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Sample records for meningomyelocele

  1. The Cognitive Phenotype of Spina Bifida Meningomyelocele

    Science.gov (United States)

    Dennis, Maureen; Barnes, Marcia A.

    2010-01-01

    A cognitive phenotype is a product of both assets and deficits that specifies what individuals with spina bifida meningomyelocele (SBM) can and cannot do and why they can or cannot do it. In this article, we review the cognitive phenotype of SBM and describe the processing assets and deficits that cut within and across content domains, sensory…

  2. The reading man flap for closure of large meningomyelocele defects.

    Science.gov (United States)

    Mutaf, Mehmet; Temel, Metin; Günal, Ertan

    2012-05-01

    Closure of the skin defect in myelomeningocele repair is an essential step that determines the quality of the surgical result. In large myelomeningoceles, however, adequate skin coverage may not be accomplished by direct closure or skin undermining. In such cases, the skin defect is best repaired using flaps. The aim of this study is to evaluate the reading man procedure for closure of large meningomyelocele defect. In this procedure, after neurosurgical repair and closure of the placode, the defect surgically becomes a circle in shape. Then, the circular defect is closed by transposition of two skin flaps designed in an unequal Z-plasty manner. Over 5 years, the reading man procedure was used for closure of large meningomyelocele defects in seven patients (four females and three males), aged between 1.5 and 6 months. The defect size was 10.5×7.25 cm (8.5×5.3 and 12.6×9.5 cm) on average. The localisation of the lesions was thoracolumbar in two patients and lumbosacral in five patients. In all patients, a successful tension-free one-stage closure was obtained without dog-ear formation. Except for one patient with minimal tip necrosis, healing was uneventful without any complications. There was no patient with late breakdown of the wound during 1.5 years (8 months-4 years) of mean follow-up. The reading man procedure enables the surgeon to achieve a tension-free defect closure of considerably large meningomyeleocele defects. Using two well-vascularised fasciocutaneous flaps, it provides a durable coverage and soft tissue padding over the neural tissues with no suture seam at midline. With these advantages, the Reading Man Procedure seems to be a useful and safe alternative for closure of large meningomyelocele defects. Copyright © 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  3. Intracranial structures of meningomyelocels studied by computed tomography

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    Asada, M.; Tamaki, N.; Matsumoto, S. (Kobe Univ. (Japan). School of Medicine)

    1980-10-01

    We have had experience with forty-two patients with meningomyeloceles; their intracranial structures have been studied and analysed by CT. Six patients (16%) revealed no abnormalities, while thirty-six (82%) had hydrocephalus. All the cases have undergone repair of the meningomyelocele, and thirty-five parties, a shunt operation for hydrocephalus. The CT analysis of the intracranial structures of the meningomyelocele revealed that scaphocephalus was present in 40%, posteriorly dominant ventricular dilatation in the lateral ventricle in 73%, and enlarged massa intermedia in 54%. The following postoperative changes were found: sharp edges of the anterior and posterior horns were found in 81%, prominent dilatation of quadrigeminal and retrothalamic cisterns in 76%, and the interdigitation of the medial cerebral cortex in 69%. In the posterior fossa, a hypoplastic cerebellar tentorium was found in 70% of the cases and a pear-shaped deformity of the upper cerebellum in 62%. The brain stem was enclosed laterally by the anteriorly situated ventral portion of the cerebellum in 74%, and the fourth ventricle was collapsed or narrowed in 76%. An absence of the septum pellucidum was associated in six cases; a quadrigeminal cyst, in two, and a cavum septi pellucidum, in one. CT is a useful and safe apparatus for evaluating the intracranial structures of Arnold-Chiari malformations. Trivial morphological changes, such as the ectopic gray matter, beaking tectum, enlarged accessory commissure, and aqueduct stenosis observed in autopsy cases, are still impossible to demonstrate on CT. It is, however, hoped that with the improvement of the CT image, the sagittal image will become more precise for evaluating the downward displacement of the brain stem and the fourth ventricle.

  4. Effects of Electrical Stimulation, Exercise Training and Motor Skills Training on Strength of Children with Meningomyelocele: A Systematic Review

    Science.gov (United States)

    Dagenais, Liese M.; Lahay, Erin R.; Stueck, Kailey A.; White, Erin; Williams, Lindsay; Harris, Susan R.

    2009-01-01

    This systematic review provides a critical synthesis of research regarding the effects of electrical stimulation, exercise training, and motor skills training on muscle strength in children with meningomyelocele. Nine databases were searched using terms related to meningomyelocele and physical therapy interventions. Of 298 potentially relevant…

  5. Meningomyelocele Repair in a Premature Newborn with Hydrocephalus: Anaesthetic Confronts and Management

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    Sandhya Ghodke

    2017-07-01

    Full Text Available Deficit of neural tube closure in the initial phases of intrauterine development leads to a gamut of abnormalities ranging from spina-bifida occulta, a relatively benign condition, to encephalocele and meningomyelocele, an anomaly in vertebral bodies, spinal cord and sometimes involving brainstem (in cervical meningomyelocele. Meningomyelocele is the most common nonlethal malformation in the spectrum of neural tube deficits. The intrinsic challenges associated with the latter disorder warrants tailor-made approaches for providing anaesthesia to the requisite therapeutic surgical interventions. Pediatric patients pose a set of natural barriers because of their ever budding and maturing neurophysiological status, apart from the central neural disease process. Hence, in order to provide optimal neuro-anaesthetic care, the anaesthesiologist must have the knowledge of the outcomes of various pharmacologic interventions on cerebral aerodynamics apart from his professional experience in pediatric neuroanaesthesia. The current case report accounts for a challenging anaesthetic management in a premature newborn having hydrocephalus and lumbosacral meningomyelocele, presented for surgical repair within four hours of delivery.

  6. MRI in the evaluation of spina bifida patients in the remote period after meningomyelocele repair

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    Williamson, M.R.; Glasier, C.M.; Chadduck, W.M.; Angtuaco, E.J.C.; Binet, E.F.

    1989-07-01

    MRI was performed on 22 patients ranging in age from 3 months to 16 years who had closure of their meningomyelocele shortly after birth. These patients had developed new clinical findings suggestive of spinal cord dysfunction. MRI showed low placement of the spinal cord in all 22. Six patients had lipomas, five had diastematomyelia and six had hydromyelia. Four patients had an obviously dysplastic terminal cord. (orig.).

  7. Spinal extradural inclusion dermoid cyst mimicking pseudomeningocele, appearing after 17 years of meningomyelocele repair

    Science.gov (United States)

    Yerramneni, Vamsi Krishna; Patibandla, M. R.; Venkateswararao, K.; Mudumba, VS

    2013-01-01

    Dermoid cysts are congenital cystic tumors arising from embryonic rests and commonest site is in the thoracic region of the spinal canal. The authors reported a case of dermoid cyst in a 17-year-old boy appearing after lumbar meningomyelocele repair at 2 months of age. The boy presented with 6 months history of gradually progressive globular swelling at the site of previous scar and weakness of the left foot. Preoperatively small extradurally protruding placode was seen attached to the swelling. The swelling was completely excised. At 1 year follow-up patient had improvement in foot weakness with magnetic resonance imaging showing no residual or recurrent lesion. PMID:24082940

  8. [Neurogenic bladder function disorders in patients with meningomyelocele: S2k guidelines on diagnostics and therapy].

    Science.gov (United States)

    Stein, R; Assion, C; Beetz, R; Bürst, M; Cremer, R; Ermert, A; Goepel, M; Kuwertz-Bröking, E; Ludwikowski, B; Michael, T; Pannek, J; Peters, H; Rohrmann, D; Rübben, I; Schröder, A; Trollmann, R; Thüroff, J W; Wagner, W

    2015-02-01

    The treatment of children and adolescents with meningomyelocele has experienced a clear change in the last 30 years. The establishment of pharmacotherapy, clean intermittent catheterization (CIC) and infection prophylaxis have improved the prognosis for patients and have led to new therapeutic strategies. The interdisciplinary cooperation between neonatologists, neurosurgeons, pediatric neurologists, pediatric urologists, pediatric nephrologists, pediatric orthopedists and pediatric surgeons leads to optimization of individualized therapy. These guidelines present definitions and classifications, investigations and timing which are described in detail. The conservative and operative therapy options for neurogenic bladder function disorders are described and discussed with reference to the current literature. The brief overview provides in each case assistance for the treating physician in the care of this patient group and facilitates the interdisciplinary cooperation.

  9. Syringohydromyelia following meningomyelocele surgery - role of metrizamide myelography and computed tomography

    International Nuclear Information System (INIS)

    Stanley, P.; Senac, M.O. Jr.; Segall, H.D.; Park, T.S.

    1984-01-01

    Five children developed symptoms attributable to syringohydromyelia several years following neonatal surgery for lumbosacral meningomyelocele. Metrizamide injection followed by computed tomography (CT) was performed in all five patients. In two patients, there was direct opacification of both the subarachnoid space and the syringohydromyelia. In a further two patients, delayed CT demonstrated late opacification of the central cavity from contrast introduced into the subarachnoid space. In the fifth patient via a suboccipital puncture, there was fortuitous filling of a caudally displaced fourth ventricle communicating with a central cavity within the cord, but there was no visualization of the subarachnoid space. Surgery with decompression and plugging of the obex halted the progression of the disease in all the patients. (orig.)

  10. Velo-Cardio-Facial syndrome and DiGeorge sequence with meningomyelocele and deletions of the 22q11 region

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    Nickel, R.E.; Pillers, D.M.; Merkens, M.; Magenis, R.E.; Zonana, J. [Oregon Health Sciences Univ., Portland, OR (United States); Driscoll, D.A.; Emanuel, B.S. [Univ. of Pennsylvania Medical Center, Philadelphia, PA (United States)

    1994-10-01

    Approximately 5% of children with neural tube defects (NTDs) have a congenital heart defect and/or cleft lip and palate. The cause of isolated meningomyelocele, congenital heart defects, or cleft lip and palate has been largely thought to be multifactorial. However, chromosomal, teratogenic, and single gene causes of combinations of NTDs with congenital heart defects and/or cleft lip and palate have been reported. We report on 3 patients with meningomyelocele, congenital heart defects, and 22q11 deletions. Two of the children had the clinical diagnosis of velo-cardio-facial syndrome (VCFS); both have bifid uvula. The third child had DiGeorge sequence (DGS). The association of NTDs with 22q11 deletion has not been reported previously. An accurate diagnosis of the 22q11 deletion is critical as this micro-deletion and its associated clinical problems is transmitted as an autosomal dominant trait due to the inheritance of the deletion-bearing chromosome. We recommend that all children with NTDs and congenital heart defects, with or without cleft palate, have cytogenetic and molecular studies performed to detect 22q11 deletions. 31 refs., 3 figs.

  11. Prenatal diagnosis of a 22q11 deletion in a second-trimester fetus with conotruncal anomaly, absent thymus and meningomyelocele: Kousseff syndrome.

