New classification of the penoscrotal positional anomalies.

BACKGROUND: The aspect of sexual differentiation and the mechanism controlling the position of genitalia, which represents one of the most substantial differences between the sexes, is still poorly understood. Minor cases and some variants of penoscrotal transposition (PST) are unreported, and obvious cases were classified broadly and confused with other unrelated anomalies. METHODOLOGY: Relevant literature published till 2022 were reviewed then organized, recapitulated, and presented in comparison with the findings and data of 65 child diagnosed with PST. So, an integrated comprehensive approach to this uncommon condition enabled a new classification including few unreported variant cases, which were complemented. RESULTS: PST is classified herein into a cephalic or caudal scrotal migration, the cephalic type subdivided into major and minor subtypes the latter type subdivided into bilateral, unilateral or central subtypes. Cases of caudal scrotal regression is an unreported anomaly in which the scrotum located caudally, as constant association with epispadias/exstrophy anomalies leaving a wide distance between the fixed penis and the scrotal sacs. CONCLUSION: PST is not rare as it was believed, it occurs in two directions; cephalic and caudal directions. Scrotal caudal regression anomaly was not described before, as well the PST presented as an inguinal hernia.

Variant Presentations of the Exstrophy-Epispadias Complex: A 40-Year Experience.

OBJECTIVE: To investigate the diagnosis, surgical management, and outcomes in patients with variant EEC. Variant presentations of the exstrophy-epispadias complex (EEC) span a wide range of abnormalities. The rarity and diversity of EEC variants can lead to challenges in the diagnosis and subsequent management of this population. METHODS: The authors reviewed an institutional database of 1336 EEC patients from 1975 to 2018 for variant presentations of EEC. Variant presentations included those with skin covered bladder exstrophy (BE), duplicate bladders, superior vesical fistula, and epispadias with major bladder prolapse. Surgical management and outcomes were assessed. RESULTS: In total, 44 EEC variants were identified. Nineteen (43%) presented with a skin-covered BE variant. Five patients presented with duplicate BE, while 6 presented with superior vesical fistula. Fourteen patients (32%) presented with epispadias with major bladder prolapse. Overall, 36 (82%) EEC variants underwent primary bladder closure, at a median of 135 days after birth (range 1-2010), with 21 (58%) undergoing pelvic osteotomy. Primary closures were successful in 89% of cases. Continence procedures were performed in 17 patients. This includes 5 patients who underwent bladder augmentation. However even without a continence procedure, continence with volitional voiding was found in 8 patients. CONCLUSION: The most common EEC variant is the skin-covered form of BE. In order to expedite appropriate management, accurate diagnosis upon initial presentation is crucial. Still, successful surgical reconstruction often results in continence that is similar to, or better than, nonvariant EEC presentations.

Is Concealed Epispadias a Rare Variant?

OBJECTIVE: To present our experience with concealed epispadias and to estimate its actual share in the isolated male epispadias cases and its effect on the surgical outcome. MATERIALS AND METHODS: Consecutive patients with isolated male epispadias treated in our center between 2008 and 2015 were classified into concealed and classic epispadias. The 2 groups were compared regarding age at presentation, meatal location, incontinence, dorsal curvature, success rate, and complications. RESULTS: Out of 51 patients with isolated male epispadias, 11 (21.6%) were concealed: 7 balanic and 4 penile shaft epispadias. Concealed epispadias cases were found to have significantly delayed age at presentation, more distal meatal location, and less incontinence rate than classic epispadias cases. None of the surgical outcome parameters showed significant difference between the 2 groups. CONCLUSION: Concealed epispadias represents about one-fifth of isolated male epispadias cases. Impediment and delay of diagnosis are its main clinical impacts, with insignificant effect on the surgical outcome.

The surgical management of male epispadias in the new millennium.

Male epispadias is a rare and challenging urologic condition. As part of the epispadias-exstrophy complex of genitourinary anomalies, it covers a broad spectrum with the mildest forms being the rarest. The anatomic classification into glanular, penile, and penopubic is based on the location of the urethral meatus. However, this classification fails to include some of the suprapubic malformations that may be present such as pubic diastasis, bladder neck abnormalities, and vesicoureteral reflux. Careful preoperative evaluation should allow the identification of these anomalies and will help achieve the goals of surgical reconstruction. These goals include cosmetic and functional reconstruction of the penis and urethra as well as, when needed, creation of a continence mechanism that will allow for normal bladder function, storage, and evacuation of urine. This review will focus on: 1) the preoperative evaluation and management of male patients with epispadias and 2) surgical management based on the anatomic classification.

Bladder exstrophy-epispadias complex.

The bladder exstrophy-epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BEEC cases are classified as non-syndromic and the etiology of this malformation is still unknown. This review presents the current state of knowledge on this multifactorial disorder, including historical retrospect, phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components. These published lines of evidence argue strongly that BEEC occurs as a result of strong genetic predisposition that is yet to be deciphered.

Variants of the exstrophy complex: a single institution experience.

PURPOSE: Variants of the bladder/cloacal exstrophy complex are rare. Different presentations and subsequent management and outcome are discussed. MATERIALS AND METHODS: We performed a retrospective review of our database of more than 815 patients with the exstrophy complex. Patients with variants of classic epispadias or bladder or cloacal exstrophy were identified. Anatomical presentation, surgical management, type of continence procedures and final outcome were evaluated. RESULTS: Of the 25 patients with variants 13 were treated primarily at our institution and 12 were referred. Time until primary bladder closure ranged from 1 day to 4 years. Followup after continence procedure ranged from 1 month to 39 years. Seven of the 25 patients are awaiting a continence procedure. Six patients are dry without a continence procedure, of whom 4 have superior vesical fistulas. A total of 11 patients underwent bladder neck reconstruction (BNR), of whom 3 are dry, 2 are dry during the day but are wet at night, 1 had a failed procedure and 5 are dry after continent diversion (CD). One additional patient underwent CD initially and is dry. Referred cases of epispadias with bladder prolapse were not recognized at birth and had delayed closure. Impaired bladder growth or failed BNR required CD in 4 patients, and 2 are awaiting a continence procedure. Skin covered and duplicate exstrophy had comparable outcomes to the classic presentations. Duplicated organs were used for reconstructive procedures. Of the 6 patients with cloacal variant 2 are continent of stool and 2 await a Pena procedure. One of these patients has an ileal stoma and 1 has a colostomy. CONCLUSIONS: The initial presentation of exstrophy variants can be confusing, often delaying initial treatment. Superior vesical fistulas permit continence without BNR due to an intact urinary sphincter. Variants such as epispadias with bladder prolapse and duplicate or skin covered exstrophy should be closed at birth with standardized techniques to promote bladder growth for later BNR. These cases are faced with the same long-term problems as the classic presentation. Cloacal variants can present with intact anal innervation, allowing a later Pena procedure.

[Three cases report on the epispadiac urethral duplication in male].

A case of complete type and two cases of incomplete type of epispadiac urethral duplication are reported. In the complete one, the accessory urethra (14 cm in length) opened 1.5 cm proximal to the dorsum of the penis. In two cases of incomplete type, the epispadiac openings located at the base of the penis or higher. The sinuses were lined with transitional epithelium proximally, and with squamous cells on the distal half of the accessory urethra, which suggests a developmental origin. We conclude that these sinuses may etiologically represent the identical origin without regard to complete type or incomplete type.