Shruti Sharma, an 11-year old girl, was diagnosed with Prader-Willi syndrome (PWS) at a young age. PWS is a complex genetic disorder that results in short stature, disorders of metabolism, cognitive disabilities, behavioral problems and low muscle tone, leading often to spinal deformities such as scoliosis. One of the hallmarks of the disorder is obesity and an insatiable appetite, often leading to Type 2 diabetes. Shruti was certainly the most challenging case the OSS team faced in 2017.
Shruti originally presented to the OSS team the previous year for consideration of corrective surgery for her thoracic scoliosis. But her diabetes was newly diagnosed and poorly controlled, so the team thought it best to delay her surgery until their return trip. That would give Shruti time for her local physicians to bring her diabetes under control. By 2017 her left thoracic scoliosis curvature had progressed to 92 degrees.
Shruti’s surgery lasted about 6 hours and consisted of a posterior correction of the scoliosis and the placement of spinal rods and fusion from the 2nd thoracic vertebra to the 3rd lumbar vertebra. Due to her relatively small size for 11, small 4.5mm rods and implants were used, followed by special bands known as “Jazz Bands”, attached to several vertebra. Surgeons slowly tightened the Jazz Bands, gradually straightening the severe curvature of Shruti’s spine and anchoring it to the spinal rods.
Shruti remained intubated in the ICU for the first 24 hours and by the 4th day she was smiling, walking, and coloring pictures.