Hypospadias with Intact Prepuce: A Spectrum of Anomalies and their Reconstruction.

Aims: The aim of the study was to perform a detailed analysis of the clinical characteristics of megameatus with intact prepuce (MIP) and to audit our results of reconstructive surgeries on MIP. Materials and Methods: Design: Retrospective analysis. Setting: Pediatric surgery department of tertiary level. Subjects, methods: Hospital records and pictures of MIP operated over a 9-year period. Age, size of penis, circumcised or not, shape of glans, external urethral meatus (EUM), urethral plate (UP), chordee, distal urethra, reconstructive surgery, and complications were analyzed. Postoperative result was objectively assessed by Hypospadias Objective Scoring Evaluation (HOSE). Results: Twelve of 254 hypospadias were MIP (incidence = 4.72%). The mean age at operation was 38.25 months (12-87 m). Patients sought circumcision or surgical correction of anomaly. Two patients were precircumcised. MIP was coronal 7, subcoronal 3, and glanular 2. Meatus was wide in 10 and normal in 2. Glans penis was wide in 9 and conical in 3. UP was wide (9), moderately wide (2), or narrow and shallow (1). In two cases of wide deep UP, distal septum was present. Distal urethra was nondilated in all but 1, which had megalourethra. Reconstructive surgery was Tubularized Urethral Plate Urethroplasty (7 cases) or classical Snodgrass (Tubularised incised plate urethroplasty (TIPU), with superimposed vascular dartos flap (5 cases). The megalourethra underwent partial excision and TIPU. Distal UP-septum was incised. Urethral injury (2 cases) and UP injury (1 case) were intraoperative complications. One postoperative complication (ventral glans necrosis) resulted. The mean follow-up period was 4.79 months (1-12 m). In the postoperative follow-up, 11 (92%) had HOSE score 14-16, whereas one had HOSE 13. Conclusion: Some hypospadias cases which have intact prepuce have no megameatus; hence, they cannot be termed MIP. All cases of hypospadias having intact prepuce can be covered by the umbrella term "Hypospadias with Intact Prepuce (HIP);" MIP is a large subgroup under HIP. HIP presents with a spectrum of anomalies of glans, EUM, and UP. Repair by tubularization of UP without or with midline incision gives excellent results.

Rare case of an isolated scaphoid congenital megalourethra: before and after reconstruction.

Congenital megalourethra, first described in 1955, is a rare urethral anomaly resulting from dysgenesis of the penile corpus spongiosum, with or without corpus cavernosum involvement, leading to dilatation of the penile urethra. Presentations come in two forms, scaphoid and fusiform, with the former being more common and resulting from deficient or absent corpus spongiosum. Fusiform types are much rarer, and consist of absence of both the corpus spongiosum and cavernosum.3 Here, we present a case involving the surgical correction of an isolated scaphoid-type congenital megalourethra with significantly improved postoperative cosmetic and functional outcomes.

Prenatal diagnosis of congenital megalourethra with imperforate anus.

BACKGROUND: Congenital megalourethra is a rare prenatal finding while prenatal diagnosis of imperforate anus poses high challenge. This is the first prenatally ultrasound diagnosed case which had congenital megalourethra and imperforate anus. This case demonstrated the possibility of using the prenatal imaging findings to evaluate the postnatal prognostic outcomes in multi-organ anomalies. CASE: We present a case of congenital megalourethra, diagnosed prenatally at 22 weeks' gestation, in which the penis appeared severe dilated with complete absence of the corpora spongiosa and cavernosa. This case also revealed absence of perianal muscle which was in associated with imperforate anus. Detailed prenatal ultrasonographic findings predicted the high possibility of poor outcome of the fetus in the pulmonary, renal, and sexual functions. CONCLUSION: This case serves to identify not only the marked bilateral hydronephrosis features but also the striking lower urethral malformation with obstruction flow effect of the penis. Indeed we believe this is the first case report of a rare case of fetal megalourethra associated with imperforate anus at early second trimester on ultrasonography imaging.

Prenatal Diagnosis and Perinatal Outcomes of Congenital Megalourethra: A Multicenter Cohort Study and Systematic Review of the Literature.

