Ipsilateral Renal Agenesis Research Articles

Laparoscopic metroplasty: reconstructive surgery for unicornuate uterus with noncommunicating, functional uterine horn

To demonstrate laparoscopic surgery for a patient with unicornuate uterus and a large hematometra in the noncommunicating uterine horn. Narrated video featuring the diagnostic tests and surgical management. Academic tertiary hospital. A 13-year-old woman, gravida 0 with menarche at 9 years old, had severe dysmenorrhea during her recent menstrual cycles. The results of transrectal three-dimensional sonography, hysteroscopy, and magnetic resonance imaging (MRI) were consistent for a uterine didelphys composed of a right unicornuate uterus and a left hemiuterus with hematometra and no connection to the cervix. Left adnexal endometrioma and ipsilateral renal agenesis were also noted. Laparoscopic adhesiolysis and enucleation of the left ovarian endometrioma were first performed. Then, implementing the concepts of Strassman unification method, a horizontal incision was made on the medial side of each hemicorpus, starting from the left rudimentary horn and ending at the fundal area of the right hemiuterus. The incision was carried deep enough to reach the endometrium. The opposing, reverted myometrium was then reapproximated from the lower end of the uterus to the upper fundal part in two layers, using absorbable bidirectional monofilament barbed suture. The outer serosal layer was closed with 1-0 Monocryl via a running continuous suture. On postoperative day 2, the patient was discharged uneventfully. Symptomatic relief and restoration of normal and functional anatomy. The patient has regular menstrual cycles with no recurrence of dysmenorrhea or ovarian endometrioma. At a 2-year postoperative follow-up evaluation, hysteroscopic examination revealed a single uterine cavity with a small fundal septum. The MRI scans showed an anteverted, symmetrical uterus. In comparison with the widely applied hemihysterectomy, reconstructive laparoscopic metroplasty not only effectively achieves symptomatic relief but also offers better uterine symmetry and a larger intrauterine cavity via unification of the uterus.

Favorable pregnancy outcome for a patient with Robert's uterus, bicornuate uterus, and ipsilateral renal agenesis.

A case of a patient diagnosed with hybrid Robert's uterus and bicornuate uterus, and unique ipsilateral renal agenesis, who successfully delivered a baby after surgery.

PERANAN MRI ABDOMEN PADA KASUS HERLYN WERNER WUNDERLICH

Background: Herlyn-Werner-Wunderlich syndrome (HWW) is a very rare genetic syndrome, a combination of anomalies between the mullerian and mesonephric ducts characterized by uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis, also known as Obstructed Hemivaginal syndrome and Ipsilateral Renal Anomaly (OHVIRA). Magnetic Resonance Imaging (MRI) is an effective diagnostic support tool for finding out anatomical abdominal abnormalities with the right choice of protocol sequences to obtain optimal MRI images.
 Method: This study used a qualitative descriptive approach by presenting a case of a 20 year old female patient who had complaints of lower abdominal pain, no previous medical history and irregular menstruation. MRI Abdomen without contrast was performed using an MRI 1.5 Tesla signa explorer GE Healthcare at EMC Tangerang Hospital. Using the Coronal T2 SSFSE sequence, T1 fat sat LAVA, Axial T2 SSFSE, T1 fat sat LAVA breath hold method and Coronal 3D IFIR (MRA renal without contrast). Specifically for the pelvic area with the Axial T2 Propeller, Coronal T2 fat sat, and Sagittal T2 sequences.
 Result: In the Axial T2 propeller sequence, pelvic area retrieval revealed two separate uterine corpuses (uterus didelphys) accompanied by a heterogeneous oval lesion most likely originating from the left side of the vagina (hemivagina). Whereas in the Coronal T2 sequence the whole abdomen was taken and confirmed by the Coronal 3D IFIR, the right kidney was anatomically visible while the left kidney was not visible (agenesis).
 Conclusion: Abdominal MRI is a very accurate diagnostic tool to perform Herlyn-Werner-Wunderlich (HWW) syndrome without contrast agent with SSFSE T2 sequences, T2 propeller and 3D IFIR which have an important role in visualizing the quality of MRI images.

Cystic Dysplasia of the Rete Testis: Does Pathophysiology Guide Management?

