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.
Objectives: Arterio-ureteralfistula (AUF) is an infrequent but potentially life-threatening condition. The aim of this study was reviewing the literature to build a flow-chart useful for an early and effective diagnosis and treatment of this pathology.Materials and methods: A literature search in PubMed was con-ducted. In addition, retrieved articles were cross-referenced. Data parameters included oncologic, vascular and urological history, diagnostics, treatment, and follow up were collected using a standard template by 2 independent reviewers. Results: A total of 140 cases of AUF out of 172 available in the literature at the time of the review, were considered. All patients presented gross hematuria. Chronic indwelling ureteral catheter (CIUC); history of pelvic surgery (HPS) and history of pelvic radiotherapy (HRT) were present respectively in 81%, 62.1%and 58.6% of the sample. The most predominant location of AUF was at the common iliac artery ureteral crossing. Angiography with provocative measures had the highest diag-nostic sensitivity (50%) and endovascular treatment with stent-graft placement across the fistula is the current state of the art treatment choice.Conclusions: Failure to diagnose can postpone a potentially life-saving targeted therapy and lead to complications. The identifi-cation of the Trifecta hematuria, history of pelvic surgery (HPS) and history of pelvic radiotherapy (HPR) would allow the identi-fication of patients at high risk of AUF, who may benefit from more sensitive early diagnostic investigations such as CT angiography and provocative angiography. The treatment of choice in case of AUF to date consist in endovascular prosthesis placement.
Objective: To present a retrospective analysis on the oncological and functional outcomes of a single-center experience on a large series of extraperitoneal laparoscopic radical prostatectomies (eLRP) with an extended follow-up. Materials and methods: Herein we present a retrospective review of patients who underwent eLRP. Oncological and functional follow-up data were collected by means of outpatient visits and telephone interviews, assessing overall mortality and biochemical recurrence-free survival. Patients with clinical T4 stage prostate cancer (PCa), previous surgery for benign prostatic hyperplasia (BPH), previous androgen deprivation, radiotherapy, concomitant chemotherapy and/or experimental therapies, and with insufficient follow-up data were excluded. Preoperative data recorded were age, body mass index, ultrasound prostate volume, preoperative PSA and clinical stage of PCa. Operative data (operative time, nerve sparing technique and any perioperative complication) and pathological findings were obtained by consulting the surgical and pathological reports. Oncological and functional follow-up were collected during follow-up visits and telephone interview. Results: Between January 2001 and December 2019, overall 938 eLRP were performed at our Institution. The median follow-up was 132 months. 69.7% of the patients had complete dataset. The estimated overall biochemical recurrence (BCR)-free survival was 71.4% at 5 years and 58.9% at 10 years. Cancer specific survival was 84,5%. Erectile function was preserved in the most of patients as postoperative IIEF-5 score within 12 months after surgery was > 12 in the 82.1%. About the urinary incontinence, 0.76% of the patients presented severe incontinence (continued and persistent loss of urine) and 7.0% were mildly incontinent (using up to one pad per day). Conclusions; eLRP has shown oncological and functional results comparable to other minimally invasive techniques and to open radical prostatectomy (ORP), with favorable perioperative outcomes than the open technique and a reduced complication rate.
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