Case Study: Congenital Scoliosis in an 18 Month-old Boy

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baby scoliosis surgery

18-month old Arannya Adhakary with congenital scoliosis waiting for his surgery

Operation Straight Spine (OSS) is an annual mission trip to Kolkata, India, where doctors, nurses and spinal monitoring technicians from the US, England and India treat patients with spinal disorders. During the 2017 trip, Dr. Jeffrey McConnell and the OSS team treated an 18-month old boy with congenital scoliosis.

Arannya Adhakary, 18 months old, was the youngest patient treated during OSS 2017.  He was diagnosed with congenital scoliosis when he was just 3 months of age.

 Congenital scoliosis can be caused by a number of different defects in the development of the spine during the first trimester of fetal growth. Abnormalities occur when part of a vertebra fails to form or several vertebra fail to separate during the very earliest stages of fetal development when the spine and other major organs are starting to form. Patients can have a single abnormality or multiple defects throughout the spine. 

Arannya’s congenital spine defect was known as a hemivertebra, which occurs when the vertebra is triangular in shape instead of the normal rectangular shape.   This type of congenital defect has a very high propensity to cause a severe and progressive deformity of the spine, usually a kyphosis or scoliosis,  as the child grows.

Arannya’s hemivertebra was located at the 11th thoracic vertebra, approximately in the middle of his spine. Hemivertebra are best treated at an early age by removing the abnormal vertebra, correcting the deformity and fusing the growth plates above and below the hemivertebra so the deformity does not recur. Arannya’s surgery consisted of removal of the T11 hemivertebra, correction of the local kyphosis and scoliosis, and limited fusion from T10 to T12.  To stabilize the spine and help fuse where the hemivertebra was removed, two small screws and a single 3.5mm diameter rod was placed from T10 to T12 on the right side.

Arannya did amazingly well after surgery, a testament to his young age, and by the 4th postoperative day was going on walks in the courtyard, smiling, carried in his mother’s arms.

scoliosis deformity xray

Pre-operative MRI of the thoracic spine showing the T11 hemivertebra and acutely angled scoliosis deformity.

scoliosis postop xray

Post-operative X-ray after removal of the T11 hemivertebra and placement of spinal screws and rod.

india scoliosis

Four days after surgery on walk with his mother.

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

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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