What is the best course of treatment for a patient with uterine didelphys, unilateral hematocolpos and hematometra, and unilateral renal (kidney) agenesis?

Surgical Management of Uterine Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis

The definitive treatment is complete excision of the vaginal septum to drain the hematocolpos and hematometra, which should be performed as soon as the diagnosis is confirmed to prevent long-term complications including endometriosis, pelvic adhesions, and infertility. 1, 2

Diagnostic Confirmation

Before surgical intervention, confirm the complete anatomic picture:

• Pelvic MRI is essential to delineate the exact anatomy of both uterine horns, identify the obstructed hemivagina, confirm hematocolpos/hematometra, and assess for associated complications 1
• Renal ultrasound to document ipsilateral renal agenesis, which occurs in virtually 100% of these cases 3, 2
• Laparoscopic evaluation should be strongly considered at the time of surgery to assess for endometriosis, pelvic adhesions, and tubal patency, as retrograde menstruation through the obstructed system commonly causes these complications 4, 2

Surgical Approach: Three Viable Options

Option 1: Vaginoscopic/Hysteroscopic Resection (Preferred for Adolescents)

This minimally invasive approach preserves the hymen and provides excellent visualization 5:

• Perform vaginoscopic approach under continuous ultrasonographic guidance 5
• Use resectoscopic loop to incise the vaginal septum at the point of maximal bulging corresponding to hematocolpos 5
• Continue resection until complete or near-complete excision of the septum is achieved 5
• Advantages: Preserves hymenal integrity, minimal tissue trauma, excellent drainage, low morbidity 5

Option 2: Combined Laparoscopy and Vaginoscopy

This approach is superior when vaginoscopic visualization alone is inadequate 4:

• Allows simultaneous evaluation and treatment of endometriosis, pelvic infection, and adhesions 4
• Provides direct visualization of both uterine horns and tubes to assess fertility potential 4
• Enables creation of adequate drainage under direct vision 4

Option 3: Traditional Vaginal Septum Excision with Laparotomy

Reserved for complex cases or when minimally invasive approaches are unavailable 1, 2:

• Perform complete excision of the vaginal septum via vaginal approach 2
• Laparotomy allows direct assessment of both uterine horns and treatment of endometriosis 1, 2
• Avoid Strassman metroplasty unless specifically indicated, as simple septum resection provides optimal outcomes 2

Critical Surgical Principles

The key to success is complete septum excision, not partial incision 5, 2:

• Incomplete resection leads to recurrent obstruction and symptom recurrence 2
• The communication between the two vaginas must be large and permanent 5
• Drainage must be confirmed intraoperatively by ultrasound showing resolution of hematocolpos and hematometra 5

Management of Associated Endometriosis

Conservative surgical treatment of endometriosis at the time of septum resection is recommended 2:

• Endometriosis occurs frequently due to retrograde menstruation through the obstructed system 2
• Ablate or excise visible endometriotic implants conservatively 2
• Avoid aggressive surgery that might compromise future fertility 2

Common Pitfalls to Avoid

Do not delay surgery once diagnosis is confirmed 1, 2:

• Progressive accumulation of menstrual blood increases risk of endometriosis and adhesions 2
• Earlier intervention correlates with better long-term fertility outcomes 2

Do not perform hysterectomy or hemihysterectomy 2:

• These patients have excellent obstetrical outcomes after conservative surgery 2
• Pregnancy rates and outcomes are similar to patients with uncomplicated uterus didelphys 2

Do not overlook the contralateral system 3, 2:

• Ensure the non-obstructed hemivagina and uterine horn are normal 2
• Document patency and function of both systems 2

Postoperative Considerations

Follow-up at 2 and 6 months postoperatively is essential 5:

• Confirm complete resolution of symptoms 5
• Verify persistent large communication between the two vaginas by vaginoscopy 5
• Assess for recurrent obstruction or stenosis 5

Long-term fertility counseling should be provided 2:

• Obstetrical outcomes are favorable with appropriate management 2
• Patients should be informed about the didelphic uterus and potential obstetric implications 2