Mullerian Duct Remnants Research Articles

Neophallus and Urethroplasty Revisions: Step-by-step Surgical Techniques and Key Reconstructive Maneuvers

Abstract Objective: The aim of this video is to demonstrate step-by-step surgical techniques for neophallus and urethroplasty revisions, emphasizing key reconstructive maneuvers and improving patient outcomes. Materials and Methods: Neophallus and urethroplasty revisions can be challenging procedures associated with complications. We present a 25-year-old male with a history of ambiguous genitalia and micropenis due to 17-hydroxylase deficiency. The patient presented with bothersome penile skin redundancy at the base of the neophallus and a history of intermittent urinary incontinence, necessitating “milking the base of the neophallus” to completely evacuate his urethra. The patient also reported foul-smelling discharge with urine. Imaging evaluation, including retrograde urethrogram and voiding cystogram, revealed a smooth cystic dilatation of the posterior urethra with a normal bladder contour and capacity. Further evaluation with cystoscopy identified a Mullerian duct remnant in the urethra. We recommend surgical excision of the remnant and neophallus skin revision using intraoperative SPY Indocyanine green to carefully delineate the blood supply to the neophallus and mitigate potential risks and complications. Results: This paper demonstrates step-by-step surgical techniques for neophallus and urethroplasty revisions, highlighting key surgical maneuvers such as using intraoperative SPY Indocyanine green to delineate blood supply, excision of the Mullerian duct cystic lesion, and urethroplasty. These techniques aim to improve patient outcomes by addressing penile skin redundancy and urinary incontinence while minimizing potential complications. Conclusion: As neophallus reconstruction becomes increasingly common, familiarity with surgical anatomy, complications management, and outcomes is essential. This video provides a valuable resource for surgical education and may aid in the improvement of patient outcomes.

Robot-Assisted Excision of Persistent Mullerian Duct Remnants: A New Standard of Care?

Robot-Assisted Excision of Persistent Mullerian Duct Remnants: A New Standard of Care?

Infected Ciliated Cyst of the Vulva in an Adolescent Patient: A Case Report

Background: Ciliated cyst of the vulva is a rare benign entity and is a variant of cutaneous ciliated cyst. Mullerian duct remnants, another rare entity that is even more rarely on the vulva, is considered in the differential diagnosis. To our knowledge, this is the first case of a sepsis originating from a ciliated cyst of the vulva.

V02-09 ROBOTIC EXTRAVESICAL MULLERIAN DUCT REMNANT EXCISION

You have accessJournal of UrologyMisc. Benign Disease & Transplant & Renovascular (V02)1 Sep 2021V02-09 ROBOTIC EXTRAVESICAL MULLERIAN DUCT REMNANT EXCISION Laura Kidd, Chinonyerem Okoro, and Daniel Eun Laura KiddLaura Kidd More articles by this author , Chinonyerem OkoroChinonyerem Okoro More articles by this author , and Daniel EunDaniel Eun More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000001979.09AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: We present a 13-year-old male with history of mixed gonadal dysgenesis who presented with abdominal pain and urinary frequency. He was admitted for work-up, which revealed a negative urine culture but a 13.9 cm Mullerian duct remnant (MDR). The patient underwent cystoscopic drainage, which provided temporary relief, but seven months later his pain recurred and he developed gross hematuria. Repeat imaging showed the same MDR, now 10 cm in size. He was referred for robotic surgical repair. METHODS: Cystourethroscopy showed a mound of heaped of tissue at 6 o’clock in the prostatic urethra, corresponding to the opening of the remnant. This was not cannulated, to avoid draining the remnant prior to its dissection. The patient was placed in steep Trendelenburg and a three-port configuration was used (right, camera, left), with no assistant port. The bladder was filled and the vesicouterine plane was carefully developed, taking care to control any encountered vessels feeding the MDR. The remnant was dissected down to its neck. Near infrared fluoroscopy (NIF) and white light from the cystoscope in the urethra was used to confirm the exact insertion point. This was also visualized by the bedside assistant in the cystoscopic view, with gentle prodding from the robotic instrument. The MDR was then amputated at the insertion point and the defect was closed in a single layer. Intraoperative cystoscopy confirmed excellent closure with no luminal narrowing or back-wall suture bites. A coude catheter was placed to end the case. RESULTS: Estimated blood loss was 50cc, total operative time was 97 minutes. No complications were encountered. The patient was discharged on post-operative day 0. Pathology was consistent with Mullerian duct remnant. Catheter was removed at 2 weeks and he continues to do well at 2-month follow-up. CONCLUSIONS: Mullerian duct remnants are rare and presenting symptoms can vary. Endoscopic management can be attempted, but failure often necessitates more definitive repair. This can safely be performed robotically, and intraoperative urethroscopy can help identify the urethral insertion point if cannulation of the remnant is not, or cannot be, performed pre-operatively. In the limited literature, as well as in our experience, perioperative outcomes are favorable. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e136-e136 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Laura Kidd More articles by this author Chinonyerem Okoro More articles by this author Daniel Eun More articles by this author Expand All Advertisement Loading ...

