Insights into enlarged prostatic utricles and Müllerian duct system remnants associated with posterior hypospadias.

INTRODUCTION: The prostatic utricle (PU) consists of the caudal remnant of the Müllerian duct and the urogenital sinus. The term "vagina masculina" is used if other Müllerian structures are associated with the PU. This work aims to investigate the incidence, management, and follow up of enlarged PUs and Müllerian remnants in males with posterior hypospadias. PATIENTS AND METHODS: This study presents a retrospective review of cases presented with posterior hypospadias over a 5-year period. Prior to hypospadias repair, retrograde urethrograms were used to investigate enlarged PU. Subsequently, they were classified according to the Ikoma score and further assessed by karyotyping and cystoscope. Surgical excision was indicated in cases with symptomatic utricles or vagina masculina. RESULTS: Thirty patients were included in the study in the period between 2015 and 2020 (Table). All cases were asymptomatic initially. Twelve patients were diagnosed with enlarged PU; three of them had vagina masculina. One case with perineal hypospadias had a separate perineal opening for PU. Following hypospadias repair, three of the eight cases treated conservatively turned symptomatic. DISCUSSION: The incidence of enlarged PU and Müllerian remnants varied among different studies. However, it increased as the severity of hypospadias increased. Preoperative urethrogram was helpful in the diagnosis and classification of PU, but it had its limitations. Cystoscope was more advantageous in diagnosing vagina masculina. Although most cases were asymptomatic, some turned symptomatic after hypospadias repair. Some cases with perineal hypospadias had PU with a separate perineal opening. CONCLUSION: The incidence of enlarged PUs or Müllerian remnants was 40%. Although cases were asymptomatic before hypospadias surgery, some cases turned into symptomatic after hypospadias repair. In some cases, the PU or Müllerian remnants had a separate perineal opening. They can be classified as a particular form of Ikoma grade III necessitating surgical intervention.

Autologous platelet gel improves outcomes in tubularized incised plate repair of hypospadias.

BACKGROUND: hypospadias is one of the most widespread male congenital anomalies, occurring in 1:250 to 1:300 live births. Several repair techniques have been developing to improve the outcomes. PURPOSE: a randomized prospective controlled study was adopted to evaluate effectiveness of autologous platelet gel in healing promotion and improving the outcomes of hypospadias repair. METHODS: thirty children who aged between 6 months and 12 years were recruited and subdivided into two groups; group A had tubularized incised plate (TIP) repair with autologous platelet gel application and group B had TIP repair without autologous platelet gel. RESULTS: there was no significant difference in duration of operation between both groups. All patients in groups A and B had slit-like meatus shape in the distal glans. While all those of group A had one urine stream, yet only 11 of group B had one. There were complications that happened exclusively in group B such as spray stream (27%) and fistula (20%). Whereas other complications occurred insignificantly more in group B than in A including meatal stenosis (53 versus 27%), glans dehiscence, (20 versus 7%), bleeding (33 versus 13%), infection (33 versus 27%), edema (27% versus13), respectively. The incidence of skin necrosis was equal in both groups. CONCLUSION: autologous platelet gel usage in TIP hypospadias repair can be a reliable technique to promote wound healing, and to limit of postoperative surgical complications.

Vesico-cutaneous fistula: A modified technique for intermittent catheterization in children with impaired bladder function.

