A Rare Case of Hematometrocolpos due to Transverse Vaginal Septum along with Distal Vaginal Atresia in an Adolescent Girl

Abstract

Primary amenorrhoea due to Müllerian malformations is rare, with 1 in 4500 cases and 2%–8% of cases presenting as infertility. Obstructive Müllerian anomalies present as hematometra and hematocolpos during puberty. Timely surgical intervention is required to relieve acute pelvic pain and restore functional anatomy. A 15-year-old girl presented to OPD with complaints of severe pain in her lower abdomen and lower back for the last 2–3 weeks, not relieving on medication. She has not attained menarche and has been having cyclical pain and low backache for 7–8 days every month for the last year. Physical examination showed a suprapubic lump with vaginal agenesis. Magnetic resonance imaging revealed hematometrocolpos due to transverse vaginal septum and distal vaginal atresia. Pull-through vaginoplasty along with complete excision of transverse vaginal septum was performed. Vaginal dilator therapy was done after the healing of the sutures. In follow-up, the patient attained menstruation with a patent vagina. Obstructive Müllerian anomalies should be identified early by detailed clinical examination and targeted investigations to prevent long-term morbidity and infertility.

Introduction

Hematometra and hematocolpos are the presenting features of vaginal obstruction. Causes can be classified as Congenital Mullerian malformations, such as imperforate hymen, transverse vaginal septum, distal vaginal agenesis, and obstructed hemivagina with ipsilateral renal anomaly or acquired due to infection and trauma.[1] These cases present at puberty as primary amenorrhoea, dysmenorrhoea, pelvic pain, recurrent vaginal discharge, or infertility.[2]

Primary amenorrhoea is defined as the absence of menses at the age of 15 years in the presence of secondary sexual characteristics. Clinical evaluation of primary amenorrhoea should be started as early as 13 years in the absence of secondary sexual characteristics.[3]

Primary amenorrhoea due to Müllerian malformations is a rare occurrence with an incidence of 1 in 4500 cases and 2%–8% of cases present as infertility.[4,5]

During embryological development, the upper and lower parts of the vagina are derived from the Müllerian ducts and sinovaginal bulbs, respectively. The sinovaginal bulb is formed by urogenital sinus, which forms the lower third of the vagina by 17–18 weeks of gestation.[6]

Transverse vaginal septum is formed due to the failure of tissue resorption between the sinovaginal bulb and the caudal tip of the fused Müllerian ducts.[7] The prevalence of transverse vaginal septum is reported to be 1 in 30,000 to 1 in 84,000 females.[8]

The most common location of the transverse septum is the upper vagina (46%), followed by the mid vagina (35%) and the lower vagina (14%).[9]

The prevalence of vaginal agenesis has been reported to be 1 in 4000 to 1 in 10,000 females.[10] Vaginal agenesis is classified as complete, proximal, and distal agenesis, with the latter, estimated to be 5% of the total number.[11]

We report a rare case of hematometrocolpos due to transverse vaginal septum with distal vaginal atresia in a 15-year-old adolescent girl.

Case Report

A 15-year-old girl presented to OPD with complaints of severe pain in lower abdomen and lower back for the last 2–3 weeks, not relieving on medication. She has not attained menarche and is having cyclical pain and low backache for 7–8 days every month for the last 1 year. On examination, her secondary sexual characteristics were well-developed, there was a soft to firm, globular swelling noted in suprapubic region with mild tenderness. External genitalia showed a blind vagina with a shallow dimple at the level of hymen without bulge.

Abdominal ultrasound showed hematometrocolpos with normal findings in the upper abdomen. Magnetic resonance imaging (MRI) of whole abdomen and pelvis confirmed the finding of hematometrocolpos as hyperintense collection in the endometrial cavity and vagina with obstruction and atresia of distal portion of the vagina [Figure 1a]. The patient was managed by surgical procedure, which involved creating neovagina in the distal atretic portion by pull-through technique till we reached up to the mid-transverse vaginal septum, which was bulging as a balloon; a transverse incision was given; and approximately 400 cc of brownish colored fluid, which was collected menstrual blood, was drained. Resection of the septum was done, and the edges were sutured with the hymenal ring. This procedure did not required graft because the atretic portion of the vagina was less than 3 cm [Figure 1b]. Postoperatively, a mould made of 10 cc syringe without plunger, covered with gauze and condom was put as dilator and changed intermittently by her mother after the sutures get healed. In follow-up, the patient attained normal menstrual flow with a patent vaginal canal.

Figure 1.

(a) Blue arrow—hematometra; Red arrow—hematocolpos; Green arrow—transverse vaginal septum; and Yellow arrow—distal vaginal atresia. (b) Postoperative image of reconstructed vagina

Discussion

Obstructive Müllerian anomalies generally present after puberty as amenorrhoea, pelvic pain, urinary frequency, or urinary retention. A digital rectal examination can be helpful in delineating the size of the uterus, cervix, the hematocolpos, and the distance of the level of obstruction from the introitus. MRI is the preferred imaging modality to confirm the findings. Management depends on the age of presentation and the symptoms. This is considered a surgical emergency in cases of severe pain, acute urinary retention, and mandate for definitive surgical procedures. There is a risk of ascending infection if simple incision and drainage are performed for hematocolpos. These conditions are commonly associated with vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb anomalies.[2]

In adolescents with nonurgent condition, menstrual suppression can be given in the form of progesterone-only pills, injections, combined oral contraceptive pills, or gonadotropin-releasing hormone agonists.[12] A pull-through vaginoplasty with an interposition graft is done if the length of the proximal vagina is 3 cm or more from the introitus. Complete excision of the transverse vaginal septum and anastomosis of proximal vagina to the distal vagina by using the Z-plasty technique is performed to increase the length of vaginal flaps. Postoperative care will require placement of a vaginal stent or mould, bed rest with indwelling urinary catheter, and antibiotics. Dilator therapy should be initiated after the healing of the graft or suture line, and follow-up should be done every 1–2 weeks till the pain is relieved.[13]

Conclusion

Obstructive Müllerian anomalies pose a challenge in the diagnosis and management of adolescent patients. Evaluation is best carried out by thorough medical history, physical examination, and imaging to understand the structural anomaly and to plan the definitive procedure. Preoperative counselling of patients and family members and postoperative care is required to obtain the best long-term results.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.