Herlyn-Werner-Wunderlich Syndrome Complicated with Pyocolpos: An Unusual Cause of Postabortal Sepsis.

Obstructive mullerian anomalies give rise to a spectrum of clinical presentations and are uncommon in routine gynecologic practice. The patient usually becomes symptomatic in early reproductive years. Recurrent pelvic pain, dysmenorrhea, enlarging abdominopelvic mass, and abnormal vaginal discharge are the common presenting symptoms. We describe a rare case of a mullerian anomaly getting diagnosed 13 years after attaining menarche during the evaluation of postabortal sepsis. Patient presented 2 weeks following evacuation carried out for missed abortion, with acute abdominal pain, fever and foul smelling discharge per vaginum. The anomaly was identified as uterus didelphys with obstructed left hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) complicated by pyocolpos. She was successfully managed by single-stage transvaginal septum resection under laparoscopic control.

INTRODUCTION

Uterus didelphys with obstructive hemivagina and ipsilateral renal anomaly is a rare congenital abnormality of the female urogenital tract.[1] It is difficult to estimate the exact incidence of obstructive Mullerian anomalies but is reported to be around 0.1-3.8%.[2] The anomaly is difficult to diagnose due to its variable clinical presentation and variations in the age at the time of diagnosis. It may go undetected in the early reproductive years and may come to light after commencement of sexual activity or when childbearing is attempted. The time of diagnosis and clinical presentation can be influenced by the level and degree of completeness of the uterovaginal obstruction.

CASE REPORT

A 26-year-old nulliparous lady presented to the gynecology department with lower abdominal pain, fever, and foul smelling discharge per vaginum since 2 days. She had undergone dilatation and evacuation for missed abortion 2 weeks back. It was a spontaneous conception, 2 years following marriage, and early pregnancy evaluation at 6 weeks revealed a viable singleton pregnancy in the right horn of a bicornuate uterus and a 9 cm × 7 cm cystic lesion with internal echoes in the region of left adnexa, posterior to urinary bladder. Repeat scan at 8 weeks revealed absent cardiac activity and evacuation was carried out at a local hospital. Immediate postabortal period was uneventful.

She was febrile with significant tachycardia and no pallor. Abdominal examination revealed tenderness in left iliac fossa and hypogastrium with no palpable mass. Vulval inspection revealed asymmetry at the introitus with a bulge on the left side and unhealthy vaginal discharge. Swab was taken for C and S. Per speculum examination not carried out due to severe tenderness. On bimanual examination cervix felt high-up and to the right side, cephalad and posterior to a tender cystic mass of about 10 cm × 8 cm on the left side, uterine body normal in size, and tender on palpation. Lab parameters showed normal Hb, TLC – 12,500 with neutrophilia, and highly elevated CRP. USG was reported as uterus didelphys (as the uterine horns were widely separated) with a large collection of 10.3 cm × 6.8 cm with pockets of air seen on the left side, posterolateral to the bladder, normal ovaries and absent left kidney. An obstructive mullerian anomaly was suspected and MRI was carried out to confirm the same [Figures 1a–c].

Figure 1a

MRI pelvis (axial image) – Uterus didelphys, normal endometrial cavity, normal ovaries, with large collection in left hemivagina

Figure 1c

Coronal image – Collection compressing the normal patent vagina on right side (open arrow)

Open arrow showing communication of collection with left uterine body

Patient was started on antibiotics and planned for surgery. Local examination findings were reconfirmed before starting the procedure. Left lateral vaginal mass was palpated and Foley's catheter placed to define the limits of the bladder. There was a tiny fistulous opening on the vaginal septum 4 cm above the hymenal ring. Diagnostic laparoscopy was carried out [Figures 2a and b]. The extent of the left paravaginal mass noted and limits of the bladder were identified [Figure 3]. Cruciate incision was made transvaginally, at the site of the tiny fistulous opening on the longitudinal vaginal septum, and around 200 cc of foul smelling, frothy, purulent material drained. Specimen was sent for C and S. Digital palpation through the cruciate incision helped in defining the extent of the vaginal septum. It was excised using electro cautery until the left cervix was reached. Fine absorbable sutures were placed on the resected septum to attain hemostasis. Post-operative period was uneventful and she conceived 3 months after surgery.

Figure 2a

Laparoscopic view – Both uterine bodies with adnexa are normal. No pelvic collection or purulent exudate through the tubes

Figure 2b

Posterior view of the collection

Figure 3

Anatomical limits of collection identified in relation to the bladder

DISCUSSION

The specific association of uterus didelphys, obstructed hemivagina with ipsilateral renal anomaly is well recognized.[3] The triad has been described as Herlyn-Werner-Wunderlich syndrome.[45] More recently, the acronym Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is used to describe patients with OHVIRA, that is, occurrence of two out of the three components of the triad.[6] It allows inclusion of uterine anomalies other than uterus didelphys, like septate or bicornuate uterus, which are estimated to occur in about 20% of such cases.

Uterus didelphys results due to complete failure of lateral fusion of the paramesonephric ducts. Incomplete fusion and resorption leads to bicornuate or septate uterus, which are common accompaniments in OHVIRA syndrome. The occurrence of partial or complete vaginal septum is due to vertical fusion defect of paramesonephric ducts with the urogenital sinus. Associated arrest in the development of the metanephric ducts leads to a variety of renal tract abnormalities. These include renal agenesis with or without agenesis of the ureter, duplication of the ureters, or multicystic dysplastic kidney.[7]

The obstructed hemivagina leads to development of hematometrocolpos and secondary effects of reverse menstruation, such as endometriosis, and pelvic adhesions subsequently. These may have a negative impact on future fertility. Recurrent cyclic abdominal pain, dysmenorrhea, enlarging lower abdominal mass,[3] coital difficulty, and abnormal vaginal discharge are various presenting symptoms. The pocketed vaginal collection with degenerated blood products can get secondarily infected giving rise to symptoms of pelvic inflammatory disease.[2] The patient often gives history of regular normal menstrual cycles and can be asymptomatic for years after menarche.[8] Diagnosis can therefore be elusive and requires high degree of clinical suspicion.

Among the imaging modalities, MRI is considered as the gold standard in the diagnosis of Mullerian anomalies.[2] The multiplanar imaging helps to delineate the uterovaginal anatomy with high accuracy.[7] In addition, it aids in identifying associated tubo-ovarian pathologies, characterizing the thickness and location of the septum, the type of collection in the obstructed hemivagina, and associated malformations of the urinary tract, which commonly coexist with Mullerian anomalies.

The treatment is aimed at the complete resection of the vaginal septum and establishing continuity of the obstructed side. Transvaginal resection can be performed either as a single or a double-stage procedure.[6] Laparoscopy prior to resection helps to rule out additional adnexal pathology and confirm the diagnosis if preoperative MRI is not feasible. Resection under laparoscopic control also helps to avoid or detect an inadvertent bladder injury as the paravesical anatomy is distorted in such cases. Hysteroscopic vaginal septum resection under transabdominal ultrasound guidance is preferable in young females in order to preserve the hymenal integrity.[9] The surgical outcome is excellent and associated with successful reproductive performance in future life.[2410]