Acute paediatric compartment syndrome of the hand caused by hereditary angiooedema

Compartment syndrome of the leg and forearm are well described in the literature. However, compartment syndrome of the hand is rare and in children it is even rarer. Atraumatic hand compartment syndrome has not to our knowledge been previously reported. We describe a case of an atraumatic compartment syndrome of the hand in a child who underwent an urgent fasciotomy. The child was diagnosed with hereditary angiooedema. We highlight a rare but serious complication of a hereditary disease not commonly seen by the surgical community. We hope that this report raises the awareness of this condition, thereby reducing delays in reaching a prompt diagnosis.