A Report on a Case of Ruptured Angiomyolipoma in a 31-year old, 27 weeks AOG Gravid Patient with Tuberous Sclerosis Complex

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A Report on a Case of Ruptured Angiomyolipoma in a 31-year old, 27 weeks AOG Gravid Patient with Tuberous Sclerosis Complex
Mary Ann
Espina
Maia Celeste Arbatin miansolisespina@gmail.com Chong Hua Hospital Adult Nephrology CEBU CITY
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Angiomyolipomas are benign renal tumors and bleeding are uncommon. However, patients with Tuberous Sclerosis Complex have more complex lesions with higher risk of rupture especially in pregnant patients. The paucity of data on which it is encountered poses a therapeutic dilemma. 

 

This is a case of a 31-year-old  gravid female patient, G2PO (0010) pregnancy uterine, 27 1/7 wks AOG diagnosed with Tuberous Sclerosis Complex. Clinical features include shagreen patch, periungal fibroma and facial angiofibroma  associated with findings of cortical tubers on MRI of the brain and multiple angiomyolipomas on both kidneys with largest size situated in the upper pole of the left kidney measuring 10 cm. Patient came in due to severe left flank pain associated with oliguria, elevated serum creatinine and anemia (hemoglobin 6.7 mg/dL). MRI of abdomen showed perirenal hematoma. Blood transfusion and Gel Foam Selective Arterial Embolization were performed. Patient was stabilized and pregnancy was continued until term. 

Angiomyolipoma associated with Tuberous Sclerosis requires preassessment of risk of complications to guide management. In high risk population including female patients with possibility of pregnancy, close monitoring and early intervention with embolization or mTOR inhibitor could prevent complications. In a setting of active bleeding that threatens pregnancy, multidisciplinary team is recommended to improve both maternal and fetal outcomes.

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