Comparison of the PCPLC Database to NSQIP-P: A Patient Matched Comparison of Surgical Complications Following Repair of Anorectal Malformation.

BACKGROUND: Anorectal malformations (ARM) are rare and heterogenous which creates a challenge in conducting research and offering recommendations for best practice. The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) was formed in 2016 to address this challenge and created a shared national data registry to collect information about pediatric colorectal patients. There has been no external validation of the data collected. We sought to evaluate the database by performing a patient matched analysis comparing 30-day outcomes identified in the PCPLC registry with the NSQIP-P database for patients undergoing surgical repair of ARM. METHODS: Patients captured in the PCPLC database from 2016 to 2021 at institutions also participating in NSQIP-P who underwent ARM repair younger than 12 months old were reviewed for 30-day complications. These patients were matched to their NSQIP-P record using their hospital identification number, and records were compared for concordance in identified complications. RESULTS: A total of 591 patient records met inclusion criteria in the PCPLC database. Of these, 180 patients were also reviewed by NSQIP-P. One hundred and fifty-six patient records had no complications recorded. Twenty-four patient records had a complication listed in one or both databases. There was a 91 % concordance rate between databases. When excluding complications not tracked in the PCPLC registry, this agreement improved to 93 %. CONCLUSION: Including all patients evaluated for this subpopulation, a 91 % concordance rate was observed when comparing PCPLC collected complications to NSQIP-P. Future efforts can focus on further validating the data within the PCPLC for other patient populations. LEVEL OF EVIDENCE: V.

Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis.

OBJECTIVES: Inflammation on diagnostic rectal biopsy for children with suspected Hirschsprung disease (HSCR) is reported on pathology, and its significance is unknown. We describe the management and outcomes of a cohort with inflammation on rectal biopsy compared to those without. Specifically, to address the hypothesis that inflammation on diagnostic biopsy is associated with increased complication rates irrespective of intervention type and timing. METHODS: A single institution retrospective review of children with HSCR who underwent biopsy and endorectal pull-through (ERPT) from 2010 to 2020 was performed. The primary outcome was overall complications at 30-days following ERPT. Secondary outcomes included timing and type of operative intervention as well as postoperative enterocolitis diagnosed within 6-months of ERPT. RESULTS: Forty-nine children were identified; inflammation was present on diagnostic biopsy for 17 children. Those with inflammation were more likely to have clinical evidence of enterocolitis at the time of biopsy (p = 0.001) and were more likely to undergo leveling colostomy before ERPT (p = 0.01). Children with inflammation had a higher anastomotic leak rate (p = 0.04). Subgroup analysis of patients with inflammation undergoing primary ERPT versus leveling colostomy demonstrated no significant difference in outcomes following definitive ERPT. CONCLUSIONS: Our study suggests inflammation on diagnostic rectal biopsy for HSCR is associated with increased anastomotic leak rates. While additional prospective studies are indicated, attention to methods of mitigating inflammation and confirming its resolution before definitive pull-through may be of benefit for improving clinical outcomes in patients found with inflammation on diagnostic rectal biopsy.

Functional Outcomes of Patients Who Underwent Anorectal Malformation Repair Using MRI Guidance.

BACKGROUND: Despite the initiation of minimally invasive laparoscopic techniques, the majority of patients who undergo anorectal malformation repair still experience functional bowel issues in childhood, including constipation and fecal incontinence. In this study, we evaluate the functional outcomes of a procedure in which magnetic resonance imaging guidance is used during initial laparoscopic repair to better locate the epicenter of the sphincter muscle complex and pelvic floor with the goal of more accurate placement of the neoanus and improved functional outcomes. METHODS: A retrospective chart review evaluated demographic, operative, and outcome details for patients who underwent this procedure. A telephone survey was employed to determine levels of social continence using the validated Baylor Continence Scale and to determine what type of bowel management is used. RESULTS: Twenty-six patients were included. Median age at operation was 7 months, and median age at follow-up was 4 years old, with a range of 1-9. Bowel management regimen results revealed that 19 % (n = 5) use no bowel management regimen, 58 % (n = 15) use laxatives only, and 23 % (n = 6) use enemas. Enema use was not associated with different spine or sacral anomalies (p = 0.77). Fifteen patients (58 %) answered the Baylor Continence Scale questions and had a median score of 14. No difference was found in scores when accounting for lesion level (p = 0.43), quality of needle placement (p = 0.46), or quality of sphincter muscles (p = 0.75). CONCLUSIONS: Using MRI guidance in the repair of anorectal malformations shows promise in both the qualitative and quantitative functional outcomes of this complex patient population. LEVEL OF EVIDENCE: Level III.