BACKGROUND: Clean intermittent catheterization (CIC) is fundamental in managing children with impaired bladder function. However, it is not always feasible via the native urethra predominately males with intact sensation. AIM: In the current study, the experience regarding a modified vesico-cutaneous fistula (VCF) technique for CIC in children is reported. PATIENTS AND METHODS: From January 2018 to January 2020, the modified VCF was performed to cases with impaired bladder function for CIC. Before surgery, patients were evaluated thoroughly. The anteroposterior diameter (APD) of the renal pelvis was considered as an indicator of the degree of affection of the upper urinary tract. Postoperative attacks of UTIs, leakage from the fistula, fistula stenosis, and the need for revision were recorded. The technique was accomplished relying on the principles of open Stamm gastrostomy (Fig. 1). RESULTS: Eight cases underwent a modified VCF due to different pathologies. Their ages, at time of the surgery, ranged from 1 month to 144/12 years. The median postoperative follow up period was 12 months. A leakage between catheterizations was experienced in 1 case. Stenosis of the fistula occurred in 1 case and it required surgical revision. No symptoms or signs of urinary tract infection were encountered. The mean APD of the renal pelvis was reduced from 29.8 mm (SD: ±14.6 mm), in the preoperative evaluation, to 17.6 mm (SD: ±10.85 mm) in the postoperative follow up. DISCUSSION: Vesico-cutaneous fistula is a new technique that provides easy and direct way for CIC. In the current study, a modified VCF technique was introduced to the enrolled patients as a simple maneuver, to make easy self-catheterization. Despite the limitations of this study due to the lack of a control group, the small number of cases, and the short follow up period, this modified technique was able to create a short and straightforward channel to access the bladder for intermittent catheterization. It could be done in redo cases. Results showed an acceptable degree of continence and low rate of complications. Moreover, it was socially accepted among our patients. CONCLUSION: Intermittent catheterization in children, with abnormal bladder function, can be done easily and efficiently via a modified VCF.

Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.

Congenital haemangioma (CH) is a rare benign vascular tumour presenting at birth with excellent prognosis. Usually, CH regresses without treatment within the first few months of life. Kaposiform Haemangioendothelioma (KHE) is another type of vascular tumours that has been described as benign with locally aggressive potential. Although the diagnosis of vascular tumours is usually straightforward based on typical clinical presentation, yet some confusing similarities may exist with congenital sarcomas.Conclusion: Data of cases managed at the vascular anomaly clinic during the period 2015 through 2019 were retrospectively analysed. The study included three groups of patients: cases diagnosed as congenital haemangioma (9 cases), cases of Kaposiform Haemangioendothelioma who presented in the neonatal period (7 cases), as well as cases of congenital fibrosarcoma (4 cases) that were referred to the vascular anomaly clinic because of apparent similarity with vascular tumours. The hallmark of the study was to compare clinical and imaging features in the three groups to facilitate differentiation and remove diagnostic confusion when managing these rare cases in the future. What is Known: • Congenital haemangioma is a rare benign vascular tumour presenting at birth. • Kaposiform Haemangioendothelioma is another type of vascular tumours that has been described as benign with locally aggressive potential. What is New: • Confusing similarities may exist between vascular tumours and congenital sarcomas.

Posterior cloaca: A urogenital rather than anorectal anomaly.

BACKGROUND: 'Persistent Cloaca' refers to one of the major groups of anorectal anomalies in the female when a single perineal orifice is located at the normal site of the urethra draining both urogenital and alimentary tracts. However, 'posterior cloaca' is a new term developed by Pena to describe a unique defect in females in which a common urogenital sinus is deviated posteriorly to open into normally located anorectum (type A) or in the perineum slightly anterior to the anus (type B). METHODS: The study included seven girls diagnosed as posterior cloaca. Their age at presentation ranged from 1 to 108 months (median 12 months). The main presentation was abnormal external genitalia, while two cases underwent colostomy (±vaginostomy) at birth. Surgical reconstruction varied according to the degree of deviation from normal anatomy. Partial urogenital sinus mobilization (PUM) was used to correct minor forms; while at the other end of the spectrum (absent urinary bladder), continent urinary diversion was performed. Due to the high incidence of renal and urological anomalies, regular follow up at paediatric nephrology/urology clinics was advised for affected cases. RESULTS: All seven cases had a common urogenital confluence characteristically deviated posteriorly. The degree of backward deviation of the common urogenital orifice was variable ranging from mild to severe posterior displacement. In six cases (85%), the common urogenital orifice was shifted backwards in the perineum approaching the anal verge (type B). In one case, the common urogenital orifice opened internally into the anorectum (type A). Major urinary tract anomalies were quite common (5 of 7 cases; 71%): absent urinary bladder (2 cases); single kidney (2 cases); crossed ectopic kidney (1 case); hydroureteronephrosis (2 cases). CONCLUSION: Posterior cloaca is a rare anomaly in the female essentially affecting the lower urogenital tract with a high incidence of associated renal anomalies. Management should be tailored according to the degree of developmental defect.