Magnitude of post-urethroplasty urinary tract infections in children with hypospadias at a tertiary hospital in Kenya

Children who have undergone urethroplasty procedure for hypospadias are at a higher risk of getting urinary tract infections (UTI). This may be due to urethral scarring following urethroplasty that acts as a nidus for UTI. The risk is also attributed to urine stasis due to complications of urethroplasty such as urethral diverticulum, urethral stricture, meatal stenosis or breakdown of urethroplasty. Hypospadias is associated with mullerian duct remnants such as mullerian duct cysts and enlarged prostatic duct utricle which may lead to recurrent UTI. To determine the magnitude of urinary tract infections after urethroplasty for hypospadias in children at Kenyatta National Hospital (KNH), a tertiary hospital in Kenya. This was a prospective descriptive cross-sectional study of male children with hypospadias who had undergone urethroplasty between January 2014 to December 2018 (5years) at KNH with age range from 6 months to 18 years. Appropriate urine collection method was used to obtain the urine specimen for microscopy, culture and sensitivity tests. Data collected was analyzed using STATA 15. Significance of the results was considered at 95% confidence interval. The total number of patients seen and urine samples collected was 83 boys. The prevalence of UTI following urethroplasty for children with hypospadias was 6% (5/83). They all had UTI symptoms. Of the patients who had UTI, 60% was due to Enterobacter coli, 20% Pseudomonas aeruginosa and 20% Enterobacter cloaca complex. E.coli was 100% sensitive to nitrofurantoin, amoxicillin/clavulanic acid and cefazolin while 33% resistant to ciprofloxacin and Trimethoprim/sulfamethoxazole. Majority of the patients with UTI (80%) had penoscrotal hypospadias and 60% of them developed complications post-operatively. Urinary tract infections is not a common finding in children who have undergone urethroplasty for hypospadias at KNH. Patients with penoscrotal hypospadias appears to be at a higher risk of developing UTI in our institution with E. Coli being the commonest bacteria implicated. Antibiotic protocol should be based on local culture and sensitivity pattern.

SUN-074 Loss-Of-Function Mutations in GATA4 in Patients with 46,XY Disorders of Sex Development Without Cardiac Defects

