RESUMEN
INTRODUCTION: Zinner Syndrome (ZS), a rare congenital malformation of the mesonephric duct, combines seminal vesicle cyst (SVC) with ipsilateral upper urinary tract abnormalities. Typically asymptomatic in childhood, ZS manifests between 2nd to 4th decades with bladder symptoms, perineal pain and infertility. Diagnostic confirmation with additional imaging is needed when either renal or seminal abnormalities are identified. MATERIALS AND METHODS: A retrospective study spanning 22 years identified 20 pediatric ZS cases through clinical analytics. Demographic, clinical, and radiological data were analyzed, including presenting complaints, imaging modalities (ultrasound, CT, MRI), and surgical findings. The study was HIPAA-compliant and IRB-approved. RESULTS: Among 20 cases (mean age: 7.3 years), clinical presentations included asymptomatic cases, urinary symptoms, and abdominal pain. Imaging revealed renal anomalies (agenesis, multicystic dysplastic kidney) and seminal vesicle abnormalities. Surgical interventions (n = 12) addressed symptomatic cases, often involving robotic or laparoscopic procedures. DISCUSSION: ZS, though rare, presents with varied clinical features, necessitating a multidisciplinary approach. Early diagnosis is facilitated by prenatal identification of renal abnormalities. Surgical intervention is reserved for symptomatic cases, with techniques such as vesiculectomy and resection of remnant structures employed. CONCLUSION: This study highlights ZS's diverse clinical and radiological spectrum, emphasizing the need for vigilance in detecting overlapping entities. Timely identification, utilizing advanced imaging techniques, is crucial for accurate diagnosis and appropriate management of Zinner Syndrome in the pediatric population.
Asunto(s)
Vesículas Seminales , Humanos , Estudios Retrospectivos , Masculino , Niño , Vesículas Seminales/anomalías , Vesículas Seminales/diagnóstico por imagen , Preescolar , Síndrome , Femenino , Adolescente , Anomalías Múltiples/diagnóstico , Conductos Mesonéfricos/anomalías , Quistes/diagnóstico , Quistes/cirugía , LactanteRESUMEN
Zinner's syndrome is a rare congenital malformation characterized by the association of unilateral renal agenesis with ipsilateral seminal vesicle cyst and ejaculatory duct obstruction. Most patients are asymptomatic until the third or fourth decade of life when the syndrome is associated with dysuria, perineal pain, infertility, and painful ejaculation. In this report, we present the common imaging findings of this rare developmental anomaly involving the mesonephric duct in a 48-year-old male patient experiencing pelvic pain, recurrent dysuria, and pollakiuria.
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Enfermedades de los Genitales Masculinos , Riñón , Masculino , Humanos , Persona de Mediana Edad , Riñón/diagnóstico por imagen , Riñón/anomalías , Disuria , Vesículas Seminales/diagnóstico por imagen , Vesículas Seminales/anomalías , Conductos Mesonéfricos/diagnóstico por imagen , Conductos Mesonéfricos/anomalías , SíndromeRESUMEN
The abdominal cocoon syndrome is a rare cause of recurring intestinal obstruction in children. It refers to encasement of the small bowel by a fibrocollagenous membrane forming a cocoon. We report a nine year old male presenting with abdominal pain, distension, bilious vomiting and inability to pass stool and flatus for two days. In view of a persistently increasing bilious nasogastric output, an urgent exploratory laparotomy was performed. The small bowel loops were matted together forming a cocoon densely adherent to the parietal peritoneum with supra-colic fibrous bands. The bands histologically displayed multiple ductal remnants with epithelium resembling that of ductus deferens. These structures showed immunopositivity for pan-cytokeratin and basal CD10.Workup for tuberculosis and other etiological causes was unremarkable. This is the first documented case of abdominal cocoon in a pediatric subject associated with supernumerary wolffian remnants.