Y-Type Urethral Duplication: A True Variant of the Anomaly or a Misnomer?

Objectives The objective of this study was to define anatomical and radiological features of the so-called Y-type urethral duplication. Methods The study included four male patients and one female patient with congenital connection between the urogenital tract and the external anal orifice. Investigations included renal sonography, urethrograms, and magnetic resonance imaging pelvis in the last patient. The urethrograms of male patients were carefully reviewed, in addition to available urethrograms of similar cases that could be obtained through searching the literature. Results Unlike cases of urethral duplication, the male patients had always a complete prepuce and a functioning anterior urethra in 25%. The accessory uroanal channel had almost always a constant origin from the posterior urethra. Some tension seems to be exerted by the urethroanal tract pulling on and causing a kink in the posterior urethra. Management was simple in patients without anterior urethral hypoplasia (one male and the female patient). Both were treated by simple excision of the communicating ano-urogenital tract through a perineal approach with an excellent outcome. Histopathological examination of excised tracts revealed stratified squamous cell in the former and transitional cell lining in the latter. In patients with hypoplastic anterior urethra, staged urethral reconstruction was performed in two, and progressive dilatation of hypoplastic anterior urethra was tried in the last patient. Conclusion Several observations would support diagnosing the congenital connection between the urinary tract and the external anal orifice in the male as a congenital fistula rather than an accessory urethra. Confirming and accepting this information may have its impact on changing the current surgical approach.

The double urethra: revisiting the surgical classification.

AIM: The aim of this article was to describe our experience with 14 patients with double urethra. PATIENTS AND METHODS: We retrospectively examined the patients' records including their clinical presentations, investigations, operative findings, and outcome. In addition to Effmann's classification, we used a newly proposed classification that depends on the orientation of the double urethral channels. RESULTS: During the last 15 years, 18 patients were diagnosed to have double urethra at our pediatric surgical unit. We excluded four patients with 'Y-type' urethral duplication. The remaining 14 patients were divided into either sagittal or collateral duplication. Their age at presentation ranged from the neonatal period to 9 years. The sagittal urethral duplication included 12 male patients. All patients had two urethral channels, one above the other. The dorsal urethral channel was always characterized by poor function and ectopic course. The ventral channel was always the more functioning urethra, with a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). The collateral urethral duplication included two patients. Both patients were associated with duplication of the urinary bladder and the external genitalia as a part of caudal duplication syndrome. In this group, both urethrae had comparable function, lying side by side, and each draining a separate urinary bladder. CONCLUSION: The double urethra is a diverse spectrum comprising different pathologies. Our proposed classification system of duplicated urethras is clinically relevant as it guides surgical management and allows prognostication of outcome.

Feasibility and safety of monopolar diathermy as an alternative to clip ligation in laparoscopic Fowler-Stephens orchiopexy.

PURPOSE: The aims of the study were to study the effect of Fowler-Stephens orchiopexy (FSO) on testicular histology and to assess the feasibility of using monopolar diathermy as an alternative to clip ligation during laparoscopic FSO. PATIENTS AND METHODS: The study included 20 patients with 20 intraabdominal testes and short vessels managed by laparoscopic-staged FSO. Biopsies were taken from intraabdominal testes during the first and second stages of the procedure for histologic comparison. The patients in the study were divided into 2 groups according to the method of dividing the testicular vessels in stage 1. The first 13 patients (group A) were managed by clip ligation of the vessels, whereas monopolar diathermy was used in the following 7 patients (group B). RESULTS: Biopsy findings at stage 2 revealed an overall reduction in both the total number of germ cells per tubule and mean diameter of seminiferous tubules, whereas there was no statistically significant difference between the results in groups A and B. CONCLUSION: The seminiferous cells can withstand (survive) dividing the main blood supply of the testis during FSO. The monopolar diathermy can be used as an alternative to clipping during laparoscopic procedures, having the advantages of lower expenses and using smaller instruments.