BACKGROUND: Escobar syndrome or multiple pterygium syndrome is characterized by 
a web across every flexion crease in the extremities, most notably the popliteal 
space. In addition, 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 
had a 3-year follow-up with radiographically confirmed maintenance of 
correction. Fusion was 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 patient's 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 to prevent curve progression, restrictive lung disease, and the 
need for complex salvage procedures.