OBJECTIVES: The purpose of this study was to evaluate the prenatal findings and postnatal outcomes in fetuses with congenital megalourethra. METHODS: This retrospective study reviewed our experience and the literature between 1989 and 2014. Prenatal findings were evaluated and compared with postnatal findings, including neonatal mortality and abnormal renal function (need for dialysis or renal transplantation). RESULTS: Fifty fetuses with congenital megalourethra were analyzed, including 6 cases diagnosed in our centers. Most cases (n = 43 [86.0%]) were diagnosed in the second trimester. Only 1 case was diagnosed in the first trimester, whereas 6 cases (12.0%) were diagnosed in the third trimester. Thirty-five fetuses (70.0%) survived. Bilateral hydroureters were associated with perinatal death (P= .024). Among the survivors, 41.9% of the neonates had renal impairment. The following factors were associated with postnatal renal impairment: presence of severe oligohydramnios/anhydramnios (P = .033), bilateral hydronephrosis (P = .008), and earlier gestational age at delivery (P = .022). CONCLUSIONS: In fetal megalourethra, bilateral hydroureters, bilateral hydronephrosis, and severe oligohydramnios/anhydramnios are associated with neonatal mortality and renal impairment.

Isolated megalourethra: A rare case report and review of literature.

Megalourethra is a rare congenital disorder of anterior urethra and erectile tissue of penis. It mainly appears in two types-a milder scaphoid type and severe fusiform type. Fusiform type is commonly associated with congenital anomalies of various systems of the body. Isolated megalourethra without other congenital anomalies is extremely rare. We report one such case which was detected postnatally and successfully treated by reduction urethroplasty.

A Unique Case of Inflatable Penile Prosthesis (IPP) Implantation and Mesh Phalloplasty in a Patient Born with Congenital Fusiform Megalourethra.

We present the first case of a patient born with congenital fusiform megalourethra who underwent the successful placement of an inflatable penile prosthesis (IPP) and mesh phalloplasty due to primary erectile dysfunction (ED). After an extensive psychosexual assessment and a preoperative MRI scan, an IPP was successfully implanted. The procedure was carried out through a penoscrotal approach, which offered excellent exposure to the crura and the proximal ends of the corpora cavernosa. Following the incision and the dilatation of both corpora cavernosa, the penile cylinders were inserted and a mesh phalloplasty was performed, to replace the erectile tissues lacking in the distal corpora cavernosa. After reservoir and pump placement, the device was tested, and no mechanical issues were recorded. The postoperative course was uneventful, and, at the 2-year follow-up, the patient reported highly satisfactory results, with valid functional erections.

Megalourethra and bladder foreign body in a female patient.

Key Clinical Message: Undiagnosed female megalourethra can be a cause of iatrogenic bladder foreign body. Abstract: Foreign bodies in the urinary bladder are relatively rare. Female megalourethra is an extremely rare congenital disorder which is usually associated with Müllerian anomalies. We describe a case of an iatrogenic bladder foreign body and a megalourethra in a young woman with normal gynecological organs.

CONGENITAL SCAPHOID MEGALOURETHRA: REVISITING A RARE ANOMALY.

Megalourethra is an infrequent malformation of the anterior urethra that is caused by the lack of corpus sponigosum and in severe cases is accompanied by the lack of corpora cavernosa as well. We report a five-year-old boy presented to us with scaphoid variety of megalourethra having complaints of ballooning of urethra during voiding. He was investigated with urethrogram and cystoscopy and subsequently repaired with Nesbitt Longitudinal Reduction Urethroplasty. He had smooth recovery postoperatively with normal voiding stream on follow up.

Congenital megalourethra.

Background: Congenital megalourethra is a urogenital anomaly characterized by a cystic dilatation and elongation of the penile urethra resulting from the absence and hypoplasia of the corpus spongiosum and corpus cavernosum, or anterior urethral valve. There are two clinical types: scaphoid and fusiform. Generally, the etiology is unknown, but it is thought to be a defect in mesodermal development. Fewer than 100 cases have been reported in the literature, and the exact incidence is unclear. In most cases, the surgical procedure is challenging and requires extensive reconstructive and/or replacement surgery. Case presentation: We present a 6-month-old boy suffering from a cystic dilatation of the penile urethra along with urine dribbling during micturition since birth. The patient was diagnosed with the scaphoid type of megalourethra and was operated on using reduction urethroplasty. On the 21st post-operative day, we removed the Foley catheter and followed the patient on two occasions (the 45th post-operative day and the 6th post-operative month) with excellent results. Conclusion: The anagement of megalourethra depends on the clinical type. Meticulous surgical technique, the use of fine suture materials with careful handling, and fixation of the vascularized flap are the main principles of an acceptable result. Observation of erectile function and fertility require long-term follow-up.