Asunto(s)
Obstrucción Intestinal/etiología , Intestino Delgado/patología , Conducto Deferente/anomalías , Conductos Mesonéfricos/anomalías , Niño , Humanos , Obstrucción Intestinal/diagnóstico , Obstrucción Intestinal/patología , Masculino , SíndromeRESUMEN
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is associated with congenital absence of the uterus, cervix, and the upper part of the vagina; it is a sex-limited trait. Disrupted development of the Müllerian ducts (MD)/Wölffian ducts (WD) through multifactorial mechanisms has been proposed to underlie MRKHS. In this study, exome sequencing (ES) was performed on a Chinese discovery cohort (442 affected subjects and 941 female control subjects) and a replication MRKHS cohort (150 affected subjects of mixed ethnicity from North America, South America, and Europe). Phenotypic follow-up of the female reproductive system was performed on an additional cohort of PAX8-associated congenital hypothyroidism (CH) (n = 5, Chinese). By analyzing 19 candidate genes essential for MD/WD development, we identified 12 likely gene-disrupting (LGD) variants in 7 genes: PAX8 (n = 4), BMP4 (n = 2), BMP7 (n = 2), TBX6 (n = 1), HOXA10 (n = 1), EMX2 (n = 1), and WNT9B (n = 1), while LGD variants in these genes were not detected in control samples (p = 1.27E-06). Interestingly, a sex-limited penetrance with paternal inheritance was observed in multiple families. One additional PAX8 LGD variant from the replication cohort and two missense variants from both cohorts were revealed to cause loss-of-function of the protein. From the PAX8-associated CH cohort, we identified one individual presenting a syndromic condition characterized by CH and MRKHS (CH-MRKHS). Our study demonstrates the comprehensive utilization of knowledge from developmental biology toward elucidating genetic perturbations, i.e., rare pathogenic alleles involving the same loci, contributing to human birth defects.
Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/genética , Anomalías Congénitas/genética , Conductos Paramesonéfricos/anomalías , Conductos Paramesonéfricos/crecimiento & desarrollo , Mutación , Conductos Mesonéfricos/crecimiento & desarrollo , Adulto , Proteína Morfogenética Ósea 4/genética , Proteína Morfogenética Ósea 7/genética , Codón sin Sentido , Femenino , Estudios de Asociación Genética , Pleiotropía Genética , Proteínas Homeobox A10/genética , Proteínas de Homeodominio/genética , Humanos , Factor de Transcripción PAX8/genética , Herencia Paterna , Penetrancia , Proteínas de Dominio T Box/genética , Factores de Transcripción/genética , Proteínas Wnt/genética , Conductos Mesonéfricos/anomalíasRESUMEN
RESUMEN Los quistes de los conductos de Gartner, generalmente pequeños, benignos y asintomáticos, son vestigios del canal mesonéfrico de Wolff. Representan el 11 % de los quistes vaginales, esta es su localización más frecuente según la literatura consultada. Se presentó un caso operado en el Hospital Militar de Matanzas "Dr. Mario Muñoz Monroy", de localización en la cara posterior del istmo uterino (AU).
ABSTRAC Gartner's duct cyst, mostly little, benign and asymptomatic, are vestiges of the Wolffian mesonephric duct representing 11 % of the vaginal cysts; this location is the most frequently reported and published one up to date. The authors presented the case of a patient who underwent a surgery in the Military Hospital "Dr. Mario Muñoz Monroy¨ with a cyst in the posterior side of the uterine isthmus (AU).
Asunto(s)
Humanos , Femenino , Adulto , Conductos Mesonéfricos/anomalías , Quistes/epidemiología , Útero/anomalías , Conductos Mesonéfricos/cirugía , Ultrasonografía/métodos , Quistes/cirugía , Quistes/diagnósticoRESUMEN
El síndrome de Herlyn-Werner-Wünderlich es una anomalía congénita rara del tracto urogenital que afecta a los conductos de Müller y de Wolff con la tríada útero didelfo, obstrucción de hemivagina y agenesia renal ipsilateral. Las pacientes suelen estar asintomáticas hasta la menarquía, y a partir de entonces la clínica dependerá del grado de obstrucción vaginal, apareciendo frecuentemente masa y dolor pélvico. El diagnóstico suele ser radiológico, siendo de elección la resonancia de abdomen y pelvis que nos confirmará las alteraciones genitourinarias en su totalidad, y el tratamiento es quirúrgico con resección del tabique y drenaje de la vagina obstruida
Herlyn-Werner-Wünderlich syndrome is a rare congenital anomaly of the urogenital tract affecting the Müller and Wolff ducts with the uterus didelphys triad, hemivagina obstruction and ipsilateral renal agenesis. Patients are usually asymptomatic until menarche and thereafter the clinical practice will depend on the degree of vaginal obstruction, with frequently appearing mass and pelvic pain. The diagnosis is usually radiological, with the resonance of abdomen and pelvis chosen that will confirm us the genitourinary alterations in its totality and the treatment is surgical with resection of the septum and drainage of the obstructed vagina
Asunto(s)
Humanos , Femenino , Adolescente , Anomalías Urogenitales/diagnóstico por imagen , Anomalías Urogenitales/cirugía , Dismenorrea/etiología , Anomalías Congénitas/diagnóstico , Conductos Paramesonéfricos/patología , Conductos Mesonéfricos/anomalías , Conductos Mesonéfricos/patología , Hematocolpos/diagnóstico por imagen , Dolor Abdominal/etiología , SíndromeRESUMEN
A 65-year-old male presented with a cystic scrotal mass that fluctuated in size. The cyst was associated with aberrant epididymal tissue and was found to have benign epithelial proliferations, with immunohistochemistry supporting a mesonephric origin (GATA3, CD10, and WT1 positive). Clinical presentation, imaging, and pathologic findings are described.
