Compartment syndrome in a patient with familial rhabdomyolysis: a case report.

Abstract

In clinical terms, compartment syndrome is caused by an increase in the pressure of the fluid within an anatomically defined compartment and is exacerbated by a decrease in the size or elasticity of the compartment 1. The causes of increased pressure include intracompartmental bleeding due to trauma, muscle contusion leading to extravasation of fluid into the compartment, or hypoxemia secondary to arterial or venous occlusion. There have, however, been various reports of spontaneous compartment syndrome in patients without a clear history of preceding trauma 2-6. We describe an unusual case of an eight-year-old girl who had spontaneous development of bilateral anterior compartment syndrome of the leg. Despite the initial complete paralysis of the anterior compartment muscles, early recognition and appropriate surgical decompression led to complete functional recovery of the affected muscles in both legs. The case of this patient emphasizes the importance of considering the diagnosis of compartment syndrome in patients with familial rhabdomyolysis who have development of myalgia, swelling, and muscle weakness. An eight-year-old girl was in good health until two days prior to presentation, when pain and swelling developed in both legs. She was evaluated in a local hospital and then was transferred to the pediatric intensive care unit at our institution because of deteriorating renal function. Soon after she arrived, the pediatric orthopaedic team was consulted to evaluate the bilateral lower-extremity pain. There was no history of rash or drug ingestion prior to presentation and no evidence of intercurrent infections or illnesses. The patient's brother had a similar clinical problem at the age of two years. He had swelling in the compartments of the upper and lower extremities and myalgias, which led to renal and cardiac failure with a fatal outcome. The patient had a temperature of 36.8dC, a pulse rate of 140 …