Acute paediatric compartment syndrome of the hand caused by hereditary angiooedema

Abstract

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.Paediatric compartment syndrome is a rare condition. Compartment syndrome of the leg and the forearm are much more common and are well described in literature. The average annual incidence is given as 7.3/100,000 for men and 0.7/100,000 in women.1 The incidence of compartment syndrome of the hand is not documented although there are a limited number of anecdotal reports. Trauma, for example in the form of crush injuries, venomous animal bites, 2 vascular injuries and burns, 3-5 is the most common underlying aetiology behind these case reports. There is one report of Henoch-Schönlein purpura 6 causing an acute compartment syndrome of the hand. We describe the first reported case of an acute hand compartment syndrome caused by hereditary angiooedema. case historyA 13-year-old Asian, right hand dominant girl presented with progressive left hand swelling (Fig 1) and a 5-day history of fever and malaise. There was no significant history of trauma, fall or any animal or insect bites. She had suffered from previous episodes of idiopathic swelling over the last two years on a monthly basis affecting both hands and legs as well as her head and face. She was otherwise fit and healthy.The patient was referred to the orthopaedic service five days into her illness. She was haemodynamically stable and apyrexial with gross, painful, oedematous swelling of the left hand. Digital perfusion was not impaired and she had paraesthesia in a median nerve distribution. Radiographs were normal and blood tests including a full blood count and inflammatory markers were also normal.A clinical diagnosis of compartment syndrome was made and the patient was taken to emergency theatre for a fasciotomy (Fig 2). Intraoperatively, a tight carpal tunnel was released. Necrotic fascia and muscle was found in the mid palmar space. The thenar eminence was normal and the recurrent palmar branch of the median nerve was preserved. Intermetacarpal spaces were also released, which again showed evidence of a necrotic brown exudate. A sample of this fluid was sent for microscopy and culture and went on to show no evidence of any organisms. In addition, the patient's blood and urine cultures were also negative. Postoperatively, the left limb was kept elevated and intravenous antibiotics were administered prophylactically after surgery until the result of the microbiology sample was known.At the 12-week foll...

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Acute Compartment Syndrome of the Hand in a 14-Month-Old Child: A Case Report

“…The etiologies of ACS in children are numerous (Table 1); however, they could vary significantly based on the age group [5][6][7]. In general, trauma or surgical positioning are the usual causes in children above 14 years of age.…”

“…Besides trauma, the etiologies of ACS are diverse. These could include animal bites, hemorrhages, or in rare cases, hereditary angiomas, as reported in some case reports [5][6][7].…”

Perioperative anesthetic management in pediatric hereditary angioedema; case report

“…Clinical presentations in combination with a positive family history should raise suspicions regarding HAE [15]. However, 25% of HAE is the result of spontaneous mutation and a novel mutation may explain cases of angioedema with no family history [11,18].…”