Abstract
Hereditary angioedema (HAE) is a rare disorder caused by a deficiency of C1 esterase inhibitor. Minor trauma and emotional stress are the most common initiating events leading to contact system activation and excessive uncontrolled bradykinin release. This manifests as angioedema, a vascular reaction of the deeper layers of the skin and mucous membranes, with vasodilatation and increased permeability resulting in tissue swelling. Severe angioedema can occur in the perioperative period, leading to fatal airway obstruction. We describe the anaesthetic management of a child with HAE for dental rehabilitation and provide an review of the relevant literature.
Highlights
Hereditary angioedema (HAE) is a rare disorder caused by a deficiency of C1 esterase inhibitor
A 6-year-old girl weighing 15 Kg who had been diagnosed with C1 esterase inhibitor (C1-INH) deficiency was admitted to Sultan Qaboos University Hospital (SQUH), Oman, for dental rehabilitation from multiple dental caries
There were more aggressive attacks with acute dyspnoea, dysphagia and diarrhoea combined with the progressive swelling of the right side of the neck for which she required admission to the intensive care unit (ICU) for observation; no endotracheal intubation was required
Summary
إدارة التخدير لمريض الوذمة الوعائية الوراثية مع إعطاء الجرعة الوقائية من استريزC1 مثبط. Abstract: Hereditary angioedema (HAE) is a rare disorder caused by a deficiency of C1 esterase inhibitor. Minor trauma and emotional stress are the most common initiating events leading to contact system activation and excessive uncontrolled bradykinin release. This manifests as angioedema, a vascular reaction of the deeper layers of the skin and mucous membranes, with vasodilatation and increased permeability resulting in tissue swelling. Hereditary angioedema (HAE) is caused by a deficiency of C1 esterase inhibitor (C1-INH). C1-INH is a serine protease which inhibits "Activated Factor XIIa" and "Kallikrein" Deficiency of this key inhibitor leads to uncontrolled activation of the Contact System thereby generating profuse amounts of Bradykinin; which is a potent local vasodilator. Airway oedema and hypovolemic shock due to the tissue leak of fluids are especially significant in the perioperative period, challenging even the most experienced anaesthesiologist
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