Case report and literature review of patient with neovagina stricture complicated by high-grade dysplasia

Stats:

• 17-year-old female featured in case study
• No. 1 center in the nation for pediatric GI surgery
• World’s largest database of patients with congenital colorectal and urogenital conditions

Case background: cloaca and ulcerative colitis of the neovagina

Cloaca is the fusion of the vagina, urethra and rectum into a common channel that opens on the perineum. Channel lengths can vary from 1 to 10 cm, with longer channels often requiring a vaginal replacement along with surgical repair of the cloacal abnormality. The rectum, colon and small bowel can be used for vaginal replacement.

Colon vaginoplasty complications include:

• Introital stenosis
• Vaginal prolapse
• Wound dehiscence
• Hematoma
• Infection
• Ulcerative and diversion colitis (presents with blood discharge)
• Premalignant and malignant neoplasms of colonic neovagina (rare)

It has been suggested that patients with ulcerative colitis of the neovagina are at increased risk of malignancy. However, of the several cases reported in medical literature, the authors could not find any that had a history of ulcerative colitis.

Pediatric surgeons and researchers at the International Center for Colorectal and Urogenital Care at Children’s Hospital Colorado presented a clinical case report of an adolescent female patient with dysplasia in a colonic neovagina with recurrent neovaginal stenosis and history of ulcerative colitis.

Case overview: review of patient with colon vaginoplasty and history of ulcerative colitis

When she was 14, the patient presented with worsening abdominal pain at the same time she was diagnosed with a large hematometrocolpos (blood-filled distended uterus and vagina). The cervix appeared patent with a focal fluid collection inferior to the uterus consistent with a stenotic neovagina.

The patient’s pain was significantly reduced after receiving progestin to suppress menstruation and the placement of uterine drain, which evacuated approximately 700 ml of blood and decompressed the uterus.

She then underwent a vaginoplasty with buccal mucosa graft to create a patent vagina for management, with the following occurring after surgery:

• Vaginal stent removed after two weeks, then began vaginal dilation
• Proximal vagina narrowing noted at follow-ups
• Vaginal bleeding (thought to be breakthrough bleeding from menstrual suppression)
• Began vaginal douches due to diversion colitis concern
• Vaginal stenosis progressed
• Abdominopelvic pain, with labs and imaging suggesting pelvic inflammatory disease
• Required inpatient admission with IV antibiotics

The patient ultimately underwent exploratory laparotomy, hysterectomy, vaginectomy, and neovagina creation (resected in segments) with ileum. Pathology identified polypoid low-grade dysplasia with focal high-grade lesion in the background of pronounced chronic colitis, for which the patient will receive long-term surveillance.

Case discussion: vaginal stenosis an uncommon symptom of neovagina colitis

This patient’s case demonstrates the risk of premalignant and malignant lesions in colonic neovaginas affected by colitis. The patient had a personal history of ulcerative colitis, possibly caused by neovaginal ulcerative colitis and diversion colitis.

Inflammatory changes of the colonic neovagina are common, and the majority of changes are asymptomatic. Patients with diversion colitis may have mucoid vaginal discharge, bleeding and pain. Inflammatory bowel disease (IBD) can also occur, with symptoms similar to diversion colitis. Since colorectal cancer risk increases with IBD, a neovagina could increase risk of premalignant and malignant lesions.

Medical literature reports several cases of adenocarcinoma in colonic neovaginas, diagnosed three to 53 years after vaginoplasty and the majority presented with vaginal bleeding. Typical symptoms of colitis are pain, bleeding and discharge, but the primary symptom of the patient in this case was recurrent vaginal stenosis.

Key recommendations from report authors:

• Be aware of long-term complications after reconstruction in patients with cloaca history
• Patients with a sigmoid neovagina require lifelong surveillance and evaluation
• Consider medical management for patients with symptomatic colitis
  • severe cases may require neovaginectomy
  • dysplasia and adenocarcinoma require surgical resection
• If no concern for recurrent or residual dysplasia after neovaginectomy, consider long-term surveillance with serum tumor markers and imaging