    Science.gov (United States)

    Canda, Mehmet Tunc; Demir, Namik; Bal, Filiz Uyar; Doganay, Latife; Sezer, Orcun

    2012-04-01

    A 30-year-old nulliparous woman was seen for a detailed ultrasound scan at 20 weeks of gestation. The scan revealed a male fetus with truncus arteriosus, membranous ventricular septal defect, absent thymus and sacral meningomyelocele. A 46,XY karyotype with a 22q11 deletion was detected. The parents chose to terminate the pregnancy. The pathological autopsy showed normal facial structures, minimal ventricular dilatation in the brain and a sacral meningomyelocele. Overlapping toes and a left claw-hand were also noted. An aplastic thymus with absent parathyroid glands was detected. The cardiac examination was consistent with the ultrasound diagnosis. The parental karyotypes were both normal. Kousseff syndrome is caused by a chromosome 22q11 deletion. It includes sacral meningomyelocele and conotruncal heart defects, unlike DiGeorge syndrome. Obstetricians should consider this a not so rare entity when they detect conotruncal abnormalities and a meningomyelocele as part of a 22q11.2 deletion syndrome. © 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology.

  12. Achados oculares em pacientes com mielomeningocele: 72 casos Ocular findings in 72 patients with meningomyelocele

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    Mônica Fialho Cronemberger

    2000-10-01

    Full Text Available Objetivo: Estudar as alterações oculares em pacientes com mielomeningocele. Material e Método: Realizado estudo retrospectivo em 72 pacientes com mielomeningocele, descrevendo as alterações oculares presentes nessa população, além de correlacionar com a presença ou não de hidrocefalia. Resultados: Dos 72 pacientes com mielomeningocele, com idade variando de 5 meses a 18 anos, 64 (88,9% pacientes tinham hidrocefalia. Destes 64 pacientes, 36 (56,3% tinham estrabismo, sendo que 21 (58,3% eram endotrópicos, 13 (36,1% exotrópicos, 1 (2,8% exofórico e 1 (2,8% com estrabismo discinético. Do total dos 72 pacientes estudados 38 (52,8% eram estrábicos. A anisotropia foi encontrada em 16 (22,2% pacientes, sendo que em 14 (87,5% em A e em 2 (12,5% em V. Todos os 16 pacientes com anisotropia apresentavam hidrocefalia. O erro refrativo mais freqüente foi a hipermetropia encontrada em 64 (44,4% olhos. Atrofia óptica foi encontrada em 9 (12,5% pacientes. Conclusão: A mielomeningocele, associada a hidrocefalia, apresenta uma porcentagem de estrabismo maior do que o encontrado na população normal.Purpose: To study ocular findings in patients with meningomyelocele. Methods: A retrospective study of 72 patients with myelomeningocele was performed to evaluate ocular motility disorders, refractive errors and indirect ophthalmoscopy findings. Results: In a group of 72 patients with myelomeningocele, aged 5 month -- 18 years, 64 (88.9% had hydrocephalus. Of this group of 64 patients, 36 (56.3% had strabismus, 21 (58.3% of whom were esotropic, and 13 (36.1% exotropic, 1 (2.8% exophoric and 1 (2.8% presented sometimes esotropia and sometimes exotropia. Of the total of 72 studied patients, 38 (52.8% had strabismus. Anisotropia was found in 16 (22.2% patients, 14 (87.5% with an A pattern and 2 (12.5% with a V pattern. The most frequent refractive error was hyperopia, found in 64 (44.4% eyes. All the 16 patients with anisotropia had hydrocephalus

  13. A Rare Complication of Ventriculo-peritoneal Shunt : The Migration of the Distal End of the Shunt into the Pleural Cavity with Hydrothorax in a Case Meningomyelocele

    International Nuclear Information System (INIS)

    Musharbash, A.

    2003-01-01

    We report a case of a two month old baby boy with an upper lumber meningomyelocele that was repaired one day after delivery and a right posterior parietal ventriculo-peritoneal shunt inserted at the age of two weeks for the development of hydrocephalus. The patient presented with respiratory distress was found to have CSF hydrothorax due to the migration of the distal end of the ventriculo-peritoneal shunt into the pleural cavity. The CSF was infected, this was successfully treated with the removal of the shunt, antibiotics, thoracocentesis for the presistence of the respiratory distress and insertion of a new ventriculo-peritoneal shunt. The presence of an upper lumbar meningomyelocele may play a role in weakness of the diaphragmatic muscle through which the migration of the distal end of the shunt occur, especially if no congenital defect in the diaphragm could be detected. (author) 11 refs . , 2 figs

  14. Perinatal features of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS complex) associated with large meningomyeloceles and severe limb defects.

    Science.gov (United States)

    Chen, C P; Shih, S L; Liu, F F; Jan, S W; Jeng, C J; Lan, C C

    1997-05-01

    Omphalocele-Exstrophy-Imperforate anus-Spinal defects (OEIS complex), a combination of omphalocele, exstrophy of the bladder, an imperforate anus and spinal defects, arises from a single localized defect in the early development of the mesoderm that will later contribute to infraumbilical mesenchyme, cloacal septum, and caudal vertebrae. In this report, we document the perinatal features of two cases of OEIS complex associated with meningomyeloceles and severe lower limb defects, and discuss the prenatal diagnosis, inheritance, and differential diagnosis of this association of malformations. Although long-term survival can be achieved by successful corrective surgery, the associated structural defects such as large meningomyelocele and severe limb aplasia or hypoplasia, as seen in our patient, can influence the patient's quality of life. We would like to emphasize that an accurate prenatal diagnosis of OEIS complex and associated malformations is important for the detailed counseling of the family as well as appropriate perinatal management by the obstetricians, pediatric surgeons, urologists, neurosurgeons, and neonatologists.

  15. Spinal cord anomalies in patients with anorectal malformations without severe sacral abnormalities or meningomyelocele: outcomes after expectant, conservative management.

    Science.gov (United States)

    Kyrklund, Kristiina; Pakarinen, Mikko P; Taskinen, Seppo; Kivisaari, Reetta; Rintala, Risto J

    2016-12-01

    OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed. Bowel function and LUTS were assessed by questionnaire. Patients with extensive sacral anomalies or meningomyelocele were excluded. RESULTS Of 89 patients (median age 15 years, range 5-29 years), MRI was available in 90% (n = 80; 40 male patients with RUF), and 80% of patients returned the questionnaire (n = 64; 31 male patients with RUF). Spinal cord anomalies were found in 34%, comprising a filum terminale lipoma in 30%, low conus medullaris in 10%, and thoracolumbar syrinx in 6%. Bowel functional outcomes between patients with SCAs (n = 23) and those with a normal spinal cord (n = 41) were not significantly different for soiling (70% vs 63%), fecal accidents (43% vs 34%), and constipation (57% vs 39%; p = not significant for all). The LUTS, including urge (65% vs 54%), urge incontinence (39% vs 24%), stress incontinence (17% vs 22%), and straining (32% vs 29%) were also comparable between groups (p = not significant for all). No patients developed lower-limb neurological abnormalities. CONCLUSIONS The results suggest that the long-term functional outcomes for patients with SCAs who had VF/PF and RUF may not differ significantly from patients with the same type of ARMs and a normal spinal cord. The results favor a conservative approach to their management in the absence of abnormal neurological findings in the lower limbs.

  16. Effects of reading goals on reading comprehension, reading rate, and allocation of working memory in children and adolescents with spina bifida meningomyelocele.

    Science.gov (United States)

    English, Lianne; Barnes, Marcia A; Fletcher, Jack M; Dennis, Maureen; Raghubar, Kimberly P

    2010-05-01

    Spina bifida meningomyelocele (SBM) is a neurodevelopmental disorder associated with intact word decoding and deficient text and discourse comprehension. This study investigated the ability to adjust reading in accordance with specified reading goals in 79 children and adolescents with SBM (9-19 years of age) and 39 controls (8-17 years of age). Both groups demonstrated slower reading times and enhanced comprehension when reading to study or to come up with a title than when reading for specific information or for entertainment. For both groups, verbal working memory contributed to comprehension performance in those reading conditions hypothesized to require more cognitive effort. Despite their sensitivity to the goals of reading, the group with SBM answered fewer comprehension questions correctly across all reading goal conditions. The results are discussed in relation to the hypothesized cognitive underpinnings of comprehension deficits in SBM and to current models of text comprehension.

  17. Simplified correction of a meningomyelocele-like defect in the ovine fetus Correção simplificada de um defeito semelhante à meningomielocele em fetos de ovelha

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    Rogério Chaccur Abou-Jamra

    2009-06-01

    Full Text Available PURPOSE: To develop a simplified technique for antenatal correction of a meningomyelocele -like defect in fetal sheep to allow direct skin closure. METHODS: A spinal defect was surgically created at 75 days of gestation in the fetuses of 36 pregnant sheep, 23 survived the surgery. At 102 days gestation, the defect was corrected in 14 cases (9 were left untreated. Skin surrounding the defect was dissected below the dermis to permit its edges to be approximated and sutured, without interposing any material to its edges. An interface material intended to protect the neural tissue from skin adhesion was used and the skin defect was completely closed over it. Pregnancy was allowed to continue up to 138 days gestation, the fetuses were submitted to macroscopic and microscopic analysis. RESULTS: The defect was successfully corrected in 90.9% in the experimental group, and spontaneous closure occurred in 22.3% in the control group (p OBJETIVO: Desenvolver uma técnica simplificada de correção pré-natal de defeito semelhante à mielomeningocele em fetos de ovelha permitindo um fechamento direto da pele. MÉTODOS: Um defeito espinhal foi cirurgicamente criado com 75 dias de gestação, em 36 fetos de ovelha, 23 sobreviveram à cirurgia. Após 102 dias de gestação, o defeito foi corrigido em 14 casos (9 não foram tratados. A pele em volta do defeito foi dissecada abaixo da derme para permitir a aproximação direta das bordas através de sutura, sem a interposição de nenhum material entre a pele. Um material de interface foi colocado entre o tecido neural exposto e a pele, com o objetivo de evitar a adesão da medula à pele, que foi completamente fechada sobre o defeito. A gravidez foi mantida até 138 dias, os fetos foram submetidos a análises macroscópicas e microscópicas. RESULTADOS: O defeito foi corrigido em 90.9% no grupo experimental, e o fechamento espontâneo ocorreu em 22.3% no grupo controle (p < 0.05. A taxa de sobrevivência ap

  18. Uso da prótese vertical expansível de titânio para costela no tratamento da cifose congênita em portadores de mielomeningocele torácica Uso de la prótesis vertical expansible de titanio para costilla en el tratamiento de la cifosis congénita en portadores de mielomeningocele torácico Use of vertical expandable prosthetic of titanium for the rib for treating congenital kyphosis in thoracic meningomyelocele patients

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    Guilherme Rebechi Zuiani

    2009-09-01

    meningomyelocele patients using vertical expandable prosthetic of titanium for the rib (VEPTR. METHODS: a retrospective study of 19 thoracic meningomyelocele and congenital kyphosis patients that were subjected to the VEPTR treatment between October 2005 and October 2008, with radiographic evaluation and immediate post and pre-operative clinical practice. Also, the duration of surgical procedure, the need for blood transfusion and postoperative complications were assessed. RESULTS: the patients' average age was 70 months (from 32 to 130 months. The average follow-up from patients was 13.5 months (from 2 to 26 months. The average duration of the procedure was 117 minutes (variation between 70 and 195 minutes. All children reached trunk balance, 13 of whom had not showed it in the postoperative period. The average of pre-operation kyphosis was 115° (from 80° to 150° and 77° (from 50° to 104° for postoperative, with an average correction percentage of 31.2% (from 1.1 to 61.5%. The previous pre-operative imbalance of trunk was an average 7.9 cm (from 1.0 to 15.5 cm and 3.4 cm (from 0 to 8 cm for post-operative. The average correction of this imbalance was of 50.4% (from 0 to 100%. Regarding weight, in pre-operative the average was 15.4 kg (from 8 to 30 kg and 20.6 kg (from 8.5 to 35 kg for postoperative. The average gain of weight was of 36.6% (from 9.8 to 100%. Five of the 19 patients (26.3% presented postoperative complications. No patient needed blood transfusion. CONCLUSION: the use of VEPTR in thoracic meningomyelocele and congenital kyphosis patients has proven to be an effective and promising alternative for the control of physical deformity in patients with a potential for growth.

