Anaesthetic management of an obstetric patient with cleidocranial dysplasia

Abstract

Cleidocranial dysplasia (CCD) is an autosomal dominant disease, characterised by generalised skeletal dysplasia, which has an estimated incidence of 1 in 1 000 000.1 Despite being a rare genetic condition, CCD has significant anaesthetic implications due to abnormal anatomical features of the head, mouth, neck and spinal column. These features have an impact on both general and neuraxial anaesthetic techniques.

Craniofacial abnormalities resulting from CCD often present as a large brachycephalic head, with mandibular prognathism and maxillary underdevelopment. A depressed nasal bridge and hypoplastic sinuses disrupt nasal breathing, predisposing to recurrent upper respiratory tract infections.2 Permanent delayed closure of fontanelles and cranial sutures affect the majority of patients and may have implications for patient positioning under anaesthesia. Up to 94% of patients with CCD have dental findings leading to overcrowding and malocclusion.3, 4 In addition, absent or hypoplastic clavicles, short ribs, narrow upper thoracic diameter or funnel-shaped chest may lead to respiratory insufficiency, which is important for the early post-operative period, especially after general anaesthesia.2