Karl Benz

Pelvic and lower extremity immobilization for cloacal exstrophy bladder and abdominal closure in neonates and older children

INTRODUCTIONnSuccessful bladder closure in cloacal exstrophy (CE) is best accomplished through a multidisciplinary team and attention to pre- and postoperative technique. This study from a high volume exstrophy center investigates outcomes and complications of primary and reoperative bladder closures in patients immobilized with spica cast or patients with external fixation (EF) and skin traction.nnnMETHODSnThe authors reviewed an institutionally approved and daily updated database of 1311 patients with exstrophy-epispadias complex and identified patients with cloacal exstrophy born between 1975 and 2015 who had undergone primary or reoperative bladder closures. Only the closures that used spica casting or external fixation were included for analysis. Demographic, operative, and outcomes data were compared between patients with spica cast only and patients with external fixation and skin traction.nnnRESULTSnOut of 140 patients with CE or a CE variant, a total of 71 patients with 94 bladder closures (66 primary and 28 reoperative) met inclusion criteria. Median follow-up time was 8.8 years (range 1.5-29.1). There were 37 closures performed at the authors institution and 58 from outside hospitals. Pelvic osteotomy was undertaken in 66 (70.2%) of all closures, and in 36 (97.3%) of closures at the authors institution. Postoperative immobilization was achieved with spica cast alone in 46 (48.9%) closures, external fixation and skin traction in 43 (45.7%), and spica cast and external fixation in 5 (5.3%) closures. For all closures, there were 33 failures (71.7%) among those immobilized with spica cast alone versus 4 failures (9.3%) for those immobilized with external fixation and skin traction (p<0.001). When restricted to closures performed with osteotomy, the failure rates were 50.0% and 9.3% respectively (p=0.002). There was minimal differences in complication rates between spica and external fixation groups (8.7% versus 23.3%, p=0.059).nnnCONCLUSIONnFailure of CE closure can occur with any form of pelvic and lower extremity immobilization. This study, however, provides continued evidence that external fixation with skin traction is an optimal, secure technique (3.8% failure rate) for postoperative management in an older child (1-2 years).nnnLEVEL OF EVIDENCEnLevel III, Retrospective comparative study STUDY TYPE: Therapeutic study.

Combined Bladder Neck Reconstruction and Continent Stoma Creation as a Suitable Alternative for Continence in Bladder Exstrophy: A Preliminary Report

OBJECTIVEnTo explore the use of concomitant bladder neck reconstruction (BNR) and creation of a continent stoma (CS) in patients who are not quite eligible for BNR but still strongly desire volitional voiding.nnnMETHODSnThe authors retrospectively reviewed an institutional database of patients with exstrophy-epispadias complex who underwent BNR-CS between 2000 and 2015. Indications for a BNR-CS, perioperative outcomes, and continence status were evaluated. Method of voiding and continence status were analyzed for patients with greater than 6 months of follow-up after the BNR-CS.nnnRESULTSnA total of 24 patients with exstrophy-epispadias complex (15 male and 9 female) underwent BNR-CS at a median age of 8.9 years (range 5.4-17.4). This included 18 patients with classic bladder exstrophy, 5 with epispadias, and 1 with a cloacal exstrophy variant. There were 5 surgical complications (20.1%) following the BNR-CS, including 3 febrile urinary tract infections, 1 superficial wound infection, and 1 urethrocutaneous fistula. The median follow-up time from the time of BNR-CS was 1.1 years (range 0.1-14.1). Seventeen of 24 patients (71%) had a follow-up greater than 6 months and were evaluated for continence. Twelve patients (71%) were completely dry for intervals greater than 3 hours following BNR-CS. Five (29%) did not achieve continence with BNR-CS. Of those 5 patients, 3 (60%) underwent subsequent bladder neck transection.nnnCONCLUSIONnCombined BNR and CS is a suitable alternative to achieve urinary continence in patients who are not ideal candidates for BNR alone. This approach can offer a select group of patients the opportunity for volitional voiding.

