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Bilateral wünderlich syndrome caused by spontaneous rupture of renal angiomyolipomas.

Sotošek S, Markić D, Španjol J, Krpina K, Knežević S, Maričić A - Case Rep Urol (2015)

Bottom Line: Wünderlich syndrome (WS) is a urological emergency characterized by retroperitoneal hemorrhage.Because the patient was stable and the tumor was not malignant, treatment was conservative.Follow-up revealed the full recovery of kidney function and the resolution of the hematoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, University Hospital Rijeka, Tome Strižica 3, 51 000 Rijeka, Croatia.

ABSTRACT
Wünderlich syndrome (WS) is a urological emergency characterized by retroperitoneal hemorrhage. In most cases, bleeding occurs from a renal angiomyolipoma (AML) and may be the first manifestation of the disease. We report a female patient with bilateral WS due to the metachronous rupture of renal AMLs. Because the patient was stable and the tumor was not malignant, treatment was conservative. Follow-up revealed the full recovery of kidney function and the resolution of the hematoma.

No MeSH data available.


Related in: MedlinePlus

CT scan revealed a perirenal hematoma around the left kidney with an angiomyolipoma in the ventral part of the kidney.
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fig1: CT scan revealed a perirenal hematoma around the left kidney with an angiomyolipoma in the ventral part of the kidney.

Mentions: A 49-year-old Caucasian female presented to the emergency department with abrupt-onset pain in the left flank, radiating to the left inguinal region. She had no concomitant diseases and no known history of trauma, anticoagulation, or bleeding diathesis. Physical examination revealed a mildly obese female with severe tenderness in the left flank and left upper quadrant. The patient was afebrile with normal blood pressure and without tachycardia. The patient's hemoglobin was 136 g/L, creatinine was 68 μmol/L, and the estimated glomerular filtration rate was 88 mL/min. Urine sediment was normal. Abdominal ultrasound examination revealed a large retroperitoneal mass around the left kidney with heterogeneous echogenicity of the lower pole of the left kidney. For further evaluation, computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast was performed, revealing a large left perinephric hematoma that distended the renal fascia and displaced the peritoneal and retroperitoneal structures. The estimated size of the hematoma was 15 cm in the cephalocaudal, 4 cm in the anteroposterior, and 7 cm in the transverse dimensions. In addition, a hypodense mass without calcification was detected in the lower pole of the left kidney that was poorly marginated from the perinephric hematoma (Figure 1). These findings were highly suggestive of the rupture of the angiomyolipoma of the left kidney with massive retroperitoneal hemorrhage, a condition known as WS. Because the patient was hemodynamically stable without significant changes in the hemoglobin level, conservative treatment was chosen, including the monitoring of vital signs (blood pressure and pulse), blood testing (hemoglobin and creatinine levels), and repeated ultrasound scans. She was initially administered intravenous fluids, after which she was placed on an oral diet and prescribed a broad-spectrum antibiotic. The patient remained stable throughout the hospital stay and was discharged ten days after admission. Follow-up examinations (including assessments of the hemoglobin and creatinine levels, ultrasound of the kidney, and CT scan) were performed 3 and 6 months after the initial presentation, revealing the complete morphological recovery of the left kidney with complete resolution of the perinephric hematoma (Figure 2). After five years, a similar clinical scenario was observed, but on the right side (Figure 3). The same diagnostic pathway with conservative management was performed with the complete resolution of the hematoma (Figure 4). Now, 10 years later, the patient has normal renal function without retroperitoneal hematoma. On ultrasound, both kidneys appear normal with two AMLs (3 cm on the left and 4 cm on the right sides). Because the renal AMLs are bilateral and the patient has experienced episodes of bleeding on both sides, she is educated about the possibility of repeated bleeding. She has also been informed to report immediately in the case of sudden abdominal pain. At this time, our patient has refused any proposed surgical procedures (nephron-sparing surgery).


