A primigravida in the extremist-affected region of a third-world nation gave birth to a newborn who was remotely consulted through video rounds from the capital of the state. Unfortunately, these abnormalities are often overlooked and left untreated. The baby had multiple limb defects, gastroschisis, exstrophy of the bladder and spina bifida. Tragically, the newborn did not survive due to the lack of clinical and surgical expertise in the area. It is crucial to emphasise the importance of establishing e-clinics for expectant mothers in underserved areas, providing them with access to high-quality anomaly scans.

OBJECTIVE: To answer the question of whether it is possible to achieve complete corporal covering of the urethral closure using incomplete penile disassembly in classic bladder exstrophy. We hypothesize that mobilization of the corpora under Buck\'s fascia, their dorsal translocation through the incisions in Buck\'s fascia and suturing corporal convex sides above the urethra would allow extend corporal covering of the urethra, reducing the risk of urethra-cutaneous fistula formation.
METHODS: A prospective follow-up on all boys who underwent the modified Cantwell-Ransley primary penile reconstruction was conducted. Inclusion criteria comprised bladder exstrophy closure in our institution, ensuring a postoperative follow-up period of no less than 24 months. The key innovation of the technique lies in a deep dissection of the dependent corpora under Buck\'s fascia, followed by their dorsal relocation through extended dorsal incisions in Buck\'s fascia, and limited external corporal rotation 90 degrees only at the base of the penis.
RESULTS: Between November 2019 and March 2022, 18 boys aged 11 to 35 months met the inclusion criteria and underwent the modified penile reconstruction. Surgical procedures and postoperative period did not include any major complications. Total corporal covering of the urethral sutures was achieved in 15 of 18 patients. No urethra-cutaneous fistulas were observed within 2 years of follow-up. All individuals demonstrated spontaneous erections, and the absence of dorsal curvature was documented.
CONCLUSIONS: The modified technique of incomplete penile disassembly applied in a homogenous group of patients with classic bladder exstrophy allows penile shaft elongation, improved aesthetic outcomes, preserved erections, and eliminates dorsal curvature. The technique demonstrated feasibility and reliability while maintaining positive effects on tissue circulation. The absence of urethra-cutaneous fistulae is attributed to the complete corporal covering of the urethral sutures and supports the initial hypothesis.

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The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars\' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

BACKGROUND: The assessment of pubic diastasis is important for the surgical planning of patients with bladder exstrophy-epispadias complex. Understanding how the diastasis changes during surgical follow-up may help predict patient morbidity. Radiography can follow diastasis but may be affected by patient and technical imaging factors including body size, imaging protocol, and equipment. Using imaging calibration and anatomic ratios may mitigate differences due to these aspects.
OBJECTIVE: Use imaging phantoms to assess the effect of radiographic calibration on measurements of pubic diastasis and an internal anatomic ratio as a child grows.
METHODS: Radiographic images were obtained of three different sizes of computed tomography phantoms (older child, child, and infant) using three imaging techniques that include the osseous pelvis in children. All phantoms were imaged with abdomen and pelvis techniques. The infant phantom was additionally imaged using a thoracoabdominal technique. These exposures were all repeated with systems from three manufacturers. Linear measurements were made between radiographic markers placed to simulate pubic diastasis and sacral width. A ratio was also created between these distances. Measurements with and without image calibration were made by two pediatric radiologists using rulers placed at the time of image acquisition.
RESULTS: There was excellent interrater agreement for measurements, ICC >0.99. Anterior distances were more affected by magnification than posterior ones with a significant difference between uncalibrated versus calibrated anterior distances (p=0.04) and not for posterior ones (p=0.65). There was no difference between radiographic equipment manufacturers without or with calibration (p values 0.66 to 0.99). There was a significant difference in simulated pubic distance between thoracoabdominal and abdomen (p=0.04) as well as pelvic (p=0.04) techniques which resolved with calibration, each p=0.6. The ratio between the simulated pubic diastasis and sacral width differed by phantom size (all p<0.01) and imaging technique (p values 0.01 to 0.03) with or without calibration. However, the numerical differences may not be clinically significant.
CONCLUSIONS: Image calibration results in more uniform measurements that are more accurate than uncalibrated ones across patient size, imaging techniques, and equipment. Image calibration is necessary for accurate measurement of inter-pubic distances on all projection imaging. Small differences in the pelvic ratio likely are not clinically significant, but until there is a better understanding, image calibration may be prudent.

