Journal of Pediatric Orthopaedics - 2026-07-23 - Journal Article
Outcomes of Spinal Deformity Treatment in Asphyxiating Thoracic Dystrophy.
Kourpas KM, Stelzer JW, Jordan JV, Montgomery BK, Flynn JM, Cahill PJ, Anari JB
Topics
Key Takeaway
In 6 ATD patients undergoing PSF at median age 14 years, median blood loss was 500 mL with a grade IIIb complication rate of 1/6 (17%), suggesting PSF is feasible despite severe restrictive lung disease and prior VEPTR surgery.
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Summary
This retrospective series examined perioperative and postoperative outcomes of PSF for EOS in 6 patients with Jeune syndrome, 5 of whom had prior VEPTR expansion thoracoplasty. Median preoperative major coronal curve was 60°; instrumentation spanned T2 to T12–L4. Median ICU stay was 4 days, total hospitalization 6 days, and one patient required emergency bronchoscopy (Clavien-Dindo-Sink grade IIIb).
Key Limitation
With only 6 patients and no reported postoperative radiographic or pulmonary function follow-up, neither curve correction durability nor respiratory trajectory after PSF can be assessed.
Original Abstract
BACKGROUND
Asphyxiating thoracic dystrophy (ATD), or Jeune syndrome, is a rare skeletal dysplasia marked by a severe hypoplastic chest wall and restrictive lung disease, leading to an often-lethal thoracic insufficiency syndrome. Early-onset scoliosis (EOS) can complicate ATD, but details of operative management remain limited. This study aims to analyze the peri- and postoperative course of children with ATD who underwent posterior spinal fusion (PSF).
METHODS
A retrospective review was conducted for all patients with ATD treated for EOS who underwent PSF at our institution. Preoperative spinal deformity at the time of definitive fusion and prior growth-friendly procedures were assessed. Intra- and postoperative data, including instrumentation levels, operative time, estimated blood loss, hospital and ICU stay, and complications, were collected and analyzed descriptively.
RESULTS
Six patients met inclusion criteria. Five had previous expansion thoracoplasties with VEPTR instrumentation; median age at first surgery was 9 months. Median age at scoliosis onset and PSF was 35 months and 14 years, respectively. Median preoperative major coronal curve was 60 degrees. The sagittal profile ranged from severe lordosis to hyperkyphotic. Three patients had VEPTR instrumentation at the time of PSF. The most common upper instrumented vertebra was T2, and the lowest instrumented level ranged from T12 to L4. Median estimated blood loss was 500 mL with 175 mL of autologous blood salvage. Two patients required intraoperative blood transfusions. Median intensive care unit and total hospitalization were 4 and 6 days, respectively. Using the modified Clavien-Dindo-Sink classification system, 1 patient had a grade 0, 1 had a grade I, and 1 had a grade IIIb complication due to an emergency bronchoscopy.
CONCLUSIONS
ATD is well-known for resulting in thoracic insufficiency syndrome due to a volumetric decrease of the thoracic cage. This series indicates that ATD is not unlike other pediatric non-idiopathic scoliosis undergoing PSF. Despite the pulmonary complexity and history of multiple prior thoracic cage procedures, ATD patients can and should safely undergo spinal deformity correction if indicated.
LEVELS OF EVIDENCE
Level IV.