Delhi doctors treat boy with inherited spinal deformity

New Delhi, Jul 5 (IANS): Doctors at the Indian Spinal Injuries Centre (ISIC) here gave a new lease of life to a 12-year-old boy suffering from an inherited spinal disorder that curved his spine 140 degrees to the left.

Kinshuk Swalkar from Kota in Rajasthan has been suffering from Scoliotic deformity -- his spine resembled an "S"-like or "C"-like shape. A previous attempt of corrective surgery in another hospital in 2015 had to be abandoned as Swalkar also suffered malignant hyperthermia -- a life-threatening clinical syndrome of hypermetabolism involving the skeletal muscles that is triggered by certain anaesthetic agents.

"Kinshuk was first brought to ISIC in January. We were facing three major challenges before undertaking the surgery. The first was his malignant hyperthermia. Since he has a history, operating on him without having dantrolene on standby was out of the question -- the condition has a very high mortality rate of 80 per cent and can be brought down to 2 per cent using the drug," said Dr Rajat Mahajan, Spine Surgeon at ISIC.

The doctors imported Dantrolene -- the only medicine that can stabilise malignant hyperthermia -- from Germany as it was not readily available in India.

"The second challenge was the magnitude of structural deformity -- it was not possible to correct the huge 140-degree Cobb angle (the degree of side-to-side spinal curvature) and pelvic obliquity (a misalignment of the pelvis where one hip is higher than the other) at one go. The third was how to use anaesthesia during surgery since the traditional method would have triggered hyperthermia," Mahajan said.

As the doctors waited for dantrolene, they put Kinshuk on halo gravity traction -- a technique to pull the head and spine upward by stretching the spine slowly -- for 3 weeks in the first week of February that improved the curve to 100 degrees.

This also helped to address the iliocostal impingement -- a painful disabling condition in which ribs start to touch the iliac crest (hip bone).

"The remaining curve and misalignment of hip were corrected with spinal fusion from vertebrae D2 to pelvis during a 6-hour-long surgery in the first week of March," Mahajan said.

Since inhalational anaesthetic agents and suxamethonium are the trigger for the rare malignant hyperthermia, the doctors chose not to use these agents for general anaesthesia. Instead, they opted for total intravenous anaesthesia (TIVA) in which intravenous anaesthetic agents are used. It worked well in the patient's favour and he remained stable throughout the peri-operative period, the doctors said.



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