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Characterization of the development of acute-on-chronic exertional compartment syndrome: a case report of symmetric compartment syndromes and review of the literature.

Abstract

Acute-on-chronic exertional compartment syndrome is a rare and severe progression of the likely common and more benign chronic exertional compartment syndrome. This is a report of one 17-year-old male on a pediatric inpatient service with bilateral anterior leg pain of unknown origin. Because of the nonspecific nature of pain, a high level of suspicion is required for timely diagnosis to avoid compartment ischemia and irreversible soft tissue and nerve damage. While high-energy orthopaedic trauma, orthopaedic surgery, or closed reduction and casting are common preceding events for compartment syndrome, this patient presented with acute-on-chronic exertional compartment syndrome. A dearth of literature of this condition hampered its morbidity-sparing diagnosis. While there is a spectrum of clinical findings for the acute decompensation of chronic exertional compartment syndrome, like any compartment syndrome, pain disproportionate to physical exam is the most sensitive sign. Understanding the exertional compartment syndrome spectrum is tantamount to avoid the devastating complications of a missed diagnosis of acute compartment syndrome.

Chronic exertional compartment syndrome (CECS) is a rarely diagnosed but likely common clinical entity. It was first noted in 1956 in a professional soccer player who complained of insidious onset of tightness-like pain that spanned the full length of the anterior leg, below the knee, eventually involving both legs. (1) The prevalence of this condition is difficult to discern, as onset is generally insidious, resulting in reduction or cessation of inciting factors, without seeking medical care.

Acute-on-chronic exertional compartment syndrome (ACECS) is a rare sequela of CECS. The cardinal feature of this devastating condition is unrelenting pain that previously had resolved with rest. Findings on presentation include the variable constellation of the major symptoms of any acute compartment syndrome (ACS): pain, pulselessness, pallor, paresthesias, paralysis, and poikylothermia. (2-6) However, while one study demonstrated that nearly 90% of patients with (ACS) of any etiology presented with pain, at least 25% did not have any other aforementioned symptom. (7) More specific symptomatology is associated with advanced disease and poor prognosis. CECS is an exceedingly rare precipitant of ACS (thus ACECS) that is poorly described in the literature, thus serves as a risk for great morbidity.

We present the first known case of a pediatric patient with ACECS after years of training in parkour, an evolving sport that involves movement (i.e., jumping, scaling, self-propelling, and four limb involvement) to overcome natural obstacles to reach a predetermined location. (8)

Case Report

A 17-year-old boy with mild intermittent asthma and sickle cell trait presented with a 1-day history of persistent pain in his anterior lower legs. He had a 3-year history of transient, achy, non-localizing pain in the anterior lower legs that was made worse by activity and improved by rest, never requiring analgesic treatment. However, one day prior to presentation, he felt an acute increase in pain bilaterally while jogging for approximately 3 minutes to catch a bus, despite no injury. The new leg pain was squeezing in nature and did not remit with rest. Later that day, he played laser tag despite persistent, but tolerable, pain. At home, he used 400 mg/76 mg of oral ibuprofen/diphenhydramine and 650 mg of oral acetaminophen without relief. Additionally, neither shin massaging nor stretching the anterior legs by plantar flexing his ankles improved his pain. A review of systems was otherwise negative. Social history was significant for denial of drug and alcohol use and 3 to 6 hours per week of attending a gym that teaches advanced parkour.

Emergency department (ED) exam revealed vital signs that were noteworthy for bracial blood pressure of 155/77. The patient was reserved and did not show signs of significant discomfort. His legs were nonedematous and normal in color and temperature, but there was symmetric, mild tenderness to palpation between the tibial tubercle and the ankle on the anterior surface without point tenderness. Range of motion at the knee and ankle were full, but the patient was unsure whether his shin pain changed with passive or active plantar flexion bilaterally. Examination of the thigh and remainder of the lower legs were normal. Pulses in the dorsalis pedis and posterior tibial arteries were present and normal bilaterally. Sensation to soft touch and pinprick were normal throughout both legs. The feet appeared normal in color and temperature without swelling or pain. There was no peripheral motor defect.

