INTRODUCTION: There are multiple case reports of Sickle Cell Trait (SCT) with rhabdomyolysis in literature but only two case reports appear during Pubmed search having SCT with compartment syndrome (CS) and there is only one case report of SCT with compartment syndrome and rhabdomyolysis. We are presenting a case of a young army recruit with SCT who developed rhabdomyolysis and CS after strenuous exercise.
CASE PRESENTATION: A 25 year old African American active duty recruit presented to emergency department with sudden onset of bilateral leg pain following a 1.5 mile run. A similar episode of lesser severity was reported few months prior requiring no intervention. He had history of sickle cell trait (SCT). He had a family history of SCT and no drug or alcohol use. He complained of acute leg pains and was restless but cooperative. Physical examination revealed bilateral tense thighs and lower extremities with decreased lower extremity pulses on palpation. His initial laboratory workup revealed hyperkalemia, acute renal failure, very low bicarbonate and an elevated lactic acid. He was also coagulopathic and showed evidence of DIC with decreasing platelets. His hematocrit dropped to 17 within a few hours and initial CPK was found to be 70K. This quickly rose to over 200K. He was aggressively resuscitated in the medical ICU with IV fluids, packed red blood cells and fresh frozen plasma. Due to the DIC and coagulopathy, fasciotomy in legs to relieve the compartment syndrome was delayed for a few days. His ICU course was complicated by sepsis and ARDS while intubated, both of which he recovered from initially. Subsequently, he developed renal failure requiring hemodialysis. His ICU course was marked by an initial recovery followed by septic shock and subsequent death.
DISCUSSIONS: It has been well documented, over many years, the issue of acute rhabdomyolysis in African American military recruits who show evidence of sickle cell trait(SCT). In asymptomatic patients sickling is known to occur in low oxygen tension environment-most notably in the spleen and in the renal medulla. Severe exertion can cause rhabdomyolysis, limited compartment syndrome and renal failure in healthy individuals without sickle cell trait. Sickling diathesis does lower the threshold for the occurrence of compartment syndromes. In SCT patients during severe exertion, the plasma water loss causes hemoconcentration which causes increased blood viscosity and slowing of flow. This in turn causes oxygen deprivation to overworked muscles which promotes erythrocyte sickling and lactic acidosis. They then start to develop rhabdomyolysis which leads to compartment syndrome and sets up vicious cycle which fuels each other. They also develop myoglobinuria, acute renal failure, DIC, further acidosis and electrolyte abnormalities. These complications do not occur in all people with SCT. There are a number of possible risk factors why some are more susceptible to acute rhabdomyolysis and sudden death. The risk factors are deconditioning, age, exposure to extreme of temperatures, high altitude, use of weight loss supplements and last, a rare but significant factor is the co-existence of G-6-PD deficiency.
CONCLUSION: Patients with SCT are prone to develop acute rhabdomyolysis and CS after strenuous exercise. CS may be more frequent than reported and should be suspected in patients with SCT who present with rhabdomyolysis. At the same time, patients with SCT should be counseled about risk factors including strenuous exercise associated with rhabdomyolysis and compartment syndrome.
DISCLOSURE: Siddharth Shah, No Financial Disclosure Information; No Product/Research Disclosure Information