INTRODUCTION: Sarcoidosis is characterized by multiorgan involvement of granulomatous inflammation. It rarely affects female genital tract and even rarer to cause fistulizing disease. Majority of cases of female genital tract sarcoidosis have been seen in uterus with sporadic cases involving ovaries, fallopian tubes, cervix and vagina.
CASE PRESENTATION: A 30 year old African American woman with no past medical histroy presented to our hospital with lower abdominal pain of few months duration. She was diagnosed with fibroids and underwent myomectomy .The pathology revealed areas of hyalinization with foci of necrosis. Her abdominal symptoms persisted and she got readmitted to the hospital. She was started on antibiotics with presumed diagnosis of pelvic inflammatory disease (PID). CT abdomen and chest done at that time showed hilar, bilateral axillary and retroperitoneal lymphadenopathy. Cervical lymph node biopsy was done which revealed well formed granulomas with negative GMS and AFB stains. She was diagnosed with pulmonary sarcoidosis and was started on low dose of steroids. Her abdominal symptoms resolved simultaneously. On tapering her steroids she started to experience recurrent episodes of pelvic pain. On examination she had cervical motion tenderness. CT scan pelvis revealed free fluid in cul-de-sac with multiseptated left ovarian mass. She was treated for PID with antibiotics without much relief. Blood, urine, and cervical cultures also came back negative. She continued with symptoms and soon underwent exploratory laparotomy with left salpingoopherectomy. The pathology reported granulomatous salpingitis with granulomatous oophoritis. A biopsy taken from pelvic wall bowel nodule revealed nodular fibrous and fibrovascular tissue with granulomatous inflammation along with vegetable material in the sample which raised concerns of gastrointestinal perforation. She underwent colonoscopy, small bowel series and lower GI studies which failed to reveal any abnormalities. Soon after her discharge from the hospital she again developed abdominal pain and was found to have right sided tubo-ovarian abscess. She underwent repeat exploratory laparotomy with total hysterectomy and right salpingoophorectomy. The biopsy was consistent with generalized granulomatous disease in the ovary, fallopian tube and the uterus with negative microbiology. Her post operative course got complicated by enterocutaneous fistula followed by enterovaginal fistula. Repeat colonoscopy ruled out any pathological evidence of Inflammatory Bowel Disease. Patient was started on high dose steriods. Patient's stool output from the fistula decreased markedly over a span of one week on treatment suggesting sarcoidosis as the culprit of the fistulas.
DISCUSSION: Sarcoidosis also called Boeck's disease was first identified over 100 years ago in Norway. It is very crucial that before the patient is diagnosed with genital tract sarcoidosis more obscure causes of the granulomatous inflammation are ruled out including coccidiomycosis, lymphogranuloma inguinale, foreign body reaction, tuberculosis and leprosy. Thus microbiological proof is vital to differentiate sarcoidosis from other forms of granulomatous inflammation. Here we present a unique case of sarcoidosis which fulfills the criteria of diagnosis of on the basis of histopathology and clinical response of the patient to the anti-sarcoid chemotherapy. The fistulizing form of sarcoidosis in the female genital tract has not been reported yet. There has been one case report of anal fistula due to sarcoidosis. The fistulizing form also responds adequately to steriods as noted in our case, therefore adequate time should be taken before decision to reoperate the patient is made.
CONCLUSIONS: Non- infectious cases for recurrent tubo-ovarian abscesses should be kept in mind. Sarcoid although rare in the female genital tract can present with features of pelvic inflammatory disease. Fistulizing disease has not been clearly reported in association to sarcoidosis. We report a unique case of female genital tract sarcoidosis with extensive involvement of ovaries, fallopian tubes and uterus, later complicating with fistulas which showed excellent response to steriods.
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DISCLOSURE: The following authors have nothing to disclose: Maximiliano Tamae Kakazu, Ashima Sahni, Aiyub Patel
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