Treatment of Urethral Intercourse and Impact on Female Sexual Function.

BACKGROUND: Urethral intercourse is a very rare entity which usually presents as urinary incontinence during and after intercourse and is most commonly seen in patients with vaginal agenesis (Mayer-Rokitansky-Hauser Syndrome) or hypoplasia, or other rear vaginal anomalies. AIM: To evaluate management and outcomes for vaginal and urethral consequences of urethral intercourse, including urinary incontinence. METHODS: Between February 2006 and March 2021, 8 women aged from 17 to 22 years underwent genital and urethral reconstruction due to consequences of urethral sexual intercourse. Vaginal reconstruction included sigmoid vaginoplasty and introitoplasty with division of the vaginal septum in cases of vaginal agenesis (5 cases) and vaginal duplication (3 cases), respectively. Incontinence was treated by sling procedures in 5 women with longer history of urethral coitus and evident bladder neck prolapse. OUTCOMES: Sexual and psychosexual outcomes assessment was based on the Female Sexual Function Index and standardized questionnaires. RESULTS: Follow-up ranged from 9 to 188 months (mean 78 months). Good esthetical and functional results were achieved in all 8 women. All patients reported satisfactory sexual intercourse. All 5 incontinent women who had underwent sling procedure were continent. In one of 3 nontreated cases, additional sling treatment was indicated 6 months after vaginal reconstruction with satisfactory outcome. One patient with vaginal duplication reported a successful pregnancy with a Caesarean section delivery. CLINICAL IMPLICATIONS: Urinary incontinence with megalourethra in young women, along with the presence of Mullerian anomalies should raise suspicion of urethral coitus. Surgical treatment includes correction of vaginal anomalies and management of consequences. STRENGTHS AND LIMITATIONS: This study represents one of the largest series for urethral intercourse, with assessment of psychosexual outcome. The limitation is the lack of statistical analysis due to small sample size. CONCLUSION: Urethral intercourse is very rare, but it can cause severe consequences. It is important to recognize this occurrence and treat it by well-known vaginal or urethral reconstructive procedures. Djordjevic ML, Bizic M, Stojanovic B, et al. Treatment of Urethral Intercourse and Impact on Female Sexual Function. Sex Med 2022;10:100534.

Prenatal diagnosis of congenital megalourethra: case report and literature review.

BACKGROUND: Congenital megalourethra is an uncommon cause of lower urinary tract obstruction that is rarely prenatally diagnosed in second trimester sonographic examination as a cystic genital mass. CASE: In the presented case, the megalourethra was accompanied with bilateral mild pelviectasis. The newborn had no morbidity during follow-up period. To review the literature, electronic databases including PubMed, Web of Science and Google Scholar were searched up to February 15, 2021. In 51 prenatally diagnosed cases in the literature, most of the cases had accompanying congenital anomalies, especially structural abnormalities in the genitourinary. CONCLUSIONS: In the absence of associated abnormalities, the condition of the upper urinary tract is the main determinant of postnatal outcome. The outcome of congenital megalourethra may be good as in our case, but there may also be serious disorders such as renal failure, pulmonary hypoplasia, erectile dysfunction and fertility issues.

Prenatal diagnosis of congenital megalourethra in the second trimester of pregnancy.

Congenital megalourethra is abnormal dilation of the penile urethra due to aplasia of erectile tissue, leading to lower urinary tract obstruction. This condition should be considered when fetal penis with typical dilation is seen on prenatal scan. The dilated structure, however, can be easily missed or misinterpreted as the umbilical cord. Perinatal prognosis is poor due to direct consequences on urinary and respiratory systems, or due to severe associated anomalies. A large number of survived cases experience urinary sequelae and renal impairment. We report one case of fetal congenital megalourethra diagnosed at 17 weeks and 6 days of pregnancy.

