Nov 11, 1988 - submucosal lipoma of the large bowel. Br J Surg 1987; ... Submucous lipoma of ... artery, proximal stenoses in the superior mesenteric artery,.
Journal of the Royal Society of Medicine Volume 82 October 1989 7 Creasy TS, Baker AR, Talbot IC, Veitch PS. Symptomatic submucosal lipoma of the large bowel. Br J Surg 1987; 74:984-6 8 Ginzburg L, Weingarten M, Fischer MG. Submucous lipoma of the colon. Ann Surg 1958;148:767-72 9 Hurwitz MM, Redleaf PD, Williams HJ, Edwards JE. Lipoma of the gastrointestinal tract. Am J Roentgerol 1967;99:84-9
Rapidly progressive aortic incompetence and coronary artery disease in a patient with Takayasu's disease
K Wong MB BS G E Venn MB BS FRCS M J Hershman MB BS FRCS R N Sapsford ChM FRCS Department of Cardiothoracic Surgery, Hammersmith Hospital, London Keywords: Takayasu's disease; arteritis; aortic incompetence; coronary artery disease
A patient with aortic incompetence and coronary artery disease in Takayasu's disease is described. Although, the underlying aetiology is an arteritis we recommend early surgery for rapidly developing symptoms.
Case report A 33-year-old Caucasian woman first presented with weight loss, malaise and pyrexia in 1982. In 1985, she developed headaches and photophobia. Temporal artery biopsy was normal. These symptoms improved following an empirical
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10 Bar-Meir S, Halla A, Baratz M. Endoscopic removal of colonic lipoma. Endoscopy 1981;13:135-6
(Accepted 26 April 1989. Correspondence to WSL Stebbings, Professorial Surgical Unit, St Bartholomew's Hospital, West Smithfield, London EClA 7BE)
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Figure 2. Selective right coronary arteriogram showing a tight ostial stenosw with normal distal vessel
course of steroids. One year later, she developed exertional dyspnoea, chest pain and palpitations which precipitated her admission.
On examination, she had signs of aortic incompetence which had not been previously documented. Her erythrocyte sedimentation rate and C-reactive protein were elevated. She had a normochromic, normocytic anaemia, normal biochemistry and negative syphilitic serology. Cardiac catheterization revealed impaired left ventricular function, a dilated ascending aorta with moderately severe aortic incompetence (Figure 1) and a tight right coronary ostial stenosis but with normal distal vessel (Figure 2). In October 1987, she developed hypertension and widespread arterial bruits with unequal blood pressures in her upper limbs. Visceral angiography revealed an occluded coeliac artery, proximal stenoses in the superior mesenteric artery, left internal carotid artery-and both renal arteries. On the basis of these findings -a diagnosis of Takayasu's disease was made. Over the ensuing months, her dyspnoea rapidly worsened. This was, initially, felt to represent impairment of her left ventricular function scndary to the disease process. Her symptoms, howevet, did not improve -on increased immunosuppression. It was, -then, appreciated that her deterioration represented a rapid progresion of her valvular disease. She was admitied for surgery in August 1988. Median sternotomy revealed a grossly thickened aorta. The aortic ring was dilated but the cusps were macrofcopically normal. The valve was replaced with a Starr-Edwards size 10 prosthesis and coronary artery bypass graft to the right coronary artery was performed. Postoperatively, she made an uneventful recovery and remains haemodynamically well three months later.
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Discussion
friable but, it is in fact tough and thickened due to the
Takayasu's arteritis is a rare, chronic arteritis affecting the aorta and/or its main branches and is often called the pulseless disease. It is of unknown aetiology and affects predominantly women between 20 and 35 years of age1. Its highest incidence is in Japan although it has been reported worldwide. The pathological process is that of a panarteritis2. This may result in arterial stenosis, occlusion or aneurysm formation. Patients usually present with generalized, constitutional symptoms and later develop clinical manifestations secondary to the arterial lesions. The more common clinical manifestations include absent pulses, cerebral or ocular ischaemia and secondary hypertension3. Diagnosis is usually confirmed by angiography4. Aortic incompetence occurs in 10% of patients with Takayasu's disease5"6. It usually develops insidiously many years after disease onset and is mild to moderate in severity. Rapidly progressive aortic incompetence as in our patient is rare'-9. Aortic incompetence is due to dilatation of the annuloaortic ring and the disease process may involve the valves resulting in valve retraction. Our patient had, also, coronary artery disease. Indeed, 30% of Takayasu patients with aortic incompetence ultimately develop coronary artery disease'. This is due to extension of the disease process into the coronary artery resulting in proximal or ostial stenosis2. Very few Takayasu patients with aortic incompetence have had aortic valve replacement. It is possible that surgery was avoided in these patients due to concern that the disease process may lead to the breakdown of suture sites or aneurysm formation3. However, reported cases of aortic valve replacement have shown good results54. From our own experience, active disease does not make the aortic wall
fibrosis.
