Inpatient management of Hirschsprung's associated enterocolitis treatment: the benefits of standardized care.

INTRODUCTION: Patients with Hirschsprung's disease (HSCR) remain at risk of developing Hirschsprung-associated enterocolitis (HAEC) after surgical intervention. As inpatient management remains variable, our institution implemented an algorithm directed at standardizing treatment practices. This study aimed to compare the outcomes of patients pre- and post-algorithm. METHODS: A retrospective review of patients admitted for HAEC was performed; January 2017-June 2018 encompassed the pre-implementation period, and October 2018-October 2019 was the post-implementation period. Demographics and outcomes were compared between the two groups. RESULTS: Sixty-two episodes of HAEC occurred in 27 patients during the entire study period. Sixteen patients (59%) had more than one episode. The most common levels of the transition zone were the rectosigmoid (50%) and descending colon (27%). Following algorithm implementation, the median length of stay (2 vs. 7 days, p < 0.001), TPN duration (0 vs. 5.5 days, p < 0.001), and days to full enteral diet (6 days vs. 2 days, p < 0.001) decreased significantly. Readmission rates for recurrent enterocolitis were similar pre- and post-algorithm implementation. CONCLUSION: The use of a standardized algorithm significantly decreases the length of stay and duration of intravenous antibiotic administration without increasing readmission rates, while still providing appropriate treatment for HAEC. LEVEL OF EVIDENCE: III level. TYPE OF STUDY: Retrospective comparative study.

Variability in perioperative evaluation and resource utilization in pediatric patients with suspected biliary dyskinesia: A multi-institutional retrospective cohort study.

INTRODUCTION: Biliary dyskinesia (BD) is a common indication for pediatric cholecystectomy. While diagnosis is primarily based on diminished gallbladder ejection fraction (GB-EF), work-up and management in pediatrics is controversial. METHODS: We conducted a multi-institutional retrospective review of children undergoing cholecystectomy for BD to compare perioperative work-up and outcomes. RESULTS: Six hundred seventy-eight patients across 16 institutions were included. There was no significant difference in gender, age, or BMI between institutions. Most patients were white (86.3%), non-Hispanic (79.9%), and had private insurance (55.2%). Gallbladder ejection fraction (EF) was reported in 84.5% of patients, and 44.8% had an EF <15%. 30.7% of patients were initially seen by pediatric surgeons, 31.3% by pediatric gastroenterologists, and 23.4% by the emergency department with significant variability between institutions (p < 0.001). Symptoms persisted in 35.3% of patients post-operatively with a median follow-up of 21 days (IQR 13, 34). On multivariate analysis, only non-white race and the presence of psychiatric comorbidities were associated with increased risk of post-operative symptoms. CONCLUSION: There is significant variability in evaluation and follow-up both before and after cholecystectomy for BD. Prospective research with standardized data collection and follow-up is needed to develop and validate optimal care pathways for pediatric patients with suspected BD. STUDY TYPE: Case Series, Retrospective Review. LEVEL OF EVIDENCE: Level IV.

Enteric duplication in children.

INTRODUCTION: Enteric duplication is a congenital anomaly with varied clinical presentation that requires surgical resection for definitive treatment. This had been approached with laparotomy for resection, but has changed with minimally invasive technique. The purpose of our study was to determine the demographics, natural history, operative interventions, and outcomes of pediatric enteric duplication cysts in a contemporary cohort. METHODS: With IRB approval, we performed a retrospective chart review of all patients less than 18 years old treated for enteric duplication between January 2006 and August 2016. Demographics, patient presentation, operative technique, intraoperative findings, hospital course, and follow-up were evaluated. Descriptive statistical analysis was performed; all medians were reported with interquartile range (IQR). RESULTS: Thirty-five patients underwent surgery for enteric duplication, with a median age at surgery of 7 months (2.5-54). Median weight was 7.2 kg (6-20). Most common patient presentations included prenatal diagnosis 37% (n = 13). Thirty-four patients (97%) had their cyst approached via minimally invasive technique (thoracoscopy or laparoscopy) with only three (8%) requiring conversion to an open operation. Median operative time was 85 min (54-133) with 27 (77%) patients requiring bowel resection. Median length of bowel resected was 4.5 cm (3-7). Most common site of duplication was ileocecal (n = 15, 42%). Postoperative median hospital length of stay was 3 days (2-5) and median number of days to regular diet was 3 (1-4). No patients required re-operation during their hospital stay. Median follow-up was 25 days (20-38). CONCLUSION: In our series, most enteric duplication cysts were diagnosed prenatally. These can be managed via minimally invasive technique with minimal short-term complications, even in neonates and infants.

The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study.

BACKGROUND: The management of malrotation in patients with congenital abdominal wall defects has varied among surgeons. We were interested in investigating the risk of midgut volvulus in patients with gastroschisis and omphalocele to help determine if these patients may benefit from undergoing a Ladd procedure. METHODS: A retrospective chart review was performed for all patients managed at three institutions born between 1/1/2000 and 12/31/2008 with a diagnosis of gastroschisis or omphalocele. Patient charts were reviewed through 12/31/2012 for occurrence of midgut volvulus or need for second laparotomy. RESULTS: Of the 414 patients identified with abdominal wall defects, 299 patients (72%) had gastroschisis, and 115 patients (28%) had omphalocele. The mean gestational age at birth was 36.1±2.3weeks, and the mean birth weight was 2.57±0.7kg. There were a total of 8 (1.9%) cases of midgut volvulus: 3 (1.0%) patients with gastroschisis compared to 5 patients (4.4%) with omphalocele (p=0.04). CONCLUSIONS: Patients with omphalocele have a greater risk of developing midgut volvulus, and a Ladd procedure should be considered during definitive repair to mitigate these risks. LEVEL OF EVIDENCE: III; retrospective comparative study.