31

Volume 68 December 1975

805

Clinical Section President Ian Burn FRCS

Meeting 10 January 1975

(continuedfrom November 'Proceedings' p 748)

Cases Perihepatic Hiematoma in Polyarteritis Nodosa D H Fitchett MB MRCP (for C M Oakley MD FRCP) (Royal Postgraduate Medical School, Ducane Road, London W12 OHS) W N, man aged 21 History: Presented in April 1972 to Charing Cross Hospital (Dr R A Parkin) with a three-month history of anorexia, weight loss, aching muscles, epigastric pain and fever. He was found to have hepatosplenomegaly, a right lateral popliteal nerve lesion, and to be hypertensive. A diagnosis of polyarteritis nodosa (PAN) was confirmed by muscle biopsy. His symptoms rapidly resolved with corticosteroid therapy and the hypertension was controlled with methyldopa. In August 1974, three days after returning from Spain, he presented at Hammersmith Hospital with severe oropharyngeal ulceration and high fever. A clinical diagnosis of Vincent's angina was confirmed by bacteriological examination. Despite treatment with metronidazole and later parenteral penicillin, it was six days before the fever resolved and he was able to return to some activity. This coincided with a dramatic rise in blood pressure to 180/120 mmHg. One week after hospital admission he had a sudden onset of epigastric pain, became shocked, with an unrecordable blood pressure, and was noted to have marked hepatomegaly (6 cm below costal margin). The shock responded rapidly to transfusion; over the next 3 hours the hepatomegaly lessened (2 cm below costal margin). Investigations suggested an intrahepatic heemorrhage or infarction: WBC 22 300/mm3, amylase 175 u/l, bilirubin 3.1 mg/100 ml, SGOT 260, alkaline phosphatase 140 u/l. Chest X-ray: elevation of right hemidiaphragm. Liver scan: large area of decreased uptake in right lobe. One week later, following a further episode of severe epigastric pain and transient hypotension, a coeliac angiogram was performed. This demon-

strated (Fig 1) deviation of the origin of the hepatic artery to the left of the vertebral column. The intraparenchymal branches showed multiple small aneurysms and stenoses, and several large (1-2 cm diameter) aneurysms were seen in both lobes of the liver. During the hepatogram phase it was shown that right margin of the liver was separated from the diaphragm and chest wall by a considerable distance (10 cm). No abnormalities were demonstrated by abdominal aortic and selective renal arteriography. Treatment with antihypertensive therapy remains the basis of his future management. Discussion

Polyarteritis is a disease of the medium- and smallsized blood vessels, and is characterized by multiple foci of fibrinoid necrosis, which form in the media and later involve the intima and

Fig 1 Selective hepatic arteriogram, right posterior oblique view. There is displacement of the right liver edge (arrowed)from the abdominal wall. Many aneurysms andstenoses are demonstrated in the intraparenchymal arteries. Some of the aneurysms are large and multilocular

