It was concluded that opiate and alcohol induced coma had resulted in ischaemic rhabdomyolysis in the left deltoid and shoulder girdle muscles, although either ...
Journal of the Royal Society of Medicine Volume 86 February 1993 Within minutes of arrival the patient had an asystolic cardiac arrest from which he was resuscitated using adrenaline, atropine, bicarbonate and naloxone. Urgent investigations revealed plasma sodium 140 mmol/l, potassium 8.1 mmol/l, creatinine 292 ;mom/l. Catheterization produced 20 ml of dark brown urine, osmolality 328 mOsmn kgH20. On recovery repeat potassium was 5.7 mmol/1. One hour later he had a second asystolic cardiac arrest, electrolytes were sodium 137 mmol/l, potassium 9.0 mmol/l, urea 12.5 mmol/l, phosphate 2.4 mmol/l, calcium 1.99 mmol/l and subsequently he required haemofiltration to control hyperkalaemia. Urine drug screen detected morphine. Both plasma and urine were dark brown in colour, compatible with rhabdomyolysis, and plasma AST was 1280 iu/l. The haemoglobin concentration was stable, and urine was positive for peroxidase activity, indicating myoglobinuria. Renal function deteriorated, requiring dialysis, and during recovery he became transiently hypercalaemic (4.01 mmol/l). It was concluded that opiate and alcohol induced coma had resulted in ischaemic rhabdomyolysis in the left deltoid and shoulder girdle muscles, although either a contaminant or direct opiate toxicity may have contributed14. Discussion Rhabdomyolysis, myoglobinuria and acute renal failure have been widely reported following opiatel'2, cocaine5, methadone3 and combined alcohol and opiate4 abuse. The classical biochemical picture of hyperkalaemic, hypocalcaemic renal failure with hypercalcaemia during recovery was reproduced in this case4. However, before the clinical syndrome was suspected the patient twice developed hyperkalaemic asystole.In any person where alcohol or drug abuse is suspected, rapid analysis of plasma potassium may prevent, or facilitate prompt treatment of, cardiac arrest. A high index of suspicion should be maintained because apart from the usual clues of
Hypertrophic obstructive cardiomyopathy with LEOPARD (Moynihan's) syndrome: surgical treatment
S Kolvekar MS MCh B T Williams FRCS G E Venn MS FRCS Department of Cardiothoracic Surgery, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH Keywords: hyperobstructure cardiomyopathy; surgery
Hypertrophic obstructive cardiomyopathy (HOCM) may present as a discrete entity or as part of a disease complex. The natural history of HOCM is poor with 25% of patients dying within 10 years of diagnosis in the absence ofsurgical treatment. The surgical approach to the condition has varied. over the years with a number of surgical -techniques used with the intent of improving prognosis. These procedures have included mitral valve surgery, trans aortic myotomy/ myectomy and trans ventricular outflow tract myectomy. Latterly the technique of trans aortic-myectomy, performed with the aid of a diathermy loop has been described1. Correspondence to: Mr Graham E Venn, Department of Cardiothoracic Surgery, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH
drug misuse (such as venepuncture marks and pupillary constriction), there may only be local signs of rhabdomyolysis such as a red, tender, swollen limb4'5, red skin alone6 or anaesthetic, pulseless limb'. Furthermore it should be remembered that the complete syndrome has been repeatedly reported in the absence of coma3 and with no physical signs of muscle necrosis5. There has been much speculation about the cause of hyperkalaemia; probably a combination of release from damaged myocytes, acute renal failure (mainly secondary to ferrihaemate poisoning of renal tubular cells7) and consequent acidosis. None the less, though some 50 years have passed since Bywaters8 described the sequelae of the crush syndrome, we would like to emphasize that unless hyperkalaemia is anticipated and corrected, the patient may die long before the other more widely reported manifestations of this syndrome become apparent. References 1 Trewby PN, Kalfayan PV, Elkeles RS. Heroin and hyperkalaemia. Lancet 1981;i:327 2 Gibb WR, Shaw IO. Myoglobinuria due to heroin abuse. J R Soc Med 1985;78:862-3 3 Nanji AA, Filipenko JD. Rhabdomyolysis and acute myoglobinuric renal failure associated with methadone intoxication. J Toxicol Clin Toxicol 1983;20:353-60 4 Nicholls K, Niall JF, Moran JE. Rhabdomyolysis and renal failure. Complications of narcotic abuse. Med J Aust 1982;2:387-9 5 Herzlich BC, Arsurs EL, Pagala M, Grob D. Rhabdomyolysis related to cocaine abuse. Ann Intern Med 1988;4:335-6 6 Thjs LG, Balk E., Bronsveld W, Thijs JC. Hyperkalaemia in drug addicts. Lancet 1980Xi:1245 7 Chaikin HL. Rhabdomyolysis secondary to drug overdose and prolonged coma. South Med J 1980;73:990-4 8 Bywaters EGL. Fifty years on: the crush syndrome. BMJ 1990;301:1412-15
(Accepted 22 October 1991)
