Review Question - QID 215547

The patient is presenting with newly diagnosed congenital scoliosis which should be further evaluated for coexisting cardiac and renal defects.

Congenital scoliosis is abnormal vertebral development that typically occurs at 4 to 6 weeks of gestation. As a result, hemivertebra or block vertebrae can form, which can lead to asymmetric growth of the spinal column and progressive deformity. The heart and kidneys also develop during weeks 4-6 of gestation, which may be affected by the same process causing the vertebral abnormalities. As such, patients with congenital scoliosis should be evaluated for cardiac and renal defects with an echocardiogram and renal ultrasound.

Pahys and Guille reviewed the recent literature on the treatment of congenital scoliosis. The literature supports delaying surgical treatment with derotational casting to avoid the crankshaft phenomenon with early fusion and minimize the complication rate associated with growth-friendly constructs. Recent literature has demonstrated magnetic rods to be as effective with respect to curve correction as traditional growing rods in pre-adolescent patients in short-term follow-up studies.

Figure A is the AP radiograph of the spine with a congenital fully segmented hemivertebrae at L3.

Incorrect answers
Answer 1: TLSO bracing has not shown to have any effect on curve progression in congenital scoliosis. Derotational casting has shown to delay the need for growing constructs.
Answer 3: The best indication for an isolated hemivertebrae excision is for a fully segmented hemivertebrae at the lumbosacral junction prior to the age of 5 years. A long thoracolumbar fusion at 18 months of age is not ideal as this would likely lead to crankshaft phenomenon and limit T1-S1 growth.
Answer 4: VEPTR rods are ideal for patients at risk for thoracic insufficiency syndrome. This patient's curve is limited to the lumbar spine and not likely to benefit from VEPTR rods.
Answer 5: Routine follow-up may be an appropriate option if there is minimal curve progression with serial radiographs. However, this patient will need a cardiac and renal evaluation prior to any further treatment decision.