Genitourinary and perineal vascular anomalies in children: A Seattle children's experience

Abstract

We sought to evaluate the initial presentation and diagnosis, to compare presenting symptoms of these anomalies with respect to location, and describe the management course and review the treatment outcomes of vascular anomalies (VA) in genitourinary and perineal area. We described changes in the evaluation and management approach over time. After IRB approval, we reviewed the Vascular Anomalies Program 3,780 patient database from 1998-2013, identifying 85 patients with GUP VA. Demographics, presentation, anatomic location, imaging modalities and treatments were reviewed. For the 76 patients that were managed at our institution, we analyzed data for management approach change over time, duration of follow-up, complications, and treatment outcomes. Using Chi-squared test, we analyzed the treatment eras for any difference in presenting symptoms, or management approach. From the VAP database, we identified 85 patients with GUP VA. Vascular tumors (VT) were more common than vascular malformations (VM) (62/85, 72.9% vs. 23/85, 27.1%). The 62 patients with VT were subdivided into infantile hemangioma (IH) (49, 79%) and congenital hemangioma (CH) (13, 20.9%). The 23 patients with VM were subdivided into simple (18, 78.2%) and complex (2, 8.7%), and one patient had Klippel-Trenaunay syndrome. Overall, genital VA were more common than perineal (43/85, 54.2% vs. 24/85, 28.2%). None of our patients had urinary involvement. Ultrasound was used in 34% (29/85) of VA patients, the majority were VM (18/23, 78%). Majority of patients were symptomatic with ulceration, pain and bleeding (54/85, 63.5%). Malformations were more complex, presenting as extensive lesions (6/23, 26.1%), which included pelvic extension in 5 (21.7%). Seventy-six patients were managed at our institution. Treatment was utilized more frequently than observation overall (46/76, 60% vs. 30/76, 40%), and more frequently for VM than VT (12/18, 63% vs. 34/58, 57%). After introduction of oral propranolol for treatment of IH in 2010, fewer patients presented with ulceration (p=0.213), and more were treated with propranolol over procedural therapy (p=0.157). Overall, complete treatment of these lesions was achieved in 82.9% (63/76), with low complication rate (6/76, 7.9%). Evaluation and management of VA is best performed with expert multidisciplinary team at tertiary care center. Imaging studies are mostly used in evaluation of VM. Symptomatic VA involving the perineum and genitalia require therapeutic management. With the introduction of propranolol for treatment of IH, oral therapies were used more often with excellent outcomes. A multidisciplinary approach is essential to treatment innovation, patient-centered therapies and improving therapeutic outcomes.