Congenital Scaphoid Megalourethra: A Case Report.

A congenital megalourethra is an enlargement of the pendulous urethra without evidence of distal obstruction. A 1-month-old boy presented to us with complaint of weak stream, ballooning of the penis before and during voiding and post voiding dribbling, since birth. Physical examination and cystourethroscope confirmed the diagnosis of congenital scaphoid megalourethra. He underwent reduction urethroplasty. During postoperative follow up, he had normal looking penis with good urinary stream.

The Association of Congenital Urethral Duplication and Double Megalourethra.

BACKGROUND: Urethral duplication and megalourethra are rare urethral anomalies. However, the concomitance of urethral duplication and double megalourethra has not been reported previously. CASE REPORT: A newborn was presented with penile swelling during voiding. Physical examination revealed a retractable foreskin and two external meatus of a double urethra. Retrograde urethrography demonstrated two complete megalourethras. Urethro-urethrostomy and urethroplasty were performed when the patient was 10 months old. The patient was followed up for one year without any urinary problems and has good cosmetics and urinary continence. CONCLUSION: The concomitance of these two rare anomalies and more importantly its surgical treatment makes this case report unique and valuable.

Urethral Coitus in a Case of Vaginal Agenesis - Is Only Vaginoplasty Enough to Treat the Urinary Problems?

Urethral coitus is an extremely rare condition. Megalourethra and urinary incontinence due to urethral coitus in vaginal agenesis are unusual manifestations because these patients usually present with primary amenorrhea before becoming sexually active and receive treatment. A 24-year-old woman came to our clinic because of primary amenorrhea, sexual dysfunction, dyspareunia, megalourethra and urinary incontinence five months after her marriage due to urethral coitus. Based on these clinical and radiological findings a diagnosis of Mayer-Rokitansky-Kuster-Hauser Syndrome was made and patient underwent modified McIndoe Vaginoplasty. The elasticity of female urethra permits repeated coitus, together with the physical damage can probably lead to incontinence. In this case, after eliminating the underlying cause of disease with vaginoplasty, no other treatment was required. The integrity of sphincteric function and structural support of urethra might be regained without subjecting the patient to aggressive reconstructive surgical procedures.

Congenital anterior urethrocutaneous fistula at the penoscrotal junction with proximal penile megalourethra: A case report.

Congenital anterior urethrocutaneous fistula and megalourethra are both rare anomalies. These anomalies are commonly associated with other anorectal or genitourinary anomalies and evaluated with voiding cystourethrography. We examined a 34-month-old boy who presented with a fistula at the penoscrotal junction. A voiding cystourethrogram showed a jet of urine coming through the fistula and proximal saccular dilatation of the penile urethra. We present the imaging findings of the first case of an association between a congenital anterior urethrocutaneous fistula at the penoscrotal junction and a proximal penile megalourethra. We also discuss the etiology, management, and differential diagnosis of this entity, and review the literature.

Megalourethra: a case report managed with a single intrauterine bladder aspiration.

Congenital megalourethra is a rare mesenchymal anomaly of the male urethra, characterised by severe dilatation of the penile urethra due to congenital absence of the corpus spongiosum and/or corpus cavernosum. Postnatal complications include voiding and erectile dysfunction as well as renal insufficiency and pulmonary hypoplasia. We present a unique case of congenital megalourethra diagnosed prenatally in the early second trimester. The parents opted to continue pregnancy and vaginal delivery of a live neonate occurred with a favourable outcome.

A Case of Congenital Megalourethra / 대한비뇨기과학회지

Megalourethra is a rare congenital abnormaly characterized by severe dilatation of urethra, the disorder at first reported by Nesbitt in 1955. The scaphoid type is due to poor development of the corpus spongiosum, whereas in the more severe fusiform variety the corpora cavernosa also are affected. We present a case of congenital scaphoid megalourethra associated with acute renal failure, sepsis and grade IV bilateral vesicoureteral reflux. Initial management of this patient was vesicocutaneostomy and urethrocutaneostomy followed later by urethral reconstruction.