Delivery of anesthesia and complications for children with Fraser syndrome: a review of 125 anesthetics.

OBJECTIVES

To perform a retrospective, anesthesia case note review of patients with Fraser syndrome.

AIM

To identify the perioperative and postoperative anesthetic management and complications in this patient group.

BACKGROUND

Fraser syndrome is a rare, autosomal recessively inherited disorder characterized by cryptophthalmos, cutaneous syndactyly, and ambiguous genitalia. It also has variable association with cardiac, laryngeal, tracheal, and gastrointestinal abnormalities. Children with Fraser syndrome present for a variety of surgical and radiological procedures, and there are a number of single case reports in the literature.

METHODS

A retrospective case note review was undertaken on 10 children with Fraser syndrome who presented to our institution over a 30-year period. Analysis of the medical notes and general anesthetic records was undertaken, and the conduct of anesthesia, spectrum of disease, genetic markers, and perioperative complications were recorded. There were a total of 125 procedures performed under general anesthesia during this period.

RESULTS

There were a total of ten anesthetic complications in the review, all related to management of the airway. There were two accidental extubations, five recorded incidents of airway obstruction (which were relieved with continuous positive airway pressure (CPAP)) and one posttracheostomy airway bleed. One child was a difficult intubation requiring an ID 2.5 mm oral endotracheal tube (ETT), and one child was an impossible intubation that required an emergency tracheostomy to secure the airway.

CONCLUSIONS

There was a low incidence of complications with this group of patients. However, there is a relatively high incidence of difficult or impossible tracheal intubation (20%) due to glottic stenosis and one patient required an emergency tracheostomy despite no previous clinical evidence of airway narrowing.