Background: Disorders of sex development (DSD) encompass a wide range of conditions associated with numerous causative genes. In about 50-60% of 46,XY DSD individuals, the underlying molecular cause remains uncertain. GATA4 haploinsufficiency has been described in patients with congenital heart defects (CHD), while only a few studies reported mutations related to 46,XY DSD phenotype. This study investigated clinical phenotypes and molecular characteristics of two 46,XY DSD patients with GATA4 mutations. Methods: Mutation analysis was performed in patients with 46,XY DSD by whole exome sequencing (WES) using Illumina NextSeq platform. Clinical and endocrine characteristics were reviewed retrospectively. GATA4 variants identified by WES were verified by Sanger sequencing. Functional activity of GATA4 variants was tested by luciferase reporter assay on the SRY and AMH promoter using two different cell systems including HEK293 and NCI-H295R. Results: Subject 1 presented with micropenis and hypospadias at the age of 5 months. Karyotype was 46,XY. Mullerian duct remnants were not found in pelvic ultrasound. The patient underwent urethroplasty at the age of 10 months and was reared as a male. Subject 2 with complete female external genitalia was referred to our hospital because of 46,XY karyotype on G-scanning. The patient underwent laparoscopic orchiectomy at the age of 1.8 years and was assigned as a female. Both patients were responsive to hCG stimulation tests and did not have CHD. Subject 1 harbored a novel heterozygous variant of c.643A>G (p.R215G)] in GATA4, whereas a previously reported variant of c.1220C>A (p.P407Q) was identified in Subject 2. In vitro luciferase reporter assays using SRY and AMH promoter revealed decreased transcriptional activity of both p.P407Q and p.R215G. Conclusions: This study expanded phenotypic spectrum of mutations in GATA4 in patients with 46,XY DSD without CHD. GATA4 mutations in patients with 46,XY DSD may not be associated with CHD. Possible explanations for phenotypical variability comprise incomplete penetrance, variable expressivity, and oligogenic mechanisms.

LAPAROSCOPIC MANAGEMENT OF A GIANT PROSTATIC UTRICLE: A CASE REPORT AND REVIEW OF LITERATURE

Prostatic utricle is a Mullerian duct remnant with an incidence of 1 %. Excision of utricle is challenging because of the close proximity of seminal vesicle, ejaculatory ducts, bladder, rectum, ureter, and nerve plexus. Here, we report the case of a 23-year-old male presented with complaints of painful terminal hematuria associated with clots along with retrograde ejaculation. The abdominal and local examination was within normal limits. MRI pelvis showed a large non-communicating cystic structure present in the pelvic cavity, compressing posterior wall of the urinary bladder, seminal vesicle, anterior wall of the rectum and also causing left hydroureteronephrosis. After evaluation, the patient was diagnosed with giant prostatic utricle cyst. Laparoscopic excision of prostatic utricle cyst was done successfully. Postoperative period was uneventful and the patient was discharged in satisfactory condition. Laparoscopic excision of prostatic utricle cyst is technically challenging but with acceptable complications and good surgical results.

Primary peritoneal carcinosarcoma arising from the secondary Müllerian system: case report and literature review

Carcinosarcomas, also known as malignant mixed Mullerian tumours (MMMT), are aggressive neoplasms that are biphasic as they contain both carcinomatous and sarcomatous elements. Most commonly arising from the endometrium, extragenital carcinosarcomas are extremely rare and most cases develop from the peritoneum. The case is reported of a 70-year-old female who presented with abdominal pain and distention. On evaluation a large abdomino-pelvic mass with ascites was noted. She underwent complete cytoreductive surgery and the histopathology reported carcinosarcoma of primary peritoneal origin, of heterologous type arising de novo from the secondary Mullerian system with no synchronous or metachronous carcinomas or endometriosis. She declined adjuvant treatment and re-presented with disseminated abdominal disease and unfortunately succumbed to the disease within four months. Carcinosarcomas of the extragenital sites have been postulated to arise from pre-existing foci of endometriosis, Mullerian duct remnants, or the secondary Mullerian system, all of which are derivatives of the coelomic epithelium. They are extremely aggressive, and there is little knowledge concerning their natural history and scant data regarding their management. (Full text available online at www.medpharm.tandfonline.com/ojgo ) South Afr J Gynaecol Oncol 2018; DOI: 10.1080/20742835.2018.1454144

Cystoscope-Assisted Laparoscopic Resection of Mullerian Duct Remnant in a Male Presenting with Uterus and Vagina

