Autologous Buccal Mucosa Vaginoplasty in 22 Patients with Congenital Adrenal Hyperplasia
Anjali Nambiar, BS1, Michelle K. Arevalo, BS1, Gwen Grimsby, MD2, Ellen Wilson, MD2, Linda A. Baker, MD2.
1University of Texas Southwestern Medical Center, Dallas, TX, USA, 2Children's Health, Dallas, TX, USA.
Background: Reconstruction of the urogenital sinus (UGS) in females with congenital adrenal hyperplasia (CAH) is most challenging in patients with intermediate and high UGS at birth or in those with postsurgical persistent urogenital sinus or vaginal stricturing later in life. Several surgical techniques have been used, some which may sacrifice final vaginal length and female genital cosmesis. We report medical-surgical and patient-centered outcomes in 22 patients with CAH who underwent reconstruction employing autologous buccal mucosa vaginoplasty (ABMV) at our single institution.
Methods: With IRB approval, a retrospective chart review was performed on all CAH patients undergoing primary UGS surgery or secondary postsurgical repair via ABMV from 2004-2017. Demographics, pre-operative patient history, patient anatomy, operative procedures, and surgical outcomes including serial vaginal calibrations were collected from the medical record. Patient-centered outcomes were obtained via non-validated questionnaires completed either over telephone or in office.
Results: Twenty-two patients underwent ABMV at a median age of 15 years (IQR: 11 – 19, Table 1). Median clinic follow up was 14 months (IQR: 6 – 28); none were lost to follow up. Nine vaginoplasties were primary (no previous perineal surgery) for intermediate or high UGS. Thirteen were secondary (stenosis: 13, persistent urogenital sinus: 3). Buccal was harvested from the lower lip (n=3), unilateral (n=6) or bilateral (n=13) cheek. Concomitant procedures with ABMV are detailed in Table 1. No intraoperative complications occurred. Perioperative complications included prolonged recovery from epidural-induced foot neuropathy (n=1). Post-ABMV stricturing developed in 3/9 primary and 0/13 secondary cases (p = 0.054). Strictures occurred at the introitus (n=1, 4.5%) requiring revision after failed home dilation or as an anastomotic hourglass deformity (n=2, 9%), one severe enough to prevent successful intercourse. Two patients had oral complaints following buccal harvest (cheek tightness which resolved spontaneously n=1 and buccal mucosa web requiring minor division n=1). No patients experienced new urinary incontinence postoperatively. Excluding pre-pubertal girls, mean vaginal length was 9.1 cm ± 1.7 cm and mean vaginal diameter was 2.7 ± 0.9 cm at a median of 350 days follow up. Questionnaires were completed in 55% of patients at median follow-up of 32 months (IQR: 6 – 72). Patients reported high subjective satisfaction with their surgery (mean: 4.0/4.0 scale), and 5/7 patients attempting intercourse achieved successful, pain-free penetration (Table 2). One patient achieved pregnancy.
Conclusions: ABMV is a safe, effective procedure in females with CAH that results in excellent vaginal size, high levels of patient satisfaction and low revision rate in both primary and even secondary complex vaginoplasty.
| Table 1. Patient Demographics | |
| Age at surgery (years) | 15 (IQR: 11 - 19) |
| Median Follow-up (months) | 14.2 (IQR: 6.0 - 28.5) |
| Pubertal Stage at Surgery | |
| Pre-pubertal (Tanner 1) | 4 |
| Pubertal (Tanner 2-4) | 13 |
| Post-pubertal (Tanner 5) | 5 |
| Race | |
| White/Caucasian | 8 |
| Black/African American | 4 |
| Hispanic | 9 |
| Other | 1 |
| Type of Vaginoplasty | |
| Primary Vaginoplasty | 9 |
| Secondary Vaginoplasty | 13 |
| Mean Urogenital Sinus Length (cm) | |
| Primary Repair (n = 9) | 3.5 ± 0.8 |
| Recurrent UGS Repair (n = 3) | 1.2 ± 1.0 |
| Concomitant Procedures | |
| Clitoroplasty | 13 |
| Labioplasty | 12a |
| Female urethroplasty | 13 |
| Perineoplasty | 3b |
| a3 had buccal mucosa labioplasty b1 had buccal mucosa perineoplasty | |
| Table 2. Surgical and Patient-Centered Outcomes | |
| Peri-Operative Complications | 1a |
| Post-Operative Complications | 3 |
| Hourglass deformity | 2 |
| Introital Stenosisb | 1 |
| Post-Operative Vaginal Caliber | |
| Length (cm) | 9.1 ± 1.7 |
| Diameter (cm) | 2.7 ± 0.9 |
| Subjective Patient Satisfaction (4.0 Scale) | |
| Appearance | 3.7 |
| Overall Satisfaction with Surgery | 4 |
| Patients Achieving Intercourse | 5/7 (71%) |
| aepidural-induced neuropathy | |
| brequired revision | |
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