Moir, Christopher R.

We report a rare case of biliary aplasia in an infant who presented with persistent jaundice and elevated direct bilirubin despite having cholic stools. Imaging confirmed the absence of extrahepatic bile ducts, consistent with biliary atresia. Intraoperatively, there was a complete absence of an extrahepatic biliary tree consistent with biliary hypoplasia. We identified a cryptogenic hepatic plate directly fused to the right portal vein on further dissection. Despite the atypical anatomy, a modified Kasai portoenterostomy was successfully performed. The patient showed progressive clinical improvement with complete resolution of jaundice and normalisation of liver function tests over the following months. This case underscores a rare anatomical variant that can still permit a favourable outcome with a timely Kasai procedure. To our knowledge, this is one of the few documented cases of hepatic plate–portal vein fusion in biliary atresia.

Background: Videoscopic left cardiac sympathetic denervation (LCSD) is an effective antifibrillatory, minimally invasive therapy for patients with potentially life-threatening arrhythmia syndromes like long QT syndrome (LQTS). Although initially used primarily for treatment intensification following documented LQTS-associated breakthrough cardiac events while on beta-blockers, LCSD as 1-time monotherapy for certain patients with LQTS requires further evaluation. We are presenting our early experience with LCSD monotherapy for carefully selected patients with LQTS. Methods: Among the 1400 patients evaluated and treated for LQTS, a retrospective review was performed on the 204 patients with LQTS who underwent LCSD at our institution since 2005 to identify the patients where the LCSD served as stand-alone, monotherapy. Clinical data on symptomatic status before diagnosis, clinical, and genetic diagnosis, and breakthrough cardiac events after diagnosis were analyzed to determine efficacy of LCSD monotherapy. Result: Overall, 64 of 204 patients (31%) were treated with LCSD alone (37 [58%] female, mean QTc 466±30 ms, 16 [25%] patients were symptomatic before diagnosis with a mean age at diagnosis 17.3±11.8 years, 5 had [8%] ≥1 breakthrough cardiac event after diagnosis, and mean age at LCSD was 21.1±11.4 years). The primary motivation for LCSD monotherapy was an unacceptable quality of life stemming from beta-blocker related side effects (ie, beta-blocker intolerance) in 56/64 patients (88%). The underlying LQTS genotype was LQT1 in 36 (56%) and LQT2 in 20 (31%). There were no significant LCSD-related surgical complications. With a mean follow-up of 2.7±2.4 years so far, only 3 patients have experienced a nonlethal, post-LCSD breakthrough cardiac event in 180 patient-years. Conclusions: LCSD may be a safe and effective stand-alone therapy for select patients who do not tolerate beta-blockers. However, LCSD is not curative and patient selection will be critical when potentially considering LCSD as monotherapy.

Abstract Background The pediatric resuscitation and trauma outcome (PRESTO) model was developed to aid comparisons of risk‐adjusted mortality after injury in low‐ and middle‐income countries (LMICs). We sought to validate PRESTO using data from a middle‐income country (MIC) trauma registry and compare its performance to the Pediatric Trauma Score (PTS), Revised Trauma Score, and pediatric age‐adjusted shock index (SIPA). Methods We included children (age < 15 years) admitted to a single trauma center in South Africa from December 2012 to January 2019. We excluded patients missing variables necessary for the PRESTO model—age, systolic blood pressure, pulse, oxygen saturation, neurologic status, and airway support. Trauma scores were assigned retrospectively. PRESTO's previously high‐income country (HIC)‐validated optimal threshold was compared to MIC‐validated threshold using area under the receiver operating characteristic curves (AUROC). Prediction of in‐hospital death using trauma scoring systems was compared using ROC analysis. Results Of 1160 injured children, 988 (85%) had complete data for calculation of PRESTO. Median age was 7 (IQR: 4, 11), and 67% were male. Mortality was 2% ( n = 23). Mean predicted mortality was 0.5% (range 0–25.7%, AUROC 0.93). Using the HIC‐validated threshold, PRESTO had a sensitivity of 26.1% and a specificity of 99.7%. The MIC threshold showed a sensitivity of 82.6% and specificity of 89.4%. The MIC threshold yielded superior discrimination (AUROC 0.86 [CI 0.78, 0.94]) compared to the previously established HIC threshold (0.63 [CI 0.54, 0.72], p < 0.0001). PRESTO showed superior prediction of in‐hospital death compared to PTS and SIPA (all p < 0.01). Conclusion PRESTO can be applied in MIC settings and discriminates between children at risk for in‐hospital death following trauma. Further research should clarify optimal decision thresholds for quality improvement and benchmarking in LMIC settings.