Cystic dysplasia of the rete testis (CDT) is a rare, benign, cause of testicular mass in the pediatric population. The mass appears on sonography as multiple small cysts of varying size surrounded by normal or compressed testicular tissue. CDT is often associated with other genitourinary anomalies, commonly presenting with agenesis or dysplasia of the ipsilateral kidney. The pathophysiology and the management remains controversial. We report a case of a 3-year-old presenting with an enlarged testicular mass later presumed to be CDT associated with ipsilateral renal agenesis, review the literature, and propose an evaluation and management algorithm.

OHVIRA Syndrome: A Case Report

OHVIRA syndrome or Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of female urogenital tract which is represented by the triad of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. This rare variant of Müllerian duct anomalies represent failure of vertical and the lateral fusion of Müllerian ducts around 9 weeks of gestation. OHVIRA syndrome comprises about 2-3% of Müllerian abnormalities. A 13 years old girl got admission in FMCH on 15.3.2018 with lower abdominal pain and acute retention of urine. Her menarche was established 6 months back with regular cycle and associated with dysmenorrhoea. Imaging studies revealed uterine didelphys, highly dilated cervix with hematocolpos and absence of right kidney. The vaginal septum was excised. The patient recovered fully and was under follow up for 6 months. There was no evidence of further outflow tract obstruction. Faridpur Med. Coll. J. Jan 2019;14(1): 54-56

Diagnosis and treatment of Herlyn Werner Wunderlich syndrome: a case report

Background: Herlyn Werner Wunderlich syndrome (HWWS) is a rare female urogenital tract abnormality, isolated by presenting a didelfous uterus, transverse vaginal septum and ipsilateral renal agenesis. Objective: A case report of a patient with Werner Herlyn Wunderlich syndrome. Case report: Female patient of 17 years of age, single, nubile, menarche at 14 years, infrequent menstrual cycles, normal volume, with dysmenorrhea EVA 7/10, attends to present disabling pelvic pain, ultrasound was found finding, hematocolpos and hematometra, is complemented with hysteroscopy, computed tomography (CT) and magnetic resonance imaging (MRI), a diagnosis of HWWS syndrome is integrated by confirming the presence of a didelfo uterus, obstructed right hemivagina and right renal agenesis, with distention of the vagina and cervix secondary to hematometra and hematocolpos, normal ovaries. The approach in this patient was by minimal invasion. Conclusion: Herlyn Werner Wunderlich syndrome is a rare mullerian defect, it is often diagnosed late, the diagnosis is usually made after menarche, associated with chronic pelvic pain or palpable pelvic mass; 2D ultrasound along with physical examination performs the suspected diagnosis; This should be complemented with RM. The treatment of choice is vaginal septum resection assisted by minimally invasive techniques.

Cystic dysplasia of testes: case report and review of literature

Cystic dysplasia of the testis is a rare benign congenital defect in which the testis is a formation of multiple irregularly shapes cystic spaces, first described in 1973 by Leissring and Oppenheimer. We report a case and review the literature to identify clinically useful associations and management options. Literature review. A comprehensive literature search was performed on electronic databases and search platforms. Only English publications were included. Hand search was additionally performed separately by two investigators, with data extracted from relevant publications included patient characteristics, management workup, associated conditions and outcomes. Case report. A male newborn with VACTERL association was noted to have an enlarged right testis and impalpable left testis. Ultrasonography of the testis revealed cystic lesions and tumour markers were normal. Pole to pole biopsy confirmed cystic dysplasia. Mild involution has been noted at 3 months follow-up. Literature review: 40 publications including 55 cases of CDT were identified. Diagnosis was made at median 4.7 years (range 0-12 years) with ultrasound being the main investigation to achieve diagnosis. Strong associations were found between CDT and ipsilateral renal agenesis (50%), MCDK (15%), VACTERL (10%), renal duplication (3%) and Potter’s Syndrome (6%). The most common management by far was orchidectomy. Of those cases which were treated conservatively with observation, almost all were found to have involution on follow up imaging. Clinicians should be aware of CDT as a rare differential for neonatal testicular enlargement. Management should be conservative due to negligible long term malignancy risk and investigations are recommended to check for known associations which have clinical significance.

