effective and safe method,which might become the therapy of first choice. (authors)

  2. Imaging of arteriovenous malformation following stereotactic radiosurgery

    International Nuclear Information System (INIS)

    Tranchida, J.V.; Mehall, C.J.; Slovis, T.L.; Lis-Planells, M.

    1997-01-01

    Background. Stereotactic radiosurgery allows for a high dose of focused radiation to be delivered to a small lesion such as an arteriovenous malformation (AVM). The clinical change and brain response over time to this localized high-dose radiation can be quite striking. Objective. The objective of this study to describe and analyse the imaging changes following radiotherapy for AVMs. Materials and methods. The clinical presentation and the imaging changes following radiotherapy in two patients were studied over the course of 1-2 years. Results. The imaging findings include diffuse low attenuation and contrast enhancement on CT. High-signal lesions were apparent on T2-weighted MR images with prominent contrast enhancement on T1-weighted images. Ring enhancement occurred over time. While new changes appeared over 12 months, these changes diminished during the second year. Conclusion. Radiotherapy induces inflammatory changes that are generally reversible but can lead to parenchymal destruction. These imaging changes are often nonspecific and therefore must be interpreted in light of clinical symptomatology and the time course since treatment. These patients should receive routine MR imaging within 3 months after radiosurgery with follow-up imaging at 6, 12, and 18 months. (orig.). With 8 figs

  3. Recurrent Streptococcus Pneumoniae Meningitis in a Child with Split Hand and Foot Malformation and Undiagnosed Mondini Dysplasia.

    Science.gov (United States)

    Katarzyna, Mazur-Melewska; Jarosław, Szydłowski; Katarzyna, Jończyk-Potoczna; Wojciech, Służewski; Magdalena, Figlerowicz

    Recurrent bacterial meningitis is a life-threatening infection of the central nervous system that is mostly connected with anatomical abnormalities of the skull, chronic parameningeal infections and immunodeficiencies. It's rarely seen, but when it occurs an extensive investigation should be carried out to discover the responsible factor, so that further episodes can be prevented. We report on a child with split hand and foot (SHFM), confirmed incorrect karyotype 46, XY, t(7:12)(q21.2;q21.3) and a fourth episode of fulminant meningitis caused by penicillin-resistant Streptococcus pneumoniae . After a broad evaluation of factors predisposing to recurrent meningitis, the undiagnosed malformation of his inner and middle ears - Mondini dysplasia was found. We suggest examining all children with SHFM for hearing impairment before they develop recurrent meningitis. The time when the radiological procedure for searching for inner ear dysplasia should be performed could be a controversial issue: before or after the first episode of meningitis. From the epidemiological point of view, high-resolution computer tomography scanning of the temporal bones should be considered after the diagnosis of deafness in a child with SHFM related to 7q21 deletion.

  4. Detecting congenital malformations - Lessons learned from the Mpepu study, Botswana.

    Directory of Open Access Journals (Sweden)

    Gbolahan Ajibola

    Full Text Available A large and increasing number of HIV-infected women are conceiving on antiretroviral treatment (ART. While most antiretrovirals are considered safe in pregnancy, monitoring for rare pregnancy and infant adverse outcomes is warranted.We conducted a retrospective secondary analysis nested within a clinical trial of infant cotrimoxazole vs. placebo prophylaxis in Botswana (the Mpepu Study. Infants were examined at birth, and at least every 3 months through 18 months of age. Abnormal physical findings and diagnostic testing revealing malformations were documented. Post hoc, a geneticist classified all reported malformations based on available documentation. Structural malformations with surgical, medical or cosmetic importance were classified as major malformations. We present a descriptive analysis of identified malformations.Between 2011 and 2014, 2,933 HIV-infected women who enrolled in the Mpepu study delivered 2,971 live-born infants. Study staff conducted 2,944 (99% newborn exams. One thousand eighty-eight (38% women were taking ART at conception; 1,147 (40% started ART during pregnancy; 442 (15% received zidovudine monotherapy; and 223 (7% received no antiretroviral during pregnancy. Of 33 reported anomalies, 25 (76% met congenital malformations criteria, 10 (30% were classified as major malformations, 4 (40% of which were identified after the birth exam.Our results highlight the importance of staff training on identification of congenital malformations, programmatic monitoring beyond the birth examination and the value of geneticist involvement in the malformations classification process in resource-limited settings. These elements will be important to fully define antiretroviral drug safety in pregnancy.Surveillance systems for monitoring the safety of antiretroviral use during pregnancy among HIV-infected women in resource-limited setting are lacking. The World Health Organization's published programmatic recommendations for such

  5. Presbycusis: do we have a third ear?

    Science.gov (United States)

    Reis, Luis Roque; Escada, Pedro

    Age-related hearing changes are the most frequent cause of sensorineural hearing loss in adults. In the literature no studies exist concerning the importance of speechreading in individuals with presbycusis. Equally, no such studies have been carried out with speakers of the Portuguese (Portugal) language. To evaluate whether the intelligibility of words in presbycusis is improved by speechreading, in such a way that looking at the interlocutor's face while he is talking functions like a "third ear", and to determine the statistical relevance of the intelligibility improvement by speechreading. Eleven individuals (22 ears) with bilateral and symmetrical sensorineural hearing loss compatible with presbycusis were evaluated. The subjects were aged between 57 and 82 years, with an average of 70±11.51 years and median of 69.5 years. A complete medical and audiological profile of each patient was created and all patients were submitted to a vocal audiogram, without and with observation of the audiologist's face. A descriptive and analytical statistical analysis was performed (Shapiro-Wilk and t pairs tests) adopting the significance level of 0.05 (5%). We noticed better performance in intelligibility with speechreading. The p-value was zero (ppresbycusis in this study, performed better on spoken word intelligibility when the hearing of those words was associated with speechreading. This phenomenon helps in such a way that observation of the interlocutor's face works like a "third ear". Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  6. Analytical model of internally coupled ears

    DEFF Research Database (Denmark)

    Vossen, Christine; Christensen-Dalsgaard, Jakob; Leo van Hemmen, J

    2010-01-01

    Lizards and many birds possess a specialized hearing mechanism: internally coupled ears where the tympanic membranes connect through a large mouth cavity so that the vibrations of the tympanic membranes influence each other. This coupling enhances the phase differences and creates amplitude...... additionally provides the opportunity to incorporate the effect of the asymmetrically attached columella, which leads to the activation of higher membrane vibration modes. Incorporating this effect, the analytical model can explain measurements taken from the tympanic membrane of a living lizard, for example...

  7. Ear replanatation: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Krešimir Božikov

    2013-10-01

    Full Text Available Background: Total ear amputation is a rare accident. The most common causes are traffic accidents (33 %, followed by fights (28 %. In 1980, Pennington et al. reported the first successful microsurgical ear replantation in a 29-year old man.Methods: An English literature review of trauma cases of total ear amputation showed only 13 successful replantations with arterial and venous microanastomoses. We present a case report of successful total ear replantation with arterial and vein microanastomoses in a 17-year old boy.Results: Our ear replantation with both arterial and venous anastomoses performed was successful and we achieved an excellent aesthetic outcome.Conclusion: The reason for such a low number of successful ear replantations is technical challenge due to small vessel diameter, difficult vessel identification, vessel approach and concomitant avulsion injury. The best aesthetic result in ear reconstruction is achieved by microsurgical replantation. The surgical technique depends on the intraoperative findings. Since ear replantation is a very challenging procedure, a microsurgeon needs to discuss with the patient the risk of partial/total necrosis of the replanted ear and the possibilities of other reconstructive options.

  8. Incorporating anthropometry into design of ear-related products.

    Science.gov (United States)

    Liu, Bor-Shong

    2008-01-01

    To achieve mass customization and collaborative product design, human factors and ergonomics should play a key development role. The purpose of this study was to provide product designers with the anthropometic dimensions of outer ears for different demographic data, including gender and age. The second purpose was to compare the dimensions of various ear-related products (i.e., earphone, bluetooth earphone and ear-cup earphone) with the anthropometic database and recommend appropriate solutions for design. Two hundred subjects aged 20-59 was selected for this study and divided into four age stratifications. Further, three different dimensions of the outer ear (i.e., the earhole length, the ear connection length and the length of the pinna) were measured by superimposed grid photographic technique. The analysis of variance (ANOVA) was used to investigate the effects of gender, and age on ear dimensions. The results showed that all ear dimensions had significant gender effects. A comparison between the anthropometric dimensions and those of current products revealed that most current ear-related products need to be redesigned using anthropometric data. The shapes of earhole and pinna are not circular. Consequently, ear products need to be elongated so that users may feel more comfortably and not have the product slip off easily.

  9. Histology and synchrotron radiation-based microtomography of the inner ear in a molecularly confirmed case of CHARGE syndrome.

    Science.gov (United States)

    Glueckert, Rudolf; Rask-Andersen, Helge; Sergi, Consolato; Schmutzhard, Joachim; Mueller, Bert; Beckmann, Felix; Rittinger, Olaf; Hoefsloot, Lies H; Schrott-Fischer, Anneliese; Janecke, Andreas R

    2010-03-01

    CHARGE (Coloboma of the iris or retina, heart defects, atresia of the choanae, retardation of growth and/or development, genital anomalies, ear anomalies) syndrome (OMIM #214800) affects about 1 in 10,000 children and is most often caused by chromodomain helicase DNA-binding protein-7 (CHD7) mutations. Inner ear defects and vestibular abnormalities are particularly common. Specifically, semicircular canal (SCC) hypoplasia/aplasia and the presence of a Mondini malformation can be considered pathognomonic in the context of congenital malformations of the CHARGE syndrome. We obtained a temporal bone (TB) of a patient with CHARGE syndrome who died from bacteremia at 3 months of age. The clinical diagnosis was confirmed in the patient by direct DNA sequencing and the detection of a de novo, truncating CHD7 mutation, c.6169dup (p.R2057fs). We assessed changes of the TB and the degree of neural preservation, which may influence the potential benefit of cochlear implantation. The TB was analyzed using synchrotron radiation-based micro computed tomography, and by light microscopy. The vestibular partition consisted of a rudimentary vestibule with agenesis of the SCCs. The cochlea was hypoplastic with poor or deficient interscaling and shortened (Mondini dysplasia). The organ of Corti had near normal structure and innervation. Modiolus and Rosenthal's canal were hypoplastic with perikarya displaced along the axon bundles into the internal acoustic meatus, which may be explained by the arrest or limited migration and translocation of the cell nuclei into the cochlear tube during development. (c) 2010 Wiley-Liss, Inc.

  10. Ear-to-Ear On-Body Channel Model for Hearing Aid Applications

    DEFF Research Database (Denmark)

    Kvist, Søren Helstrup; Thaysen, Jesper; Jakobsen, Kaj Bjarne

    2015-01-01

    that the head is mod- eled more accurately, and the radiation pattern is sampled in more points. The model is able to take the on-body radiation pattern of the antenna, as well as arbitrary he ad contours into account. The model is validated by the use of measurements and Ansys HFSS simulations on the specific......The deterministic ear-to-ear on-body channel is modeled by the use of a number of elliptically shaped paths. The semi-major axes of the elliptica lly shaped paths are adjusted such that they trace the outline of the head. The path gain converges when the number of paths is increased, su ch...... anthropomorphic mannequin (SAM) head. The model is used with a g enetic algorithm in order to synthesize a radiation pattern that is optimal for use with the ear-to-ear on-body channel. The radiation pattern is synthesized in terms of the spherical wave expansion coefficients of the hypothetical small antenna...

  11. Pattern of Gross Congenital Malformations in a Tertiary Referral Hospital in Northeast India.

    Science.gov (United States)

    Baruah, Jenita; Kusre, Giriraj; Bora, Reeta

    2015-10-01

    To explore the prevalence of structural congenital malformations among newborns and study some of the fetal and maternal characteristics of the malformed babies in North east India where such systematic study on congenital malformation has not been undertaken before. A cross sectional study was undertaken from May 2010 through Feb 2013 for estimation of gross congenital malformations among live birth and stillbirth children born in Assam Medical College. All live births were clinically examined for detection of gross congenital malformations and autopsy was carried out on still births and neonates dying within 24 h of birth to detect gross congenital malformations in the internal organs. All malformations were classified as per ICD 10 classification. The mothers of the newborns with congenital malformations were interviewed in a predesigned, pretested proforma. The variables included maternal age, antenatal registration, antenatal history of drug intake, consanguinity and previous history of malformations. Statistical analysis was done using chi square test. A total 18,192 births including live births and still births were examined and 206 cases of structural malformations were observed. Prevalence of congenital malformations was 1.2 % of the total live births. Distribution of malformation was predominant among males than in females (60.67 vs. 37.37 %; p malformation of the cardiovascular system was found to be very low. Malformations among stillbirths and newborns born to unregistered mothers were significantly more. Percentage of malformations in babies born to mothers of more than 30 y of age was higher than other age groups (2.2 %). Occurrence of malformations in low birth weight babies were significantly more (p malformations was 1.2 % of the total live births. Musculoskeletal system was the most common system involved. Congenital malformations were significantly associated with sex of the new born, registration of the mother and birth weight of the

  12. Monitoring congenital malformation among inhabitants of town

    International Nuclear Information System (INIS)

    Pawel, I.

