Title

Rapidly progressive scoliosis in a patient with Marshall-Smith Syndrome

Authors

Michael Korn, Henry Ford Health SystemFollow
Mohsin Fidai, Henry Ford Health System
Kyle Morgenstern, Henry Ford Health System
Joseph Perra, Henry Ford Health System

Files

Program

Orthopedic Surgery

Training Level

Resident PGY 1

Institution

Henry Ford Hospital

Abstract

Case: Marshall-Smith syndrome is a rare genetic condition due to mutations in the NFIX gene resulting in a multitude of congenital abnormalities, including atlantoaxial instability and ligamentous laxity. This case report describes a patient with Marshall-Smith syndrome who developed atlantoaxial rotatory subluxation and subsequent rapidly progressive scoliosis after occiput-C2 fusion. She was treated with 4 weeks of halo traction, followed by an occiput to T3 posterior spinal fusion, segmental fixation, and magnetic growth rods from T3-L4. The thoracic curve was reduced from 125 to 34 degrees.Conclusion: The presence of atlantoaxial rotatory subluxation in the setting of generalized ligamentous laxity likely contributed to the rapid progression of scoliosis in this patient and should be recognized.

Presentation Date

5-2019

Recommended Citation

Korn, Michael; Fidai, Mohsin; Morgenstern, Kyle; and Perra, Joseph, "Rapidly progressive scoliosis in a patient with Marshall-Smith Syndrome" (2019). Case Reports. 67.
https://scholarlycommons.henryford.com/merf2019caserpt/67