Síndrome de Herlyn-Wener-Wunderlich

Introducción: El Síndrome de Herlyn-Wener-Wunderlich es una malformación de los conductos de Müller y de Wolf, debido a una falta de fusión de los mismos, es muy rara y se caracteriza por presentar un útero didelfo, con una hemivagina obstruida y agenesia renal ipsilateral, más frecuente del lado derecho.
 Objetivo: Describir un caso clínico de una paciente adolescente con cuadro de dismenorrea secundario a Sindrome de Herlyn-Wener-Wunderlich.
 Material y métodos: Se realizó un estudio descriptivo, retrospectivo y documental sobre dismenorrea secundaria, malformaciones mullerianas y presentación de un caso clínico.
 Resultados: Se describe el caso de una paciente de 13 años tratada en la Unidad Materno Infantil del Hospital Teodoro Maldonado Carbo, con cuadro de dismenorrea severa desde el inicio de la menarca, presenta masa pélvica dolorosa a la palpación, se realiza ecografía abdominal y pélvica encontrando dos tumoración de contenido líquido con sedimento que se extienden hasta canal vaginal y que compromete cérvix bilobulado, la ecografía abdominal reporta ausencia de riñón derecho por lo que fue sometida a laparotomía exploratoria confirmando el diagnóstico de malformación uterina con útero didelfo, hemivagina derecha ciega y hematocolpos. 
 Conclusiones: Las malformaciones Mullerianas corresponde a anomalías congénitas poco comunes y el Síndrome de Herlyn-Wener-Wunderlich es una entidad mucho menos común, causante de cuadros de dismenorrea severa, abdomen agudo, su diagnóstico clínico es difícil y requiere estudios complementarios. El tratamiento es conservador con el fin de preservar la función reproductiva de la paciente

Colpolithiasis: A mini review

Objective: Vaginal calculus is an unusual finding. In this study we describe a case of primary vaginal calculus in a rather rare urogenital anomaly- Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome. This syndrome is shrouded in controversies. Here we present a mini review about vaginal calculus and theory of embryological development of female genital tract as related to OHVIRA syndrome. Case: A 50 years-old unmarried lady with primary amenorrhoea presented with pain abdomen. She was found to have calculus in the pelvis by x-ray which was thought to be bladder calculus initially. On local examination the vaginal opening was absent. Imaging studies such as NCCT and MRI confirmed that it is a vaginal calculus, located below the uterine cervix, with absent right kidney. She was diagnosed with OHVIRA syndrome with colpolithiasis. On laparotomy she was detected to have unicornuate uterus with absence of right Mullerian structures. The calculus occupied the whole of upper part of vagina, which was a blind pouch. The patient underwent total abdominal hysterectomy, left salpingo-ophorectomy with removal of calculus after opening the vaginal vault. Postoperative recovery was uneventful. Chemical analysis of the calculus revealed calcium magnesium oxalate which is of hematic origin. Keywords: Primary vaginal calculus, Mullerian duct anomaly, Obstructed hemivagina, OHVIRA syndrome, Herlyn-Werner-Wunderlich syndrome.

Herlyn‐Werner‐Wunderlich syndrome with cervical atresia complicated by ovarian endometrioma: A case report

Herlyn-Werner-Wunderlich syndrome (HWWs) is a rare congenital malformation that involves uterus dideslphys, obstructed hemivagina and ipsilateral renal agenesis. The obstructed vagina affects menstrual flow and causes related clinical features after menarche. Pelvic endometriosis is one of the common complications of HWWs. Resection of the vaginal septum can release the obstruction and result in good outcomes. However, in the case of cervical atresia, a rare variant of HWWs, ipsilateral hysterectomy is recommended because it is difficult to canalize cervical atresia surgically. Here we present a case of HWWs with cervical atresia complicated with pelvic endometriosis. She was treated with laparoscopic ovarian cystectomy followed by hormonal therapy. Six months after surgery, there is no evidence of recurrence of endometrioma.

