Info Teu
structures by the tumour. The mode of presentation includes a swelling in the neck, middle ear disease, or symptoms indicative of involvement of the cerebellum, brainstem, or skull base. Although normally nonfunctional, it occasionally produces catecholamines. It must be distinguished from glomus tumours, which arise from the tympanic plexus. Surgical removal is either through the auditory canal or via a mastoid approach in the presence of extensive spread, postoperative complications are...
TURP syndrome
A syndrome which may occur during transurethral resection of the prostate, in which large quantities of glycine 1.5 irrigating fluid are absorbed into the circulation through open veins in the prostatic bed, although some is also absorbed more slowly from the retroperitoneal or perivesical spaces. Glycine 1.5 is a nonelectrolytic, slightly hypotonic solution 2.1 would be isotonic , which on absorption is mainly confined to the ECF. Plasma sodium levels are decreased by more than would be caused...
Info Hpw
Dutka DP, Donnelly JE, Nihoyannopoulos P et al 1999 Marked variation in the cardiomyopathy associated with Friedreich's ataxia. Heart 81 141 7. Fantus IG, Seni MH,Andermann E 1993 Evidence for abnormal regulation of insulin receptors in Friedreich's ataxia. Journal of Clinical Endocrinology amp Metabolism 76 60 3. Finley GA, Campbell AM 1992 Spinal anesthesia and Friedreich's ataxia. Anesthesia amp Analgesia 74 318. Kubal K, Pasricha SK, Bhargava M 1991 Spinal anaesthesia in a patient with...
Thyrotoxic crisis or storm see also
The abrupt onset of symptoms of a severe hypermetabolic state, associated with the output of thyroxine, in a patient with pre-existing thyroid disease.This is a clinical, not a bichemical, diagnosis, and biochemically it is difficult to distinguish between the two. However, serum free T4 concentrations are significantly higher in a thyroid crisis compared with thyrotoxicosis. It may occur in a patient with occult thyroid disease, in whom a crisis may be precipitated by an acute medical,...
Laryngeal papillomatosis
Benign, warty tumours of the larynx that occur mainly in children, but sometimes in adults, and are caused by one or more of the human papillomaviruses, the majority type 11, but sometimes type 6.They arise most commonly on the true vocal cords with extension onto the ventricles. Frequently recurrent, they may be present in other parts of the respiratory tract. Extension into the subglottis is common, but involvement of the trachea, proximal bronchus and lung parenchyma is rare. In children,...
Acromegaly
A rare, chronic disease of insidious onset, which usually presents in middle life.An increased secretion of growth hormone GH , associated with high insulin-like growth factor somatomedin, IGF-1 levels, results in an overgrowth of bone, connective tissue, and viscera. It is most commonly caused by an adenoma of the eosinophil cells of the pituitary. Lifespan is considerably shortened, and deaths are primarily from cerebrovascular, respiratory and cardiovascular complications. Death from...
Zenkers diverticulum
A pharyngeal pouch, which is formed by an outpouching of pharyngeal mucosa in the posterior wall of the hypopharynx at its junction with the oesophagus through Killian's dehiscence. At this site there is an area of weakness that lies between the cricopharyngeus and the inferior pharyngeal constrictor muscles.There may be a functional component to the condition, because neuromuscular dysfunction, particularly in the relaxation of cricopharyngeus, has been found. 1. The main complaints are of...
Drowning and near drowning
Drowning is one of the commonest causes of accidental death in young people, but is potentially remediable if appropriate treatment is instituted without delay. Early animal studies led to undue emphasis being placed on the differences between immersion in salt or fresh water, the accompanying osmotic changes, and, in fresh water, the possibility of massive haemolysis. In practice, the inhaled volumes are much less than those induced experimentally, the haemolysis is not significant, and, in...
Autosomal dominant
1. Facioscapulohumeral Landouzy Dejerine, FSHD has onset in adolescence, first affecting the shoulders, facial weakness, winging of scapulae, proximal arm wasting. It is slowly progressive.There is an increased incidence of cardiac arrhythmias suggesting that conduction tissue may be involved. Uneventful anaesthesia using atracurium Dressner et al 1989 , and vecuronium Nitahara et al 1999 , showed no increased sensitivity, but more rapid recovery times than normal. 4. Oculopharyngeal Landrum...