Outcomes From Colonic Pull-Through for Cloacal Exstrophy Differ by Colon Length: A Multi-Institutional Study.

BACKGROUND: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population. METHODS: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher's exact tests. RESULTS: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005). CONCLUSION: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE. LEVEL OF EVIDENCE: III.

Thoracoscopic Patch Repair of Congenital Diaphragmatic Hernia: Can Smaller Incisions Treat Larger Defects?

INTRODUCTION: Thoracoscopic CDH repair is increasingly performed for Type A and small Type B defects that are amenable to primary repair. However, the thoracoscopic approach is controversial for larger defects necessitating a patch due to technical complexity, intraoperative acidosis, and recurrence risk. We aim to compare clinical outcomes between thoracoscopic and open patch repair of Type B/C defects, using a standardized technique. METHODS: This is a single-center retrospective review of thoracoscopic and open CDH patch repairs January 2017-December 2021. We excluded primary repairs, Type D hernias, repairs on ECMO, recurrent repairs. Various preoperative, intraoperative, and postoperative variables were compared. Primary outcome was recurrence rate. Secondary outcomes included intraoperative pH and pCO2, operative time, and complication rates. RESULTS: Twenty-nine patients met inclusion criteria (open = 13, thoracoscopic = 16). The open cohort had lower o/e total fetal lung volume (29 vs 41.2%, p = 0.042), higher preoperative peak inspiratory pressures (24 vs 20 cm H2O, p = 0.007), were more frequently Type C defects (92.3 vs 31%, p = 0.002) and had liver "up" in left-sided hernias (46 vs 0%, p < 0.0001). Intraoperatively, median lowest pH and highest pCO2 did not differ; neither did overall median pH or pCO2. Operative times were similar (153 vs 194 min, p = 0.113). No difference in recurrence rates was identified, however postoperative complications were higher in the open group. There were no mortalities. CONCLUSIONS: Although we demonstrate higher disease severity of patients undergoing open repair, thoracoscopic patch repair for Type B/C defects is safe and effective in patients with favorable physiologic status, alleviating concerns for intraoperative acidosis, operative length, and risk of recurrence. LEVEL OF EVIDENCE: II.

Rectal Prolapse Following Repair of Anorectal Malformation: Incidence, Risk Factors, and Management.

BACKGROUND: The incidence and optimal management of rectal prolapse following repair of an anorectal malformation (ARM) has not been well-defined. METHODS: A retrospective cohort study was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium registry. All children with a history of ARM repair were included. Our primary outcome was rectal prolapse. Secondary outcomes included operative management of prolapse and anoplasty stricture following operative management of prolapse. Univariate analyses were performed to identify patient factors associated with our primary and secondary outcomes. A multivariable logistic regression was developed to assess the association between laparoscopic ARM repair and rectal prolapse. RESULTS: A total of 1140 patients met inclusion criteria; 163 (14.3%) developed rectal prolapse. On univariate analysis, prolapse was significantly associated with male sex, sacral abnormalities, ARM type, ARM complexity, and laparoscopic ARM repairs (p < 0.001). ARM types with the highest rates of prolapse included rectourethral-prostatic fistula (29.2%), rectovesical/bladder neck fistula (28.8%), and cloaca (25.0%). Of those who developed prolapse, 110 (67.5%) underwent operative management. Anoplasty strictures developed in 27 (24.5%) patients after prolapse repair. After controlling for ARM type and hospital, laparoscopic ARM repair was not significantly associated with prolapse (adjusted odds ratio (95% CI): 1.50 (0.84, 2.66), p = 0.17). CONCLUSION: Rectal prolapse develops in a significant subset of patients following ARM repair. Risk factors for prolapse include male sex, complex ARM type, and sacral abnormalities. Further research investigating the indications for operative management of prolapse and operative techniques for prolapse repair are needed to define optimal treatment. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: II.

