Stesura Seveso Archivio Italiano di Urologia e Andrologia 2013; 85, 4210 INTRODUCTION Wunderlich’s syndrome is a clinical condition defined as a spontaneous renal bleeding of non traumatic origin, con- tained within the Gerota’s fascia. In 1700, Bonet was the first one who described this con- dition, while C.R.A. Wunderlich was the first to make a clinical de scription in 1856 (1). Coenen used the term Wunderlich’s syndrome for the first time in 1910 (2). Various authors find as underlying causes: nephritis, tu - mours, vascular diseases, cysts rupture (3-7). Classically it presents with acute flank pain, tender palpa- ble mass and clinical hemodynamic deterioration. These symptoms are defined as the Lenk’s classic triad (8). We present three cases of spontaneous renal bleeding. CASE 1 A 72 years old man presented to the emergency depart- ment with severe generalized abdominal pain. He described a 3-hour history of acutely worsening abdominal and left lumbar pain and vomiting. Neither urinary symp- toms nor history of trauma. He had a previous medical his- tory positive for hypertension, dyslipidaemia and hyper- uricemia. Clinically he had hypertension (160/100 CASE REPORT Wunderlich’s syndrome: Three cases of acute spontaneous renal bleeding, conservately treated Andrea Guttilla, Alessandro Crestani, Francesco Cattaneo, Fabio Zattoni, Claudio Valotto, Massimo Iafrate, Fabrizio Dal Moro, Filiberto Zattoni Urology Clinic, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padua, Italy. Wunderlich’s syndrome is a clinical condition defined as a spontaneous renal bleeding of non traumatic origin, contained within the Gerota’s fascia. Wunderlich’s syndrome is rare. Spontaneous bleeding of kidney tumors, either benign or malignant, represents the more common causes. Classically it presents with acute flank pain, tender palpable mass and clinical hemo- dynamic deterioration. These symptoms are defined as the Lenk’s classic triad. We present three cases of spontaneous renal bleeding. KEY WORDS: Kidney; Spontaneous bleeding; Angiography; Kidney tumours. Submitted 21 December 2012; Accepted 31 March 2013 No conflict of interest declared Summary mmHg), tachycardia, a voluminous lumbar tumefaction and haematoma (Figure 1). Haemoglobin was 7.1 g/dL. A computed to mography (CT) was performed, showing a Figure 1. Case 1: voluminous lumbar tumefaction and haematoma. DOI: 10.4081/aiua.2013.4.210 211Archivio Italiano di Urologia e Andrologia 2013; 85, 4 Wunderlich’s syndrome: Three cases of acute spontaneous renal bleeding, conservately treated large (16 cm) left perinephric haema toma with an active bleed- ing in the sub capsular and perirenal space. Normal contro- lateral kidney (Figure 2). The patient’s haemoglobin levels continued to decrease, and he was transfused with 4 units of blood. The patient underwent emer- gency em bolization (Figu re 3) of a 3-cm avascular area in the left kidney at the middle third (possible sub capsular lesion) with an active bleeding from a thin arterial capsular branch of the lower renal pole. Then, he was admitted to the In - tensive Care Unit (ICU) for close observation and strict bed-rest. His haemoglobin level stabi- lized, and he was treated con- servatively. He was discharged from the Urology Department after 30 days. CT scans before leaving the hospital and the one made after 90 days showed significant reduction of the perirenal haema toma (Figures 4, 5). One year after the patient is fine. The haematoma is completely disappeared (Figures 6, 7). He still has problems of blood pressure that he is controlling with different drugs. CASE 2 A 75 years old woman presented to the emergency department with severe left lumbar pain. Neither urinary symptoms nor history of trauma. She had a previous medical history posi- tive for ischemic heart disease, atrial fibrillation in therapy with anticoagulants, hyperten- sion, cronic kidney failure with an atrophic right kidney. Clinically he had hypotension (110/70 mmHg), tachycardia, haemoglobin 7 g/dL, serum creatinine 300 mmol/L. The patient’s haemoglobin lev- els continued to decrease, and she was transfused with 5 units of blood. The patient underwent a CT scan that showed an impor- tant lumbar haematoma (Figure 8). The patient underwent emergency renal arteriography that described: stenosis of the left renal artery, with no parenchymal vascularisation. The left artery was embolized with kidney exclusion. Then, she was admit- ted to the ICU for close observation and strict bed-rest. The haemoglobin levels during the staying in the ICU Figure 2. Case 1: computed