Laboratory exam was significant for a creatinine kinase (CK) of 8,563 and heme-positive urine and otherwise normal for electrolytes, blood urea nitrogen (BUN) and creatinine, complete blood count, and urine drug screen. Radiographs of both legs were normal (Fig. 1). Orthopaedic ED consult was negative, and he was admitted to the inpatient pediatric floor for intravenous fluids to protect against rhabdomyolysis, with myoglobinuria suspected to be secondary to overuse. The working diagnosis for his pain at this time was medial tibial stress syndrome (shin splints).

Over the next 48 hours, the patient was continued on IV fluids and started on 650 mg of acetaminophen every 6 hours as needed and 5 mg of oxycodone every 4 hours as needed; the patient requested acetaminophen sporadically, five times, and never requested oxycodone. Acetaminophen continued to provide no relief. His lab results were significant for an uptrending creatine kinase (CK) on consecutive morning blood draws: 12,333 and 24,723 U/L. By day 2 of admission, urinalysis became negative for heme, with a persistently normal serum creatinine, blood urea nitrogen (BUN), and electrolytes. After 55 hours on the inpatient service, the patient described a progression in pain. Exam now revealed warmth and tension of both anterior lower legs without any other changes noted in sensation, motor function, pulse quality, or color. Repeat pediatric orthopaedic, as well as vascular, consults were obtained on suspicion of compartment syndrome, and given the warmth of the leg--an MRI was ordered to rule out fasciitis, myositis, and osteomyelitis.

MRI of the lower extremities showed anterior leg compartments edema bilaterally but was otherwise negative (Fig. 2). Following that exam, pressure transduction of the bilateral anterior compartments revealed pressures of 71 and 73 mmHg, with all other leg compartment pressures being normal. Blood pressure at this time was 139/70. Given the history of chronic activity-associated anterior lower leg pain, frequent parkour training, and acute increase in pain while running, the diagnosis of ACECS was made. Emergent bilateral anterior fasciotomies were performed under general anesthesia 57 hours after hospital admission. The wounds were not closed at this time; they were managed with daily wet to dry dressing changes, and staged closure by primary intention or skin graft was planned after reevaluation of tissue viability and resolution of edema. The immediate postoperative course was complicated by complete and persistent loss of dorsiflexion bilaterally, postoperative day 4 Pseudomonal wound infection of necrotic tissue requiring two rounds of soft tissue debridement in the anterior and lateral compartments bilaterally, and ultimately split-thickness skin grafting to cover the wounds. The patient's complex course ultimately resulted in bilateral foot drop, likely secondary to both nerve injury and muscular compromise.

Discussion

We present a case of parkour-induced CECS that decompensated into ACECS during a short jog by a young patient with sickle cell trait and lower leg pain with an otherwise normal physical exam. While professionally-trained parkour greatly emphasizes safety, it does so by distributing maximal vertical force, a measure of landing intensity, to muscular tissue to avoid skeletal injury. (8) As a consequence, muscle demand is great, and muscular injury is the second most prevalent injury in the sport. (9) Because of the rarity of ACECS and scarcity of description in literature combined with this patient's seemingly benign presentation coupled with his stoic nature there was a low index of suspicion for compartment syndrome.

CECS is thought to be common in the running athlete and active military members. A retrospective study of all United States military personnel from 2006 to 2011 was performed using the Defense Medical Epidemiology Database, (10) revealing that the diagnosis of CECS was made in 0.49 cases per 1,000 person-years. Although there are no published guidelines for this condition, a case series of 15 patients with CECS revealed mildly elevated pressure to an average of 22 mmHg (normal: < 10), with exercise pressures elevated to 80 mmHg (normal: < 30), and return to resting pressure in an average of 40 minutes. (11) It is thought that chronic compartment pressures above 30 mmHg result in an inability for intracapillary pressure to overcome compressive forces. (11) The pathophysiology of CECS is hypothesized to be the summation of a poorly compliant fascial sheath that cannot respond to the increase in volume secondary to augmented blood flow during muscular activity and chronic hypertrophy of the muscle. (12)

