1/19. The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention.OBJECTIVE: To determine how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. methods: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. RESULTS: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. CONCLUSION: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/19. Segmental costovertebral malformations: association with neural tube defects. Report of 3 cases and review of the literature.patients with spondylocostal dysostosis (SCD) have vertebral abnormalities and numerical or structural rib anomalies that produce thoracic asymmetry. Rib anomalies and dysmorphism are the typical features that differentiate this syndrome from spondylothoracic dysostosis (STD). Jarcho-Levin syndrome is a severe form with involvement of the whole vertebral column. Other associated findings such as congenital heart defects, abdominal wall malformations, genitourinary malformations and upper limb anomalies may be found; in addition, neural tube defects (NTDs) have been associated with this malformation. SCD is transmitted both in a recessive form and as a dominant defect. We report on 3 children with SCD; 2 also had NTDs. All of them were studied with x-rays and spinal magnetic resonance (MR), and over the same period they underwent multidisciplinary clinical functional evaluation. One of our cases with NTD also presented polythelia, which has not previously been described in patients with SCD. The common association of segmental costovertebral malformations with NTDs could be related to an early gastrulation genomic defect, or one after gastrulation, when there are two independent somitic columns. The latter sometimes progresses and then involves primary and secondary neurulation. Also, the association of SCD with NTDs could be related to the interaction of different genes, resulting in this complex phenotype. Therefore, additional genetical and embryological studies are necessary to provide evidence of an etiological link between SCD and NTD.- - - - - - - - - - ranking = 0.125keywords = complex (Clic here for more details about this article) |
3/19. An unusual fetus with complete absence of thoracic, lumbar and sacral vertebrae, bilateral renal agenesis, VSD, meningomyelocele, imperforate anus, and teratoma.We present a 30 week old male fetus who had a very interesting malformation complex which can not be explained by teratogenic or hereditary diseases. The aim of this paper is to discuss this complicated entity and compare it with other reported cases.- - - - - - - - - - ranking = 0.125keywords = complex (Clic here for more details about this article) |
4/19. Partial caudal duplication in a newborn associated with meningomyelocele and complex heart anomaly.BACKGROUND: Caudal duplication is a spectrum of rare congenital anomalies with a possible heterogeneous pathogenesis including incomplete separation of monovular twins. methods: We report an autopsy case of a full-term infant with incomplete caudal duplication syndrome associated with multiple anomalies. RESULTS: These anomalies included a duplicated penis; double urinary bladder with an attenuated tunica muscularis; duplication of lower bowel with two ilia, appendices and colons; colonic hypogangliosis and left imperforated anus associated with rectourethral fistula. Other anomalies consisted of sacral meningomyelocele, sacral duplication with hypoplastic left sacrum and pelvic bones, muscle atrophy and hypoplasia of the left lower extremity, abnormal lobation of liver with stomach entrapment, omphalocele, and right atrial isomerism syndrome. The complex pattern of anomalies suggests the possibility that partial caudal duplication might be part of the spectrum of conjoined twinning.- - - - - - - - - - ranking = 0.625keywords = complex (Clic here for more details about this article) |
5/19. Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy.This short paper illustrates a case with cervical myelomeningocoele, a Chiari malformation (CM), hydrocephalus (HC) and cervical syringomyelia treated by neuroendoscopic third ventriculostomy (NTV) with resolution of both the hydrocephalus and the syrinx. Two similar cases are discussed. The technique is advocated for the treatment of such complex dysraphic conditions.- - - - - - - - - - ranking = 0.125keywords = complex (Clic here for more details about this article) |
6/19. Computer-aided image-guided endoscopic sinus surgery in unusual cases of sphenoid disease.The vital neurovascular structures that border the sphenoid sinus make extensive sphenoid sinus surgery hazardous despite the advent of endoscopic sinus surgery (ESS). Computer-aided image-guided endoscopic sinus surgery (CAIGESS) has facilitated safer surgery by providing real-time analysis of complex, three-dimensional anatomic landmarks. We present 6 cases of atypical sphenoid disease, which greatly benefited from the unique superiority of CAIGESS in avoding surgical complications. Two cases of cerebrospinal-fluid (CSF) leak with concomitant meningoencephalocele of the sphenoid sinus were successfully managed with this technique. An inverting papilloma originating from the sphenoid sinus was successfully exenterated using CAIGESS. One