Method of home tube feeding and 2-3-year neurodevelopmental outcome.

OBJECTIVE: To describe the Bayley Scales of Infant Development 3rd Edition (Bayley-III) of infants discharged home receiving tube feeds. STUDY DESIGN: Retrospective review of infants discharged with nasogastric or gastrostomy tube feeds and completed a Bayley-III assessment at 2-3-years of age through a neonatal follow-up program. Results were reported using descriptive statistics. RESULTS: Of infants discharged with nasogastric feeds, median Bayley-III scores were in the low-average to average range, and full oral feeds were achieved in 75%. Of infants discharged with gastrostomy tube feeds, median Bayley-III scores were in the extremely low range, and full oral feeds were achieved in 36%. Our data set did not demonstrate a distinct patient demographic that correlated to the type of feeding tube at discharge. CONCLUSION: Neurodevelopmental outcome at 2-3 years does not appear to be negatively impacted by the decision to discharge an infant from the NICU with home NG feedings.

Inflammatory Causes of Dysphagia in Children.

Gastroesophageal reflux (GER) and eosinophilic esophagitis (EoE) are the most common inflammatory causes of pediatric dysphagia, but several other less prevalent conditions should be considered. These conditions can affect one or several aspects of the swallowing process. In some inflammatory conditions dysphagia may be an early symptom. Esophagoscopy and instrumental swallow studies are often needed to determine the underlying diagnosis and best treatment plan. In some inflammatory conditions dysphagia can portend a worse outcome and need for more aggressive treatment of the underlying condition. Consultations with speech language pathology, gastroenterology, dietetics, allergy/immunology and/or rheumatology are often needed to optimize management.

Feeding dysfunction in NICU patients with cramped synchronized movements.

Patients admitted to the neonatal intensive care unit (NICU) have higher association for neurodevelopment deficits, specifically cerebral palsy (CP). We identified patients with risk for CP using abnormal Pretchl's General Movement Assessment (GMA) and sub-category of cramped synchronized movements (CSM) and reported their feeding outcomes at discharge. Over 75 % of these patients required either nasogastric (NGT) or gastrostomy tube (GT) at discharge. Of these, 57 % weaned off their NGT or GT at home and 43 % of patients still needed a GT one year after discharge. Of those that could not wean off their NGT or GT, these patients had longer hospital stay, took lower percentage by mouth, and an older post-menstrual age at discharge. We did not find a difference in NGT or GT use between patients with IVH, ELBW, nor between their birthweight or gestation age at birth. This study provides further clinical characteristics in NICU patients who have higher risk of CP, and supports the need for skilled feeding therapy and resources both during and after NICU admission.

Flexible Sigmoidoscopy Utility in the Diagnosis of Pediatric Gastrointestinal Disorders.

AIM:  Although flexible sigmoidoscopy (FS) is utilized in children for the diagnosis of pediatric gastrointestinal conditions, such as inflammatory bowel disease and juvenile polyp disorders, the diagnostic yield of FS in pediatric patients is unknown. MATERIALS AND METHODS:  We retrospectively reviewed FS cases in children under 18 years of age over a five-year period at our institution. Indications for the procedure, endoscopic visual findings, histologic findings, final diagnosis, and any management changes based on FS findings were included. RESULTS:  A total of 354 cases were included in the analysis for which 40 cases (11.3%) had abnormal visual findings, 48 cases (13.6%) had abnormal histologic findings, and 13 cases (3.7%) had both abnormal endoscopic visual and histologic findings. Of the 88 cases with abnormal visual and/or histologic abnormalities, only the results of 34 of these FS cases led to a change in management based on endoscopic findings (9.6%). Most patients with a non-diagnostic FS had a final diagnosis of functional abdominal pain; most patients with a diagnostic FS had a final diagnosis of colitis, not otherwise specified. CONCLUSION:  Our findings suggest that FS is not a helpful diagnostic endoscopic intervention in pediatric patients, especially in children with reassuring history and physical exam findings.

