Recurrent retroperitoneal hemorrhage in a patient with tuberous sclerosis complex: a case report

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.

Renal hematoma: An unusual cause of acute abdomen and fetal demise in severe Pre-Eclampsia

“…[5] Trans-arterial embolization or stent-grafting of the bleeding vessels is effective in controlling hemorrhage and can be repeated if bleeding recurs (redo angio-embolization). [9] Angiography was done in our case with the aim to confirm the diagnosis and treat the ongoing hemorrhage by selective embolization. Though selective arterial embolization is reported in the literature, it was not possible in this patient in view of multiple vessel involvement.…”

Wunderlich’s Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma

“…Tuberous sclerosis, or Bournville–Pringle disease, is a rare genetic condition that occurs sporadically or as a familial form, and affects cellular differentiation and proliferation, which results in hamartomatous lesions in many organs, most commonly in the brain, heart, eyes, kidneys, skin, and lungs [ 1 , 2 ]. Renal involvement is described in tuberous sclerosis, with renal angiomyolipoma being the most common association [ 2 , 4 ]. The prevalence of renal angiomyolipoma in patients with tuberous sclerosis is reported to be between 50–80%, which means that careful screening for the presence of renal tumors is advised in these patients [ 3 , 4 ].…”

“…Renal involvement is described in tuberous sclerosis, with renal angiomyolipoma being the most common association [ 2 , 4 ]. The prevalence of renal angiomyolipoma in patients with tuberous sclerosis is reported to be between 50–80%, which means that careful screening for the presence of renal tumors is advised in these patients [ 3 , 4 ]. Renal angiomyolipoma usually has a benign course, but can be a source of bleeding, with Wunderlich’s syndrome, spontaneous non-traumatic retroperitoneal hemorrhage, being one of the most serious complications, occurring in 10% of patients with angiomyolipoma [ 2 , 4 ].…”

“…The prevalence of renal angiomyolipoma in patients with tuberous sclerosis is reported to be between 50–80%, which means that careful screening for the presence of renal tumors is advised in these patients [ 3 , 4 ]. Renal angiomyolipoma usually has a benign course, but can be a source of bleeding, with Wunderlich’s syndrome, spontaneous non-traumatic retroperitoneal hemorrhage, being one of the most serious complications, occurring in 10% of patients with angiomyolipoma [ 2 , 4 ]. An increase in the size of the renal angiomyolipoma (>4.0 cm in diameter), multifocal tumors, and an association with tuberous sclerosis increases the risk of bleeding [ 2 , 5 ].…”

The role of tyrosine kinase inhibitors in tuberous sclerosis

“…Tuberous sclerosis (TS) is an autosomal dominant neurocutaneous syndrome characterised by widespread benign hamartomatous lesions in several organs (Shwartz et al , 2007; Chiarugi et al , 2008; Curatolo et al , 2008). Although the primary treatment of TS is surgical, there is growing evidence regarding medical treatment alternatives (Shwartz et al , 2007; Zhang et al , 2007; Curatolo et al , 2008; Huang & Manning, 2008).…”