TL;DR: Eight cases of uterus didelphys with obstructed hemivagina referred to the Gynecology service at Baylor College of Medicine over a 2-year period were reviewed, demonstrating the difficulty in diagnosis, heterogeneity in presentation, and need for expertise in this rare congenital anomaly.
TL;DR: One of the largest reviews of HWWS in pediatric patients to date finds good long-term outcome occurs after vaginal septectomy, and this diagnosis should be suspected in females with a pelvic mass and ipsilateral renal agenesis.
TL;DR: A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical intervention as well as decreased long-term morbidity.
TL;DR: R rarity and variable presentation of congenital vaginal obstructions can lead to delayed diagnosis and erroneous management, so a high index of suspicion and cross-sectional imaging help in early diagnosis and associated renal anomalies.
Abstract: Congenital obstructing lesions of vagina, hydrometrocolpos, and hematocolpos, present at a variable time during early childhood and adolescence to different medical and surgical specialties. Twenty-six cases presenting over an 18-years period (1987–2005) were divided into three groups; Group A: neonates (6), Group B: adolescents (18), and Group C: adults (2). Common presentations in neonates (Group A) were abdominal mass (5), neonatal sepsis (3), and respiratory distress (2); whereas abdominal pain (18), voiding dysfunctions (13), and backache (7) were prevalent in adolescents (Group B). Adults (Group C) presented with inability to consummate and infertility (2). Four patients received erroneous treatment; exploratory laparotomy (1) and appendectomy (3). Urinary symptoms and associated urinary abnormalities were present in more than 50% of cases, especially those with complex anomalies. Management included excision of imperforate hymen (16) and transverse vaginal septum (8) through perineal (20) and abdominoperineal approach (4). Patients with urogenital sinus (1) and cloacal malformation (1) had staged reconstruction at 2.5 years of age following preliminary vesicostomy and colostomy at birth. On follow up (range 1–15 years; mean 7) more than 60% patients have menstrual irregularity (11), endometriosis (5), and infertility (4). In conclusion, rarity and variable presentation of congenital vaginal obstructions can lead to delayed diagnosis and erroneous management. A high index of suspicion and cross-sectional imaging help in early diagnosis and associated renal anomalies. A comprehensive management is imperative to preserve the reproductive potentials, as significant proportion of patients may experience sexual difficulties, menstrual irregularity, and infertility.
TL;DR: Although surgical correction is the mainstay of hymenal anomalies, septa, obstructed hemivaginas, and distal vaginal agenesis, first-line therapy for Müllerian aplasia is progressive dilation.
Abstract: Summary of accurate diagnosis and surgical management of vaginal anomalies. Imperforate hymen, transverse septa, and distal vaginal agenesis present similarly with pain and hematocolpos. Likewise, imperforate hymen, transverse septa, distal vaginal agenesis, and Mullerian aplasia may appear similarly on examination. Imaging should be used to better differentiate the anomaly. Although surgical correction is the mainstay of hymenal anomalies, septa, obstructed hemivaginas, and distal vaginal agenesis, first-line therapy for Mullerian aplasia is progressive dilation. Many surgical techniques are also described but no consensus exists as to the best one. Clinicians should be aware of the appropriate evaluation, differential diagnosis, and management options available for vaginal anomalies.