The most common presentation of a patient with CECS is an endurance athlete complaining of bilateral (68% of the time) leg claudication-like pain localized to the anterior compartment (in 70%). (13) The pain generally begins within minutes of starting the offending exercise and improves with rest. The transient nature of CECS results in a normal physical exam and rare emergent presentation. Because exertional pain is nonspecific, a broad differential diagnosis must be considered, including medial tibial stress syndrome, stress fracture, popliteal artery entrapment syndrome, nerve entrapment, muscular strain, Baker's cyst, periostitis, and fascial weakness leading to muscle herniation. (13,14)

While CECS is a well-described condition, there are currently no accepted explanations for the deleterious progression of CECS into ACECS owing to the paucity of cases of ACECS. (2-5) Multiple studies report sickle cell trait, a known condition in our patient, as a possible precipitating factor for sudden irreversibility of increased compartment pressure. (4,6) A mechanism has been proposedby Dincer and Raza (Fig. 3) in which sickle cell trait can trigger a compartment syndrome following physical activity--even during typical activity for a well-trained individual. (6) An exercised and poorly-perfused muscle becomes a functionally hypoxemic focus for sickling, allowing for the cascade described in Figure 3. (4,6) We propose that this patient's apparent CECS led to a subclinical cycle of rhabdomyolysis caused by muscle ischemia and elevated compartment pressures. Chronic and repeated sickling-induced minor vaso-occlusive events during his parkour training sessions peaked when the patient switched exercise modes to endurance running, and alterations in exercise patterns have been hypothesized to be another contributing factor in acute exacerbation of CECS. (3)

ACECS is a rarely described condition with a seemingly benign presentation due to lack of clear injury. Due to its vascular sequelae, examining physicians may cite normal pulses as evidence against ACS. However, in ACECS, the anterior tibial artery is the affected arterial source; its distal continuation, dorsalis pedis, may be normal due to augmented collateralization from the peroneal artery in a patient with CECS.

Unfortunately, disease rarity and challenge in recognition are complicated by the risk of permanent neurologic deficit, amputation, or even mortality with delayed diagnosis. (6) Retrospective examination of this case reveals classic ACS pain descriptors that should have raised immediate suspicion of this condition but that did not because of the poor characterization of this phenomenon. We recommend recognition of additional features, such as chronic bilateral anterior leg pain, a history of high-impact exercise with exertional leg pain, (12) a persistently elevated CK greater than 4,000 U/L, (15) and sickle cell trait, (4,6) as additional criteria that should raise concern for ACECS.

Conclusion

Despite its atraumatic origin, cases of ACECS will have an orthopaedic consult by the emergency department. In a fit and active patient with a relatively benign presentation for their bilateral lower leg pain, the patient is at risk for being discharged with outpatient follow-up. To consider ACECS, it is critical to understand the pathophysiology and risk factors for CECS and the possibility of its decompensation into ACECS. Suspicion should be based on history consistent with CECS and pain characterization typical of any ACS, regardless of the absence of other symptoms. Compartment pressures should be measured immediately, and the operating room should be activated in preparation for fasciotomy. Rapid recognition and clinical diagnosis of this rare entity is imperative for avoiding treatment delays leading to permanent sequelae such as this patient's foot drop and past reports of mortality.

Disclosure Statement

None of the authors have a financial or proprietary interest in the subject matter or materials discussed, including, but not limited to, employment, consultancies, stock ownership, honoraria, and paid expert testimony.

References

(1.) Mayor GE. The anterior tibial syndrome. J Bone Joint Surg Br. 1956 May;38-B(2):513-7.

(2.) Uzel A-P, Lebreton G, SocrierM. Delay in diagnosis of acute on chronic exertional compartment syndrome of the leg. Chir Organi Mov. 2009 Dec;93(3):179-82.

(3.) Mueller M, Dunnet W. Acute on chronic peroneal compartment syndrome. Injury. 2004 Nov;35(11):1196-9.

