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Summary Background The decreased penile length in patients born with BE results partly from pubic symphysis diastasis and the separation of the corporal bodies, which causes a shortened penis as the corporal length is lost in traversing the distance between the pubic rami. However, in some cases there is an intrinsic penile abnormality and dorsal chordee. Furthermore, multiple surgeries has in some cases, resulted in cutaneous and subcutaneous scarring, which contributed to the problem of the short phallus and dorsal tethering to the abdominal wall (figure). Objective Herein we evaluated the outcome of penile lengthening, repair of penile upward tethering to the abdominal wall and dorsal curvature in males born with bladder exstrophy and epispadias (BEE). Study design We reviewed the records of 34 patients (11–29 years old) born with BE (31pts.) and epispadias (3 pts.). The parents and/or the young men were referred because of their dissatisfaction with and complains of short penile length, and upward tethering/chordee. The penile lengthening was achieved by detaching the corporal bodies from the pubic rami and suturing the corporal bodies as reported by Johnston (figure) However, in 11 patients who had had multiple surgeries the periosteum was incised and the corporal dissection was performed subperiosteally to protect the erectile tissues. In 16 pts dermal grafts of the dorsal corporal wall was performed to correct the dorsal curvature. 13 patients underwent single stage augmentation urethroplasty. Results Surgical complications were encountered in 4 pts (11.7%). Subjective evaluation by the patient and/or parents reported satisfactory and/or very satisfactory results in 31/34 (91%). The degree of penile lengthening measured at 6 months and one year postoperatively showed increased length which varied between 50% and 150% of the preoperative penile length. Conclusions As children born with BEE transition to adolescence and adulthood, the external genitalia acquire greater importance. The short phallus and/or dorsal chordee and/or upwards tethering can be corrected successfully in the majority of patients. Download : Download high-res image (492KB) Download : Download full-size image Summary Figure .

Recent articles document successful classic bladder exstrophy (CBE) closure without osteotomy. Still, many patients require osteotomy if they have a large bladder template and pubic diastasis, or non-malleable pelvis.To understand the indications and outcomes of bladder closure with and without pelvic osteotomy in patients younger than 1 month of age.An institutional database of 1217 exstrophy-epispadias patients was reviewed for CBE patients closed at the authors' institution within the first month of life. Patient demographics, closure history, pubic diastasis distance, bladder capacity, and outcomes were recorded and compared using chi-square tests between osteotomy and non-osteotomy patients. Failure was defined as bladder dehiscence, prolapse, vesicocutaneous fistula, or bladder outlet obstruction requiring reoperation. Bladder capacity100 mL was deemed sufficient for bladder neck reconstruction (BNR).One hundred CBE patients were included for analysis: 38 closed with osteotomy (26 male, 12 female), and 62 closed without osteotomy (42 male, 20 female). There were four failed closures in the osteotomy group (2 dehiscence, 2 prolapse) and four failed closures in the non-osteotomy group (2 dehiscence, 2 prolapse). This corresponded to statistically equivalent rates of failure between the osteotomy and non-osteotomy groups (10.5% vs. 6.5%, p = 0.466). There was no statistically significant difference between the groups' ability to achieve bladder capacity sufficient for BNR (82% vs. 71%, p = 0.234).A successful primary bladder closure, regardless of the use of osteotomy, has been shown to be the single most important predictor of eventual continence. Because of the complexity of exstrophy manifestations, a multidisciplinary team approach is of the utmost importance. Based on our institutional experience, closure without osteotomy is considered when patients are72 h of life, have a pubic diastasis4 cm, malleable pelvis, and pubic apposition without difficulty. Rates of successful closure and attaining sufficient capacity for BNR were both statistically equivalent across groups. This retrospective study is limited by selection bias and the significant difference in follow-up time between groups. Nevertheless, as a high-volume exstrophy center this study draws from one of the largest cohorts available.Regardless of the type of closure undertaken, there clearly is a role for newborn CBE closure without pelvic osteotomy in patients considered suitable for closure by both the pediatric urologist and orthopedic consultant. However, if there is any doubt concerning pubic diastasis width, pelvic malleability, or ease of pubic apposition, an osteotomy is highly recommended.