Problem fracture in Paget's disease of bone
through the lateral cortex (Figure la). Treatment with salmon calcitonin 100 units subcutaneously thrice weekly and a protective non-weight bearing regimen was instituted. Two weeks later, however, the pain worsened uy, and a completed transverse fracture through one of the.. fissure fractures was found (Figure lb). An interlocking intra-medullary nail was inerted, followed by mobiliation. In April 1988 when walking across a road the patient felt a 'crack' associated with a severe pain in the left thigh. X-rays showed the intra-medullary nail to have fractured (Figure lc). This was removed and replacedw.th. a noninterlocking nail, following which fracte heHng has b6en slow (Figure ld).
R E S Gray MA MRCP' E A Jenkins MBMRCPs M A Hall MB MRCP' M Swann FRCS2 B M Anserl MD FRCP' Departments of 'Rheumatology and 2Orthopaedic Surgery, Wexham Park Hospital, Slough, Berkshire Keywordls: Paget's disease, fracture; internal fixation
Fracture is a well-recognized complication of Paget's disease of bone. Management by internal fixation may be difficult because of poor bone quality"4, deformity37, or excessive bleeding during surgery'-2,i6'8. We report a case further complicated by metal failure of the internal fixation.
Case report A 52-year-old male with rheumatoid arthritis and Paget's disease of bone in the left femur had radiological examination of his hips in 1974 because of reduced range of movement. There was no pain associated with the Paget's disease and no treatment was undertaken. Serum alkaline phosphatase activity increased from 364 iu/l in -1979 to 741 iu/l in 1987 (reference range 60-280 iu/l) but his left femur remained painfree until November 1987 when a severe pain developed in the left knee and thigh, worse on weight bearing, unrelated to any trauma. X-rays showed characteristic features of Paget's disease in the upper two-thirds of the left femur, with three fissure fractures
We therefore recommend that aortic valve replacement be considered as an option for treatment of Takayasu patients who develop progressive aortic incompetence. References 1 Schwartzman RJ, Parker Jr JC. The aortic arch syndrome. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology, vol. 39, part 2. Amsterdam: North Holland Publishing, 1980: 213-38 2 Cipriano P, Silverman JF, et al. Coronary arterial narrowing in Takayasu's aortitis. Am J Cardiol 1977;39:744-50 3 Ishikawa K. Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu's disease). Am J Cardiol 1981; 47:1026-32 4 Land. A, Rossi P. The value oftotal angiography in the diagnosis of Takayasu's arteritis. Radiology 1975;114:287-97 5 Ducailar C, Thulmond A, Semler H, Starr A. Aortic valve replacement for Takayasu's disease. Ann Thorac Surg 1987;
48:102-4 6 Duborg 0, Thomas D, Sirinelli A, Frja G, Gandjbakhch I, Lecerc JP, Bardet J, Grosgogeat Y, Bourdarias JP. Aortic regititatio n in Takayasu's disease: 3 operated cases and a review f the literature. Arch Mal Coeur 1984;77:998-1005 7 Nakan 'T, Isaka N, Takezawa H, Kusagawa M. Successful treatment of acute severe aortic regurgitation caused by Takayasu' arteritis: a case report. Angiology 1986;37:544-9 8 Shimokawa H, Koiwaya Y, Kaku T. Annuloaortic ectasia in a case of Takayasu's arteritis associated with Hashimoto's disease. Br Heart J 1983;49:94-7 9 Akikusa B, Kondo Y, Muraki N. Aortic insufficiency caused by Takaysan's arteritis without clinical features. Arch Pathol Lab
Med 1981'106:650-1
(Accepted 13 April 1989)
Discussion Fracture after minimal trauma is often the presenting feature of Paget's disease1l37,9, but many cases are discovered as- an incidental finding during biochemical or radiological examination for other.reasons10, as was our patient. Indeed, only about 5% O patients with Paget's st bone to suffer disease are symptomatic10. The c Pagetic fracture is the femur1"11, often through a preex1'*73, as in this existing fissure fracture in the latervl case. Commonly the fracture is heralded by several weeks or months of new or worsening pain. otendernessl',3'3 " 2. Approximately 20% of these fractures occur in the upper third of the femoral shaft'. Successfil results have been i and lso by obtained by bed rest and t pl interal fixation with an intra-medullary nfl- althouhb when the femur is very bowed the latter is nically more difficult and better alignment may -be obtained with conservative treatment1'3. However, mortality within three months of the fracture appears lower if prompt surgery is undertaken8. There has been one previous report of failure of treatment due to fracture of the intra-medullary nail6.
Case presented to Section of Rheumatology & Rehabilitation
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