    2004-01-01

    In Russia rendering of medical care of inhabitants of town located not far from works of nuclear industries be provided for system of special referral centers. Now the congenital malformation (CM) is one of the most issue of the day unresolved problem protection of genetic health of populations. CM account weighty part of structure incidence nursery every where. The most of CM lead to developmental disability, substantively restrict to life span and fertility. for the present moment the treatment CM developed for isolated instances therefore special prophylaxis to take on special significance. The one way to prophylaxis is simultaneous monitoring of CM and chief factors of disutility. In the framework of the State system of monitoring of CM our research laboratory of the State Research Centre Institute of Biophysics to Make a reality monitoring CM in the families of personnel of units of the atomic industry. From 2000 and during the present moment we are logged data about 21 a species of CM. In any case monstriparity with one of these CM in the families of workers of the atomic industry we investigated this case. Pro hac vice we are logged data about professional contacts parents this child with any factors of professional disutility including ionizing radiation. During 2002 we was obtained reliable information from 13 special referral centers about 33 case of birth of baby with CM. It's average about 1/1000 from all case of birth. From this case only 12 babies with CM was birth in the families of personnel of the atomic industry. (Author)

  13. Open resections for congenital lung malformations

    Directory of Open Access Journals (Sweden)

    Mullassery Dhanya

    2008-01-01

    Full Text Available Aim: Pediatric lung resection is a relatively uncommon procedure that is usually performed for congenital lesions. In recent years, thoracoscopic resection has become increasingly popular, particularly for small peripheral lesions. The aim of this study was to review our experience with traditional open lung resection in order to evaluate the existing "gold standard." Materials and Methods: We carried out a retrospective analysis of all children having lung resection for congenital lesions at our institution between 1997 and 2004. Data were collected from analysis of case notes, operative records and clinical consultation. The mean follow-up was 37.95 months. The data were analyzed using SPSS. Results: Forty-one children (13 F/28 M underwent major lung resections during the study period. Their median age was 4.66 months (1 day-9 years. The resected lesions included 21 congenital cystic adenomatoid malformations, 14 congenital lobar emphysema, four sequestrations and one bronchogenic cyst. Fifty percent of the lesions were diagnosed antenatally. Twenty-six patients had a complete lobectomy while 15 patients had parenchymal sparing resection of the lesion alone. Mean postoperative stay was 5.7 days. There have been no complications in any of the patients. All patients are currently alive, asymptomatic and well. None of the patients have any significant chest deformity. Conclusions: We conclude that open lung resection enables parenchymal sparing surgery, is versatile, has few complications and produces very good long-term results. It remains the "gold standard" against which minimally invasive techniques may be judged.

  14. Management of Orbital and Periorbital Venous Malformation

    Directory of Open Access Journals (Sweden)

    Lara A. Benoiton

    2017-05-01

    Full Text Available BackgroundTo review our management of common venous malformation (VM affecting the orbit and/or periorbital area.MethodsConsecutive patients with orbital and/or periorbital VM were identified from our vascular anomalies database. Demographic details of the patients, anatomic site(s affected, symptoms and signs, presence of a family history of VM, and types of treatment(s were collected, supplemented by chart review.ResultsA total of 24 patients’ age 1–68 (mean, 30 years with orbital and/or periorbital VM presented with cosmetic concerns (n = 17, 71%, distensibility (n = 15, 63%, pain (n = 9, 38%, diplopia (n = 4, 17%, and spontaneous thrombosis (n = 1, 8%. The VM caused globe dystopia (n = 13, 54%, enophthalmos (n = 6, 25%, proptosis (n = 3, 12%, exotropia (n = 3, 12%, and pseudoptosis with visual obstruction (n = 3, 13%. A total of 11 (46% patients were managed conservatively. 13 (54% patients underwent active treatment. Ethanol sclerotherapy (ES was performed in six patients with extensive facial VM associated with orbital/periorbital involvement, resulting in symptomatic improvement in five patients, one of whom developed skin necrosis and another patient developed reduced infraorbital nerve sensation. Surgery was performed for localized lesion (n = 3, 23%, for extensive lesions (n = 4, 31% and as an adjunct to ES (n = 6, 46% resulting in symptomatic improvement in all patients. One patient required correction of lower lid ectropion.ConclusionOrbital and/or periorbital VMs are heterogeneous, and management needs to be individualized. Surgery is used for localized lesions aiming for complete excision, as a debulking procedure for extensive orbital/periorbital VM when ES was not possible, or following ES for extensive facial VM with orbital and/or periorbital involvement.

  15. Induced thermal resistance in the mouse ear

    International Nuclear Information System (INIS)

    Law, M.P.; Coultas, P.G.; Field, S.B.

    1979-01-01

    The mouse ear (pinna) was used to investigate the effect of two hyperthermic treatments. Heating was by immersion in hot water at 43.5 0 C. A single treatment of about 50 minutes was required to cause necrosis in 50% of the ears treated. When heat treatment was given in two equal fractions the total heating time had to be increased if the interval between fractions was greater than four hours. By 24 hours a total treatment of about 100 minutes was required, indicating almost complete recovery from the first heating. Priming treatments at 43.5 0 C induced thermal resistance to a second heat treatment at 43.5 0 C. Maximum resistance was observed one day after a 20 minute priming and two days after a 40 minute priming, when the heating time had to be increased to 120 minutes, an increase by a factor of 2.4. Shorter priming treatments induced less resistance, the minimum heating time to produce an effect being two minutes. In all cases the effect decreased during the next four to five days. These results indicate that the reduced response of tissues to fractionated hyperthermia is due both to the repair of sublethal heat damage and induction of thermal resistance. (author)

  16. Middle ear function in sinonasal polyposis.

    Science.gov (United States)

    Bakhshaee, Mehdi; Ardakani, Hossein Payedar; Ghazizadeh, Amir Hossain; Movahed, Rahman; Jarahi, Lida; Rajati, Mohsen

    2016-10-01

    Nasal airway patency has long been considered a major factor in ear health. The aim of this study was to determine the effect of sinonasal polyposis on middle ear and eustachian tube (ET) functionality. Forty-four individuals with polyposis, 23 with non-polyposis nasal obstruction, and 23 healthy controls were enrolled. Demographic, clinical and imaging data of all participants were collected and ET function tests and audiologic tests were performed. Hearing loss (p = 0.02), flat tympanogram (p = 0.02), disturbed Toynbee and Valsalva tests (p = 0.01), and the prevalence of allergy (p = 0.04) and purulent nasal discharge (p polyposis group than the other groups. Regression analysis revealed that infection and allergy have more important roles in ET function than the nasal obstruction. Polyposis could impede ET function; however, it is probably not because of its obstructive nature, but because of the associated increased risk of infection.

  17. Wearable ear EEG for brain interfacing

    Science.gov (United States)

    Schroeder, Eric D.; Walker, Nicholas; Danko, Amanda S.

    2017-02-01

    Brain-computer interfaces (BCIs) measuring electrical activity via electroencephalogram (EEG) have evolved beyond clinical applications to become wireless consumer products. Typically marketed for meditation and neu- rotherapy, these devices are limited in scope and currently too obtrusive to be a ubiquitous wearable. Stemming from recent advancements made in hearing aid technology, wearables have been shrinking to the point that the necessary sensors, circuitry, and batteries can be fit into a small in-ear wearable device. In this work, an ear-EEG device is created with a novel system for artifact removal and signal interpretation. The small, compact, cost-effective, and discreet device is demonstrated against existing consumer electronics in this space for its signal quality, comfort, and usability. A custom mobile application is developed to process raw EEG from each device and display interpreted data to the user. Artifact removal and signal classification is accomplished via a combination of support matrix machines (SMMs) and soft thresholding of relevant statistical properties.

  18. Enhanced visualization of inner ear structures

    Science.gov (United States)

    Niemczyk, Kazimierz; Kucharski, Tomasz; Kujawinska, Malgorzata; Bruzgielewicz, Antoni

    2004-07-01

    Recently surgery requires extensive support from imaging technologies in order to increase effectiveness and safety of operations. One of important tasks is to enhance visualisation of quasi-phase (transparent) 3d structures. Those structures are characterized by very low contrast. It makes differentiation of tissues in field of view very difficult. For that reason the surgeon may be extremly uncertain during operation. This problem is connected with supporting operations of inner ear during which physician has to perform cuts at specific places of quasi-transparent velums. Conventionally during such operations medical doctor views the operating field through stereoscopic microscope. In the paper we propose a 3D visualisation system based on Helmet Mounted Display. Two CCD cameras placed at the output of microscope perform acquisition of stereo pairs of images. The images are processed in real-time with the goal of enhancement of quasi-phased structures. The main task is to create algorithm that is not sensitive to changes in intensity distribution. The disadvantages of existing algorithms is their lack of adaptation to occuring reflexes and shadows in field of view. The processed images from both left and right channels are overlaid on the actual images exported and displayed at LCD's of Helmet Mounted Display. A physician observes by HMD (Helmet Mounted Display) a stereoscopic operating scene with indication of the places of special interest. The authors present the hardware ,procedures applied and initial results of inner ear structure visualisation. Several problems connected with processing of stereo-pair images are discussed.

  19. Inflammatory diseases of the middle ear

    International Nuclear Information System (INIS)

    Boasquevisque, Gustavo Santos; Andrade, Carlos Ramon de; Boasquevisque, Edson Mendes

    2008-01-01

    Objective: to determine the aspects and frequency of middle ear alterations at computed tomography and magnetic resonance imaging in patients with clinical suspicion for middle ear inflammatory disease. Material and method: imaging examination results of 95 patients (95 computed tomography and 1 magnetic resonance imaging) were compared with the results of otology, imaging and surgical findings. Results: fifty-two patients had normal imaging results. Forty-three patients had radiologic alterations compared to the physical, otology and histopathologic exams: acute otomastoiditis in 2 patients (4.6%), unilateral chronic otomastoiditis in 9 (21.0%), bilateral chronic otomastoiditis in 2 (4.6%), unilateral cholesteatoma in 26 (60.5%), and bilateral cholesteatoma in 4 (9.3%). Conclusion: computed tomography was able to demonstrate the characteristics of the lesions and found complications in majority of the cases. Magnetic resonance imaging was used only in one patient to rule out scar and cholesteatoma. The knowledge of the clinical and otologic data enables the radiologist to elaborate a more appropriate interpretation of the imaging findings. (author)

  20. Genetic causes of congenital brain malformations in epilepsy patients

    DEFF Research Database (Denmark)

    Møller, Rikke Steensbjerre

    2008-01-01

    The search for genetic causes of congenital brain malformations, severe epilepsy and mental retardation plays an important role in neuropediatrics and neurology. Disclosure of the aetiology of the intellectual disabilities, seizures and the underlying brain malformation may be of psychological va...... genes for developmental brain defects. The overall aim of the present study has been to identify new candidate genes or predisposing factors involved in congenital brain malformations in epilepsy patients.......The search for genetic causes of congenital brain malformations, severe epilepsy and mental retardation plays an important role in neuropediatrics and neurology. Disclosure of the aetiology of the intellectual disabilities, seizures and the underlying brain malformation may be of psychological...... value for the family, and it is essential for proper genetic counselling. The human brain is one of the most complex structures known, and probably many of the 25.000- 30.000 genes that comprise the human genome are involved in its development, which means that thousands of genes could be candidate...

  1. Brain Vascular Malformation Consortium: Overview, Progress and Future Directions.

    Science.gov (United States)

    Akers, Amy L; Ball, Karen L; Clancy, Marianne; Comi, Anne M; Faughnan, Marie E; Gopal-Srivastava, Rashmi; Jacobs, Thomas P; Kim, Helen; Krischer, Jeffrey; Marchuk, Douglas A; McCulloch, Charles E; Morrison, Leslie; Moses, Marsha; Moy, Claudia S; Pawlikowska, Ludmilla; Young, William L

    2013-04-01

    Brain vascular malformations are resource-intensive to manage effectively, are associated with serious neurological morbidity, lack specific medical therapies, and have no validated biomarkers for disease severity and progression. Investigators have tended to work in "research silos" with suboptimal cross-communication. We present here a paradigm for interdisciplinary collaboration to facilitate rare disease research. The Brain Vascular Malformation Consortium (BVMC) is a multidisciplinary, inter-institutional group of investigators, one of 17 consortia in the Office of Rare Disease Research Rare Disease Clinical Research Network (RDCRN). The diseases under study are: familial Cerebral Cavernous Malformations type 1, common Hispanic mutation (CCM1-CHM); Sturge-Weber Syndrome (SWS); and brain arteriovenous malformation in hereditary hemorrhagic telangiectasia (HHT). Each project is developing biomarkers for disease progression and severity, and has established scalable, relational databases for observational and longitudinal studies that are stored centrally by the RDCRN Data Management and Coordinating Center. Patient Support Organizations (PSOs) are a key RDCRN component in the recruitment and support of participants. The BVMC PSOs include Angioma Alliance, Sturge Weber Foundation , and HHT Foundation International . Our networks of clinical centers of excellence in SWS and HHT, as well as our PSOs, have enhanced BVMC patient recruitment. The BVMC provides unique and valuable resources to the clinical neurovascular community, and recently reported findings are reviewed. Future planned studies will apply successful approaches and insights across the three projects to leverage the combined resources of the BVMC and RDCRN in advancing new biomarkers and treatment strategies for patients with vascular malformations.

  2. Magnetic resonance venography of congenital vascular malformations of the extremities

    International Nuclear Information System (INIS)

    Laor, T.; Burrows, P.E.; Hoffer, F.A.