Ultrasound-Guided Enteropexy for Repair of Ostomy Prolapse.

Background: Enterostomies provide fecal diversion for numerous conditions, but anatomical complications-prolapse, stricture, and retraction-occur in up to 25%. Given up to 76% of these complications require surgical intervention, effective minimally invasive repair techniques for their management are needed. This article describes a new technique for prolapse repair utilizing image-guided surgery for incisionless repair of ostomy prolapse. Methods: To perform the procedure, the prolapsed bowel is reduced and evaluated for feasibility for ultrasound repair. Under direct ultrasound guidance sutures are used to pexy the bowel loop to the overlying fascia. Sutures are tied with knots and sutures buried below the skin to securely tack the bowel to the abdominal wall. Results: Four patients aged 2-10 years underwent ultrasound-guided enteropexy for repair of major prolapse of an end ileostomy (2 patients), loop colostomy, and end colostomy. All patients remained free of major prolapse for 3-10 months after the procedure, 2 of whom have progressed to ostomy takedown without complications. Conclusions: Ultrasound-guided enteropexy is a noninvasive effective way to manage ostomy prolapse.

A Pilot Study: Transcriptional Profiling, Functional Analysis, and Organoid Modeling of Intestinal Mucosa in Hirschsprung Disease.

BACKGROUND: Hirschsprung disease (HSCR) is a congenital colonic aganglionosis. Many HSCR patients develop enterocolitis despite surgical resection. The pathophysiology of this inflammatory process is poorly understood. We compared transcriptional profiles and function of ganglionic and aganglionic tissue in HSCR patients. METHODS: RNA sequencing was performed on mucosal tissues from HSCR patients (n = 6) and controls (n = 3). Function of matched ganglionic and aganglionic regions were investigated utilizing organoids generated from these tissues. RESULTS: Transcriptional differences observed in ganglionic and aganglionic regions of HSCR patients included upregulation of genes involving inflammation, cell differentiation and proliferation as well as decreased expression of genes encoding mucins compared to controls. Organoids derived from ganglionic and aganglionic regions of HSCR patients were similar in epithelial cell differentiation, epithelial barrier formation and response to stimulation with bacterial metabolites and pro-inflammatory cytokines. CONCLUSIONS: Despite normal ganglionic structure, the section of colon adjacent to the aganglionic region in HSCR patients has perturbed gene expression which resembles the aganglionic segment. Transcriptional and functional changes in colonic epithelium are persevered in the ganglionic colon used for pull-through surgery. This may explain persistence of enterocolitis despite surgical excision of aganglionic colon and subsequent endorectal pull-through performed with ganglionic colon during correction of HSCR. LEVEL OF EVIDENCE: N/A.

Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group.

BACKGROUND/PURPOSE: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients. METHODS: Retrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale. RESULTS: There were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD. CONCLUSION: More than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment. LEVEL OF EVIDENCE: Level 4.

Do preoperative factors predict success of antegrade continence enemas in children?

INTRODUCTION: Antegrade continent enemas (ACE) procedures are one treatment option in children with medically refractory constipation or encopresis and predicting success is difficult. We hypothesize that there are preoperative factors that can be identified to help with patient selection and family counseling. METHODS: We conducted a retrospective study of children who underwent a cecostomy or appendicostomy for an ACE program between 2015 and 2021. Underlying diagnosis, pre-operative bowel regimen and imaging were analyzed. Patients were reviewed for success at 3-, 6- and 12-months post-procedure. Data was analyzed with Fisher's Exact, Kruskal-Wallis and logistic regression where applicable with significance defined as p < 0.05. RESULTS: Forty-three children were identified; 28 were male, 15 were female, mean age at time of operation was 8 years old. 76% were considered successful at 3-months, 86% at 6-months, and 87% at 12-months post- procedure. Univariate analysis showed that a pre-ACE retrograde enema program predicted success at 3-months (94% vs. 64% p = 0.03) but no difference at 6- or 12-months. At one year after ACE procedure there was a significant reduction in number of enteral medications (2 to 0, p < 0.01) and 94% of patients were on one or fewer at one year follow-up. Age, gender, weight at time of operation, contrast enema, anorectal manometry and colonic transit time results were not predictive of outcomes. CONCLUSION: In this study, we characterized expected time to success in our population as well as identified use of a pre-operative retrograde enema program as a potential predictor of success at 3-months in children undergoing an ACE procedure. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Prognosis study.