  19. Arithmetic Achievement in Children with Cerebral Palsy or Spina Bifida Meningomyelocele

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    Jenks, Kathleen M.; van Lieshout, Ernest C. D. M.; de Moor, Jan

    2009-01-01

    The aim of this study was to establish whether children with a physical disability resulting from central nervous system disorders (CNSd) show a level of arithmetic achievement lower than that of non-CNSd children and whether this is related to poor automaticity of number facts or reduced arithmetic instruction time. Twenty-two children with CNSd…

  20. Idiom Comprehension Deficits in Relation to Corpus Callosum Agenesis and Hypoplasia in Children with Spina Bifida Meningomyelocele

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    Huber-Okrainec, J.; Blaser, S.E.; Dennis, M.

    2005-01-01

    Idioms are phrases with figurative meanings that are not directly derived from the literal meanings of the words in the phrase. Idiom comprehension varies with: literality, whether the idiom is literally plausible; compositionality, whether individual words contribute to a figurative meaning; and contextual bias. We studied idiom comprehension in…

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    African Journals Online (AJOL)

    Rickham PP. The swing of the pendulum: The indications for operating on meningomyelocele. Med J Aust 1976; 2: 743—746. 5. Lorber J. Results of treatment of myelomeningocele: An analy- sis of 524 unselected cases, with special reference to possible selection for treatment. Dev. Med. Child Neurol 1971; 13: 279—. 303.

  2. Muscle ultrasound density in human fetuses with spina bifida aperta

    NARCIS (Netherlands)

    Verbeek, R. J.; van der Hoeven, J. H.; Sollie, K. M.; Maurits, N. M.; Bos, A. F.; den Dunnen, W. F. A.; Brouwer, O. F.; Sival, Deborah

    Background: In fetal spina bifida aperta (SBA), leg movements caudal to the meningomyelocele (MMC) are transiently present, but they disappear shortly after birth. Insight in the underlying mechanism could help to improve treatment strategies. In fetal SBA, the pathogenesis of neuromuscular damage

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

    African Journals Online (AJOL)

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

  4. prenatal ultrasound diagnosis of discordant occipital encephalocele ...

    African Journals Online (AJOL)

    drclement

    old gravida 14, Para 13+1 with a diagnosis of discordant encephalocele multiple (twin) pregnancy made ... 13+1 with five alive, lady referred from an urban maternity in Edo State to the antenatal centre of the University of ... especially trisomy 18 are also associated features. Others are meningo-myeloceles or meningoceles.

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

    African Journals Online (AJOL)

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

  6. The split notochord syndrome with dorsal enteric fistula.

    Science.gov (United States)

    Hoffman, C H; Dietrich, R B; Pais, M J; Demos, D S; Pribram, H F

    1993-01-01

    Split notochord syndrome with dorsal enteric fistula is an extremely rare congenital anomaly that may be associated with meningomyelocele or meningocele, and genitourinary anomalies. This case presented with an additional finding of bladder exstrophy, raising the possibility of a relationship between this syndrome and the OEIS complex.

  7. Involvement & Participation. National Conference on Physical Activity for the Exceptional Individual (11th, San Diego, California, November 19-20, 1982).

    Science.gov (United States)

    Greaves, Edward R.; Richmond, Alan

    This publication contains papers, presented at a conference about physical activities for the exceptional individual, concerning: (1) student interest/motivation; (2) swimming; (3) games; (4) wheelchairs; (5) movement education; (6) physical stress and bone growth; (7) parent involvement; (8) meningomyelocele; (9) blind athletes; (10) Project…

  8. Spinal hemorrhages are associated with early neonatal motor function loss in human spina bifida aperta

    NARCIS (Netherlands)

    Sival, Deborah; Verbeek, R. J.; Brouwer, O. F.; Sollie, K. M.; Bos, A. F.; den Dunnen, W. F. A.

    Background: In spina bifida aperta (SBA), leg movements caudal to the meningomyelocele are present in utero, but they disappear shortly after birth. It is unclear whether leg movements disappear by impact of the neuro-developmental malformation or by superimposed traumatic damage. If superimposed

  9. Magnetic resonance imaging of spinal dysraphism

    Energy Technology Data Exchange (ETDEWEB)

    Akino, Minoru; Isu, Toyohiko; Iwasaki, Yoshinobu; Abe, Hiroshi; Abe, Satoru; Miyasaka, Kazuo; Nomura, Mikio; Saito, Hisatoshi.

    1988-04-01

    Nineteen patients with lumbosacral spina bifida were studied by magnetic resonance imaging (MRI) and were divided into two groups: those with lumbosacral lipoma and those with meningomyelocele. All of the patients with meningomyelocele underwent surgery soon after birth for closure of the skin defect. Whenever possible, examination was not confined to the lumbosacral area but also included the brain and other portions of the spinal cord. Certain similarities and differences in pathology were ascertained in the two groups. The tethered cords were the same in both groups. However, Chiari malformations were observed only in patients with meningomyelocele, and hydrocephalus occurred only in patients with Chiari malformations. Syringomyelia and scoliosis were detected in both groups, but scoliosis was more prevalent in the meningomyelocele group. There appeared to be a correlation between scoliosis and syringomyelia; in five of the seven cases of syringomyelia, the locations of the scoliosis and syringomyelia were the same. With MRI, these complex pathologies, including tethered cord, syringomyelia, scoliosis, Chiari malformations, and hydrocephalus, were easily visualized. The superiority of MRI over conventional X-ray technology has been well established. First, a direct image of the spinal cord is obtained. Second, there is no necessity for injection of contrast material into the intrathecal space. Third, any scanning field is possible. There are also some disadvantages with MRI. First, the spatial resolution is inferior to that of high-resolution computed tomography. Second, MRI cannot provide information concerning bone cortex. Therefore, bone involvement cannot be accurately diagnosed. However, in the assessment of spinal dysraphism, MRI is an excellent diagnostic tool and should be the preferred method of diagnosing spinal dysraphism.

  10. 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

  11. G6PD Deficiency with Arnold-Chiari Malformation.

    Science.gov (United States)

    Verma, Shilpi; Bhatia, Pradeep Kumar; Sharma, Vandana; Sethi, Priyanka; Singh, Yogendra Raj

    2016-11-01

    A neonate with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Arnold-Chiari Malformation (ACM) type 2 underwent lumbar meningomyelocele (MMC) repair. Patients with G6PD deficiency are prone to develop haemolysis following any kind of oxidative stress and in ACM, there is a disturbed cranio-spinal pressure relationship. The neonate was managed under general anaesthesia with propofol for induction as well as for maintenance along with fentanyl and oxygen-nitrous mixture.

  12. Comparative studies of spinal celes in Switzerland, Jamaica, and Nigeria, and possible relationship with environmental pollution

    OpenAIRE

    M.O.N. Ibe

    2009-01-01

    Objective and Background:The need to sanitize our environment from elements hazardous to life on earth is hereby high-lighted, as well as a properly organized and efficiently run prenatal health care system. Clinical Methods and Materials:From 1975 to 2004,17 spinal celes (including 2 meningoceles) were routinely repaired in Imo and Ebonyi States of Nigeria, and 5 in Jam aica,the West Indies; none in Easel, Switzerland. All 20 meningomyeloceles were incontinent of urine and faeces, had se...

  13. [Congenital spinal malformations].

    Science.gov (United States)

    Ertl-Wagner, B B; Reiser, M F

    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.

  14. 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

  15. Imaging of the lumbar spine after diskectomy; Imagerie du rachis lombaire apres discectomie

    Energy Technology Data Exchange (ETDEWEB)

    Laredo, J.D.; Wybier, M. [Hopital Lariboisiere, 75 - Paris (France)

    1995-12-31

    The radiological investigation of persistent or recurrent sciatica after lumbar diskectomy essentially consists of demonstrating recurrent disk herniation. Comparison between plain and contrast enhanced CT or MR examinations at the level of the diskectomy is the main step of the radiological survey. The meanings of the various radiological findings are discussed. Other lesions that may induce persistent sciatica after lumbar diskectomy include degenerative narrowing of the lateral recess, spinal instability, stress fracture of the remaining neural arch, pseudo-meningomyelocele after laminectomy. (authors). 34 refs., 5 tabs.

  16. A case of prenatal diagnosis of fetal hydantoin syndrome by ultrasound

    Directory of Open Access Journals (Sweden)

    Gollop Thomaz Rafael

    1999-01-01

    Full Text Available Fetal hydantoin syndrome (FHS is a set of disruptions occasionally present in fetuses exposed in utero to phenytoin or other anticonvulsants. Administration of phenytoin in early pregnancy may impair proper psychomotor performance expected for children's development. Several combined phenotypic markers delineate the syndrome, but the presence of single clinical signs is more common. There is controversy about the etiology of FHS. Associated disruptions may be related to a deficiency in a detoxifying enzyme (epoxide hydrolase, vascular problems, and/or factors not yet known. Genetic causes are believed to influence susceptibility to the drug. This text reports an unusual pattern of malformations detected in an ultrasound scan (gastroschisis, sacral meningomyelocele, and absence of the right lower limb and in the anatomopathological study (left-side gastroschisis, sacral meningomyelocele, scoliosis, left clubfoot, absence of the right lower limb, and pectus carinatum of a fetus whose mother took phenytoin. These defects may have been provoked by exposure to the drug during embryogenesis. In view of similar malformations observed in cases of prenatal exposure to cocaine, a recognized vasoconstrictor, it is suggested that vascular disruptions of hemodynamic origin constituted the event leading to some of the anomalies caused in the developing embryo. A complication of the chorionic villus sampling procedure, used for cytogenetic analysis, is another possibility.

