Diffuse lymphangiomatosis with genital involvement—evaluation with magnetic resonance lymphangiography

Abstract

Objective To assess, for the first time, the morphology of the lymphatic system in patients with diffuse lymphangiomatosis and genital involvement by magnetic resonance lymphangiography (MRL). Materials and methods Ten patients with diffuse lymphangiomatosis and genital involvement were examined by MRL. Three locations were examined: first, the lower leg and foot region; second, the upper leg and the knee region; and third, the pelvic with retroperitoneal region. MR imaging was performed with a 1.5-T system equipped with high-performance gradients. For MRL, a T1-weighted 3D-spoiled gradient-echo and a T2-weighted 3D-TSE sequence were used. Results The size of the genital lymphangiomas, which were revealed in all patients, varied between 6 and 85 mm. In 60% of the patients, lymphangiomas were additionally detected at the level of the lower legs, and in 70% patients at the level of the upper leg. Furthermore, lymphangiomas were seen in the inguinal and retroperitoneal regions in 80%, and in the pelvic region and anterior abdominal wall in 90% of the patients examined. The genital lymphangiomas feeding lymphatic vessels were detected in 80% of the patients in the anterior abdominal wall and in 90% of the patients in the inguinal and pelvic regions; 90% of the patients suffered consecutively from a lymphedema of the lower extremities. All patients suffered from recurrent infections in the genital region; 80% of the patients repeatedly experienced genital lymphorrhea due to lympho-cutaneous fistulas and lymphcysts. Conclusion MRL is a safe and accurate minimal-invasive imaging modality for the evaluation of the lymphatic circulation in patients with diffuse lymphangiomatosis and genital involvement. Because the site and extent of the lymphangiomas with their feeding lymphatic vessels are important prognostic factors, performing radiologic evaluation with a high resolution is crucial for the therapeutic planning of patients.