Bilateral wünderlich syndrome caused by spontaneous rupture of renal angiomyolipomas.

Sotošek S, Markić D, Španjol J, Krpina K, Knežević S, Maričić A - Case Rep Urol (2015)

CT scan revealed a perirenal hematoma around the left kidney with an angiomyolipoma in the ventral part of the kidney.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4352461&req=5

fig1: CT scan revealed a perirenal hematoma around the left kidney with an angiomyolipoma in the ventral part of the kidney.
Mentions: A 49-year-old Caucasian female presented to the emergency department with abrupt-onset pain in the left flank, radiating to the left inguinal region. She had no concomitant diseases and no known history of trauma, anticoagulation, or bleeding diathesis. Physical examination revealed a mildly obese female with severe tenderness in the left flank and left upper quadrant. The patient was afebrile with normal blood pressure and without tachycardia. The patient's hemoglobin was 136 g/L, creatinine was 68 μmol/L, and the estimated glomerular filtration rate was 88 mL/min. Urine sediment was normal. Abdominal ultrasound examination revealed a large retroperitoneal mass around the left kidney with heterogeneous echogenicity of the lower pole of the left kidney. For further evaluation, computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast was performed, revealing a large left perinephric hematoma that distended the renal fascia and displaced the peritoneal and retroperitoneal structures. The estimated size of the hematoma was 15 cm in the cephalocaudal, 4 cm in the anteroposterior, and 7 cm in the transverse dimensions. In addition, a hypodense mass without calcification was detected in the lower pole of the left kidney that was poorly marginated from the perinephric hematoma (Figure 1). These findings were highly suggestive of the rupture of the angiomyolipoma of the left kidney with massive retroperitoneal hemorrhage, a condition known as WS. Because the patient was hemodynamically stable without significant changes in the hemoglobin level, conservative treatment was chosen, including the monitoring of vital signs (blood pressure and pulse), blood testing (hemoglobin and creatinine levels), and repeated ultrasound scans. She was initially administered intravenous fluids, after which she was placed on an oral diet and prescribed a broad-spectrum antibiotic. The patient remained stable throughout the hospital stay and was discharged ten days after admission. Follow-up examinations (including assessments of the hemoglobin and creatinine levels, ultrasound of the kidney, and CT scan) were performed 3 and 6 months after the initial presentation, revealing the complete morphological recovery of the left kidney with complete resolution of the perinephric hematoma (Figure 2). After five years, a similar clinical scenario was observed, but on the right side (Figure 3). The same diagnostic pathway with conservative management was performed with the complete resolution of the hematoma (Figure 4). Now, 10 years later, the patient has normal renal function without retroperitoneal hematoma. On ultrasound, both kidneys appear normal with two AMLs (3 cm on the left and 4 cm on the right sides). Because the renal AMLs are bilateral and the patient has experienced episodes of bleeding on both sides, she is educated about the possibility of repeated bleeding. She has also been informed to report immediately in the case of sudden abdominal pain. At this time, our patient has refused any proposed surgical procedures (nephron-sparing surgery).

Bottom Line: Wünderlich syndrome (WS) is a urological emergency characterized by retroperitoneal hemorrhage.Because the patient was stable and the tumor was not malignant, treatment was conservative.Follow-up revealed the full recovery of kidney function and the resolution of the hematoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, University Hospital Rijeka, Tome Strižica 3, 51 000 Rijeka, Croatia.

ABSTRACT
Wünderlich syndrome (WS) is a urological emergency characterized by retroperitoneal hemorrhage. In most cases, bleeding occurs from a renal angiomyolipoma (AML) and may be the first manifestation of the disease. We report a female patient with bilateral WS due to the metachronous rupture of renal AMLs. Because the patient was stable and the tumor was not malignant, treatment was conservative. Follow-up revealed the full recovery of kidney function and the resolution of the hematoma.

No MeSH data available.


Related in: MedlinePlus