Case Of the Week (COW) 03 May 2015
Diagnosis: klippel - feil anomaly- type 2 with butterfly vertebra with isolated case of true anterior thoracic meningocele
Findings:
Fusion of cervical vertebra between C3-C7 with butterfly vertebra involving T1,T2 vertebra with ventral herniation of spinal cord with meninges through ventral defect in butterfly vertebra . Syringomyelia above and below the level of herniation.
Discussion:
Spinal meningocele is herniation of meninges through bone defects or foramina to form a CSF filled sac.Congenital meningocele is relatively rare . More than 80%- of congenital meningocele are located posteriorly in lumbosacral area. Anterior meningocele is rarer and is described generally in thoracic and sacral region. Most of the reported thoracic meningoceles is usually through a previously enlarged intervertebral foramen. The anterior meningocele through ventral defect in vertebral body is known as true anterior meningocele and is very rare.The anterior meningocele is usually associated with NF-1 or other generalized mesenchymal dysplasia. In this case the herniation is through a wide midline bony defect of vertebral bodies . So it is a case of true anterior thoracic meningocele. Patient presented in this case report had no association with with NF-1 or other generalized mesenchymal dysplasia. So this is a isolated case of true anterior thoracic meningocele. In the review of the literature, Andrade et al found 134 cases of intrathoracic meningoceles reported before 1992. 69% of these thoracic meningoceles were in association with NF-1, only 22% were isolated cases. In the same article, a total of 16 patients with thoracic meningocele between 1992 and 2003 were reported. Twelve of these 16 patients were together with NF-1 and the remaining four being isolated cases Treatment: Surgical. Small and medium-sized- Approach is through a laminectomy and an intradural repair . For larger lesions, as in case presented here, a transthoracic access is indicated.
Contributed By:
Dr. V Dheebha. Dr. S Babu Peter, Dr. Geetha
Diagnosis: klippel - feil anomaly- type 2 with butterfly vertebra with isolated case of true anterior thoracic meningocele
Findings:
Fusion of cervical vertebra between C3-C7 with butterfly vertebra involving T1,T2 vertebra with ventral herniation of spinal cord with meninges through ventral defect in butterfly vertebra . Syringomyelia above and below the level of herniation.
Discussion:
Spinal meningocele is herniation of meninges through bone defects or foramina to form a CSF filled sac.Congenital meningocele is relatively rare . More than 80%- of congenital meningocele are located posteriorly in lumbosacral area. Anterior meningocele is rarer and is described generally in thoracic and sacral region. Most of the reported thoracic meningoceles is usually through a previously enlarged intervertebral foramen. The anterior meningocele through ventral defect in vertebral body is known as true anterior meningocele and is very rare.The anterior meningocele is usually associated with NF-1 or other generalized mesenchymal dysplasia. In this case the herniation is through a wide midline bony defect of vertebral bodies . So it is a case of true anterior thoracic meningocele. Patient presented in this case report had no association with with NF-1 or other generalized mesenchymal dysplasia. So this is a isolated case of true anterior thoracic meningocele. In the review of the literature, Andrade et al found 134 cases of intrathoracic meningoceles reported before 1992. 69% of these thoracic meningoceles were in association with NF-1, only 22% were isolated cases. In the same article, a total of 16 patients with thoracic meningocele between 1992 and 2003 were reported. Twelve of these 16 patients were together with NF-1 and the remaining four being isolated cases Treatment: Surgical. Small and medium-sized- Approach is through a laminectomy and an intradural repair . For larger lesions, as in case presented here, a transthoracic access is indicated.
Contributed By:
Dr. V Dheebha. Dr. S Babu Peter, Dr. Geetha