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Approaches to Female Congenital Genital Tract Anomalies and Complications

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Approaches to Female Congenital Genital Tract Anomalies and Complications Int Surg 2017;102:367–376 DOI: 10.9738/INTSURG-D-16-00124.1 Approaches to Female Congenital Genital Tract Anomalies and Complications Emine Ince1, Pelin Oguzkurt˘ 1, Semire Serin Ezer1, Abdulkerim¨ Temiz1, Hasan Ozkan¨ Gezer1,Sxenay Demir2, Akgun¨ Hi¸csonmez¨ 1 1Departments of Pediatric Surgery and 2Radiology, Baskent University Faculty of Medicine, Ankara, Turkey Objective: Female congenital genital tract anomalies may appear with quite confusing and deceptive complications. This study aims to evaluate the difficulties in diagnosis and treatment of female congenital genital tract anomalies that frequently present with complications. Summary: During a 10-year period, we evaluated 20 female patients with congenital genital tract anomalies aged between 3 days and 16 years. All patients were retrospectively analyzed in terms of the results of diagnostic studies, surgical intervention, and treatment. Methods: Ultrasonography and magnetic resonance imaging revealed hydromucocolpos or hematocolpometra, imperforate hymen, distal vaginal atresia, didelphys uterus, an obstructed right hemivagina, uterovaginal atresia, a unicornuate uterus with a noncom- municating rudimentary horn, a vesicovaginal fistula, a utero-rectal fistula, intraabdominal collection, and a vaginal calculus. Results: Two patients had Mayer-Rokitansky-Ku¨ ster-Hauser syndrome and 6 patients had obstructed hemivagina and ipsilateral renal anomaly syndrome. Definitive surgical interventions were hymenotomy, vaginal pull-through, vaginovaginostomy, and vesico- vaginal fistula repair using a transvesical approach. In conclusion, female congenital genital tract anomalies may appear with a wide range of complications. Conclusions: There is a potential to do significant harm, if the patient’s anatomic problems are not understood using detailed imaging. Revealing the anatomy completely and defining the complications that have already developed are critical to tailor the optimal treatment strategies and surgical approaches. Corresponding author: Emine I˙nce, MD, Baskent U¨ niversitesi TıpFaku¨ ltesi, Adana Uygulama ve Arastırma Hastanesi, C¸ ocuk Cerrahisi Klinig˘i, Seyhan Hastanesi Baraj Yolu, 1. Durak Seyhan /Adana, 01150, TURKEY. Tel.: 90 322 458 68 68 1000; Fax: 90 322 459 26 22; E-mail: [email protected] Int Surg 2017;102 367 INCE FEMALE CONGENITAL GENITAL TRACT ANOMALIES Key words: Female congenital genital tract anomalies – Hematocolpos – Hydromucocolpos Imperforate hymen – Mullerian¨ anomalies emale congenital genital tract anomalies may region and to exclude associated urological malfor- present with different clinical appearances and mations. In more complex cases, magnetic reso- deceptiveF complications. The most common condi- nance imaging (MRI), a dimercaptosuccinic acid tion seen in congenital malformations of the genital (DMSA) scan, voiding cystourethrography (VCUG), tract is imperforate hymen (IH) with an incidence of intravenous pyelography, and colon radiography 1 in 1000 to 16,000 live births.1 Mullerian¨ duct were performed. All definitive surgeries were anomalies (MDAs), which are rarely seen, result performed by 2 surgeons who had scientific from the interruption of normal development of the knowledge, skills, and experience at the same levels. female genital tract, which involves a series of All patients were followed after hospital discharge complex processes characterized by the differentia- with a mean follow-up of 2 years, and no eligible tion, migration, fusion, and subsequent canalization patients were excluded during the study. of the Mullerian¨ system.2,3 Mullerian¨ duct anoma- lies usually present with obstructive symptoms. The Results mass and compression symptoms due to mucocol- pos are distinct in the neonatal and infantile periods, Twenty patients with a median age of 12.3 years whereas hematometra and hematocolpos are prom- (range: 3 days to 16 years) were included in the 1– inent after menarche during the adolescent period. study. The presenting complaints were abdominal 3 Mullerian¨ duct anomalies such as uterus didel- pain (n ¼ 15), acute urinary retention or AUR (n ¼ phys, unicornuate uterus, rudimentary horn, distal 11), abdominal distention (n ¼ 4), antenatally vaginal atresia, transverse vaginal septum, and detected cystic abdominal mass (n ¼ 2). Physical ipsilateral distal vaginal atresia are rare, and the examination revealed an abdominal mass (n ¼ 17), exact incidence in the general population or infertile vaginal dimple (n ¼ 2), lower abdominal tenderness 2 population still remains unclear. Urinary system (n ¼ 1), and bulging of hymenal membrane (n ¼ 5) anomalies are most frequently defined as anomalies (Tables 1 and 