Anaesthesia Management In Patient With Hereditary Spherocytosis
1. Surgery should be avoided in the presence of a haemolytic crisis. 2. Prophylaxis before and after splenectomy. Three vaccines should be given, preferably 2 weeks before splenectomy Pneumovax 0.5 ml sc or im, and repeat once between 5 and 10 years later. HIB vaccine 0.5 ml sc or im once only. Mengivac A C 0.5 ml sc or im, or Meningitec C 0.5 ml im. Repeat every 2 years. Continuous antibiotic prophylaxis benzylpenicillin 600 mg 12 hrly iv or oral penicillin V 250 mg 12 hrly. If penicillin...
Info Gce
phaeochromocytoma 384, 389 Pompe's disease 405, 406 pulmonary hypertension 420 pulmonary oedema 424 6 relapsing polychondritis 439 rheumatoid arthritis 448, 449 sarcoidosis 452, 453 scleroderma 455 scoliosis 458 Sturge Weber syndrome 472, 473 systemic lupus erythematosus 474, 475 Takayasu's arteritis 477, 478 thalassaemia 485 thyrotoxicosis 488, 489-90 tuberous sclerosis 496 Turner's syndrome 497, 498 Wegener's granulomatosis 510, 511 Williams syndrome 513, 514, 515 carnitine palmitoyl...
Osborne Glycopyrrolate Agitation
1. Unexpected delayed recovery from anaesthesia, stupor, restlessness, agitation, amnesia, hallucinations, respiratory depression, confusion, or seizures. In the perioperative period, after premedication with morphine 10 mg and hyoscine 0.4 mg, a young man developed hyperthermia 39.3 C increasing to 42 C , tachycardia, and confusion which resolved immediately after physostigmine 3 mg Torline 1992 .A young woman given glycopyrrolate 0.2 mg became hot and dry and developed headache, tachycardia,...
Management Lrv
1. Dehydration must be avoided, and a good cardiac output and peripheral perfusion maintained. 2. Blood viscosity and platelet adhesiveness can be reduced by dextran, and the prior administration of pyridoxine. 3. Dextrose infusion will prevent hypoglycaemia. 4. Early mobilisation, elastic stockings, and low-dose heparin therapy will help to decrease the chance of postoperative thromboembolism. 5. In the pregnant patient, heparin 10 000 u bd may be needed to reduce thrombotic risk. In the...
Cd Co
6. Insulin resistance. Insulin resistance occurs 3 in a number of circumstances. Normally the u ratio of insulin to glucose required is 15 u insulin to 500 ml 10 dextrose. In patients with severe infection, obesity, liver disease, steroid therapy, or undergoing cardiopulmonary bypass, the dose of insulin may have to be increased by up to four times the normal ratio. 7. Impaired conscious level in diabetics. Causes include hypoglycaemia, diabetic ketoacidosis, hyperglycaemic hyperosmolar...
Bibliography Uro
Anan R, Nakagawa M, Miyata M et al 1995 Cardiac involvement in mitochondrial diseases a study on 17 patients with documented mitochondrial DNA defects. Circulation 91 955-61. Barohn RJ, Clanton T, Sahenk Z et al 1990 Recurrent respiratory insufficiency and depressed ventilatory drive complicating mitochondrial myopathies. Neurology 40 103-6. Blake LL, Shaw RW 1999 Mitochondrial myopathy in a primigravid pregnancy. British Journal of Obstetrics amp Gynaecology 106 871-3. Burns AM, Shelly MP 1989...
Pierre Robin syndrome and the
A rare syndrome in which the combination of severe micrognathia and posterior prolapse of the tongue results in respiratory obstruction in infancy, failure to thrive, and, occasionally, cor pulmonale. Other congenital abnormalities, such as cleft palate and oesophageal atresia, may occur. The 'Robin sequence', a term that is increasingly being used in the specialist surgical literature, describes a spectrum of anomalies, the common features of which include mandibular hypoplasia, glossoptosis...
