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Scoliosis in Escobar Syndrome: Retrospective Review of Surgical Outcomes, Risks, and Radiographic Patterns
Andrea Fabregas1,2, Norman Ramirez2, Tyler Sparks1
1Department of Orthopedic Surgery, Shriners Children's Philadelphia, Philadelphia, PA.
Journal of Pediatric Orthopedics
|March 3, 2026
Summary
Escobar syndrome (ES) frequently involves severe scoliosis and intraspinal anomalies like tethered spinal cord. Surgical correction is effective, but VEPTR procedures have high complication rates, necessitating careful planning.
Area of Science:
- Orthopaedics
- Genetics
- Pediatric Surgery
Background:
- Escobar syndrome (ES) is a rare multiple pterygium syndrome.
- ES commonly presents with joint contractures, pterygia, craniofacial anomalies, and scoliosis.
- Limited literature exists on ES comorbidities and intraspinal abnormalities.
Purpose of the Study:
- To characterize orthopaedic manifestations in ES.
- To determine scoliosis prevalence and severity in ES.
- To identify associated neurological anomalies in ES patients.
Main Methods:
- Retrospective review of 20 ES patients with scoliosis (2000-2024).
- Data included demographics, comorbidities, spinal deformities, intraspinal MRI findings, and surgical outcomes.
- Minimum 2-year follow-up for all patients.
Main Results:
- 100% of patients had craniofacial anomalies; 43% had pulmonary and 23% cardiac issues.
- 50% of patients had intraspinal anomalies, including tethered spinal cord (40%).
- Scoliosis improved post-surgery (77° to 51°), but VEPTR had high complication rates (233%).
Conclusions:
- Scoliosis is a significant manifestation of ES, often with comorbidities and intraspinal anomalies.
- Growth-friendly spinal surgery can be effective but requires careful consideration of risks.
- Thorough preoperative imaging and surgical planning are crucial for managing ES patients.
