Expedited Laparoscopic Cholangiogram and Liver Biopsy in the Workup of Biliary Atresia.

PURPOSE: Recent studies demonstrate the success of Kasai portoenterostomy for biliary atresia (BA) is linearly related to infant age at time of Kasai. We sought to review the feasibility and safety of laparoscopic needle micropuncture cholangiogram with concurrent core liver biopsy (if needed) for expedited exclusion of BA in patients with direct conjugated hyperbilirubinemia. METHODS: Expedited laparoscopic cholangiogram and liver biopsy were instituted at our facility for infants with direct hyperbilirubinemia for whom clinical exam and laboratory workup failed to diagnose. A retrospective chart review was performed in infants <1 year with hyperbilirubinemia from 2016 to 2021. Demographics, preoperative evaluation, procedure details, and complications were reviewed. RESULTS: Two hundred ninety-seven infants with unspecified jaundice were identified, of which, 86 (29%) required liver biopsy. Forty-seven percutaneous liver biopsies were obtained including 8 (17%) in whom BA could not be excluded. Laparoscopic cholangiogram was attempted in 47 infants following basic workup; BA was diagnosed in 22 infants (47%) of which 3 were <18 days old. Biliary patency was demonstrated laparoscopically in 22 of 25 (88%); 3 (12%) required conversion to open cholangiogram. Infants with percutaneous liver biopsy had an average delay of 3 days (range: 2-36) to cholangiogram. Preoperative studies and liver biopsy alone did not reliably exclude the diagnosis of BA. CONCLUSION: Laparoscopic cholangiogram with liver biopsy is a safe procedure resulting in the confirmation or exclusion of BA in infants. Forty-seven percent of infants who underwent laparoscopic cholangiogram were found to have BA; those who were surgical candidates underwent Kasai during the same operation.

Multi-Institutional Review From the Pediatric Colorectal and Pelvic Learning Consortium of Minor Spinal Cord Dysraphism in the Setting of Anorectal Malformations: Diagnosis, Treatment, and Outcomes.

BACKGROUND/RATIONALE: Anorectal malformations (ARM) are associated with congenital anomalies of the spine, but the impact of a minor spinal cord dysraphism (mSCD) on fecal continence in the setting of ARM remains unclear. MATERIALS/METHODS: A retrospective review was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. The patient cohort was reviewed for ARM type, mSCD screening/incidence/neurosurgical intervention and age-based BMP utilization. RESULTS: 987 patients with ARM were categorized into mild (38%), moderate (32%) or complex (19%). 694 (70%) had normal spinal (NS) status. 271 (27.5%) patients had mSCD. MRI alone (49%) was the most common screening test for mSCD. US screening had a positive predictive value of 86.3% and a negative predictive value of 67.1%. Surgical intervention rates for mSCD ranged between 13% and 77% at a median age of 0.6-5.2 years. 726 (73.6%) patients were prescribed BMP (74.4% NS, 77.5% mSCD). Laxatives were most utilized BMP in all groups <5yo. ≥5yo, enema utilization increased with ARM complexity independent of spine status (with or without neurosurgical intervention). Neurosurgical intervention did not affect BMP utilization at any age or with any ARM when mSCD was identified. CONCLUSIONS: MSCD influence on bowel function in the setting ARM remains unclear. No significant impact of mSCD was noted on ARM patient bowel management program utilization. Variability exists within PCPLC site with screening and intervention for mSCD in patients with ARM. Future studies with standardized care may be needed to elucidate the true impact of mSCD on long term patient outcomes in ARM patients. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: III.

High Infertility Rates and Pregnancy Complications in Female Physicians Indicate a Need for Culture Change.

