Original Study The Obstructed Hemivagina, Ipsilateral Renal Anomaly, and Uterine Didelphys Triad and the Subsequent Manifestation of Cervical Aplasia S. Sabdia MBBS, DRANZCOG 1 , B. Sutton MBBS, FRANZCR, Dip Fetal Medicine 2 , R.M.N. Kimble MBBS, FRANZCOG 1 ,3, * 1 Department of Obstetrics and Gynaecology, Queensland Statewide Paediatric and Adolescent Gynaecology Service, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia 2 Department of Medical Imaging, Royal Brisbane and Women’s Hospital, Brisbane, Queensland, Australia 3 University of Queensland School of Medicine, Brisbane, Queensland, Australia abstract 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 asym- metry 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. Key Words: Obstructed hemivagina, Uterine didelphys, Absent kidney, Cervical aplasia, 16p11.2 Introduction The obstructed hemivagina, ipsilateral renal anomaly, and uterine didelphys triad is an uncommon congenital M€ ullerian abnormality that usually presents with progres- sively worsening dysmenorrhea in adolescence. We present a case series of 10 patients with this constellation of anomalies managed between 2005-2013 at a tertiary center for adolescent gynecology. Various similarities and differ- ences have been observed in this case series compared with the current literature and these are discussed, along with the experience at this center of the subsequent diagnosis of cervical aplasia and need for further surgery. Method Case notes were reviewed for all 10 patients managed with the triad of obstructive hemivagina, ipsilateral renal anomaly, and uterine didelphys between 2005-2013 at a tertiary center for adolescent gynecology. Data collected from patient charts included age at menarche, time interval from symptoms to definitive surgery, presentation, affected side, imaging, operative management, concurrent pathol- ogy, and outcome at follow up. Results Patient details were reviewed and include 4 cases previ- ously described in the literature to reflect an accurate case series from this institution. 1 Age at menarche ranged from 10 to 14 years old, with a mean age of twelve years. Time in- terval from symptoms to definitive surgery ranged from 4 days to 14 years. The most frequent presentation was progressively worsening dysmenorrhea, although 3 patients presented with acute abdominal pain and 1 case was an incidental finding. Magnetic resonance imaging (MRI) was performed for all patients in the case series. Seven of the 10 cases had an affected right side. Notable abnormalities at time of surgery included endometriosis in 6 of the 10 cases, unilateral ovarian agenesis in 1 patient, and 2 patients with cervical aplasia. Genetic testing revealed the microdeletion of 16p11.2 in 1 patient who was noted to have developmental delay, learning difficulties, and seizures in addition to fea- tures of the triad. Vaginal septum division was performed in 8 cases and excision in 1 case. All patients who underwent vaginal septum division have had no major concerns on follow-up, with the longest follow-up from time of surgery being 8 years. Vaginal constriction was found in the 1 patient who underwent vaginal septum excision. The authors indicate no conflicts of interest. * Address correspondence to: Rebecca Kimble, MBBS, FRANZCOG, Women's and Newborn Services, Level 5 Ned Hanlon BuildingRoyal Brisbane and Women's HospitalButterfield Street, Herston 4029Australia; Phone: 3646 8536; fax: 3646 7798 E-mail address: rebecca.kimble@health.qld.gov.au (R.M.N. Kimble). 1083-3188/$ - see front matter Crown Copyright Ó 2014 Published by Elsevier Inc. on behalf of North American Society for Pediatric and Adolescent Gynecology. All rights reserved. http://dx.doi.org/10.1016/j.jpag.2014.02.001