Tubal replacement of cervix for treatment of type II vaginal atresia complicated with cervical dysplasia: a case report

Abstract

Vaginal atresia is a rare obstructive disease of the reproductive tract. It is characterized by the absence or underdevelopment of the vaginal canal and results in various clinical manifestations. Hysterectomy can physically and mentally burden young female patients with a congenital cervix and complete vaginal atresia. This report presents a case of type II vaginal atresia complicated by cervical dysplasia in a female patient >10 years of age. Our team opted to preserve the patient’s uterus, innovated a fallopian tube transplantation technique, and performed cervicovaginal reconstruction using natural channels instead of the cervical canal. The patient experienced menarche within the first 2 weeks postoperatively, and follow-up at 6 months revealed no abnormalities.

Introduction

Vaginal atresia is an obstructive disease of the genital tract characterized by the absence or underdevelopment of the vaginal canal. The vagina is partially or completely replaced by fibrous tissue, forming a complete or partial obstruction. Vaginal atresia can be either congenital or acquired, with congenital cases being more common in clinical settings. Female reproductive tract malformations are relatively rare, with an incidence of approximately 1/4000 to 1/10,000, and distal atresia accounts for only 5% of all cases.^1,2 Typically, patients have normal or partially normal ovarian, uterine, and endometrial function. During puberty, the endometrium undergoes periodic shedding; however, vaginal atresia obstructs menstrual blood flow, leading to clinical symptoms such as amenorrhea, abdominal pain, and endometriosis. ³ Surgery is the only method of treatment, with the main objectives being to relieve symptoms, restore anatomical integrity, enhance sexual function, and preserve fertility.^1,2,4 However, the perineal operation field is small and the risk of postoperative adhesion is high, making surgical repair a significant challenge. ³ Although surgical procedures for cervicovaginal hypoplasia have been explored, these procedures are complex, and no consensus or guidelines have been established regarding the surgical methods for patients with congenital cervicovaginal atresia.^5,6 Finding a minimally invasive treatment that can protect a patient’s reproductive function remains a significant challenge.

Our team successfully performed fallopian tube transplantation and vaginal reconstruction in a patient with congenital obstructive cervicovaginal dysplasia, type II vaginal atresia. The detailed analysis of this case can serve as a valuable reference for future treatment of patients with vaginal atresia. This study complies with the CARE guidelines. ⁷

Case report

General information

A young girl in her early teens was admitted to the hospital with a 2-year history of periodic lower abdominal pain. She had not menstruated since developing secondary sex characteristics. During the previous 2 years, she had experienced monthly episodes of lower abdominal pain, each lasting 3 to 7 days. The pain was not associated with fever, but its severity progressively worsened. The pain adversely affected her daily life and required oral analgesics. The patient was initially misdiagnosed and treated for chronic appendicitis with anti-inflammatory therapy at a local hospital, and her pain remained unresolved. A pelvic ultrasound at the local hospital indicated uterine effusion. During the abdominal pain episodes, she had no vaginal bleeding or fluid discharge.

At our hospital, magnetic resonance imaging suggested the possibility of vaginal atresia with cervical atresia, significant blood and fluid accumulation in the uterine cavity and cervical canal, a left chocolate cyst, massive fluid accumulation in the pelvic cavity, and a small right kidney. Pelvic ultrasound showed that the uterus measured 4.7 × 4.2 × 4.5 cm, the cervix was approximately 1.6 × 2.0 cm, and the upper section of the vagina was approximately 1.0 cm long. The distance between the upper blind end of the vagina and the body surface was approximately 4.9 cm. The right kidney measured 6.2 × 2.6 cm, while the left kidney measured 10.4 × 4.3 cm. Chromosome karyotype analysis of peripheral blood showed 46XX. Reproductive hormone test results were as follows: follicle-stimulating hormone: 7.09 mIU/mL, luteinizing hormone: 6.07 mIU/mL, prolactin: 17.25 ng/mL, estradiol: 43.87 pg/mL, progesterone: 2.36 ng/mL, and testosterone: 0.45 ng/mL. Other laboratory tests, including routine blood tests, blood biochemical markers, and tumor markers, showed no significant abnormalities.

