Zinner's Syndrome: Case report of a Developmental Anomaly of the Mesonephric Duct.

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.

Dedifferentiated Mesonephric-like Adenocarcinoma of the Uterine Corpus.

BACKGROUND/AIM: We present a case of uterine dedifferentiated mesonephric-like adenocarcinoma (MLA). CASE REPORT: A 54-year-old woman underwent total hysterectomy for a uterine mass under the impression of a uterine sarcoma. Histologically, MLA exhibited various growth patterns including tubular and glandular architecture. Undifferentiated carcinoma (UC) displayed discohesive tumor cells without any obvious architecture. Immunohistochemically, UC was positive for epithelial markers in very few scattered tumor cells. MLA exhibited the wild-type p53 expression pattern, whereas UC showed a uniform and strong p53 immunoreactivity. Targeted sequencing analysis revealed an identical Kirsten rat sarcoma viral oncogene homolog (KRAS) mutation in both components. A pathogenic missense tumor protein 53 (TP53) mutation was detected in UC, but not in MLA. CONCLUSION: The mutant p53 expression pattern exclusively detected in UC was concordant with the presence of missense TP53 mutation. Our observations suggested that TP53 mutation is associated with the possible transformation from MLA to UC.

Hiperplasia mesonéfrica difusa: a propósito de un caso / Diffuse mesonephric hyperplasia: a case report

La persistencia de restos derivados del mesonefros suele presentarse en las paredes laterales del cérvix de forma frecuente. En cambio, la evolución hacia una hiperplasia mesonéfrica o un carcinoma mesonéfrico es muy poco frecuente. Presentamos a una paciente de 50 años, intervenida de histerectomía por útero miomatoso, en cuya pieza quirúrgica se describe una hiperplasia mesonéfrica difusa. La presencia de restos procedentes del mesonefros o hiperplasia, constituyen en casi la totalidad de las ocasiones un hallazgo benigno que no precisa tratamiento. Es necesario conocer las características de este tipo de lesiones derivadas del mesonefros, para evitar tratamientos innecesarios

The persistence of mesonephric remnants is often present on the side walls of the cervix. However, the evolution towards mesonephric hyperplasia or mesonephric carcinoma is very rare. The case is presented of a 50-year-old female patient, who underwent hysterectomy for myomatous uterus, and in which surgical specimen was described a diffuse mesonephric hyperplasia. The presence of remains from mesonephros or hyperplasia is almost always a benign finding that does not require treatment. It is necessary to know the characteristics of this type of lesion derived from mesonephros, in order to avoid unnecessary treatments

Mayer-Rokitansky-Küster-Hauser syndrome with rare findings of inferior crossed-fused renal ectopia and Gartner's duct cyst: a video case report.

OBJECTIVE: To describe the treatments of a patient using the laparoscopic Davydov's method for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and ureteral reimplantation for hydronephrosis and hydroureter. DESIGN: Surgical video article. A consent form from the patient was obtained as appropriate; the nature of the study did not necessitate ethics committee approval. There were no conflicts of interest. SETTING: University hospital. PATIENT(S): A 28-year-old woman who presented at our gynecology department with the symptoms of primary amenorrhea and difficult intercourse. She had repaired congenital rectovestibular fistula and imperforate anus at the age of 8. At physical examination, she had a phenotypically normal vulva with a vaginal small pouch (0.5 cm). Magnetic resonance imaging of the pelvis revealed normal ovaries, a primordial uterus, absence of vaginal canal, and a 4.0 × 4.2 × 4.0 cm cystic structure posterior to the bladder. Magnetic resonance urography showed right to left renal crossed-ectopia with inferior fusion, and hydronephrosis and hydroureter from the superior kidney with Grade Ⅳ vesicoureteral reflux. Karyotype was 46, XX. INTERVENTION(S): Saline solution 300 mL was injected into the rectovesical space with an infusion of diluted adrenaline (1:200,000). The goal of this injection was to aid in the identification of tissue planes and reduce blood loss. The space between urethra/bladder and rectum progressively was dissected. Blunt dissection was performed initially with digital separation of tissues. Then, an 8-cm-long neovaginal vault of about 3 cm in diameter was created. The mobilized peritoneum was pulled downward with eight Vicryl sutures and connected to the vaginal epithelium. By cystoscope, we found the left orifice but could not find the right orifice of the hydroureter. Then we ligated the hydroureter by 2-0 absorbable suture near the cyst and cut off the hydroureter, and then incised of all the layers at the top of the bladder to make a bladder flap. We placed the 5 Fr double J stent in the hydroureter and the bladder and anastomozed with the ureteral stump (3-0 Vicryl). Then we removed the cyst laparoscopically. We performed a purse-string stitch to create the apex of the neovagina by taking posterior serosa of the bladder, the pelvic peritoneum between the ovary and rectum, primordial uterus, and anterior rectal serosa. MAIN OUTCOME MEASURE(S): Measurement of the final canal length, sexual function (Female Sexual Function Index), and degree of hydronephrosis. RESULT(S): Three days later, we started to change the vaginal mold and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. Findings confirmed the cyst was Gartner's duct cyst. One year after the surgery, the final canal length was 9 cm and Female Sexual Function Index score was 28. The ultrasound showed that the degree of hydronephrosis of upper moiety was mild. CONCLUSION(S): The distal Wolffian ducts in the female are absorbed but may persist as vestigial remnants (Gartner's duct cysts). A few cases of the combined urogenital-Wolffian anomalies are reported; most of them are associated with the anomalies of müllerian duct fusion, such as Herlyn-Werner-Wunderlich syndrome (uterus didelphys, obstructed hemivagina, and mesonephric duct anomalies). The embryogenesis of the combined anomalies is not completely understood. With comprehensive preoperative assessments, laparoscopic surgery could be a safe and effective treatment to these cases.

