Spontaneous retroperitoneal haemorrhage

J. M. WOLFF,* P. K. JUNG,* G. ADAMI AND G. JAKSE*
*Department of Urology and #Department of Radiology, R WTH Aachen, Aachen, Germany

Spontaneous retroperitoneal haemorrhage is an uncommon entity. It is even rarer when the underlying cause is associated with renal disease. In most cases the non-traumatic rupture of a kidney requires an emergency surgical intervention as the underlying disease only becomes clear intra-operatively. Most patients with a spontaneous kidney rupture have a renal tumour. Of these renal tumours, angiomyolipomas show a particular tendency to rupture. The cases are reported of five patients who presented with an acute onset of spontaneous retroperitoneal haemorrhage from different kidney disorders. The importance of considering the possibility of spontaneous kidney rupture in the evaluation of patients presenting with spontaneous retroperitoneal haemorrhage is stressed.

Keywords: retroperitoneal haemorrhage, spontaneous kidney rupture, urinary tract.

Non-traumatic retroperitoneal haemorrhage is an uncommon, but dramatic, clinical entity. It can be due to the rupture of an abdominal aortic aneurysm, adrenal bleeding, blood dyscrasia, hypertension and, rarely, renal disorders.¹Spontaneous rupture of the kidney affects either the collecting system or parenchyma and, in most cases, the non-traumatic rupture is associated with underlying diseases of the kidney.²Whereas rupture of the collecting system can be managed satisfactorily by drainage (either a percutaneous nephrostomy or ureteral tube drainage), rupture of the renal parenchyma accompanied by severe renal haemorrhage demands surgical exploration of the kidney. In many instances a nephrectomy will be necessary, especially in patients with a ruptured tumour, where nephrectomy remains the treatment of choice. We report here the cases of five patients with a traumatic renal rupture seen in our department. After evaluation all patients presented with stable circulation and did not require resuscitation. In all patients the kidney was exposed through a flank incision and, after the renal pedicle was controlled, Gerota’s fascia was opened and the kidney was explored. Both diagnosis and management are discussed.

A 29-year-old woman in her 22nd week of gestation was referred to our department due to the sudden onset of left flank pain. She did not have any history of trauma. So far, the course of her pregnancy had been uneventful, with no symptom related to the urinary tract. Physical examination showed a tender palpable mass in her left flank. Laboratory studies, including a complete blood count, electrolytes, renal function tests, coagulation studies and urine analysis, were normal, with the exception of a low serum haemoglobin concentration, a slightly increased white blood cell count and a microscopic haematuria. Abdominal ultrasound and computed tomography showed a rupture of her left kidney, with a peri-renal blood extravasation extending to the left psoas muscle. A left nephrectomy was performed through a flank incision and histological examination of the specimen showed the rupture of an angiomyolipoma.

A 50-year-old woman was directed to our department because of left flank pain and an increasing mass in her left flank. Her urological history was normal and she did not have a history of trauma. Physical examination showed a palpable mass and a tenderness in her left flank. Laboratory studies, including a complete blood count, electrolytes, renal function tests, coagulation studies and urine analysis, were normal, with the exception of a low serum haemoglobin concentration and a microscopic haematuria. Abdominal ultrasound and computed tomography showed a large peri-renal haematoma due to a rupture of the left kidney. The kidney was explored through a flank incision and a left nephrectomy was performed. Histological examination of the specimen disclosed a rupture of an angiomyolipoma.

A 23-year-old man presented with acute right flank pain and temperatures up to 40⁰C. Laboratory findings, including a complete blood count, electrolytes, renal function tests, coagulation studies and urine analysis, were normal except for a slightly increased white blood cell count and a C-reactive protein concentration of 123 mg/L. Abdominal ultrasound and computer tomography showed a peri-renal haematoma and a tumour of the right kidney, 10 cm in diameter. The right kidney was exposed by flank incision and a right nephrectomy was performed. The histological examination showed an oncocytoma of the kidney.

A 55-year-old woman was referred to our department due to the sudden onset of left flank pain, fever and nausea. Her physical examination showed a tender mass in her left flank. Laboratory studies showed a low serum haemoglobin concentration of 101 g/L, a white blood cell count of 20.7 g/L; and a C-reactive protein concentration of 135 mg/L. Coagulation studies showed a prolonged coagulation time and prothrombin time. Abdominal ultrasound and computed tomography showed a rupture of the left kidney with a peri-renal blood extravasation near a ruptured kidney cyst (Figure 1). A left nephrectomy was performed through a flank incision and histological examination of the nephrectomy specimen disclosed a rupture of a renal cyst.

	Figure 1 (a) Plain abdominal computed tomography scan in the prone position shows a large parenchymal haematoma around the left kidney.
	(b) After intravenous contrast administration the ruptured cyst is visible.

