Compartment Syndrome of the Thigh in a Patient with Fibrodysplasia Ossificans Progressiva

Frederick S Kaplan; Mona Al Mukaddam; Robert J Pignolo

doi:10.13107/jocr.2020.v10.i03.1770

Compartment Syndrome of the Thigh in a Patient with Fibrodysplasia Ossificans Progressiva

J Orthop Case Rep. 2020 May-Jun;10(3):103-107. doi: 10.13107/jocr.2020.v10.i03.1770.

Authors

Frederick S Kaplan^{1

2

3}, Mona Al Mukaddam^{1

2

3}, Robert J Pignolo⁴

Affiliations

¹ Department of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA.
² Department of Medicine, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA.
³ The Center for Research in FOP and Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA 19104, USA.
⁴ Department of Medicine, The Mayo Clinic, Rochester, MN 55905, USA.

Abstract

Introduction: The severe pain that commonly accompanies appendicular flare-ups of fibrodysplasia ossificans progressiva (FOP) is often ascribed to compartment syndrome, but no documentation exists.

Case report: We revisited the case of an adult with classic FOP who underwent measurement of compartment pressure of the thigh during an acute, severely painful flare-up of the thigh. The intracompartmental pressure of the thigh was measured at 95--110 mm of mercury (normal compartment pressure is 0--8 mmHg). A fasciotomy of the thigh was performed. Despite immediate post-operative relief of pain, progressive heterotopic ossification and loss of function of the hip and knee occurred.

Conclusion: This unique case documents and confirms the suspected presence of compartment syndrome during an acute flare-up of FOP and has vital implications for understanding the pathophysiology and care of patients with acute appendicular flare-ups of FOP and for the design of emerging clinical trials.

Keywords: Fibrodysplasia ossificans progressivaa (FOP); activin receptor A; bone morphogenetic protein signaling; compartment syndrome; heterotopic ossification; type I (ACVR1).

Publication types

Case Reports