OBJECTIVE: This survey study aims to determine the prevalence of pregnancy complications and infertility in female physicians in comparison to the general population. Risk factors, workplace environment, and education are also examined. BACKGROUND: Physicians undertake long training and have stressful work environments during optimal childbearing years. While growing literature indicates increased rates of pregnancy complications and infertility in female surgeons, the prevalence in female physicians of all specialties is unknown. METHODS: An anonymous, voluntary survey was distributed to female physicians via private physician social media groups. It queried pregnancy demographics and complications, infertility diagnosis and treatment, workplace environment, and prior education on these topics. Results were compared with general population data, between medical and surgical subspecialties, and between physicians who were and were not educated on the risks of delaying pregnancy. RESULTS: A total of 4533 female physicians completed the survey. Compared with the general population, female physicians were older at first pregnancy, more often underwent infertility evaluation and treatment, and had higher rates of miscarriage and preterm birth. During training, only 8% of those surveyed received education on the risks of delaying pregnancy. Those who were educated were significantly less likely to experience miscarriage or seek infertility evaluation or treatment. Compared with physicians in nonsurgical specialties, surgeons had fewer children, were older at first pregnancy, had more preterm births and fetal growth problems, and were more likely to be discouraged from starting a family during training and practice. CONCLUSIONS: Female physicians, particularly surgeons, have a significantly greater incidence of miscarriage, infertility, and pregnancy complications compared with the general population. The culture of medicine and surgery must continue to evolve to better support women with family planning during their training and careers.

Early postoperative feeding in single-stage repair of anorectal malformation with vestibular or perineal fistula is not associated with increased wound complications.

INTRODUCTION: The objective of this study is to assess the postoperative outcomes of single-stage repair of anorectal malformations with vestibular (VF) or perineal fistula (PF) and early initiation of postoperative feeding. METHODS: A retrospective review of patients undergoing single-stage repair of isolated low anorectal malformations (VF and PF) from 2017 to 2020 was conducted. All patients underwent an anterior anoplasty with complete mobilization of the rectal fistula, or posterior sagittal anorectoplasty (PSARP), without protective colostomy. The variables examined include age, timing of postoperative feeding initiation, length of stay (LOS), and complications. RESULTS: Nineteen patients with VF or PF underwent a single-stage repair. 12/19 (63%) patients were female. All 7 males and 9/12 females had a PF. The range of age at surgery was 2 days to 3 years with median age of 92 days [IQR 1,3: 9,193]. The median postoperative day for initiation of feeds was day 0 [IQR 1,3: 0,1] and median LOS was 1 day [IQR 1,3: 1,4.5]. 18/19 (95%) patients were evaluated in follow-up and there were no wound infections, wound dehiscences, or recurrent fistulas. Within 90 days postoperatively, no patients were seen in the emergency department for postoperative issues. Within 6 months, 2/19 (11%) patients required an unplanned return to the operating room for anal dilation. CONCLUSION: In single-stage repair of isolated low anorectal malformations, VF and PF, early initiation of postoperative feeding is safe, results in a short length of stay, and does not lead to increased wound complications. Early enteral feeding eliminates the need for parenteral nutrition and central venous access, and their associated complications. LEVEL OF EVIDENCE: Level IV.

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.

Child physical abuse and COVID-19: Trends from nine pediatric trauma centers.

BACKGROUND: Economic, social, and psychologic stressors are associated with an increased risk for abusive injuries in children. Prolonged physical proximity between adults and children under conditions of severe external stress, such as witnessed during the COVID-19 pandemic with "shelter-in-place orders", may be associated with additional increased risk for child physical abuse. We hypothesized that child physical abuse rates and associated severity of injury would increase during the early months of the pandemic as compared to the prior benchmark period. METHODS: We conducted a nine-center retrospective review of suspected child physical abuse admissions across the Western Pediatric Surgery Research Consortium. Cases were identified for the period of April 1-June 30, 2020 (COVID-19) and compared to the identical period in 2019. We collected patient demographics, injury characteristics, and outcome data. RESULTS: There were no significant differences in child physical abuse cases between the time periods in the consortium as a whole or at individual hospitals. There were no differences between the study periods with regard to patient characteristics, injury types or severity, resource utilization, disposition, or mortality. CONCLUSIONS: Apparent rates of new injuries related to child physical abuse did not increase early in the COVID-19 pandemic. While this may suggest that pediatric physical abuse was not impacted by pandemic restrictions and stresses, it is possible that under-reporting, under-detection, or delays in presentation of abusive injuries increased during the pandemic. Long-term follow-up of subsequent rates and severity of child abuse is needed to assess for unrecognized injuries that may have occurred.

