Management of congenital urogenital and perineal vascular malformations: correlation of clinical findings with diagnostic imaging for treatment decision.

PURPOSE: Analysis of clinical and diagnostic findings in rare urogenital and perineal vascular malformations only occurring in 2-3 % of vascular anomalies with regard to clinical symptoms and treatment decisions. MATERIALS AND METHODS: All 25 out of 537 patients presenting with congenital urogenital and perineal vascular malformations at our institution from 2014 to 2021 were included. Vascular anomaly classification, anatomical location, clinical symptoms at presentation, diagnostic imaging, and pain intensity were retrospectively assessed from the patient record and therapy management was evaluated. RESULTS: In total, 25 patients (10 females (40 %), 15 males (60 %)), aged 6 to 77 years were included. Diagnoses were: 10 (40 %) venous malformations (VMs), 5 (20 %) lymphatic malformations (LMs) and 10 (40 %) arteriovenous malformations (AVMs). Malformation manifestations were: 12 (32 %) lesser pelvis, 12 (32 %) external genitalia, and 13 (34 %) perineal/gluteal region. One AVM was located in the kidney. The leading clinical symptom was pain. The mean intensity was 6.0/10 for LM, 5.7/10 for VM, and 4.5/10 for AVM. Further major symptoms included physical impairment, local swelling, and skin discoloration. Bleeding complications or sexual dysfunction were rare findings. Patients with VM reported significantly more symptoms than patients with AVM (p = 0.0129). In 13 patients (52 %) minimally invasive therapy was indicated: 10 (77 %) sclerotherapies and 3 (23 %) transcatheter embolization procedures. Complete symptomatic remission was achieved in 9 (69 %) patients, partial response in 3 (23 %) patients, and 1 patient showed no clinical response to therapy. Follow-up appointments without the need for immediate minimally invasive therapy were significantly more common in patients with AVMs than in patients with VMs (p = 0.0198). CONCLUSION: To create a higher awareness of congenital urogenital and perineal vascular malformations. Awareness of this rare condition avoids misdiagnosis. Therapy decisions should be symptom-oriented. Emergency intervention is rarely required, even in fast-flow vascular malformations. KEY POINTS: · Venous malformations cause more symptoms with higher pain intensity than arteriovenous malformations.. · Diagnosis and adequate treatment can be hampered by a lack of awareness of the clinical presentation.. · Bleeding complications are rare, even in high-flow vascular malformations.. · Pain and physical impairment are the most commonly observed symptoms in these patients..

Diagnostic radiology findings and spectrum of therapeutic interventions in gynaecological and urogenital vascular anomalies.

Vascular anomalies represent a rare congenital disease with manifestation at diverse anatomical sights and presenting with heterogenous symptoms. Undetected, they can progress and create acute and chronic complications with functional impairment. The manifestation in the female and male pelvis and the urogenital tract represents a multidisciplinary challenge for physicians. Especially outpatient management in gynaecology and urology is affected. Diagnostic Radiology holds an important supportive role in early diagnosis of the underlying urogenital vascular anomaly and referral to interventional radiology, either for minimal invasive treatment, or to surgery for further assessment. This pictorial review creates awareness for the spectrum of vascular anomalies of the gynaecological and urogenital tract, their characteristic imaging findings and dedicated interventional treatment options. The individual description of vascular anomalies, based on an appropriate nomenclature and classification standard, is a guide for radiologists to distinguish the underlying vascular anomaly from other vascular disorders and to accelerate diagnosis as well as therapeutic proceedings. In consequence, interdisciplinary management of patients with vascular anomalies of the female and male pelvis will benefit.