The patient had undergone transabdominal teratoma exfoliation at 40 days of age after an abdominal mass was found. She had engaged in no sexual activity. During the examination, the patient exhibited a painful facial expression, held her hands over her abdomen, and assumed a bowed posture. A specialist examination revealed normal vulva development and a slight depression in the middle of the vestibular area with no opening or purple-blue mass. The uterus was uniformly full and normal in size, the cervix was empty without an obvious mass, and the vagina was completely atretic without a protruding mass (Figure 1). Based on the patient’s history, gynecological examination, and imaging findings, the diagnosis was congenital type II vaginal atresia combined with cervical atresia.

Figure 1.

Imaging findings and physical examination in the present case. (a) Vulval examination. (b) Ultrasound image and (c) Magnetic resonance image.

Treatment

Considering the patient’s young age, physical and mental health, and future fertility requirements, we recommend uterine-sparing cervical canal and vaginal reconstruction. After preoperative communication, the patient and her family agreed to the procedure. Our approach included traditional surgical methods and fallopian tube transplantation, using the natural channel to replace the cervical canal.

Laparoscopic exploration revealed that the uterine body was slightly enlarged, the right fallopian tube and ovary were adhered, the right fallopian tube fimbria was closed, and the ampulla was effused. The left fallopian tube appeared normal, and the left ovary had an endometriosis cyst. The cervix was empty, clearly stunted, and stringy (Figure 2).

Figure 2.

Laparoscopic direct view of the pelvis. (a) Right tubal atresia and (b) Empty cervix.

During the operation, the pelvic adhesions were separated, the anatomy was restored, and the left ovarian cyst was removed. A cavity was created along the rectal and urethral bladder space until it connected to the abdominal cavity. The right fallopian tube was freed from the tubal ligament and vessels, and the tubal isthmus and closed fimbria were trimmed and removed (Figure 3).

Figure 3.

Vaginoplasty and fallopian tube preparation. (a) Vaginoplasty and (b) Trimming of the free right fallopian tube.

The lower muscle layer of the anterior wall of the uterus was cut on the right side until the cervix was reached. The cervix was explored as a cord, and the cervical canal was bluntly separated using a vascular clamp. The vascular clamp was then reversed downward to pull the trimmed ampulla of the fallopian tube. The upper end was cut into the front and back of the uterus, and the vaginal end was trimmed again and sutured around the cervix (Figure 4).

Figure 4.

Surgical reconstruction of the cervix and vagina. (a) Blunt dilatation of the striated cervix. (b) Placement of the free, tipped fallopian tube into an artificial channel. (c) Fixing the fallopian tubes to the surrounding tissue and (d) Shaped artificial cervix and vagina.

The vestibular perineum and skin and mucous membrane of the inner thigh were freed, pulled, and pushed with a pedicle, then sutured to the outer cervix. The exposed part was covered with an amniotic membrane. A Foley catheter was inserted into the uterine cavity, and 4 mL of normal saline was injected. A vaginal mold was also inserted. The patient was discharged 1 week after surgery and menstruated approximately 2 weeks later. After menstruation, ultrasound and hysteroscopy were performed again, the Foley catheter was removed, and a lengthening tail T-ring was inserted into the uterine cavity to prevent cervical duct adhesion. A vaginal mold was placed in the vagina (Figure 5).

Figure 5.

Postoperative outcomes and stent placement. (a) One week after removal of the stitches from the vulvovaginal incision. (b) Ultrasound image 2 weeks postoperatively after the onset of menstruation. (c) Intrauterine placement of a T-ring stent to prevent adhesion of the artificial channel. (d) Appearance of the stent. (e) The end of the elongated tail filament reaches beyond the cervical opening and (f) Ultrasound image after stent placement.

