Diastomatomyelia is relatively a rare condition where there is a bony spur that projects across the spinal canal and splits the spinal cord into two. The patients end up having extensive cutaneous markers like subcutaneous lipomas, dermal sinuses and localized hypertrichosis (hairy patches). It is caused by an incomplete fusion of the mid-line mesenchymal, bony or neural elements of the spine. As skin and nervous tissue are of ectodermal origin, anomalies of both may occur simultaneously.
They may also have extensive spinal column abnormalities like scoliosis and kyphosis.
Mechanism of neuronal injury
The spinal cord gets damaged due to traction on the neurons by the bony spur as the patient grows up. Dynamic injury to the cord occurs during flexion /extension due to tethering. Spinal cord injury also happens due to stretch on the vasculature.
Indications for removal of bony spur
o Patients presenting with progressive neurological deficit
o If correction of spinal deformity (scoliosis/kyphosis) is contemplated
CASE STUDY
Synopsis
A 12 year old female child from Tamil Nadu presented to us with progressive difficulty in walking for the last 6 months, difficulty in using both her upper limbs – 3 months. She had developed urinary urgency for 1 month and incontinence for the last 1 week. Most worrisome for the team, was the respiratory muscle weakness with distress for the last 3 to 4 days. Patient had undergone a failed attempted surgery elsewhere 2 yrs ago. The patient was referred to Sagar Hospital-Neurological Dept.
INVESTIGATION
She was thin built with significant cervico-thoracic scoliosis, she had cutaneous marker in the form of hairy patch, dermal sinuses and lipoma at cervico – dorsal level, weakness of all the four limbs, with no movement in the lower limbs at all (grade 0/5). She had complete loss of bowel and bladder control and was in respiratory failure with single breath of 1 – 2, due to complete intercostal muscle paralysis and a weak diaphragm
o Imaging – X ray showed extensive cervico thoracic scoliosis with lung compromise, CT/MRI scan showed complete Diastomatomyelia with two spinal cords extending from cervical (C4) up to upper thoracic level (T5) with a tumour ( neurenteric cyst) compressing the cord at the cervical level
o Treatment challenges – The risk of surgery was very high as the patient’s general condition was very poor and the proposed surgery was going to be a redo – surgery with extensive tissue adhesions in an already complicated anatomy.
Perop – mother holding up the child demonstrating poor leg strength, cutaneous markers and surgical incisions from previous failed attempted surgery.
Chest X-ray – showing extensive spinal and chest cage deformities
MRI scan showing complete division of spinal cord into two with cystic tumour at the upper end
CT scan showing extensive bony abnormalities
Surgery – Under the leadership of Dr H V Madhusudan (neuro and spinal surgeon) – patient underwent an extensive 18 hours corrective surgery to remove the tumour from the spinal cord (neurenteric cyst), release the spinal cord tissue of all adhesion and create a single spinal canal. Patient underwent an elective tracheotomy for respiratory care.
The surgical team was excellently supported by the anaesthetic team– difficult airway; long duration of surgery, extensive blood loss, fluid electrolyte imbalance and a poor lung function were some of the issues they had to deal with.
Intensive care unit (ICU) – The pulmonologist (also head of intensive care) and his team did a fantastic job in the post – op period including pain management and physiotherapy.
Follow up – At 3 months she is presently walking independently, wound well healed normal bowel and bladder control, awaiting tracheotomy closure.
Post op patient standing independently, with well healed wounds.
STAR PERFORMER:
Dr H V Madhusudan , MBBS, M Ch (Neuro), Fellowship in spinal surgery, Arizona (USA)
Fellowship in Neuro- Oncology, Gamma Knife and Functional Neurosurgery, New- York (USA).
He completed his neuro-surgical training from NIMHANS, Bangalore; followed by ECFMG (USMLE) certification then received a fellowship in spinal surgery at University of Arizona and finally from the University of Buffalo – Roswell Park Cancer Institute, he received a fellowship in Neuro Oncology, Gamma Knife and Functional Neurosurgery.
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Sr.Manager,Digital Marketing & Media ,Mr.Ghosh , email:srmgr.dm@sagarhospitals.in