    1996-01-01

    Contrast angiography can demonstrate the vascular components of a vascular malformation, but can be technically challenging in small patients with complex venous anomalies. We reviewed the role of magnetic resonance venography (MRV) in the evaluation of children with predominantly low-flow, vascular malformations of the extremities. MRV (2D time-of-flight technique) and magnetic resonance (MR) imaging examinations were performed in ten young patients with congential predominantly low-flow vascular malformations of the extremities. MR imaging was used to characterize and determine the extent of the malformations, and MRV to evaluate the deep and superficial venous channels. In all patients, MRV studies were reviewed in conjunction with contrast angiograms, considered the gold standard, to confirm the findings. All signficant channel anomalies seen with contrast angiography were identified with MRV. In addition, MRV demonstrated some veins that were not intentionally opacified during contrast studies. MRV demonstrates both the superficial and deep conducting veins, whereas contrast angiography is a more directed study, evaluating only those channels intentionally opacified. Together, MR imaging and MRV data can non-invasively form the basis for determining the prognosis and choosing the individual treatment of congenital vascular malformations of the extremities. (orig.)

  3. Gross congenital malformation at birth in a government hospital.

    Science.gov (United States)

    Sachdeva, Sandeep; Nanda, Smiti; Bhalla, Kapil; Sachdeva, Ruchi

    2014-01-01

    A hospital-based cross-sectional study was undertaken to determine proportion of gross congenital malformation (GCMF) occurring at intramural births. Rate of GCMF was found to be 16.4/1000 consecutive singleton births (>28 weeks) with three leading malformation as anencephaly (44.68%), talipes equinovarus (17.02%) and meningomyelocele (10.63%). Higher risk of malformed births were noticed amongst un-booked (2.07%) in-comparison to booked (1.01%) mothers; women with low level of education (up to 8 years [2.14%] vs. at least 9 years of schooling [0.82%]); gravida status of at least 3 (2.69%) followed by 1 (1.43%) and 2 (1.0%) respectively; pre-term (5.13%) vs. term (0.66%); cesarean section (4.36%) versus vaginal delivery (0.62%). Mortality was significantly higher among congenitally malformed (17.35%) than normal (0.34%) newborns. With-in study limitation, emergence of neural tube defect as the single largest category of congenital malformation indicates maternal malnutrition (especially folic acid) that needs appropriate attention and management.

  4. Antithyroid Drugs and Congenital Malformations: A Nationwide Korean Cohort Study.

    Science.gov (United States)

    Seo, Gi Hyeon; Kim, Tae Hyuk; Chung, Jae Hoon

    2018-03-20

    Untreated or insufficiently treated Graves disease in pregnancy may pose risks to both mother and fetus. Antithyroid drugs (ATDs) are the treatment mainstay, but the potential teratogenic effect of these drugs has prompted clinicians to question the safe management of this vulnerable population. To examine the association between maternal prescriptions for ATDs and congenital malformations in live births. Nationwide cohort study. Korean National Health Insurance database. A cohort of 2 886 970 completed pregnancies linked to live-born infants in 2 210 253 women between 2008 and 2014. Maternal prescriptions for ATDs in the first trimester. The risk for overall and organ-specific congenital malformations in offspring, with logistic regression models used to control for potential confounders. 12 891 pregnancies (0.45%) were exposed to ATDs during the first trimester. The prevalence of malformations in exposed offspring was 7.27%, compared with 5.94% in offspring of women who were not prescribed ATDs during pregnancy (P 495 mg) during the first trimester was associated with an increased risk for malformations compared with a low dose (1 to 126 mg) (adjusted odds ratio, 1.87 [CI, 1.06 to 3.30]). The study used a prescription claims database to assess ATD exposure. Exposure to ATDs during the first trimester was associated with increased risk for congenital malformations, particularly for pregnancies in which women received prescriptions for MMI or both ATDs. None.

  5. Movement of the external ear in human embryo.

    Science.gov (United States)

    Kagurasho, Miho; Yamada, Shigehito; Uwabe, Chigako; Kose, Katsumi; Takakuwa, Tetsuya

    2012-02-01

    External ears, one of the major face components, show an interesting movement during craniofacial morphogenesis in human embryo. The present study was performed to see if movement of the external ears in a human embryo could be explained by differential growth. In all, 171 samples between Carnegie stage (CS) 17 and CS 23 were selected from MR image datasets of human embryos obtained from the Kyoto Collection of Human Embryos. The three-dimensional absolute position of 13 representative anatomical landmarks, including external and internal ears, from MRI data was traced to evaluate the movement between the different stages with identical magnification. Two different sets of reference axes were selected for evaluation and comparison of the movements. When the pituitary gland and the first cervical vertebra were selected as a reference axis, the 13 anatomical landmarks of the face spread out within the same region as the embryo enlarged and changed shape. The external ear did move mainly laterally, but not cranially. The distance between the external and internal ear stayed approximately constant. Three-dimensionally, the external ear located in the caudal ventral parts of the internal ear in CS 17, moved mainly laterally until CS 23. When surface landmarks eyes and mouth were selected as a reference axis, external ears moved from the caudal lateral ventral region to the position between eyes and mouth during development. The results indicate that movement of all anatomical landmarks, including external and internal ears, can be explained by differential growth. Also, when the external ear is recognized as one of the facial landmarks and having a relative position to other landmarks such as the eyes and mouth, the external ears seem to move cranially. © 2012 Kagurasho et al; licensee BioMed Central Ltd.

  6. Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain.

    Science.gov (United States)

    Pichi, Francesco; Freund, K Bailey; Ciardella, Antonio; Morara, Mariachiara; Abboud, Emad B; Ghazi, Nicola; Dackiw, Christine; Choudhry, Netan; Souza, Eduardo Cunha; Cunha, Leonardo Provetti; Arevalo, J Fernando; Liu, T Y Alvin; Wenick, Adam; He, Lingmin; Villarreal, Guadalupe; Neri, Piergiorgio; Sarraf, David

    2018-04-01

    Congenital retinal macrovessel (CRM) is a rarely reported venous malformation of the retina that is associated with venous anomalies of the brain. To study the multimodal imaging findings of a series of eyes with congenital retinal macrovessel and describe the systemic associations. In this cross-sectional multicenter study, medical records were retrospectively reviewed from 7 different retina clinics worldwide over a 10-year period (2007-2017). Patients with CRM, defined as an abnormal, large, macular vessel with a vascular distribution above and below the horizontal raphe, were identified. Data were analyzed from December 2016 to August 2017. Clinical information and multimodal retinal imaging findings were collected and studied. Pertinent systemic information, including brain magnetic resonance imaging findings, was also noted if available. Of the 49 included patients, 32 (65%) were female, and the mean (SD) age at onset was 44.0 (20.9) years. A total of 49 eyes from 49 patients were studied. Macrovessel was unilateral in all patients. Color fundus photography illustrated a large aberrant dilated and tortuous retinal vein in all patients. Early-phase frames of fluorescein angiography further confirmed the venous nature of the macrovessel in 40 of 40 eyes. Optical coherence tomography angiography, available in 17 eyes (35%), displayed microvascular capillary abnormalities around the CRM, which were more evident in the deep capillary plexus. Of the 49 patients with CRM, 39 (80%) did not illustrate any evidence of ophthalmic complications. Ten patients (20%) presented with retinal complications, typically an incidental association with CRM. Twelve patients (24%) were noted to have venous malformations of the brain with associated magnetic resonance imaging. Of these, location of the venous anomaly in the brain was ipsilateral to the CRM in 10 patients (83%) and contralateral in 2 patients (17%), mainly located in the frontal lobe in 9 patients (75%). Our study has

  7. Cerebral cavernous malformations. Serial magnetic resonance imaging findings in patients with and without gamma knife surgery

    International Nuclear Information System (INIS)

    Yoon Pyeong-Ho; Kim, Dong-Ik; Jeon Pyoung; Ryu, Young-Hoon; Hwang, Geum-Joo; Park, Sang-Joon

    1998-01-01

    To classify the cerebral cavernous malformations and to investigate the natural history of cavernous malformations according to the classification, 41 patients with 61 cavernous malformations (40 cavernous malformations from 22 patients treated with gamma knife surgery) were regularly followed up using MR imaging for a mean period of 25.5 months in treated cavernous malformations and 20.7 months in untreated cavernous malformations, respectively. Cavernous malformations were classified into four types. Follow-up MR images were analyzed to evaluate changes in size, signal intensity, rebleeding, and perilesional adverse reaction of irradiation. A total of 61 cavernous malformations including 17 in type I, 23 in type II, 10 in type III, and 11 in type IV showed usual degradation of blood product in 22 cavernous malformations, no change in shape and signal intensity in 31 cavernous malformations, and eight cavernous malformations with rebleedings in the serial MR images. In these eight cavernous malformations with rebleedings, six occurred in type II and two in type III, but none in type I or IV. Rebleedings were more frequent in type II than in other types. Adverse reaction of irradiation was observed in five of 22 patients treated with gamma knife surgery. Although most cerebral cavernous malformations showed evolution of hemorrhage or no change in size or shape on follow-up MR images, cerebral cavernous malformations represented as mixture of subacute and chronic hemorrhage with hemosiderin rim (type II) have a higher frequency to rebleed than other types of cerebral cavernous malformations. Cerebral cavernous malformations represented as hemosiderin deposition without central core (type IV) have a lower tendency to rebleed than other types and do not need any treatment. Most of the adverse reaction of irradiation after gamma knife surgery around cavernous malformations are transient findings and are considered to be perilesional edema. (K.H)

  8. Pattern of congenital brain malformations at a referral hospital in Saudi Arabia: An MRI study

    International Nuclear Information System (INIS)

    Alorainy, Ibrahim A.

    2006-01-01

    More than 2000 different congenital cerebral malformations have been described in the literature, for which several classification systems have been proposed. With the help of these classification systems, it is now possible, with neuroimaging, to time neuroembtyologic events. Magnetic resonance imaging (MRI), in particular, is useful in studying these malformations. This study evaluated the pattern of congenital brain malformations in a university referral hospital setting. The records of all MRI brain examinations at our hospital over a period of 3 years for children younger than 15 years of age were reviewed. Cases of congenital cerebral malformations were analyzed by sex, age at presentation, type of congenital cerebral malformation and other associated congenital cerebral malformations. Of the 808 MR examinations of different parts of the body for children in the study period, 719 (89%), on 581 patients, were of the brain. Eighty-six children (14.8%) were found to have single or multiple congenital brain malformations. In these children, 114 congenital brain malformations were identified, the commonest being cortical migrational defects (25 patients, 22%), neural tube closure defects (22 patients, 19%), and corpus callosum dysgenesis (22 patients 19%). The least common was vascular malformation. Sixteen patients (18.6%) had more than one congenital brain malformations. Neural tube closer defects, cortical migrational abnormalities, and corpus callosum anomalies were the commonest congenital brain malformations, while vascular malformations were the least common. Most of the identified malformations demonstrated the usual pattern, but a few showed unusual patterns and associations. (author)

  9. Antidepressant use during pregnancy and the risk of major congenital malformations in a cohort of depressed pregnant women: an updated analysis of the Quebec Pregnancy Cohort.

    Science.gov (United States)

    Bérard, Anick; Zhao, Jin-Ping; Sheehy, Odile

    2017-01-12

    Antidepressant use during gestation has been associated with risk of major congenital malformations but estimates can lack statistical power or be confounded by maternal depression. We aimed to determine the association between first-trimester exposure to antidepressants and the risk of major congenital malformations in a cohort of depressed/anxious women. Data were obtained from the Quebec Pregnancy Cohort (QPC). All pregnancies with a diagnosis of depression or anxiety, or exposed to antidepressants in the 12 months before pregnancy, and ending with a live-born singleton were included. Antidepressant classes (selective serotonin reuptake inhibitors (SSRI), serotonin-norepinephrine reuptake inhibitors (SNRI), tricyclic antidepressants (TCA) and other antidepressants) and types were individually compared with non-exposure during the first trimester (depressed untreated). Major congenital malformations overall and organ-specific malformations in the first year of life were identified. 18 487 pregnant women were included. When looking at the specific types of antidepressant used during the first trimester, only citalopram was increasing the risk of major congenital malformations (adjusted OR, (aOR) 1.36, 95% CI 1.08 to 1.73; 88 exposed cases), although there was a trend towards increased risk for the most frequently used antidepressants. Antidepressants with serotonin reuptake inhibition effect (SSRI, SNRI, amitriptyline (the most used TCA)) increased the risk of certain organ-specific defects: paroxetine increased the risk of cardiac defects (aOR 1.45, 95% CI 1.12 to 1.88), and ventricular/atrial septal defects (aOR 1.39, 95% CI 1.00 to 1.93); citalopram increased the risk of musculoskeletal defects (aOR 1.92, 95% CI 1.40 to 2.62), and craniosynostosis (aOR 3.95, 95% CI 2.08 to 7.52); TCA was associated with eye, ear, face and neck defects (aOR 2.45, 95% CI 1.05 to 5.72), and digestive defects (aOR 2.55, 95% CI 1.40 to 4.66); and venlafaxine was associated with

  10. Congenital brain abnormalities: an update on malformations of cortical development and infratentorial malformations.

    Science.gov (United States)

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2014-07-01

    In the past two decades, significant progress in neuroimaging and genetic techniques has allowed for advances in the correct definition/classification of congenital brain abnormalities, which have resulted in a better understanding of their pathogenesis. In addition, new groups of diseases, such as axonal guidance disorders or tubulinopathies, are increasingly reported. Well-defined neuroimaging diagnostic criteria have been suggested for the majority of congenital brain abnormalities. Accurate diagnoses of these complex abnormalities, including distinction between malformations and disruptions, are of paramount significance for management, prognosis, and family counseling. In the next decade, these advances will hopefully be translated into deeper understanding of these disorders and more specific treatments. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  11. Antidepressant exposure during early pregnancy and congenital malformations

    DEFF Research Database (Denmark)

    Pedersen, Lars Henning

    are reassuring, however, an association with heart malformations has been suggested for e.g. paroxetine. A potential biological explanation will be reviewed. The potential teratogenic potential of antidepressants needs to be balanced against the obvious problems associated with under-treated maternal depression......Pharmacological treatment of pregnant women with depression is hampered by concerns for the developing fetus. The presentation will summarize existing knowledge on the potential association between antidepressants and congenital malformations, elaborate on the scientific background, and discuss...... the clinical significance. Most information on malformations in humans is derived from epidemiological studies. The strengths and limitations of the different designs need careful consideration, including issues of confounding by indication, recall bias, and power. For most antidepressants existing data...