Zinner’s Syndrome – The Value of Clinical Imaging and Morphopathological Findings for Diagnosis

Abstract Introduction: Cystic congenital malformations of the seminal vesicle are unusual. More than half of them are associated with ipsilateral renal agenesis. This disease was first described by Zinner in 1914, and since then, more than 200 cases have been reported. Most of the patients with this congenital disease present few symptoms until the middle-age. Case presentation: A 61-year-old male was referred to the Urology Clinic with intermittent pain and sensibility in his lower left abdomen in the last 12 months accompanied by intermittent bowel obstruction. The digital rectal examination could not assess the prostate due to a rectal stenosis. On computed tomography and magnetic resonance imaging (MRI) a cystic tumor with close relations with the bladder wall and prostate, associated with left kidney agenesis was identified. The cystic tumor was surgically removed. The histopathological examination of the tumor revealed a multilocular seminal vesicle cyst, with a muscular wall. Conclusions: Seminal vesicle cysts combined with ipsilateral renal agenesis are rare urological anomalies. Imaging allows a comprehensive investigation of renal and seminal vesicle anomalies. MRI seems to better visualize cysts and differentiate them from ureteroceles, while the histopathological examination contributes to a correct diagnosis. A multidisciplinary team (urologists, radiologists, and morphologists) is necessary for the proper diagnosis of Zinner’s syndrome.

Vaginoscopic Incision of Oblique Vaginal Septum in Adolescents with OHVIRA Syndrome

This study is to evaluate the efficacy and safety of vaginoscopic incision of oblique vaginal septum in adolescents with Obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. It is about Fourteen adolescents with OHVIRA syndrome managed by vaginoscopic incision of the oblique vaginal septum using a “No-touch” technique over an 8-year period. In all fourteen adolescents with OHVIRA the oblique vaginal septum was incised successfully without any intraoperative complications. Postoperative pain was unremarkable and each patient’s symptoms resolved postoperatively. The 3-month postoperative follow up office vaginoscopy revealed that the vaginal septum had not reformed nor was any vaginal stenosis noted. Vaginoscopic incision of the oblique vaginal septum using a “No-Touch” technique is a safe, minimally invasive, and effective approach for treating OHVIRA syndrome in adolescents with hematocolpos. This technique may be utilized to minimize disruption to the undeveloped vaginal wall and postoperative pain while providing excellent surgical visualization throughout the procedure.

Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome?

AimTo review the experience of a single tertiary center with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) and evaluate if MRI findings correlate with outcome. MethodsPatients (n = 32) diagnosed with OHVIRA syndrome between 2001 and 2019 were analyzed. Presenting symptoms, age of menarche, age at operation, MRI findings, management, and outcome were reviewed. In sagittal MRI planes, distance from hematocolpos to perineum was measured. Measurements were compared among patients who underwent single-stage vaginoplasty and hemihysterectomy. ResultsMean age at diagnosis and mean age of menarche was 16.8 ± 6.4 (10−33) and 12.8 ± 1.0 (10–15). Main presenting complaints were abdominal pain/dysmenorrhea. Eighteen anomalies (56.3%) were on right side. Twenty-eight had ipsilateral renal agenesis, 3 patients had normal renal anatomy and one had unilateral multicystic dysplastic kidney. In MRI, 21 patients had hematocolpos, 11 patients had both hematocolpos and hematometra. 28 patients underwent single-stage vaginoplasty and vaginal septum resection. One had hemihysterectomy due to sepsis at presentation. Three patients had hemihysterectomy due to proximal vaginal septum and impossibility of vaginoplasty. During follow-up, 7 cases (21.9%) had married and 5 of these (71.4%) were pregnant or had delivered. Five patients had reoperation during follow-up. MRI images of 19 patients indicated mean distances from hematocolpos to perineum whom underwent vaginoplasty or hemihysterectomy were 33.9 ± 18.1 mm (10–79 mm) and 87.3 ± 11.0 mm (80–100), respectively (p = .009). ConclusionsGold standard treatment of OHVIRA syndrome is single-stage vaginoplasty. Distance from hematocolpos to perineum in MRI may correlate with surgical outcome. Hemihysterectomy may be an alternative for extreme proximal vaginal septum or infectious complications.

Classifying seminal vesicle cysts in the diagnosis and treatment of Zinner syndrome: A report of six cases and review of available literature

This study aims to emphasise the importance of imaging in the diagnosis and treatment decision-making in Zinner syndrome and provide a classification for seminal vesicle cysts. The data of six patients with Zinner syndrome in a single institution were collected. All patients underwent a contrast-enhanced computed tomography (CT) exam. Among these patients, five patients also underwent an magnetic resonance imaging (MRI). These results were combined with the review of available literature to classify the seminal vesicle cysts. Among these patients, two patients had urinary urgency and frequency, while four patients had no urinary symptoms. No reproductive-system symptoms were revealed. The imaging revealed left-sided involvement in two patients and right-sided involvement in four patients. The associated features included ipsilateral renal agenesis, seminal vesicle cyst or agenesis, and ejaculatory duct obstruction. Either an ipsilateral ureterocele or an ipsilateral small testis was noted. The seminal vesicle cysts demonstrated varying attenuation or intensity in the imaging. Imaging (CT and especially MRI) can be critical in the noninvasive diagnosis of Zinner syndrome and in allowing aberrant anatomy to be displayed for possible surgery. The proposed seminal vesicle cyst imaging classification could potentially contribute to clinical decision-making.

Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report

BackgroundHerlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh.Case presentationA 15-year-old Asian girl presented with lower abdominal pain of 3 months’ duration. She had had menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. Abdominal examination found a tender mass on the right iliac fossa. Further evaluation with ultrasound revealed distended endometrial cavity filled with complex fluid and nonvisualization of the right kidney. Pelvic magnetic resonance imaging showed absent right kidney and two separate endometrial stripes surrounded by endometrium and a muscular layer. The right endometrial cavity and cervix were distended with blood. This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. The vaginal septum was resected for vaginoplasty. She was discharged 5 days after surgery and came for follow-up after 7 days. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. She also informed us that she had started regular menstruation without any pain 30 days after the operation.ConclusionAn unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to complications such as endometriosis and infertility. Awareness is required; otherwise, misdiagnosis clearly can occur.

Herlyn-Werner-Wunderlich Syndrome with Central Precocious Puberty: A Case Report

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa. Key words: HWW syndrome, Central precocious puberty, Renal agenesis, Hemivagina, Uterus didelphys

Fetal magnetic resonance imaging is a useful modality for prenatal diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome

The authors report the first case of prenatal fetal MRI diagnosis of unilateral hydrocolpos, uterus didelphys, and ipsilateral renal agenesis in a case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. A 33-year-old woman was referred to the present institution because of a fetal pelvic cyst. Detailed ultrasound revealed a female fetus with a retrovesical cyst and right renal agenesis. Fetal MRI confirmed unilateral hydrocolpos, uterus didelphys, and normal intestinal tract of the fetus. Fetal MRI is a useful modality for prenatal diagnosis of OHVIRA as a method of evaluating the pelvic structures and excluding other cloacal anomalies.

An Unusual Cause of Recurrent Urinary Retention in an Adolescent Female

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We present the case of an otherwise healthy 16-year-old female with acute urinary retention secondary to HWWS. The diagnosis was established with abdominal ultrasound and Magnetic Resonance Imaging (MRI). The patient subsequently underwent surgical resection of the vaginal septum resulting in relief of obstruction. Clinical symptoms in patients with HWWS typically present after menarche with progressive hematometra causing pain and compression of localized structures. Even though ultrasound can help in the diagnosis, MRI is the best choice of imaging for the visualization of these anomalies. The diagnosis of HWWS is important to consider in young females of reproductive age presenting with symptoms of obstruction of adjacent structures. Our patient presented with acute urinary retention which is a rare symptom in this entity. A high index of clinical suspicion and awareness of the syndrome are required to make a speedy diagnosis and prevent future complications.

Síndrome de Herlyn-Werner-Wünderlich y diagnóstico tardío: a propósito de un caso

El síndrome de Herlyn-Werner-Wünderlich es una anomalía congénita del tracto urogenital. Se caracteriza por la tríada de útero didelfo, hemivagina obstruida y agenesia renal unilateral. La mayoría de pacientes se mantienen asintomáticas hasta la menarquia, cuando debutan con dismenorrea, dolor pélvico y masa palpable debido al hematocolpos asociado. Presentamos el caso de una paciente de 32 años en estudio por problemas de fertilidad. Se realizó estudio ecográfico 3D e histerosalpingografía, donde se observó útero bicorne completo con doble canal cervical, con impresión diagnóstica de vagina homolateral ciega. A la especuloscopia durante el periodo menstrual se observó cérvix de hemivagina normal y orificio de salida en cara lateral, por donde drenaba sangre de hemivagina ciega (probablemente fistulizada). Se realizó una ecografía abdominal que constató agenesia renal izquierda, confirmándose el diagnóstico.

Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn–Werner–Wunderlich Syndrome

IntroductionHerlyn–Werner–Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients. MethodsWe carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date. ResultsThere were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina. ConclusionOur case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment. Study designCase series, level IV evidence.