ABSTRACTObjectives:Pancreatitis is a known complication of endoscopic retrograde cholangiopancreatography (ERCP). Our aim was to assess the prevalence and severity of ERCP‐associated pancreatitis using established criteria.Materials and Methods:Retrospective review of patients younger than 18 years undergoing ERCP complicated by post‐ERCP pancreatitis defined by the 1991 consensus statement. Patients with chronic pancreatitis were studied separately using modified criteria. Risk factors for post‐ERCP pancreatitis were analyzed.Results:Three hundred forty‐three ERCPs were performed in 224 patients. Two hundred seventy‐six ERCPs were performed in patients without chronic pancreatitis, 7 of which were complicated by post‐ERCP pancreatitis (prevalence 2.5%). Patients undergoing diagnostic‐only ERCP were less likely to develop post‐ERCP pancreatitis (P < 0.01). Sixty‐seven procedures were performed on patients with chronic pancreatitis; 10 developed postprocedure pain requiring or prolonging hospitalization (prevalence 14.9%). Pancreatic duct stenting was a risk factor for post‐ERCP pain in this subset of patients (P = 0.02).Conclusions:The prevalence of post‐ERCP pancreatitis is low—2.5% excluding patients with chronic pancreatitis and 4.96% overall. Therapeutic procedures and the presence of chronic pancreatitis are risk factors for post‐ERCP pancreatitis.

Abstract Background: Operative maneuvers to increase mesenteric length during ileal pouch-anal anastomosis (IPAA) are frequently utilized in adults, but limited data exist on the need for their use in children. Materials and Methods: A retrospective chart review of children (age <18) considered for IPAA creation at two affiliated tertiary referral centers from 2007 to 2017 was conducted, and patient factors, operative details, and 30-day postoperative complications were abstracted. Body mass index (BMI) was normalized to BMI percentile-for-age-and-sex and classified as underweight (BMI <5th percentile), healthy weight (5th ≤ BMI percentile <85th), or overweight/obese (BMI ≥85th percentile). Maneuvers were identified from operative notes. Univariate analysis and multivariable logistic regression were performed to determine independent factors associated with the use of maneuvers. Results: A total of 94 patients underwent attempt at IPAA creation, which was successful in 91 (97%). Fourteen (15%) of 91 patients were classified as overweight or obese. The 3 failures occurred secondary to inability to reach in 3 patients, with specific mention of patients' obesity in 2 and pouch ischemia in 1. Sixty (66%) patients required maneuvers to lengthen the mesentery. Overweight/obese patients required maneuvers more often than nonoverweight/obese patients (93% versus 61%, P = .03). There were no differences in 30-day maximum Clavien-Dindo scores between patients with and without maneuvers performed ( P = .83). Being overweight/obese was an independent risk factor for requiring maneuvers (odds ratio: 9.3, 95% confidence interval: 1.1–82.8) after adjusting for age, sex, height, operative stage, and surgeon. Conclusion: Surgeons should be prepared to perform mesenteric lengthening maneuvers when operating on pediatric patients to ensure minimal tension on the IPAA, and more so when operating on obese children. Whether these maneuvers have an impact on long-term pouch function is undetermined.

Abstract Purpose: Restoration of intestinal continuity by ileal pouch-anal anastomosis (IPAA) following subtotal colectomy may not require a temporary, protective ileostomy. Diversion contributes to patient discomfort, cost, and additional operative risk at the time of subsequent reversal. We compared the outcomes of pediatric patients undergoing modified two-stage to three-stage IPAA after recovering from subtotal colectomy. Methods: We reviewed children (age <18) who underwent IPAA creation for ulcerative or indeterminate colitis from January 1, 2007 to December 31, 2017. Patient characteristics, operative details, 30-day complications, and postoperative length of stay (LOS) were abstracted. Total LOS for the three-stage group included both the IPAA and the ileostomy reversal operations. Univariate comparisons between patients undergoing modified two-stage and three-stage operations were performed. Results: A total of 43 patients underwent IPAA after subtotal colectomy; 32 (74%) underwent a three-stage approach, and 11 (26%) had a modified two-stage approach. Operative approach was laparoscopic in 33 (77%), planned open in 9 (21%), and converted to open in 1 (2%). Single-incision technique was used in 12 of 33 (36%) laparoscopic cases. Modified two-stage procedures had shorter total median LOS (7 days versus 9 days, P = .005). Incidence of postoperative leak, readmission, return to the operating room, and maximum 30-day Clavien–Dindo scores at the time of IPAA creation did not differ between modified two- and three-stage approaches (all P > .05). Conclusion: The modified two-stage approach to IPAA creation resulted in fewer hospital days compared to the three-stage approach. Considering the risks and patient burdens of diversion, further research is needed to assist in decisions regarding protective ileostomy after completion proctectomy with IPAA.