Gartner's Duct Cyst of the Vagina: A Case Report.

Paramesonephric duct or Mullerian ducts forms female genital organs whereas mesonephric duct forms male genital organs. The remnant of the mesonephric duct or Wolffian duct in females sometimes forms a mesonephric cyst or Gartner's duct cyst. They are usually asymptomatic and <2 cm but sometimes can be bigger. It is diagnosed with pelvic examination. It is treated with surgical excision of the cyst. This is a unique case in urogynecology as it confuses with pelvic organ prolapse and the mode of treatment is completely different. We report a case of 32-years old lady who presented in urogynecology outpatient department with complain of pelvic organ prolapse. After examination she was diagnosed as vaginal cyst and excision was done and confirmed as Gartners cyst in histopathological examination.

Ultrasound in the assessment of pelvic organ prolapse.

Imaging is increasingly being used in urogynaecology. Because of low cost and universal availability, ultrasound (US) is the most commonly used diagnostic modality, which allows the observation of manoeuvres such as Valsalva and pelvic floor muscle contraction in real time. The ability to see beyond surface anatomy is particularly important in the posterior compartment and in obstructed defecation where this method may replace defecation proctography. Imaging is especially useful in the form of 3D/4D multiplanar and tomographic translabial US, as these modalities give access to the axial plane and the levator ani. This allows assessment of both avulsion, i.e. major maternal birth trauma, and hiatal overdistension, i.e. ballooning. Both are major risk factors for both prolapse and prolapse recurrence. This review will outline current clinical utility, introduce recent research in the respective field and provide an overview of likely future utility of imaging in the investigation of pelvic organ prolapse.

A Gartner Duct Cyst Masquerading as Anterior Vaginal Prolapse.

BACKGROUND: Gartner duct cysts are embryologic remnants of the mesonephric ducts that may present as a vaginal mass or cyst. CASE: A patient was referred for surgical management of prolapse whose bulging anterior vagina was actually a Gartner duct cyst that required excision as a result of symptoms. Preoperative magnetic resonance imaging helped confirm the diagnosis. CONCLUSION: A Gartner duct cyst may present as a vaginal cyst that, if large, may mimic pelvic organ prolapse. The diagnosis should be considered when a patient's individual prolapse compartments are inconsistent or when physical examination is suggestive of another process.

Zinner Syndrome: A Diagnostic Challenge. The Aid of Morphology, Embryology, and Immunohistochemistry.

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.

Neonatal Gartner Duct Cyst: Two Case Reports and Literature Review.

Vaginal cysts are rare, particularly in the newborn. They usually present as one of these three entities in the newborn: paraurethral cysts (Skene duct cysts), Gartner duct cysts (mesonephric ductal remnants) or a covered ectopic ureter. Abdominal ultrasound should always be included in the clinical evaluation in search of renal anomalies. We report two cases of Gartner cysts in neonates.

Conservative treatment and follow-up of vaginal Gartner's duct cysts: a case series.

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.

Herlyn-Werner-Wunderlich syndrome: pre- and post-surgical MRI and US findings.

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.

A modified approach of cystic puncture with contrast medium injection for diagnosis of obstructed hemivagina and urinary tract anomalies.

This study investigated the diagnostic value of a modification of the conventional technique of X-ray imaging after cystic puncture with injection of contrast medium for obstructed hemivagina and related urinary tract anomalies in girls. The modified procedure made the following diagnostic findings: ipsilateral obstructed hemivagina in one patient with renal agenesis; vaginal ectopic ureter and ipsilateral obstructed hemivagina in one patient with renal dysplasia; and vaginal ectopic ureter, Gartner's duct cyst and ipsilateral obstructed hemivagina in six patients with renal dysplasia or aplasia. This modified method might have improved diagnostic value over the traditional method, and accurately identify genitourinary tract anomalies. It could therefore serve as an alternative and complementary method of sonography.