Composite Anterolateral Thigh and Fascia Lata Free Flap for Abdominal and Groin Reconstruction in a Pediatric Patient.

Large abdominal wall and groin defects present complex reconstructive challenges. These defects typically require free flap reconstruction to bring in healthy vascularized tissue and recreate the complex full-thickness defect. A 6-year-old previously healthy girl presented to our trauma center after sustaining a close-range shotgun injury resulting in a full-thickness defect to the inferior hemi-abdomen and groin. A composite anterolateral thigh flap with fascia lata free flap was performed to reconstruct the myofascial, skin, and subcutaneous tissue of the abdomen and groin. We present the first composite anterolateral thigh flap with fascia lata for full-thickness abdominal wall and groin reconstruction in a pediatric patient.

IMPPACT (Intravenous Monotherapy for Postoperative Perforated Appendicitis in Children Trial): Randomized Clinical Trial of Monotherapy Versus Multi-drug Antibiotic Therapy.

BACKGROUND: Perforated appendicitis is the most common cause of intraabdominal abscess (IAA) in children. The optimal postoperative antibiotic regimen to reduce IAA has evolved in the last decade from triple-drug to 2-drug therapy (CM). Recent retrospective studies show decreased infectious complications with monotherapy PT. To date prospective comparative data are lacking. Therefore, a prospective randomized trial comparing PT versus CM was conducted. METHODS: A multi-institutional prospective randomized trial was performed in children with perforated appendicitis comparing postoperative antibiotic regimens PT or CM. The primary outcome was 30-day postoperative IAA formation. Perforation was strictly defined as a hole in the appendix or fecalith in the abdomen, documented with intraoperative photographs. RESULTS: One hundred sixty-two patients were enrolled during the study period. No differences in age, weight, or duration of presenting symptoms were identified. In addition, length of stay, duration of intravenous antibiotic treatment, discharge oral antibiotic treatment, and antibiotic-related complications did not differ between groups. Compared to the CM group, the PT group had significantly lower IAA rate [6.1% vs 23.8%, odd ratio (OR) 4.80, P = 0.002], lower postoperative computed tomography imaging rate (13.9% vs 29.3%, OR 2.57, P = 0.030), and fewer emergency room visits (8.8% vs 26.4%, OR 3.73, P = 0.022). Multivariate logistic regression analysis found the use of CM versus PT (OR 9.21, P = 0.021) to be the most significant predictor for developing IAA. CONCLUSIONS: In children with perforated appendicitis, postoperative monotherapy with PT is superior to standard 2-drug therapy with CM and does not increase antibiotic-related complications or antibiotic exposure duration.

Mentorship in pediatric surgery: A need for structure?

INTRODUCTION: Mentorship in surgical training is critical but differs greatly from the early apprenticeship model and often spans generations. This study evaluates the current state of and desire for structured mentorship in pediatric surgical training from the perspective of program directors (PDs) and trainees. METHODS: A survey addressing demographics, presence of or desire for structured mentorship, and proposed mentoring topics was emailed to pediatric surgery PDs (n = 58) and trainees completing fellowship in 2018-2020 (n = 72). RESULTS: The response rate was 38.5%. 50% of trainees were female versus 15% of PDs (p = 0.02). 19% of trainees reported having a structured mentorship program versus 26% of PDs (p = 0.72). The majority, 83%, of trainees felt a structured mentorship program is warranted versus 40% of PDs (p = 0.002). There were differing opinions between trainees and PDs regarding important components of a mentoring program. Trainees felt the following were more important: transition to practice, job negotiation, CV review, financial planning and performance review. PDs felt the following were more important: quality improvement projects and work/life balance. Both agreed academic development and job search were important. CONCLUSIONS: The majority of pediatric surgery trainees desire structured mentorship programs; however, few institutions have them. Training programs and program directors warrant a response to this gap. LEVEL OF EVIDENCE: IV.

