Anaesthesia in the prune-belly syndrome

Between 1959 and 1984, 36 children with the prune-belly syndrome underwent 133 operations at the Hospital for Sick Children, Great Ormond Street. The anaesthetic and surgical problems have been reviewed. Minor postoperative respiratory tract infections followed eight anaesthetics. Three deaths occurred in the postoperative period. In two of these there were multifactorial causes, not directly related to the prune-belly syndrome. One death was related to the sequelae of the syndrome. Normal doses of muscle relaxants are recommended when intermittent positive pressure ventilation is used during anaesthesia. Monitoring of the patient's respiratory state and active physiotherapy are advisable postoperatively. Analgesics should be used with caution.     

Anaesthesia in a patient with Noonan''s syndrome and cardiomyopathy

A 5-year-old girl with Noonan's syndrome and a hypertrophic cardiomyopathy presented for myelography and subsequently, laminectomy. The anaesthetic management of the patient is described and the potential problems of the facial, cardiovascular and skeletal abnormalities associated with the condition are discussed.     

Anaesthesia in a child with Patau's syndrome

A 5-year-old girl who had Patau's syndrome associated with double outlet right ventricle and pulmonary stenosis required general anaesthesia for the formation of a modified Blalock-Taussig shunt.     

Anaesthesia for cardiac surgery in children with nemaline myopathy

Nemaline myopathy is a rare congenital myopathy associated with skeletal deformities and respiratory complications. Three children with nemaline myopathy who underwent cardiac surgery are described where the heart rate decreased during induction of anaesthesia and body temperature increased during or after the surgery. The anaesthetic implications in the management of patients with nemaline myopathy are discussed.     

Acute superior vena caval syndrome with airway obstruction following elective mediastinoscopy

A 47-year-old female patient had a subclinical superior vena caval syndrome which developed into the 'full blown' acute condition when she was placed into the left lateral position after mediastinoscopy. She developed airway obstruction requiring urgent re-intubation and subsequent admission to the intensive care unit. This subclinical condition might have been suspected pre-operatively if closer attention had been paid to the history, physical examination and review of the computerised axial tomography scan: she had a history of intermittent dyspnoea, wheeze and cough which was worse on waking and improved as the day progressed, she had a positive Pemberton's sign and the computerised axial tomography scan showed that the lesion was encroaching on the superior vena cava.     

The laryngeal mask airway in children

The laryngeal mask airway was used in 200 children during a variety of surgical procedures. Some problem with the use of the device was encountered in 47 cases (23%), but in only five cases (2.5%) were the problems serious enough to warrant abandonment of its use. A clear airway was ultimately achieved in 191 children. Downfolding of the epiglottis over the laryngeal inlet was identified in eight out of 24 patients where flexible laryngoscopy was performed, clinically all these had unobstructed airways. The mask was used in 16 children with known airway problems. It is concluded that the size 2 laryngeal mask airway can be successfully used within the weight range 6-30 kg.     

Anaesthesia for Proteus syndrome

A 14-year-old boy with Proteus syndrome presented for orthopaedic surgery to his legs. No report in the literature exists on anaesthesia for this condition. Our patients posed airway problems that were managed by tracheal intubation under sedation using a fibreoptic bronchoscope. Anaesthesia was induced with thiopentone and maintained with nitrous oxide and isoflurane in oxygen. His lungs were ventilated mechanically throughout surgery, which was uneventful.     

Anaesthesia for Shy—Drager syndrome

Anaesthesia for nephrectomy in a patient with Shy-Drager syndrome is described. The potential problems and pre-, per- and postoperative management are discussed with particular reference to the monitoring of cardiovascular function by pulmonary artery catheterisation.     

The laryngeal mask airway in children

Clinical and fibreoptic assessment of the positioning of the laryngeal mask airway was performed in 100 children. Clinical observation indicated a patent airway in 98% and severe airway obstruction in 2% of cases. Perfect positioning, as judged by fibreoptic laryngoscopy, was found in 49% and the epiglottis was within the mask in 49%. Fibreoptic evidence of partial airway obstruction in 17% was not detected clinically.     

Anaesthesia in an infant with Joubert''s syndrome

The presentation and management of a child with Joubert's syndrome is outlined with specific reference to the problems of opioid sensitivity and abnormal control of respiration.     

The laryngeal mask airway in paediatric anaesthesia

Forty-eight children, aged between 2 and 10 years, admitted as day cases for otological surgery were allocated at random into two groups. The first group was anaesthetised using a standard facemask, and the second with a laryngeal mask airway. The laryngeal airway produced a satisfactory airway in all children, and was inserted on the first attempt in 67% of patients. Hypoxia was significantly less frequent in the laryngeal airway group (p less than 0.05), and there were significantly fewer interruptions to surgery than in the facemask group (p less than 0.001). Patient safety, operating and anaesthetic conditions were all considered superior in the laryngeal airway group.     

A comparison of deep vs. awake removal of the laryngeal mask airway in paediatric dental daycase surgery. A randomised controlled trial

Dental anaesthesia provides a potential conflict between anaesthetist and surgeon because of the shared airway. The laryngeal mask airway (LMA) has helped to improve airway control for these procedures, but there is little evidence for best practice on the timing of their removal after airway surgery in the paediatric population. We compared 'awake' and 'deep' removal of the LMA in 196 patients aged from 2 to 15 years in a randomised, controlled study. We found that average peripheral oxygen saturation (SpO2) was lower in the deep group and this was statistically significant (96.2% vs. 94.9%, p = 0.04). It was also found that the deep group had a higher incidence of patients with SpO2 < 95% (p = 0.003) and of patients who coughed (p = 0.003). We conclude that the LMA should be taken out awake in these patients.