  12. Identifying position, visibility, dimensions, and angulation of the ear.

    Science.gov (United States)

    Mohamed, Kasim; Christian, Jayanth; Jeyapalan, Karthigeyan; Natarajan, Shanmuganathan; Banu, Fathima; Veeravalli, Padmanabhan T

    2014-01-01

    We selected 254 subjects between the ages of 18 and 30 yr to assess the ear position, angulations of the ear in relation to the nose, visibility from the frontal view, and dimensions of the ear by using various anthropometric points of the face. Subjects were divided into four groups based on facial form. A reference plane indicator, facial topographical measurements, metal ruler, and digital photography were used. While considering the position of the ear, in all facial forms except square tapering, the most samples showed a tendency for the subaurale being in line with subnasale. Regression analysis showed a tendency to gnathion distance is the most dependent variable with length of the ear kept as a constant predictor, while both interalar distance and exocanthion to endocanthion distance correlate highly significantly to the width of the ear. In all subjects, the visibility of the ear when viewed from the front was an average of 1.5 mm. Regardless of facial form, ear angulation was generally less than nose angulation.

  13. Ad Hoc Constitution of Topical Antibiotics Solution for Ear Dressing ...

    African Journals Online (AJOL)

    Background/ Purpose: In the management of chronic suppurative otitis media and otitis externa there are few cases where although an organism is cultured and isolated from the ear, there are either no antibiotic sensitive to the microbiological flora or the sensitive antibiotic is not available in the form of an ear drop, limiting ...

  14. Why do elephants flap their ears? | Wright | African Zoology

    African Journals Online (AJOL)

    The blood flow in the ear of the African elephant Loxodonta africana was measured In anaesthetized animals using the dye dilution technique at the same time as the arterio-venous temperature difference. The calculated heat loss from the ear is shown to be a substantial proportion of the total metabolic heat-loss ...

  15. Economic Evaluation pf Antibacterial Usage in Ear, Nose and Throat ...

    African Journals Online (AJOL)

    Purpose: To carry out economic evaluation of antibacterial usage for Ear, Nose and Throat infections in a tertiary health care facility in Nigeria. Methods: Antibacterial utilisation evaluation was carried out retrospectively over one year period by reviewing 122 case notes containing 182 prescriptions of patient with Ear Nose ...

  16. A break-even analysis of major ear surgery.

    Science.gov (United States)

    Wasson, J D; Phillips, J S

    2015-10-01

    To determine variables which affect cost and profit for major ear surgery and perform a break-even analysis. Retrospective financial analysis. UK teaching hospital. Patients who underwent major ear surgery under general anaesthesia performed by the senior author in main theatre over a 2-year period between dates of 07 September 2010 and 07 September 2012. Income, cost and profit for each major ear patient spell. Variables that affect major ear surgery profitability. Seventy-six patients met inclusion criteria. Wide variation in earnings, with a median net loss of £-1345.50 was observed. Income was relatively uniform across all patient spells; however, theatre time of major ear surgery at a cost of £953.24 per hour varied between patients and was the main determinant of cost and profit for the patient spell. Bivariate linear regression of earnings on theatre time identified 94% of variation in earnings was due to variation in theatre time (r = -0.969; P break-even time for major ear surgery of 110.6 min. Theatre time was dependent on complexity of procedure and number of OPCS4 procedures performed, with a significant increase in theatre time when three or more procedures were performed during major ear surgery (P = 0.015). For major ear surgery to either break-even or return a profit, total theatre time should not exceed 110 min and 36 s. © 2015 John Wiley & Sons Ltd.

  17. The acoustical significance of age-dependent ear elongation

    DEFF Research Database (Denmark)

    Christensen, Flemming

    2015-01-01

    , corresponding to what is reported in the literature. For female ears, virtually no acoustical effect was found. For male ears directional dependent effects in the range up to 5 dB on average was found for certain directions and frequencies. Implications on age dependent hearing loss (presbycusis...

  18. Profile of Ear Diseases among Elderly Patients in Sagamu, South ...

    African Journals Online (AJOL)

    The importance of screening for hearing impairment in the elderly patients was also stressed. KEYWORDS: Cerumen, Ear disease, Elderly, Otitis, Presbycusis. Erratum Note: Olusola AS on the article “Profile of Ear Diseases among Elderly Patients in Sagamu, South-Western Nigeria” on Page Nig. J. Med 2013. 143-147.

  19. 14 CFR 67.205 - Ear, nose, throat, and equilibrium.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Ear, nose, throat, and equilibrium. 67.205..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman..., vertigo or a disturbance of equilibrium. ...

  20. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Ear, nose, throat, and equilibrium. 67.105..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium. ...

  1. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Ear, nose, throat, and equilibrium. 67.305..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman... by, or that may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium. ...

  2. Directional characteristics for different in-ear recording points

    DEFF Research Database (Denmark)

    Møller, Anders Kalsgaard; Christensen, Flemming; Hoffmann, Pablo Faundez

    2015-01-01

    -back the sound. In this paper, measurements with different in-ear recording positions are conducted on nine subjects. The measurements are used for finding the optimal microphone position. The results show that spatial information equivalent to ears without insert earphones can be preserved up to around 4-5 kHz....

  3. Concha headphones and their coupling to the ear

    DEFF Research Database (Denmark)

    Blanchard, Lola Justine Kydia Olivia; Agerkvist, Finn T.

    2009-01-01

    The purpose of the study is to obtain a better understanding of concha headphone. Concha headphones are the small types of earpiece that are placed in the concha. They are not sealed to the ear and therefore, there is a leak between the earpiece and the ear. This leak is the reason why...

  4. 15 CFR 734.2 - Important EAR terms and principles.

    Science.gov (United States)

    2010-01-01

    ... 15 Commerce and Foreign Trade 2 2010-01-01 2010-01-01 false Important EAR terms and principles. 734.2 Section 734.2 Commerce and Foreign Trade Regulations Relating to Commerce and Foreign Trade... OF THE EXPORT ADMINISTRATION REGULATIONS § 734.2 Important EAR terms and principles. (a) Subject to...

  5. Preventing Cauliflower Ear with a Modified Tie-Through Technique.

    Science.gov (United States)

    Dimeff, Robert J.; Hough, David O.

    1989-01-01

    Describes a quick, simple tie-through suture technique (in which a collodion packing is secured to the auricle with two buttons) for preventing cauliflower ear following external ear trauma in wrestlers and boxers. The technique ensures constant compression; multiple treatments for fluid reaccumulation are rarely necessary. (SM)

  6. Comparison of Microbiological Flora in the External Auditory Canal of Normal Ear and an Ear with Acute Otitis Externa.

    Science.gov (United States)

    Ghanpur, Asheesh Dora; Nayak, Dipak Ranjan; Chawla, Kiran; Shashidhar, V; Singh, Rohit

    2017-09-01

    Acute Otitis Externa (AOE) is also known as swimmer's ear. Investigations initiated during World War II firmly established the role of bacteria in the aetiology of Acute Otitis Externa. To culture the microbiological flora of the normal ear and compare it with the flora causing AOE and to know the role of normal ear canal flora and anaerobes in the aetiology. A prospective observational study was conducted on 64 patients clinically diagnosed with unilateral AOE. Ear swabs were taken from both the ears. Microbiological flora was studied considering diseased ear as test ear and the normal ear as the control. Aerobic and anaerobic cultures were done. Severity of the disease was assessed by subjective and objective scores. Effect of topical treatment with ichthammol glycerine pack was assessed after 48 hours and scores were calculated again. Patients with scores < 4 after pack removal were started on systemic antibiotics and were assessed after seven days of antibiotics course. Data was analysed using Paired t-test, Wilcoxon signed ranks test and Chi-square test. A p-value < 0.05 was considered significant. Pseudomonas aeruginosa (33%) was the most common bacteria cultured from the ear followed by Methicillin Resistant Staphylococcus aureus (MRSA) (18%). Patients with anaerobic organism in the test ear had severe symptoms and needed systemic antibiotic therapy. Most of the cases may respond to empirical antibiotic therapy. In cases with severe symptoms and the ones refractory to empirical treatment, a culture from the ear canal will not be a tax on the patient. This helps in giving a better understanding about the disease, causative organisms and helps in avoiding the use of inappropriate antibiotics that usually result in developing resistant strains of bacteria.

  7. Classification of venous malformations in children and implications for sclerotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Puig, Stefan [Department of Radiology, University of Vienna, Waehringer Guertel 18-20, 1090 Vienna (Austria); Department of Paediatric Radiology, Hopital Necker-Enfants Malades, Paris (France); Aref, Hussein [Department of Paediatric Radiology, Hopital Necker-Enfants Malades, Paris (France); Department of Radiology, Alexandria Faculty of Medicine, Alexandria (Egypt); Chigot, Valerie; Brunelle, Francis [Department of Paediatric Radiology, Hopital Necker-Enfants Malades, Paris (France); Bonin, Beatrice [Paediatric Maxillofacial Surgery, Trousseau Hospital, Tours (France)

    2003-02-01

    The purpose of this work is to present a simple and descriptive classification system for venous malformations (VMs) that may serve as a basis for interventional therapy, and to test its usefulness in a sample of consecutively referred paediatric patients. The classification system we developed includes four types: type I, isolated malformation without peripheral drainage; type II, malformation that drains into normal veins; type III, malformation that drains into dilated veins; and type IV, malformation that represents dysplastic venous ectasia. The system was prospectively tested using phlebography in a sample of 43 children and adolescents with VMs who were referred for treatment during a 10-month period. Our hypothesis was that the type of VM would determine whether low-risk sclerotherapy was indicated. Thirteen (30%) patients had a type-I VM, 16 (37%) had a type-II, 9 (21%) had a type-III, and 5 (12%) had a type-IV malformation. In more than 90% of patients with a type-I or type-II lesion, sclerotherapy could be performed without any problems. In one third of patients with a type-III VM, sclerotherapy had to be withheld and one of nine (11%) developed a severe complication after therapy. Of the five patients with type-IV lesions, three (60%) had to be excluded from sclerotherapy. Our initial results indicate that sclerotherapeutic intervention in patients with type-III and type-IV VMs must be carefully considered, while it can be safely performed in low-risk patients with type-I and type-II lesions. (orig.)

  8. [Epidemiological analysis of selected congenital limb malformations in Hengyang].

    Science.gov (United States)

    Li, Na-Na; Yuan, Yu-Mei; Liu, Yong; Dai, Li; Deng, Chang-Fei; Nie, Xing-Hui; Zheng, Xiang-Chi; Hu, Yan-Zhen; Liu, Yun-Rong

    2013-07-01

    To describe the epidemiological characteristics of selected congenital limb malformations (CLM) in newborns of Hengyang. During the period of 2008-2010, cluster sampling survey was adopted to investigate the congenital limb malformations of neonates born to women resident in Hengyang, including Nanyue District, Zhuhui District, Changning City and Hengshan County. Each newborn was examined for the screening of CLM after birth. Limb malformations were grouped into the isolated (ILM) and the syndromic (SLM) form, depending on associated malformations of the affected. Prevalence rates, CLM spectrum and clinical manifestations were analyzed. A total of 170 CLM cases were identified among 52,307 newborns during the study period, resulting overall rate of 32.50/10(4). The rates for isolated and syndromic CLM were 28.29 and 4.21 per 10 000 births respectively. The rates for polydactyly, congenital talipes equinovarus, syndactyly and limb reduction defects were 13.00/10(4), 9.56/10(4), 5.16/10(4) and 3.63/10(4), respectively. No significant difference in rates of overall CLM or specified CLM was observed across urban-rural, gender and maternal age groups. Of the cases affected by polydactyly, syndactyly and limb reduction defects, malformation involved upper limbs, lower limbs and the both accounted for 68.14%, 14.16% and 17.70%. Preterm birth, low birth-weight, still birth and neonatal death were observed more frequently in syndromic cases than in isolated patients. The high CLM prevalence rate and fatality rate in Hengyang suggest that effective measures should be taken to prevent malformations and to improve survival of the affected.

  9. Magnetic resonance imaging in inflammatory lesions of the middle ear

    International Nuclear Information System (INIS)

    Tono, Tetsuya; Saku, Kazuaki; Miyanaga, Satoshi; Kano, Kiyo; Morimitsu, Tamotsu; Suzuki, Yukiko.