tomography (CT) showing a large (16 cm) left perinephric haematoma with an active bleeding in the sub capsular and perirenal space. Figure 3. Case 1: emergency embolization of a 3-cm avascular area in the left kidney at the middle third (possible sub capsular lesion) with an active bleeding from a thin arterial capsular branch of the lower renal pole. Figures 4-5. Case 1: CT scans at dismissal and after 90 days showed significant reduction of the perirenal haematoma. Archivio Italiano di Urologia e Andrologia 2013; 85, 4 A. Guttilla, A. Crestani, F. Cattaneo, F. Zattoni, C. Valotto, M. Iafrate, F. Dal Moro, F. Zattoni 212 started to rise up. She was discharged from the Urology Department after 12 days with haemoglobin value of 10.3 mg/dl. The patient was lost at follow-up. CASE 3 A 57 years old man presented to the emergency depart- ment with acute right lumbar pain. Neither urinary symptoms nor history of trauma. He had a previous medical history positive for hypertension. Clinically he had normal blood pressure (140/80 mmHg). Haemoglobin was 12.1 g/dL. A CT was performed, show- ing a large (10 cm) right perinephric haematoma with no evidence of active bleeding and a 6 cm sub capsular mass. Normal contralateral kidney. We decided to treat him conser- vately and to treat the mass surgi- cally after the acute problem was resolved. He was discharged from the Urology Department after 12 days. CT scan before leaving the hospital showed significant reduc- tion of the perirenal haematoma and confirmed the presence of a solid area. One month after, the patient underwent open right partial nephrectomy. The pathological report showed a type 2 papillary RC, Fuhrman grade 3, pT1aNx. DISCUSSION Wunderlich’s syndrome is a rare syndrome, with about 300 cases described in the literature. The most common causes of these spontaneous haemorrhages are represented by neoplasm (61%) (9). As benign lesion, angiomy- olipoma (31.5%) is the most common, while renal cell carci- noma is the most common malignant one (10, 11). Some authors have also described some other different causes as vasculi- tis, arteriovenous fistulas, rup- ture of renal artery aneurism rup- ture and nephritis (12). The imaging studies, for patient with suspicious of a spontaneous renal bleeding, are CT scan or angiography (9, 10). CT scan can give the opportunity to know the entity of the haematoma and the presence of an active bleed- ing. Selective angiography with embolization is often useful in the acute phase of the haemor- rhage in order to control bleed- ing, contribute to diagnosis and reduce the need for sur- gery. The management is dictated by the clinical condi- tion of the patient and by the underlying aetiology. Sometimes subjects with unstable haemodynamic condi- tion can require an emergency nephrectomy especially if the cause of the bleeding is clear (i.e. renal cancer). In other cases, as the one described above, a conservative treatment to preserve renal function can be the best choice. Also in kidney bleeding following a trauma, many Authors recommend a conservative treatment if major complications, as other abdominal injuries, are not present (13, 14). Eventually, if the imaging done during the follow-up shows a clear diagnosis a nephrectomy must be per- Figures 6-7. Case 1: CT scan after 1 year showing that haematomais completely disappeared. Figure 8. Case 2: CT scan showing an important lumbar haematoma. 213Archivio Italiano di Urologia e Andrologia 2013; 85, 4 Wunderlich’s syndrome: Three cases of acute spontaneous renal bleeding, conservately treated formed. The follow-up, after patient discharge, should be done with CT scans to monitor the haematoma reduc- tion and with measurements of blood pressure to exclude Page syndrome (15, 16). CONCLUSION Wunderlich’s syndrome is rare. Spontaneous bleeding of kidney tumours, either benign or malignant, represents the more common cause. CT scan and angiography are the preferred diagnostic tools. 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Correspondence Andrea Guttilla, MD (Corresponding Author) andrea.guttilla@gmail.com Alessandro Crestani, MD alessandro.crest@gmail.com Francesco Cattaneo, MD i.francescocattaneo@gmail.com Fabio Zattoni, MD fabiozattoni@gmail.com Claudio Valotto, MD claudio.valotto@sanita.padova.it Massimo Iafrate, MD massimo.iafrate@unipd.it Fabrizio Dal Moro, MD fabrizio.dalmor@gmail.com Filiberto Zattoni, MD filiberto.zattoni@unipd.it Urology Clinic, Department of Surgical, Oncological and Gastroenterological Sciences, University of Padua, Via Giustiniani, 2 - 35100 Padua, Italy