Feeding and Growth Outcomes in Infants with Type C Esophageal Atresia Who Undergo Early Primary Repair.

OBJECTIVES: To describe growth and feeding outcomes in patients with type C esophageal atresia who underwent early primary repair and to identify predictors for poor growth. STUDY DESIGN: This single-center, retrospective, cohort study included all patients with type C esophageal atresia who underwent early primary repair from 2013 to 2019. Weight-for-age z score (WAZ) was calculated at birth, and every 6 months until 3 years postoperatively. Longitudinal median regression was used to evaluate WAZ over time. A multivariable logistic regression model explored predictors of growth outcomes. RESULTS: Of 46 infants who met the inclusion criteria, 72% were term. The median age at repair was 1.5 days of life (IQR, 1-2 days of life) and the hospital length of stay was 20 days (IQR-14, 30 days). Two patients had esophageal leak (4.3%). The median WAZ at birth was below average (-0.72; IQR, -1.37 to -0.40), but improved to reach average by 3 years (-0.025; IQR, -0.85 to 0.97, P < .001). At discharge, 72% of patients were receiving full oral nutrition, which improved to 95% by 3 years. The only independent predictor of poor growth at 1 year (WAZ < -1 [33%]) was WAZ at discharge (P = .02). CONCLUSIONS: Infants with esophageal atresia who undergo early primary repair are capable of achieving standard growth curves by 3 years of age. However, poor discharge WAZ score was predictive of poor WAZ score at 1 year. Efforts to identify at-risk patients and institute targeted inpatient and outpatient nutrition interventions are needed to improve their growth trajectory.

Hirschsprung Disease: The Rise of Structured Transition and Long-term Care.

Hirschsprung disease, like most surgically correctable congenital anomalies, is now survivable well into adulthood. Patients are, therefore, presenting later in life with problems that were previously infrequently encountered by colorectal surgeons or gastroenterologists. The goals of our review are to describe the current state of care transition for this patient population, identify the specific needs for these patients, and propose guidelines which could inform a future model of transition from the pediatric to adult setting. This is a review of the current state of care transition and long-term outcomes for patients with Hirschsprung disease. Although these patients report a generally good quality of life, the majority suffer from some degree of lifelong gastrointestinal complication, regardless of the type of operative repair. A more formalized transition of care will provide a guide for pediatric surgeons and patients, alleviate colorectal surgeon and gastroenterologist concerns, and provide better long-term care for these patients.

External Validation of University of Wisconsin's Clinical Criteria for Obtaining Maxillofacial Computed Tomography in Trauma.

OBJECTIVES: Patients sustaining multisystem trauma are at risk for oral and maxillofacial fractures. Although the University of Wisconsin established criteria to help guide the clinician in obtaining additional cross-sectional imaging to evaluate possible facial fractures, it has not been externally validated. Our aim was to evaluate whether the University of Wisconsin's Criteria is generalizable to external institutions through validation and to report modern practice patterns at a level 1 trauma center. METHODS: A retrospective case study was performed of all patients who had computed tomography of the facial bones (CT face) at a tertiary, academic, Level 1 trauma center over the 6-month period ending on June 30, 2015. The electronic medical record was reviewed for the 5 University of Wisconsin criteria (bony step off or instability, periorbital ecchymosis, malocclusion, tooth absence, and glasgow coma scale). Final interpretation of CT face findings by board-certified radiologists (facial fractures, intracranial hemorrhage, and cervical spine injury) were also captured. Our modeling was similar to that described by the reference study, the internal validation study. Sensitivity, specificity, negative, and positive predictive values with 95% confidence intervals were evaluated. A P < 0.05 was considered significant. RESULTS: The presence of any ≥1 of the 5 criteria identified on physical examination resulted in 81% sensitivity for any facial fracture, which is lower than the sensitivity initially described (98%) and subsequently internally validated (97%). The absence of all 5 physical examination criteria had a negative predictive value of 60%, again lower than that initially described (87%) and then internally validated (81%). CONCLUSION: We were unable to validate the University of Wisconsin criteria for predicting facial fractures. These criteria may be institutionally specific and not generalizable to other trauma centers. Further research to refine the criteria for CT of the face is needed to improve resource allocation.

Quality of dictated feedback associated with SIMPL operative assessments of pediatric surgical trainees.

