237 Early Diagnosis of Labial Fusion Enables Medical Treatment in an Office Setting

Track: Poster Abstracts
Wednesday, February 11, 2015, 6:45 PM-7:45 PM
Grand Hall CD (Manchester Grand Hyatt)
Claire Scrivani, BS , NHLBI, NIH, Bethesda, MD
Melissa Merideth, MD , National Human Genome Institute, Bethesda, MD
Tajana Klepac Pulanic, MD , Eunice Kennedy Shriver National Institute of Child Health and Human Development, Program in Reproductive and Adult Endocrinology, Bethesda, MD
Steven Z. Pavletic, MD , Experimental Transplantation and Immunology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD
Richard W. Childs, MD , Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, MD
Matthew Hsieh, MD , National Institutes of Health, Bethesda, MD
Pamela Stratton, MD , Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD
Presentation recording not available for download or distribution as requested by the presenting author.

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

 

Disclosures:
Nothing To Disclose