    1988-01-01

    Eighteen patients with chronic otitis media, middle ear cholesteatoma, and postoperative inflammatory diseases of the middle ear underwent high resolution computerized tomography (CT) and magnetic resonance imaging (MRI) before surgical exploration of the middle ear. Results showed that CT provides higher detail resolution in middle ear structures, but provides limited density resolution in displaying inflammatory soft tissue lesions. By contrast, MRI differentiates among soft tissue lesions such as fluid-filled spaces, granulation tissues, and cholesteatomatous debris. Cholesterin granulomas show a particularly characteristic signal pattern with a very high intensity area in both T1 and T2 weighted images. It is concluded that MRI is useful in differentiating soft tissue density masses when used in conjunction with CT in middle ear inflammatory diseases. (author)

  10. Acoustic impedances of ear canals measured by impedance tube

    DEFF Research Database (Denmark)

    Ciric, Dejan; Hammershøi, Dorte

    2007-01-01

    During hearing sensitivity tests, the sound field is commonly generated by an earphone placed on a subject ear. One of the factors that can affect the sound transmission in the ear is the acoustic impedance of the ear canal. Its importance is related to the contribution of other elements involved...... in the transmission such as the earphone impedance. In order to determine the acoustic impedances of human ear canals, the standardized method for measurement of complex impedances used for the measurement of the audiometric earphone impedances is applied. It is based on the transfer function between two microphone...... locations in an impedance tube. The end of the tube representing the measurement plane is placed at the ear canal entrance. Thus, the impedance seen from the entrance inward is measured on 25 subjects. Most subjects participated in the previous measurement of the ratio between the pressures at the open...

  11. Aberrant internal carotid artery in the middle ear

    Energy Technology Data Exchange (ETDEWEB)

    Roh, Keun Tak; Kang, Hyun Koo [Dept. of Radiology, Seoul Veterans Hospital, Seoul (Korea, Republic of)

    2014-10-15

    The knowledge about the aberrant internal carotid artery (ICA) in the middle ear is essential for clinicians, because a misdiagnosis of the aberrant ICA could have serious consequences such as excessive aural bleeding during a middle ear surgery. A 38-year-old woman presented with tinnitus and hearing difficulties of the left ear that had started 5 years ago. During otoscopy, an anteroinferior bluish mass was seen in the tympanic space. Computed tomography and magnetic resonance imaging demonstrated a left-side aberrant ICA with bony dehiscence of the carotid canal in the middle ear and a reduced diameter of the tympanic ICA. Herein we report a case of an aberrant ICA in the middle ear. We also review the literature regarding this important vascular anomaly of the temporal bone which may lead to disastrous surgical complications.

  12. Aberrant internal carotid artery in the middle ear

    International Nuclear Information System (INIS)

    Roh, Keun Tak; Kang, Hyun Koo

    2014-01-01

    The knowledge about the aberrant internal carotid artery (ICA) in the middle ear is essential for clinicians, because a misdiagnosis of the aberrant ICA could have serious consequences such as excessive aural bleeding during a middle ear surgery. A 38-year-old woman presented with tinnitus and hearing difficulties of the left ear that had started 5 years ago. During otoscopy, an anteroinferior bluish mass was seen in the tympanic space. Computed tomography and magnetic resonance imaging demonstrated a left-side aberrant ICA with bony dehiscence of the carotid canal in the middle ear and a reduced diameter of the tympanic ICA. Herein we report a case of an aberrant ICA in the middle ear. We also review the literature regarding this important vascular anomaly of the temporal bone which may lead to disastrous surgical complications.

  13. Direct cost comparison of totally endoscopic versus open ear surgery.

    Science.gov (United States)

    Patel, N; Mohammadi, A; Jufas, N

    2018-02-01

    Totally endoscopic ear surgery is a relatively new method for managing chronic ear disease. This study aimed to test the null hypothesis that open and endoscopic approaches have similar direct costs for the management of attic cholesteatoma, from an Australian private hospital setting. A retrospective direct cost comparison of totally endoscopic ear surgery and traditional canal wall up mastoidectomy for the management of attic cholesteatoma in a private tertiary setting was undertaken. Indirect and future costs were excluded. A direct cost comparison of anaesthetic setup and resources, operative setup and resources, and surgical time was performed between the two techniques. Totally endoscopic ear surgery has a mean direct cost reduction of AUD$2978.89 per operation from the hospital perspective, when compared to canal wall up mastoidectomy. Totally endoscopic ear surgery is more cost-effective, from an Australian private hospital perspective, than canal wall up mastoidectomy for attic cholesteatoma.

  14. Intrauterine exposure to carbamazepine and specific congenital malformations: systematic review and case-control study

    DEFF Research Database (Denmark)

    Jentink, Janneke; Dolk, Helen; Loane, Maria A

    2010-01-01

    To identify specific major congenital malformations associated with use of carbamazepine in the first trimester of pregnancy.......To identify specific major congenital malformations associated with use of carbamazepine in the first trimester of pregnancy....

  15. Effects of Co60 gamma radiation on Biomphalaria glabrata (Say, 1818) Embryo. II. Malformations

    International Nuclear Information System (INIS)

    Okazaki, K.; Kawano, T.

    1990-01-01

    The morphogenetic effects of ionizing radiation were investigated in Biomphalaria glabrata embryos irradiated in the cleavage, blastula, gastrula, young trochophore and trochophore stages with 5 to 25 Gy doses of 60 CO gamma radiation. The number of malformed embryos rapidly increased with increasing radiation dose, reaching a maximum between 5th to 8th day after irradiation in all stages analyzed. Susceptibility to malformation induction was higher the younger than the age of the irradiated embryo. However, for the cleavage stage the frequency of malformed embryos was inversely proportional to radiation dose for the same radiation dose. Several types of morphogenetic malformations were obtained, among then cephalic malformations, exogastrula, shell malformations and embryos with everted stomodeum, unspecific malformations being the most frequent. The results show that the types of malformation induced by radiation probably are not radiation-specific and do not depend on the dose applied [pt

  16. Imaging features of ductal plate malformations in adults

    Energy Technology Data Exchange (ETDEWEB)

    Venkatanarasimha, N., E-mail: nandashettykv@yahoo.com [Department of Radiology, Derriford Hospital, Plymouth (United Kingdom); Thomas, R.; Armstrong, E.M.; Shirley, J.F.; Fox, B.M.; Jackson, S.A. [Department of Radiology, Derriford Hospital, Plymouth (United Kingdom)

    2011-11-15

    Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis.

  17. [The genetic background for the eye malformations anophthalmia and microphthalmia].

    Science.gov (United States)

    Roos, Laura Sønderberg; Grønskov, Karen; Jensen, Hanne; Tümer, Zeynep

    2012-03-12

    Anophthalmia and microphthalmia (AO/MO) are rare congenital eye malformations, in which the eyeball is apparently absent or smaller than normal, which causes various degrees of visual impairment. Over 200 different AO/MO-related syndromes have been described, but the genetic background is unknown in many cases. The aim of this article is to give an overview of AO/MO, focusing on the genetic background. It is illustrated that the future identification of new AO/MO related genes will benefit in the genetic counseling of AO/MO patients, and in the understanding of eye development and congenital eye malformations.

  18. Berry syndrome in association with familial limb malformation.

    LENUS (Irish Health Repository)

    Shahdadpuri, R

    2012-02-01

    We describe a newborn boy diagnosed with Berry syndrome consisting of a distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, and interruption of the aorta. The child was noted to have reduplication of the right thumb. The child\\'s mother had a claw malformation of her left hand but a normal cardiovascular status. Genetic analysis for TBX5 and SALL4 mutations were negative in both the patient and his mother. This case describes the first ever report of Berry syndrome in an infant with reduplication of the right thumb and familial limb malformation.

  19. Diffusion imaging and tractography of congenital brain malformations

    International Nuclear Information System (INIS)

    Wahl, Michael; Barkovich, A.J.; Mukherjee, Pratik

    2010-01-01

    Diffusion imaging is an MRI modality that measures the microscopic molecular motion of water in order to investigate white matter microstructure. The modality has been used extensively in recent years to investigate the neuroanatomical basis of congenital brain malformations. We review the basic principles of diffusion imaging and of specific techniques, including diffusion tensor imaging (DTI) and high angular resolution diffusion imaging (HARDI). We show how DTI and HARDI, and their application to fiber tractography, has elucidated the aberrant connectivity underlying a number of congenital brain malformations. Finally, we discuss potential uses for diffusion imaging of developmental disorders in the clinical and research realms. (orig.)

  20. Cancer risk in siblings of children with congenital malformations

    DEFF Research Database (Denmark)

    Sun, Yuelian; Wu, Chunsen; Arah, Onyebuchi A

    2016-01-01

    PURPOSE: Cancer and birth defects cluster in families more often than expected by chance, but the reasons are neither well known nor well studied. METHODS: From singletons born alive in Denmark between 1 January 1977 and 31 December 2007, we identified children who had no congenital malformations...... but had a full or half sibling with a congenital malformation (CM) diagnosed in the first year of life; this constituted the exposed group, while children whose siblings had no such condition constituted a reference group. We estimated cancer risks for children who had a full sibling or a half sibling...

  1. Radiographic assessment of congenital malformations of the upper extremity

    International Nuclear Information System (INIS)

    Winfeld, Matthew J.; Otero, Hansel

    2016-01-01

    Congenital and developmental malformations of the upper extremity are uncommon and their diagnosis can challenge radiologists. Many complex classification systems exist, the latest of which accounts for the complex embryology and pathogenetic mechanisms that govern the formation of these anomalies. Using appropriate descriptors allows for more specific diagnosis and improved consultation with referring pediatricians and surgeons, helping to guide medical and surgical interventions and, if indicated, further investigation for associated abnormalities and underlying syndromes. We review the imaging characteristics of upper limb malformations to help pediatric radiologists better understand the classification and workup necessary in these cases. (orig.)

  2. Radiographic assessment of congenital malformations of the upper extremity

    Energy Technology Data Exchange (ETDEWEB)

    Winfeld, Matthew J. [Perelman School of Medicine at the University of Pennsylvania, Department of Radiology, Musculoskeletal Division, Penn Medicine University City, Philadelphia, PA (United States); Otero, Hansel [Children' s National Medical Center, Department of Radiology, Washington, DC (United States)

    2016-09-15

    Congenital and developmental malformations of the upper extremity are uncommon and their diagnosis can challenge radiologists. Many complex classification systems exist, the latest of which accounts for the complex embryology and pathogenetic mechanisms that govern the formation of these anomalies. Using appropriate descriptors allows for more specific diagnosis and improved consultation with referring pediatricians and surgeons, helping to guide medical and surgical interventions and, if indicated, further investigation for associated abnormalities and underlying syndromes. We review the imaging characteristics of upper limb malformations to help pediatric radiologists better understand the classification and workup necessary in these cases. (orig.)

  3. Two Cases of Arnold-Chiari Malformation with Respiratory Failure

    Directory of Open Access Journals (Sweden)

    Sinem Iliaz

    2014-03-01

    Full Text Available Arnold–Chiari malformation is defined as downward displacement of the brainstem and cerebellum through the foramen magnum. It has different clinical presentations and four subtypes. It is known that downward migration of posterior fossa components through the foramen magnum and associated lower cranial nerve palsy and brainstem compression can cause respiratory failure. Acute respiratory failure could mark the onset of the disease. Posterior fossa decompression performed to treat primary disease can improve the central sleep abnormalities. As respiratory failure is rarely seen, this paper presents two cases of Arnold–Chiari malformation with respiratory failure.

  4. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  5. Neonatal Hemoperitoneum Secondary to Hemorrhage from Omental Venous Malformation

    Directory of Open Access Journals (Sweden)

    So-Hyun Nam

    2015-12-01

    Full Text Available Hemoperitoneum in the neonate is uncommon. We report our experience with a neonate who suddenly developed hemoperitoneum in late gestation. Ascites with right pleural effusion initially suggested fetal hydrops. However, a decrease in hemoglobin with bloody ascites from the paracentesis gave the impression of hemoperitoneum. Ultrasonography and computed tomography showed no abnormality in the solid organs. Using 3-mm laparoscopic exploration, we found the omental mass and excised it. It was histologically confirmed as a venous malformation. Venous malformation of the omentum with significant bleeding requiring exploration is a very rare condition in the neonate, but it should be considered as a cause of neonatal hemoperitoneum.

  6. Physiological artifacts in scalp EEG and ear-EEG.

    Science.gov (United States)

    Kappel, Simon L; Looney, David; Mandic, Danilo P; Kidmose, Preben

    2017-08-11

    A problem inherent to recording EEG is the interference arising from noise and artifacts. While in a laboratory environment, artifacts and interference can, to a large extent, be avoided or controlled, in real-life scenarios this is a challenge. Ear-EEG is a concept where EEG is acquired from electrodes in the ear. We present a characterization of physiological artifacts generated in a controlled environment for nine subjects. The influence of the artifacts was quantified in terms of the signal-to-noise ratio (SNR) deterioration of the auditory steady-state response. Alpha band modulation was also studied in an open/closed eyes paradigm. Artifacts related to jaw muscle contractions were present all over the scalp and in the ear, with the highest SNR deteriorations in the gamma band. The SNR deterioration for jaw artifacts were in general higher in the ear compared to the scalp. Whereas eye-blinking did not influence the SNR in the ear, it was significant for all groups of scalps electrodes in the delta and theta bands. Eye movements resulted in statistical significant SNR deterioration in both frontal, temporal and ear electrodes. Recordings of alpha band modulation showed increased power and coherence of the EEG for ear and scalp electrodes in the closed-eyes periods. Ear-EEG is a method developed for unobtrusive and discreet recording over long periods of time and in real-life environments. This study investigated the influence of the most important types of physiological artifacts, and demonstrated that spontaneous activity, in terms of alpha band oscillations, could be recorded from the ear-EEG platform. In its present form ear-EEG was more prone to jaw related artifacts and less prone to eye-blinking artifacts compared to state-of-the-art scalp based systems.

