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Genitourinary and perineal vascular anomalies in children: A Seattle children's experience
Katie Willihnganz-Lawson1, Jennifer Gordon2, Jonathan Perkins1
1Seattle Children's Hospital, Seattle, WA, USA.
Insights
Genitourinary and perineal vascular anomalies (VA) are often symptomatic, with vascular tumors being more common than malformations. Expert multidisciplinary care and newer therapies like propranolol have led to excellent treatment outcomes.
Area of Science:
- Vascular Surgery
- Pediatric Surgery
- Dermatology
Background:
- Vascular anomalies (VA) in the genitourinary and perineal (GUP) region present unique diagnostic and management challenges.
- Understanding the presentation, location, and treatment outcomes of these anomalies is crucial for optimizing patient care.
Purpose of the Study:
- To evaluate the initial presentation and diagnosis of GUP vascular anomalies.
- To compare presenting symptoms based on anomaly location.
- To describe the management course and treatment outcomes, noting changes in approach over time.
Main Methods:
- Retrospective review of 85 patients with GUP VA from a specialized Vascular Anomalies Program database (1998-2013).
- Analysis of demographics, presentation, location, imaging, and treatments for all patients.
- Detailed review of management, follow-up, complications, and outcomes for 76 patients managed at the institution.
- Chi-squared test used to compare treatment eras regarding symptoms and management.
Main Results:
- Vascular tumors (72.9%) were more frequent than vascular malformations (27.1%).
- Genital anomalies (54.2%) were more common than perineal (28.2%); no urinary involvement was noted.
- Symptomatic presentations (ulceration, pain, bleeding) occurred in 63.5% of patients.
- Introduction of oral propranolol post-2010 correlated with decreased ulceration and increased use of oral therapy for infantile hemangioma.
- Overall complete treatment was achieved in 82.9% with a low complication rate (7.9%).
Conclusions:
- Expert multidisciplinary team management at a tertiary care center is optimal for GUP VA.
- Imaging is primarily utilized for evaluating vascular malformations.
- Symptomatic GUP VA necessitate therapeutic intervention, with propranolol showing excellent outcomes for infantile hemangioma.
- A multidisciplinary approach drives innovation and improves patient-centered therapeutic outcomes.
Aims:
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.
Methods:
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.
Results:
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%).
Conclusions:
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.
