Escobar syndrome (Multiple pterygium syndrome) associated with Thoracic Kyphoscoliosis, Lordoscoliosis, and Severe Restrictive Lung Disease: A Case Report

HSS Journal


Oheneba Boachie-Adjei, MD

Oheneba Boachie-Adjei, MD

Chief Emeritus of Scoliosis Service, Hospital for Special Surgery
Attending Orthopedic Surgeon, Hospital for Special Surgery
Professor of Orthopedic Surgery, Weill Cornell Medical College

Christopher C. Dodson, MD
Scoliosis Service
Department of Orthopedic Surgery
Hospital for Special Surgery

Abstract
Background: Escobar syndrome or multiple pterygium syndrome is characterized by a web across every flexion crease in the extremities, most notably the popliteal space. Additionally, this syndrome is associated with two other structural anomalies: a vertical talus and congenital lordoscoliosis. We present a case report of a patient with escobar syndrome who was initially managed conservatively, and subsequently, had severe and debilitating progression and respiratory decompensation ultimately requiring surgical intervention.

Study Design: Case report

Methods: After preoperative evaluation by a pediatrician, pulmonologist, and otolaryngologist, the patient underwent one stage anterior and posterior spinal fusion with instrumentation as well as multiple osteotomies, rib resections and vertebrectomies.

Results: The patient's postoperative course was complicated by wound necrosis requiring irrigation and debridement, a urinary tract infection, and a tracheostomy for persistent atelectasis. The patient eventually recovered from all complications. There were never any focal neurologic deficits. The patient has 3 year follow up with radiographically confirmed maintenance of correction. Fusion has been obtained in the anterior and posterior segments. Clinically, the patient is able to stand upright, can participate in functional activities, and has not required any pain medication. The patients' functional vital capacity improved from 23% predicted preoperatively to 60% predicted postoperatively.

Conclusions: Patients with severe spinal deformity secondary to escobar syndrome can be successfully treated surgically. We propose early surgical intervention in this group in order to prevent curve progression, restrictive lung disease and the need for complex salvage procedures.

This article appears in HSS Journal: Volume 1, Number 1.
View the full article at springerlink.com.


About the HSS Journal
HSS Journal, an academic peer-reviewed journal, is published twice a year, February and September, and features articles by internal faculty and HSS alumni that present current research and clinical work in the field of musculoskeletal medicine performed at HSS, including research articles, surgical procedures, and case reports.

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