Vaginal Discharge, Lymphangiography, Therapeutic Embolization

26세 여자 환자로 18년 된 외음부 및 질 림프유출과 외음부의 다수의 좁쌀같은 병변들이 있었다. MR 영상에서 양cmr 외음부와 골반 내에 다수의 T2 고신호 병변들이 보였다. Lipiodol 림프조영술에서 우하복벽에서의 두드러진 림프관에서 기원하여 외음부로의 림프 역류가 보였다. 이 두드러진 림프관을 경피적으로 천자하여 NBCA의 상방 파급을 막기 위해 미세코일로 천자된 림프관의 원위부를 색전하였고 림프관을 NBCA/Lipiodol 혼합물로 색전하였다. 이 시술 후 일년 째 추적 검사에서 피부 병변과 증상은 소실되었다.

A 26-year-old woman had an 18-year long history of vulvar and vaginal lymphorrhea and multiple millet-like lesions on her vulva. ON MRI, multiple T2 high signal intensities were noted at the bilateral vulvar areas and pelvic cavity. Conventional lipiodol lymphangiography showed lymphatic reflux to the vulvar areas, possibly originating from prominent tubular lymphatics in the right lower abdominal wall. After percutaneous puncture of this tubular lymphatic structure, its distal portion was embolized using microcoils to prevent upward glue propagation; this was followed by glue embolization of the tubular lymphatic structure. At one year followup, the patient's skin lesions disappeared.

Lymphorrhea is an uncommon but potentially serious complication after surgical lymphatic injury or chronic lymphedema. Among the many types of lymphorrhea, vulvar and/or vaginal lymphorrhea is a rare event of lymphatic reflux (1, 2). Interventional techniques such as lymphangiography and assisted embolization are known to be safe and effective alternatives for managing lymphorrhea that do not respond to conservative treatment. Most reported lymphorrhea treated with interventional techniques includes chylothorax or chylous ascites, but vulvar and/or vaginal lymphorrhea had not been reported so far. Here, we report a successful result of conventional lipiodol lymphangiography and adjunctive embolization for a patient with vulvar and vaginal lymphorrhea.

증례 26세/여자 임상소견 A 26-year-old woman was referred for the evaluation of vulvar and vaginal lymphorrhea. She had an 18-year history of vulvar and vaginal secretion and multiple millet-like lesions on her vulva. When she was 10-years old, she underwent multiple percutaneous sclerotherapy and laser therapy sessions. At the age of 20, she underwent percutaneou s radiofrequency ablation to obliterate the dilated lymphatics at her vulva. These remedies were ineffective. At 24, she underwent surgical lymphovenous connection, which she did not achieve satisfactory results. There was no sign of lymphedema. 진단명 Vulvar and vaginal lymphorrhea 영상소견 On MRI, multiple dots and serpentine structures of T2 high signal intensity were observed in the bilateral vulvar areas and pelvic cavity, compatible with lymphangiectasia (Fig. 1). Therefore, conventional lipiodol lymphangiography with or without adjunctive embolization was planned through a multi-disciplinary discussion. 시술방법 및 재료 Intranodal lymphangiography through the right inguinal lymph node was performed using a 25 G spinal needle (Tae-Chang Industrial, Gongju, Korea) under ultrasound guidance. The injected ethiodized oil (Lipiodol; Guerbet, Paris, France) moved quickly toward the bilateral pelvic cavities as well as the vulvar areas (Fig. 2A). Reflux of lymphatic flow to the vulvar areas seemed to originate from a prominent tubular lymphatic structure in the right lower abdominal wall (Fig. 2A). It appeared that coil embolization of the distal portion of this tubular lymphatic structure could reduce the lymphatic flow going upwards, and subsequent NBCA embolization of this structure could block the lymphatic reflux into the vulvar areas. As planned, the tubular structure was punctured using a Chiba needle (Cook, Bloomington, IL, USA) under ultrasound guidance, and a microguidewire (Meister; Asahi Intecc, Nagoya, Japan) was inserted. Clear fluid leaked through the puncture site (Fig. 2B). A microcatheter (Progreat Lambda 1.7Fr; Terumo, Tokyo, Japan) was advanced over the micro guidewire. Subsequently, the distal portion of this tubular lymphatic structure was embolized using four microcoils (Micronester, Cook, USA) to prevent upward propagation of the NBCA. Coil embolization was followed by embolization of the tubular lymphatic structure using a 1:2 mixture of NBCA (Histoacryl; B.Braun, Tuttingen, Germany) and Lipiodol (Fig. 2C). Prominent lymphatic channels were opacified with lipiodol in the abdominal and pelvic retroperitoneum. 추적관찰 The multiple millet-like vulvar lesions had almost disappeared at the two-week follow-up visit with complete resolution of vulvar or vaginal secretions. In addition, the patient reported no skin lesions or symptoms at the one-year followup after the procedure.