Tuberous sclerosis
A neurocutaneous disease associated with hamartomas in multiple organ systems, skin lesions, and learning difficulties. Skeletal muscle is not involved. It is an autosomal dominant condition and recent genetic linkage studies have implicated abnormalities of the 9q34 protein encoded by the tuberin and hamartin genes. Both are tumour suppressor genes on chromosomes 9 and 16 O'Callaghan 1999 . For full diagnostic criteria, see Webb and Osbourne 1995 . 1. Cutaneous. Facial and ungual angiofibroma....
Info Bha
and infused according to a sliding scale Blood sugar 3 5 mmol l-1 actrapid Blood sugar 6-9 mmol l-1 actrapid 1.5 ml h-1. Blood sugar 10-12 mmol l-1 actrapid Blood sugar 13-16mmoll-1 actrapid 4 ml h-1. Blood sugar gt 16 mmol l-1 actrapid 6 ml h-1. Glucose 10 500 ml, with or without 10 mmol potassium chloride is infused at 100 ml h-1. Obviously, care must be taken not to stop one without the other being stopped. ii GIK technique Alberti 1991 . Preoperative FBG should be lt 13 mmol l-1....
Sarcoidosis
A multisystem, granulomatous disorder, of variable natural history and prognosis, most frequently presenting in young adults with bilateral hilar lymphadenopathy, pulmonary infiltration, cutaneous and ocular lesions. In some, an acute onset may resolve spontaneously in others, slow onset may herald progressive disease with serious complications, such as pulmonary fibrosis, blindness, cardiac involvement, nephrocalcinosis, and renal failure. Heart and lung involvement are the most significant...
Info Fxw
with the syndrome of haemolysis, elevated liver enzymes and low platelets. Canadian Journal of Anaesthesia 38 227 33. De Boer K, Buller HR,Ten Cate JW et al 1991 Coagulation studies in the syndrome of haemolysis, elevated liver enzymes and low platelets. British Journal of Obstetrics amp Gynaecology 98 42 7. Geary M 1997 The HELLP syndrome. British Journal of Obstetrics amp Gynaecology 104 887-91. Isler CM, Rinehart BK,Terrone DA et al 1999 Maternal mortality associated with HELLP hemolysis,...
Management Lpf
1. In severe hypothyroidism, elective surgery should be postponed whilst treatment is instituted.There is some argument about the management of patients requiring coronary artery bypass surgery. Although suggestions have been made that treatment should not be given before surgery, for fear of precipitating cardiac ischaemia or heart failure Finlayson amp Kaplan 1982, Drucker amp Borrow 1985,Vanderpump et al 1996 , hypothyroidism is not protective in cardiovascular disease and untreated patients...
Mucopolysaccharidoses MPS
A group of inherited connective tissue syndromes that result from enzyme deficiencies. The mucopolysaccharides or glycoaminoglycans are constituents of connective tissue, and are made up of repeating disaccharide units connected to protein.They are normally broken down in the cell lysosomes to monosaccharides and amino acids. In the absence of certain enzymes, accumulation of intermediate products of degradation process takes place.These substances increase cell size and cause impairment of...
Cushings syndrome and Cushings disease
Cushing's syndrome is the general term used for a disorder caused by excess circulating glucocorticoid. Cushing's disease specifies one of its causes that of pituitary-dependent adrenal hyperplasia, secondary to ACTH secretion.This accounts for about 70 80 of cases of Cushing's syndrome. Other important causes are adrenal cortical tumour 5 10 , and ectopic ACTH producing tumour 5 10 .The diagnosis and treatment of Cushing's syndrome, particularly during pregnancy, is more complex than...
Amyloidosis
Amyloidosis is a general term for a variety of different disease processes involving the deposition of fibrillary material in tissues.This is formed of protein subunits sharing a common beta-pleated sheet structure, but derived from proteins of great chemical diversity. These fibrils are resistant to normal proteolytic digestion and share a common histochemical staining property to Congo red. In the light of advances in the understanding of the structural chemistry of amyloid, the disease is...