patient, who experienced a lateral-rectus muscle palsy from sphenoid sinusitis, underwent successful sinusotomy with CAIGESS. Another patient, who had refractory left-sided sphenoid sinusitis despite 2 ESS procedures, was found to have an obliquely oriented intersinus septum which misled the previous surgeons to enter mistakenly the contralateral sphenoid sinus. CAIGESS allowed accurate identification and removal of the intersinus septum and relief of the sinusitis. Finally, a sphenoid-sinus mucocele that developed after a prior pituitary surgery was safely decompressed with CAIGESS. This surgical approach offers a new and effective adjunct to ESS in selected revision or difficult sinus cases and has proven invaluable in complicated sphenoid cases where the surrounding neurovascular anatomy could otherwise be jeopardized.- - - - - - - - - - ranking = 0.125keywords = complex (Clic here for more details about this article) |
7/19. Terminal myelocystocele.Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin covered lumbosacral spina bifida. This condition is often associated with OEIS complex i.e. opmphalocele, exstrophy of the bladder, imperforate anus and spinal abnormality. We studied 4 cases of terminal myelocystocele. They revealed no preoperative neurological deficit. None of these had associated OEIS complex. One of the cyst was unique due to presence of copious amount of pus in its cavity. All four cases underwent successful repair and surgery remained uneventful in all of them. No child showed neurological deterioration during the follow-up.- - - - - - - - - - ranking = 0.25keywords = complex (Clic here for more details about this article) |
8/19. Prenatal ultrasound diagnosis of cloacal exstrophy associated with myelocystocele complex by the 'elephant trunk-like' image and review of the literature.A case of cloacal exstrophy (CE) was detected by ultrasound as early as 22 weeks of gestation in association with myelocystocele complex, an unusual form of occult spinal dysraphism often associated with such a disease. The ultrasonographic diagnosis was made through the detection of a wavy cord-like segment of soft tissue protruding from the anterior abdominal wall, just below the umbilical cord insertion, strongly resembling the trunk of an elephant. Our article enforces the suggestion that the ultrasound elephant trunk-like image should be added to the existing major criteria for making prenatal diagnosis of CE.- - - - - - - - - - ranking = 0.625keywords = complex (Clic here for more details about this article) |
9/19. Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: a longitudinal case study.Myelomeningocele is often accompanied by hydrocephalus (MMH), making it a potentially unstable neurological condition requiring shunt placement and possible revisions. Serial neuropsychological assessment is an important tool in monitoring children with MMH, as cognitive changes can indicate shunt malfunction and hydrocephalus. We present the case of a girl with MMH who had five neuropsychological assessments (ages 5, 7, 11, 12, and 14). Despite a lack of overt neurological symptoms or report of behavioral decline, testing at age 11 revealed decline in multiple neurobehavioral domains, and imaging at that time showed increased hydrocephalus, requiring shunt revision. Subsequent neuropsychological assessment conducted after a 2-year period of medical stability showed improvement and/or a return to baseline levels in some skill areas (i.e., working memory, verbal memory, visuomotor integration, and sustained attention), yet more lasting impairments in others (i.e., Verbal IQ, processing speed, organization, and response inhibition). These lasting cognitive deficits potentially impact independent completion of complex medical self-care tasks. This pattern of recovery highlights vulnerability of brain systems supporting executive functions in children with hydrocephalus and shunt failure.- - - - - - - - - - ranking = 0.125keywords = complex (Clic here for more details about this article) |
10/19. Proximally situated osseous septum in complex spina bifida. Case report.In Type I split cord malformation (SCM) lesions, the osteocartilaginous spur and its dural sleeve are almost always located in the caudal extent of the median cleft. The authors present a case of lumbar myelomeningocele associated with a thoracic Type I SCM in which an osseous septum is uniquely situated in the proximal extent of the median cleft. Split cord malformations are cord-tethering lesions, which may be associated with a myelomeningocele that causes additional tethering, even in the opposite direction; therefore, both lesions should be treated. In view of this unique case, however, the surgical approach used for tethering lesions in such cases of complex spina bifida should perhaps be tailored. Whenever feasible, meticulous preoperative examination of a patient with spina bifida, including entire craniospinal magnetic resonance imaging followed with perioperative dynamic evaluation, is important for the effectiveness and safety of the surgery.- - - - - - - - - - ranking = 0.625keywords = complex (Clic here for more details about this article) |
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