Apnoea episodes in Hirschsprung's disease and the anaesthesia implications of neurocristopathies

Perioperative Care of a Patient With Waardenburg Syndrome

“…As with other chromosomal and genetic abnormalities, Waardenburg syndrome results in a specific pattern of endorgan involvement and anatomical malformations which may require surgical intervention. Although the disease has an incidence of 1 in 40,000, there remains limited information regarding anesthetic care for these patients [2,5,6,[11][12][13][14][15][16][17][18]. As with all intraoperative care, the anesthetic approach begins with a thorough preoperative evaluation and identification of the endorgan involvement.…”

Anesthetic management of Shah-Waardenburg syndrome: Experience of two cases and review of literature

“…[3] Neurocristopathies are disorders caused by an alteration in the migration of the neural crest cells during the embryonic phase, which causes the association of different anomalies. [4] Other features associated with WS include urinary system abnormalities, neural tube defects, Sprengel shoulder, cleft-lip or palate, facial nerve palsy and plicated tongue, laryngomalacia,[5] and severe cyanotic cardiopathy. [6] [Mutations in PAX gene on chromosome 2 are seen in WS I and III, and MITF mapped on chromosome 3 in type II.…”

Congenital central hypoventilation syndrome in children: a Hong Kong perspective

“…Complications can occur after neuromuscular block. [60][61][62] Patients with CCHS are sensitive to sedatives and narcotics. Significant respiratory depression can occur after non-oral opioid administration 60,63,64 ; short-acting sedatives are recommended.…”

“…[60][61][62] Patients with CCHS are sensitive to sedatives and narcotics. Significant respiratory depression can occur after non-oral opioid administration 60,63,64 ; short-acting sedatives are recommended. 60,65 Electrocardiography can detect bradycardia and arrhythmias; glucose monitoring can identify hypoglycaemia or hyperglycaemia.…”