Anaesthesia for Rett syndrome.

Rett syndrome is a devastatingly disabling neurological disease that is only observed in girls. Scoliosis occurs in roughly half the girls and surgery may be required. Anaesthesia is described in three patients. Sudden death may be a feature of the disease which occurred four weeks postoperatively in one case. Although a long QTc interval may be seen, it did not occur in any of our cases.

An unusual case of obstructive pulmonary oedema.

A four-year-old boy was admitted to intensive care via casualty having suffered an apnoeic episode due to compression of his chest by a heavy cattle bar. A petechial rash was noted on the upper chest and arms. A chest radiograph showed pulmonary oedema which resolved over a few hours following positive pressure ventilation and positive end expiratory pressure. We believe this to be an unusual presentation of obstructive pulmonary oedemia (Negative Pressure Pulmonary Oedema).

Anaesthesia for Cockayne syndrome. Three case reports.

Cockayne syndrome is a rare autosomal recessive condition producing a dwarfed, mentally retarded infant or child. Problems with airway management and an increased risk of gastric aspiration are the main anaesthetic concerns. Anaesthetics given to three patients with Cockayne syndrome are described. In two of these, tracheal intubation was difficult and the use of a laryngeal mask airway proved invaluable.

Myotonic and neuromuscular blocking effects of increased doses of suxamethonium in infants and children.

The myotonic effects and duration of action of several doses of suxamethonium were determined in 24 infants and 16 children during thiopentone-fentanyl-nitrous oxide anaesthesia. Infants received suxamethonium 2, 3 or 4 mg kg-1; children received 1 or 2 mg kg-1. The increase in muscle tone during onset of neuromuscular block was independent of dose. Onset of block was faster in children who received suxamethonium 2 mg kg-1 compared with those who received 1 mg kg-1, and in infants given 2 mg kg-1 than in children given the same dose. Compared with adults given suxamethonium 1 mg kg-1, infants required 3-4 mg kg-1 and children at least 2 mg kg-1 to produce 6-8 min of neuromuscular block. These results provide a clear indication for increasing the intubating doses of suxamethonium in infants and children, and a explanation for the unduly high rate of "masseter spasm" in some paediatric centres.

Anaesthesia and Prader-Willi syndrome.

A review of a case series of sixteen anaesthetics in eight cases was undertaken to determine whether children with Prader-Willi syndrome present particular problems to the anaesthetist. Children in an early stage of the condition who are below their centile for weight present no specific problems. Children who are heavier than 97th centile weight have problems associated with their obesity: difficult intravenous access and sleep apnoea. Scoliosis was noted in both groups and was not associated with problems after minor surgery.

The caudal canal in children: a study using magnetic resonance imaging.

The anatomy of the caudal (sacral extradural) space was studied in 41 children, using magnetic resonance imaging. The distance from the upper margin of the sacrococcygeal membrane to the dural sac, the length of the membrane and the maximum depth of the caudal space were each measured. Age, height, weight and body surface area were recorded and, using multiple linear regression (stepwise technique), equations predicting the length of the membrane and the distance between its upper margin and the lower limit of the dural sac were obtained. Wide variability limits the clinical usefulness of these equations. In all patients, the maximum depth of the caudal space was found to be at the upper margin of the sacrococcygeal membrane. No correlation was found between this maximum depth and the age, height, weight or body surface area of the child.

Postintubation tracheal stenosis in an 11-year-old boy: a surgical and anaesthetic challenge.

We present a case of postintubation tracheal stenosis in an 11-year-old boy occurring after a relatively short period of intubation. He had been intubated and ventilated in a paediatric intensive care unit after a road traffic accident. Clinical symptoms manifested by oxygen desaturation and wheeziness, finally leading to deterioration of the level of consciousness, occurred a few hours after the first attempt at extubation after 48 h requiring reintubation. Endoscopic examination performed a few weeks later revealed a tracheal stenosis. Consequently, he underwent an initial period of conservative treatment consisting of balloon dilatation and intralesional injection of steroids, followed by a tracheal resection and reconstruction. The anaesthetic management of patients with tracheal stenosis presenting for laryngo-tracheobronchoscopy and balloon dilatation is discussed.

A bronchogenic cyst in an infant causing tracheal occlusion and cardiac arrest.

A 3-month-old infant treated for 3 weeks for suspected bronchiolitis, developed episodes of profound desaturation. A lateral X-ray showed displacement and compression of the trachea. Respiratory arrest, from which she was successfully resuscitated, occurred just before MRI scan. The mass was removed at thoracotomy and a histological diagnosis of a bronchogenic cyst was made. Mediastinal masses in babies are relatively rare, and the situation in which they present with acute respiratory distress may prove extremely challenging to the anaesthetist. Bronchogenic cysts are difficult to diagnose pre-operatively and awareness may assist in the peri-operative management of these infants.