  7. Mozart Ear Deformity: a Rare Diagnosis in the Ear Reconstruction Clinic.

    Science.gov (United States)

    Telich-Tarriba, Jose E; Victor-Baldin, Andre; Apellaniz-Campo, Armando

    2017-07-01

    Mozart ear is a rare auricular deformity; clinically the auricle is characterized by the bulging appearance of the anterosuperior portion of the auricle due to fusion of the crura of the antihelix, an inversion in the normal form of the cavum conchae resulting in its convexity and a slit-like narrowing of the orifice of the external auditory meatus.A retrospective review of clinical and photographic records of patients attended at the ear reconstruction clinic of our hospital between June of 2010 and May 2016 was performed; out of 576 consecutive patients only 3 fulfilled the inclusion criteria, with a prevalence of 0.5%. The authors present these patients.Surgical interventions mainly focus on the correction of the convex concha; however, the procedure should be tailored to the severity of the deformity and the wishes of the patient.

  8. [Risk factors for inner ear diseases].

    Science.gov (United States)

    Doroszewska, G; Kaźmierczak, H; Doroszewski, W

    2000-11-01

    The aim of this study was to evaluate the occurrence of obesity and arterial hypertension in patients suffering from vertigo, and/or tinnitus and/or hearing loss of unknown origin. 48 patients (25 women and 23 men) were included into this study. All patients had a negative previous medical history of any metabolic, cardiovascular or neurological disorders. Our results were compared to the control group of 31 healthy persons (16 women and 15 men). All subjects had a complete neurootologic examination, appropriate audiometric and vestibular studies. In most cases inner ear pathology was recognised. BMI, systolic and diastolic blood pressure were measured in all cases. Patients were overweight significantly more often comparing to the control group. Systolic and diastolic hypertension was found significantly more often in men from the patients than control group.

  9. The effect of different sowing depths on fresh ear yield and some ear characteristics of sweet corn

    Directory of Open Access Journals (Sweden)

    Bekir Atar

    2017-12-01

    Full Text Available The research was conducted with aim to investigate effect on fresh ear yield and some ear characteristics of sweet corn of sowing at different depths during 2015 and 2016 years in Isparta. The experiments were set up according to randomized complete block design with three replicates using BATEM TATLI sweet corn cultivar. Furrows were opened at depths of 10 and 20 cm after the soil preparation, and seeds were sown in the 4-5 cm depth in to these furrows. According to means of years, while furrow sowing increased ear diameter, ear weigh, number of kernels per ear and fresh ear yield compared to control, it was not effect on ear length. In the research, between 10 cm and 20 cm furrow sowing wasn’t significant statistically. Fresh ear yield in control, 10 cm and 20 cm furrow sowing were measured as 1110.9 kg ha-1 , 1228.4 kg ha-1 and 1289.4 kg ha-1 , respectively. According to results of research, 5 cm deep sowing in 10 cm furrows should be advised in sweet corn cultivation.

  10. Presbycusis: do we have a third ear?

    Directory of Open Access Journals (Sweden)

    Luis Roque Reis

    Full Text Available Abstract Introduction: Age-related hearing changes are the most frequent cause of sensorineural hearing loss in adults. In the literature no studies exist concerning the importance of speechreading in individuals with presbycusis. Equally, no such studies have been carried out with speakers of the Portuguese (Portugal language. Objectives: To evaluate whether the intelligibility of words in presbycusis is improved by speechreading, in such a way that looking at the interlocutor's face while he is talking functions like a “third ear”, and to determine the statistical relevance of the intelligibility improvement by speechreading. Methods: Eleven individuals (22 ears with bilateral and symmetrical sensorineural hearing loss compatible with presbycusis were evaluated. The subjects were aged between 57 and 82 years, with an average of 70 ± 11.51 years and median of 69.5 years. A complete medical and audiological profile of each patient was created and all patients were submitted to a vocal audiogram, without and with observation of the audiologist's face. A descriptive and analytical statistical analysis was performed (Shapiro-Wilk and t pairs tests adopting the significance level of 0.05 (5%. Results: We noticed better performance in intelligibility with speechreading. The p-value was zero (p < 0.05, so we rejected the null hypothesis, showing that there was statistically significant difference with speechreading; the same conclusion was obtained by analysis of the confidence intervals. Conclusions: Individuals with presbycusis in this study, performed better on spoken word intelligibility when the hearing of those words was associated with speechreading. This phenomenon helps in such a way that observation of the interlocutor's face works like a "third ear".

  11. Inner ear anomalies causing congenital sensorineural hearing loss: CT and MR imaging findings

    International Nuclear Information System (INIS)

    Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan

    2005-01-01

    Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cased had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss

  12. Passage of albumin from the middle ear to the inner ear in otitis media in the chinchilla

    International Nuclear Information System (INIS)

    Goldberg, B.; Goycoolea, M.V.; Schleivert, P.M.; Shea, D.; Schachern, P.; Paparella, M.M.; Carpenter, A.M.

    1981-01-01

    A study of the permeability of the middle ear-inner ear interface for macromolecules was carried out in chinchillas with open and obstructed eustachian tubes utilizing tritiated human serum albumin and immunoelectrophoresis. Tritiated albumin was placed in the round window niche area or normal animals and animals in which the eustachian tubes had been obstructed for 24 hours or 14 days. The tritiated albumin was allowed to remain in the middle ear cavity for 24 hours, Samples of middle ear effusion, perilymph, blood and cerebrospinal fluid were collected and measured for radioactivity. Radioactivity was demonstrated in the perilymph. Samples of middle ear effusions and perilymph were also studied by immunoelectrophoresis with goat antihuman albumin. Albumin placed in the round window niche of an experimental animal could be recovered unchanged in the perilymph. The results suggest a pathophysiologic explanation for the association of otitis media and sensorineural hearing loss or endolymphatic hydrops

  13. ANAESTHETIC MANAGEMENT IN A PATIENT WITH ARNOLD-CHI ARI MALFORMATION TYPE I AND SYRINGOMYELIA

    OpenAIRE

    Kartika; Pratap; Vijayalaxmi; Kalyan Chakravarthy; Nagaraju

    2013-01-01

    ABSTRACT: Syringomyelia is an unusual neurological condition characterised by the the presence of cystic cavity in the spinal cord resultin g in neurological manifestations. Here, we report a safe anesthetic management of patient with Arnold-Ch iari malformation type I and syringomyelia posted for foramen magnum decompression . INTRODUCTION: Arnold-Chiari malformation (ACM) is a developmental malformation characterised by downward displacement of cere...

  14. A rare association of rectal and genitourinary duplication and anorectal malformation

    Institute of Scientific and Technical Information of China (English)

    王俊; 施诚仁; 余世耀; 吴燕; 徐长辉

    2003-01-01

    @@ It is very rare to see multiple malformations occurring in both the urogenital and digestive systems in a case of congenital anorectal malformation. In this particular care, an imperforated anus occurred with other multiple malformations, including a double kidney, urethral duplication and rectal duplication, etc.

  15. Prenatal diagnosis of congenital cystic adenomatoid malformation of the lung: A case report

    International Nuclear Information System (INIS)

    Shin, Hyun Ja; Shin, M. J.; Yoo, Y. J.; Park, J. M.; Kim, J. R.

    1990-01-01

    Congenital cystic adenomatoid malformation is one of a rare congenital malformation usually unilateal in volving a part of lobe or a whole lobe of the fetal lung, characterized by excessive growing of terminal respiratory element. We made a prenatal diagnosis in a case of congenital cystic adenomdtoid malformation with diffuse bilateral involvement, Stocker Type III which is associated with fetal hydrops

  16. Formation and malformation of the enteric nervous system

    NARCIS (Netherlands)

    J.H.C. Meijers (Johan)

    1989-01-01

    textabstractTo clarify pathogenetic mechanisms of congenital malformations of the ENS, the formation of the ENS was investigated in chicken and murine embryos. The experimental work was concentrated on several aspects of the interaction between neural crest cells and the enteric microenvironment.

  17. Congenital malformations in 4224 children conceived after IVF

    NARCIS (Netherlands)

    Anthony, S.; Buitendijk, S. E.; Dorrepaal, C. A.; Lindner, K.; Braat, D. D. M.; den Ouden, A. L.

    2002-01-01

    BACKGROUND: The percentage of children born after IVF will continue to increase due to demographic changes such as increasing maternal age and new developments in assisted reproduction techniques. IVF conceptions may carry an increased risk of congenital malformations. METHODS: We compared overall

  18. Screening for congenital heart malformations in child health centres

    NARCIS (Netherlands)

    R.E. Juttmann (Rikard)

    1999-01-01

    textabstractThe objective of this thesis is to clarify the effectiveness and the efficiency of screening for congenital heart malformations in Dutch child health centres and the possibilities to optimise this prevention programme. To this end the following main questions will be addressed. 1. Does

  19. Surgical Treatment of Dermatomal Capillary Malformations in the Adult Face

    Directory of Open Access Journals (Sweden)

    Yoojeong Kim

    2012-03-01

    Full Text Available BackgroundFacial capillary malformations (CMs rarely recede; they often become darker and raised in proportion to their growth. These malformations may hypertrophy in adulthood, resulting in increased disfigurement and dysfunction. Laser treatment is considered a first-line therapy for focal CMs, but thick wide lesions, which are accompanied by hypertrophy and have a well-circumscribed nodularity, may be treated with surgical excision and reconstruction.MethodsWe retrospectively reviewed the records of 25 consecutive patients who had undergone complete or partial excisions of facial capillary malformations in our unit. After the excisions, the defects that encompassed their facial aesthetic units were subsequently covered by various methods, including primary closures, local flaps, expanded flaps, split-thickness skin grafts, and full thickness skin grafts.ResultsThe data demonstrated satisfactory results and reliability. Our patients were treated without significant complications, and all of the patients were moderately or fully satisfied with the outcome of their surgeries.ConclusionsAmong the many reconstructive options for adult patients with facial capillary malformations, thick split-thickness skin grafts can be a good choice for the coverage of widely excised wounds.

  20. Genetics of Congenital Heart Malformations: Clinical and Molecular Studies

    NARCIS (Netherlands)

    M.W. Wessels (Marja)

    2009-01-01

    markdownabstract__Abstract__ Congenital heart malformations (CHM) are among the most common congenital defects, occurring in 8 out of 1000 live-births. In the past decade significant progress has been made in the identification of genes implicated in the signaling pathways involved in

  1. Cardiac outflow tract malformations in chick embryos exposed to homocysteine

    NARCIS (Netherlands)

    M.J. Boot (Marit); R.P.M. Steegers-Theunissen (Régine); R.E. Poelmann (Robert); L. van Iperen (Liesbeth); A.C. Gittenberger-De Groot (Adriana)

    2004-01-01

    textabstractIncreased homocysteine concentrations have been associated with cardiac outflow tract defects. It has been hypothesized that cardiac neural crest cells were the target cells in these malformations. Cardiac neural crest cells migrate from the neural tube and contribute to the condensed

  2. Cleft lip and palate malformations: essential knowledge for the ...

    African Journals Online (AJOL)

    Cleft lip and palate malformations are a common group of congenital abnormalities, and are therefore frequently encountered by the general practitioner, who is often the primary coordinator in the management of these patients. This is especially true in a South African setting, where specialist treatment is not always readily ...

  3. Magnetic resonance angiography of arteriovenous malformation in the brainstem

    Energy Technology Data Exchange (ETDEWEB)

    Oyama, Hirofumi; Kida, Yoshihisa; Kobayashi, Tatsuya; Tanaka, Takayuki; Iwakoshi, Takayasu; Kai, Osamu; Hirose, Mitsuhiko [Komaki City Hospital, Aichi (Japan)

    1993-11-01

    The magnetic resonance (MR) angiography appearance of arteriovenous malformation (AVM) in the tegmentum and pons is described. The interpeduncular perforating branches of the posterior cerebral artery and median pontine branches of the basilar artery were seen more clearly by MR angiography than by conventional angiography. MR angiography was very useful for the follow-up of AVM after stereotactic radiosurgery. (author).

  4. Magnetic resonance angiography of arteriovenous malformation in the thalamus

    Energy Technology Data Exchange (ETDEWEB)

    Oyama, Hirofumi; Kida, Yoshihisa; Kobayashi, Tatsuya; Tanaka, Takayuki; Iwakoshi, Takayasu; Kai, Osamu; Hirose, Mitsuhiko [Komaki City Hospital, Aichi (Japan)

    1993-11-01

    A comparative study of magnetic resonance angiography and conventional angiography of arteriovenous malformation in the thalamus showed that both methods clearly visualized the feeding arteries: perforating branches of the posterior cerebral artery, posterior choroidal artery and lenticulostriate artery. Draining veins such as the internal cerebral vein were also demonstrated well. (author).

  5. Paraspinal arteriovenous malformation Onyx embolization via an Ascent balloon.

    Science.gov (United States)

    Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F

    2014-04-01

    Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM.