The Role of Human Acellular Dermis in Preventing Fistulas after Bladder Neck Transection in the Exstrophy-Epispadias Complex

OBJECTIVEnTo evaluate human acellular dermis (HAD) as an adjunct during bladder neck transection (BNT) by comparing surgical outcomes with other types of tissue interposition.nnnMETHODSnA prospectively maintained institutional database of exstrophy-epispadias complex (EEC) patients was reviewed for those who underwent a BNT with at least 6 months follow-up. The primary outcome was the occurrence of BNT-related fistulas.nnnRESULTSnIn total, 147 EEC patients underwent a BNT with a mean follow-up time of 6.9 years (range 0.52-23.35 years). There were 124 (84.4%) classic exstrophy patients, 22 (15.0%) cloacal exstrophy patients, and 1 (0.7%) penopubic epispadias patient. A total of 12 (8.2%) BNTs resulted in fistulization, including 4 vesicoperineal fistulas, 7 vesicourethral fistulas, and 1 vesicovaginal fistula. There were 5 (22.7%) fistulas in the cloacal exstrophy cohort and 7 (5.6%) fistulas in the classic bladder exstrophy cohort (Pu2009=u2009.019). Using either HAD or native tissue flaps resulted in a lower fistulization rate than using no interposed layers (5.8% vs 20.8%; Pu2009=u2009.039). Of those with HAD, the use of a fibrin sealant did not decrease fistulization rates when compared to HAD alone (6.5% vs 8.8%, Pu2009=u2009.695). There was no statistical difference in surgical complications between the use of HAD and native flaps (8.6% vs 5%, Pu2009=u2009.716).nnnCONCLUSIONnUse of soft tissue flaps and HAD is associated with decreased fistulization rates after BNT. HAD is a simple option and an effective adjunct that does not require harvesting of tissues in patients where a native flap is not feasible.

Bladder Re-augmentation in Classic Bladder Exstrophy: Risk Factors and Prevention

OBJECTIVEnTo characterize the causes of re-augmentation in patients with classic bladder exstrophy (CBE).nnnMETHODSnA prospectively maintained institutional database of 1327 exstrophy-epispadias complex patients was reviewed for patients with CBE who underwent more than 1 augmentation cystoplasty (AC) procedure. Data regarding bladder capacities, complications following AC, and reasons for re-augmentation were evaluated.nnnRESULTSnA total of 166 patients with CBE underwent AC. Of these, 67 (40.4%) were included in the control group and 17 (10%) patients underwent a re-augmentation. There were several indications for re-augmentation including continued small bladder capacity (17 of 17), inadequate bladder necks (8 of 17), failed rattail augmentation (2 of 17), stomal incontinence (1 of 17), a urethrocutaneous fistula (1 of 17), and an hourglass augmentation (1 of 17). Of note, 5 of the 17 patients (29%) had a re-augmentation procedure with a ureteral reimplantation. The sigmoid colon was the most commonly used bowel segment in the failed initial AC (8 patients), whereas the ileum was the most commonly used segment during re-augmentation (12 patients). In the re-augmentation cohort, the mean amount of bowel used during the first AC procedure was 12u2009cm (standard deviation [SD] 3.6) compared with 19u2009cm (SD 5.0) during re-augmentation. The mean amount of bowel used for control group augmentations was 20.8u2009cm (SD 4). The mean re-augmentation preoperative bladder capacity of 100u2009mL (SD 60) immediately increased after re-augmentation to 180.8u2009mL (SD 56.4) (Pu2009=u2009.0001).nnnCONCLUSIONnBladder re-augmentation is most commonly required in the setting of a small bladder capacity after an initial AC, when an insufficient amount of bowel is used during the first AC procedure.