Background and Aim: Mullerian duct remnant (MDR) is a rare condition and is sometimes accidentally diagnosed during surgery for an inguinal hernia and undescended testes. MDR is always found near the seminal duct and conservatively preserved, preventing injury of the seminal duct. However, recent reports have revealed that MDR has the potential to become malignant.1,2 Cases of deaths among male adolescents have also been reported. As such, surgical extirpation of MDR is recommended. Laparoscopic and robotic approaches have been applied as surgical intervention.3,4 In this video, the combination of laparoscopy and cystoscopy to safely extirpate an MDR is shown. Materials and Methods: Case: A four-month-old boy was initially found to have coronal hypospadias and undescended testes. Laparoscopy was performed at 11 months of age because of nonpalpable testes. A left streak gonad and MDR were found to be presenting as a complete uterus and vagina. Chromosomal examination revealed mixed gonadal dysplasia (46XY (23)/45X (7)). A cystoscope showed that the MDR was connected to the prostatic urethra. At 18 months of age, this patient underwent extirpation of the left streak gonad and urethroplasty for hypospadias. Subsequently, resection of the MDR was planned because of repeated urinary tract infections. Operative Findings and Procedure: The patient was placed in a lithotomy position and a cystoscope was used to confirm the fistula orifice at the urethra. A 5 mm, 30° laparoscope was inserted through the umbilical longitudinal incision. Pneumoperitoneum was established at 8 mmHg CO2 inflation. Two 5 mm ports (left upper abdomen and left lower abdomen) and one 3 mm port (right lower abdomen) were inserted. MDR presenting as a complete uterus and vagina was recognized. The bladder was suspended using extracorporeal 3-0 proline insertion. The bilateral uterus ligaments were coagulated and divided using a vessel sealing system (LigaSure Maryland; Medtronic, Inc., Dublin, Ireland). After opening the peritoneal reflection, the distal side of the MDR was carefully dissected using a 3 mm bipolar (RoBi, KarlStorz, Tuttling, Deutschland) to prevent thermal injury of the pelvic nerve. The distal mullerian duct had a vaginal appearance and was connected to the urethra as a fistula. Before ligation of the fistula, the depth of dissection was confirmed using a cystoscope. Under inspection with the cystoscope, the dissected fistula was retracted, and the level of dissection was confirmed. A transfixing 4-0 PDS (Ethicon, Cincinnati, OH) suture was used to ligate the fistula, which was then resected using a vessel sealing system and ligated using an Endoloop (Ethicon). No injury to the bilateral ureter or urethra was recognized. Results and Conclusion: The postoperative course was uneventful. A pathological diagnosis revealed that the resected MDR showed age-appropriate development. The patient has had a good clinical course since the operation. A laparoscopic approach is feasible and low invasive for the extirpation of an MDR, even in infants. The fistula was safely ligated using a combination of laparoscopy and a cystoscope. This combination approach is useful for preventing injury to the urethra. Acknowledgments: We thank Brian Quinn for his comments and help with the article. This study was supported by grants-in-aid for scientific research from the Japan Society for the Promotion of Science (JSPS, No. 25293360 and No. 26670765), JFE (The Japanese Foundation for Research and Promotion of Endoscopy), J-CASE (Japanese Consortium of Advanced Surgical Endoscopy), Foundation for Promotion of Cancer Research, Foundation for Shinnihon Zaidan, a research grant from the President's Discretionary Expenses of Kagoshima University, a research grant from The UBE Foundation, and a research grant from Japan Medical Education Foundation. No competing financial interests exist. Runtime of video: 6 mins 49 secs

Laparoscopic correction of transverse testicular ectopia with persistent Müllerian duct syndrome

Abstract Transverse testicular ectopia (TTE) with persistent Mullerian duct syndrome (PMDS) is a rare genitourinary anomaly. Herein, we report a case of TTE with PMDS corrected laparoscopically. An 11-month old male patient visited our hospital with swelling of the right inguinal area. The right testis was palpable in the right scrotum while left testis was not in the left scrotum. During laparoscopic exploration, the left testis was found around the right inguinal area, with the left spermatic cord across the pelvic cavity from left side. When the left testis was pulled, a tubular structure, Mullerian duct remnant, and the right spermatic cord came into the abdominal cavity. The Mullerian duct seemed like a uterus was located between both vas deferens. We divided it because the length of the bilateral vas deferens was not sufficient for orchiopexy. After laparoscopic right orchiopexy and left trans-scrotal approach orchiopexy, bilateral inguinal herniorrhaphy was performed laparoscopically.