  6. Psychosocial job strain and risk of congenital malformations in offspring

    DEFF Research Database (Denmark)

    Larsen, Allan Boye Vagn; Hannerz, H; Thulstrup, A M

    2014-01-01

    OBJECTIVE: To investigate if maternal exposure to psychosocial job strain at work (high demands and low control) measured by questionnaire early in pregnancy (median week 15) is associated with malformations in the offspring. DESIGN: Population-based cohort study. SETTING: The Danish National Bir...

  7. Tetraphocomelia with the Waardenburg syndrome and multiple malformations.

    Science.gov (United States)

    Wu, Hue-Tsi; Wainwright, Helen; Beighton, Peter

    2009-04-01

    A male infant delivered spontaneously at the 29th week of pregnancy had gross tetraphocomelia and features of the Waardenburg syndrome. There were no relevant factors in the pregnancy nor family history. It is possible that microdeletions or contiguous gene defects are involved in the pathogenesis of these malformations.

  8. Transcatheter arterial ethanol embolization for congenital renal arteriovenous malformations

    International Nuclear Information System (INIS)

    Wang Jingbing; Wang Han; An Xiao; Wang Linchuan; Gao Liqiang; Zhou Zhiguo; Zhang Guixiang

    2010-01-01

    Objective: To discuss the effect and safety of trans-microcatheter arterial embolization with ethanol for the treatment of congenital renal arteriovenous malformations. Methods: Clinical data of 11 patients with congenital renal arteriovenous malformations manifested mainly as gross hematuria were retrospectively analyzed. Selective renal angiography was performed in all 11 patients. After the diagnosis was confirmed, super-selective catheterization of the diseased arteries was carried out and the trans-microcatheter arterial embolization with ethanol was conducted. Results: A total of 12 procedures were completed in 11 patients. The ethanol dose used in one procedure was 5-25 ml. Successful embolization of the congenital renal arteriovenous malformations was obtained in all patients. The gross hematuria disappeared within 24-48 hours after the treatment. Lumbago at treated side, low fever, abdominal distension, nausea, vomiting, etc. occurred within one week and no other serious complications developed. During the follow-up period lasting for 4-96 months, no recurrence of hematuria was observed and the renal function remained normal. Conclusion: Transcatheter arterial ethanol embolization is an economic, safe and effective treatment for congenital renal arteriovenous malformations. (authors)

  9. Congenital pulmonary arteriovenous malformation: a rare cause of ...

    African Journals Online (AJOL)

    Pulmonary arteriovenous malformation (PAVM) is a rare condition in which there is abnormal connection between pulmonary arteries and veins. The disorder usually appears in late childhood or early adult life, with dyspnea on exertion, clubbing or cyanosis. We present two patients with severe cyanosis and their work-up ...

  10. The Profile Of Congenital Malformation Among Newborn Infants In ...

    African Journals Online (AJOL)

    Objective: The study pattern of congenital malformations (CM) among newborn infants in Calabar, South-south Nigeria. Patients and Methods: Medical records of all inborn and out-born neonates who were admitted and treated for CM in University of Calabar teaching Hospital (UCTH) from 1997 to 2006 (10 years) were ...

  11. Congenital malformations in 4224 children conceived after IVF.

    NARCIS (Netherlands)

    Anthony, S.; Buitendijk, S.E.; Dorrepaal, C.A.; Lindner, K.; Braat, D.D.M.; Ouden, A.L. den

    2002-01-01

    BACKGROUND: The percentage of children born after IVF will continue to increase due to demographic changes such as increasing maternal age and new developments in assisted reproduction techniques. IVF conceptions may carry an increased risk of congenital malformations. METHODS: We compared overall

  12. Colostomy for anorectal malformation in a tertiary center in Nigeria ...

    African Journals Online (AJOL)

    Background: Anorectal malformations constitute a wide spectrum of congenital anorectal abnormalities ranging from absent anus to anal membranes and fistulae. It is one major indication for the creation of colostomy in early neonatal life. This study was aimed at o describe and highlight the place of colostomy in the ...

  13. Paediatric ocular and adnexa malformation in Birnin Kebbi ...

    African Journals Online (AJOL)

    Objectives: Congenital eye and adnexa malformations although rare, are however a major challenge to health professionals in terms of diagnosis. There are also emotional, financial and stigmatization challenges experienced by the patients and their relatives. Appropriate management of these patients requires ...

  14. Congenital malformations in 4224 children conceived after IVF

    NARCIS (Netherlands)

    Anthony, S.; Buitendijk, S.E.; Dorrepaal, C.A.; Lindner, K.; Braat, D.D.M.; Ouden, A.L. den

    2002-01-01

    Background: The percentage of children born after IVF will continue to increase due to demographic changes such as increasing maternal age and new developments in assisted reproduction techniques. IVF conceptions may carry an increased risk of congenital malformations. Methods: We compared overall

  15. Pulmonary sequestration with histologic changes of cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Morin, C.; Filiatrault, D.; Russo, P.

    1989-01-01

    Pulmonary sequestration and congenital cystic adenomatoid malformation (CCAM) are two infrequent congenital pulmonary diseases. The combination of these two entities is rare. We report a case where the antenatal ultrasonography showed a left pulmonary mass suggesting CCAM. The US done after birth revealed an aberrant vascularisation. Pathologic examination confirmed the association of both lesions. (orig.)

  16. Chronic intestinal bleeding caused by congenital arteriovenous malformations

    NARCIS (Netherlands)

    Haringsma, J.; Tytgat, G. N.

    1988-01-01

    A case of vascular malformation over the entire length of the colon and small intestine in a 41-year-old male with an almost life-long history of gastrointestinal hemorrhage, is presented. The patient's history, in connection with the findings at colonoscopy and surgery, was highly suggestive of

  17. Anorectal malformations in neonates | Mirza | African Journal of ...

    African Journals Online (AJOL)

    Background: Anorectal malformations (ARM) are associated with congenital anomalies and other risk factors, yielding a poor prognosis, especially in neonatal life. Objectives: This study was performed to identify the congenital anomalies as a factor of poor prognosis (mortality) in such patients. Settings: Department of ...

  18. Valproic Acid Monotherapy in Pregnancy and Major Congenital Malformations.

    NARCIS (Netherlands)

    Jentink, J.; Loane, M.A.; Dolk, H.; Barisic, I.; Garne, E.; de Jong-van den Berg, L.T.W.; Morris, Joan K.

    2010-01-01

    Background: The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited. Methods: We first combined data from eight published cohort studies (1565 pregnancies in which the women

  19. [Multi-channel cochlear implants in patients with Mondini malformation].

    Science.gov (United States)

    Li, Yong-xin; Han, De-min; Zhao, Xiao-tian; Chen, Xue-qing; Kong, Ying; Zheng, Jun; Liu, Bo; Liu, Sha; Mo, Ling-yan; Zhang, Hua; Wang, Shuo

    2004-02-01

    To describe clinical experiences with multi-channel cochlear implantation in patients with Mondini malformation. Among 300 patients who received multi-channel cochlear implants from 1996 to 2002 in Beijing Tongren Hospital, 15 patients were diagnosed with Mondini malformation. A retrospective analysis was performed dealing with the surgical techniques, mapping and rehabilitations characteristics after surgery. 15 patients with normal cochlear structure are consider as control group. Gusher is found more common than the normal cochlear implantation, most of them are serious. The electrodes are inserted in the "cochleostomy" in full length of 13 Patients, 2 pairs of electrodes remains outside of "cochleostomy" in 2 patients. No serious complications occurred after implantation. All patients have auditory sensations. The impedance of the electrodes, the T level, C level and the hearing threshold are similar with the normal cochlear implantation group. The results have no significant difference in compare with normal cochlear group(P > 0.05). Multi-channel cochlear implantation could be performed safely in patients with Mondini malformation. The primary outcome for patients with Mondini malformation are similar to those with normal cochlear structure following the multi-channel cochlear implantation.

  20. Echotomography in the study of the uropathologic malformation in children

    Energy Technology Data Exchange (ETDEWEB)

    Accarino, B; Salvi, V; Maioli, A; Del Vecchio, E

    1985-01-01

    The authors report on the usefulness of ultrasound in the study of uropathology malformations in children. They analize some caracteristic ecotomographic patterns of this pathology and emphasize validity and usefulness of the technique in diagnosis and the follow-up of these cases. 31 refs.

  1. Imaging findings and therapeutic alternatives for peripheral vascular malformations

    International Nuclear Information System (INIS)

    Monsignore, Lucas Moretti; Nakiri, Guilherme Seizem; Santos, Daniela dos; Abud, Thiago Giansante; Abud, Daniel Giansante

    2010-01-01

    Peripheral vascular malformations represent a spectrum of lesions that appear through the lifetime and can be found in the whole body. Such lesions are uncommon and are frequently confounded with infantile hemangioma, a common benign neoplastic lesion. In the presence of such lesions, the correlation between the clinical and radiological findings is extremely important to achieve a correct diagnosis, which will guide the best therapeutic approach. The most recent classifications for peripheral vascular malformations are based on the blood flow (low or high) and on the main vascular components (arterial, capillary, lymphatic or venous). Peripheral vascular malformations represent a diagnostic and therapeutic challenge, and complementary methods such as computed tomography, Doppler ultrasonography and magnetic resonance imaging, in association with clinical findings can provide information regarding blood flow characteristics and lesions extent. Arteriography and venography confirm the diagnosis, evaluate the lesions extent and guide the therapeutic decision making. Generally, low flow vascular malformations are percutaneously treated with sclerosing agents injection, while in high flow lesions the approach is endovascular, with permanent liquid or solid embolization agents. (author)

  2. Umbilical site for temporary colostomy in anorectal malformations: is ...

    African Journals Online (AJOL)

    Umbilical site for temporary colostomy in anorectal malformations: is it cosmetically preferable? Taha Alkhatrawi, Radi Elsherbini and Djamal Ouslimane. Purpose In an attempt to minimize the scars and improve the cosmetic outcome in children, the umbilical site has been chosen for colostomy formation in patients with.

  3. Congenital malformations and maternal occupational exposure to glycol ethers

    NARCIS (Netherlands)

    Cordier, S; Bergeret, A; Goujard, J; Ha, MC; Ayme, S; Calzolari, E; DeWalle, HEK; KnillJones, R; Candela, S; Dale, [No Value; Dananche, B; deVigan, C; Fevotte, J; Kiel, G; Mandereau, L

    Glycol ethers are found in a wide range of domestic and industrial products, many of which are used in women's work environments. Motivated by concern about their potential reproductive toxicity, we have evaluated the risk of congenital malformations related to glycol ether exposure during preg

  4. Radiation dermatitis after spinal arteriovenous malformation embolization: case report

    International Nuclear Information System (INIS)

    Carstens, G.J.; Horowitz, M.B.; Purdy, P.D.; Pandya, A.G.

    1996-01-01

    Few cases of radiation injury related to lengthy interventional neuroradiologic procedures have been reported, although concern has been heightened, as evidenced by a 1994 FDA Public Health Advisory. We report a case of radiation-induced dermatitis in a patient undergoing multiple diagnostic and embolization procedures for treatment of a spinal arteriovenous malformation. (orig.). With 2 figs

  5. Three-dimensional spiral CT of craniofacial malformations in children

    International Nuclear Information System (INIS)

    Binaghi, S.; Gudinchet, F.

    2000-01-01

    Objective. To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial malformations. Materials and methods. Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations, underwent craniofacial spiral CT. 3D reformatting was performed using an independent workstation. Results. 3D CT allowed the preoperative evaluation of 16 patients with craniosynostosis and the post-surgical management of 2 patients. 3D CT clearly depicted malformations of the skull base involving the petrous bone in seven patients (four cases of Goldenhar-Gorlin syndrome, one case of Treacher-Collins syndrome and two cases of Crouzon's disease). Four patients with craniofacial clefts were also evaluated. Radiological findings were confirmed by the clinical and intraoperative findings in all patients that underwent surgical treatment. Movement artefacts and ''Lego effect'' related to abrupt change of cranial vault border were encountered and are discussed. Conclusions. 3D CT of the skull can safely and reliably identify paediatric craniofacial malformations involving bone, and it should be used as morphological mapping to help the surgeon in planning surgical treatment. (orig.)

  6. Congenital Cystic Adenomatoid Malformation of Lung-Rare Case Report

    Directory of Open Access Journals (Sweden)

    N. S. Kamakeri

    2016-10-01

    Full Text Available Congenital cystic adenomatoid malformation of lung associated with Cystic dysplasia of kidney, cystic disease of liver with mixed gonadal dysgenesis is rare and is not reported in literature so far. Hence an attempt is made to present this rarest entity.

  7. Malformations vasculaires au cours du syndrome de Williams ...

    African Journals Online (AJOL)

    Le syndrome de Williams-Beuren est une maladie génétique rare, il associe classiquement une dysmorphie faciale assez spécifique, des malformations cardiovasculaires et un profil neuropsychologique particulier. Nous rapportons les observations de trois enfants atteints du syndrome de Williams-Beuren en insistant ...

  8. Current management of anorectal malformation in Egypt: a survey of ...

    African Journals Online (AJOL)

    Background/aim: Anorectal malformation (ARM) represents a wide spectrum of anomalies. Its management includes various strategies. This survey aims at detecting the current preferences of Egyptian pediatric surgeons regarding the management of ARM. Materials and methods: A survey was circulated individually to the ...