Novel Anatomical Observations of the Prostate, Prostatic Vasculature and Penile Vasculature in Classic Bladder Exstrophy Using Magnetic Resonance Imaging

Purpose: We used magnetic resonance imaging to define the innate pelvic neurovascular course and prostatic anatomy in infants with classic bladder exstrophy before the pelvis was altered by surgery. Materials and Methods: Pelvic magnetic resonance imaging was performed in male infants with classic bladder exstrophy and compared to a group of age matched controls. Data collected included prostatic dimensions as well as course of the prostatic artery, periprostatic vessels and pudendal neurovasculature. Results: The prostate was larger in the transverse (p <0.001) and anteroposterior (p <0.001) dimensions in patients with classic bladder exstrophy compared to those with normal prostates but was smaller in the craniocaudal dimension (p <0.001). This finding resulted in a larger calculated prostate volume in patients with classic bladder exstrophy compared to controls (p = 0.015). The pelvic vasculature and prostatic artery followed a similar course in patients with classic bladder exstrophy and controls. Relative to each other, the lateral to medial course of the prostatic arteries in males with classic bladder exstrophy was less pronounced than in normal males. A similar externally rotated pattern was seen when both sides of the pudendal vasculature were compared in males with classic bladder exstrophy. Conclusions: The prostate in infants with classic bladder exstrophy has a consistent configuration and dimensions that differ from those in normal infants. When both sides are compared, the periprostatic vasculature and penile sensory neurovascular bundles are externally rotated in infants with classic bladder exstrophy. However, these components course along the same landmarks as in normal patients.

Novel Observations of Female Genital Anatomy in Classic Bladder Exstrophy Using 3-Dimensional Magnetic Resonance Imaging Reconstruction

Purpose: Understanding the distinct female anatomy in classic bladder exstrophy is crucial for optimal reconstructive and functional outcomes. We present novel quantitative anatomical data in females with classic bladder exstrophy before primary closure. Materials and Methods: 3‐Dimensional reconstruction was performed in patients undergoing pelvic magnetic resonance imaging, and pelvic anatomy was characterized, including measurements of the vagina, cervix and erectile bodies. Results: We examined magnetic resonance imaging of 5 females (mean age 5.5 months) with classic bladder exstrophy and 4 age matched controls (mean age 5.8 months). Mean distance between the anal verge and vaginal introitus was greater in patients with classic bladder exstrophy (2.43 cm) than in controls (1.62 cm). Mean total vaginal length in patients with classic bladder exstrophy was half that of controls (1.64 cm vs 3.39 cm). All 4 controls had posterior facing cervical ora, while 4 of 5 females with exstrophy had anterior facing cervical ora located in the anterior vaginal wall. Lateral deviation of the cervical ora was also seen in all 5 patients with classic bladder exstrophy but in only 1 control. Clitoral body length was comparable in both groups (26.2 mm and 28.0 mm). However, the anterior cavernosa‐to‐posterior (pelvic rami associated) cavernosa ratio was much greater in patients with classic bladder exstrophy (6.4) compared to controls (2.5). Conclusions: This study uncovers the uniquely novel finding that contrary to their male counterparts, females with classic bladder exstrophy have the majority of the clitoral body anterior to the pelvic attachment. This discovery has surgical and embryological implications.

