Labial fusion is a rare form of female genital chronic graft-versus-host disease (GvHD). We report on the successful treatment of five patients who presented with labial fusion between 23 months and 8 years post-hematopoietic cell transplantation (HCT) (Table). Of these five women, three had severe sclerotic GvHD (Patient 1, 3, 4), one woman had bronchiolitis obliterans (Patient 2), and one woman had no other GvHD sites (Patient 5). In all cases, labial fusion was classified as severe vulvovaginal GVHD (Stratton-Turner Grade III). Patient 1 presented with worsening difficulty with micturition, vulvar fullness and inability to have intercourse; she had only a pinpoint opening for passage of urine. Severe sclerotic GvHD also significantly narrowed her mouth opening. Her complete labial fusion and vaginal adhesions were lysed under general anesthesia. Three of five women (Patient 2, 3, 4) presented with dyspareunia. The remaining woman (Patient 5) was not sexually active; labial fusion was noted during routine gynecology examination. The labia of these four women were fused between the clitoris and urethra resulting in a narrowed vaginal opening without urethral obstruction. Their labial adhesions were successfully lysed during an office procedure using local anesthesia. In all patients, topical skin adhesive (dermabond) was applied to the denuded labial surfaces to maintain labial separation during healing. Once the labial mucosa healed, patients applied topical clobetasol ointment and topical estrogen to the affected area for several weeks to prevent reagglutination. On follow-up, all women were significantly improved and dyspareunia resolved. These patient histories highlight the importance of asking women, especially those with severe chronic GvHD, about dyspareunia and pain with urination. Those with genital symptoms warrant referral to a gynecologist skilled in evaluation and treatment of women post-HCT. These cases illustrate that labial fusion from chronic GvHD, if diagnosed early enough, may be treated successfully with an office procedure and medical therapy.
Patient
| Underlying disease/ indication for transplant
| Conditioning regimen; GvHD prophylaxis at the time of genital GvHD diagnosis
| Time point post- HCT
| Other sites of GvHD
| Symptoms of genital GvHD
| |
1
| Hodgkin lymphoma then MDS
| Unknown; sirolimus, and steroids | 8 years
| sclerotic skin eyes, mouth, esophageal strictures
| inability to have intercourse
| |
2
| AML
| BuCy2 conditioning; no GVHD prophylaxis
| 2 yr 4 mo
| BOS
| dyspareunia
| |
3
| Ewing sarcoma then AML
| Cy/TBI conditioning; tacrolimus, cellcept, prednisone
| 1 yr 11 mo
| Sclerotic skin
| dyspareunia
| |
4
| myelofibrosis
| Fludarabine/busulfan conditioning; tacrolimus, prednisone, pomalidamide
| 5 yr 9 mo
| sclerotic skin, eyes, mouth, joint and gyn | dyspareunia
| |
5
| Sickle cell anemia
| 300 rads TBI, alemtuzumab; sirolimus
| 4 yrs
| none
| none
| |
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