Impact on Patient Care of a Multidisciplinary Center Specializing in Colorectal and Pelvic Reconstruction

Aim of the study: Many patients with an anorectal malformation (ARM) or pelvic anomaly have associated urologic or gynecologic problems. We hypothesized that our multidisciplinary center, which integrates pediatric colorectal, urologic, gynecologic and GI motility services, could impact a patient's anesthetic exposures and hospital visits.Methods: We tabulated during 2015 anesthetic/surgical events, endotracheal intubations, and clinic/hospital visits for all patients having a combined procedure.Main results: Eighty two patients underwent 132 combined procedures (Table 1). The median age at intervention was 3 years [0.2-17], and length of follow up was 25 months [7-31]. The number of procedures in patients who underwent combined surgery was lower as compared to if they had been done independently [1(1-5) vs. 3(2-7) (p < 0.001)]. Intubations were also lower [1[1-3] vs. 2[1-6]; p < 0.001]. Hospital length of stay was significantly lower for the combined procedures vs. the theoretical individual procedures [8 days [3-20] vs. 10 days [4-16]] p < 0.05. Post-operative clinic visits were fewer when combined visits were coordinated as compared to the theoretical individual clinic visits (urology, gynecology, and colorectal) [1[1-4] vs. 2[1-6]; p = < 0.001].Conclusions: Patients with anorectal and pelvic malformations are likely to have many medical or surgical interventions during their lifetime. A multidisciplinary approach can reduce surgical interventions, anesthetic procedures, endotracheal intubations, and hospital/outpatient visits

Detectable clonal mosaicism and its relationship to aging and cancer

In an analysis of 31,717 cancer cases and 26,136 cancer-free controls from 13 genome-wide association studies, we observed large chromosomal abnormalities in a subset of clones in DNA obtained from blood or buccal samples. We observed mosaic abnormalities, either aneuploidy or copy-neutral loss of heterozygosity, of >2 Mb in size in autosomes of 517 individuals (0.89%), with abnormal cell proportions of between 7% and 95%. In cancer-free individuals, frequency increased with age, from 0.23% under 50 years to 1.91% between 75 and 79 years (P = 4.8 × 10(-8)). Mosaic abnormalities were more frequent in individuals with solid tumors (0.97% versus 0.74% in cancer-free individuals; odds ratio (OR) = 1.25; P = 0.016), with stronger association with cases who had DNA collected before diagnosis or treatment (OR = 1.45; P = 0.0005). Detectable mosaicism was also more common in individuals for whom DNA was collected at least 1 year before diagnosis with leukemia compared to cancer-free individuals (OR = 35.4; P = 3.8 × 10(-11)). These findings underscore the time-dependent nature of somatic events in the etiology of cancer and potentially other late-onset diseases

Update on plication procedures for Peyronie’s disease and other penile deformities

Plication techniques are not a panacea for deformities associated with Peyronie’s disease or congenital curvature. However, they do provide certain advantages, both theoretic and real, over competing procedures such as grafting. Depending on the technique, plication procedures have minimal risk of de novo erectile dysfunction, minimal risk of injury to the dorsal neurovascular bundle, and may be used for a variety of angulation deformities, including multiplanar curvature and severe degrees of curvature. A variety of incisions may be used, including the classic circumcision with degloving but also ventral raphe, dorsal penile inversion, and penoscrotal. These may be helpful in preventing postoperative morbidity and in sparing the prepuce if desired. Plication may also be combined with procedures such as penile prosthesis for correction of residual curvature. Lastly, despite its complications, plication techniques are very well tolerated, are relatively simple to perform and result in the very high satisfaction rates

The Association Between BXO and Obesity in Boys Undergoing Circumcision

This study investigated whether boys with balanitis xerotica obliterans (BXO) have increased rates of obesity compared with boys with no concern for BXO (NCB). Boys ≤18 years old with circumcision pathology–confirmed BXO were compared with an age-matched group who had NCB during circumcision. Boys with BXO were found to have a mean body mass index of 70.64 percentile for age compared with 52.43 percentile in age-matched controls ( P = .0005). The rate of obesity was significantly higher in boys with BXO (42%) compared with 12.4% in boys with NCB (odds ratio = 5.12; 95% CI = 2.6 to 10.06). Given the increasing rates of childhood obesity and the long-term health consequences of both BXO and obesity, special attention should be paid to this population. Further research is needed to determine if BXO in obese children may represent an early indicator of a systemic disease process where intervention may be warranted

Erratum to “Cloaca reconstruction: A new algorithm which considers the role of urethral length in determining surgical planning” [YJPSU 53/1 (2018) 90-95](S0022346817306449)(10.1016/j.jpedsurg.2017.10.022)

