Wunderlich syndrome

Case contributed by Dr Maxymyshyn Nazar

Presentation

Shortness of breath which had been worsening over weeks. Her symptoms began after physical activity and after she felt pain in her left flank. Low Hgb levels on laboratory anaylsis. The patient also had a "benign" brain tumor and epistaxis.

Patient Data

Age: 25 years
Gender: Female
CT

CT shows enlarged, dysmorphic kidneys with multiple small hypoattenuating lesions that are compatible with angiomyolipomas. The left kidney demonstrates a subcapsular heterogeneous mass compressing the spleen and pancreas, and which occupies almost the whole left hemiabdomen. Lower lobe of left lung is compressed with free pleural fluid. There is also free fluid seen in the abdominal cavity. The basal part of the lung shows multiple thin walled cysts.

Case Discussion

Wunderlich syndrome refers to spontaneous nontraumatic subcapsular renal hemorrhage. It is characterized by Lenk's triad (acute flank pain, flank mass, and hypovolemic shock). Angiomyolipomas are one of the commonest causes of Wunderlich syndrome. 

In this case we have findings in multiple organs

Added to the history of a "benign" brain tumor, these findings suggest that this patient has tuberous sclerosis (TS).

A subseptate uterus is also noted, which is not associated with TS but a developmental anomaly.

PlayAdd to Share

Case information

rID: 29386
Published: 23rd May 2014
Last edited: 29th Aug 2019
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.