Case Study: Severe Scoliosis in an 11-Year Old Girl with Rare Syndrome

Shruti Sharma during her pre-operative evaluation with the OSS 2017 team.

Kolkata, India,  January 25, 2017

During their annual mission trip to Kolkata, India, Dr McConnell and the other members of the Operation Straight Spine (OSS) team were faced with a very challenging case of severe scoliosis in an 11 year-old girl.

Shruti Sharma was diagnosed with Prader-Willi syndrome (PWS) at a young age.  PWS is a complex genetic disorder affecting approximately one in 15,000-30,000 individuals. The disorder results in short stature, disorders of metabolism, cognitive disabilities and behavioral problems. One of the hallmarks of the disorder is insatiable appetite and obesity which often leads to childhood diabetes mellitus (Type 2 diabetes).  Due to low muscle tone, patients will often develop spinal deformities such as scoliosis. Shruti was certainly the most challenging case the OSS team faced this year, not only from a medical management standpoint, but also by the severity of her spinal deformity.

Shruti originally presented to the OSS team in 2016 for consideration of corrective surgery for her thoracic scoliosis, however her diabetes was newly diagnosed and poorly controlled. The team thought it best to delay her surgery until their return trip in 2017 so that her diabetes could be brought under better control. By 2017 her left thoracic scoliosis curvature had progressed to 92 degrees.

Pre-operative X-ray showing severe, 92 degree thoracic scoliosis

Shruti’s scoliosis surgery

After Shruti was placed under anaesthesia, blood started collecting in her right lung for unknown reasons. This delayed her surgery for several hours. The bleeding eventually stopped and she was stable enough to complete the procedure, which lasted about 6 hours. It 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, small 4.5mm rods and implants were used. Then, special bands resembling zip ties, known as  “Jazz Bands”, were attached to several vertebra. By slowly tightening the Jazz Bands the severe curvature of Shruti’s spine was gradually straightened and anchored to the spinal rods.

Shruti’s postop and recovery

Shruti’s postoperative course was somewhat stormy.  She remained intubated in the ICU for the first 24 hours. as her blood pressure was unstable and she ran a high temperature. On post-op day #1 the breathing tube was removed, but her right lung had filled with fluid and she required aggressive manual pulmonary therapy to help clear the lung. Ultimately, Shruti did very well and by the 4th post-op day she was smiling, walking, and coloring pictures.

About 3-4 weeks after her scoliosis surgery, Shruti had a slight setback when part of her spinal incision came open and developed an infection.  Luckily, Dr Ujjwal Debnath, based in India, was able to re-operate on Shruti’s back, wash out the wound and clear the infection. She required additional hospitalization and antibiotic therapy but ultimately recovered.

Post-operative X-ray showing excellent correction of the scoliosis deformity.

Shruti walking on 4th post-op day with OSS team member Bethan Salmon, RN