Asunto(s)
Quistes/diagnóstico , Epitelio/patología , Conductos Mesonéfricos/anomalías , Anciano , Humanos , Masculino , TestículoRESUMEN
INTRODUCTION: Zinner syndrome is a rare developmental anomaly of the Wolffian (mesonephric) duct which is characterized by a triad of obstruction of the ejaculatory duct, the ipsilateral seminal vesicle cyst, and the ipsilateral renal agenesis. Usually is totally asymptomatic, however it can also determine symptoms such as lower urinary tract symptoms, perineal pain, ejaculatory disorders such as painful ejaculation or hematospermia, and infertility. CASE REPORT: We present a case of a 51 years old men with a 3-year history of lower urinary tract symptoms, perineal pain, obstructed defecation, recurrent urinary tract infections and infertility. CT scan showed a voluminous cystic neoformation of the left seminal vesicle, hypoplasia of the left kidney and ipsilateral ureteronephrosis. The mass was removed using laparoscopic "en block" seminal vesiculectomy with associated ipsilateral nephroureterectomy. No post-operative complications occurred. At 2-month post-operative control the patient reported an improvement of urinary and rectal symptoms.
Asunto(s)
Riñón/cirugía , Laparoscopía/métodos , Nefroureterectomía/métodos , Vesículas Seminales/anomalías , Quistes/cirugía , Estudios de Seguimiento , Humanos , Infertilidad Masculina/etiología , Riñón/anomalías , Síntomas del Sistema Urinario Inferior/etiología , Masculino , Persona de Mediana Edad , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Vesículas Seminales/cirugía , Síndrome , Tomografía Computarizada por Rayos X , Infecciones Urinarias/etiología , Conductos Mesonéfricos/anomalíasRESUMEN
INTRODUCTION: Renal agenesis and multicystic dysplastic kidney (MCDK) are usually associated with either an absent or atretic ureter. Occasionally, these renal anomalies may be associated with a dilated tortuous ureter, ureterocele or other cystic malformation of mesonephric duct (MND) remnants. OBJECTIVES: The objective of this study was to identify and classify anatomical variants of tubulocystic remnants of the MND, with a secondary focus on natural history and management outcomes. PATIENTS AND METHODS: A retrospective cohort study of patients seen in the study institution between 2007 and 2014 with a tubulocystic abnormality of MND structures associated with either MCDK or renal agenesis was conducted. Medical imaging and progress notes were reviewed for all patients. Data collected included anatomical information, surgical intervention, histology and outcomes of both conservatively managed and surgically resected MND remnant structures. RESULTS: Nineteen patients were identified, 5 girls and 14 boys. Median age at presentation was 4.6 years. Anomalies of the MND occurred on the left in 9 patients and on the right in 10 patients. Mean follow-up was 3.4 years. Patients fell into 3 distinct anatomical groups: Type I, including orthotopic remnants corresponding to ureteric bud structures (ureter and trigone); Type II, including ectopic MND remnants of ureteric bud structures, and Type III, including complex remnants corresponding to MND structures other than those from ureteric bud (vas, epididymis and seminal vesicles). Anomalies of structures arising from urogenital sinus and paramesonephric ducts were also identified. Most patients were asymptomatic and successfully managed conservatively. Transvesical puncture of trigonal cysts provided effective decompression in 5 patients. Partial or complete MCDK regression was seen in 7 patients, whereas MND cystic anomalies did not regress spontaneously. DISCUSSION: When MND tubulocystic structures persist along with renal agenesis or MCDK, most arise from ureteric bud structures in an orthotopic position as a ureterocele with or without a blind-ending ureter-like structure. Less commonly, ureteric bud structures insert ectopically into the urogenital tract, or tubulocystic structures arising from the remainder of the MND occur. Embryogenesis of other urogenital structures may also be abnormal, and conditions such as Zinner syndrome and obstructed hemivagina and ipsilateral renal agenesis syndrome should be considered. CONCLUSIONS: Complications are uncommon, and surgical intervention should be limited to symptomatic patients. Remnants of metanephric blastema may involute, but MND remnants persist.
