Thirty-Year Follow-Up of Patient With Gorham Disease (Massive Osteolysis) Treated With Hip Arthroplasty

Gorham disease (also known as massive osteolysis or disappearing bone disease) is an exceedingly rare, peculiar entity of uncertain cause and unpredictable prognosis. There is no proven mode of therapy or consensus on treatment. We present 30-year follow-up on a patient successfully treated with resection, total hip arthroplasty, and radiation with no recurrence of disease. Our observations in this case suggest that surgical treatment and reconstruction can lead to good long-term function and disease-free survival.     

Gorham disease of the lumbar spine with an abdominal aortic aneurysm: a case report

Background contextReports of Gorham disease of the lumbar spine complicated by abdominal aortic aneurysms are rare.PurposeWe herein report the case of a patient with Gorham disease of the lumber spine involving an abdominal aortic aneurysm (AAA).Study designCase report.MethodsA 49-year-old man had a 1-month history of right leg pain and severe low back pain. Plain lumbar radiography revealed an osteolytic lesion in the L4 vertebral body. Computed tomography images demonstrated the presence of an extensive osteolytic lesion in the L4 vertebral body and an AAA in front of the L4 vertebral body.ResultsThe patient underwent mass resection, spinal reconstruction, and blood vessel prosthesis implantation. During surgery, it was found that the wall of the aorta had completely disappeared and was shielded by the tumor mass; therefore, we speculated that the mass in the lumbar spine had directly invaded the aorta.ConclusionsThe patient was able to walk without right leg or low back pain 1 year after undergoing surgery. No recurrence was demonstrated in the magnetic resonance images taken 1 year and 10 months after surgery.     

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.     

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.     

Shy-Drager syndrome A review and a description of the anaesthetic management

Autonomic failure in patients with the Shy-Drager syndrome may produce cardiovascular instability during anaesthesia and surgery. The syndrome is reviewed and the anaesthetic management of a case is described. The choice between general and regional anaesthesia seems to be less important than adequate cardiovascular monitoring and the maintenance of blood pressure with intravenous fluids. Sympathomimetic drugs, if used at all, should be administered in very dilute solutions to avoid hypertension from denervation hypersensitivity. In the postoperative period, symptoms from orthostatic hypotension may be severe and their control requires prolonged postural training, by elevation of the head of the bed, and therapy with 9-alpha-fludrocortisone.     

The anaesthetic management of a patient with familial dysautonomia

Familial dysautonomia is a rare inherited disease of the nervous system, involving mainly peripheral sensory and sympathetic nerves. The autonomic instability is of greatest importance for the anaesthetist although the sensory defect is the most dramatic symptom of the disease. A case report of a 15-year-old girl undergoing two operations for severe scoliosis is presented with a discussion of the anaesthetic problems encountered in this disease.     

Larsen's syndrome: anaesthetic implications. Six case reports

Six cases were examined to review the important anaesthetic implications of Larsen's syndrome. Potential problems arising in these patients are highlighted and emphasis placed on cervical spine and airway/respiratory management.     

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.     

Anasthesia in Marfan''s syndrome

Thirteen patients with Marfan's syndrome who underwent surgery between 1968 and 1983 were studied to document the anaesthetic morbidity in this rare disorder. One of the 13 patients studied died intra-operatively, one died in the immediate postoperative period and one patient developed postoperative complications and further surgery was postponed indefinitely. This represents a high mortality/morbidity rate and the findings are recorded. The two deaths were unexpected and could not be explained.     

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.     

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.     

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.     

The anaesthetic management of Caesarean section for placenta praevia: a questionnaire survey

The purpose of this study was to determine current UK anaesthetic practice regarding the use of regional anaesthesia in the management of patients with placenta praevia presenting for Caesarean section. We asked the members of the Obstetric Anaesthetists Association to complete a postal questionnaire in which a range of clinical situations involving varying degrees of placenta praevia were presented. In each case respondents were asked whether they would be willing to use regional anaesthesia. A wide variety of clinical practice was demonstrated. Anaesthetists with two or more obstetric sessions were more willing to use regional anaesthesia for Caesarean section in the presence of placenta praevia in both elective and emergency situations associated with haemorrhage.