[Surgical management of spinal deformity in a patient with Escobar syndrome: review of the literature].

BACKGROUND: The non-lethal variant of the Escobar or multiple pterygium syndrome is an entity of autosomal recessive inheritance linked to the X chromosome; it is characterized by multiple pterygia (hence its name) located mainly in the neck (95%) and armpits (55%), as well as other orthopedic malformations such as a vertical talus, congenital hip dislocation, and congenital scoliosis.

OBJECTIVE: To present an optional surgical technique for the management of severe spinal deformities.

CASE REPORT: Twelve-year-old female diagnosed with Escobar syndrome with severe scoliosis which conditions malformations of the chest with lung involvement, producing mechanical ventilatory restriction and increasing the risk of severe lower respiratory tract infection. We performed a hands-free posterior instrumentation with PASS LP system and Smith-Petersen osteotomies.

CONCLUSIONS: The Cobb angle improved from 62° to 23° and the sagittal balance from 125 mm to 73 mm.