Giant Thoracolumbosacral Myelomeningocele: A Case Report

Abstract

Neural tube defects are a broad spectrum of congenital developmental anomalies that include cranial defects and open and closed spinal dysraphism. Myelomeningocele is a common form of spinal dysraphism most commonly seen in the lumbosacral region. Giant myelomeningoceles are rare occurrences. We hereby present the case of a 7-month-old infant with a history of large swelling over the thoracic lumbar region (circumference of 42 cm and diameter of 12.5 cm) that was progressively increasing in size with occasional cerebrospinal fluid (CSF) leakage from the swelling. There was associated progressive increasing size of the head size circumference (51 cm) as well for the past 6 months. There was no movement of both lower limbs since birth without any sensation up to the groin. On evaluation, the infant was found to have gross hydrocephalous. The Evans index was 0.4 with Chiari 2 malformation and a giant thoracolumbosacral myelomeningocele. The patient underwent a low-pressure ventriculoperitoneal (VP) shunt on the right side, followed by the repair of the giant myelomeningocele. The post-op period was uneventful with no CSF leakage or skin necrosis. The infant was discharged on day 5. Giant myelomeningoceles are quite a challenge for neurosurgeons due to the high risk of skin edge necrosis, CSF leaks, meningoencephalitis, etc. Here we managed our case with utmost intraoperative surgical skills and postoperative management due to which our patient was discharged in a satisfactory condition without any complication. This case is supposed to be largest myelomeningocele in the world literature to date.

Keywords

myelomeningocele - neurosurgery - spinal dysraphism - tethered cord

Authors' Contribution

All the authors contributed to the study's conception and design. A.A. contributed to the conceptualization of the study and writing of the first draft. A.K.M. was responsible for preparation of the figures. S.P. contributed to the editing and writing of the manuscript. R.C.D. contributed to the editing of the manuscript. S.B.S. contributed to the editing and the second draft of the manuscript.

Publication History

Article published online:
23 February 2024

• References

• 1 Agrawal A, Sampley S. Spinal dysraphism: a challenge continued to be faced by neurosurgeons in developing countries. Asian J Neurosurg 2014; 9 (02) 68-71
• 2 Patnaik A, Mahapatra AK. A rare case of giant multiseptated thoracic myelomeningocele with segmental placode. Surg Neurol Int 2015; 6: 170
• 3 Bakketun T, Gilhus NE, Rekand T. Myelomeningocele: need for long-time complex follow-up-an observational study. Scoliosis Spinal Disord 2019; 14: 3
• 4 Mahapatra AK. Spinal dysraphism controversies: AIIMS experiences and contribution. Indian J Neurosurg 2012