Magnetic Resonance Imaging Findings of Ovotesticular Disorder of Sex Development with Bilateral Gonadoblastoma

: Ovotesticular disorder of sex development (DSD) is a very rare chromosomal anomaly characterized by the presence of both ovarian and testicular tissues. We reported a case of delayed diagnosis in a 18-year-old man presenting with nonpalpable testes. Pre-operative magnetic resonance imaging (MRI) demonstrated associated anomalies (agenesis of seminal vesicle, configuration of urethra) and Mullerian duct remnants. The presence and location of both ovary and testis, and restricted diffusions of gonads were detected on MRI. On histopathology, malignancies were found in both gonads. Pre-operative MRI findings were helpful for surgical approach. We emphasized the importance of pre-operative MRI on treatment planning.

Excision of Mullerian duct remnant for persistent Mullerian duct syndrome provides favorable short- and mid-term outcomes

ObjectiveIn dealing with persistent Mullerian duct syndrome (PMDS), excision of Mullerian duct remnant (MDR) has been rarely mentioned in the past, but recent discussions have taken place. This study aimed to evaluate the operative feasibility and outcomes. Materials and methodsThree patients with PMDS operated on with excision of MDR between 2000 and 2009 were enrolled. Medical records were retrospectively collected and reviewed. ResultsBilateral undescended testis was manifested in all cases. Two patients presented with incarcerated hernia, requiring emergency herniorrhaphy at the ages of 6 months and 10 days, respectively. Reconstruction comprising simultaneous MDR excision and orchiopexy was made at the age of 1 year. MDR was incidentally found in another patient during operation for undescended testis. Immediate reconstruction was accomplished. Follow-up periods were 12.0, 3.5, and 2.5 years, respectively. Worse outcomes were noted on the two testes with repeated operations for incarcerated hernias, whereas the outcomes on the other four testes with a single operation were favorable. ConclusionsExcision of MDR is technically feasible, and provides favorable outcomes in cases of a single operation. For experienced surgeons, immediate reconstruction should be the priority when this abnormality is incidentally encountered at an age suitable for orchiopexy.

VCUG and the recurring question of sedation: preparation and catheterization technique are the key