Asunto(s)
Anomalías Múltiples , Riñón/anomalías , Riñón Displástico Multiquístico/complicaciones , Conductos Mesonéfricos/anomalías , Anomalías Múltiples/diagnóstico , Anomalías Múltiples/cirugía , Preescolar , Estudios de Cohortes , Femenino , Humanos , Riñón/cirugía , Masculino , Riñón Displástico Multiquístico/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Conductos Mesonéfricos/cirugíaRESUMEN
The epithelial Wolffian duct (WD) inserts into the cloaca (primitive bladder) before metanephric kidney development, thereby establishing the initial plumbing for eventual joining of the ureters and bladder. Defects in this process cause common anomalies in the spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). However, developmental, cellular, and molecular mechanisms of WD-cloaca fusion are poorly understood. Through systematic analysis of early WD tip development in mice, we discovered that a novel process of spatiotemporally regulated apoptosis in WD and cloaca was necessary for WD-cloaca fusion. Aberrant RET tyrosine kinase signaling through tyrosine (Y) 1062, to which PI3K- or ERK-activating proteins dock, or Y1015, to which PLCγ docks, has been shown to cause CAKUT-like defects. Cloacal apoptosis did not occur in RetY1062F mutants, in which WDs did not reach the cloaca, or in RetY1015F mutants, in which WD tips reached the cloaca but did not fuse. Moreover, inhibition of ERK or apoptosis prevented WD-cloaca fusion in cultures, and WD-specific genetic deletion of YAP attenuated cloacal apoptosis and WD-cloacal fusion in vivo Thus, cloacal apoptosis requires direct contact and signals from the WD tip and is necessary for WD-cloacal fusion. These findings may explain the mechanisms of many CAKUT.
Asunto(s)
Apoptosis/genética , Cloaca/embriología , Quinasas MAP Reguladas por Señal Extracelular/metabolismo , Proteínas Proto-Oncogénicas c-ret/genética , Anomalías Urogenitales/genética , Conductos Mesonéfricos/embriología , Proteínas Adaptadoras Transductoras de Señales/genética , Animales , Proteínas de Ciclo Celular , Cloaca/anomalías , Cloaca/metabolismo , Riñón/embriología , Sistema de Señalización de MAP Quinasas , Ratones , Mutación , Fosfoproteínas/genética , Proteínas Proto-Oncogénicas c-ret/metabolismo , Uréter/embriología , Conductos Mesonéfricos/anomalías , Conductos Mesonéfricos/metabolismo , Proteínas Señalizadoras YAPRESUMEN
We investigate a patient with right kidney agenesis. Imaging showed the presence of a cystic mass dislocating the bladder. The specimen showed three formations: a kidney remnant, a ureter with blind-ending branch, and a cyst, from which departed another tubular structure, considered the deferential duct. The specimen was sampled. The supposed kidney was formed by cystic structures. Examination of the tubular structures disclosed smooth muscle fibers with no lumen, whereas the cyst was surrounded by fibrous and hemorrhagic walls. Collaboration among specialists allowed the diagnosis of Zinner syndrome, a congenital malformation due to an abnormal development of the Wolffian duct.