Multi-institutional review of bowel management strategies in children with anorectal malformations.

BACKGROUND/RATIONALE: To describe current bowel management program (BMP) strategies in anorectal malformation (ARM) patients based on patient-level predictors using data from a multi-institutional consortium. MATERIALS/METHODS: Patient bowel function and BMP were reviewed from Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) data. The PCPLC is comprised of multidisciplinary specialists researching colorectal and pelvic disorders. Seven US institutions submitted de-identified clinical data on ARM patients into a centralized patient registry. RESULTS: The primary ARM of 624 patients was categorized into Mild (45.2%), Moderate (40.4%) or Complex (14.2%) anomaly classifications. Patient-specific BMP were examined based on age and on the presence of spinal cord/sacral anomalies. 418 (67%) enrolled patients were prescribed BMP (<5 yo 56.4%; ≥5-<12 yo 86.7%; ≥12 81.5%). Constipation was the primary chief complaint (80.2%). Forty percent of patients on a BMP were toilet trained and approximately one-half (48.5%) reported daytime stool accidents. Secondary surgical interventions for antegrade continence enemas (ACE) were examined; 14.5% of patients employed ACE strategies and utilization increased with age and varied based on anatomic anomalies. CONCLUSIONS: This is the first report on BMP strategies for patients with ARM from the Pediatric Colorectal and Pelvic Learning Consortium. Individual patient characteristics are explored for their impact on bowel management strategy utilization. LEVEL OF EVIDENCE: IV.

Evolving indications for surgical intervention in patients with differences/disorders of sex development: Implications of deferred reconstruction.

Rare medical conditions are difficult to study due to the lack of patient volume and limited research resources, and as a result of these challenges, progress in the care of patients with these conditions is slow. Individuals born with differences of sex development (DSD) fall into this category of rare conditions and have additional social barriers due to the intimate nature of the conditions. There is also a lack of general knowledge in the medical community about this group of diverse diagnoses. Despite these limitations, progress has been made in the study of effective ways to care for patients who are born with chromosomal or anatomical differences of their internal reproductive organs or external genitalia. Advocacy groups have placed a spotlight on these topics and asked for a thoughtful approach to educate parents of newborns, medical providers, and the adolescents and young adults themselves as they mature.1 There is growing interest in the approaches to surgical reconstruction of the genitalia and the management of internal gonads, specifically the timing of procedures and the indications for those procedures.2 Advocates suggest deferring surgical procedures until the affected individual can participate in the decision-making process. This approach requires a roadmap for addressing the long-term implications of delayed surgical management. Presented here is a review of the specific issues regarding the complex management of the various categories of DSD.

Thoracoscopic Cardiac Sympathetic Denervation: Adjunct Therapy for Secondary Prevention of Life-Threatening Ventricular Arrhythmias in Children.

