Vulval elephantiasis

Introduction

Elephantiasis as a result of chronic lymphedema is characterized by gross enlargement of the arms, legs or genitalia, and occurs due to a variety of obstructive diseases of the lymphatic system. Genital elephantiasis usually follows common filariasis and lymphogranuloma venereum. It may follow granuloma inguinale, carcinomas, lymph node dissection or irradiation and tuberculosis but this happens rarely. Vulval elephantiasis as a consequence of extensive lymph node destruction by tuberculosis is very rare. We present two very unusual cases of vulval elephantiasis due to tuberculous destruction of the inguinal lymph nodes.

Case report

A 40 year old Indian woman presented with progressively increasing vulval swellings over a period of five years. She also described a loss of appetite and weight. There was a history of fever with a rise in the evenings, night sweats and vaginal discharge, although her menstrual periods were normal. Eight years prior to presentation, she had generalized lymph node tuberculosis with discharging cervical and inguinal sinuses, for which she received a full course of anti-tubercular therapy. Her tuberculosis was completely cured by the anti-tubercular therapy and she did not show any evidence of a recurrence. Her genital swellings were extremely large and caused her to experience difficulty in walking. Sexual intercourse was not possible.

Our examination revealed that she was poorly nourished, with a marked pallor. There was no lower limb edema. She had two giant vulval swellings measuring 35 × 25 cm on the right side and 45 × 30 cm on the left side (Figure 1 ​and 2). In the standing position, the left vulval swelling extended below her knees. While walking, she had to tuck the swellings between her buttocks. The skin overlying the swellings was thick and rugose.

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Figure 1
Vulval elephantiasis involving both labia majora (35 × 25 cm on the right side and 45 × 30 cm on the left side) with well-healed scars of previously discharging inguinal sinuses. The lower limbs are normal
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Figure 2
The pre-operative appearance

Both her inguinal and cervical regions had puckered scars of healed sinuses without any palpable lymph node. The rest of our physical examination, including her vaginal wall, chest and abdomen was normal.

She was found to be severely anemic (Hb 6 gm%) with a normal leucocyte count. Our other investigations, including blood urea nitrogen, serum electrolytes, creatinine, Mantoux test, night blood smear, chest X-ray, ultrasonography of her abdomen and pelvis, and pap smear were normal.

She was taken up for surgery after the correction of her anemia. A wide local excision with a primary closure was performed. Part of the fibro-fatty tissue of her labia majora was preserved to give them a natural bulging appearance (Figures 2,3,4,5). There was considerable oozing of lymph during surgery and in the post-operative period, but healing occurred with primary intention. The swellings removed from her right and left labia weighed 20 lb and 16 lb respectively. The immediate post-operative period was uneventful. Nearly six years of follow up revealed a satisfactory recovery, although in the immediate post-operative period and in the early follow-up period she presented with seroma formation under the skin flaps that was managed by aspiration and pressure bandaging. She also experienced episodes of serous discharge from the site that was self limiting and was managed by pressure bandaging.

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Figure 3
The resected specimen (the left weighing 20 pounds and the right weighing 16 pounds)
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Figure 4
Post-operative appearance after resection of the lesions
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Figure 5
The specimen bisected, showing grayish-white tissue loaded with lymph

Copyright ©2010 Chintamani et al; licensee BioMed Central Ltd.

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