The voiding cystourethrogram (VCUG) is the most frequently performed fluoroscopic examination in pediatric radiology departments for the investigation of the lower urogenital tract in children. Avariety of clinical indications exists and include urinary tract infections (UTI), vesico-ureteral reflux (VUR), prenatal hydronephrosis, congenital renal anomalies, posterior urethral valves (PUV), bladder diverticula, hypospadias, cloacal abnormalities, Mullerian duct remnants, imperforate anus, bladder and urethral trauma, hematuria, urolithiasis, renal transplantation and assessment of the unstable bladder [1–3]. The VCUG is not likely to be replaced any time soon. If one only considers vesicoureteral reflux, an estimated 50,000 children are diagnosed with VUR after urinary tract infection each year in the United States [4]. The number of children undergoing screening examinations, therefore, is likely in the many hundreds of thousands. The VCUG examination requires transurethral catheterization (excluding suprapubic puncture), instillation of contrast agent for bladder filling, and voiding under direct fluoroscopic imaging. Optimal evaluation includes an awake and cooperative child, limited and optimized image acquisition utilizing last-image-hold techniques and pulsed digital fluoroscopy units, minimal radiation burden and a short examination time [1, 5]. The vast majority of VCUG examinations are performed primarily on an outpatient basis, unfortunately often with little or no preparation of the child or parent [6]. The VCUG examination can therefore be perceived as a painful investigation associated with high levels of distress and anxiety for the child, parents and even the medical staff [1, 6–14]. The VCUG experience has been described by one author as follows: “The child may be separated from his or her parents; his or her legs and genitalia are spread apart, often forcibly; probing and intruding into a “private” area not usually touched by strangers; firm pressure may be used to clean the genitalia; a urinary catheter is inserted; and the child must tolerate the discomfort of an unusually full bladder and the indignity of voiding in public. As the child lies flat and still, cold and exposed, huge cameras approach within inches of his or her body and rotate around it” [6]. If this description of the VCUG is the perception of the lay public, then it is not surprising that an uninformed parent would desire, if not demand, that their child be sedated, preferably with general anesthesia. This misunderstanding is certainly doing our families and patients a great disservice. Unfortunately, many children have been irreparably traumatized by previous catheterizations as well as other invasive medical procedures and the mere thought of undergoing an unknown or repeat procedure is unbearable. I believe the use of sedation is entirely appropriate in these patients. As a pediatric radiologist, it is my job to provide the safest, most productive and least distressing examination possible. In my opinion, however, if thorough pre-test preparation of the parent and child were combined with appropriate catheterization techniques, the VCUG examination could be performed without the need for sedation in the vast majority of patients. The first component of this discussion revolves around patient education. The VCUG examination process actually begins in the ordering physician’s office. First and foremost, the physician needs to communicate to the family the reason for the examination, its medical importance to the health of Related articles can be found at doi:10.1007/s00247-011-2322-x and doi:10.1007/s00247-011-2323-9).

Unusual Presentation of a Mullerian Remnant in an Infant with Recurrent Epididymo-orchitis

Mullerian duct remnants result from incomplete regression, a defect that occurs during sexual differentiation. Ninety percent of patients with mullerian remnants also have an associated disorder of sexual differentiation such as hypospadias. Presenting signs and symptoms are recurrent bladder infection, perineal pain, dysuria, or infertility. The purpose of this case report is to recount an unusual presentation of a mullerian duct remnant with recurrent epididymo-orchitis and to discuss the embryology, diagnosis, and management of this condition.

Clinical Features and Diagnostic Approach in Patients with Undervirilised External Male Genitalia

Background. Sex development in humans is a complex and multiple step process. SRY gene on Y chromosome has met the criteria for a testis determining factor, however subsequent stages of differentiation require the expression of several other genes. Male pseudohermaphroditism refers to genetically XY male with differentiated testes and varying degree of undermasculinization of the external genitalia. Aim. Our study aimed at exploring the clinical characteristics, cytogenetic and SRY gene analyses in a group of 14 pediatric patients with male pseudohermaphroditism. Material and Methods. Clinical presentations were: micropenis and/or penoscrotal/perineal hypospadia in 86%, altered appearance of the scrotum in 79% and Mullerian duct remnants in 50% of the patients. Results. Cytogenetic analyses revealed a normal male karyotype in all patients. SRY gene was found positive in all 14 patients. Conclusion. We concluded that SRY gene analysis in patients with male pseudohermaphroditism confirms the clinical diagnosis and directs further investigations. SRY gene analysis is a quick and relatively simple diagnostic method that can be included at the early stage diagnostic of sex differentiation disorders.

Carcinosarcoma in the pouch of Douglas

Only one case of carcinosarcoma in the pouch of douglas has been reported in the English literature. The author herein reports a case of carcinosarcoma of the pouch of douglas. A rapidly growing tumor (10 cm in diameter) was found in the pelvic cavity of a 51-year-old woman, and an operation was performed for the clinical diagnosis of left ovarian malignancy. However, it was found that the tumor was located in the pouch of douglas, free of ovarian involvement. Because the tumor was impossible to resect totally, an incisional biopsy was performed. Histologically, the tumor showed a mixture of squamous cell carcinoma, atypical epithelial cells occasionally forming tubules, myxomatous spindle cell sarcoma, and chondrosarcoma. The tumor showed abundant vessels, hemorrhage and necrosis. Immunohistochemically, the squamous cell carcinoma and atypical epithelium components were positive for cytokeratins, CEA, CA125, and p53 protein. The spindle cell sarcoma and chondrosarcoma components were positive for vimentin and p53 protein. The overall Ki-67 labeling was 30%. The author diagnosed the present case as carcinosarcoma of heterologous type. The present case shows that carcinosarcoma can occur in the pouch of douglas. It may arise from misplaced Mullerian duct remnants in the pouch of douglas.

