Anaesthesia for open-heart surgery in a patient with Weaver's syndrome

Abstract

EDITOR: We describe the management of a child with Weaver's syndrome who presented for operation with an atrial septal defect (ASD). Of prime concern for the anaesthetist is the possibility of a difficult intubation due to the skeletal abnormalities of the face, which includes micrognatia. Weaver's syndrome was first described in 1974 [1]. It is characterized by excessive growth of prenatal onset and accelerated skeletal maturation during infancy. The children are usually large at birth. They have an unusual facies with a large bifrontal diameter, a flat occiput, large ears, ocular hypertelorism and relative micrognathia. The skeletal anomalies also include prominent finger pads, camptodactyly, broad thumbs, clinodactyly of the toes, and limited elbow and knee extension, which improves with age. Other common features are thin hair, inguinal or umbilical hernia, and a hoarse low-pitched cry. The aetiology is unknown. No definite endocrine or metabolic aberration has been found. Mildly altered concentrations of serum amino acids have been reported, but it may be a nonspecific finding related to the excessive growth. Similar changes have been reported in children with cerebral gigantism [1]. Our patient was a 1-yr-old female who had been previously diagnosed with Weaver's syndrome. Her body weight was 23 kg (the median weight for normal children is 9.8 kg). She was 93 cm tall (the median height for normal children is 74.2 cm). Cardiac catheterization had revealed an ASD and a patent ductus arteriosus (PDA). The child also had an umbilical hernia. Electrcardiographic (ECG) findings were consistent with right axis deviation, hypertrophy of the right ventricle and dilation of the right atrium. She was in sinus rhythm. Routine preoperative blood chemistry was within normal ranges, except for a mild anaemia (haemoglobin 9.7 g dL−1, haematocrit 31%). Serum aminoacid studies showed normal values for the branched chain aminoacids. Urinary aminoacid and mucopolysaccharide concentrations were normal. Anaesthesia was induced with etomidate (0.3 mg kg−1) and atracurium (0.5 mg kg−1) for muscle paralysis, i.v. Endotracheal intubation was abnormally difficult: the child had a short neck. Her jaw was small with a receding chin and a relatively underdeveloped mandible. During direct laryngoscopy, the anterior commissure of the larynx could not be seen (Mallampati grade 2 [2]). A guide was used for intubation and a 6 mm (26-French) plain endotracheal tube was passed at the first attempt. The normal estimated tube size for a 1-yr-old child is 4 mm (18-French) [3]. Maintenance of anaesthesia was provided with isoflurane (1 MAC) and N2O (50%) in O2. Vital signs were stable and within normal limits throughout the course of the operation. The duration of cardiopulmonary bypass was 30 min and the aorta was cross-clamped for 18 min. The child was extubated in the intensive care unit 4 h following surgery. On the second postoperative day, she was transferred to the general ward, and the rest of the postoperative course was uneventful. The patient was discharged from the hospital in good condition 10 days following surgery. Surgical correction of congenital cardiac defects has not been reported in Weaver's syndrome previously [1,4-7]. In our patient, the indication for surgery was the closure of an ASD and a PDA. There are two previous reports documenting surgery in three patients and in only one of them is the anaesthetic technique described [4,5]. Difficult tracheal intubation has been noted indicating the need for preoperative precautions as availability of fibreoptic bronchoscopy and emergency tracheostomy facility [7]. Alternative methods to facilitate tracheal intubation in difficult patients include an awake intubation, a blind nasal intubation and fibreoptic guidance. Apart from the potential difficulty with endotracheal intubation, no particular anaesthetic considerations have to be taken. B. Celebioǧlu F. Yener Ankara, Turkey