Isolated giant intrathoracic meningocele associated with vertebral corpus deformity (original) (raw)
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Isolated true anterior thoracic meningocele
AJNR. American journal of neuroradiology
Intrathoracic meningocele is rare and is usually associated with neurofibromatosis type I. Most of the reported thoracic meningoceles are not strictly anterior in location, but also lateral or anterolateral. We report a case of true anterior thoracic meningocele with no associated generalized mesenchymal dysplasia.
Giant true dorsal thoracic meningocele in a school-age child
Journal of Neurosurgery: Pediatrics, 2008
✓A meningocele is a common form of spinal dysraphic lesions, but it is extremely uncommon in children in the upper thoracic region. The authors describe a rare case in which they found a giant true dorsal meningocele in the upper thoracic region in a symptomatic child, which, to their knowledge, is the first such reported case. A school-age child, who harbored a giant dorsal mass, complained of restriction of function. He underwent successful surgery in which the meningocele sac was totally removed.
Cervical meningocele and associated spinal anomalies
Child's Nervous System, 1987
Simple meningoceles are infrequent forms of dysraphism and are often benign. They have been associated with other spinal anomalies. The uncommon cervical meningocele may have a higher propensity to be associated with other spinal anomalies. Four patients with cervical meningocele are presented with radiographic evaluation and clinical course. Multiple abnormalities were documented radiographically and operatively, including hydrocephalus, Chiari malformation, hydromyelia, lipomeningomyelocele, tethered cord, thickened filum terminale, diastematomyelia, Klippel-Feil syndrome, and thoracic hemivertebrae. Prior to the development of any late neurological abnormality from associated spinal anomalies, magnetic resonance imaging is recommended early in a child born with a simple meningocele.
Neurosurgical Review, 1995
We report two cases of ventrolateral thoracic and lumbar meningoceles associated with neurofibromatosis type 1, and present a review of the literature. Thoracic and lumbar meningoceles are rare lesions which are associated with neurofibromatosis in 60% to 85% of cases. Large meningoceles may cause pain, neurological signs, or respirtory complaints when located intrathora-ciealLly. Surgery is indicated in such cases, as well as when small meningoceles enlarge with time. The etiology of meningoceles in neurofibromatosis remains controversial. The authors postulate that ventrolateral lesions of the thoracic and lumbar spine are secondary to congenital mesodermal dysplasia and hypoplastic bone changes. MRI is the diagnostic method of choice, since it most adequately visualizes the pathological anatomy of the spinal cord, meninges, and adjacent structures in every sectional plane. CT scans are particularly useful in assessing the spinal bony changes.
Pediatric Neurosurgery
Background: Lateral meningocele syndrome (LMS) is an exceedingly rare connective tissue disease with phenotypic anomalies similar to those seen in Marfan syndrome, Ehler-Danlos syndrome, and Loeys-Dietz syndrome. However, this syndrome is invariably associated with the presence of multiple lateral thoracolumbar spinal meningoceles: a distinct point of phenotypic divergence from other connective tissue disorders. The etiopathogenesis of this syndrome has recently been linked to truncating mutations within exon 33 of NOTCH3. Despite numerous reports, neurosurgical management of multiple spinal meningoceles remains poorly defined in the literature. We conducted a literature review to provide insight into the nosology, clinical significance, and neurosurgical management strategies of this distinct connective tissue disorder. Summary: Our literature search revealed 11 articles (16 cases) of LMS, which included 9 males
Lateral meningocele syndrome: Vertical transmission and expansion of the phenotype
American Journal of Medical Genetics Part A, 2005
Lateral meningoceles were first described by ; J Pediatr 90: 49-54] in a patient with other skeletal findings and distinctive craniofacial features. Subsequently, six more patients with the so-called lateral meningocele syndrome (LMS) have been reported. We describe the findings in three new cases and expand the phenotype. The existence of an affected mother and daughter supports the hypothesis that LMS is a dominant disorder affecting primarily the connective tissue.
True cervicothoracic meningocele: a rare and benign condition
Cervical meningoceles are rare spinal dys-raphism, accounting for approximately 7% of all cystic spinal dysraphism. In spite of the rarity , the clinical course is most of the times benign. The surgical treatment includes resec-tion of the lesion and untethering, when presented. We present a 14-day-old female child with true meningoceles who underwent to surgical excision and dura-mater repair. Retrospect analysis of the literature concerning true cervical meningocele is performed. By reporting this illustrative case, we focus on its classification and its differentiation from other types of cervical spinal dysraphism, such as myelocystocele and myelomeningocele. Although its course is benign, it is mandatory a continuum follow up with periodic magnetic resonance imaging of spinal cord, since late neurological deterioration has been described.
Journal of neurosurgery, 2004
The authors report the case of a 7-year-old girl who presented with persistent low-back and left leg pain and was diagnosed with expanding occult intrasacral meningocele (OIM), diastematomyelia, tethered cord, and multiple vertebral anomalies. She was followed for 27 months. Removal of a bone spur and sectioning of the tight terminal filum did not relieve her left leg and back pain. Serial magnetic resonance (MR) imaging after initial detection of the OIM revealed gradual but significant enlargement of the lesion. Fifteen months after terminal filum release and bone spur resection, the OIM was excised. After this surgery, the back and leg pain resolved. No case of OIM reported to date contains documented serial MR imaging confirmation of enlargement of OIM. Furthermore, this is the first report of OIM associated with diastematomyelia. Because most OIMs expand, the authors recommend that, in addition to surgery for associated congenital anomalies, the OIM be totally excised to resolv...
Journal of neurosurgery. Pediatrics, 2016
Lumbo-costo-vertebral syndrome (LCVS) is a rare disorder in children that is characterized by hemivertebrae, congenital absence of ribs, meningocele, and hypoplasia of the truncal and abdominal wall presenting as a congenital lumbar hernia. An otherwise healthy 12-month-old girl was referred to the authors' hospital with soft swelling on her left middle back; scoliosis had been present since birth. Imaging revealed a thoracic meningocele, ectopia of the spleen suggesting lumbar hernia, multiple anomalies of the thoracic vertebral columns, and defects of the ribs; thus, LCVS was diagnosed. Surgical observation revealed that the meningocele was firmly anchored to part of the diaphragm, which created stretching tension in the meningocele continuously with exhalation. Once detached, the meningocele shrank spontaneously and never developed again after cauterization. In this case, continuous or pulsatile pressure in the presence of a vertebral defect was thus considered to be an impor...