A patient presented with retroperitoneal fibrosis but without any ureteric obstruction. The diagnosis was made by an abdominal CT scan and also at laparotomy. Post-operatively, she developed hypertensive encephalopathy. An isotope renogram with captopril was abnormal but not diagnostic of renal artery stenosis. The patient's condition improved with steroid and antihypertensive treatment. A follow-up CT scan showed complete resolution of peri-aortic thickening. A causative link is postulated between retroperitoneal fibrosis, trauma during laparotmy, and onset of acute hypertension.
- retroperitoneal fibrosis
- hypertensive encephalopathy
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Retroperitoneal fibrosis, first described by Ormond in 1948, characteristically affects the peri-aortic tissues often involving the ureters, leading to ureteric obstruction. The pathogenesis of this disease, though not fully understood, is now recognised to be immune mediated. Although no therapeutic trials have been conducted because of low incidence of the disease, steroid treatment has been found to be effective in most cases. Patients often present with non-specific symptoms and occurrence of hypertension has been rarely reported in these patients. The present case report is related to a patient with retroperitoneal fibrosis complicated by hypertensive encephalopathy, the first case report of its kind. We discuss the possibility of a causal relationship between these two conditions and the role of steroid treatment in preventing such complications.
A 50-year-old woman presented with a 3-month history of anorexia and weight loss of 14 lbs. She had undergone hysterectomy and bilateral salpingo-oophorectomy several years earlier and was subsequently put on hormone replacement therapy. She specifically denied previous exposure to ergot, methysergide or practolol. Blood pressure, fundoscopy and a resting electrocardiogram (ECG) were all found to be normal at initial screening. On abdominal palpation, a para-umbilical mass was noted but without any evidence for hepatosplenomegaly or ascites. Full blood count and electrolytes were normal but erythrocyte sedimentation rate (ESR) was raised to 94 mm/h. Auto-immune screen for antibodies was negative and immunoglobulin levels were normal. Abdominal computed tomography (CT) scan showed a fairly well defined cuff of tissue surrounding the aorta from the level of the renal vessels to the distal common iliac arteries on both sides of the pelvis with no abnormality of the other intra-abdominal organs (figure 1).
A laparotomy was performed to exclude lymphoma but extensive thickening of the aorta, superior mesenteric, renal and common iliac arteries was noted. Histology of a cuff of tissue dissected from the peri-aortic region showed predominantly fibrous tissue infiltrated with plasma cells and lymphocytes, the latter often aggregated in places in the form of follicles.
On the second postoperative day her blood pressure progressively rose to 230/120 mmHg, precipitating a generalised convulsion. CT scan of the brain demonstrated oedema over the vertex region with effacement of the cortical sulci but no haemorrhage. The total volume of intravenous fluid replacement in the postoperative period was only 2 litres. 24-Hour urinary catecholamine levels were normal. Systemic blood pressure was controlled with 50 mg intravenous labetalol followed by oral prednisolone (40 mg) and bendrofluazide (2.5 mg daily). An isotope renogram showed no initial abnormality but reduced bilateral renal function following a single dose of captopril. The classical delay in clearance, associated with renal artery stenosis, was not noted. More than a year after discharge from the hospital the patient remains well with normal blood pressure and an ESR of 5 mm/h. A repeat CT of the abdomen (figure 2) showed resolution of the inflammatory oedema and fibrosis around the aorta, seen in the previous CT. Her current treatment comprise atenolol 50 mg, nifedipine 20 mg and prednisolone 5 mg.
The clinical condition of retroperitoneal fibrosis, presenting as ureteric obstruction and peri-aneurysmal thickening of the aortic wall has long been recognised, but wider use of CT scanning detects more asymptomatic cases. This condition is thought to be secondary to leakage of material from atheromatous plaque in the diseased aorta, generating an auto-immune inflammatory response1 and the presenting symptoms depend on the organs or vascular structures involved.
Mild hypertension has been previously reported with this condition,2 but no studies have been carried out to determine a causal relationship. However, hypertensive encephalopathy, manifest by cerebral oedema and a generalised convulsion has not been previously reported. The precise reason for severe hypertension in this woman is subject to speculation, but may involve inflammatory narrowing of the renal arteries, as evidenced by the abnormal post-captopril isotope renogram, with further local tissue oedema resulting from retroperitoneal biopsy at laparotomy. Steroid treatment may have controlled the inflammation improving the patency of the renal arteries. Our patient improved with steroids and antihypertensive therapy and could not be persuaded to undergo renal angiography.
This is the first case report documenting occurrence of hypertensive encephalopathy following operative biopsy in a patient with retroperitoneal fibrosis. The possibility of such a complication should prompt consideration of steroid treatment before invasive procedures or laparotomy is undertaken.
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