Anesthetic management of a child with chromosome 22q11 deletion syndrome

Abstract

Chromosome 22q11 deletion syndrome (22q11DS) is a congenital anomaly characterized by cardiovascular, oropharyngeal, immunologic, endocrine, and neurodevelopmental abnormalities. We successfully managed a 6-year-old girl with 22q11DS with general anesthesia. Potential problems in anesthetic management of patients with 22q11DS are reviewed.

CHD associated with syndromic diagnoses: peri-operative risk factors and early outcomes

“…25 Congenital malformations including cleft palate, sub-mucous clefts, retrognathia, Pierre Robin sequence, congenital laryngeal web, and vascular ring may complicate airway management. 81,94,95 Bronchomalacia and bronchospasm have been observed in patients with 22q11 deletion and pulmonary atresia with ventricular septal defect, which may be related to compression by aortopulmonary collateral vessels. 96,97 Although prolonged mechanical ventilation was not observed after unifocalisation of the major aortopulmonary collateral arteries, 98 increased post-operative respiratory complications including prolonged intubation and post-extubation stridor have been observed.…”

Síndrome de deleção 22q11.2: compreendendo o CATCH22

“…Assim, o reconhecimento da síndrome não apenas permite identificar e tratar anormalidades associadas, mas também influencia no aconselhamento genético aos familiares (6,12,52) . Além disso, a presença de anormalidades orofaríngeas, laringobrônquicas, imunológicas, endócrinas, psiquiátricas e cardiovasculares, frequentes na SD22q11, pode levar a várias complicações clínicas tanto no momento da indução anestésica como durante e após a realização de procedimentos cirúrgicos (53) . Recentemente, houve relatos de que esses pacientes apresentam predisposição maior do que a população em geral para o desenvolvimento de certos tumores pediátricos, como o hepatoblastoma (54)(55) .…”