BACKGROUND: SIMPL is a workplace-based operative performance assessment tool which allows for dictated feedback (DF). To better understand the value of DF, we sought to characterize the type and quality of DF generated during SIMPL evaluations. METHODS: Thematic analysis of DF from SIMPL assessments between June 2017 and December 2018 at a single pediatric surgery fellowship program was performed. Comments were categorized as specific, encouraging or corrective. Categories were combined to determine DF quality as effective, mediocre or ineffective. RESULTS: Of 781 SIMPL assessments (21 faculty, 5 trainees), 451 (57%) had DF. Most comments were encouraging (93%) and specific (65%). Only 21% were corrective, 17% had entrustment features, and 8% had an explicit learning plan. Feedback quality was deemed mediocre (45%), ineffective (33%) and effective (21%). CONCLUSION: SIMPL dictated feedback was mostly encouraging and specific. To improve quality, feedback should incorporate learning plans as well as corrective and entrustment features.

Nutrition delivery and growth outcomes in infants with long-gap esophageal atresia who undergo the Foker process.

BACKGROUND: Predictors of growth outcomes in patients with long-gap esophageal atresia (LGEA) are not known. We examined nutrition and growth in-hospital and post-discharge in LGEA patients who underwent the Foker Process (FP). METHODS: Single-center, retrospective cohort study of infants with LGEA undergoing primary (non-rescue) FP from 2014 to 2020. Weight-for-age z scores (WAZ, 0 = average), macronutrient prescription, anthropometry, and clinical variables were collected. Longitudinal median regression evaluated differences in WAZ over time. Multivariable median regression examined variables associated with change in WAZ at 1 year. RESULTS: 45 patients met criteria, with median (IQR) age at repair of 4 (2, 5.8) months and WAZ of -0.96 (-1.55, -0.40). On admission, 11% were moderately (WAZ < -2) and 9% were severely (WAZ < -3) malnourished. Lower admission WAZ was significantly associated with improvement in WAZ at 1-year follow-up (p = 0.002); EA type (59% type A), esophageal leak (16%), median days paralyzed (13), ventilated (21), on parenteral nutrition (35), or to full enteral nutrition (35) were not associated with change in WAZ. Median WAZ remained stable while in-hospital, and patients maintained their growth curves through 3-year follow-up. CONCLUSION: Throughout infancy, most primary FP LGEA patients have weight for age that is below average. Using targeted nutritional intervention, those who present with malnutrition can still achieve adequate growth despite prolonged and complicated hospital courses.

Resident Education in End-of-Life Communication and Management: Assessing Comfort Level to Enhance Competence and Confidence.

OBJECTIVES: Our primary objective was to understand residents' baseline comfort with end-of-life (EOL) communication and management and to compare this with their comfort after completion of their surgical intensive care unit (SICU) rotation. We also evaluated the association between prior training with perceived level of comfort with EOL issues, and whether the resident believed in the concept of a "better death." DESIGN, SETTING, PARTICIPANTS: As a quality improvement initiative, we conducted surveys of trainees before and after their rotation in the Yale New Haven Hospital SICU. Prerotation and postrotation surveys were administered to all residents who rotated during the 2016-2017 academic year and the first half of 2017-2018. The survey consisted of 34 questions querying residents on their level of training in EOL care, their comfort with management and discussions in different EOL domains, and their beliefs about what measures would have improved their ability to provide EOL care. Residents surveyed were from general surgery, emergency medicine, or anesthesia departments. RESULTS AND CONCLUSIONS: Our study demonstrates that there is a significant correlation between resident comfort with EOL communication and experience providing EOL care. However, concepts in medicolegal aspects of palliative care could be taught through formal didactics, and structured training may allow residents the opportunity to reflect on the importance of a "better death."

Expanding the phenotypic spectrum of Mabry Syndrome with novel PIGO gene variants associated with hyperphosphatasia, intractable epilepsy, and complex gastrointestinal and urogenital malformations.

Mabry syndrome is a glycophosphatidylinositol (GPI) deficiency characterized by intellectual disability, distinctive facial features, intractable seizures, and hyperphosphatasia. We expand the phenotypic spectrum of inherited GPI deficiencies with novel bi-allelic phosphatidylinositol glycan anchor biosynthesis class O (PIGO) variants in a neonate who presented with intractable epilepsy and complex gastrointestinal and urogenital malformations.

Doxycycline sclerotherapy for post-traumatic inguinal lymphocele in a child.

Lymphoceles are abnormal collections of lymphatic fluid caused by a disruption in the lymphatic channels and leakage of lymph. This most commonly occurs after surgical procedures, but occasionally lymphoceles may be the result of trauma, more commonly penetrating trauma. Lymphoceles resulting from blunt trauma are rare in both adults and children. In the adult population, there are few published case reports, and management principles vary. To date, there are no reports of traumatic lymphoceles in the paediatric population, and therefore there is no precedent for treatment. Here, we report the case of a young boy who developed an inguinal lymphocele from a bicycle handle bar injury which was successfully treated with doxycycline sclerotherapy.