Management and Outcomes of Wilms Tumor With Suprarenal Intravascular Extension: A Pediatric Surgical Oncology Research Collaborative Study.

OBJECTIVE: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus. BACKGROUND: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported. METHODS: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed. RESULTS: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P =0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P =0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P =0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival. CONCLUSIONS: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary.

Redo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy-epispadias complex complications. Technical principles and pearls based on case scenarios.

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.

A survey of management preferences for bladder exstrophy and cloacal anomalies among Canadian pediatric surgeons and urologists.

PURPOSE: This study examined the current referral patterns and preferences of Canadian Association of Paediatric Surgeons (CAPS) and Pediatric Urologists of Canada (PUC) members for management of bladder exstrophy and cloacal anomalies (BECA). METHODS: We invited CAPS and PUC members to participate in an online survey using RedCap. Demographic variables, years in practice, current referral patterns and local expertise at the participants' institution were collected. Participants' preferences towards three distinct referral models were assessed using Likert scales: no centralization of care, centralization in one or two national centres of excellence, or a consortium-based approach. RESULTS: There were 82 survey respondents (2/3 were CAPS members, 35.4% female, 72% in practice for >10 years). Although >90% of participants agreed/somewhat agreed that surgical volumes impact outcomes, 58% reported not referring out BECA patients for treatment; about 50% recognized the existence of a local dedicated expert. In terms of referral preferences, 84% of participants favoured identification of a few centres with expertise based on geographic location (a consortium-based approach), while only 7% chose a one or two national centres of excellence model. Over half of participants agreed/somewhat agreed with participating in trials of a consortium-based approach in Canada. CONCLUSION: Most CAPS and PUC members do not refer BECA patients elsewhere for treatment. Nonetheless, most surgeons recognize the importance of volume to improve outcomes and show willingness to participate in trials to concentrate experience; most participants favour a consortium-based approach through identification of a few centres of excellence based on geographic location.

Comparing quality of life improvement after antegrade continence enema (ACE) therapy for patients with organic and functional constipation / encopresis.

BACKGROUND: We compared patient- and family-reported overall and stool-related quality of life (QoL) before and after an antegrade continence enema (ACE) procedure (cecostomy tube insertion) for refractory chronic constipation or fecal incontinence (CCFI). We hypothesized that patients with functional diagnoses experience similar improvements in QoL compared to those with organic diagnoses. METHODS: This is a cross-sectional study of patients undergoing cecostomy tube insertion for CCFI at a tertiary pediatric hospital from 2012 to 2019. Patients and/or primary caregivers completed validated stooling and overall QoL surveys based on three time points: before surgery, three months after surgery, and at the time of survey / date of last follow-up. Repeated measures analyses compared scores over time between subjects and within the diagnostic groups. RESULTS: The response rate was 65% (22/34 patients, 12 organic and 10 functional diagnoses). Mean age was 8.3 years and 32% of the participants were female. Organic diagnoses were: spina bifida (6), anorectal malformation (5), and Hirschsprung Disease (1). There was substantial improvement in stool-related and overall QoL at three months post-ACE procedure (both p<0.001) for all patients; both scores continued to improve significantly until the date of last follow-up (median 4.1 years, IQR 2.3-5.6, p<0.001). There was no statistically significant difference in scores between patients with organic and functional diagnoses. CONCLUSIONS: Caregivers perceive a significant, sustainable improvement in stooling habits and QoL following ACE therapy. The improvement is comparable between patients with a functional diagnosis and those with an underlying organic reason for their CCFI.

Incidence of fertility preservation procedures in prepubertal individuals with cancer.

BACKGROUND: Fertility Preservation (FP) for children and adolescents with cancer is underutilized. In prepubertal individuals, ovarian and testicular tissue can be frozen; however, this is still considered largely experimental. Our objective was to identify trends of FP in prepubertal individuals. METHODS: We performed a retrospective study of prepubertal children with cancer identified through the Pediatric Health Information System from 2011 to 2020. Children who underwent a testicular or ovarian biopsy were included. Any patients with testicular or ovarian malignancy, or other diagnoses which may have required a gonadal biopsy were excluded. RESULTS: A total of 418 boys under 13 and 333 girls under 12 who underwent a gonadal biopsy were identified. There was a total of 66,929 new cancer diagnoses in girls and 86,001 new cancer diagnoses in boys during this time. The most common cancer diagnosis was hematologic in both boys (50.96%) and girls (36.64%). A concurrent procedure at time of gonadal biopsy was performed in 84% of boys and 62% of girls, with line insertion being the most common. The only predictive variable of receiving a gonadal biopsy was increasing year. Overall, only 0.04% of children had a gonadal biopsy for FP during this time period. CONCLUSIONS: Gonadal biopsy rates have increased in prepubertal children with cancer, presumably for FP. While recent international guidelines support FP in this group, our findings highlight the need to establish protocols and tracking for FP procedures in the US.

Individualized care for patients with intersex (disorders/differences of sex development): Part 3.

The focus of this article is to review the complex determinants of gender assignment in a child with a disorder of sex development using four different clinical cases. While the care of patients with DSD may be shared across several specialties and opinions regarding their management may vary, this may be further complicated by psychosocial, cultural and economic factors. In this regard, access to behavioral health specialists with experience and specialization in the treatment of patients with DSD should be a foundational component of the standard of care and can greatly assist in the complex decision-making regarding gender assignment. We recommend an individualized approach by a multidisciplinary team utilizing a range of evolving strategies, including outcome data (or lack thereof) to support families during the decision-making process.

Predictors for surgical intervention and surgical outcomes in neonates with cystic fibrosis.

BACKGROUND/PURPOSE: To identify prenatal and perinatal predictors of surgery and describe surgical findings/outcomes of neonates with Meconium Ileus (MI) secondary to Cystic Fibrosis (CF). METHODS: Potential risk factors (prenatal bowel echogenicity, CF genotype, birthweight, prematurity and sex) for MI and surgery were examined in a retrospective cohort of neonates with CF presenting to a tertiary center between 1997 and 2015. Following univariable analysis, predictors of MI and surgery were determined using multivariable logistic regression. For surgical patients, detailed operative findings and outcomes were examined. RESULTS: MI was diagnosed in 26/120 (21.7%) neonates with CF and 19/26 (73.0%) required surgery. Prematurity was significantly associated with increased risk of MI and operative intervention (p-value 0.022 and p-value 0.016 respectively); lower birthweight was associated with operative intervention (p-value 0.039); genotype and echogenic bowel were associated with neither. Surgical data were available for 17/19 patients; median age at surgery was 2 days (IQR1-3), 4/17 had an atresia and 6/17 received an ostomy. Median NICU and hospital stays were 34.5 and 70 days while median time on TPN and time to ostomy reversal were 28.5 and 97 days, respectively. CONCLUSIONS: In patients with CF, prematurity and lower birthweight were identified as risk factors for meconium ileus and need for surgery. Specific genotypes and echogenic bowel were not predictors of either. LEVEL OF EVIDENCE: Level III.