  17. Neurogenic Bladder

    Directory of Open Access Journals (Sweden)

    Peter T. Dorsher

    2012-01-01

    Full Text Available Congenital anomalies such as meningomyelocele and diseases/damage of the central, peripheral, or autonomic nervous systems may produce neurogenic bladder dysfunction, which untreated can result in progressive renal damage, adverse physical effects including decubiti and urinary tract infections, and psychological and social sequelae related to urinary incontinence. A comprehensive bladder-retraining program that incorporates appropriate education, training, medication, and surgical interventions can mitigate the adverse consequences of neurogenic bladder dysfunction and improve both quantity and quality of life. The goals of bladder retraining for neurogenic bladder dysfunction are prevention of urinary incontinence, urinary tract infections, detrusor overdistension, and progressive upper urinary tract damage due to chronic, excessive detrusor pressures. Understanding the physiology and pathophysiology of micturition is essential to select appropriate pharmacologic and surgical interventions to achieve these goals. Future perspectives on potential pharmacological, surgical, and regenerative medicine options for treating neurogenic bladder dysfunction are also presented.

  18. Retrospective analysis of the use of amniotic membranes and xenografts in spinal surgery and anterior cranial fossa operations

    International Nuclear Information System (INIS)

    Jafri Malim Abdullah

    1999-01-01

    To determine the suitability of amniotic membrane an bovine bone xenografts for the use in spinal surgery and anterior cranial for a generations. Fifteen patients with anterior cranial fossa defects and spinal bone fractures received bovine bone xenografts and 10 patients with meningomyeloceles received amniotic membranes (produced by the Malaysian National Tissue Bank) were analysed retrospectively. Clinical criterias like fever, signs of inflammation, breakdown of graft implant, non specific reaction to the nervous tissue were analysed haematological and radiologically. All patients who received the bovine grafts and amniotic membranes did not show any evidence of inflammation or fever. There were no graft implant breakdowns. There was no radiological or clinical evidence of specific or non specific reaction to the nervous tissue after 12-36 months followup Amniotic membranes and bovine xenografts may be used in the healing and reconstruction of spinal and cranial defects. Despite no evidence of rejection and infection after 36 months, a long term followup is still needed

  19. Successful use of botulinum toxin a in intractable, severe muscle spasms in spinal cord injury: A case report

    Directory of Open Access Journals (Sweden)

    Madhuri A Lokapur

    2017-01-01

    Full Text Available Botulinum toxin is a protein produced by Clostridium botulinum, which inhibits muscle contraction by transiently blocking the release of acetylcholine at the neuromuscular junction. At a neuromuscular junction, the toxin inactivates some of the fusion proteins, such as SNAP-25, syntaxin, or synaptobrevin, which are essential for cellular function. This process involves the temporary inhibition of presynaptic acetylcholine release; consequently, its effects are restricted to motor neurons that depend on the cholinergic transmission (muscular plate, gland innervating cells. Injections of botulinum toxin A have been shown to be useful in the treatment of etiologically diverse types of muscle spasms. Ultrasonography (USG has been used as a guide for confirming muscle fasciculations and also is an effective tool for confirming precise needle positioning and correct drug placement. We describe a case of a 25-year-old man with meningomyelocele and paraparesis with painful muscle spasms in bilateral thighs treated by USG-guided botulinum toxin injection.

  20. [Duplication of the anus and rectum in an adult. Report of 1 case].

    Science.gov (United States)

    Labastida Muñoz, H; Tamayo Pérez, R; Corichi Barceinas, R

    1978-01-01

    It is reported a case of duplication of the anus and rectum. A 48 year old man, had two functioning rectae, one ending in its normal anatomical topography and the functioning but nor functional supernumerary rectum exteriorized in the left gluteal region trough a rudimentary anus. Histological studys demonstrated a rudimentary sphincter in the ectopic anus, and focal carcinamotous transformation of one of the four adenomatous polyps, encountered protruding through the normally located anus. The anal anomaly was associated with partial agenesis of the sacrum, absence of coccyx, sacral anterior meningomyelocele and hypoplasia of the twelve right rib. The frequency of the anomaly, its histology and embriology, as well as its classification within the malformations of the terminal gut, were revised in the medical bibliography. It is concluded that this type of anomaly has not been classified or describe, before.

  1. Arnold Chiari malformation with spina bifida: a lost opportunity of folic Acid supplementation.

    Science.gov (United States)

    Ganesh, Deepa; Sagayaraj, Benjamin M; Barua, Ravi Kumar; Sharma, Nidhi; Ranga, Upasana

    2014-12-01

    In Arnold Chiari (kee-AHR-ee) II malformation elongated cerebellar tonsils are displaced inferiorly through the Foramen Magnum into the upper cervical spinal canal. It is a complex anomaly with skull, dura, brain, spine and cord manifestations. Meningomyelocele is seen in all cases. We present a case of type II Arnold Chiari Malformation diagnosed in utero in a pregnant lady .There was no periconceptional folic acid supplementation. As the role of the Methylene Tetra Hydro Folate Reductase gene polymorphism in neural tube defects is becoming evident, a simple opportunity as folic acid supplementation should not be missed. Folate supplementation as fortification of cereal grains will also prevent other conditions like congenital heart defects, urinary tract anomalies, orofacial defects, limb defects and pyloric stenosis.

  2. Vesicoureteric reflux in children

    Directory of Open Access Journals (Sweden)

    Jameela A Kari

    2013-01-01

    Full Text Available Aim: This study aimed to identify the differences between primary and secondary vesicoureteric reflux (VUR and the effect of associated bladder abnormalities on kidney function. Patients and Methods: We retrospectively reviewed the medical records of children with VUR who were followed up at King Abdulaziz University Hospital from January 2005 to December 2010. The review included results of radiological investigations and kidney function tests. We used Chi-square test for statistical analysis and paired t-test to compare group means for initial and last creatinine levels. Results: Ninety-nine children were included in this study. Twenty (20.2% had primary VUR, 11 had high-grade VUR, while 9 had low-grade reflux. All children with low-grade VUR had normal dimercaptosuccinic acid (DMSA. Renal scars were present in 72% of the children with high-grade VUR. The mean creatinine levels (initial and last for both groups were normal. Seventy-nine (79.8% children had secondary VUR, which was due to posterior urethral valves (PUV (46.8%, neurogenic bladder caused by meningomyelocele (25.3%, non-neurogenic neurogenic bladder (NNB (21.5%, or neurogenic bladder associated with prune belly syndrome (6.3%. Children with NNB, meningomyelocele and PUV had high creatinine at presentation with no considerable worsening of their kidney functions during the last visit. Renal scars were present in 49.4% of the children with secondary VUR. Conclusion: Children with primary VUR and normal bladder had good-functioning kidneys, while those with secondary VUR associated with abnormal bladder caused by NNB, spina bifida or PUV had abnormal kidney functions. DMSA scans were useful in predicting higher grades of VUR in children with primary reflux.

  3. Spina bifida and other neural tube defects.

    Science.gov (United States)

    Northrup, H; Volcik, K A

    2000-01-01

    experimental design. We hope the search for genes involved in the risk and/or development of NTDs will lead to the development of strategies for prevention and treatment. The most recent achievement in treatment of NTDs involves the repair of meningomyelocele through advancements in fetal surgery. Convincing experimental evidence exists that in utero repair preserves neurologic function, as well as resolving the hydrocephalus and Arnold-Chiari malformation that often accompany meningomyelocele defects. However, follow-up is needed to completely evaluate long-term neurologic function and overall improved quality of life. And in the words of Olutoye and Adzick, "until the benefits of fetal [meningomyelocele] repair are carefully elucidated, weighed against maternal and fetal risks, and compared to conventional postnatal therapy, this procedure should be restricted to a few centers that are committed (clinically and experimentally) to investigating these issues."

  4. Desempenho funcional de crianças com mielomeningocele Functional performance of children with myelomeningocele

    Directory of Open Access Journals (Sweden)

    Luanda André Collange

    2008-01-01

    Full Text Available Este trabalho visou analisar o impacto dos níveis de lesão, deambulação e alterações neurológicas associadas ao quadro clínico da mielomeningocele sobre o desempenho funcional de seus portadores. Neste estudo transversal prospectivo foram examinadas 40 crianças com mielomeningocele, avaliando-se nível de lesão, deambulação, malformação de Arnold-Chiari, hidrocefalia, hidromielia, medula ancorada e incontinência esfincteriana. O desempenho funcional foi avaliado pelo Pediatric Evaluation of Disability Inventory (PEDI, para um registro quantitativo da capacidade funcional e autonomia nas atividades cotidianas, nas áreas de autocuidado, mobilidade e função social. Os dados coletados foram analisados estatisticamente, estabelecendo-se o nível de significância em pThis study aimed at analysing the impact of level of lesion, locomotion, and neurological changes associated to meningomyelocele clinical framework concerning functional performance. For this prospective cross-sectional study 40 children with meningomyelocele were assessed as to level of lesion, ambulatory ability, Arnold-Chiari malformation, hydrocephalus, hydromyelia, tethered cord, and sphincter incontinence. Functional performance was evaluated by the Pediatric Evaluation of Disability Inventory (PEDI which quantitatively assesses functional performance and independence in daily activities in the areas of self-care, mobility, and social functioning. Collected data were statistically analysed and the significance level set at p<0.05. Lesion levels and ambulatory ability had a significant (p<0.01 impact on scores of all three PEDI areas. Limitation degree was found to be directly proportional to level of lesion. Functional performance was more severely affected in self-care and mobility activities, and the best functional scores being obtained in social functioning. In patients with thoracic lesion level, symptomatic hydromyelia proved to be a significant factor for

  5. Spinal neural tube defects in Lagos University Teaching Hospital, Nigeria.

    Science.gov (United States)

    Bankole, O B; Arigbabu, S O; Kanu, O O

    2012-01-01

    The incidence of neural tube defects is known to vary among regions. Very little has been reported about the incidence in Sub-Saharan Africa except for the general impression that the prevalent rates are low. To determine the profile of patients presenting with neural tube defects in Lagos, Nigeria We studied all patients with congenital midline back swellings presenting to one of two neurosurgical services in the state over a 5-year period to establish the incidence of spina bifida and develop demographic data. Data collected included the age at presentation, maternal age, education and parity, presence of co-existing anomalies and the social status of the parents. One hundred and eight patients with congenital midline swellings of the back were studied. Meningomyelocele accounted for 96% of the cases seen. Half the patients presented within the first two weeks of life and although fifty percent of mothers had ultrasound scans done during pregnancy none of the patients were diagnosed prenatally. Seventy-three percent of mothers of affected children were from a low socio-economic class. The commonest co-existing congenital anomaly was lower limb deformity (Talipes equino-varus). Spina bifida is the commonest indication for neurosurgical clinic referral with the exception of trauma in our environment. The prevalence is higher among women in the lower socio-economic groups. Improved perinatal care is required to ensure that children with such birth defects get prompt medical attention and thereby prevent worsening of an already complex problem.