BACKGROUND: Cardiac sympathetic denervation (CSD) is a surgical option for patients with life-threatening ventricular arrhythmias. Previously described cohorts included populations in which CSD was performed for primary and secondary prevention. We report the efficacy of CSD as adjunct therapy in children with medically refractory life-threatening arrhythmias. MATERIALS AND METHODS: Retrospective review of patients undergoing thoracoscopic CSD at one institution between January 2008 and July 2017. Patient demographics, indications, procedural details, complications, length of stay, and effectiveness were evaluated. RESULTS: Ten thoracoscopic CSD procedures were performed in 8 patients. Mean age was 8.2 years (8 days-19 years); mean weight was 32.6 kg (2.7-57 kg); and 50% were female. Four had long QT syndrome, 3 catecholaminergic polymorphic ventricular tachycardia, and 1 short QT syndrome. All patients had at least two (2 to >40) episodes of resuscitated ventricular arrhythmia and were maximized on medical therapy. Six patients had implantable cardioverter-defibrillators (ICD) with a mean of 11.9 appropriate discharges (1-40) before CSD. All patients underwent left CSD; 2 subsequently required right CSD. Four of the 6 ICD patients experienced dramatic improvement (total 48 ICD discharges pre-CSD; 3 post-CSD). Two patients noncompliant with medical therapy had no significant improvement (24 ICD discharges pre-CSD; 23 post-CSD) and also underwent right CSD, again with no improvement (23 discharges pre-right CSD; 28 post-right CSD). CONCLUSIONS: Thoracoscopic CSD can be safely performed in the neonate and pediatric populations. When utilized with medication therapy, CSD is an effective adjunct in reducing ICD discharges and arrhythmias.

Hiatal and paraesophageal hernia repair in pediatric patients.

Hiatal and paraesophageal hernia (HH/PEH) can be congenital, resulting from embryologic abnormalities/genetic predisposition, or acquired, most commonly after gastroesophageal surgery such as fundoplication. Minimizing circumferential esophageal dissection at the time of Nissen fundoplication has been shown to decrease the risk of acquired HH/PEH from 36.5% to 12.2%. Gastrointestinal, respiratory, and constitutional symptoms, including anemia and failure to thrive, are common with high rates of associated gastroesophageal reflux. Chest x-ray is often abnormal and upper GI confirms the diagnosis. Treatment is surgical with the goal of reducing the hernia contents, excising the hernia sac, closing the crura, and performing an antireflux procedure. The laparoscopic approach is safe and effective.

Injuries and outcomes associated with recreational vehicle accidents in pediatric trauma.

PURPOSE: To identify injuries and outcomes from Recreational/Off-Highway Vehicles (RV/OHV) accidents at a pediatric trauma center. METHODS: A retrospective review of a prospective pediatric trauma registry was performed to identify patients sustaining injuries from an RV/OHV between January 2007 and July 2015. Vehicles included: all-terrain vehicles (ATV), dirt bikes, utility-terrain vehicles (UTV), golf carts, go-karts, and dune buggies. RESULTS: Five hundred twenty-eight patients were injured while on an RV/OHV: 269 ATV, 135 dirt bike, 42 UTV, 38 golf cart, 34 go-kart, and 10 dune buggy. The majority (n=381, 72%) had at least one injury with an Abbreviated Injury Scale ≥2; 39% (n=204) had orthopedic injuries and 22% (n=116) had central neurologic injuries. Over three-fourths (n=412, 78%) were admitted. For the 48% (n=253) of patients requiring surgery, 654 surgical procedures were performed. Median hospital charge was $27,565 (IQR: $15,553-$44,935). Excluding golf carts, helmet use was 49% (n=231); 16% (n=76) wore protective clothing. Only 22% (n=26) wore a restraining belt. CONCLUSION: Severe injuries occur in children who ride RV/OHV often warranting admission and surgical intervention. Improved understanding of RV/OHV injuries may guide caregivers in decision-making about pediatric RV/OHV use and encourage use of protective gear. LEVEL OF EVIDENCE: Level II, Prognosis Study.

Influence of sub-specialty surgical care on outcomes for pediatric emergency general surgery patients in a low-middle income country.