Summary Introduction/Objective Secure closure of the pubic diastasis during bladder exstrophy and epispadias repair decreases the abdominal wall tension at the time of reconstruction. Pelvic osteotomies are routinely performed at the time of abdominal wall and bladder reconstruction in order to more easily facilitate pubic symphyseal diastasis approximation. Postoperative pelvic immobilization is performed by methods that include modified Buck's traction, modified Bryant's traction, and spica casting. People undergoing closure often require inpatient hospitalization for 2–8 weeks because of the pelvic immobilization. The present study examined the findings from a clinical pathway for early discharge after complete primary repair of exstrophy (CPRE) and proximal epispadias repair with spica casting. Methods The present study is a retrospective review of patients that underwent pelvic osteotomies with spica casting at the time of CPRE or proximal epispadias repair from November 2006 to March 2013. All patients had anterior innominate osteotomies and spica cast pelvic immobilization. Results Pelvic osteotomies and spica cast pelvic immobilization were performed on 17 children. The median postoperative stay was 6.0 days and the subdivided results are in Table. No children experienced an abdominal or orthopedic complication. A few children required minor cast adjustments to relieve pressure. After cast removal, no skin breakdown, pressure necrosis, or nerve palsy were found. The median length of casting without pinning was 31 (26–48) days. Table . Length of hospital stay Total Male/Female Median age at surgery (days) Median postoperative stay (days) Primary bladder exstrophy 11 5/6 4.0 (1–125) 6.5 (3–15) a Bladder exstrophy revision 4 2/2 404 (79–1764) 5.5 (5–8) Epispadias 2 1/1 134 (71–197) 5.0 (4–6) Total 17 8/9 15.0 (1–1764) 6.0 (3–15) a Exclusion of one patient with prolonged hospital course secondary to seizure disorder. Discussion The use of spica cast pelvic immobilization after exstrophy and epispadias repair is safe and allows for earlier discharge when compared to other methods of pelvic immobilization. However, although the family appreciates early discharge and additional bonding, the priority is the success of the closure. The present findings demonstrate, and are corroborated by other spica cast publications, that spica casting is as effective as modified Bryant's traction or modified Buck's traction. The success rates for CPRE with spica casting are similar to published staged repairs and have the benefit of allowing for bladder cycling, which potentially enables better bladder growth and development. If success and complication rates are comparable amongst the different pelvic immobilization groups, then variables including hospital length of stay and cost become appropriate comparisons. Conclusion The shortened discharge time, along with a significant decrease in acuity of care leads to significant decreases in hospital costs. Additional hospital stay when using modified Bryant's traction or modified Buck's traction with external fixation will accrue significant hospital costs.

Purpose Although the management of patients born with bladder exstrophy (BE) may include pelvic osteotomies, nothing is known about the long-term natural history of the pelvic deformity. We assessed the orthopaedic outcome in adult patients. Material and methods 22 BE patients treated with ureterosigmoidostomies between 1945 and 1968 were evaluated clinically using Harris hip scores and radiographically using Tonnis classification. Results The mean age of the patients was 50 ±12 years. Clinical evaluation revealed an excellent outcome resulting in 91-100 points of the Harris Hip Score in 80 % of the left and in 76 % of the right hips. Good outcomes of 81-90 points were found in 10 % of the left hips and in 14 % of the right hips, while fair outcomes of 71-80 points were found in the remaining 10 % of both hips. No patient scored less than 70 points. 16% of the patients were classified as Tonnis grade 0, 63% as grade 1 and 21% were classified as grade 2. No patient was classified as Tonnis grade 3. The mean pubic symphysis diastasis was 87 mm (range: 30-152 mm). The mean CE angle on the right hand side varied from 24-44° (mean CE angle 35°) and on the left from 24-50° (mean CE angle 36°). No instability of the symphysis nor the sacroiliac joint was detected. Conclusions We concluded that patients with bladder exstrophy and an untreated pelvic deformity are not at risk of a higher incidence or earlier onset of hip arthrosis than normals. Long-term follow-up reveals no pelvic instability.