Extensive pelvic lymphatic malformations presenting with hematospermia: A case report

Lymphatic malformations are rare vascular abnormalities that usually occur in the head and neck but can occur elsewhere in the body. Here, we present an unusual case of a 14-year-old male who presents with hematospermia, hematochezia, and hematuria. Following work-up which included urinalysis, ultrasound, and magnetic resonance imaging (MRI), the patient was diagnosed with pelvic lymphatic malformations (LMs), including a perirectal lesion and a lesion involving the right spermatic cord. He was started on sirolimus therapy, which resolved the hematospermia and hematuria, and decreased the frequency of the hematochezia. However, the hematochezia did not resolve completely and the patient wished to discontinue sirolimus. The decision was made to pursue doxycycline sclerotherapy for the perirectal lesion. The hematochezia resolved for two weeks following sclerotherapy, but resumed soon thereafter. Options to further manage the patient's symptoms include re-starting sirolimus or repeating doxycycline sclerotherapy. To our knowledge, this is the first report of a lymphatic malformation presenting with hematospermia. Pelvic lymphatic malformations should be considered as a possible etiology for otherwise unexplained bleeding episodes in the adolescent genitourinary system.