Info Itu
with severe life-threatening hypoxaemic spells, refractory to other treatment, responded to phenylephrine 5 m g kg-1 plus an infusion of 0.4-2 m gkg-1min-1 . 3. Dehydration in the presence of polycythaemia and high plasma viscosity may combine to increase the incidence of cerebral thrombosis. Polycythaemia may also be associated with coagulation defects. 4. In patients with an absent pulmonary valve, positional airway compromise occurred secondary to bronchial compression of dilated pulmonary...
Management Xat
1. If airway problems exist, assessment of the severity with CXR, CT scan, bronchoscopy, and flow volume loops. 2. Corticosteroids are the mainstay of treatment, particularly when the disease is active. Other treatment includes immunosuppressives and NSAIDs. Plasmapheresis may have a role. 3. Surgery may occasionally be required for respiratory complications, but experience is limited and results uncertain. Posterior membrane fixation of the bronchial tree, internal stenting with a tracheal...
Failure To Control The Airway In A Patient With Hunter S Syndrome
as usual, and that it, like the oropharyngeal airway, can push the epiglottis over the laryngeal inlet causing obstruction.As has already been pointed out, the laryngeal mask airway has limited value if the obstruction lies at glottic level or below. 8. Local anaesthetic techniques should be considered. Spinal anaesthesia has been reported in a patient with a previous failed intubation Sj gren amp Pedersen 1986 . Combined continuous spinal analgesia and general anaesthesia were used for upper...
Management Mgo
1. Assessment of respiratory function, including lung function tests and blood gases, if there is pulmonary involvement.There is a suggestion that bronchoalveolar lavage may help to identify patients with active alveolitis. 2. Adequate venous access may require the use of a central vein or a venous cutdown. Vasoconstriction can be reduced by keeping the theatre temperature high and by warming intravenous fluids. 3. If arterial cannulation is considered,Allen's test should be performed to assess...
Marfan syndrome
An autosomal dominant, inherited condition involving a connective tissue deficit, secondary to mutations within the fibrillin gene on chromosome 15. It affects the microfibrillar component of elastic fibres.The abnormal fibrillin alters the functional relationship between blood flow and vascular endothelial cell response Westaby 1999 .The tensile strength of collagen is reduced, while its elasticity is increased and the wall becomes weakened. Skeletal, cardiovascular and ocular features occur....
Intraoperative precautions
1. Use of latex-free anaesthetic masks, ECG electrodes and wires, blood pressure cuffs, self-inflating bag, elastic bandages, catheters, ileostomy bags, instrument mats, adhesive tapes, dental instruments and material, rectal balloons, airborne latex proteins from glove powder. Powderless gloves should be used and drugs should not be drawn from ampoules with rubber stoppers. 2. Each department should have a database of latex-free equipment Dakin amp Yentis 1998 . Ballantine JC, Brown E 1995...
Coarctation of the aorta adult
A congenital narrowing of the aorta that may be pre- or post-ductal.The preductal form is usually a long, narrow segment, and is associated with other cardiac defects.This type generally presents with heart failure before the age of 1 year and requires treatment in a paediatric cardiac surgical unit. It will not be considered further here.The postductal form, however, is often asymptomatic, and the patient may present in later life for surgery of some other condition, or for correction of the...
Anaesthetic problems Tum
1. Cardiovascular complications. Myocardial dysfunction occurs early in the disease Moran et al 2000 . Patients may require anaesthesia for complications of the disease. Kowaleski et al 1996 described anaesthesia for CABG surgery in a 10 year old with coronary artery aneurysms and stenoses following Kawasaki disease at the age of 1 year. 2. Anaesthesia was required for bleeding after injury to the tongue in a 13 month old, who was anticoagulated to prevent extension of a coronary artery...
Management Bwk
1. Assessment of general condition and nutrition. 2. Prevention of friction and trauma suture cannulae, cover facial lesions with hydrocortisone cream and cotton sponges see also Epidermolysis bullosa . 3. A variety of managements have been described, including diazepam and ketamine Vatashsky amp Aronsen 1982 , epidural anaesthesia and ketamine for cholecystectomy Jeyaram amp Torda 1974 , continuous spinal anaesthesia for hysterectomy Gilsanz et al 1992 , general anaesthesia and tracheal...