  6. Anaesthesia for urgent and emergency surgery.

    Science.gov (United States)

    Brusseau, Roland; McCann, Mary Ellen

    2010-11-01

    The general principles that guide a pediatric anesthesiologist in the care of a newborn needing urgent and emergent neonatal surgery are reviewed. These include careful evaluation and assessment of the newborn including a detailed prenatal history. A brief review of neonatal physiology including the development of the autonomic nervous system and the development of nociceptive pathways is discussed and how important it is to suppress the stress response to surgical intervention in order to improve surgical outcomes. Most of the current general anesthetics have been associated with anesthetic neurotoxicity in juvenile mammals and several epidemiologic studies in human infants and toddlers have linked surgery occurring in the first 3 years of life with neurocognitive delays in school age children. These concerns are discussed in this paper. Practical considerations about neonatal intubation, line placement and intraoperative fluid management are also reviewed. And the anesthetic management of specific neonatal conditions such as congenital diaphragmatic hernias, transesophageal fistulas, gastroschisis and omphaloceles, necrotizing enterocolitis and meningomyeloceles is examined. Copyright © 2010. Published by Elsevier Ireland Ltd.

  7. Obstetric ultrasonographic findings of Chiari type II: Case report

    Directory of Open Access Journals (Sweden)

    Alptekin Tosun

    2009-01-01

    Full Text Available Chiari malformations divided into 3 groups. Chiari type I malformation is caudal protrusion of cerebellar tonsils. Type II malformation is the most common and associate with meningomyelocele. Type III is a high cervical men-ingoencephalocele and uncommon.Spina bifida, has classified into open and closed forms as skin covered spine lesions. Cranial signs are not ac-companiment on closed type. Open type usually diag-nosed on prenatal period. Typical findings are ventricu-lomegaly, lemon sign (bifrontal indentation, banana sign (Chiari II malformation, obliteration of cisterna magna and small BPD and body measurements according to gestation age. Occipital horns are higher than 10 mm in ventriculomegaly. Choroid plexus are small and looking like tear. Limon sign defines biconcave frontal bones as looking like a lemon. Banana sign and obliteration of cis-terna magna resulted cause of hypoplasia of posterior fossa. Compression of cerebellum causing abnormal lo-calization, although cerebellar tonsils and vermis herni-ated to foramen magnum. Hemispheres are wrapping brain stem and looking like ‘‘C’’ (banana sign. Spinal longitudinal sonogram reveals open spine and skin de-fect, although dilatation on spine canal and increased in-terpedincular distance.

  8. The school inclusion and use of assistive technology with myelomeningocele sequelae students: a view of the teachers

    Directory of Open Access Journals (Sweden)

    Caroline Penteado Assis

    2011-06-01

    Full Text Available Myelomeningocele is a congenital malformation that affects the fetus during pregnancy. It can causes numerous consequences for the development of the child and consequently in their schooling process. So, there are several challenges that can be experienced by these children in their inclusion at school. The use of assistive technology in the school context has been implanted with goal to high from the functional abilities of these students. In Brazil are conducted a lot of studies to know the reality of children with myelomeningocele. Therefore, the goal of this study was to investigate, from the teacher ́s perspective of regular and multifunctional class, as was the process of schooling for students with myelomeningocele sequelae and resource utilization of assistive technology in the school context. The participants of this research were seven teachers and four students with myelomeningocele sequels enclosed in regular scholl.The researcher confectioned the instruments used in interviews. Data were analyzed using content of interview analysis. The results reveal that there are present challenges for inclusion of students with myelomeningocele sequelae and that teachers did not use assistive technology resources at school. It is considered that for the real practice of educational inclusion of these students the knowledge about meningomyelocele and various technologies that can be used in the school context must be published.

  9. 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.)

  10. OEIS complex with glomerulocystic kidney disease: a case report.

    Science.gov (United States)

    Hong, Ran; Lim, Sung-Chul; Jang, Jung-Whan; Suh, Chae-Hong; Jeon, Ho-Jong; Lee, Mi-Ja; Kim, Youn-Shin

    2007-01-01

    We present a case of OEIS complex (omphalocele, exstrophy of bladder, imperforated anus, spinal defect) combined with colonic agenesis and glomerulocystic kidney disease (GCKD). The baby was born at 35.2 weeks of gestational age, weighing 2.51 kg. A prenatal ultrasound examination showed spina bifida, hydroureter, and a unilateral polycystic kidney. The postdelivery examination, which included a physical examination, simple X-ray, and pelvic MRI, showed a lower abdominal wall defect through which a small pouch with a segment of bowel protruded, imperforated anus, ambiguous external genitalia, spina bifida with meningomyelocele at the lumbosacral junction, and nonunion of pubic symphysis. The baby underwent surgery, including nephrectomy, colostomy, and repair of the abdominal wall defect. In addition to the abnormalities mentioned, a tailgut as a result of colonic agenesis and 2 appendices were identified in the course of surgery. The result of histopathological examination confirmed the polycystic kidney identified as GCKD. These radiological, surgical, and histopathologic findings are consistent with the OEIS complex. The postoperative course was uneventful during a period of 4 months of follow up. We herein report a case of the very rare OEIS complex in a newborn male baby and review the available literature.

  11. 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.

  12. Fatores de risco para meningite bacteriana no recém-nascido Risk factors for bacterial meningitis in the newborn

    Directory of Open Access Journals (Sweden)

    Vera Lúcia Jornada Krebs

    2004-09-01

    Full Text Available O objetivo do estudo é descrever os fatores de risco para meningite bacteriana em recém-nascidos e analisar a prevalência destes fatores, considerando-se a presença ou não de baixo peso ao nascimento. Foram analisados 50 recém-nascidos com meningite bacteriana, excluindo-se aqueles com meningomielocele ou infecção congênita. Na análise estatística utilizou-se o teste exato de Fisher, considerando-se significantes os valores de p The aim of this study is to describe the risk factors for bacterial meningitis in newborns, and to analyze the prevalence of these factors, considering or not the low birth weight presence. Fifty newborns with bacterial meningitis were analyzed, excluding the ones with meningomyelocele or congenital infection. In the statistical analysis, the Fisher's exact test was used, considering significant the p < 0.05 values. This study has shown that prematurity, low birth weight and presence of previous infectious diseases in the newborn or in the mother were important risk factors for meningitis. Among low birth weight newborns, invasive procedures, especially tracheal intubation, use of central venous catheter and previous use of antibiotics, were significantly associated to the meningitis occurrence. These results indicate that the improvement in the prenatal care and in the hospital infection control are measures of high importance in the decrease of the incidence of neonatal bacterial meningitis.

  13. Sustained Attention in Children With Two Etiologies of Early Hydrocephalus

    Science.gov (United States)

    Swartwout, Maegan D.; Cirino, Paul T.; Hampson, Amy W.; Fletcher, Jack M.; Brandt, Michael E.; Dennis, Maureen

    2008-01-01

    Several studies have shown that children with spina bifida meningomyelocele (SBM) and hydrocephalus have attention problems on parent ratings and difficulties in stimulus orienting associated with a posterior brain attention system. Less is known about response control and inhibition associated with an anterior brain attention system. Using the Gordon Vigilance Task (Gordon, 1983), we studied error rate, reaction time, and performance over time for sustained attention, a key anterior attention function, in 101 children with SBM, 17 with aqueductal stenosis (AS; another condition involving congenital hydrocephalus), and 40 typically developing controls (NC). In SBM, we investigated the relation between cognitive attention and parent ratings of inattention and hyperactivity and explored the impact of medical variables. Children with SBM did not differ from AS or NC groups on measures of sustained attention, but they committed more errors and responded more slowly. Approximately one-third of the SBM group had attention symptoms, although parent attention ratings were not associated with task performance. Hydrocephalus does not account for the attention profile of children with SBM, which also reflects the distinctive brain dysmorphologies associated with this condition. PMID:18999350

  14. Respiratory depression during VP shunting in Arnold Chiari malformation Type-II, a rare complication (Case reports and review of literature).

    Science.gov (United States)

    Sahu, Sandeep; Lata, Indu; Srivastava, Vineet; Gupta, Davendra

    2009-01-01

    The VP Shunt is a common pediatric surgical procedure in our country. Hydrocephalus is commonly associated with meningomyelocele in Arnold Chiari malformation-II and the ventriculoperitoneal shunt insertion is the common surgical procedure for the management of hydrocephalus. The standard protocol is to rule out any hydrocephalus by preoperative MRI. If associated with hydrocephalus, insertion of the VP shunt is indicated before the repair of MMC whereas the absence of hydrocephalus indicates that the surgical repair of MMC is to be undertaken immediately. Anesthetic management of the patient during the insertion of ventriculoperitoneal shunt may pose problems. We report here two cases of ACM-II (lumbar MMC with associated hydrocephalus) who had respiratory depression / delayed emergence after an otherwise uneventful procedure. Although the VP shunt (first procedure) required postoperative ventilation which improved later, the phenomena of respiratory depression / delayed emergence did not occur after the MMC repair (second surgery). The possible mechanisms involved in these events and their various clinical aspects are discussed below.

  15. Arnold–Chiari malformation type 1 complicated by sudden onset anterior spinal artery thrombosis, tetraparesis and respiratory arrest

    Science.gov (United States)

    Wani, Abdul Majid; Zayyani, Najah R; Al Miamini, Wail; Khoujah, Amer M; Alharbi, Zeyad; Diari, Mohd S

    2011-01-01

    Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of cerebellar tissue posterior to the medulla and cord that extends into the cervical spinal canal; (2) caudal displacement of the medulla and the inferior part of the fourth ventricle into the cervical canal; and (3) a frequent but not invariable association with syringomyelia or a spinal developmental abnormality. Chiari recognized four types of abnormalities. Presently, the term has come to be restricted to Chiari’s types I and II, that is, to cerebellomedullary descent without and with a meningomyelocele, respectively. The association of Arnold–Chairi malformation and high cervical cord infarction is unusual. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischaemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, postsurgical, sepsis, hypotension and thromboembolic disorders. The authors present an interesting case of cervical cord infarction due to anterior spinal artery thrombosis in a patient of type 1 Arnold–Chiari malformation without any of the above predisposing factors. PMID:22701030

  16. Respiratory depression during VP shunting in Arnold Chiari malformation Type-II, a rare complication (Case reports and review of literature)

    Science.gov (United States)

    Sahu, Sandeep; Lata, Indu; Srivastava, Vineet; Gupta, Davendra

    2009-01-01

    The VP Shunt is a common pediatric surgical procedure in our country. Hydrocephalus is commonly associated with meningomyelocele in Arnold Chiari malformation-II and the ventriculoperitoneal shunt insertion is the common surgical procedure for the management of hydrocephalus. The standard protocol is to rule out any hydrocephalus by preoperative MRI. If associated with hydrocephalus, insertion of the VP shunt is indicated before the repair of MMC whereas the absence of hydrocephalus indicates that the surgical repair of MMC is to be undertaken immediately. Anesthetic management of the patient during the insertion of ventriculoperitoneal shunt may pose problems. We report here two cases of ACM-II (lumbar MMC with associated hydrocephalus) who had respiratory depression / delayed emergence after an otherwise uneventful procedure. Although the VP shunt (first procedure) required postoperative ventilation which improved later, the phenomena of respiratory depression / delayed emergence did not occur after the MMC repair (second surgery). The possible mechanisms involved in these events and their various clinical aspects are discussed below. PMID:21887176

  17. Arnold-Chiari malformation type 1 complicated by sudden onset anterior spinal artery thrombosis, tetraparesis and respiratory arrest.

