Continent stoma with Modified Mansoura Technique (Indiana W pouch with nipple valve fixation) in adult bladder exstrophy: A rare case report on step-by-step surgery technique and outcome

COMPLETE PRIMARY REPAIR OF EXSTROPHY

Surgical complications of bladder augmentation: comparison between various enterocystoplasties in 133 patients

Is Delayed Primary Bladder Exstrophy Closure the New Norm? An Analysis of the Epic Cosmos Database.

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

Evaluation of men with unilateral or bilateral undescended testes: Does age of correction make a difference?

Ureterorectostomy as a continent urinary diversion for complicated bladder exstrophy in children by using a modified Duhamel procedure: A case series

Surgical treatment of adult bladder exstrophy: A case report and literature review

Introduction: The exstrophy–epispadias complex encompasses a range of genitourinary anomalies, from less severe manifestations to the more severe classical bladder exstrophy (CBE) and cloacal exstrophy, imposing considerable physical, functional, and psychological challenges. Managing adult cases involves distinct challenges stemming from infrequent interactions and intricate patient expectations. These adults encounter specific challenges stemming from social stigma, restricted healthcare access, and unique anatomical complications, such as diminished bladder capacity, fused bony structures, altered gait patterns, variations in phallus dimensions, and an ongoing risk of malignancy in the exposed bladder. These factors lead to complications and frequently result in inferior outcomes relative to childhood repairs. Materials and Methods: In this study, we have assessed the effectiveness of various single-stage repairs. Between 2015 and 2023, seven adult patients diagnosed with CBE were treated and different surgical procedures were performed based on individual patient needs, including primary repair of exstrophy with bladder neck reconstruction, continent catheterizable pouch, ileal conduit, ureterosigmoidostomy pouch, and others. Subsequent assessments comprised the evaluation of continence, cosmesis, patient satisfaction, and complications. Results: All patients exhibited normal renal function. Six individuals attained continence, with most maintaining it for several hours, whereas two patients necessitated concomitant clean intermittent catheterization (CIC). Incontinence in one patient was managed with a suprapubic catheter. Patient satisfaction remained elevated despite the need for CIC. Two patients had infections, and one experienced bowel leakage, which was managed with intervention. All patients expressed satisfaction with the cosmetic results. Conclusion: Bladder exstrophy–epispadias, though rare, poses distinct challenges for adults. Recent surgical advancements provide significant single-stage correction and various pouch procedures, thereby expanding treatment options. These innovations markedly improve physiological function; however, they are associated with manageable postoperative complications, that affect psychological well-being and social integration.

Manitoba-oculo-tricho-anal (MOTA) syndrome is very rare syndrome characterized by aberrant hairline, eye anomalies (ocular hypertelorism, cryptophthalmos, and upper eyelid colobomas), bifid nose, omphalocele and anorectal anomalies. MOTA syndrome was first reported in 1992 in Oji-cree community from the Island Lake region of Manitoba, Canada. Till date very few cases of MOTA have been reported and none from India. We report first case of MOTA syndrome from India. A two month old male baby was brought with complaints of defect in right upper eye lid since birth. He was the second born child of a second degree consanguineously married couple at 37 weeks of gestation. On physical examination, the baby had right upper eyelid coloboma , ocular hypertelorism, bifid nose, small nasal ala and bilateral undescended testis. Investigations revealed high anorectal anomaly and right renal agenesis. Whole exome sequencing showed homozygous nonsense variation in exon 25 of the FREM1 gene that resulted in a stop codon. This case gains importance as it is the first case of MOTA being reported from India and bilateral undescended testis which was seen this case is an addition to the variable clinical spectrum of MOTA.

Salvage Procedures to Achieve Continence After Failed Bladder Exstrophy Repair

Challenges in salvaging urinary continence following failed bladder exstrophy repair in a developing country

Using social media for patient-reported outcomes: A study of genital appearance and sexual function in adult bladder exstrophy patients

EARLY OUTCOME FOLLOWING COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY IN THE NEWBORN

Testis histopathology in boys with cryptorchidism correlates with future fertility potential.

Testis Histopathology in Boys With Cryptorchidism Correlates With Future Fertility Potential