Vocal cord paralysis in the Shy-Drager syndrome

A case is presented in which unexpected and persistent postoperative respiratory problems led to the finding of bilateral abductor vocal cord paralysis and confirmed the diagnosis of the Shy-Drager syndrome. Anaesthetists should be aware that vocal cord paralysis may be a feature of this uncommon condition, and should consider the possibility of glottic obstruction as a cause of ventilatory difficulties.     

Moebius syndrome: a review of the anaesthetic implications

Moebius syndrome is a rare congenital anomaly characterized by multiple cranial nerve palsies, orofacial malformations and limb anomalies. This study retrospectively reviewed the anaesthetic records of 19 children with Moebius syndrome who had anaesthesia at the Royal Alexandra Hospital for Children over a 15 year period and analysed the complications which occurred. Affected children most commonly present for anaesthesia for correction of strabismus, or for orthopaedic procedures to improve limb function. Despite the abnormal facies and drooling of saliva, these children are almost always of normal intelligence. Problems can arise during anaesthesia, with a high incidence of difficult or failed intubation. The use of a facemask and spontaneous breathing technique, where appropriate, seems to present no problems and maintaining an airway in this way appears to be safe. However, the potential for problems with aspiration of oral secretions should be remembered and the use of antisialogogue premedication is recommended. Affected children have a high incidence of other anomalies, including congenital cardiac disease, spinal anomalies, corneal abrasions and peripheral neuropathies and a careful preoperative assessment is essential.     

Gorham syndrome: anaesthetic management

Gorham syndrome is a rare chronic disease of children and young adults, featuring massive osteolysis with pathological fractures and complicated by respiratory and neurological deficits. To date, 175 cases have been reported in the literature but information on anaesthetic management is sparse. We present a child with Gorham syndrome who underwent urgent surgical medullary decompression and who subsequently developed bilateral pleural effusions.     

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.     

Bardet-Biedl syndrome: review of anaesthetic problems

Bardet-Biedl syndrome is a rare syndrome characterized by obesity, mental retardation, polydactyly, retinitis pigmentosa and hypogenitalism. These children frequently require multiple anaesthetics for diagnostic and therapeutic measures. The anaesthetic management of a child with the syndrome is described. Eight cases presenting between 1974 and 1990 at the Royal Children's Hospital in Melbourne are reviewed. The anaesthetic problems associated with this syndrome include obesity with consequent difficulty with venous access, placement of local anaesthetic blocks and induction of anaesthesia due to behavioural activity.     

Pre-treatment with somatostatin in the anaesthetic management of a patient with carcinoid syndrome

Carcinoid syndrome produces flushing, bronchoconstriction and gastrointestinal hypermotility secondary to serotonin, histamine, bradykinin and prostaglandin release. A variety of drugs, foods and anaesthetic agents may provoke this syndrome. Under anaesthesia, the flushing produced may be associated with acute hypotension and cardiovascular collapse; this phenomenon is called a carcinoid crisis. Recently, somatostatin analogue has been used successfully to treat intraoperative carcinoid crisis. In this report, we present a 66-year-old lady with carcinoid syndrome who was pre-treated with 50 micrograms somatostatin analogue IV and IM prior to surgical manipulation. The anaesthetic course was relatively uneventful and the patient did well postoperatively.     

Long QT syndrome: anaesthetic management at delivery

We describe the anaesthetic management of a spontaneous vaginal delivery at 39 weeks' gestation in a 22-year-old patient with congenital long QT syndrome. With a strong family history of sudden deaths, the patient had an initial QT interval corrected for rate (QTc) of >600 ms. Following a once-daily 50-mg dose of atenolol over the previous 11 months, her QTc remained prolonged at 560 ms. To minimise any increase in catecholamine levels and consequent risk of malignant ventricular arrhythmias, a combined spinal-epidural technique was selected using intrathecal diamorphine and levobupivacaine, with intravenous and oral magnesium and potassium supplementation. Levobupivacaine was substituted for routine racemic bupivacaine to decrease the risk of drug-induced cardiotoxicity. Delivery outcome was successful and uneventful. We outline the pathophysiology, risks and treatments of long QT syndrome, and discuss the analgesic management of this patient in labour with congenital long QT syndrome.     