(4.) Hieb LD, Alexander AH. Bilateral anterior and lateral compartment syndromes in a patient with sickle cell trait. Case report and review of the literature. Clin Orthop Relat Res. 1988 Mar;(228):190-3.

(5.) Goldfarb SJ, Kaeding CC. Bilateral acute-on-chronic exertional lateral compartment syndrome of the leg: a case report and review of the literature. Clin J Sport Med. 1997 Jan;7(1):59-61; discussion 62.

(6.) Dincer HE, Raza T. Compartment syndrome and fatal rhabdomyolysis in sickle cell trait. WMJ. 2005 Aug;104(6):67-71.

(7.) Bae DS, Kadiyala RK, Waters PM. Acute compartment syndrome in children: contemporary diagnosis, treatment, and outcome. J Pediatr Orthop. 2001 Sep-Oct;21(5):680-8.

(8.) Puddle DL, Maulder PS. Ground reaction forces and loading rates associated with parkour and traditional drop landing techniques. J Sports Sci Med. 2013 Mar 1;12(1):122-9.

(9.) Wanke EM, Thiel N, Groneberg DA, Fischer A. [Parkour--"art of movement" and its injury risk]. Sportverletz Sportschaden. 2013 Sep;27(3):169-76.

(10.) Waterman BR, Liu J, Newcomb R, et al. Risk factors for chronic exertional compartment syndrome in a physically active military population. Am J Sports Med. 2013 Nov;41(11):2545-9.

(11.) Qvarfordt P, Christenson JT, et al. Intramuscular pressure, muscle blood flow, and skeletal muscle metabolism in chronic anterior tibial compartment syndrome. Clin Orthop Relat Res. 1983 Oct;(179):284-90.

(12.) Wilder RP, Magrum E. Exertional compartment syndrome. Clin Sports Med. 2010 Jul;29(3):429-35.

(13.) Turnipseed WD. Diagnosis and management of chronic compartment syndrome. Surgery. 2002 Oct;132(4):613-9; discussion 617-9.

(14.) Pell RF, Khanuja HS, Cooley GR. Leg pain in the running Athlete. J Am Acad Orthop Surg. 2004 Nov-Dec;12(6):396-404.

(15.) Valdez C, Schroeder E, Amdur R, et al. Serum creatine kinase levels are associated with extremity compartment syndrome. J Trauma Acute Care Surg. 2013 Feb;74(2):441-5; discussion 445-7.

Andrew Schwartz, M.D., Claudette Poole, M.D., F.A.A.P., and Charles Schleien, M.D., M.B.A.

Andrew Schwartz, M.D., Emory University Orthopaedics & Spine Hospital, Emory University School of Medicine, Atlanta, Georgia. Claudette Poole, M.D., F.A.A.R, University of Alabama Birmingham Pediatrics/UAB School of Medicine, Birmingham, Alabama. Charles Schleien, M.D., M.B.A., Northwell Health System, Hofstra School of Medicine, Hempstead, New York.

Correspondence: Andrew Schwartz, M.D., Emory University Orthopaedics & Spine Center, 59 Executive Park Drive South, Atlanta, Georgia 30329; amschw3@emory.edu.

Caption: Figure 1 A, AP radiograph of right leg; B, AP radiograph of left leg; C, lateral radiograph of right leg; D, lateral radiograph of left leg. There is evidence of mild, diffuse soft tissue swelling without findings of fractures or dislocations.

Caption: Figure 2 Coronal T2-MRI right (A) and left (B) leg; sagittal T2-MRI right (C) and left (D) leg; axial T2-MRI right (E) and left (F) leg. There is bilateral anterior compartment edema without fracture or soft tissue injury.

Caption: Figure 3 A proposed mechanism for the development of CECS in sickle cell trait patients. (Adapted from Dincer HE, Raza T. Compartment syndrome and fatal rhabdomyolysis in sickle cell trait. WMJ. 2005 Aug;104(6):67-71.)
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Author:Schwartz, Andrew; Poole, Claudette; Schleien, Charles
Publication:Bulletin of the NYU Hospital for Joint Diseases
Article Type:Clinical report
Date:Apr 1, 2017
Words:2637
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