Mesonephric remnant with tubular function in a 15-year-old female.

Congenital abnormalities of the genital or urinary tract are not uncommon and often occur together. This article discusses a unique case in which a functioning mesonephric remnant was found.

Diagnostic laparoscopy in a Gartner's duct cyst.

Gartner's duct cysts associated with renal dysgenesis are rare malformations and represent a diagnostic challenge. We report on one such case in which final diagnosis was achieved by laparoscopy and discuss the possible role of minimally invasive surgery in the management of this condition.

Diagnosis and treatment for persistent Gartner duct cyst in an infant: A case report.

Persistent Gartner duct cysts are extremely rare in infants. The authors describe the case of an infant with persistent Gartner duct cysts and discuss its management.

Recurrent giant Gartner's duct cysts. A report of two cases.

BACKGROUND: Gartner's duct cysts are cystically dilated wolffian duct remnants found in the upper anterolateral part of the vagina. Many such giant cysts are diagnosed during childhood and result from ectopic communication with the ureter or cervix. There is a paucity of literature on recurrent and giant cysts presenting among older women. CASES: A 43-year-old woman presented in 1981 with a 7 x 14-cm, left, paravaginal, cystic mass. This was initially drained vaginally, then marsupialized vaginally. Following marsupialization, the patient began to note large gushes of fluid from the vagina. Ultrasound demonstrated a 3-cm cyst thought to arise within the broad ligament. The patient required total abdominal hysterectomy/bilateral salpingo-oophorectomy for endometrial hyperplasia. Exploration revealed neither a broad ligament nor vaginal mass. Postoperatively, vaginal drainage continued. Computed tomography demonstrated a multiloculated, cystic mass left of the vaginal cuff. Exploratory laparotomy revealed the mass to be within the paravaginal space. The cyst was marsupialized into the peritoneal cavity. A 32-year-old woman was diagnosed in 1992 with an 8 x 10-cm right pelvic mass found on examination and confirmed by computed tomography. At exploratory laparotomy the mass was found to be within the paravaginal space and was resected vaginally. In 1999 the patient returned, complaining of rectal pain. Examination and ultrasound revealed a right, multiloculated pelvic mass displacing the rectum, uterus and vagina. Magnetic resonance imaging demonstrated that the mass was entirely inferior to the levator plate. The cyst was resected vaginally. CONCLUSION: Giant Gartner's cysts tend to be misdiagnosed as pelvic masses. Magnetic resonance imaging is the best imaging modality for localizing these cysts. Recurrences of giant cysts tend to be multiloculated. Management strategies for multiloculated recurrences include periodic surveillance, schlerotherapy and marsupialization into the peritoneal cavity.

Diagnosing the combination of renal dysgenesis, Gartner's duct cyst and ipsilateral müllerian duct obstruction.

PURPOSE: We describe the differential points in the diagnosis of the combination of renal dysgenesis, Gartner's duct cyst and ipsilateral müllerian duct obstruction. Various imaging studies and urological procedures were performed. We report our experience in detecting these anomalies in 10 girls and review the literature. MATERIALS AND METHODS: Ten girls, 7 to 13 years old, with this combination of anomalies were identified in the last 10 years. Imaging studies as well as urological procedures were selectively performed, especially at puberty following menarche. Patients received long-term followup with ultrasound. RESULTS: Cystic dilation of Gartner's duct protruded into the bladder and presented as a ureterocele in 5 patients and posterior to the bladder in 5. Surgical removal of a partial portion of a Gartner's duct cyst was performed in 5 patients for alleviation of urinary symptoms. Unilateral müllerian duct obstruction was demonstrated in all 10 patients. Excision of the vaginal septum was performed in 6 patients for relief of genital obstruction. CONCLUSIONS: When cystic dilatation of the pelvis, especially a ureterocele-like cyst without ureteral dilatation, is found in girls with ipsilateral renal dysgenesis, the possibility of a Gartner's duct cyst should be considered. For early detection and treatment of unilateral obstruction of duplicated müllerian ducts pelvic sonography should be performed at puberty, especially just after menarche, in girls with renal dysgenesis and ipsilateral Gartner's duct cyst.

Gartner's duct cyst with a single vaginal ectopic ureter and associated renal dysplasia or agenesis.

Two cases of Gartner's duct cyst with vaginal ectopic ureter and associated renal anomalies are reported. This unusual wolffian duct anomaly may be more common than previously suspected, especially in Asian patients. The radiologist and ultrasonographer should be aware of this anomaly and should perform a pelvic sonogram in any patient in whom there appears to be an absent or dysplastic kidney. Care should be taken to try and identify the Gartner's duct cyst extending caudally posterior to the base of the urinary bladder.