Partial Enteral Discharge Programs for High-risk Infants.

Premature infants or infants born with complex medical problems are at increased risk of having delayed or dysfunctional oral feeding ability. These patients typically require assisted enteral nutrition in the form of a nasogastric tube (NGT) during their NICU hospitalization. Historically, once these infants overcame their initial reason(s) for admission, they were discharged from the NICU only after achieving full oral feedings or placement of a gastrostomy tube. Recent programs show that these infants can be successfully discharged from the hospital with partial NGT or gastrostomy tube feedings with the assistance of targeted predischarge education and outpatient support. Caregiver opinions have also been reported as satisfactory or higher with this approach. In this review, we discuss the current literature and outcomes in infants who are discharged with an NGT and provide evidence for safe practices, both during the NICU hospitalization, as well as in the outpatient setting.

Clinical diagnostic predictive score for Meckel diverticulum.

BACKGROUND/PURPOSE: Meckel diverticulum (MD) is present in 2% of the population. Many practitioner feel the diagnosis relies upon technetium-99 m pertechnetate scintigraphy. When negative, patients undergo additional invasive procedures delaying definitive therapy. This study aims to identify features of bleeding MD and generate a risk score, which could preclude unnecessary testing and facilitate earlier operation. METHODS: All patients <18-years-old who presented with hematochezia from 2005 to 2015 were identified. MD diagnosis was based on histopathology of operative tissue. Controls were patients with hematochezia undergoing colonoscopy. A points system was used generate a risk score. RESULTS: A total of 215 patients presented with hematochezia out of which 42 patients with MD were identified. Predictive variables included infant (OR 7, 95%CI 2-29) and toddler (OR 20, 95%CI 8-50) age groups, duration <6 days (OR 18, 95%CI 8-43), presence of large blood volume (OR 16, 95% CI 7-36), hemoglobin <7 g/dL (OR 6, 95% CI 3-15) and transfusion requirement (OR 16, 95% CI 7-38). A score of 6 or higher is highly suggestive of MD. CONCLUSIONS: This scoring system identifies children with bleeding MD who may benefit from exploratory surgery without undergoing endoscopy. This novel scoring system can be applied to provide accurate clinical diagnosis, reduce unnecessary tests and allow prompt surgical management.

Creation of a Standard Model for Tube Feeding at Neonatal Intensive Care Unit Discharge.

BACKGROUND: Feeding dysfunction is a common consequence of prematurity and illness in neonates, often requiring supplemental nasogastric (NG) or gastrostomy (GT) feeding tubes. A standardized approach to the discharge of infants receiving home enteral nutrition (HEN) is currently lacking. METHODS: The Home Enteral Feeding Transitions (HEFT) program was developed to identify patients eligible for HEN and create a standard discharge process. A structured tool helped determine discharge timing and route, and a dedicated outpatient clinic was created for infants discharged on HEN. Demographic, inpatient, and outpatient data were prospectively collected and compared with a historical cohort. RESULTS: A total of 232 infants discharged from our neonatal intensive care unit (NICU) over 9 months met inclusion criteria. Ninety-eight (42%) were discharged with HEN, 68 NG and 30 GT, compared with 134 (58%) receiving full oral feeds. This represented a 10% increase in HEN utilization (P = 0.003) compared with our historical control group. Median HEN length of stay was 31.5 days compared with our historical average of 41 days (P = 0.23). Frequency of emergency department visits and admissions because of HEN was unchanged postintervention. Parents were satisfied (8.6/10), and 98% said they would choose HEN again. The median time to NG discontinuation after discharge was 13.5 days, with an estimated cost savings of $2163 per NICU day. CONCLUSION: Our program is the first of which we know to use a standard care-process model to guide the decision-making and utilization of HEN at NICU discharge. HEFT shows that HEN at NICU discharge can be safe and effective, with high parental satisfaction.

