Working Diagnosis:
Familial Chronic Exertional Compartment Syndrome

Treatment:
All the individuals underwent anterior and lateral fasciotomies. 2 out of the 6 individuals i.e., son and daughter of sibling C, underwent posterior fasciotomies as well.

Outcome:
Following anterior, lateral, and posterior fasciotomies, all individuals had complete resolution of pain, paresthesia, and foot drop with the ability to participate in higher intensity and extended activity. The individuals who did not have posterior fasciotomies continue to experience pain in the posterior part of the leg with extended activity making them avoid longer activities.

Author's Comments:
Compartment syndrome is a painful condition caused by the increased interstitial pressure within a closed osteofascial compartment of the extremities. 95% of cases occur in the legs and 5% of cases occur in forearms, thighs, hands and feet.
Chronic exertional compartment syndrome is common, especially among young adult athletes involved in running, endurance training, soccer, field hockey, lacrosse and marching military members.
Presents with bilateral lower extremity discomfort described as squeezing, cramping, aching, or burning that typically occurs during exercise and is relieved 10 to 20 minutes after cessation of activity. CECS may also cause paresthesias, numbness, and foot drop due to compression of the nerves in the affected compartments. Most often in the anterior or lateral compartments, followed by the deep posterior and superficial posterior compartments.

Gold standard for diagnosing is measuring intracompartmental pressures with a handheld manometer. Normal pressures are between 0 and 8 mmHg.
Diagnostic criteria for chronic compartment syndrome of the leg:
A pre-exercise pressure greater than or equal to 15 mmHg, a 1-minute post-exercise pressure of greater than or equal to 30 mmHg, or a 5-minute post-exercise pressure greater than or equal to 20 mmHg. Surgical release is the standard of care for patients with moderate-to-severe CECS and for patients whose symptoms are refractory even after eliminating the trigger.

CECS may have a genetic component as presented in this case. We believe that our report is the first to discuss chronic exertional compartment syndrome in multiple members of a family of different generations.

Editor's Comments:
The authors of the case have presented a rare presentation of a relatively common medical condition. To our knowledge, it has not been reported in the medical literature. The author did provide a study found on twins which is listed in references.

It is important for the reader to know the criteria presented in the author comment section on chronic exertional compartment syndrome (CECS). Clinically, when CECS is identified, the surgical treatment is often curative for the patient. Hence the importance of diagnosing CECS.

References:
1. Chandwani D, Varacallo M. Exertional Compartment Syndrome. [Updated 2023 May 23]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK544284/

2. Banerjee, Purnajyoti, and Christopher Mclean. "Chronic exertional compartment syndrome with medial tibial stress syndrome in twins." Orthopedics 34.6 (2011): e219-e221.

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