Scleroderma
A spectrum of diseases involving abnormal collagen deposition and microvascular changes in the skin and other organs.There is synthesis and deposition of excessive extracellular matrix and the presence of vascular anti-GBM glomerular basement membrane antibodies McHugh 1994 .Autoantibodies define subsets of the disease.These include anti-centromere antibody ACA and anti-topoisomerase-1 antibody ATA . Limited cutaneous scleroderma, previously known as CREST syndrome Calcinosis, Raynaud's, o...
Gauchers disease
An inherited sphingolipid storage disease in which there is a deficiency of a lysosomal enzyme, glucocerebrosidase, resulting in pathological storage of glycolipid in the macrophages of the reticuloendothelial system. There are three types, each with different features type 1 about 99 of cases , type 2 infantile, results in early death , and type 3 juvenile .There is a high incidence in Ashkenazi Jews Zimran et al 1992 . Enzyme therapy decreases the signs and symptoms, but is associated with...
Vipoma
One of the APUDomas Amine Precursor Uptake and Decarboxylation that secretes vasoactive intestinal polypeptide VIP , but may also produce other hormones. Tumours in adults are most commonly of pancreatic endocrine origin, whereas in children they are usually ganglioneuroblastomas arising from the sympathetic chain.Vipomas can also occur as a manifestation of the MEN 1 syndrome Thakker 1998 .VIP is one of a number of hormones that can be secreted by bronchial carcinomas, neuroblastomas, and...
Mitral stenosis
Normal left ventricular filling is restricted by the decreased area across the stenosed valve.The normal area of the valve is 4 cm2. Symptoms appear when this is reduced to about 2.5 cm2 below 1 cm2 the symptoms are severe. Compensation is normally achieved by increasing the pressure gradient across the mitral valve, and is dependent upon atrial contraction and the duration of diastole. Decompensation often begins with the onset of atrial fibrillation, associated with a fast ventricular rate....
Masseter muscle rigidity MMR
Masseter muscle spasm is a clinical diagnosis it has been defined as jaw tightness to a degree that it interferes with tracheal intubation, and occurs despite usually adequate doses of suxamethonium Saddler et al 1990 . Since MMR occurs in 50 of patients who are MHS, it was assumed to be an exclusive sign presaging an MH crisis. However, it is now known that an increase in masseter muscle tone may be a normal response to suxamethonium. In a study of 50 healthy patients, the majority showed...
Management Kng
1. If there is widespread pulmonary involvement and the patient is symptomatic, lung function tests, including blood gases, should be performed. 2. A preoperative ECG is essential, even in young patients. If there is evidence of a conduction defect, a temporary pacemaker should be inserted before anaesthesia. 3. Assessment and management of laryngeal 4. Treatment of hypercalcaemia. o Corticosteroids may improve symptoms and s signs of cardiac and pulmonary sarcoidosis Schaedel et al 1991 . 5....
Management Swr
1. Management of neuromuscular blockade. The method of management depends on the severity of the myasthenia, the type of operation, and the anaesthetist's personal preference.Whilst some surgery can be undertaken using analgesics and inhalational agents, abdominal surgery requires a greater degree of muscle relaxation than can be produced by inhalation agents alone. Reliable neuromuscular monitors and relaxant infusions allow good control to be achieved with minimal doses of a nondepolarising...
Anaesthetic problems Let
1. An increased risk of airway obstruction, so that tracheal intubation or tracheostomy may be required in early life Coniglio et al 1988 . Fifty six percent had choanal atresia or stenosis, 40 micrognathia, 8 laryngomalacia, 6 subglottic stenosis, and 8 had other upper airway abnormalities. 2. Upper airway collapse. Stack amp Wyse 1991 attributed the tendency for the airway to collapse during light anaesthesia to laryngomalacia. However, Roger et al 1999 felt that the inability to maintain the...
