Anaesthesia for a child with Walker–Warburg syndrome

Abstract

Walker-Warburg syndrome (WWS) is a rare lethal autosomal recessive disorder manifested by characteristic central nervous system and eye malformations. We have not come across reports of general anaesthesia in a child with WWS in the English literature. We report a case of general anaesthesia in a 12-month-old male child with WWS. The child also had bilateral cleft lip, cleft palate, urogenital malformation and hydronephrosis. Despite many potential anaesthesia concerns, anaesthesia was uneventful in this child.

Perioperative considerations in Walker–Warburg syndrome

“…Regarding preoperative fasting requirements, a previous publication suggested that WWS might be associated with reduced gastrointestinal motility and an increased risk for pulmonary aspiration of gastric contents 10. We were unable to verify this information in the cited original literature 11,12, but our patient indeed showed signs of gastric retention and gastroesophageal reflux shortly after birth.…”

“…This supports our approach of maintaining spontaneous breathing until adequate mask ventilation is established, and underlines the need to be well prepared for difficult airway management. A second report described a patient with possible WWS who also presented with a difficult airway [10]. However, this patient did not have CMD, which is considered a cornerstone in the diagnosis of WWS [1].…”

“…Concerns about an increased risk for central and obstructive apnea prompted us to avoid preoperative sedation in this child with severe neurologic impairment and muscular hypotonia [2,10]. Regarding preoperative fasting requirements, a previous publication suggested that WWS might be associated with reduced gastrointestinal motility and an increased risk for pulmonary aspiration of gastric contents [10].…”

Anaesthesia Management in a Case of Walker Warburg Syndrome-A Case Report

“…English literature search revealed only one case report of anaesthesia in WWS [5]. Here we present a case report 20 days old child with WWS who was brought to the neurocenter of our hospital and was diagnosed to have hydrocephalus which needed ventriculo-peritoneal shunt insertion.…”

Management of general anesthesia in a child with Miller–Dieker syndrome: a case report

“…We avoided the use of succinylcholine, because it can cause hyperkalemia and malignant hyperthermia. Two case reports of patients with Walker–Warburg syndrome, another type of lissencephaly, have been published [ 5 , 6 ]; both described difficulties in airway management in patients with lissencephaly.…”