Asunto(s)
Anomalías Congénitas/diagnóstico , Inmunohistoquímica/métodos , Enfermedades Renales/congénito , Riñón/anomalías , Imagen por Resonancia Magnética/métodos , Tomografía Computarizada por Rayos X/métodos , Conductos Mesonéfricos/anomalías , Adulto , Anomalías Congénitas/metabolismo , Anomalías Congénitas/cirugía , Humanos , Riñón/metabolismo , Riñón/cirugía , Enfermedades Renales/diagnóstico , Enfermedades Renales/metabolismo , Enfermedades Renales/cirugía , Masculino , Síndrome , Procedimientos Quirúrgicos Urológicos Masculinos/métodos , Conductos Mesonéfricos/diagnóstico por imagen , Conductos Mesonéfricos/cirugíaRESUMEN
STUDY OBJECTIVE: Gartner duct cysts (GDCs) are rare embryological remnants of the mesonephric duct with the majority of cases discovered incidentally in asymptomatic patients. The largest prior published series evaluating the surgical management of GDCs included 4 patients. The present study aimed to determine the manifestations and outcomes of surgically managed patients with GDCs with important implications for surveillance, monitoring, and management. DESIGN: A retrospective chart review (Canadian Task Force classification III). SETTING: A tertiary care center. PATIENTS: All women diagnosed with GDCs from January 1994 to April 2014 at our institution were identified. Patients were included if they underwent surgical management and had GDCs confirmed by pathology. One hundred twenty-four charts were manually reviewed, and 29 patients were included in the analysis. INTERVENTIONS: All patients underwent surgical management, which included vaginal excision or marsupialization. MEASUREMENTS AND MAIN RESULTS: A total of 29 patients met the inclusion criteria for this study. The median age of the patients included in the analysis was 36 years old. Eleven patients were asymptomatic at the time of diagnosis (37.9%). The reason for surgical intervention was not available in 9 of these patients. Surgical intervention was performed in 2 of the 11 asymptomatic patients because of an increasing size of the lesion during observation. Presenting symptoms included dyspareunia or pain with tampon placement (37.9%), pelvic pain or pressure (24.1%), pelvic mass or bulge (17.2%), and urinary incontinence (6.9%). Preoperative imaging studies were obtained in 62% of patients; ultrasound was used in 44.4%, computed tomographic scanning in 22.2%, magnetic resonance imaging in 16.7%, and multiple modalities in 16.7%. Approximately 10% were found to have other genitourinary anomalies, including a bladder cyst, urethral diverticulum, and a solitary right kidney with uterine didelphis and septate vagina. The average cyst size was 3.5 cm (±1.8 cm). Surgical excision of GDCs was performed in all except for 3 cases of marsupialization. No intraoperative complications occurred. The median follow-up was 82 months (range, 0-246 months). One patient had possible recurrence with dyspareunia and protruding tissue diagnosed 14 months postoperatively. There were no other postoperative complications in the follow-up period. CONCLUSION: GDCs are rare pelvic masses that are often asymptomatic but may present with dyspareunia, pelvic pain or pressure, pelvic mass or bulge, or urinary symptoms. Excision or marsupialization is successful in the majority of cases without significant morbidity.
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Quistes/cirugía , Enfermedades de los Genitales Femeninos/cirugía , Anomalías Urogenitales/cirugía , Conductos Mesonéfricos/anomalías , Adulto , Anciano , Quistes/complicaciones , Dispareunia/etiología , Femenino , Enfermedades de los Genitales Femeninos/complicaciones , Enfermedades de los Genitales Femeninos/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Dolor Pélvico/etiología , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Incontinencia Urinaria/etiología , Anomalías Urogenitales/complicaciones , Anomalías Urogenitales/diagnóstico por imagen , Útero/anomalías , Conductos Mesonéfricos/cirugía , Adulto JovenRESUMEN
A 10-month-old female toy poodle was referred to the University of Tokyo Veterinary Medical Center with a urogenital anomaly found during sterilization. An exploratory laparotomy revealed a cyst adhering to the cervix and a unilateral renal agenesis. Histopathology and immunohistochemical analysis of the cyst was consistent with remnants of the Wolffian duct or a Gartner duct cyst. This is a rare case of a canine Gartner duct cyst with renal agenesis and uterine anomaly. We discuss the similarity of this case to that of humans and introduce a classification in the literature for these complex urogenital malformations for further clinical research into the precise diagnosis and appropriate surgical planning.