Posterior sagittal approach with perirectal dissection for reconstructive surgery of severe urogenital anomalies

There are many approaches for surgery of complicated congenital and acquired urogenital anomalies in children with intact rectum. Pena advocates the mid-sagittal division of the sphincter mechanism posterior and anterior to the rectum, along with opening of the posterior and anterior rectal walls. The aim of this study is to determine whether the posterior sagittal approach with perirectal dissection (PSAPD) and elevation of the mobilized rectum would impair fecal continence when used for correction of complicated urogenital anomalies in children with normal rectum. Between 1988 and 1994 the authors performed PSAPD in eight infants and children with an intact anorectum. Indications for PSAPD were high vaginal atresia, Mullerian duct remnants, prostatic rhabdomyosarcoma, and traumatic vesicovaginal fistula. After a mean 10-year follow-up the bowel habits were assessed. Anorectal and uromanometric studies and a detailed questionnaire (modified Holschneider's scoring) sent to children or parents were evaluated. Three patients who preoperatively were clinically fecal continent had soiling only at the time of diarrhea. Early postoperative low anorectal pressure profile normalized during the follow-up. Seven patients had a fecal continence score above 23, two of them with maximum points of 26. Only one girl had a low score of 15. The authors conclude the PSAPD which offers a few advantages over the sagittal division of the rectum provides an alternative approach for selected lesions of the genitourinary tract in children with a normal rectum. Our results suggest that fecal continence is either preserved or partially affected.

A boy with acute urinary retention: a Mullerian duct remnant (2006: 3b)

Vestiges of the Mullerian duct systems are found in the male and these may occasionally give rise to symptoms. We report a case of acute urinary retention in a male child secondary to a Mullerian duct remnant. Subsequent MRI was done to reach the final diagnosis.

A CLINICAL ENTITY OF CYSTIC DILATATION OF THE UTRICLE ASSOCIATED WITH HEMOSPERMIA

We investigated the mechanism of hemospermia in patients with a midline cyst (MLC) of the prostate, focusing on cystic dilatation of the utricle (CDU) as a possible causative lesion. Of 138 patients with hemospermia 30 (22%) had an MLC, of whom 19 underwent transperineal needle aspiration of the MLC and bilateral seminal vesicles to determine the site of bleeding. Following MLC aspiration a mixture of dye and contrast medium was injected. The verumontanum was observed endoscopically and pelvic x-ray was done. Seminal vesicle fluid on 1 or 2 sides was hemorrhagic in 13 of the 19 patients (aspiration failed in 6) and fluid from the MLC was nonhemorrhagic in 5 of the 19 (aspiration failed in 7). The MLC communicated with the urethra (CDU) in 15 patients (79%) and with 1 or 2 ejaculatory ducts in 11 (58%). In 5 of 11 patients with communication with the ejaculatory duct hemospermia persisted for more than 1 year. Four of these patients were cured by transurethral unroofing of the CDU. CDU is a significant underlying lesion for hemospermia. Communication with the ejaculatory ducts was frequent in patients with chronic hemospermia and transurethral unroofing was effective for eliminating hemospermia.

An unusual case of a prenatally detected large mullerian duct remnant

Mullerian duct remnants presenting in childhood are often diagnosed incidentally and when symptomatic, typically present with recurrent infection and/or voiding disturbances. We present a rare unusual case of a male neonate who presented with a prenatally detected large pelvic cyst that turned out to be a giant prostatic utriculus.