  9. A DESCRIPTIVE STUDY OF FUNGAL INFECTIONS IN CHRONICALLY DISCHARGING EARS

    Directory of Open Access Journals (Sweden)

    Sujatha

    2015-08-01

    Full Text Available BACKGROUND : Chronic Suppurative Otitis Media (CSOM is a disease of multiple aetiology and well known for its persis tence and recurrence inspite of treatment and are the bearbug of otologist, paediatrician and general practitioner. One of the reason s for the refractoriness to treatment and chronicity is coexist ing fungal infection of the ear. OBJECTIVES: Are to find out the prevalence of fungal infections in chronic discharging ears and to identify and isolate the type of fungus prevalent in these ears . MATERIALS AND METHOD S: Tertiary care hospital level descrip tive study was conducted in 50 cases of CSOM with actively discharging ears for a period of one year starting from February 2013. For all the cases aural swabs were collected from the diseased ear and were used for direct microscopic examination in potassi um hydroxide wet mount. Ear swab was cultured on Sabouraud’s dextrose agar plate for fungal cultures. The patient characteristics were prospectively recorded and results were analysed. CONCLUSION : There is high prevalence of coexisting fungal infection in actively discharging ears of CSOM patients

  10. An in vitro model of murine middle ear epithelium.

    Science.gov (United States)

    Mulay, Apoorva; Akram, Khondoker M; Williams, Debbie; Armes, Hannah; Russell, Catherine; Hood, Derek; Armstrong, Stuart; Stewart, James P; Brown, Steve D M; Bingle, Lynne; Bingle, Colin D

    2016-11-01

    Otitis media (OM), or middle ear inflammation, is the most common paediatric disease and leads to significant morbidity. Although understanding of underlying disease mechanisms is hampered by complex pathophysiology it is clear that epithelial abnormalities underpin the disease. There is currently a lack of a well-characterised in vitro model of the middle ear (ME) epithelium that replicates the complex cellular composition of the middle ear. Here, we report the development of a novel in vitro model of mouse middle ear epithelial cells (mMECs) at an air-liquid interface (ALI) that recapitulates the characteristics of the native murine ME epithelium. We demonstrate that mMECs undergo differentiation into the varied cell populations seen within the native middle ear. Proteomic analysis confirmed that the cultures secrete a multitude of innate defence proteins from their apical surface. We showed that the mMECs supported the growth of the otopathogen, nontypeable Haemophilus influenzae (NTHi), suggesting that the model can be successfully utilised to study host-pathogen interactions in the middle ear. Overall, our mMEC culture system can help to better understand the cell biology of the middle ear and improve our understanding of the pathophysiology of OM. The model also has the potential to serve as a platform for validation of treatments designed to reverse aspects of epithelial remodelling that underpin OM development. © 2016. Published by The Company of Biologists Ltd.

  11. THE IDENTIFICATION OF EAR PRINTS USING COMPLEX GABOR FILTERS

    Directory of Open Access Journals (Sweden)

    Alexander A S Gunawan

    2012-05-01

    Full Text Available Biometrics is a method used to recognize humans based on one or a few characteristicsphysical or behavioral traits that are unique such as DNA, face, fingerprints, gait, iris, palm, retina,signature and sound. Although the facts that ear prints are found in 15% of crime scenes, ear printsresearch has been very limited since the success of fingerprints modality. The advantage of the useof ear prints, as forensic evidence, are it relatively unchanged due to increased age and have fewervariations than faces with expression variation and orientation. In this research, complex Gaborfilters is used to extract the ear prints feature based on texture segmentation. Principal componentanalysis (PCA is then used for dimensionality-reduction where variation in the dataset ispreserved. The classification is done in a lower dimension space defined by principal componentsbased on Euclidean distance. In experiments, it is used left and right ear prints of ten respondentsand in average, the successful recognition rate is 78%. Based on the experiment results, it isconcluded that ear prints is suitable as forensic evidence mainly when combined with otherbiometric modalities.Keywords: Biometrics; Ear prints; Complex Gabor filters; Principal component analysis;Euclidean distance

  12. An in vitro model of murine middle ear epithelium

    Directory of Open Access Journals (Sweden)

    Apoorva Mulay

    2016-11-01

    Full Text Available Otitis media (OM, or middle ear inflammation, is the most common paediatric disease and leads to significant morbidity. Although understanding of underlying disease mechanisms is hampered by complex pathophysiology it is clear that epithelial abnormalities underpin the disease. There is currently a lack of a well-characterised in vitro model of the middle ear (ME epithelium that replicates the complex cellular composition of the middle ear. Here, we report the development of a novel in vitro model of mouse middle ear epithelial cells (mMECs at an air–liquid interface (ALI that recapitulates the characteristics of the native murine ME epithelium. We demonstrate that mMECs undergo differentiation into the varied cell populations seen within the native middle ear. Proteomic analysis confirmed that the cultures secrete a multitude of innate defence proteins from their apical surface. We showed that the mMECs supported the growth of the otopathogen, nontypeable Haemophilus influenzae (NTHi, suggesting that the model can be successfully utilised to study host–pathogen interactions in the middle ear. Overall, our mMEC culture system can help to better understand the cell biology of the middle ear and improve our understanding of the pathophysiology of OM. The model also has the potential to serve as a platform for validation of treatments designed to reverse aspects of epithelial remodelling that underpin OM development.

  13. Trends and complications of ear piercing among selected Nigerian population

    Directory of Open Access Journals (Sweden)

    Olajide Toye Gabriel

    2017-01-01

    Full Text Available Background: The reported health and socioeconomic consequences of ear piercing, especially in modern day society, underscore the need to further research into this subject. In this study, we determine the trends and complications of ear piercing among selected Nigerian population. Aim and Objectives: The aim and objective of this study was to draw attention to the trends and complications of ear piercing with a view to prevent its associated complications. Methodology: It is a descriptive cross-sectional study carried out between February and May 2015 among selected Nigerian population from two of its six geo-political zones. A self-administered semi-structured questionnaire which had been pretested was used to collect data from 458 respondents who consented using multistage sampling technique. Results: Of 480 respondents enumerated, 458 completed the questionnaires and gave their biodata. The male:female ratio was 1:6.2. Their ages ranged from 18 to 75 years with a mean of 35.56 ± 10.16. About 35.4% of the respondents were within the age group of 31–40 years. Majority of the respondents, i.e.,79.3% practiced ear piercing on their children. Most of them (86.8% preferred single piercing. Ear piercing was performed within the 1st week of birth in 37.2% of the respondents. Large percentage (93.2% of the respondents will not encourage ear piercing in male children. Nearly 20.5% of the respondents observed complications. Conclusion: Ear piercing remained a common practice in Nigeria, with respondents preferring it on females. Majority of the piercings are done in childhood and by untrained personnel. Keloid formation was the notable complication observed by the respondents. There is a need to increase awareness about the hazards of ear piercings and to enact laws that regulate ear piercings particularly in children which is hereby stretched.

  14. 3D ear identification based on sparse representation.

    Directory of Open Access Journals (Sweden)

    Lin Zhang

    Full Text Available Biometrics based personal authentication is an effective way for automatically recognizing, with a high confidence, a person's identity. Recently, 3D ear shape has attracted tremendous interests in research field due to its richness of feature and ease of acquisition. However, the existing ICP (Iterative Closet Point-based 3D ear matching methods prevalent in the literature are not quite efficient to cope with the one-to-many identification case. In this paper, we aim to fill this gap by proposing a novel effective fully automatic 3D ear identification system. We at first propose an accurate and efficient template-based ear detection method. By utilizing such a method, the extracted ear regions are represented in a common canonical coordinate system determined by the ear contour template, which facilitates much the following stages of feature extraction and classification. For each extracted 3D ear, a feature vector is generated as its representation by making use of a PCA-based local feature descriptor. At the stage of classification, we resort to the sparse representation based classification approach, which actually solves an l1-minimization problem. To the best of our knowledge, this is the first work introducing the sparse representation framework into the field of 3D ear identification. Extensive experiments conducted on a benchmark dataset corroborate the effectiveness and efficiency of the proposed approach. The associated Matlab source code and the evaluation results have been made publicly online available at http://sse.tongji.edu.cn/linzhang/ear/srcear/srcear.htm.

  15. Imaging diagnosis of venous malformation in head and neck

    International Nuclear Information System (INIS)

    Ha, Doo Hoe; Kim, Dong Ik; Suh, Jung Ho; Jung, Tae Sub

    1992-01-01

    The venous malformation in head and neck is a development vascular disease which arises from the arrest in the certain stage of vascular embryogenesis. However, the lesion extends along the fascia and has a tendency to recur after incomplete therapy. Retrospectively, the authors reviewed radiologic studies of 20 patients diagnosed as venous malformation during the last 5 years. The diagnosis was verified by histopathology (5 patients) and direct puncture angiography (15 patients). The radiologic studies included. CT with intravenous contrast injection (20 patients), RI angiography with 99mTc-pyrophosphate (6 patients), and direct puncture angiography (15 patients). Multiplicity of venous malformation was noted in 9 patients. On CT scan, the lesions had lobulated irregular shape, with heterogeneous appearance, showed delayed enhancing characteristics, and had the phleboliths (21 lesions). The venous malformations were located at the masticator space (including masseter muscle) (n = 12), retrobulbar space (n 6), submandibular space (n = 4), paravertebral space (n = 3) and so on. In two cases, the lesions were very extensive involving entire neck and parapharynx. On RI angiography using 99m Tc-pyrophosphate, all of the lesions showed persistent and delayed uptake. With direct puncture angiography the lesions could be classified as acinar pattern (n = 17) and mixed pattern (acinar and saccular) (n = 2). Venous connections were noted in 10 lesions. In conclusion, if a soft tissue mass on head and neck shows a heterogeneous attenuation density with or without calcified phlebolith on CT scan, RI angiography is recommended as a next diagnostic study. If it shows delayed persistent uptake, venous malformation can be suspected. Finally direct puncture angiography can verify the nature and extent of the lesion

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

    Science.gov (United States)

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

    2011-03-01

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

  17. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    Directory of Open Access Journals (Sweden)

    Brouwer Oebele F

    2008-03-01

    Full Text Available Abstract Background Fetal spina bifida aperta (SBA is characterized by a spinal meningomyelocele (MMC and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA with MMC and 6 fetuses with normal cerebral development (22–41 week GA were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US. After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation

  18. Outcome of excision of megarectum in children with anorectal malformation.

    Science.gov (United States)

    Keshtgar, Alireza S; Ward, Harry C; Richards, Catherine; Clayden, Graham S

    2007-01-01

    Megarectum in association with anorectal malformation contributes to chronic constipation and fecal incontinence. Resection of megarectum in anorectal malformation improves bowel function, but neuropathy and poor sphincter quality may affect the outcome of fecal continence adversely. The aim of this study was to evaluate the benefits of resection of megarectum in anorectal malformation and to ascertain the impact of anal sphincter quality and neuropathy on the outcome. We studied 62 children with intractable fecal incontinence after repair of anorectal malformation between January 1991 and January 2005. All patients were investigated with anorectal manometry and anal endosonography under ketamine anesthesia. On endosonography, an intact or scarred internal anal sphincter (IAS) was classified as good and a fragmented or absent IAS as poor. On manometry, a resting anal sphincter pressure equal to or more than 30 mm Hg was classified as good and a lower pressure as poor. Functional assessment of fecal continence was done before and after excision of megarectum using a modified Wingfield scores. Sixteen children had excision of megarectum with median age of 9 years (range, 2-15 years) and postoperative follow-up of 5 years (range, 1-10 years). Seven had formation of antegrade continent enema stoma before excision of megarectum. Children were classified into three groups of anomalies: low (n = 6), intermediate (n = 4), and high (n = 6). All children were incontinent of feces. After excision of megarectum, of the 9 children with good IAS and no neuropathy, 7 became continent of feces. Of the remaining 7 children, 4 had poor IAS and 3 had neuropathy, 5 of whom required an antegrade continent enema stoma to be clean. Excision of megarectum in children who had previous repair of anorectal malformation results in fecal continence in the presence of a good IAS and absence of neuropathy. Patients with a poor IAS or neuropathy will often require artificial means of fecal

  19. The middle ear immune defense changes with age

    DEFF Research Database (Denmark)

    Nielsen, Michelle Christine; Friis, Morten; Martin-Bertelsen, Tomas

    2016-01-01

    of this study was to analyze the relationship between age and the mucosal immune system in the middle ear. It is hypothesized that genes involved in the middle ear immune system will change with age. A comprehensive assessment of these genetic differences using the techniques of complementary DNA has not been...... performed. Complementary DNA microarray technology was used to identify immune-related genes differentially expressed between the normal middle ear mucosa of young (10 days old) and adult rats (80 days old). Data were analyzed using tools of bioinformatics. A total of 260 age-related genes were identified...

  20. Neonatal Hairy Ear Pinnae and Gestational Diabetes: Just a Coincidence?

    Science.gov (United States)

    Valerio, Enrico; Riello, Laura; Chirico, Michela; Semenzato, Rossella; Cutrone, Mario

    2015-01-01

    A newborn girl of 36 weeks gestation was noted to have several anomalies, including bilateral low ear attachment with ear pinnae hypertrichosis, left preauricular pit, micrognathia, short lingual frenulum, and short neck. Pregnancy history revealed poorly controlled maternal gestational diabetes (GD). Localized hypertrichosis of the ear pinnae may represent a potential marker of GD and thereby alert physicians to suspect other potentially GD-associated conditions such as macrosomia, asphyxia, respiratory distress, hypoglycemia, hypocalcemia, hyperbilirubinemia, polycythemia, hypertrophic cardiomyopathy, and congenital anomalies, particularly those involving the central nervous system. © 2015 Wiley Periodicals, Inc.