A 57-year-old man was seen with the onset of acute left flank pain associated with nausea and vomiting. He did not have any recent history of trauma. Physical examination showed a localized tenderness in the left lower quadrant of the abdomen and a left flank mass. Laboratory results, including a complete blood count, electrolytes, renal function tests, coagulation studies and urine analysis, were normal except for a slightly increased white blood cell count and a microscopic haematuria. Abdominal ultrasound and computed tomography showed a rupture of a hydronephrotic left kidney and an enormous hydronephrotic mass with blood and fluid extravasation. A left nephrectomy was performed through a flank incision and histological examination of the ruptured hydranephrotic kidney showed a metastasis of adenocarcinoma of the colon in the left kidney.

Non-traumatic retroperitoneal haemorrhage due to a spontaneous kidney rupture is a known, but uncommon, entity. However, more cases have been reported in the last few years, mainly due to new imaging techniques.³The disorder was originally described by Wunderlich in 1856, who pointed out that a peri-renal haematoma may be due to spontaneous kidney rupture.⁴Since then about 200 cases have been reported. By definition, a spontaneous rupture of the kidney requires the absence of recent instrumentation, surgery or trauma.⁵At presentation the clinical picture may include the classical ‘Lenk’s triad’, consisting of acute flank pain, tenderness and symptoms of internal bleeding.⁶

In most instances the spontaneous renal rupture is associated with underlying diseases of the kidney,⁷and only occasionally has the spontaneous rupture of an otherwise normal kidney been reported.²McDougal et al.²reported the cases of four patients and reviewed the literature. Of more than 300 cases that had been reported, only 78 qualified as pathologically proved cases of spontaneous parenchymal rupture with peri-renal haematoma. The aetiology of these cases is illustrated in Table 1. Renal tumours account for most cases, with clear cell carcinoma being predominant, followed closely by angiomyolipomas. Vascular disease was the next most common cause, whereas infection, nephritis and blood dyscrasia occurred less commonly.

Table 1 Spontaneous rupture of renal parenchyma: underlying diseases and its incidence (adapted from McDougal et al.²)

Disease	Percentage of patients
Tumours	58
benign (e.g. angiomyolipoma)	42
malignant (e.g. clear cell cancer)	58
Vascular (e.g. peri-arteritis nodosa)	18
Infection	10
Nephritis	5
Blood dyscrasia	5
Miscellaneous	4

Rupture of the kidney during pregnancy is rarely reported. Whereas spontaneous rupture of the renal pelvis during pregnancy has been reported in otherwise normal kidneys, those cases of spontaneous rupture of the renal parenchyma during pregnancy have also been associated with underlying disease of the kidney.⁸’⁹ Occasionally, the intrapartum rupture of angiomyolipomas has been reported, where increased blood volume and increased renal blood flow are thought to have a causal relation.⁹

About 5% of renal tumours are renal oncocytomas, which have a wide spectrum of behaviour range from benign to malignant. The diagnosis is predominantly made on pathological examination as there are no reliable distinguishing clinical characteristics.¹⁰Furthermore, higher grade oncocytomas have metastatic potential and have led to the death of the patient.¹¹Therefore the recommended treatment of the solid renal mass lesion remains radical nephrectomy. However, in some cases of a small tumour a partial nephrectomy as a nephron-sparing surgery may offer a surgical alternative.

Simple renal cysts are common, as up to 50% of the population over the age of 50 years are reported to have one. Their complications include obstruction, infection, rupture or haemorrhage, confined either to the cyst or causing subcapsular or peri-renal haemorrhage.¹²The cause of cyst rupture with haemorrhage is unclear, as it is not known whether expansion with increased intracystic pressure occurs, with the subsequent tearing of blood vessels, or whether haemorrhage into the cyst is the first event, with subsequent rupture from cyst expansion.¹³In most cases peri-renal haemorrhage requires surgical intervention.

The metastatic spread of solid tumours, a secondary renal tumour, is often seen at necropsy as an incidental finding. It occurs in about 7% of patients in a combined necropsy series. The lung, breast and pancreas are the most common primaries which produce renal metastases. As a late event they rarely cause symptoms which lead to their clinical identification before the patient’s death. Only occasionally does a secondary renal tumour manifest itself as a clinical problem necessitating intervention. Despite the surgical removal of the primary and secondary lesion, the survival statistics for patients with renal metastases from solid tumours are uniformly poor.¹⁴

In conclusion, non-traumatic retroperitoneal haemorrhage is uncommon and even rarer when the disease derives from the kidney. Most cases are treated as an emergency and the use of imaging techniques such as ultrasound and computed tomography aid diagnosis. Although there is a case for conservative surgical treatment to preserve renal parenchyma, nephrectomy is still considered to be the treatment of choice in patients with a severe perirenal haematoma.^1,2 In all our patients the kidney was exposed through a flank incision. However, a midline transabdominal approach, which allows safer vascular control before exploring the ruptured kidney, should be considered in patients with signs of a large blood loss from heavy retroperitoneal bleeding. The high incidence of kidney tumours among these patients warrants radical surgical intervention.

Correspondence: Dr J.M. Wolff Department of Urology, RWTH Aachen, 52057 Aachen, Germany.

� 1998 The Royal College of Surgeons of Edinburgh, J. R. Coll. Surg. Edinb., 43, February, 53—56