Outcomes of Growth-Friendly Surgical Treatment of Early Onset Scoliosis in Children With Prune Belly Syndrome: A Preliminary Report

BACKGROUND: Prune belly syndrome (PBS) is a rare condition characterized by absence of abdominal musculature, cryptorchidism, and obstructive uropathy. The most common orthopaedic problem is scoliosis, yet no reports on growth-friendly surgical treatment of early-onset scoliosis (EOS) exist. Our purpose was to evaluate outcomes of distraction-based implants in children with PBS.

METHODS: This was a multicenter retrospective review. Children with PBS treated with TGR, MCGR, or VEPTR with a minimum 2-year follow-up were identified. Demographics, radiographic/surgical data, complications, and unplanned returns to the operating room (UPROR) were collected. Quality of life was measured using EOSQ-24.

RESULTS: Seven patients (100% male) were identified. The mean age at index surgery was 5.6 years. The mean length of follow-up was 4 years (range: 2 to 11 y). Implant types included MCGR (4 patients), TGR (2 patients), and VEPTR (1 patient). Two patients had pelvic fixation. The mean major curve magnitude was 87 degrees preindex, 47 degrees postindex, and 53 degrees at the last follow-up. Mean kyphosis was 55 degrees preindex, 25 degrees postindex, and 42 degrees at the most recent follow-up. Mean T1-T12 and T1-S1 lengths increased 4.4 and 4.8 cm, respectively, between preindex and last follow-up.Complications occurred in 3 patients. One patient had 3 TGR breakages resulting in 3 UPRORs to revise the rods and 1 superficial wound infection treated with antibiotics. Another patient was readmitted for respiratory failure after TGR lengthening and a third patient had a superficial infection manifested by incisional swelling after MCGR insertion that resolved with antibiotics. Two patients have undergone definitive fusion and 1 patient has retained his TGR definitively. The other patients are still undergoing rod lengthening.

CONCLUSIONS: Children with PBS can develop severe scoliosis at a young age. Distraction-based implants can successfully control scoliosis while allowing spinal growth. The complication rate is comparable to patients with idiopathic EOS treated with growth-friendly implants.

LEVEL OF EVIDENCE: Therapeutic level IV.