MRIs for vulvar and vaginal lymphorrhea A. Axial T2-weighted scan shows multiple dots and linear structures of high signal intensity (SI) involving bilateral sides of the pelvic cavity. B. Coronal T2-weighted scan shows a prominent tubular structure (arrows) of high SI involving the right lower abdominal wall and high SI involving both vulvar areas (arrowheads).

Conventional intranodal lymphangiography and adjunctive embolization A. Right inguinal lipiodol lymphangiography shows prominent lymphatic opacification in the bilateral pelvic cavities and the vulvar areas. Note the reflux of lymphatic flow (arrows) to the vulvar area from a prominent tubular lymphatic structure (arrowheads). B. The prominent tubular lymphatic structure (arrowhead) was punctured with a Chiba needle (arrow) under fluoroscopic guidance; clear fluid leaking through the puncture site is seen. C & D. The prominent tubular lymphatic structure has been embolized with microcoils (arrow in C) for the distal portion and glue (arrowheads in C) for the rest. Note the prominent lymphatic opacification in the abdominal and pelvic retroperitoneum.

Vulvar and/or vaginal lymphatic leakage can be associated with intestinal lymphangiectasia, skin xanthomatosis, or primary lymphedema (2). Its etiology can be explained by an incompetent valvular system with extensive lymphangiectasis (primary) or obstruction of the lymphatics (secondary) (1, 3). The reflux site might be anywhere in the gynecologic tract: uterus, cervix, vagina, or vulva (1). This patient was considered genital involvement of diffuse lymphangiomatosis based on her history of earlyonset symptoms and prominent retroperitoneal lymphatic channels on lymphangiography (4). Since lymphangiomatosis and lymphatic reflux were confined to the retroperitoneal and genital regions in this patient, lymphedema did not develop. It is crucial to identify the disease extent as a thorough lymphatic evaluation of the whole body. In this case, an excellent clinical outcome was achieved in the patient because lymphatic flow into the vagina and vulva was effectively blocked by percutaneous NBCA embolization. The minimal amount of reflux can persist after the embolization procedure, but it can be resorbed; therefore, symptom improvement or resolution can be expected. When blocking abnormal lymphatic channels with NBCA at a certain level, coil embolization at the distal site for reducing upward lymphatic flow may facilitate greater NBCA embolization of targeted reflux, as seen in this patient.

1. Kornreich L, Idelson A, Shuper A, Ziv N, Mimouni M, Hadar H. The CT manifestations of the primary gynecological chylous reflux syndrome in the pediatric age. Pediatr Radiol 1988;18:503-4. 2. Karg E, Bereczki C, Kovacs J, Korom I, Varkonyi A, Megyeri P, et al. Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia. Br J Dermatol 2002;146:134-7. 3. Adashi EY, Mitchell GW, Jr., Farber M. Gynecological aspects of the primary chylous reflux syndrome: a review. Obstet Gynecol Surv 1981;36:163-71. 4. Lohrmann C, Foeldi E, Langer M. Diffuse lymphangiomatosis with genital involvement-evaluation with magnetic resonance lymphangiography. Urol Oncol 2011;29:515-22.