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Conservative management of giant symptomatic angiomyolipomas in patients with the tuberous sclerosis complex
Author(s) -
Danforth Teresa L.,
Lane Brian R.,
Novick Andrew C.
Publication year - 2007
Publication title -
bju international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.773
H-Index - 148
eISSN - 1464-410X
pISSN - 1464-4096
DOI - 10.1111/j.1464-410x.2007.07059.x
Subject(s) - tuberous sclerosis , medicine , angiomyolipoma , nephrectomy , renal function , surgery , kidney , kidney disease , cystic kidney disease , conservative management , embolization , radiology
OBJECTIVE To present two patients with the tuberous sclerosis complex in whom giant angiomyolipomas (AMLs) were managed conservatively for >20 years, as large (>4 cm) symptomatic AMLs are generally treated with embolization or nephrectomy because of the risks of haemorrhage. PATIENTS AND METHODS The first patient initially presented with bilateral renal AMLs replacing >70% of his parenchymal volume, and a large left renal cyst. Since presentation he was hospitalized three times, but has had no transfusions or procedures to treat his AMLs. His renal function has remained stable during this 21‐year interval. The second patient initially presented with bilateral renal AMLs that were not amenable to nephron‐sparing surgery. After removing her nonfunctioning left kidney, the 24‐cm AML in her right kidney was managed conservatively for >20 years, during which she had 44 transfusions and 11 hospitalizations before uncomplicated right nephrectomy and subsequent need for haemodialysis. CONCLUSION Conservative management of AMLs can preserve renal function for >20 years in patients with tuberous sclerosis at high risk of end‐stage renal disease.