A New Case of Herlyn–Werner–Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney

The aim of this study was to present a new case of congenital Herlyn–Werner–Wunderlich syndrome, a rare anomaly of the female reproductive tract, and review the related literature. A 12-year-old girl presented with severe dysmenorrhea since menarche and magnetic resonance imaging showing a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. A diagnostic cystoscopy and a reduced-port robot-assisted laparoscopy with chromopertubation were performed in order to identify the anomaly. Uterine didelphys and right cervical dysgenesis with ipsilateral vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis were diagnosed on the basis of histopathologic findings, and she subsequently underwent a robotic unilateral right total hysterectomy with salpingectomy. This case report reinforces the importance of the intraoperative biopsy for an accurate diagnosis, despite magnetic resonance imaging being considered the gold-standard diagnostic tool.

Surgical therapy for herlyn-werner-wunderlich (HWW) syndrome cases

Introduction: One of the rare forms of Mullerian congenital ducts is Herlyn-Werner-Wunderlich (HWW) syndrome. HWW syndrome is usually found at puberty with nonspecific symptoms. Pelvic pain can be found both acute and chronic, dysmenorrhoea, as well as hematometra and hematocolpos. Early detection and treatment results in a good prognosis with fertility preservation. Case presentation: Two cases of Herlyn-Werner-Wunderlich (HWW) syndrome surgically performed were reported at Dr. Sardjito Yogyakarta Indonesia Central General Hospital in 2017-2018. Both cases are still children and at the age of puberty. The chief complaint in both cases it was reported to have the same main complaint, abdominal pain, with the condition having never experienced menstruation yet. On further examination it is known that pain is caused due to hematometra and hematosalping that occur in both cases. The first case with hematometra in the right hemiuterus and right hematosalpinx, while second case with hematometra in the uterus didelphys and bilateral hematosalpinx. One case with a normal vagina, and the other case of vaginal agenesis. There was previous history of surgery in both of cases. There are abnormalities in the urinary tract that occur together with these genital abnormalities. Surgical therapy is carried out with the main goal being to drain menstrual blood from hematometra and hematosalpinx. In first case which has a vagina within the normal range (9 cm long) and the inferior area of ​​the right hemiuterus which is bulging toward the vagina, an area can be identified for the drainage incision to drain the entire hematometra and hematosalpinx. In the second case with vaginal agenesis, neovagina which is made by sigmoid colon vaginoplasty surgery can be a way of menstrual blood flow for a lifetime. Conclusion: Clinical manifestations of Herlyn-Werner-Wunderlich (HWW) syndrome cases can be classified into 2 groups, complete and incomplete. Surgical treatment is based on the condition of the uterus and vagina. In the condition of cervical agenesis but with a normal vagina, surgery can be done by making an incision in the drainage of the uterine cervical tissue. If vaginal agenesis is obtained, vaginoplasty surgery can be performed, and one of the options is sigmoid colon vaginoplasty.

A Case of Congenital Urethrovaginal Fistula in a Female Child with Suspected Imperforate Hymen: A Case Report

Urethrovaginal fistula is an abnormal communication between the urethra and vagina. Urethrovaginal fistula results in urinary incontinence with urine continually leaking from the vagina. In children congenital anomaly may also be the cause. Congenital Urethrovaginal fistula is an extremely rare genitourinary anomaly. The reported five cases in the literature are all associated with urogenital abnormalities like vaginal septum, vaginal agenesis and imperforate hymen. We present a case of a 7-year-old female that was referred from a peripheral hospital for micturating cystourethrography (MCUG) on account of urinary incontinence and passage of urine from vaginal orifice. Following MCUG the urethra, proximal fistula between the urethra and vagina, distended urinary bladder, contrast opacified uterus and a fallopian tube were all demonstrated. We report this case because of its rarity in the literature.

Acute Kidney Injury After Sigmoid Vaginoplasty Procedure In Mullerian Duct Anomalies: A Case Report

Background: Development of female genital tract is a complex process and dependent upon a series of events involving cellular differentiation, migration, fusion, and canalization. Mullerian Duct Anomalies (MDA) are uncommon congenital anomalies, but can vary widely and treatable with surgical procedure. Classification system that have been used are classification from American Society for Reproductive Medicine (ASRM) and European Society of Human Reproduction and Embriology (ESHRE) and European Society for Gynaecological Endoscopy (ESGE). Surgical procedure in MDA patient had a high successful rate, but postoperative complication can arise in form of the need for further surgical requirement and acute kidney injury. Case report: A 20 year old girl admitted to obstetric gynecologic clinic with complaints of abdominal pain, amenorrhea, and redness voiding. She experienced abdominal pain since 6 years ago. Previously, patient had history of vaginal drainage procedure when she was 14 years old but she did not felt improvement in complaint and symptom. Cystoscopy and radiology imaging showed vaginal agenesis and renal dekstra agenesis, subsequently patient was planned for a sigmoid vaginoplasty procedure. Identification and exploration during surgery revealed vaginal agenesis, renal dekstra agenesis, hematometra from hemiuterus dekstra, hemiuterus sinistra with asesorius or hipoplasia uterine and hematosalping dekstra. In 5 days post operative, patient suffer anuria and acute kidney injury complication. Acute kidney injury after major surgery involving gastrointestinal was common because of surgical stress response, and agenesis renal condition aggravates this complication.