BACKGROUND: Whether adult general surgeons should handle pediatric emergencies is controversial. In many resource-limited settings, pediatric surgeons are not available. The study examined differences in surgical outcomes among children/adolescents managed by pediatric and adult general surgery teams for emergency general surgical (EGS) conditions at a university-hospital in South Asia. METHODS: Pediatric patients (<18y) admitted with an EGS diagnosis (March 2009-April 2014) were included. Patients were dichotomized by adult vs. pediatric surgical management team. Outcome measures included: length of stay (LOS), mortality, and occurrence of ≥1 complication(s). Descriptive statistics and multivariable regression analyses with propensity scores to account for potential confounding were used to compare outcomes between the two groups. Quasi-experimental counterfactual models further examined hypothetical outcomes, assuming that all patients had been treated by pediatric surgeons. RESULTS: A total of 2323 patients were included. Average age was 7.1y (±5.5 SD); most patients were male (77.7%). 1958 (84.3%) were managed by pediatric surgery. The overall probability of developing a complication was 1.8%; 0.9% died (all adult general surgery). Patients managed by adult general surgery had higher risk-adjusted odds of developing complications (OR [95%CI]: 5.42 [2.10-14.00]) and longer average LOS (7.98 vs. 5.61 days, p < 0.01). 39.8% fewer complications and an 8.2% decrease in LOS would have been expected if all patients had been managed by pediatric surgery. CONCLUSION: Pediatric patients had better post-operative outcomes under pediatric surgical supervision, suggesting that, where possible in resource-constrained settings, resources should be allocated to promote development and staffing of pediatric surgical specialties parallel to adult general surgical teams.

Rates of residual disease with close but negative margins in breast cancer surgery.

OBJECTIVES: A recent multidisciplinary consensus defined an adequate breast cancer margin as no ink on tumor. The purpose of this study was to analyze rates of residual disease at re-excision by margin width. MATERIALS AND METHODS: A prospective database at a single institution was reviewed from 2000 to 2012. Institutional protocol had been to perform re-excision surgery when margins were <2 millimeters (mm). RESULTS: There were 2520 procedures. Re-excision surgery was performed for 12% of breast conserving therapy (BCT) procedures and 2% of mastectomies; residual disease was present in 38% and 26%, respectively. The rates of residual disease for all patients with positive, 0.1-0.9 mm, and 1.0-1.9 mm margins were 40%, 38%, and 33%, respectively. Age, race, menopause status, width of closest final margin, tumor histology, hormone receptor status, triple-negative disease and presence of lymphovascular invasion (LVI) were not significantly associated with the presence of residual disease. The presence of multiple margins <2 mm trended toward significance (p = 0.06). Median follow-up was 43 months. The five-year local recurrence rates (5-year LR) were 1.1% for mastectomy patients and 1.9% for BCT patients. CONCLUSIONS: Breast cancer patients with margins of excision <2 mm have a substantial risk of residual disease but the rates far exceed LR rates. These findings suggest that using residual disease rates to determine the appropriate margin width is not reliable, but also serve as a note of caution to track LR rates as institutions conform to new national guidelines for margin management.

Role of ERCP in pediatric blunt abdominal trauma: a case series at a level one pediatric trauma center.

BACKGROUND: There is no consensus regarding the appropriate use of endoscopic retrograde cholangiopancreatography (ERCP) in pediatric trauma. We report our experience with ERCP for management of pediatric pancreatic and biliary injury following blunt abdominal trauma. METHODS: A retrospective chart review was performed for pediatric patients with blunt abdominal trauma from July 2008 through December 2012 at our pediatric trauma center. For patients who underwent ERCP, demographics, injury characteristics, diagnostic details, procedures performed, length of stay, total parenteral nutrition use, and complications were reviewed. RESULTS: There were 532 patients identified: 115 hepatic injuries, 25 pancreatic injuries and one gall bladder injury. Nine patients (mean age 7.8 years) underwent ERCP. Seven (78%) had pancreatic injuries, while two (22%) had bilateral hepatic duct injuries. The median time to diagnosis was one day (range, 0-12). Diagnostic ERCP only was performed in three patients, two of which proceeded to distal pancreatectomy. Five patients had stents placed (two biliary and three pancreatic) and four sphincterotomies were performed. Despite pancreatic stenting, one patient required distal pancreatectomy for persistent leak. Median length of stay was 11 days. CONCLUSIONS: Pediatric pancreatic and biliary ductal injuries following blunt abdominal trauma are uncommon. ERCP can safely provide definitive treatment for some patients.