Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: A Case Series With Long-term Follow-up and Prolonged Survival.

OBJECTIVES: Describe clinical characteristics, management, and outcome in a cohort of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) patients. METHODS: We conducted a retrospective chart review of MMIHS patients followed at a large transplant and intestinal rehabilitation center over a period of 17 years. RESULTS: We identified 25 patients with MMIHS (68% girls, 13 transplanted). One transplanted and 1 nontransplanted patient were lost to follow-up. We estimated 100, 100, and 86% for 5-, 10-, and 20-year survival, respectively, with only 1 death. Of the 22 patients alive at the time of study (11 transplanted, 11 nontransplanted), median age was 9.2 years (range 2.7-22.9 years). Longest posttransplant follow-up was 16 years. Seventeen patients had available prenatal imaging reports; all showed distended bladder. Eight had genetic testing (5, ACTG2; 2, MYH11; 1, MYL9). Almost all patients had normal growth with median weight z-score -0.77 (interquartile range -1.39 to 0.26), height z score -1.2 (-2.04 to -0.48) and body mass index z-score 0.23 (-0.37 to 0.93) with no statistical difference between transplanted and nontransplanted patients. All nontransplanted patients were on parenteral nutrition with minimal/no feeds, and all except 1 of the transplanted patients were on full enteral feeds. Recent average bilirubin, INR, albumin, and creatinine fell within the reference ranges. CONCLUSIONS: This is the largest single-center case series with the longest duration of follow-up for MMIHS patients. In the current era of improved intestinal rehabilitation and transplantation, MMIHS patients have excellent outcomes in survival, growth, and liver function. This observation contradicts previous reports and should alter counselling and management decisions in these patients at diagnosis.

Pediatric Nasogastric Tube Placement and Verification: Best Practice Recommendations From the NOVEL Project.

The placement of a nasogastric tube (NGT) in a pediatric patient is a common practice that is generally perceived as a benign bedside procedure. There is potential risk for NGT misplacement with each insertion. A misplaced NGT compromises patient safety, increasing the risk for serious and even fatal complications. There is no standardized method for verification of the initial NGT placement or reverification assessment of NGT location prior to use. Measurement of the acidity or pH of the gastric aspirate is the most frequently used evidence-based method to verify NGT placement. The radiograph, when properly obtained and interpreted, is considered the gold standard to verify NGT location. However, the uncertainty regarding cumulative radiation exposure related to radiographs in pediatric patients is a concern. To minimize risk and improve patient safety, there is a need to identify best practice and to standardize care for initial and ongoing NGT location verification. This article provides consensus recommendations for best practice related to NGT location verification in pediatric patients. These consensus recommendations are not intended as absolute policy statements; instead, they are intended to supplement but not replace professional training and judgment. These consensus recommendations have been approved by the American Society for Parental and Enteral Nutrition (ASPEN) Board of Directors.

Soluble Fiber Use in Pediatric Short Bowel Syndrome: A Survey on Prevailing Practices.

BACKGROUND: In pediatric short bowel syndrome (SBS), adding fiber to enteral feedings is 1 treatment method to manage increased stool output. However, there are no standardized recommendations on the use of fiber in this setting, including type, dosage, titration strategies, etc. OBJECTIVE: The aim of this study is to determine current prevailing practices on the use of fiber in the treatment of chronic high stool output in the pediatric SBS population. METHODS: An anonymous electronic survey with 13 questions was sent through health professional electronic mailing lists. The survey was completed by healthcare professionals including physicians (primary care, subspecialists, and surgeons), nurse practitioners, and registered dietitians. RESULTS: A total of 94 responses were received. The most common supplemental fiber used was pectin (62.8%). The 2 major factors considered when initiating fiber therapy were consistency of stool (74.5%) and volume of stool output (85.1%). The major factor that determined discontinuation of fiber was abdominal distention (67%). A majority of providers waited 2 weeks or less to see improvement following fiber initiation before discontinuing it. CONCLUSIONS: The goal of the survey was to gather more information with regard to fiber use in the management of SBS patients. The data collected can be used to provide future direction on determining best practices for fiber use in SBS patients.

Optimizing Transition to Home Enteral Nutrition for Pediatric Patients.

Enteral nutrition is delivered via the gastrointestinal tract when oral intake is not sufficient to maintain nutrition status. There is evidence that long-term home enteral nutrition (HEN) can be advantageous to pediatric patients by improving quality of life and function. Data from pediatric patients receiving either nasogastric tube or gastrostomy tube feeds indicate good outcomes in terms of maintaining and improving nutrition status as evidenced by anthropometric measurements. As the number of pediatric patients requiring HEN increases, development of an effective program to allow smooth transition to the home is necessary. The formation of a multidisciplinary HEN management team can achieve the important task of taking care of children requiring HEN who are technology dependent. Members of a multidisciplinary HEN team can include care coordinators, nurses, physicians, dietitians, and speech therapists. Planning for patient discharge to home should start well before the actual discharge, with timely identification of home caregivers, the family's learning style, and completion of other requirements for safe administration of HEN. Education should be provided in a continuum, starting at the identification of nutrition support while still in the hospital and continuing after discharge to address concerns and long-term complications of HEN.

Standardization of Feeding Advancement After Neonatal Gastrointestinal Surgery: Does It Improve Outcomes?

BACKGROUND: Postabdominal intestinal surgery (PAIS) infants pose many complex management issues. Utilization of feeding guidelines has been shown to improve outcomes in preterm and low-birth-weight infants. We propose that standardization of feeding for PAIS infants is safe. METHODS: We identified 163 PAIS infants: 93 prior to and 70 after implementation of a feeding guideline. The primary outcome was time to full enteral nutrition (EN). A propensity score-matched analysis was performed. RESULTS: The preimplementation and postimplementation PAIS infants were similar at baseline. No significant differences were seen in matched groups for time to full EN, parenteral nutrition days, or time to discharge, but cholestasis was less severe in the postimplementation group and breast milk use increased. Good compliance (67%) to daily guideline use was achieved during the initial 2 years. CONCLUSIONS: Utilization of a feeding guideline is safe and standardizes care within an institution, improving compliance to evidence-based practices and outcomes.

A case of pancreatitis, panniculitis and polyarthritis syndrome: Elucidating the pathophysiologic mechanisms of a rare condition.

Pancreatitis-Panniculitis-Polyarthritis (PPP) syndrome is rare and its physiopathology unclear. A 6-year old boy suffered of traumatic pancreatitis complicated by PPP syndrome. Extensive investigations demonstrated high levels of pancreatic lipase and fatty acids in the affected peripheral tissues. These findings support the sequence of peripheral lipolysis and fatty acid accumulation inducing tissue inflammation.