Abstract
RATIONALE: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous port wine capillary malformations, varicose veins with hemihypertrophy of soft tissue and bone.Pelvic and retroperitoneal vascular malformations have been described up to the 30% of patients with KTS while hemangiomas of the urinary tract have been reported in 6% PATIENT CONCERNS:: A 30-year-old man with KTS was referred to our center for primary erectile dysfunction (ED) associated with varicosities of unusual distribution and asymmetry of the lower limbs. Furthermore, he suffered from hypertension and autosomal dominant polycystic kidney disease.During penile prosthesis implantation, a significant intraoperative bleeding (1 liter) due to large scrotal venous malformations and profuse bleeding from both corpora was recorded. One month later, the day after the first prosthesis training session, the patient returned with swelling in the penoscrotal region. A large inguino-scrotal hematoma was drained. There was a complete bilateral dehiscence of corpora cavernosa with a spread venous bleeding in the scrotum. DIAGNOSES: CT scan showed hypertrophy of the right hypogastric artery with severe vascular malformations: the right pudendal artery was massively dilated with early visualization of venous drainage without evidence of arteriovenous fistulae; regular bulbocavernous capillary blush; right upper gluteus artery hypertrophic and dilated. Multiple twisting and aneurysms of the right internal pudenda artery were bleeding from multiple points. Cystoscopy showed a fistula between the proximal urethra and the penoscrotal dartos. Coagulation tests revealed the presence of factor XIII deficiency INTERVENTIONS:: The patient underwent several procedures including percutaneous scleroembolization of the internal pudendal arteries, removal of the penile implant, recombinant factor XIII (FXIII) administration, and cord blood platelet gel application. OUTCOMES: The patient was discharged after almost 3 months in hospital, hemodynamically stable. LESSONS: Experience regarding management of ED in KTS patient is limited and in case of concomitant factor XIII deficiency, the clinical scenario can be life-threatening. A multidisciplinary approach including a urologist, an interventional radiologist and a hematologist in our experience represented the key approach in case of severe bleeding following surgery for ED.