Effective Tube Weaning and Predictive Clinical Characteristics of NICU Patients With Feeding Dysfunction.

BACKGROUND: The present study evaluated the effectiveness of a multidisciplinary earlier discharge model for neonates receiving home enteral nutrition (HEN). METHODS: A retrospective data review and analysis was performed on 183 patients discharged out of the neonatal intensive care unit (NICU) receiving partial oral feeds (PO) and partial HEN from September 2016 to March 2018. These patients were followed in a multidisciplinary clinic led by a pediatric gastroenterologist, a neonatal feeding therapist, and a pediatric dietitian. Demographics and data were recorded for patients at discharge, and then chart reviews were performed for additional data. RESULTS: Of 182 patients, 121 (67%) weaned off HEN with a median time to full PO at 79 days (interquartile range [IQR] 15, 247) and had median PO intake of 20% (0, 43) at time of discharge. When comparing patients who gained 100% PO vs patients who did not wean off HEN, the weaned group consisted of 88% nasogastric tubes, with median time off feeds at 27 days (IQR 8, 79) and median PO intake of 29% (11, 50) at discharge. Only 13% of the cohort had an emergency room or hospital admission, which corresponds to 1.6 and 0.8 events, respectively, per 500 tube days specifically due to HEN complications. CONCLUSIONS: Our study supports that NICU patients with feeding dysfunction can effectively and safely discharge home earlier while receiving HEN. Our data suggest that a dedicated outpatient clinic can facilitate effective tube weaning in a majority of neonates with complex medical diseases with low rates of adverse events.

Neonatal cholestasis and hepatosplenomegaly caused by congenital dyserythropoietic anemia type 1: A case report.

BACKGROUND: Congenital dyserythropoietic anemia type 1 (CDA1) is an autosomal recessive disorder of ineffective erythropoiesis, resulting in increased iron storage. CDA1 is usually diagnosed in children and adolescents but can rarely present in the neonatal period with severe anemia at birth. There are no prior reports of neonatal liver histologic findings of CDA1. We report a case of CDA1 in a newborn presenting with severe anemia, cholestasis and liver failure, where liver biopsy helped confirm the diagnosis. CASE SUMMARY: A term infant, born via emergency Cesarean section, presented with cholestasis, hepatosplenomegaly, multiorgan failure and severe anemia at birth. A prior pregnancy was significant for fetal demise at 35 wk without autopsy or known etiology for the fetal demise. Parents are both healthy and there is no history of consanguinity. On further evaluation, the patient was found to have severe ferritin elevation and pulmonary hypertension. An extensive infectious and metabolic work-up was negative. Salivary gland biopsy was negative for iron deposition. At 2 wk of age, a liver biopsy showed findings consistent with CDA1. A genome rapid sequencing panel revealed novel variants in the CDAN1 gene. The patient's liver dysfunction, cholestasis and organomegaly resolved, however she remains transfusion-dependent. CONCLUSION: We report liver pathology findings of CDA1 with a novel genetic mutation for the first time in a newborn.

A Novel Care Model for Neonatal Intestinal Failure Patients Is Associated With Cost Savings and Improved Outcomes.

