- Department of Trauma and Emergency, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
- Department of Neurosurgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
Department of Neurosurgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
DOI:10.4103/2152-7806.169554Copyright: © 2015 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Patnaik A, Mahapatra AK. A rare case of giant multiseptated thoracic myelomeningocele with segmental placode. Surg Neurol Int 16-Nov-2015;6:170
How to cite this URL: Patnaik A, Mahapatra AK. A rare case of giant multiseptated thoracic myelomeningocele with segmental placode. Surg Neurol Int 16-Nov-2015;6:170. Available from: http://surgicalneurologyint.com/surgicalint_articles/a-rare-case-of-giant-multiseptated-thoracic-myelomeningocele/
Myelomeningocele is a common form of spinal dysraphism involving thoraco-lumbar region. Pure thoracic form is quite rare. We present a unique case of giant, multiseptated thoracic myelomeningocele with a segmental placode, both proximal and distal to which the spinal cord was normally neurulated and discuss the unique features associated with such rare variety of thoracic myelomeningocele.
A 16-month-old male child presented with huge swelling over thoracic region since birth, which was progressively increasing in size. The parents did seek for the neurosurgical consultation and were advised surgery soon after the birth but they deferred it for lack of money and risk for further neurological deficit. There was no history of leakage of fluid from the swelling. The swelling was approximately 15 cm × 12 cm × 12 cm in size, lobulated, with broad pedicle of skin, and covered with thick desquamated epithelium [Figure
Giant myelomeningoceles are challenge due to high-risk of skin breakdown, CSF leak, infection, etc., To reduce the chances of skin breakdown and subsequent CSF leak, a ventriculoperitoneal is advisable such cases. We prefer a two sitting shunt and defect repair to decrease the chances of shunt infection as we did in this case. We performed a low pressure shunt as the anterior fontanelle was open and there was no radiological signs of grossly increased intracranial pressure (periventricular lucency). In presence of gross neurologic deficits in thoracic myelomeningocele, there occurs invariably kyphosis due to unrestricted pull of the normally innervated proximal anterior abdominal and intercostal muscles. This kyphosis prevents the tensionless closure of the defect and requires same sitting kyphectomy. Fortunately in our case, due to normal neurulation of distal cord the paraspinal muscles had retained their tone and this explains the lack of kyphosis in the present case. Segmental neural placode is extremely rare and account for only 4% of all open neural tube defects. Exact mechanism of genesis of such segmental placode is not clearly known but a “square-pulse” type teratogenic insult during primary neurulation has been proposed which results in normal neurulation starting distal to an isolated failure of neural plate fusion. “Collision site” hypothesis explains the cause as failure from two adjacent neurulation sites proceeding in opposite directions.[
Thoracic and cervical region myelomeningoceles are quite rare and occur in 1–5% of all neural tube closure defects.[
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