An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy.

People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence.

Ultrasound-guided percutaneous central venous access in a neonatal porcine model.

Percutaneous catheterization is currently utilized for central venous access in adult and adolescent porcine models; however, neonatal models require a cut down to gain venous access. This approach requires general anesthesia and can result in systemic inflammation, which can confound studies investigating other inflammatory triggers. Here we present the first successful series of percutaneous, ultrasound-guided, durable central venous access in newborn piglets weighing 1 kg with a novel method of tunneling the catheter subcutaneously using a needle. Catheters (3-5 Fr, single- or double-lumen) were successfully placed in the right jugular vein of eight piglets weighing 1.3 ± 0.4 kg (mean ± standard deviation) with an average duration of catheter patency of 4.5 ± 2.1 days. There were no adverse events from catheter placement, though one catheter was inadvertently removed. This technique is a safe, minimally invasive method for obtaining secure central venous access in a neonatal porcine model.

Characterizing the use of botulinum toxin in patients with Hirschsprung disease treated at referral institutions for pediatric colorectal surgery.

PURPOSE: Botulinum toxin (BT) is used to treat pediatric patients with Hirschsprung disease (HD) with obstructive symptoms. We aimed to characterize use of BT in HD patients across pediatric colorectal surgery referral centers. METHODS: A multicenter retrospective study of BT use in children (0-18y) with HD was performed using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) between 2017 and 2021. Sites with <10 HD patients recorded were excluded. Patterns were evaluated using Fisher's exact, Wilcoxon rank-sum, Kruskal-Wallis, and Cochran-Armitage trend test. RESULTS: 494 patients at 8 centers were included. 118 (23.9%) received at least one BT injection. Among patients who required redo pullthrough procedures, 53.1% received BT compared to 22.7% of patients who only underwent one pullthrough (p<0.001). Age at pullthrough was also significantly associated (p = 0.021). A lower proportion of Hispanic patients received BT (9.6% vs. 26.3%;p = 0.006). Percentage of HD patients receiving BT varied significantly across sites (p<0.001). CONCLUSIONS: Use of BT in patients with HD varies widely with greater use in patients who underwent redo surgery and in those who underwent pullthrough at an older age. Hispanic patients received less BT. These findings highlight the need to develop consensus guidelines and for further study on timing of injections and potential disparities in care. LEVEL OF EVIDENCE: III.

An Alternative Approach to Single-Stage Cloaca Repair: Image-Guided Anorectoplasty with Delayed Vaginoplasty.

BACKGROUND: Cloacal anomalies occur when a fetus's rectum, vagina, and urethra fail to separate and result in 1 common channel at birth. They are commonly managed by complex reconstruction in the first year of life. This manuscript presents an alternative approach to management in patients with absent or nondilated Mullerian structures. CASE: Image-guided, combined endoscopic and laparoscopic surgery (CELS) was used to perform an anorectal pull-through at 5 months of age on a patient with persistent cloaca and no definite vaginal or uterine structures seen on MRI and endoscopy. Urogenital reconstruction is delayed until adolescence. SUMMARY AND CONCLUSION: We hypothesize that performing a minimally invasive anorectoplasty on patients with complicated anatomy and low risk for hydrocolpos could potentially result in improved urologic function and better psychosocial outcomes. Delaying vaginoplasty will enable determination of the function of remnant Mullerian structures and allow the patient to direct the augmentation approach.

Venogram before long-interval repeat cannulation for pediatric extracorporeal membrane oxygenation.