BACKGROUND: Neonates with intestinal failure (IF) have prolonged admissions in the neonatal intensive care unit (NICU) and require lifelong follow-up with gastroenterology (GE) as outpatients. Inpatient management of these patients typically relies on many rotating practitioners and currently discharge criteria do not exist. We sought to create standardized discharge criteria with a continuity care model for neonatal IF patients. METHODS: Inpatient care was streamlined to two GE physicians with weekly consultations. We implemented standardized discharge goals for both enteral and total parental nutrition (TPN) by: 1) Enteral feedings of at least 5 mL/h were tolerated; 2) Stable central venous access was intact; 3) TPN was cycled to 20 h/day or less; and 4) No other medical issues required NICU admission. Patient records were reviewed after 18 months of implementing standardized discharge criteria and we compared their outcomes to a historical cohort of IF patients. RESULTS: Optimal discharge criteria were met in 12 patients and a cohort of 26 historical patients was used for comparison. Patients in optimal versus historical groups had similar baseline characteristics (medians, all P values = non-significant (NS)): gestational age (36 vs. 35 weeks), birth weight (1,990 vs. 2,076 g), birth length (45 vs. 44 cm), and small bowel length after definitive surgery (63 vs. 55 cm). Compared to the historical group, the optimal cohort was discharged earlier (median length of stay 69 vs. 126 days, P < 0.01), with a reduced total stay of 684 NICU days, fewer central line-associated bloodstream infections (CLABSIs) (4 vs. 10 per 1,000 patient days, P = 0.04), and had fewer readmissions (7 vs. 17 per 1,000 patient days, P < 0.01), respectively. CONCLUSIONS: Concentrating the care of IF patients to a GE team invested in long-term care, while implementing safe discharge criteria, resulted in a dramatic length of stay reduction with fewer CLABSIs and readmissions compared to historical management. At approximately 4,000 dollars per day in NICU hospital charges, this program saved over 2.7 million dollars in care costs while allowing families and their infants more time at home. The safety and applicability of the optimal discharge criteria presented here should be studied further. Similar programs may be effective at other large NICUs.

Identification of Pediatric Patients With Celiac Disease Based on Serology and a Classification and Regression Tree Analysis.

BACKGROUND & AIMS: Celiac disease is detected using serology and endoscopy analyses. We used multiple statistical analyses of a geographically isolated population in the United States to determine whether a single serum screening can identify individuals with celiac disease. METHODS: We performed a retrospective study of 3555 pediatric patients (18 years old or younger) in the intermountain West region of the United States from January 1, 2008, through September 30, 2013. All patients had undergone serologic analyses for celiac disease, including measurement of antibodies to tissue transglutaminase (TTG) and/or deamidated gliadin peptide (DGP), and had duodenal biopsies collected within the following year. Modified Marsh criteria were used to identify patients with celiac disease. We developed models to identify patients with celiac disease using logistic regression and classification and regression tree (CART) analysis. RESULTS: Single use of a test for serum level of IgA against TTG identified patients with celiac disease with 90% sensitivity, 90% specificity, a 61% positive predictive value (PPV), a 90% negative predictive value, and an area under the receiver operating characteristic curve value of 0.91; these values were higher than those obtained from assays for IgA against DGP or IgG against TTG plus DGP. Not including the test for DGP antibody caused only 0.18% of celiac disease cases to be missed. Level of TTG IgA 7-fold the upper limit of normal (ULN) identified patients with celiac disease with a 96% PPV and 100% specificity. Using CART analysis, we found a level of TTG IgA 3.2-fold the ULN and higher to most accurately identify patients with celiac disease (PPV, 89%). Multivariable CART analysis showed that a level of TTG IgA 2.5-fold the ULN and higher was sufficient to identify celiac disease in patients with type 1 diabetes (PPV, 88%). Serum level of IgA against TTG in patients with versus those without trisomy 21 did not affect diagnosis predictability in CART analysis. CONCLUSIONS: In a population-based study, we found that serum level of IgA against TTG can identify patients with celiac disease with PPVs of about 90%. Predictive values increase greatly when levels are markedly above the ULN or when the assay is used in combination with other variables. Measurement of IgG against TTG or DGP does not increase the accuracy of detection of celiac disease based against TTG IgA levels. There is a low risk of false-positive results from serologic analysis in patients with type I diabetes or persistent increases in antibody against TTG on repeat testing.

Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI.

We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient's treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA. Early recognition of possible association with Ehlers-Danlos syndrome is important for quick intervention and successful management of pediatric patients.