Postoperative follow-up

The patient was followed up for 6 months after the operation and returned to normal life and studies. Approximately 2 weeks after the operation, the patient began to menstruate normally, with periods lasting 5 to 7 days. The menstrual blood flowed smoothly without dysmenorrhea. During follow-up visits at 1 month, 3 months, and 6 months postoperatively, physical examination showed that the vaginal depth was 7 to 10 cm, capable of accommodating two fingers. The vaginal wall mucosa was smooth, pink, and had good elasticity. There were no abnormalities in the uterus or bilateral adnexa. Ultrasonography performed 1 month after surgery also showed no abnormalities in the uterus and bilateral adnexa.

Discussion

Our team innovatively transplanted the fallopian tube, which had lost its functional value because of severe hydrops caused by the backflow of menstrual blood, to the lower end of the uterus after rehabilitation to replace the cervical tube. Although the patient had secondary sex characteristics before surgery, she had no menstruation, experienced periodic severe abdominal pain, had limited daily activities, and required pain management. The patient also suffered from anxiety and stress due to amenorrhea and pain, adversely affecting her academic performance and social life. After the surgical treatment, the patient experienced menarche approximately 2 weeks postoperatively. Her menstrual cycle normalized, dysmenorrhea remained absent, her quality of life significantly improved, and her anxiety and mental health were restored. No abnormalities were found during postoperative follow-up.

Patients with vaginal atresia should undergo surgery as soon as possible, with the primary goal of establishing a functional vaginal opening.^8,9 Most experts recommend cervical drilling, utero-vaginal anastomosis, and McIndoe surgery. Although these methods can successfully treat cervicovaginal atresia, the risk of secondary cervical stenosis after surgery is as high as 40% to 60%.^9,10 The specific procedure depends on the severity of the condition and individual patient factors and may include vaginal dilation, vaginoplasty, tissue transplantation, and the use of tissue dilators. Drugs alone do not treat vaginal atresia, but hormone therapy may be prescribed in some cases to temporarily control the menstrual cycle until surgical correction can be performed. ¹¹ Treatment should be individualized according to the specific situation of the patient.^12,13 Long-term fertility assessment is ongoing.

The main manifestations of congenital vaginal atresia in women are primary amenorrhea and periodic abdominal pain. The onset time and degree of abdominal pain vary among patients with different types of vaginal atresia. Patients with type I congenital vaginal atresia generally have better endometrial function, resulting in earlier and more severe abdominal pain. By contrast, patients with type II congenital vaginal atresia combined with cervical atresia have poorer endometrial function, leading to later-onset and relatively milder abdominal pain. Evaluating patients with suspected obstructive uterovaginal dysplasia requires a complete history, physical examination, and imaging. Accurate diagnosis is essential for proper management. Good clinical outcomes depend on thorough disease assessment, understanding the developmental abnormality, adequate preoperative counseling, meticulous surgical intervention, and long-term postoperative management.

In this case, the patient had an early age of onset and a well-developed uterus without malformations. Ultrasonography, magnetic resonance imaging, and CA125 measurement suggested a diagnosis of type II vaginal atresia, cervical dysplasia, and endometriosis. The surgery was challenging because of the atresia of the entire vagina, the small perineal surgical field, and the close proximity of the rectum and urinary system. ² Vaginoplasty, cervicoplasty, and cervicovaginal penetration surgery were necessary. The main difficulties of this surgical protocol included cervical reconstruction and anastomosis. There was no palpable mass in the cervix during the gynecological examination, and imaging indicated cervical dysplasia and immature tissue development. The direction of vaginal and cervical connection separation was difficult to determine. The urethra, bladder, and rectum are easily damaged during surgery. Furthermore, connecting the cervix to the vaginal cavity is challenging because of the narrow surgical field, tough atretic cervical tissues, and lack of columnar epithelium to protect the cervix after incision, making postoperative cervical adhesion and atresia likely to occur. Such cases require reoperation or even uterine resection.^14,15 Maintaining support after cervical plasty is crucial, often requiring stents and cervical hysterovaginal anastomosis. Cervical resection with artificial vaginal access is prone to infection and re-adhesion. Communicating the risks associated with injury, infection, adhesions, reoperation, and uterine removal in combination with endometriosis to the family is essential.