Case report A 58-year-old woman was admitted with a history of frequent syncopal attacks over the preceding 6 months, angina and palpitations with effort intolerance (New York Heart Association Class 2). She had a positive family history with her mother having died of abrupt cardiac syncope. Clinical examination revealed hypertelorism and lentiginosis compatible with a diagnosis of LEOPARD (Moynihan's) syndrome. She had an ejection systolic murmur in the aortic area. Echocardiogram showed asymmetrical septal hypertrophy with systolic anterior motion of the mitral valve and left ventircular outflow tract obstruction (LVOTO) with a peak gradient of 80 mmHg. Her left ventricular function was otherwise satisfactory. The right ventricle was small and displaced by a very large interventricular septum. Cardiac catheterization confirmed these findings. Cardiopulmonary bypass was established using aortic and bicaval cannulae. At operation there was typical cardiomegaly. The left ventricle was vented through its apex and the heart arrested by the infusion of cold crystalloid cardioplegic. An oblique aortotomy was performed. A large sub-valvular bulge in the interventricular septum occluded the outflow tract anteriorly. The right coronary cusp was retracted forward, the mitral valve apparatus protected with a wooden spatula and obstructing components of the interventricular septum resected using a diathermy loop. Clearance of obstruction was satisfactory. Following weaning from bypass the outflow tract gradient measured 5 mmHg. This simple technique allows for good relief of left ventricular outflow tract obstruction with minimal patient morbidity. The postoperative recovery was uneventful and subsequent echocardiography showed no gradient across the
Case presented to Clinical Section, 8 March 1992
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left ventricular outflow tract. The patient has been reviewed postoperatively and is presently asymptomatic. Discussion Hypertrophic obstructive cardiomyopathy is a genetically determined disorder of cardiac muscle transmitted as an autosomal dominant trait, although non-familial cases probably also occur. Lentiginosis represents hyperactivity of melanocyte activity which presents as multiple pigmented spots. Gorlin2 described a multiple lentiginosis syndrome and named it as LEoPARD syndrome; L - Lentiginosis, E - Electrocardiographic conduction defect, 0 - Ocular telorism, P Pulmonary stenosis, A - Abnormal genitalia, R - Retardation of growth, D - Deafness. Moynihan3 described the lentiginosis with hypertrophic obstructive cardiomyopathy which may be associated with one or more abnormalities described in LEOPARD syndrome. There are presently two modes of surgical treatment; to resect the part of inter-ventricular septum (via the
aorta or left ventricle) or replace the mitral valve as anterior mitral leaflet obstructs the LVOT in systole. Williams' described the use of diathermy loop for septum resection. This has proven to be a simple, effective and safe method. In summary, this report dments the s sul treatment of an unusual form of hypertrophic obstructive cardiomyopathy associated with lentiginosis. References 1 Williams BT, Armstead SH. Hypertrophic obstructive cardiomyopathy; The use of a diathermy loop for septal resection. J Cardiovasc Surg 1984;25:185-6 2 Gorlin RJ, Anderson RC, Blaw M. Multiple lentiginosis syndrome. Am J Dis Child 1969;117:652 3 Moynihan EJ, Polani PE. Progressive cardiomyopathic lentiginosis. Q J Med 1972;162:205 (Accepted 1 July 1992)
Metastatic Ewing's sarcoma of the kidney
W J Lynch MB BS FRACS' P 0 Maher FRACS ERCS2 'Department of Urology, The London Hospital Whitechapel El lBB and 2Department of Urology, Repatriation General Hospital Concord, Australia 2139 Keywords: Ewing's sarcoma; metastases; renal neoplasm
We present a case of metastatic Ewing's sarcoma of the kidney. We believe that no previous case has been reported. It is also quite unusual in the lateness of metastasis, being 9 years after the apparent successful treatment of the original tumour. Case report A 24-year-old woman developed right sided abdominal pain 2 weeks before the delivery of her first child. She was seen 8 weeks later because she noticed a mass in the right upper quadrant, which seemed to be increasing in size. It was easily ballotable and seemed to be attached to the kidney. Ultrasound and CT scan showed a complicated cyst, probably of the kidney. Nine years earlier, a Ewing's sarcoma of the distal left femur was treated with local radiotherapy and adjuvant chemotherapy - the VAC regimen (vincristine, actinomycin D and cyclophosphamide) was used (Figure 1). At surgery a huge cystic mass was found arising from the upper half of the right kidney. Its fluid was blood-stained, and, on opening the cyst, solid debris was noted on its wall. Frozen section showed a malignancy was present and right nephrectomy was performed. Histopathology confirmed that the renal ma8s was a metastasis from the original Ewing's sarcoma (Figure 2). Lung and bone scans showed no other secondary deposits. It was felt that further treatment should be given in the form of six courses of chemotherapy similar to the first regimen, followed by radiotherapy to the renal bed. The patient is well, with no evidence of further tumour 12 months after completing her chemotherapy. Correspondence to: Dr W J Lynch, 105 Burraneer Bay Road, Cronulla, NSW, Australia 2230
Figure 1. Sections show a small cell neoplasm. Nuclei are uniform with dispersed chromatin and very rare mitoses. PAS positive granules are present in the cytoplasm
Figure 2. Sections show small round cell tumour. Confirmed as Ewing's sarcoma by positive NSE, equivocal S100 and negative keratin and LCA Electron microscopy findings supported light microscopy diagnosis of Ewing's