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Quistes/veterinaria , Enfermedades de los Perros/congénito , Riñón/anomalías , Anomalías Urogenitales/veterinaria , Enfermedades Uterinas/veterinaria , Conductos Mesonéfricos/anomalías , Anomalías Múltiples/veterinaria , Animales , Quistes/patología , Quistes/cirugía , Perros , Femenino , Histerectomía/veterinaria , Enfermedades Renales/congénito , Enfermedades Renales/veterinaria , Ovariectomía/veterinaria , Anomalías Urogenitales/patología , Enfermedades Uterinas/congénitoRESUMEN
BACKGROUND: In women, during embryologic development, the paired Müllerian (paramesonephric) ducts fuse distally and develop into the uterus, cervix, and upper vagina. If the Wolffian ducts persist in vestigial form, they can lead to Gartner's cysts, mainly located in the right wall of the vagina. This is one of the few studies of Gartner's cysts with a series of consecutive cases over a long period of time who were exclusively subject to clinical observation. Although Gartner's cysts are found in approximately 0.1 to 0.2% of women, controversy exists regarding the course of action to be taken. CASE PRESENTATION: We describe the cases of four women who were 38-years old, 53-years old, 37-years old, and 49-years old at their first appointment and who were of mixed ethnicity, mixed ethnicity, black, and mixed ethnicity respectively. The follow-up of these patients ranged from 2 to 17 years. In these four cases the location of the cysts was the right wall of the vagina. Transvaginal ultrasound was the test of choice for diagnostic confirmation. In the cases presented in this study, the women were asymptomatic and chose to be observed clinically. CONCLUSIONS: This is the first study reporting long-term clinical observation of these lesions. This study shows that conservative treatment can be a safe option for asymptomatic patients with vaginal Gartner's duct cysts.
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Tratamiento Conservador , Quistes/terapia , Enfermedades Vaginales/terapia , Conductos Mesonéfricos/anomalías , Adulto , Enfermedades Asintomáticas , Quistes/diagnóstico por imagen , Femenino , Humanos , Persona de Mediana Edad , Ultrasonografía , Enfermedades Vaginales/diagnóstico por imagen , Espera Vigilante , Conductos Mesonéfricos/diagnóstico por imagenRESUMEN
Epididymal agenesis is defined as the absence of the epididymis totally or segmentally, unilateral or bilateral, which is secondary to the Wolffian duct malformation (1). Rete testis, epididymis, vas deferens and seminal vesicle are believed to develop from Wolffian ducts.
Asunto(s)
Epidídimo/anomalías , Enfermedades de los Genitales Masculinos/etiología , Conductos Mesonéfricos/anomalías , Adulto , Epidídimo/cirugía , Enfermedades de los Genitales Masculinos/cirugía , Humanos , Masculino , Conductos Mesonéfricos/cirugíaRESUMEN
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Müllerian duct anomalies with mesonephric duct anomalies. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. Patients generally present with non-specific symptoms after menarche. Pelvic pain, dysmenorrhea, and palpable mass due to hematocolpos or hematometra are the most common findings. Pyohematocolpos and pyosalpinx may appear as acute complications, while endometriosis and pelvic adhesions constitute potential long-term complications. When a prenatal diagnosis of unilateral renal agenesis in newborn girls is known, a gynecological imaging study should be performed to exclude uterine and vaginal abnormalities. These patients should be followed up to ensure that a timely surgical correction is performed. The diagnosis of HWWS is difficult due to the lack of specific symptoms or findings upon physical examination. An accurate imaging description of these congenital anomalies is crucial to guide patients toward surgical treatment, relieving acute complications, and preserving the normal fertility. The authors provide a pictorial review of the magnetic resonance imaging and ultrasonography findings of the HWWS with correlation to embryological, clinical, and surgical features.
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Imagen por Resonancia Magnética , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/patología , Cuidados Preoperatorios , Anomalías Urogenitales/diagnóstico por imagen , Anomalías Urogenitales/patología , Anomalías Congénitas/diagnóstico por imagen , Anomalías Congénitas/patología , Anomalías Congénitas/cirugía , Femenino , Humanos , Riñón/anomalías , Riñón/diagnóstico por imagen , Riñón/patología , Riñón/cirugía , Enfermedades Renales/congénito , Enfermedades Renales/diagnóstico por imagen , Enfermedades Renales/patología , Enfermedades Renales/cirugía , Conductos Paramesonéfricos/anomalías , Conductos Paramesonéfricos/diagnóstico por imagen , Conductos Paramesonéfricos/patología , Síndrome , Ultrasonografía , Anomalías Urogenitales/cirugía , Conductos Mesonéfricos/anomalías , Conductos Mesonéfricos/diagnóstico por imagen , Conductos Mesonéfricos/patologíaRESUMEN
Epididymal agenesis is defined as the absence of the epididymis totally or segmentally, unilateral or bilateral, which is secondary to the Wolffian duct malformation (1). Rete testis, epididymis, vas deferens and seminal vesicle are believed to develop from Wolffian ducts.