Info Kat
injections, and rectal medication Shah et al 1998 . 3. Treatment depends on the severity and type of vWD. In the less severe forms of the disease type I or Ila , clinical and laboratory improvement is associated with both pregnancy and the administration of oestrogens or desmopressin DDAVP .These have the benefits of avoiding the use of blood products. For adenotonsillectomy, in patients shown to respond, desmopressin 0.3 m gkg-1 was given 20-30 min preoperatively Allen et al 1999 . It has been...
Wegeners granulomatosis
A systemic granulomatous vasculitis, usually affecting the age group 20 40 years, in which granulomas of the upper and lower respiratory tract are associated with a focal necrotising glomerulonephritis, and a widespread vasculitis involving other organs.The clinical features overlap with microscopic polyarteritis, and the antineutrophil cytoplasmic antibody is positive in both.A survey of 85 cases showed that all patients had either upper or lower respiratory tract involvement, and 85 had...
Hereditary spherocytosis
A familial haemolytic anaemia of autosomal dominant inheritance in which premature destruction of intrinsically abnormal erythrocytes occurs in the spleen. Specific molecular defects have been found.The clinical severity of the disease varies considerably Eber et al 1990 .Anaesthesia may be required for splenectomy and or cholecystectomy. However, a conservative approach to splenectomy is being adopted avoidance of surgery in those with mild to moderate disease is now recommended. After...
Air embolism
Air embolism can occur in any situation in which there is an open vein and a subatmospheric pressure.When a patient is in an upright position, air inadvertently entering the venous system will normally be carried to the right side of the heart, where it localises initially at the junction of the right atrium and the superior vena cava. Some air may remain in the upper part of the right atrium, while the rest is carried through the tricuspid valve and into the pulmonary artery. There is...
Preoperative abnormalities Hbe
1. Varying degrees of muscle weakness are present, with initial involvement of the thighs and pelvis.The calf muscles are enlarged by fatty tissue pseudohypertrophy and, if the shoulder girdle is affected, there is winging of the scapulae. Relentlessly progressive scoliosis, which may require spinal fusion to improve stability and comfort, usually occurs after this Shapiro et al 1992 .The child is often obese and eventually becomes confined to a wheelchair. 2. Vital capacity decreases...
Info Dxd
accurately determined, but it is easy to visualise how a serious arrhythmia might occur in a myopathic heart with a combination of even mild respiratory obstruction, light anaesthesia, and halothane.That halothane, isoflurane, and sevoflurane given alone are all capable of producing muscle breakdown is demonstrated by the reports of myoglobinuria following anaesthetics in which suxamethonium was not used Rubiano et al 1987, Obata et al 1999 . Avoidance of suxamethonium and volatile agents has...
Info Nrb
iii Fosphenytoin 15 mg kg 1 iv at up to 150 mg min-1. f If fits cannot be controlled, the patient must be transferred to an ITU for more specialist treatment. If still in status give thiopentone atracurium or vecuronium, tracheal intubation, and IPPV g If the patient is hypoglycaemic give 25 ml 50 glucose. If alcohol is likely to have been the precipitating factor, give thiamine 100 mg. 5. Psychogenic seizures can occasionally occur, but there are a number of features that can help to...
Presentation Atu
a After a known dural tap. Cardiovascular collapse usually takes place immediately after the epidural injection, although delays of up to 45 min have been reported Woerth et al 1977 .Three cases of total spinal anaesthesia occurred when epidural injections of local anaesthetic were given into the interspace adjacent to an inadvertent dural perforation Hodgkinson 1981 . In all three incidents, the patients were in active labour. It was suggested that frequent uterine contractions can result in...
Hypercalcaemia
When artefactual causes of an increased serum calcium level have been excluded, the commonest causes of hypercalcaemia are malignancy and hyperparathyroidism. Sarcoidosis, thyrotoxicosis and vitamin D toxicity are uncommon. Other causes are extremely rare. Occasionally a patient with hypercalcaemia may present for anaesthesia. Severe hypercalcaemia gt 3.2 mmol l 1 may be dangerous and, in consultation with a physician, urgent lowering of the level may be required.