    Science.gov (United States)

    Wani, Abdul Majid; Zayyani, Najah R; Al Miamini, Wail; Khoujah, Amer M; Alharbi, Zeyad; Diari, Mohd S

    2011-04-15

    Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of cerebellar tissue posterior to the medulla and cord that extends into the cervical spinal canal; (2) caudal displacement of the medulla and the inferior part of the fourth ventricle into the cervical canal; and (3) a frequent but not invariable association with syringomyelia or a spinal developmental abnormality. Chiari recognized four types of abnormalities. Presently, the term has come to be restricted to Chiari's types I and II, that is, to cerebellomedullary descent without and with a meningomyelocele, respectively. The association of Arnold-Chairi malformation and high cervical cord infarction is unusual. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischaemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, postsurgical, sepsis, hypotension and thromboembolic disorders. The authors present an interesting case of cervical cord infarction due to anterior spinal artery thrombosis in a patient of type 1 Arnold-Chiari malformation without any of the above predisposing factors.

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

    Directory of Open Access Journals (Sweden)

    Rafael Fabiano M. Rosa

    2009-09-01

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

  19. Spinal sonography in newborns and infants - part II: spinal dysraphism and tethered cord.

    Science.gov (United States)

    Deeg, K-H; Lode, H-M; Gassner, I

    2008-02-01

    Patients with cutaneous markers in the lumbo-sacral region as well as infants with bladder and bowel dysfunction, orthopedic anomalies and progressive neurological dysfunction are at risk for spinal dysraphism and tethered cord. Three types of spinal dysraphism can be distinguished: Type I - open spinal dysraphisms with a non-skin covered back mass; type II - closed spinal dysraphisms with a skin covered back mass; type III - occult spinal dysraphisms without a back mass. All spinal dysraphisms can be associated with a tethered cord, characterized by a low position of the conus medullaris below L3. Type I dysraphisms are meningomyeloceles and myeloceles, which are associated with CHIARI-II malformations characterized by the low position of the cerebellar vermis within the foramen magnum. Type II dysraphisms are lipomyeloceles, lipomyelomeningoceles, posterior meningoceles and myelocystoceles. Lipomeningoceles and lipomyelomeningoceles are characterized by a subcutaneous echogenic mass which communicates with the spinal canal and may cause tethered cord. Posterior meningoceles are, dorsal cystic space occupying lesions without internal neural tissue. Myelocystoceles are characterized by a cystic dorsal mass which communicates with a dilated central canal characteristic of syringo-hydromyelia. Type III dysraphisms without a back mass are frequently associated with cutaneous markers in the lumbo-sacral region. Sonographically dermal sinus tracts, diastematomyelia, tight filum and lipoma of the filum terminale and the caudal regression syndrome have to be distinguished. Dermal sinuses are characterized by an echogenic tract from the skin to the spinal canal, often associated with a spinal dermoid. Diastematomyelia is characterized by a complete or partial duplication of the spinal cord which can only be shown on axial images. Tight filum terminale or lipoma of the filum terminale is characterized by a thick echogenic filum with a diameter of more than 2 mm, and a conus

  20. Malformation/dysplasia syndrome (neural tube defect, hypospadias neuroblastoma) associated with an extra dicentric marker chromosome 15 ({open_quotes}inversion duplication 15{close_quotes})

    Energy Technology Data Exchange (ETDEWEB)

    Reitnauer, P.J.; Rao, K.W.; Tepperberg, J.H. [Univ. of North Carolina, Chapel Hill, NC (United States)

    1994-09-01

    Extra dicentric 15 marker chromosomes are associated with variable degrees of mental retardation but not major structural birth defects. We have studied a unique patient, a male infant who was prenatally diagnosed with lumbar meningomyelocele and an extra pseudodicentric marker chromosome: 47,XY,+psu dic(15)t(15;15)(?q12,?q12)mat. Hairy ears and a coronal hypospadias were noted at birth. At three months of age, a stage I thoracic neuroblastoma was primarily resected. Tumor cells, skin fibroblasts and peripheral blood lymphocytes contained the dicentric 15. The mother is mosaic for the marker chromosome. Fluorescence in situ hybridization (FISH) studies using a classic 15 satellite probe (D15Z1 [Oncor]) confirmed the presence of 2 number 15 centromeres in the marker. The marker is felt to be the result of a translocation rather than an inverted duplication because the G-band morphology of the short arm/satellite complexes differ from one another, implying that the arms originate from 2 different number 15s. FISH analysis using cosmid probes for the Prader-Willi/Angelman critical region (D15S11 and GABRB3 [Oncor]) revealed 2 copies of this region, indicating that these loci are duplicated in the marker. Although some features of the patient`s phenotype such as developmental delay and hypotonia have been associated with dicentric chromosome 15 markers, this is the first malformation/dysplasia syndrome or neuroblastoma reported to our knowledge. The association of neuroblastoma with chromosome 15 aberrations in this case provides speculation as to the role of chromosome 15 loci in cell division control.

  1. Sonographic pattern of hydrocephalus among the under five children in Sokoto North Western Nigeria

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    Sule Ahmed Saidu

    2015-01-01

    Full Text Available Background: Hydrocephalus among children is an important medical problem in view of its neurological sequelae in the growing child. This situation is compounded by the acute shortage of neurosurgeons in third world countries like Nigeria; hence, the need for its early detection and proper management. Objective: Evaluation of the ultrasound (US appearances in children under 5 years of age presenting with clinical signs of raised intracranial pressure suggestive of hydrocephalus. Patients and Methods: A retrospective review of transfontanelle US scans done in 64 children (39 boys and 25 girls attending the Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria over a period of 2 years was carried out. The patients had a mean age of 5.0 ± 4.6 months (range: 1–60 months at the time of their US examination. Some of the clinical indications for US scan included: Congenital hydrocephalus, encephalocele, meningomyelocele, and meningitis. All scans were performed through the anterior fontanelle using SIUI Apogee 800 PLUS scanner with a curvilinear probe using multi-frequency transducer of 2–5 MHz. Results: Fifty-two patients (81.3% had hydrocephalus of congenital origin. Eleven cases (17.2% had postmeningitic hydrocephalus while only 1 case (1.6% was posthemorrhagic. Twenty-five patients (48.0% of the congenital cases were due to cerebral aqueduct stenosis. Eleven (21.2% of the congenital cases were from obstruction at the exit foramina of Luschka and Magendie resulting in the communicating type of hydrocephalus. Conclusion: Hydrocephalus is a known cause of neurological morbidity among infants in developing countries. Majority of the cases are congenital in origin and most commonly due to cerebral aqueduct stenosis. Transfontanelle US is cheap, affordable, nonhazardous, and more accessible than other imaging modalities. It should serve as the first-line investigation of infants with suspected hydrocephalus for early

  2. The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis

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    Breysem, L.; Bosmans, H.; Dymarkowski, S.; Demaerel, P.; Vanbeckevoort, D.; Smet, M. [Department of Radiology, University Hospitals, Herestraat 49, 3000, Leuven (Belgium); Schoubroeck, D.Van; Witters, I.; Deprest, J. [Department of Obstetrics and Gynecology, University Hospitals, Herestraat 49, 3000, Leuven (Belgium); Vanhole, C.; Casaer, P. [Department of Pediatrics, University Hospitals, Herestraat 49, 3000, Leuven (Belgium)

    2003-07-01

    The aim of this study was to evaluate the role of MR imaging of the fetus to improve sonographic prenatal diagnosis of congenital anomalies. In 40 fetuses (not consecutive cases) with an abnormality diagnosed with ultrasound, additional MR imaging was performed. The basic sequence was a T2-weighted single-shot half Fourier (HASTE) technique. Head, neck, spinal, thoracic, urogenital, and abdominal fetal pathologies were found. This retrospective, observational study compared MR imaging findings with ultrasonographic findings regarding detection, topography, and etiology of the pathology. The MR findings were evaluated as superior, equal to, or inferior compared with US, in consent with the referring gynecologists. The role of these findings in relation to pregnancy management was studied and compared with postnatal follow-up in 30 of 40 babies. Fetal MRI technique was successful in 36 of 39 examinations and provided additional information in 21 of 40 fetuses (one twin pregnancy with two members to evaluate). More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. This improved parental counseling and pregnancy management in 15 pregnancies. In 3 cases, prenatal MRI findings did not correlate with prenatal ultrasonographic findings or neonatal diagnosis. The MRI provided a more detailed description and insight into fetal anatomy, pathology, and etiology in the vast majority of these selected cases. This improved prenatal parental counseling and postnatal therapeutic planning. (orig.)

  3. The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis

    International Nuclear Information System (INIS)

    Breysem, L.; Bosmans, H.; Dymarkowski, S.; Demaerel, P.; Vanbeckevoort, D.; Smet, M.; Schoubroeck, D.Van; Witters, I.; Deprest, J.; Vanhole, C.; Casaer, P.

    2003-01-01

    The aim of this study was to evaluate the role of MR imaging of the fetus to improve sonographic prenatal diagnosis of congenital anomalies. In 40 fetuses (not consecutive cases) with an abnormality diagnosed with ultrasound, additional MR imaging was performed. The basic sequence was a T2-weighted single-shot half Fourier (HASTE) technique. Head, neck, spinal, thoracic, urogenital, and abdominal fetal pathologies were found. This retrospective, observational study compared MR imaging findings with ultrasonographic findings regarding detection, topography, and etiology of the pathology. The MR findings were evaluated as superior, equal to, or inferior compared with US, in consent with the referring gynecologists. The role of these findings in relation to pregnancy management was studied and compared with postnatal follow-up in 30 of 40 babies. Fetal MRI technique was successful in 36 of 39 examinations and provided additional information in 21 of 40 fetuses (one twin pregnancy with two members to evaluate). More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. This improved parental counseling and pregnancy management in 15 pregnancies. In 3 cases, prenatal MRI findings did not correlate with prenatal ultrasonographic findings or neonatal diagnosis. The MRI provided a more detailed description and insight into fetal anatomy, pathology, and etiology in the vast majority of these selected cases. This improved prenatal parental counseling and postnatal therapeutic planning. (orig.)

