The obstructed hemivagina, ipsilateral renal anomaly, and uterine didelphys triad and the subsequent manifestation of cervical aplasia

STUDY OBJECTIVE: To compare a case series of the obstructed hemivagina, ipsilateral renal anomaly and uterine didelphys triad with the literature, with a focus on a subset of patients with cervical aplasia. DESIGN, SETTING, AND PARTICIPANTS: A retrospective case series was conducted of all patients with the triad managed between 2005-2013 at a tertiary center for adolescent gynecology. RESULTS: Similarities in this cohort, compared to the literature, included heterogeneity of presentation, presence of endometriosis, and asymmetry of affected side. Notable differences included 1 patient with a 16p11.2 microdeletion and 2 patients with subsequent unilateral cervical aplasia. All patients underwent magnetic resonance imaging for diagnosis. Vaginal septum division was performed in 8 cases and excision in 1 case. Both cases with cervical aplasia ultimately underwent hemi-hysterectomy, and highlight the implications of this rare variant in regards to its existence, limitations of magnetic resonance imaging in this context, and suggestions for improvement in diagnosis and management. CONCLUSION: The complexity of these cases, especially the evolving manifestation of cervical aplasia postoperatively, illustrates the need to recognize limitations in imaging and divergence in definitive management.