UNASSIGNED: This case report describes the successful use of a latissimus dorsi muscle flap attached to the femoral vessels to cover a large mass loss in a 27-year-old woman with bladder exstrophy. The two-stage reconstruction showed excellent results six months after surgery.
METHODS: A 27-year-old woman with bladder exstrophy and previous enterocystoplasty with Mitrofanoff-type urinary diversion combined with a late fascial flap presented 10 years later with disembowelment near the pubic symphysis. A two-stage reconstruction using a latissimus dorsi flap and polypropylene mesh was performed with excellent aesthetic and functional results.
UNASSIGNED: The latissimus dorsi muscle flap, described by J.M. Servant in 1984 as the \"apple turnover\" technique, is highly reliable for reconstructing large substance losses with minimal functional sequelae. The procedure in this case resulted in very satisfactory aesthetic and functional results at six months postoperatively. This approach provided a safe and effective technique of last resort.
CONCLUSIONS: the two-stage latissimus dorsi flap technique was a reliable, safe and effective solution for this complex reconstructive challenge.

UNASSIGNED: Bladder exstrophy epispadias complex (BEEC) is a rare congenital anomaly of unknown etiology, although, genetic and environmental factors have been associated with its development. Variants in several genes expressed in the urogenital pathway have been reported as causative for bladder exstrophy in human and murine models. The expansion of next-generation sequencing and molecular genomics has improved our ability to identify the underlying genetic causes of similarly complex diseases and could thus assist with the investigation of the molecular basis of BEEC.
UNASSIGNED: The objective was to identify the presence of rare heterozygous variants in genes previously implicated in bladder exstrophy and correlate them with the presence or absence of bladder regeneration in our study population.
UNASSIGNED: We present a case series of 12 patients with BEEC who had bladder biopsies performed by pediatric urology during bladder neck reconstruction or bladder augmentation. Cases were classified as \"sufficient\" or \"insufficient\" (n = 5 and 7, respectively) based on a bladder volume of greater than or less than 40% of expected bladder size. Control bladder tissue specimens were obtained from patients (n = 6) undergoing biopsies for conditions other than bladder exstrophy. Whole exome sequencing was performed on DNA isolated from the bladder specimens. Based on the hypothesis of de novo mutations, as well as the potential implications of autosomal dominant conditions with incomplete penetrance, each case was evaluated for autosomal dominant variants in a set of genes previously implicated in BEEC.
UNASSIGNED: Our review of the literature identified 44 genes that have been implicated in human models of bladder exstrophy. Our whole exome sequencing data analysis identified rare variants in two of these genes among the cases classified as sufficient, and seven variants in five of these genes among the cases classified as insufficient.
UNASSIGNED: We identified rare variants in seven previously implicated genes in our BEEC specimens. Additional research is needed to further understand the cellular signaling underlying this potentially genetically heterogeneous embryological condition.

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BACKGROUND: Achieving urinary continence is a key goal in children born with the bladder exstrophy-epispadias complex (BEEC). Unfortunately, this goal is only moderately achieved despite sometimes extensive surgical treatment. Undergoing repeated hospitalization and operations may consequently have a negative impact on quality of life. We therefore believe that other, conservative treatment options should be explored in an earlier stage of incontinence treatment in BEEC patients. As part of this, an intensive urotherapy program based on was offered to patients with persistent incontinence after reconstructive surgery for BEEC.
OBJECTIVE: The aim of this study is to evaluate the benefits of intensive urotherapy on incontinence after reconstructive surgery in children with BEEC.
METHODS: A retrospective chart study was performed including all children who were enrolled in an intensive urotherapy program because of persistent incontinence after reconstructive surgery for BEEC. Urotherapy consisted of a ten-day inpatient training program based on cognitive behavioral therapy, with intensive follow-up by experienced urotherapists. Main outcome measurement was continence, expressed as the percentage of children that achieved complete continence (good result; 100% dry) or 50-99% decrease of wet days a week (improved result) after treatment.
RESULTS: Data of 33 patients with a mean age of 10.6 years were analyzed. In 61% of cases (20/33) an improved or good result was reported on incontinence after urotherapy. Children with classic bladder exstrophy more often achieved a good or improved result (13/16; 81%), compared to children with epispadias (6/16; 38%). The only patient with a cloacal exstrophy completed treatment with an improved result. From the group of patients with persistent incontinence, 75% (12/16) reported that the complaints were socially acceptable at the end of follow-up.
CONCLUSIONS: By following our intensive urotherapy program the majority of patients achieved complete continence or improved incontinence. In addition, our results show that the inpatient training program has a positive impact on acceptance in cases of persistent incontinence. The urotherapists offer individualized care and clear guidance, which we deem essential elements of successful treatment. Considering that repeated surgery may impede progress and offers no guarantee of continence, we recommend giving preference to conservative treatment options.
CONCLUSIONS: Our results show that 37% (12/33) of patients with BEEC who were enrolled in our intensive urotherapy program because of persistent incontinence after reconstructive surgery, achieved complete continence after urotherapy and 63% (21/33) still experienced some degree of incontinence. 75% of patients who did not achieve complete continence, described the remaining incontinence as socially acceptable. These findings strongly support counselling patients with BEEC to consider conservative treatment before opting for further surgery.