Esmolol in the anaesthetic management of a boy with Romano–Ward syndrome

The Romano–Ward syndrome is a congenital form of long Q-T syndrome which frequently causes syncope, dysrhythmias and sudden death. Certain anaesthetic drugs can precipitate dangerous dysrhythmias. Chronic β-adrenergic blockade is the therapy of choice for symptomatic patients and establishment of complete and total β-blockade is recommended for symptomatic patients having anaesthesia and surgery. We report the successful use of esmolol to establish rapid and total β-blockade before a short elective out-patient procedure and suggest that esmolol may be useful for other patients with Romano–Ward syndrome having anaesthesia and surgery.     

The anaesthetic management of a case of Kawasaki's disease (mucocutaneous lymph node syndrome) and Beckwith-Weidemann syndrome presenting with a bleeding tongue

An unusual case of a 13-month-old child with Kawasaki's disease and the Beckwith-Weidemann syndrome is presented. The child, while anticoagulated with warfarin and aspirin to prevent extension of a coronary artery thrombus, fell and lacerated the tongue resulting in haemorrhage and significant swelling. The ongoing haemorrhage, combined with difficulty in securing venous access resulted in the child becoming shocked. Surgical intervention was required to stem the haemorrhage. The anaesthetic management of a shocked child with a coronary artery aneurysm and thrombosis, a potentially difficult airway and a full stomach is described.     

A systematic review of the aetiology and management of post cholecystectomy syndrome

Background

10% of patients who undergo a cholecystectomy go on to develop post-cholecystectomy syndrome (PCS). The majority of these patients may suffer from extra-biliary or unrelated organic disorders that may have been present before cholecystectomy. The numerous aetiological causes of PCS result in a wide spectrum of management options, each with varying success in abating symptoms. This systematic review aims to provide a summary of the causative aetiologies of post cholecystectomy syndrome, their incidences and efficacy of available management options.

Larsen syndrome and its anaesthetic considerations

Larsen syndrome is a complex syndrome with genetic heterogeneity, and with both autosomal dominant and autosomal recessive patterns of inheritance. It is characterized by congenital dislocation of joints, flat faces and complicated by issues relating to respiratory, cardiac, musculoskeletal and central nervous systems. This report describes the anaesthetic management of two patients with Larsen syndrome. The first case is a 4-year-old patient who had cervical cord compression secondary to cervical instability and who was scheduled for anterior corpectomy with fusion of cervical vertebrae and placement of halo frame. This patient had transient loss of evoked potentials during positioning and a stormy postoperative course requiring reintubation and a prolonged stay in the intensive care unit. The second case is a 22-month-old child who was scheduled for a repeat posterior cervical spinal fusion due to failure of her initial fusion procedure. This patient had an uneventful perioperative course. Relevant anaesthetic issues in patients with Larsen syndrome are discussed.     

Morquio's syndrome and its anaesthetic considerations

Morquio's syndrome is an inherited disorder characterized by excessive excretion of keratan sulphate in the urine. The anaesthetic care of these patients should take into consideration respiratory, craniofacial, cardiac, skeletal, ocular and hepatic abnormalities. We report the case of a child with Morquio's syndrome who presented for stabilization of the cervical spine, and discuss the issues relevant to the anaesthesiologist.     

Subclavian vein obstruction and thoracic outlet syndrome: A review of etiology and management

This is a comprehensive review of acute and chronic venous obstruction, both thrombotic and nonthrombotic. It covers the anatomy, etiology, clinical features, management, and results of treatment of this uncommon condition. The typical clinical symptoms are pain and swelling in the arm, aggravated by exercise. Venography is the essential diagnostic test. The treatment options for acute thrombosis and their results include: anticoagulants, 49% improvement in 185 cases; fibrinolysis, 74% improvement in 62 cases, 36 of whom later had first rib resection and three of whom had angioplasty; and thrombectomy, 94% improvement in 33 cases. For chronic obstruction, treatment alternatives are first rib resection, 77% improvement in 97 patients; claviculectomy, 83% improvement in six patients; scalenectomy and soft tissue division, 83% improvement in 23 cases; endovenectomy, 90% improvement in 10 cases; and venous bypass, 67% improvement in 15 cases. The definitions of improvement are variable and sometimes ambiguous. The data do not provide a rigid guideline for specific management of each condition; rather, the options are presented with their supporting data to permit an individualized approach.     

Moyamoya disease - a review of clinical experience and anaesthetic management

Moyamoya disease is a rare neurovascular condition that affects both children and adults. Increasingly these patients present for surgical management to improve the cerebral circulation since medical therapy is essentially ineffective. Because of the precarious cerebral circulation, these patients represent an anaesthetic challenge. In this report we review the pathophysiology of moyamoya disease, summarize our experience with seven patients and discuss the anaesthetic management.