It is rare for children to receive more than one course of support with extracorporeal membrane oxygenation, and in those who do undergo multiple episodes, the interval is usually days to weeks between events. Little data exists on re-cannulation years after an initial extracorporeal membrane oxygenation run, and late repeat cannulation can pose unique challenges. We report the case of a 10-year-old male patient with right jugular vein occlusion due to a previous course of extracorporeal membrane oxygenation as a neonate, who was successfully supported via central cannulation. This case demonstrates the importance of adequate imaging of target vasculature prior to attempting re-cannulation of a previously used vessel. Establishing a thoughtful strategy for late repeat cannulation is essential to achieve safe access in unusual and challenging situations.

Salvage Laparoscopic-Assisted Anorectoplasty after Failed Vestibular Fistula Repair Using Magnetic Resonance Image Guidance.

Patients with vestibular fistula have a good functional outcome after posterior sagittal anorectoplasty (PSARP). While continence is often preserved, close follow-up and management of constipation are often required. Redo anorectal surgery has been associated with worse functional outcomes compared with primary procedures, possibly due to injury and scarring of the pelvic floor musculature and sphincter complex. Our group has a growing experience in the use of intraoperative real-time magnetic resonance imaging (MRI) for anorectal malformation repairs. We present a case of salvage operation of a failed PSARP for vestibular fistula.

Robotic resection of recurrent pediatric lipoblastoma.

This case demonstrates successful resection of a rare, recurrent presacral-pelvic lipoblastoma in a 19-year-old female patient. Because of the anatomical location of the mass and its proximity to vital structures, the robotic approach allowed for both optimal visualization and effective debulking of the mass. Furthermore, with the use of an articulating laparoscopic camera, key visualization of the posterior lateral pelvis was possible. Using a wide breadth of technologies and resources is essential to broadening the surgical armamentarium and achieving resectability in otherwise challenging cases.

Obtaining central access in challenging pediatric patients.

PURPOSE: Central catheter placement is one of the most commonly performed procedures by pediatric surgeons. Here, we present a case series of patients where central access was obtained at our institution with the utilization of a novel ultrasound-guided technique. This series represents the first of its kind where the native, parent vessels were inaccessible, resulting in a challenging situation for providers. METHODS: A retrospective chart review was performed in pediatric patients (0-17 years) at a tertiary care institution between July 2012 and November 2017 on all central line procedures where ultrasound was utilized to cannulate the brachiocephalic or superior vena cava in face of proximal occlusion. Our group has previous experience utilizing an image-guided in-plane approach to central line placement in the pediatric population. Demographics, operative characteristics, and postoperative complications were reviewed. RESULTS: A total of 11 procedures were included in this case series where the BC (N = 9) or SVC (N = 2) were cannulated for access. Internal jugular vein cannulation was attempted on each patient unless preoperative imaging demonstrated occlusion. The median operative time was 43 ± 23 min. Most procedures were performed on the right sided (63%), with catheters ranging from 4.2F single lumen to 14F double lumen. Since being placed, three (27%) catheters have been removed, with one due to non-use, one due to sepsis, and the final one due to malposition. CONCLUSION: With the continued need for long-term central access in the pediatric population, distal vein occlusion or inaccessibility can prove challenging when attempting to obtain central access. Here, demonstrated a safe alternative technique that provides an additional option in the pediatric surgeon's armamentarium for patients with difficult central access.

Development of the necrotizing enterocolitis society registry and biorepository.

Necrotizing enterocolitis (NEC) is a devastating disease affecting premature infants. New advances in diagnostic and treatment options are desperately needed. Accordingly, the NEC Society initiated a research collaborative with a group of investigators dedicated to advancing the state of NEC-associated knowledge. Recent advances in high-content molecular interrogation and bio-computation (e.g., genomics, transcriptomics, proteomics, and metabolomics) can provide new insights from afflicted infants with NEC, however, individual centers do not have sufficient cases to conduct these studies independently. The development of a NEC Society Biorepository (NSB) has emerged to advance collaboration among institutions through the shared use of biologic samples in the dedicated pursuit of molecular indicators of disease and to gain greater pathophysiologic insights through research. The NSB will provide key infrastructure across several centers to harness the potential for new discoveries, while ensuring specimens are processed consistently, appropriate clinical data is collected, and privacy is maintained. The NSB will provide a comprehensive framework for sharing biological samples and clinical data through a robust and secure system that supports the investigation of research studies on NEC.