806 Proc. roy. Soc. Med. Volume 68 December 1975

adventitia. Clinical presentation depends upon the site and extent of involvement. In our patient the presenting features are characteristic of widespread disease with no pulmonary involvement. The diagnosis usually depends upon histological examination, often of skeletal muscle. As skeletal muscle is involved in only 20-30% of cases the diagnostic dividends of muscle biopsy cannot be high. The almost universal visceral involvement has led to the increasing use of hepatic and renal histology as an aid to diagnosis. It is also well recognized, as in the present case, that despite the presence of many macroscopic vascular lesions, needle biopsy of the liver may be normal (Bron & Gajaraj 1970). Selective visceral angiography may therefore provide diagnostic evidence of widespread aneurysmal disease which is pathognomonic of PAN. Most reported cases of PAN studied by angiography have demonstrated renal involvement, but similar changes are described in multiple sites (Efsen & Lorenzen 1968). Abdominal crises are often an early feature of PAN. Visceral hEemorrhage, infarction, perforation or even pancreatitis may lead to a multitude of presenting symptoms, from focal pain to a diffuse pain without findings at laparotomy. PAN is the most common cause of massive hepatic infarction: with both hepatic arterial and venous radical involvement, sufficient ischemia may result to produce true infarction of the liver parenchyma. Perihepatic hkmatoma, producing a large tender intra-abdominal tumour, results from the rupture of a superficial arteritic lesion. The present case demonstrates widespread hepatic abnormalities, without renal or other abdominal visceral involvement. Some of the aneurysms are large and multilocular (Fig 1). The clinical, liver scan and angiographic evidence also suggests that the patient suffered massive hepatic hemorrhage on at least two occasions. It is thought that on the first occasion the hmemorrhage occurred centrally in the right lobe. Subsequently it spread to the outer part of the right lobe and displaced the rest of the liver substance to the left. It is likely that the perihepatic heemorrhage resulted from a short period of uncontrolled hypertension. At the time of his most recent illness the cause of the severe oral ulceration was not apparent; because of the possibility of drug hypersensitivity, methyldopa was stopped for a short period. As the patient's activity increased his blood pressure became uncontrolled, preceding the intra-abdominal haemorrhage. At no time was there evidence for active vasculitis (ESR 7 mm in 1 hour, Westergren, absent clinical symptoms). The hiemorrhage probably resulted from a rupture of previously diseased vessels.

32

The future prognosis of this patient probably depends upon careful antihypertensive therapy to prevent further hepatic htmorrhage. However, the presence of hypertension almost certainly indicates glomerular involvement (Rose & Spencer 1957), even in the absence of abnormalities on angiographic, urographic and biochemical investigations. Although steroids may dramatically alleviate the acute symptoms, and aneurysms may regress (Robins & Bookstein 1972) there is little evidence to suggest that corticosteroid therapy improves the long-term survival. This case illustrates the classical presentation of PAN; and the complication of massive hepatic hemorrhage from visceral aneurysms, later demonstrated by angiography. Medical treatment is currently successful in preventing further hemorrhage; however, ligation of the common hepatic artery will be seriously considered should another hepatic bleed occur. REFERENCES

Bron K M & Gajaraj A (1970) New England Journal ofMedicine 282, 1024-1025 Efsen F & Lorenzen U (1968) Acta Radiologica 7,225-230 Robins J M & Bookstein J J (1972) Radiology 104, 39-42 Rose G A & Spencer H (1957) Quarterly Journal of Medicine 216, 43

Pulseless Disease with Optic Atrophy, Aortic Incompetence and Recurrent Peptic Ulceration M P Linnemann1 BA MB (for V WJohnson MB MRCP) (New Cross Hospital, Wolverhampton) M T, woman aged 57 History: Sudden blindness in left eye at age 52, in 1969. Intermittent claudication of right arm for at least five years and postprandial epigastric pain for several years. She was premenopausal. No family history of premature arterial disease. On examination: Left central retinal artery occlusion. No arcus cornea. Right radial pulse was variably reported to be absent or scarcely palpable, with diminution of right subclavian and left radial pulses. Bruits audible over common carotid arteries, left supraclavicular fossa, right brachial artery, abdominal aorta, iliac and femoral arteries and renal areas. Initially, blood pressure unrecordable in right arm, 210/130 mmHg in left arm, 170/120 in right leg, 190/130 in left leg. Left external jugular vein was distended and nonpulsatile and a Corrigan's pulse and aortic incompetence were present. Recently, a warm, firm, pulsatile swelling developed in upper right thigh and buttock. Investigations: Postero-anterior chest X-ray taken with increased penetration, as recommended by 'Present address: Royal Hospital, Wolverhampton

Perihepatic haematoma in polyarteritis nodosa.

31 Volume 68 December 1975 805 Clinical Section President Ian Burn FRCS Meeting 10 January 1975 (continuedfrom November 'Proceedings' p 748) Cas...
431KB Sizes 0 Downloads 0 Views