The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy

INTRODUCTIONnNewborns with classic bladder exstrophy (CBE) may present with a bladder template that is inadequate for closure in the neonatal period (figure). In these cases, a delayed primary closure (DPC) is conducted to permit growth of the bladder template. This study reports the surgical and long-term urinary continence outcomes of poor template CBE patients undergoing DPC and compares them to patients who underwent DPC for reasons unrelated to bladder quality (i.e., prematurity, comorbidities, or a late referral).nnnMETHODSnAn institutionally approved, prospectively maintained database of 1330 exstrophy-epispadias complex patients was reviewed for CBE patients who underwent DPC at the authors institution. A bladder template was considered inadequate for neonatal closure if found to be inelastic, <3xa0cm in diameter, and/or covered in hamartomatous polyps.nnnRESULTSnIn total, 63 patients (53 male and 10 female) undergoing DPC were identified. Of these, 36 had poor bladder templates (group 1). The remaining 27 patients (group 2) had adequate templates and their bladder closure was delayed for reasons unrelated to bladder quality. At the time of DPC, those in group 1 were relatively than those in group 2 (median of 229 vs. 128 days, pxa0=xa00.094). All 36 group 1 patients and 26 (96%) group 2 patients underwent pelvic osteotomy during DPC (pxa0=xa00.429). All patients in this study had a successful primary closure. There was little difference in longitudinal bladder capacities between group 1 and group 2 (pxa0=xa00.518). Also, there was minimal difference in the median number of continence procedures between groups, with both groups having 1 (IQR 1-1) continence procedure (pxa0=xa00.880). Eight patients in group 1, and three patients in group 2 underwent a bladder neck transection with urinary diversion. Of the 13 and 16 patients who have undergone a continence procedure in group 1 and 2, respectively, 11 (84.6%) and 13 (81.3%) are continent of urine. The age of first continence procedure was different between groups 1 and 2 at 8.0 years (5.8-9.9 years) and 4.8 (3.5-6.0 years), respectively pxa0=xa00.009. The majority of patients in group 1 established continence at a relatively later age when compared to those in group 2, at 11.4 (8.0-14.8) years and 7.9 (2.6-13.2) years of age respectively pxa0=xa00.087.nnnDISCUSSIONnIn the authors view, neonatal bladder closure is ideal for CBE patients as it minimizes potential damage to exposed bladder mucosa. However, prior studies indicate that the rate of bladder growth for patients undergoing a delayed primary closure does not differ from patients with a neonatal closure. Results from this study show continued evidence that patients with poor templates who undergo delayed closure have excellent primary closure outcomes, which is critical for further management. Furthermore, this study shows that an inadequate bladder does not affect DPC outcomes or the continence outcomes in DPC patients. However, the inadequate template does affect the type of continence procedure available to a DPC patient, the age of first continence procedure, and the age of continence.nnnCONCLUSIONSnDPC of the exstrophic bladder has a high rate of success when pelvic osteotomy is utilized as an adjunct. Patients having a DPC for reasons of an inadequate bladder template have comparable rates of bladder growth when compared to DPC of an adequate bladder template. The inadequate bladder template affects the type of continence procedure, with the majority of patients requiring urinary diversion for continence. Patients with an inadequate bladder template have a later age of first continence procedure and a relatively later age of continence, because of an inherently smaller bladder template at birth. The inadequate bladder template patients require a longer period of surveillance to access bladder growth and capacity in preparation of a continence procedure. Furthermore, as the majority of inadequate bladder template patients require a catheterizable channel for continence, the age of continence is also likely influenced by the patients preparation as they transition from volitional voiding to catheterization.

Predictors and outcomes of perioperative blood transfusions in classic bladder exstrophy repair: A single institution study

BACKGROUNDnPrimary bladder closure of classic bladder exstrophy (CBE) is a major operation that occasionally requires intraoperative or postoperative (within 72xa0h) blood transfusions.nnnOBJECTIVEnThis study reported perioperative transfusion rates, risk factors for transfusion, and outcomes from a high-volume exstrophy center in primary bladder closure of CBE patients.nnnSTUDY DESIGNnA prospectively maintained, institutional exstrophy-epispadias complex database of 1305 patients was reviewed for primary CBE closures performed at the authors institution (Johns Hopkins Hospital) between 1993 and 2017. Patient and surgical factors were analyzed to determine transfusion rates, risk factors for transfusions, and outcomes. Patients were subdivided into two groups based upon the time of closure: neonatal and delayed closure.nnnRESULTSnA total of 116 patients had a primary bladder closure during 1993-2017. Seventy-three patients were closed in the neonatal period, and 43 were delayed closures. In total, 64 (55%) patients received perioperative transfusions. No transfusion reactions were observed. Twenty-five transfusions were in the neonatal closure group, yielding a transfusion rate of 34%. In comparison, 39 patients were transfused in the delayed closure group, giving a transfusion rate of 91%. Pelvic osteotomy, delayed bladder closure, higher estimated blood loss (EBL), larger pubic diastasis, and longer operative time were all associated with blood transfusion. In multivariable logistic regression, pelvic osteotomy (OR 5.4; 95% CI 1.3-22.8; Pxa0<xa00.001), higher EBL-to-weight ratio (OR 1.3; 95% CI 1.1-1.6; Pxa0=xa00.029), and more recent years of primary closure (OR 1.1; 95% CI 1.0-1.2; Pxa0=xa00.018) remained independent predictors of receiving a transfusion (Summary Table). No adverse transfusion reactions or complications were observed.nnnDISCUSSIONnThis was the first study from a single high-volume exstrophy center to explore factors that contribute to perioperative blood transfusions. Pelvic osteotomy as a risk factor was unsurprising, as the osteotomy may bleed both during and immediately after closure. However, it is important to use osteotomy for successful closure, despite the increased transfusion risk. The risks accompanying contemporary transfusions are minimal and osteotomies are imperative for successful bladder closure.nnnCONCLUSIONSnMore than half of CBE patients undergoing primary closure at a single institution received perioperative blood transfusions. While there was an association between transfusions and osteotomy, delayed primary closure, larger diastasis, increased operative time, and increased length of stay, only the use of pelvic osteotomy, higher EBL-to-weight ratio, and recent year of closure independently increased the odds of receiving a transfusion on multivariate analysis.

