‘NOT SO WONDERFUL’ WUNDERLICH SYNDROME – CASE REPORT OF A RUPTURED LARGE RENAL ANGIOMYOLIPOMA

1. ‘NOT SO WONDERFUL’ WUNDERLICH
SYNDROME – CASE REPORT OF A
RUPTURED LARGE RENAL
ANGIOMYOLIPOMA
Roshan Valentine, Reddi Prasad Yadavalli
Department of Interventional Radiology
Manipal Hospital, Bangalore

2. INTRODUCTION
• Angiomyolipoma (AML) is the most common benign neoplasm of the kidney.
• First described histologically by Fischer et al in 1911 and named by Morgan et al
in 1951
• Histologically composed of mature adipose tissue, smooth muscle and abnormal
blood vessels – hence prone for rupture
• Syndromic association with Tuberous Sclerosis
• Usually asymptomatic but can present with spontaneous life threatening
retroperitoneal hemorrhage referred to as WUNDERLICH SYNDROME.
• First line treatment is Endovascular embolization, preferably with Polyvinyl
alcohol(PVA) particles.
• Lesions more than 4cm and intratumoral pseduoaneurysm less than 5mm
warrants intervention.

3. CASE REPORT
• 49 year old female, presented
with vague right flank pain
and giddiness to Emergency
room
• She was tachycardic and
hypotensive on initial
assessment
• Point of Care Ultrasound
– Free fluid in the right
perinephric region
• CT scan showed a ruptured
right renal AML with
characteristic CLAW sign
• Active contrast extravasation
with a small pseudoaneurysm
in the right upper pole was
seen.

4. Fig.A &B) Selective right renal angiogram shows active
contrast extravasation with gradual filling of the upper pole
pseudoaneurysm(yellow arrow).
Fig.C) Post Embolisation (300-500microns PVA particles) shows
complete occlusion of the pseudoaneurysm and AML.
FIGURE A FIGURE B FIGURE C

5. DISCUSSION
• Spontaneous rupture of the renal AML is not uncommon.
• No clear concord exists with the treatment of choice for AML, however renal
artery embolization is seen to cause regression and disappearance of the
angiomyomatous component which was noted to cause bleeding in these lesions.
• Coil embolization of the renal AML can hinder reinterventions and can promote
collateral reformations.
• Proper risk stratification of AML based on the size of the lesion, growth rate, size
of intralesional pseudoaneurysm and syndromic association can guide the
interventional radiologist in early intervention and thus protecting the kidneys
and the life of the patients.

6. REFERENCES
• Hatano T, Egawa S. Renal angiomyolipoma with tuberous sclerosis complex: How it
differs from sporadic angiomyolipoma in both management and care. Asian J Surg.2020
:8–13.
• Ramon J, Rimon U, Garniek A, Golan G, Bensaid P, Kitrey ND, et al. Renal
Angiomyolipoma: Long-term Results Following Selective Arterial Embolization. Eur Urol.
2009;
• Xu XF, Hu XH, Zuo QM, Zhang J, Xu HY, Zhang Y. A scoring system based on clinical
features for the prediction of sporadic renal angiomyolipoma rupture and hemorrhage.
Medicine (Baltimore). 2020 May 1;99(20):e20167