The longer the history of the disease, the more severe the symptoms of endometriosis and adenomyosis, leading to poorer surgical outcomes. However, patients with type II disease are often young and still have some time before childbearing age, during which the risk of surgical upstream infection and cervical re-stenosis is high. Fujimoto et al. ¹⁰ found that the success rate of conservative surgery for patients with normal vaginal development and cervical atresia alone was 68%, whereas the success rate for patients with vaginal atresia combined with cervical atresia was only 43% and was associated with significantly higher incidences of postoperative infections and cervical re-stenosis.^9,16 The treatment goal for patients with congenital vaginal atresia is to relieve the obstruction of the uterine outflow tract, restore the normal anatomical structure, and preserve reproductive function. As diagnostic and treatment capabilities improve, the original plan of removing the uterus can be replaced by a plan to preserve it, warranting increased attention from specialists.^9,17

The vaginal opening operation for type II vaginal atresia is particularly difficult. The patient is young and underdeveloped, with brittle and elastic tissue, making the direction challenging to master. The urethral-rectal space is narrow and tight, making separation difficult, and the urethra, bladder, and rectum are easily damaged. Even if the vaginal tunnel is opened, it is prone to adhesion because of the difficulty in covering it with mucous membranes. Poorly developed cervices make connecting the cervix to the vagina challenging, often resulting in adhesion and atresia,^18–20 increasing the risk of infection, and requiring surgery through the uterine cavity and vagina. Postoperatively, long-term cervical stenting is necessary to prevent adhesions. This situation often presents a medical dilemma due to infection, adhesions, and atresia, leading to another surgery ending in hysterectomy. The first operation is crucial and must ensure continuous patency of the reproductive tract and related physiological functions, preventing scarring and stenosis.

Patients require close postoperative care and observation. They are prone to infection after vaginal surgery, leading to cervical narrowing and blockage that necessitate a second operation or hysterectomy. Postoperative measures should prevent cervical tube adhesion and maintain the patency of the artificial vagina. ²¹ All patients with obstructive uterovaginal dysplasia are at high risk of developing endometriosis and adenomyosis. Patients with obstructive septal or distal atresia are at risk of postoperative restenosis and infection, requiring long-term follow-up and proactive fertility problem-solving. Complete disease assessment, an understanding of developmental anomalies, application of superior surgical resources, adequate preoperative counseling, thorough surgical interventions, and comprehensive humane and individualized long-term postoperative management are essential to achieve optimal long-term outcomes in patients with obstructive uterovaginal anomalies.^22,23

Conclusion

Congenital vaginal atresia and cervical atresia require early detection and treatment. Preoperative examinations should be thorough, and surgical plans should be tailored to the individual patient’s condition. During surgery, it is crucial to assess uterine lesions, uterine volume, and fallopian tube patency to determine whether to preserve the uterus. Postoperative care should focus on preventing cervical and vaginal restenosis and occlusion. Preservation of the uterus and fertility is vital for patients, with the primary treatment goals being to remove obstructions, restore normal anatomy, and preserve fertility.

Supplemental Material

sj-pdf-1-imr-10.1177_03000605241272532 - Supplemental material for Tubal replacement of cervix for treatment of type II vaginal atresia complicated with cervical dysplasia: a case report

Supplemental material, sj-pdf-1-imr-10.1177_03000605241272532 for Tubal replacement of cervix for treatment of type II vaginal atresia complicated with cervical dysplasia: a case report by Xin Jin, Sicong Liu, Nannan Wang, Nana Liu and Guilin Li in Journal of International Medical Research