2). Two patients, one of whom was a 4–7 that accompany genital tract anomalies. Due to newborn baby, had impaired renal functions. All inappropriate physical examinations and laboratory patients underwent USG evaluation. Abdominal evaluations in patients with recurrent abdominal MRI was performed in 16 patients. Additional pain and urinary tract infections, many types of imaging modalities were DMSA (n ¼ 4), VCUG (n complications may occur in these patients. ¼ 5), and barium enema (n ¼ 1). Abdominal USG Genital tract anomalies and accompanying com- revealed a distended uterus and a vagina filled with plications have been introduced in individual case hematocolpometra (n ¼ 12), mucocolpos (n ¼ 2), reports in the literature. In the present study, we hydrocolpos (n ¼ 3), uterus didelphys (n ¼ 5), aimed to evaluate the difficulties in diagnosis and absences of uterovaginal structure (n ¼ 2), torsion of treatment of female congenital genital tract anoma- the left ovary (n ¼ 1), and bladder stone (n ¼ 1). lies, which frequently present with complications in Unilateral renal agenesis (n ¼ 7), hydroureteroneph- children to increase attention to this subject. rosis or HUN (n ¼ 3), and pelvic abscess (n ¼ 1) has been identified as additional urinary system anom- Materials and Methods alies. Laryngeal web (n ¼ 1), esophageal atresia with a tracheoesophageal fistula (n ¼ 1), polydactyly (n ¼ The study included 20 children with the diagnosis of 1), corpus callosum agenesis (n ¼ 1), and partial congenital genital tract anomalies in our institution sacral agenesis (n ¼ 1) were detected as other system between January 2006 and April 2016. Patients with anomalies (Tables 1 and 2). MRI was not needed intersex anomalies and persistent cloacal anomalies because the diagnosis of the imperforate hymen was were excluded from the study. The records of the confirmed based on clinical and USG findings in 4 patients were reviewed for age, complaints, diag- patients. Magnetic resonance imaging showed a nostic studies, associated malformations, surgical normal uterus with distal vaginal atresia (n ¼ 5), techniques used, and treatment results retrospec- normal uterovaginal structure with HI (n ¼ 3), tively. Ultrasonography (USG) was performed to absence of uterus and vagen (n ¼ 2), uterus evaluate the internal genital organs and the pelvic didelphys and dublicated vagen with right distal 368 Int Surg 2017;102 Int Surg FEMALE CONGENITAL GENITAL TRACT ANOMALIES 2017; 102 Table 1 Clinical characteristics of patients with vaginal anomalies Physical Association Drainage Complaint examination USG MRI with anomalies tube Diagnosis Operating n (n) (n) (n) (n) (n) (n) (n) (n) Classification 7 AAP (6) Suprapubic Hematocolpometra Cystic mass along Left HUN (1) No (6) IH (7) Hymenotomy (7) AUR (6) mass (6) (6) the upper part Right renal Yes (1) Vaginal Cystoscopy (1) Abdominal Bulging Mucocolpos (1) of the vagina (3) hypoplasia (1) stone (1) Removal of distension (1) IH (5) Hydrocolpos (2) causing Laryngeal web (1) vaginal stones (1) Abdominal compression on mass (1) the urinary (2) Vaginal stone (1) 4 AAP (3) Suprapubic Hematocolpometra Cystic mass along Left nephrectomy Yes (3) Distal Cystoscopy (1) Antenatal mass (3) (2) the upper part (1) vaginal Laparotomy (1) abdominal Mucocolpos (1) of the vagina (4) CRF (1) atresia (4) Vaginal cystic mass (1) Distal vaginal Partial sacral pull-through (4) atresia (4) agenesis (1) Left HUN (1) Polydactyly (1) 1 Antenatal Giant Hydrocolpos Vaginal cavity Left HUN Yes Distal Laparotomy, abdominal abdominal was filled with vaginal repair of cystic mass cystic mass hydrocolpos, atresia vesicovaginal distal vaginal VVF fistula, atresia Vaginal pull-through AAP, acute abdominal pain; AUR, acute urinary retention; CRF, chronic renal failure; HUN, hydroureteronephrosis; IH, imperforate hymen; MRI, magnetic resonance imaging; USG, ultrasonography; VVF, vesicovaginal fistula. INCE 369 370 Table 2 Clinical characteristics of patients with Mullerian¨ duct anomalies INCE Association with Drainage Complaint Physical USG MRI anomalies tube Diagnosis Syndrome Operating n (n) examination (n) (n) (n) (n) (n) (n) (n) Classification 2 AAP (2) Vaginal Uterovaginal Uterovaginal Solitary No Uterovaginal MRKH Laparoscopy Dimple atresia (2) atresia (2) ectopic agenesis (2) (2) (2) (2) Torsion of the Torsion of the Kidney Left left ovary (1) left ovary (1) (1) oophorectomy EAþTEF (1) (1) 4 AAP (3) Suprapubic Didelphys uterus (4) Didelphys Right renal No Didelphys HWW Vaginoscopy AUR (4) mass (3) Obstructed right uterus (4) agenesis (2) uterus (4) (4) (4) Abdominal hemivagina (4) Obstructed right (4) Yes Obstructed Vaginostomy mass (1) Hematocolpometra hemivagina Corpus (2) right (4) (4) (4) callosum hemivagina (4) Hematocolpometra agenesis (4) (1) 1 AAP, AUR Lower Uterus didelfis, Unicornuate uterus Right renal Yes Unicornuate OHVIRA Colostomy,
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