  4. Pediatric Hydrocephalus in Ethiopia: Treatment Failures and Infections: A Hospital-Based, Retrospective Study.

    Science.gov (United States)

    Laeke, Tsegazeab; Tirsit, Abenezer; Biluts, Hagos; Murali, Deeptha; Wester, Knut

    2017-04-01

    Treatment of pediatric hydrocephalus in sub-Saharan countries is associated with significant complication rates. The objective of this study is to analyze the management of hydrocephalus and complication rates of surgical intervention in the Ethiopian setting to improve future quality. A retrospective cohort study was conducted in a neurosurgical teaching hospital. Two cohorts separated by 2.5 years were analyzed. A total of 128 (58.6% male and 41.4% female) children with isolated hydrocephalus, meningomyelocele (MMC)-related hydrocephalus, or MMC without hydrocephalus were included. Their age ranged from 1 day to 5 years, for a mean age of 7.2 months (median age 2 months). One hundred thirteen patients had hydrocephalus, of whom 57 (44.5.3%) had isolated hydrocephalus and 56 (43.8%) had hydrocephalus associated with MMC. Seventy-seven (74.7%) patients underwent ventriculoperitoneal (VP) shunting, whereas 24 (23.3%) underwent endoscopic third ventriculostomy (ETV). The incidence of shunt infection was 23.4%. Reoperation was needed in 54 (52.4%) patients, with the most common indication being shunt failure. ETV failed in 14 (58.3%) of the 24 patients undergoing ETV. VP shunt insertions had unacceptably high infection rate despite the presence of a protocol for the procedure. Intraoperative guidelines should be developed further and followed strictly to reduce infections. Such measures should include restricting the number of surgeons performing the procedure. In our opinion, one should avoid insertion of a VP shunt as the primary treatment. ETV has proved to be a good alternative in other studies and the decreasing pattern of ETV failure in our study also suggest ETV as a better alternative to VP shunt. Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

  5. Diagnostic value of CA 19-9 in pregnancies complicated by spinal neural tube defects: a preliminary study.

    Science.gov (United States)

    Timur, Hakan; Tokmak, Aytekin; Yucel, Aykan; Ali Inal, Hasan; Buyukkagnici, Umran; Sirvan, Levent; Danisman, Nuri

    2016-01-01

    Various physiological and pathological conditions can induce significant variations in plasma concentrations of tumor markers, such as CA 19-9, which is present in the serum and amniotic fluid of pregnant women. Herein, we aimed to determine the clinical importance of maternal serum CA 19-9 levels in the diagnosis of neural tube defects (NTDs). A total of 100 women were included in this controlled cross-sectional study. Thirty-three patients whose pregnancies were complicated by isolated meningocele or meningomyelocele constituted the study group, whereas 33 normal, healthy pregnant women constituted the control group, and 34 age- and body mass index (BMI)-matched non-pregnant women were chosen for the validation group. The mean maternal serum CA 19-9 levels were 17.2 ± 17.0 IU/mL, 7.1 ± 5.9 IU/mL, and 4.7 ± 3.6 IU/mL in the study, control, and validation groups, respectively (p < 0.001). ROC analyses showed that elevated CA 19-9 values may predict NTDs (p < 0.001). The cut-off value for CA 19-9 was found to be 9.6 IU/mL at 70% (51%-84%, 95% CI) sensitivity and 84% (74%-92%, 95% CI) specificity. CA 19-9 may be a promising noninvasive marker for NTDs. Further studies are needed to reveal the clinical applicability and diagnostic potential of maternal serum CA 19-9 levels in the identification of NTDs.

  6. Nasogastric tube as abdominal pressure sensor in urodynamics-Proof of concept of a novel approach.

    Science.gov (United States)

    VijayGanapathy, Sundaramoorthy; Karthikeyan, Vilvapathy Senguttuvan; Mallya, Ashwin; Poonawala, Ali; Keshavamurthy, Ramaiah

    2018-02-01

    The standard sensor for abdominal pressure (Pabd) measurement in urodynamics (UD) is a rectal sensor. In patients where the rectum is not available due to prior surgery or when external anal sphincter (EAS) tone is poor, rectal sensor may slip, making Pabd recording unreliable. Vaginal Pabd measurement and wireless vaginal sensors have been tried. We present our preliminary series of a novel nasogastric tube (NG) sensor for Pabd measurement. We identified patients undergoing UD with a NG Pabd sensor from a prospectively maintained UD database of a tertiary care urological center between July 2013 and December 2016. Out of 1325 urodynamic procedures done, 46 (3.5%) were performed using NG Pabd sensor. The median (IQR) age was 44 (12) years. Indications for UD in these patients were neurogenic bladder in 22 (47.8%), urinary retention in 17 (37%), post-meningomyelocele repair in four (8.7%), traumatic paraplegia in two (4.3%), and cervical myelopathy in one (2.2%). The indications for NG Pabd sensor were lax EAS tone (40; 86.9%), post-abdominoperineal resection (2; 4.3%), and painful thrombosed hemorrhoids (4; 8.7%). It was possible to make definitive urodynamic diagnosis in all patients using NG Pabd sensor. Initial calibration and NG Pabd excursions throughout the study were similar to that of rectal Pabd sensor. There were no problems with NG tube tolerance. Use of nasogastric sensor is feasible, accurate, cost-effective, and viable alternative for Pabd measurement in patients with poor anal tone or absent rectum due to postoperative status. © 2017 Wiley Periodicals, Inc.

  7. Neural Tube Defect in Alive Neonates: Incidence Rate and Predisposing Factors

    Directory of Open Access Journals (Sweden)

    F Haghollahi

    2008-06-01

    Full Text Available Background: Neural Tube Defect (NTD characterized by failure of neural tube to close properly be the second most common born defect after congenital heart disease. The most prevalent forms of NTD are Anencephaly and Spinal-bifida. Many factors are involved in this anomaly. New researches suggest environmental factors like radiation, hyperthermia, Vitamin A and acid folic deficiency, anti epileptic drug like Carbamazepine, Phenobarbital, phenytoin, Folic acid antagonist like Sulfasalazine, Triametherine and systemic disease like diabet mellitus, obesity, genetic factors, the most schance 40 to 70 percentages.Methods: In this survey cross sectional study was conducted in five hospitals depend to Tehran university during three years. Study subject identified through review of admission and discharge at major hospital through regular contact with newborn nurseries and birth hospital.Results: In 38473 reported cases, 143 cases have neural tube defect. Among NTD cases, 11.9% of mothers had medical diseases in their previous history such as diabetes mellitus, epilepsy-psychiatric, and disorder-heart diseases. In this study group, 5.6% have preclampsia during pregnancy period. The most common NTD anomaly in this study was anencephaly and meningomyelocele that was different from studies in literature.Conclusion: NTD result from failure of neural tube close threats fetus health up to 28 days after conception. When is often prior to the recognition of pregnancy since many pregnancy are unplanned NTD prevention is best achieve by adequate daily folic acid intake thought of reproductive ages .educational effort to promote daily intake of folic acid supplemental by women of reproductive age and NTD risk factor should be done. Early diagnostic procedure for high risk pregnancy advised.

  8. Early onset scoliosis with intraspinal anomalies: management with growing rod.

    Science.gov (United States)

    Jayaswal, Arvind; Kandwal, Pankaj; Goswami, Ankur; Vijayaraghavan, G; Jariyal, Ashok; Upendra, B N; Gupta, Ankit

    2016-10-01

    To evaluate clinical and radiological outcomes of growing rod (GR) in the management of Early Onset Scoliosis (EOS) with intraspinal anomalies. The effect of repeated distractions following GR, in the presence of intraspinal anomalies has not been studied. During 2007-2012, 46 patients underwent fusionless surgery. Out of these 46 patients, 13 patients had one or more intraspinal anomalies. 11 patients had undergone prior neurosurgical procedure while 2 (filum terminale lipoma and syringomyelia) did not. A total of 88 procedures were conducted during the treatment period; 13 index surgeries, 74 distractions of GR and 1 unplanned surgery. The age at surgery was 6.8 ± 2.5 years (3.5-12 years). 11 patients had congenital scoliosis and 2 had idiopathic scoliosis. A total of 19 (41.30 %) intraspinal anomalies [Tethered Cord Syndrome (TCS) 08, Split Cord Malformation (SCM) 08, Syringomyelia 01, Meningomyelocele 01, Filum terminale Lipoma 01] were seen. The average lengthening procedures per patient were 5.7 (4-9) with distraction interval of 6.7 (6-7.25) months. Pre-operative Cobb angle was 78.50 ± 18.1 (54-114°) and improved to 53.10 ± 16.70 (36-84°) at final follow-up. A total of 15 complications related to implant (9), wound (2), anesthesia (2) and neurological (2) occurred in 7 patients. Among the two neurological complications, one patient sustained fall in the post-op period and reported to the emergency department with paraplegia and broken proximal screw. While other patient experienced MEP changes during index procedure. None of the patients had any neurological complications during repeated lengthening procedures. The most common cord anomalies associated with EOS in our study are TCS and SCM. Although presence of previous intraspinal anomaly does not seem to increase the incidence of neurological deficit, use of neuromonitoring is advisable for all index procedure and selected distractions. Level 4 (case series).

  9. Outcome of antegrade continent enema (ACE) procedures in children and young adults.

    Science.gov (United States)

    Masadeh, Maen M; Krein, Michael; Peterson, Joshua; Bauer, Molly; Phearman, Laura; Pitcher, Graeme; Liao, Junlin; Shilyansky, Joel

    2013-10-01

    Intractable incontinence affects a large number of children and young adults in the US. The goal of this study is to evaluate the long-term outcomes of surgical access for administration of antegrade continence enemas (ACE) in affected children and young adults. Patients who underwent surgical procedure to enable administration of ACE from 1994 to 2011 were retrospectively reviewed. Data collected included patient demographics, primary diagnosis, surgical technique, conduit used, complications, follow-up duration, and social continence. Sixty eighty patients underwent surgery to enable ACE; mean follow up was 61 months. Enteral conduit (EC) was performed in 19 patients, tube cecostomy catheters (CC) in 49. Meningomyelocele was diagnosed in 60% of patients. Mean age was 11 (1.67-53) years. Complications included tube dislodgement (43%), granulation tissue (46%), site infection (13%), leakage (32%), break in the tube (6%) and tract stenosis (6%). Complete social continence was achieved in 68%, partial continence was achieved in 29%, and no benefit was achieved in 3% of patients. The rate of complications and incontinence resolution following CC was 78% and 66%, and following EC 89% and 74%. The differences were not statistically significant. CC patients developed granulation tissue more frequently (53%) and leaks of fecal material less frequently (20%) compared to EC patients (26% and 53%) (p < 0.05 and < 0.01). Although children 7 years or younger developed more overall complications (94%) than older patients (69%; p < 0.05), there was not a significant difference in the frequency of any one complication or in the rate of continence, between the two groups. Multivariate analysis showed that EC is three times more likely to be complicated by fecal leakage. CC patients are at greater risk to develop granulation tissue (p < 0.05). Most patients achieved social continence and improved hygiene with the aid of ACE. Younger children also benefited greatly from institution

  10. [CYSTECTASY AND REHABILITATION TRAINING FOR TREATMENT OF NEUROGENIC BLADDER DYSFUNCTION WITH HYPERREFLEXIA].