© 2018 The publisher regrets to inform that in regard to the manuscript titled “Cloaca Reconstruction: A New Algorithm which Considers the Role of Urethral Length in Determining Surgical Planning” by R. J. Wood, et al, published in the January 2018 APSA issue of the Journal, a very significant Discussion was missed to be included as part of the article. It is as below. The publisher would like to apologise for any inconvenience caused. UNIDENTIFIED SPEAKER: Wow, that was beautifully presented work, and it sounds like it\u27s going to help a lot of these children. My question is, what do you think the difference is? Does it have to do with the urethral sphincter mechanism and those with longer urethras generally have more native sphincter-type function? RICHARD WOOD: I think it\u27s probably multifaceted. Thank you for the question. I think we certainly are concerned that in patients with a very short urethra and just by bringing the bladder neck really low, it appears to affect their function. We haven\u27t noted with the patients that we\u27ve left the common channel with the urethra that they have had a difficulty to empty. Obviously that\u27s something that one needs to look at and see whether over the long term more patients need intermittent cath, but that has not been our experience for now. I don\u27t know entirely. I just feel that most children with a urogenital sinus have a normal bladder function, and a lot of kids with cloaca have abnormal bladder function. If the urologists think it\u27s that important to maintain the urethral length in patients with normal bladders, how much more so is it important for us to maintain that in children who in many cases have abnormalities to their bladder function. So we\u27re not entirely sure why, but it would appear that there is benefit. JASON FRISCHER (Cincinnati, OH): Great presentation and thank you for challenging us to try to continue to make and have better outcomes in this patient population. Couple questions. One, when you measure the urethral length, you showed a cloacagram, but I assume you also perform a cystoscopy. Which measurement do you use as your more preferred measurement for determining that 1.5-cm length? My other question is, does age play a role when you are performing this? In a 2 year old, you\u27re doing redo operations. Does urethral length in a 2 year old, or if a patient was diverted and you just encountered them at 2 years of age, is that a different length than a 3 month old that you are doing your original cloacagram? And then third question is more of a comment. I guess we really need to know what the functional outcomes of these patients are, and so do you have any data on that? I look forward to this presentation in 3-5 years again. RICHARD WOOD: Thank you very much. To answer your first question, we do measure and use the cloacagram. We will present that data in time when I think we have enough to significantly say, but my impression is the cloacagram may be better because we can – I think we can kid ourselves when we\u27re doing the cystoscopy by applying pressure. Same problem with ultrasound of the perineum. Functional outcomes, whereas we don\u27t have a lot of data yet and we have to wait for these patients to get older, I think we have anatomical data that the urethra is surviving, and they are not leaking, but we don\u27t have functional data yet. All our patients get urodynamics before surgery, and we follow them with urodynamics and other parameters, so hopefully we will be able to present that data in time. JASON FRISCHER: And age? RICHARD WOOD: We haven\u27t specifically used this protocol for the redos, but we are mindful of it because I think a lot of times the decisions are often made on the urethra by the time they come to for redo, but I think it is something we definitely consider in every single patient and the goal to maintain as much urinary continence as possible is certainly forefront to the discussion. Thank you. W. HARDY HENDREN (Duxbury, MA): I don\u27t have a question, but I would like to say that I agree completely with the message that you\u27ve just given us and to add that the time to do it is the first operation if you\u27re lucky enough to get one with the first operation. I got many of these kids who had had 2-3 operations before, and it\u27s tougher each time, so the time to do it is initially if it looks too short. Thank you. RICHARD WOOD: Thank you very much. MARC LEVITT (Columbus, OH): If I may, I don\u27t mean to follow Dr. Hendren, but I just want to thank him for a career of service on this very difficult patient population and also to Dr. Pena who made a lot of strides in addition to Dr. Hendren\u27s work. I was there looking over Dr. Pena\u27s and Dr. Hendren\u27s shoulder when Alberto for the first time showed Dr. Hendren what the urogenital mobilization was all about, and I remember the fascination in his eyes that it was going to advance the field, and in fact it did. What this paper was meant to show is that it was an incredible advance, but it cannot be applied too broadly. We have to go back to the original principles that Dr. Hendren gave us of the importance of the bladder neck, the urethral length, and urinary continence in order to get the best possible result. Thank you.[Figure presented

Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning

© 2017 Elsevier Inc. Background: Cloacal malformations represent a uniquely complex challenge for surgeons. The surgical approach to date has been based on the common channel (CC) length with two patient groups considered: less than or greater than 3 cm, which we believe is an oversimplification. We reviewed 19 patients, referred after surgery done elsewhere. Eight had postoperative urinary complications, 3 had constant urinary leakage and had been left after surgery with a urethra \u3c 1 cm,.5 with an original 3 to 5 cm common channel, who had undergone total urogenital mobilization (TUM), experienced peri-operative urethral loss needing a vesicostomy, and later, a Mitrofanoff. These patients together with a review of the cloacal and urological literature led us to design a new algorithm where urethral length is a key determinant for care. Methods: We prospectively collected data on 31 consecutive cloaca patients referred to our team (2014 to 2016) and managed according to this new protocol. The CC length, urethral length, surgical technique employed, and initial outcomes were recorded. Results: Of 31 primary cases, CC length was 1 to 3 cm in 20, 3 to 5 cm in 9, and greater than 5 cm in 2. In the 1 to 3 cm and the 3 to 5 cm groups, a urethra less than 1.5 cm led us to perform an urogenital separation. We only performed a TUM if the urethra was greater than 1.5 cm. Using this protocol, we performed a urogenital separation in 1 of 20 in the 1 to 3 cm CC group, 6 of 9 in the 3 to 5 cm CC group, and 2 of 2 in the greater than 5 cm CC group. Seven patients underwent separation, who with the previous approach, would have had a TUM. Thus far, no urinary leakage or urethral loss has occurred in any patient, but follow-up is less than 3 years. Conclusion: Urethral length appears to be a vitally important component in cloacal reconstruction. A short urethra left after repair can lead to urinary leakage. A TUM done under the wrong circumstances can lead to urethral loss. We describe a new technical approach to cloacal repair which considers urethral length but recognize that long term urological outcomes will need to be carefully documented. Type of study: Clinical cohort study with no comparative group. Level of evidence: Level 4

Anatomic factors predict urinary continence in patient with anorectal malformation

© 2020 Journal of Pediatric Urology Company It has been described that patients with more complex anorectal malformations (ARM), lower sacral ratios and spinal anomalies have poorer rates of fecal and urinary continence. While the ARM subtype has been shown to be an independent predictor of fecal continence, it is not well understood how each of these anatomic factors impact urinary continence. The purpose of this study was to identify anatomic factors associated with urinary continence in children born with ARM. We performed a retrospective review of a large prospectively collected database of children with ARM. Inclusion criteria included diagnosis of ARM, age \u3e4 years, available lateral sacral ratio measurement and presence of spinal MRI. Any child with incomplete or absent continence data was excluded. Continence was defined as voiding per urethra volitionally, dry between voids and ≤1 urinary accident per week. Bivariable tests of association and log-binomial regression models were used to examine association between anatomic factors and urinary continence. A total of 434 patients were included in the study. 57.8% (n = 251) were male. Median age was 8.4 years (IQR 6.0–12.3). With regards to severity of ARM, 20.3% (n = 88) were complex, 23.3% (n = 101) were moderate and 56.5% (n = 245) were simple. Lateral sacral ratio included 11.1% (n = 48) that were \u3c0.4, 36.2% (n = 157) 0.4–0.7 and 52.8% (n = 229) \u3e 0.7. Spine status was found to be myelomeningocele in 4.4% (n = 19), low conus or tethered cord in 34.8% (n = 151) and normal or fatty filum in 60.8% (n = 264). Overall 62.2% were continent. ARM severity, lateral sacral ratio and spine status were each independent predictors of urinary continence on univariate and multivariable analysis. We conclude that in children born with ARM, the severity of ARM, lateral sacral ratio and spine status each independently predict urinary continence. These results allow us to better understand these complex patients and their ability to develop urinary continence. This is crucial in enabling proper patient and family counseling and thus, setting appropriate expectations.[Formula presented

Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study

BACKGROUND: We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study. METHODS: We performed a cross-sectional study of people with CE and covered variants managed at five participating institutions. Those with 2000). RESULTS: A total of 160 patients were included (40% male). Median follow-up was 15.2 years (36% children, 22% older children, 43% adults). While 42% of children were incontinent in diapers, 73% of older children and adults managed their bladder with CIC, followed by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%). Among older children and adults, 86% did not evacuate urine per urethra and 28% of adults had an incontinent urinary diversion. No child or adult voided per urethra. Age-adjusted odds of undergoing incontinent diversion was no different between institutions (p = 0.31) or based on birthyear (p = 0.08). Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining patients had a pull-through, half with a MACE. The probability of undergoing bowel diversion varied significantly between institutions (range: 55-91%, p = 0.001), but not birth year (p = 0.85). SUMMARY: We believe this large long-term data presents a sobering but realistic view of outcomes in CE. A limitation is our data does not assess comorbidities or patient-reported outcomes. Rarity of volitional urethral voiding in CE forces the question of whether is a potentially unachievable goal. We advocate thoughtful surgical decision making and thorough counseling about appropriate expectations, distinguishing between volitional voiding and urinary and fecal dryness. CONCLUSIONS: In this long-term, multi-institutional study of patients with CE, 94% of older children and adults manage their bladder with incontinent diversion or CIC. Nearly 80% of patients, regardless of age, have an intestinal diversion. Given that no patients were dry and voided via urethra and 86% of older patients do not evacuate urine per urethra, these data bring into question what functional goals are achievable when performing reconstructive surgery for these patients