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
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.
Post operative CT shows a loculated left paravertebral fluid collection, absent left kidney, and absent spleen. Subseptate uterus.
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
- multiple thin walled cysts of basal segments of lung: lymphangiomyomatosis (LAM)
- multiple angiomyolipomas of kidneys
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.
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