Abstract
Introduction:
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is defined by a Müllerian anomaly with a unilateral obstructed hemivagina and associated renal anomaly. 1 –3 While most reported cases have ipsilateral renal agenesis, anomalies may include duplicated or ectopic ureters and dysplastic or polycystic kidneys. 1,3 Patients typically present after menarche with unilateral pain due to hematocolpos on the obstructed side. However, presentation early in life with hydrocolpos has been described. Patients typically undergo a staged procedure with placement of a transvaginal drain or a single procedure to resect the obstructing vaginal septum. 1,3 However, vaginoplasty can be technically challenging in infancy, and minimally invasive vaginoscopic resection of the septum has been proposed. 4 –9 Here, we present the case of a female born at 37 weeks with prenatally diagnosed hydrocolpos and suspected OHVIRA syndrome. Multiple drain placements resulted in drain malfunction and reaccumulation of hydrocolpos despite robotic nephrectomy at 3 months of age, and the decision was made to proceed with vaginoscopic incision of the vaginal septum at 7 months of age.
Materials and Methods:
A 7.9 French pediatric cystoscope was placed into the vaginal introitus. With compression of the bilateral labia majora, the vagina was distended with warm sterile water. The previously placed vaginostomy tube was identified entering the inferior aspect of the vaginal septum on the left. A guidewire was placed through the vaginostomy tube, and the tube was removed. The cystoscope was passed through the prior vaginostomy site, and the left cervix was visualized. The cystoscope was then withdrawn, and the Storz infant resectoscope with a Collin’s knife was placed within the introitus. With care taken to avoid injury to the anterior and posterior vaginal walls, the resectoscope was used to incise the vaginal septum longitudinally and extend the incision cephalad. Monopolar electrocautery at 15 watts was used. The vagina was inspected intermittently to identify the upper and lower limits of the septum. A patulous opening was created between the two hemivaginas, and the left cervix was visualized. To assess whether an indwelling drainage catheter was necessary, a 10 French council tip catheter was placed over the guide wire into the left hemivagina and inflated to 3 mL, or a diameter of 2 cm. Vaginoscopy revealed that the Foley balloon easily passed through the incision with minimal tension. No indwelling drain was deemed necessary.
Results:
Repeat ultrasound 6 months post-procedure demonstrated no reaccumulation of hydrocolpos, and the patient continues to do well.
Conclusions:
Vaginoscopic resection of an obstructing vaginal septum is a minimally invasive, simple, and feasible approach in the infant with OHVIRA and clinically relevant hydrocolpos. Early diagnosis and treatment can prevent future complications.
Authors have received and archived patient consent for video recording/publication in advance of video recording of procedure.
No funding was required for production of this video.
The authors have no potential conflicts of interest to disclose.
Statement of prior presentation:
Findings were reported as a video presentation at the PCPLC Annual Meeting, Atlanta, GA, November 2024.
Runtime of video:
5 mins 0 secs