The intravesical phallus in patients with cloacal exstrophy: An embryologic conundrum

BACKGROUNDnPhalluses present inside the extrophied bladder of cloacal exstrophy (CE) newborns have been sporadically reported in the literature; this clinical entity has largely unknown origins and may represent an extremely rare anomaly of CE.nnnOBJECTIVEnAlong with nearly doubling the number of reported intravesical phalluses in the literature, this study aims to outline the common anatomic features and discuss the implications for theories of CE embryogenesis.nnnSTUDY DESIGNnThe authors retrospectively identified patients with CE and a potential intravesical phallus between 1997 and 2017xa0at two high-volume centers. Information was obtained about karyotype, age at closure, neurologic and renal anomalies, diastasis, phallus anatomy, and phallus biopsy pathology.nnnRESULTSnSix genotypic males met the inclusion criteria. Five of six had a diastasis greater than 6xa0cm. Four of six had paired corporal bodies in the intravesical phallus, one had a single corporal body, and one had a corporal-like structure. Five of six patients had a phallus located midline in the caudal aspect of the bladder; one was located midline in the bladder dome. Phallic biopsies were obtained in three of six patients. Two showed glanular and corporal tissue while the other showed vascular proliferation morphologically similar to that of erectile tissue.nnnDISCUSSIONnPrevious reports suggested that a superior vesicle fissure configuration, fusion of the corporal bodies, and fused bladder plates were common findings with an intravesical phallus. With the addition of new cases, the only consistent variable between patients is a phallus located anywhere along the bladder plate that can comprise a corporal-like structure, a single corporal body, or fused corporal bodies. These findings have implications for several embryologic theories. Although this is a retrospective review with a limited number of patients, the condition is exceedingly infrequent making it only observable retrospectively over decades at high volume centers.nnnCONCLUSIONSnThe study outlined common anatomic features of the intravesical phallus in cloacal exstrophy and discussed the subsequent embryologic implications. In cloacal exstrophy newborns with presumed aphallia, meticulous inspection of the bladder plate and biopsy of any potential phallic structures can prevent resection of phallic tissue.

Predictors of a successful primary bladder closure in cloacal exstrophy: A multivariable analysis

PURPOSEnTo investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure.nnnMETHODSnA prospectively maintained database of 1332 exstrophy-epispadias complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. Univariate and multivariable analyses were performed to identify significant factors associated with CE primary bladder closure.nnnRESULTSnOf 143u202fCE patients identified, 99 patients met inclusion criteria. Median follow-up time was 8.82 [IQR 5.43-14.26] years. In the multivariable model, the odds of having a successful closure are about 4 times greater for the staged cloacal approach compared to the 1-stage approach (OR, 3.7; 95% CI 1.2-11.5; p-valueu202f=u202f0.023). Also, having an osteotomy increases the chance of a successful closure by almost six-fold (OR, 5.8; 95% CI 1.7-19.6; p-valueu202f=u202f0.004).nnnCONCLUSIONSnUsing the staged approach with a pelvic osteotomy is paramount to a successful primary closure in CE. The authors strongly recommend using the staged approach and osteotomy as these factors independently increase the chance for a successful primary bladder closure.nnnSTUDY TYPEnTherapeutic study.nnnLEVEL OF EVIDENCEnLevel III, Retrospective comparative study.