Recurrent retroperitoneal hemorrhage in a patient with tuberous sclerosis complex: a case report.
Chiarugi M, Martino MC, Pucciarelli M, Decanini L, Vignali C -Cases journal(2008)
F4:subependymal calcification as seen at the CT scan.
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Affiliation:Department of Surgery, University of Pisa, Pisa, PI, Italy. m.chiarugi@dc.med.unipi.it.
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Bottom Line:Tuberous sclerosis complex (TSC) is an autosomal dominant disorder.It is characterized by seizures, mental retardation and hamartomatous lesions, including facial angiofibroma, subependymal giant cell astrocytoma, cardiac rhabdomyoma and renal angiomyolipoma (AML).AMLs can bleed severely in the retroperitoneal space.
Abstract

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder. It is characterized by seizures, mental retardation and hamartomatous lesions, including facial angiofibroma, subependymal giant cell astrocytoma, cardiac rhabdomyoma and renal angiomyolipoma (AML). AMLs can bleed severely in the retroperitoneal space.

Case presentation: Herein, we present the case of a TSC patient presenting with recurrent severe episodes of retroperitoneal hemorrhage from AMLs successfully managed by angio-embolization.

Conclusion: Transarterial embolization is effective in preventing and controlling hemorrhage in patients with AMLs of the kidney.

Mentions
In September 2000, a 48 year-old Caucasian male with sporadic tuberous sclerosis presented to the emergency room with acute right flank pain, large abdominal mass, signs and symptoms of internal bleeding (hypotension, tachycardia and acute anemia). Abdominal CT scan showed bilateral renal giant angiomyolipomatosis with a honey-comb aspect of the kidneys and a right retroperitoneal haematoma. Moreover, an arterial contrast blush was seen within the right retroperitoneal hemorrhagic infarction (Figure 1). The patient underwent angiography (Figure 2), which showed the right kidney to be supplied by two main arteries and the presence of multiple intraparenchymal aneurysms (arrows) in the territory of the lower artery. There was no active bleeding but due to the size of AML and of the aneurysms a selective embolization with metal coils was carried out. Adjunctive features of the syndrome in this patient included sebaceous adenoma of the face (Figure 3), and sub-ependymal calcifications (Figure 4). The course was uneventful and kidney function was maintained. A further episode of massive retroperitoneal bleeding occurred 40 months later. The bleeding source was from a AML lesion of the upper pole of the left kidney (Figure 5). Control was achieved by selective angio-embolization. Since then, there has been no recurrence of retroperitoneal bleeding, but the patient has developed a mild renal failure.
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