Spontaneous rupture of enormous renal angiomyolipoma in a pregnant tuberous sclerosis patient: a rare case

BMC Nephrology

Zeyang Fan¹  Zechuan Liu¹  Tianshi Lv¹  Haitao Guan¹  Yinghua Zou¹ 

[1] Department of Interventional and Vascular Surgery, Peking University First Hospital, Beijing, China;

关键词: Renal angiomyolipoma;    Tuberous sclerosis;    Pregnant;    Rupture;    Selective arterial embolization;    Prenatal abdominal ultrasound;    Aseptic liquefaction necrosis;   

DOI  :  10.1186/s12882-020-02124-w

来源: Springer

【 摘 要 】

BackgroundRenal angiomyolipoma (RAML) is a rare benign kidney tumour comprised of adipose tissue, smooth muscle, and blood vessels. It can cause fatal complications if it ruptures. Although there have been reports of RAMLs rupturing, it is unusual to see RAMLs rupture during pregnancy, especially in pregnant women with tuberous sclerosis (TSC). Moreover, we reported a rare complication after selective arterial embolization (SAE) for the first time, which called aseptic liquefaction necrosis.Case presentationThe case is a 16-week-pregnant woman with TSC who presented with severe flank pain, which was secondary to the rupture of a large, previously unknown RAML. This was confirmed by emergency computed tomography and successfully treated with selective arterial embolization after the patient received counselling and provided prior informed written consent for medical termination of pregnancy (MTP). The patient underwent abortion 3 weeks after the SAE. The patient required drainage 2 months after the SAE because of aseptic liquefaction necrosis. During follow-up, the patient’s lesion remained stable.ConclusionRAML rupture is a rare but rather serious complication in pregnant tuberous sclerosis patients. Selective arterial embolization (SAE) should be performed immediately, and the status of the pregnancy needs to be assessed by a multidisciplinary team. We also report for the first time the rare complication of aseptic liquefaction necrosis after SAE of RAML, for which percutaneous drainage is effective.

【 授权许可】

CC BY