Journal of pediatric orthopedics · 2026
Scoliosis in Escobar Syndrome: Retrospective Review of Surgical Outcomes, Risks, and Radiographic Patterns.
СтатьяНазвание и аннотация: Перевод в очереди
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Fabregas Andrea, Ramirez Norman, Sparks Tyler, Nice Emily, Samdani Amer
Аннотация
BACKGROUND: Escobar syndrome (ES) is a rare, nonlethal form of multiple pterygium syndrome, primarily characterized by joint contractures, pterygium formation, craniofacial anomalies, and scoliosis. Although scoliosis is frequently reported in ES, there is limited literature describing the broader spectrum of clinical comorbidities and intraspinal abnormalities. This study presents the largest reported cohort of ES patients to date, aiming to characterize their orthopaedic manifestations, scoliosis prevalence, and neurological anomalies.
METHODS: A retrospective review was conducted at a single pediatric institution from 2000 to 2024. Patients with a confirmed diagnosis of ES and scoliosis were included, with a minimum of 2 years of follow-up. Data collected included demographics, clinical comorbidities (pulmonary, cardiac, craniofacial), radiographic findings, spinal deformities, intraspinal anomalies on MRI, surgical interventions, and complications.
RESULTS: Twenty patients met the inclusion criteria. Craniofacial anomalies were present in 100% of patients, while pulmonary disorders (43%) and cardiac anomalies (23%) were also common. Intraspinal anomalies were identified in 50% of patients, including a tethered spinal cord in 40%. Fourteen patients (70%) underwent spinal surgery at a mean age of 7 years (range, 1 to 14) with a mean follow-up of 7 years (range, 2 to 14). In all, 14 patients had spinal surgery, including 10 patients with growth-friendly procedures and 4 patients with an instrumented posterior spinal fusion (iPSF). The mean major curve improved from 77 degrees (range, 16 to 113) at initial presentation to 51 degrees (range, 18 to 110) at final follow-up. VEPTR was associated with the highest complication rate (7/3 patients, 233%), followed by MCGR (2/6 patients, 33%) and PSF (1/4 patients, 25%).
CONCLUSIONS: Scoliosis is a common and often severe orthopaedic manifestation in patients with Escobar syndrome, frequently accompanied by a variety of comorbidities and intraspinal anomalies such as a tethered spinal cord. Surgical correction using growth-friendly techniques can be effective, but complication rates with VEPTR remain high. These findings highlight the importance of thorough preoperative imaging and thoughtful surgical planning.
LEVEL OF EVIDENCE: Level IV, therapeutic/prognostic studies.
METHODS: A retrospective review was conducted at a single pediatric institution from 2000 to 2024. Patients with a confirmed diagnosis of ES and scoliosis were included, with a minimum of 2 years of follow-up. Data collected included demographics, clinical comorbidities (pulmonary, cardiac, craniofacial), radiographic findings, spinal deformities, intraspinal anomalies on MRI, surgical interventions, and complications.
RESULTS: Twenty patients met the inclusion criteria. Craniofacial anomalies were present in 100% of patients, while pulmonary disorders (43%) and cardiac anomalies (23%) were also common. Intraspinal anomalies were identified in 50% of patients, including a tethered spinal cord in 40%. Fourteen patients (70%) underwent spinal surgery at a mean age of 7 years (range, 1 to 14) with a mean follow-up of 7 years (range, 2 to 14). In all, 14 patients had spinal surgery, including 10 patients with growth-friendly procedures and 4 patients with an instrumented posterior spinal fusion (iPSF). The mean major curve improved from 77 degrees (range, 16 to 113) at initial presentation to 51 degrees (range, 18 to 110) at final follow-up. VEPTR was associated with the highest complication rate (7/3 patients, 233%), followed by MCGR (2/6 patients, 33%) and PSF (1/4 patients, 25%).
CONCLUSIONS: Scoliosis is a common and often severe orthopaedic manifestation in patients with Escobar syndrome, frequently accompanied by a variety of comorbidities and intraspinal anomalies such as a tethered spinal cord. Surgical correction using growth-friendly techniques can be effective, but complication rates with VEPTR remain high. These findings highlight the importance of thorough preoperative imaging and thoughtful surgical planning.
LEVEL OF EVIDENCE: Level IV, therapeutic/prognostic studies.