    Science.gov (United States)

    Li, Luping; Fan, Yingzhong; Li, Hao; Zhang, Qian; Gou, Li

    2016-02-01

    To investigate the effectiveness of ileal mucosal seromuscular patch for bladder expansion combined with rehabilitation training for treating neurogenic bladder dysfunction (NBD) with hyperreflexia. A retrospective study was performed on the clinical data of 61 patients with NBD and hyperreflexia who were treated and followed up between July 2008 and June 2013. There were 36 males and 25 females, aged 6-23 years (mean, 10 years). The reasons included meningomyelocele operation (43 patients),surgery for lipoma in lumbar vertebra (4 patients), operation of thoracolubar teratoma (2 patients), and lumbosacral spina B3ifida (12 patients). The results of urodynamics indicated that bladder volume decreased obviously and the residual urine increased. The voiding cystourethrography (VCUG) showed the vesicoureteral reflux (VUR), including 6 cases (10 sides) of grade V, 7 cases (12 sides) of grade IV, and 6 cases (8 sides) of grade III. The color doppler ultrosound showed mild hydronephrosis in 23 cases (41 sides), moderate hydronephrosis in 25 cases (42 sides), and severe hydronephrosis in 13 cases (22 sides). The blood biochemical examination suggested chronic renal failure (CRF) in 13 cases. The treatment included augmentation for bladder and rehabilitation training after operation. The operation time was (157+/- 26) minutes; the intraoperative blood loss was (43 +/- 15) mL, and no patient was given blood transfusion. The patients were followed up 1.5-6.0 years (mean, 4.5 years). Vesical fistula occurred in 4 cases, urinary infection in 5 cases, dysuresia in 2 cases, and cystolith in 1 case after operation. At 1 year after operation, the International Consultation on Incontinence Questionnaire-Urinary Incontience Short Form (IQ-F) score was significantly better than peoperative score (H=9.813, P=0.000). The aurdynmic data showed that the difference value between observed and theoretical bladder volumes, bladder compliance, residual urine volume, maximum flow rate, and

  11. Management of urinary tract infection with intravesical amikacin may increase the risk of bladder oxidative stress in children with neurogenic bladder.

    Science.gov (United States)

    Amasyali, Akin Soner; Yilmaz, Dilek; Yilmaz, Mustafa; Kucukdurmaz, Faruk; Sonmez, Ferah; Erol, Haluk

    2017-12-01

    We evaluated the bladder oxidative stress in neurogenic bladder children treated with intravesical amikacin for recurrent UTI and whether urinary isoprostane f2 alpha (F2-IsoP) is a good biomarker in this particular condition. This prospectively designed controlled study was approved by the Adnan Menderes University institutional ethics committee (Adnan Menderes University, 2015/649). Between January 2016 and January 2017, twenty-six children with meningomyelocele who had been doing CIC were recruited. Serum and urine samples were collected during urinary tract infection (UTI) (group 1) and after management of UTI with intravesical amikacin (group 2) besides standard oral antibiotic treatment. While oxidative stress parameters SOD, GSH, GPX, MDA, F2-IsoP and NO were analyzed in the serum samples, only F2-IsoP was analyzed in the urine. All data were compared with 23 normal healthy control children (group 3). Median age, CIC duration and number of CIC per day of patients' group were 84 (60-147) months, 60 (30-90) months and 4 (4-6), respectively. Male-to-female ratio was 1:16. There was no statistical difference between groups in terms of serum oxidative stress parameters (p > 0.05). However, statistically significant urine F2-IsoP changes exist between groups (p = 0.011) (Fig. 1). But there were no correlations between urine F2-IsoP and disease clinical data such as CIC duration or number of CIC per day. Serum glutathione levels in group 2 were higher than group 1 and 3, as well (p = 0.023, Kruskal-Wallis test). Fig. 1 Comparison of median urinary isoprostane f2 alpha levels CONCLUSION: Higher urine F2-IsoP levels after management of UTI with intravesical amikacin may reflect increased lipid peroxidation and oxidative stress in children with NB. This detrimental effect on bladder should be considered in the long-term treatment period.

  12. Abordagem assistencial ao neonato portador de mielomeningocele segundo o modelo de adaptação de Roy Abordaje asistencial al neonato portador de mielomeningocele según el modelo de adaptación de Roy Care delivery to newborns with myelomeningocele according to Roy's adaptation model

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    Eloah de Paula Pessoa Gurgel

    2010-09-01

    través de estímulos, y que las metas e intervenciones implementadas fueron importantes en la sustitución de respuestas ineficaces por adaptativas.This case study aimed to identify the Nursing diagnoses, addressing physiological aspects according to Roy's Adaptation Theory, of a baby with meningomyelocele hospitalized at a Neonatal Intensive Care Unit (NICU, and to elaborate pertinent nursing actions for these aspects. The study was developed at the NICU of the Maternity Teaching Hospital Assis Chateaubriand, located in Fortaleza-Ceará, Brazil, between December 2007 and March 2008, when a script was used to survey data. During the analysis, the following diagnoses were recognized: ineffective breathing pattern; imbalanced nutrition: less than body requirements; impaired skin integrity; risk for infection and disturbed sensory perception. The use of this theory permitted recognizing that the newborn can produce positive or negative responses to stimuli and that the implemented targets and interventions were important to replace ineffective by adaptive responses.

  13. Desempenho psicolinguístico e escolar de irmãos com mielomeningocele Psycholinguistic and school performance of brothers with myelomeningocele

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    Dionísia Aparecida Cusin Lamônica

    2012-08-01

    Full Text Available TEMA: mielomeningocele e consequências para o desenvolvimento de habilidades psicolinguísticas e escolares. PROCEDIMENTOS: o objetivo deste estudo foi descrever e refletir sobre o desempenho psicolinguístico e escolar de irmãos com mielomeningocele lombar baixa. Participaram do estudo um menino de 14 anos e 5 meses (P1 e sua irmã de 6 anos e 6 meses (P2. Os procedimentos constaram de entrevista com familiares, Observação do Comportamento Comunicativo (OCC, Teste de Vocabulário por Imagens Peabody - TVIP, Teste Illinóis de Habilidades Psicolinguísticas (ITPA, Perfil de Habilidades Fonológicas (PHF, Teste de Desempenho Escolar (TDE, e Reconhecimento de letras e números (RLN. A análise dos resultados foi descritiva respeitando os manuais dos instrumentos. RESULTADOS: a linguagem oral encontra-se sem alteração evidente. No ITPA e PHF, verificou-se que P1 e P2 apresentaram dificuldade nas habilidades de memória, closura auditiva, expressão verbal, combinação de sons e consciência fonológica. P1 ainda apresentou dificuldade na habilidade de recepção visual e P2 nas habilidades de associação auditiva e closura gramatical. Ambas as crianças apresentaram alteração nas atividades de aprendizagem. CONCLUSÃO: os participantes do estudo apresentaram comportamento comunicativo, habilidades de linguagem oral e vocabulário receptivo sem alterações evidentes. P1 e P2 apresentaram alterações nas habilidades psicolinguísticas bem como no desempenho de atividades envolvendo leitura, escrita e aritmética. As dificuldades apresentadas causam impacto importante nas atividades acadêmicas, demonstrando vulnerabilidade cerebral no sistema de suporte para atividade de aprendizagem.BACKGROUND: meningomyelocele and consequences on the development of psycholinguistic and school abilities. PROCEDURES: the purpose of this study was to describe and reflect on the psycholinguistic and school performance of siblings with low lumbar

  14. Impacto das malformações congênitas na mortalidade perinatal e neonatal em uma maternidade-escola do Recife Impact of congenital malformations on perinatal and neonatal mortality in an university maternity hospital in Recife

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    Melania Maria Ramos de Amorim

    2006-05-01

    , perinatal mortality, early and late neonatal mortality coefficients were calculated. RESULTS: malformation incidence was of 2.8% (in 4.043 births. Malformation percentages among live births was of 2.7% and among stillbirths of 6.7%. The most frequent malformations involved the central nervous system (principally hydrocephaly and meningomyelocele, the skeletal and muscular system and cardiopathies. There was no association between malformation and gender, but prematurity and low birthweight were more frequent among the malformation cases. It was determined that among malformed infants early neonatal mortality was of 32.7% and late neonatal mortality was of 10.6%. Malformation cases were 6.7% of stillborn babies, 24.2% of early neonatal deaths and 25.8% the total of neonatal deaths. CONCLUSIONS: malformation incidence corresponded to 2.8% of the births. Malformation was the second more frequent neonatal death cause following prematurity.

  15. Static renal scintigraphy with 99mTc-DMSA or dynamic renal scintigraphy with 99mTc-EC at the assessment of diseases of the kidneys and the urinary tract in pediatrics

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    Oppermann, P.

    2001-10-01

    In the field of pediatric nephrourology the nephropathies of children caused by obstruction and reflux of the urinary tract are very important. The pathogenesis of these diseases begins with an outflow impairment of urine because of a congenital ureteropelvic or ureterovesical obstruction, vesicouereteral reflux respectively. Etiologically a malformed ureter, or a neuropathic bladder with voiding disorders due to meningomyelocele are responsible for these problems. This outflow impairment of urine is followed by parenchymal damage of the involved kidneys, on the one hand caused by ascending dysplasia and nephrogenic arrest during the development of the urinary tract, and on the other hand caused by ascending urinary tract infections and water hammer effect with dilatative obstructive damage. Basically there are two ways of treatment for these little patients: first the conservative therapy with watchful waiting for maturation while giving medication and some further possibilities when treating a neuropathic bladder, and second surgery with invasive removal of the outflow impairment. To choose correctly between these possibilities of treatment clarifying convincing diagnostics are necessary (identification and evaluation of the outflow impairment and of the parenchymal damage, and evaluation of the kidney function separately for each side). Since the average age of these patients is very low the strain by diagnostics should also be as low as possible, especially concerning the radiation burden. The static renal scintigraphy with 99mTc-DMSA is considered to be the gold standard for identification and evaluation of parenchymal damage and scaring of kidneys. Now the dynamic renal scintigraphy with 99mTc-EC was found to reveal parenchymal damage as well as the static renal scintigraphy. In addition to this equivalence as far as the evaluation of parenchymal function is concerned, the dynamic renal scintigraphy with 99mTc-EC shows evidence to be superior to the static