17. A retrospective review characterizing surgical and medical management of 10 patients with complex obstructive Müllerian anomalies

Müllerian anomalies can be obstructive or non-obstructive (32% vs 68% of Müllerian anomalies). If an obstructive Müllerian anomaly is not diagnosed prior to the onset of menarche, patients can present with severe pain requiring emergent care as a result of marked hematometrocolpos. Management of these patients is focused on relieving the hematometrocolpos, improving pain, and definitively relieving the obstructive process. There is a paucity of literature on the presentation and standard management of individuals with complex Müllerian obstruction. The aim of this study was to describe 10 patients with complex obstructive Müllerian anomalies who received definitive surgical management at a single tertiary center.

Our cohort consisted of ten females presenting at 14 years and 6 months old (± 1.20), of which one patient had a known complex obstructive anomaly while nine were diagnosed at time of presentation for acute symptoms. In this cohort, 70% were diagnosed in an emergency department setting while 30% were diagnosed in an outpatient ambulatory setting. Notably, 30% of our cohort had an additional diagnosis of renal anomaly. Of the girls without a known obstruction, the chief complaint was abdominal pain (89%), with only one patient presenting primarily for amenorrhea. The most common imaging modality to work-up this condition was ultrasound (80%), followed by an MRI (60%), and finally, CT (30%). Pharmaceutical hormonal suppression prior to definitive treatment consisted of GnRH agonist/antagonists (60%), oral progestin (30%), and continuous OCP's (10%). For undiagnosed patients, surgical management primarily occurred via a two-step process, though 22% of the patients underwent a one-step technique. Definitive surgical management of the obstruction occurred 23 months (± 12) after initial diagnosis. The procedure took 7.8 hours (± 4.2) to complete and most commonly required three surgeons from pediatric and adolescent gynecology, pediatric surgery, and pediatric urology. Average postoperative hospitalization was 7 days (± 3). The most common postoperative complications were restenosis (33%), vaginal infection (11%), and fistula (11%). The cohort underwent an average of three total surgeries and were followed for about 3.4 years (± 3.1) after definitive surgery.

Our case series provides new information regarding the diagnosis and definitive management of 10 girls with complex obstructive Müllerian anomalies. Management of this type of anomaly requires hormonal suppression, multiple prolonged surgeries, and multidisciplinary care. Further elucidation regarding the presentation and care for this population will aid in swift diagnosis and a stepwise standardization of management.

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