Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group.

BACKGROUND/PURPOSE: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients. METHODS: Retrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale. RESULTS: There were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD. CONCLUSION: More than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment. LEVEL OF EVIDENCE: Level 4.

Impact of Multidisciplinary Standardization of Care for Gastroschisis: Treatment, Outcomes, and Cost.

BACKGROUND/PURPOSE: Elimination of unnecessary practice variation through standardization creates opportunities for improved outcomes and cost-effectiveness. A quality improvement (QI) initiative at our institution used evidence and consensus to standardize management of gastroschisis (GS) from birth to discharge. METHODS: An interdisciplinary team utilized best practice evidence and expert opinion to standardize GS care. Following stakeholder engagement and education, care standardization was implemented in September 2014. A comparative cohort study was conducted on consecutive patients treated before (n=33) and after (n=24) standardization. Demographic, treatment, and outcome measures were collected from a prospective GS registry. Direct costs were estimated, and protocol compliance was audited. RESULTS: BW, GA, and bowel injury severity were comparable between groups. Key practice changes were: closure technique (pre-88% primary fascial, post-83% umbilical cord flap; p<0.001), closure location (pre-97% OR, post-67% NICU; p<0.001), and GA avoidance (pre-0%, post-48%; p<0.001). Median post-closure ventilation days were shorter (pre-4, post-1; p<0.001), and SSI rates trended lower (pre-21%, post-8%; p=0.3) in the post-implementation group with no differences in TPN days or LOS. No significant difference was seen in average per-patient costs: pre-$85,725 ($29,974-221,061), post-$76,329 ($14,205-176,856). CONCLUSION: Care standardization for GS enables practice transformation, cost-effective outcome improvement, and supports an organizational culture dedicated to continuous improvement. LEVEL OF EVIDENCE: III.

Dressed for success? Silver impregnated nanocrystalline dressing for initial treatment of giant omphalocele.

OBJECTIVE: The purpose of this study was to describe outcomes and resource utilization in patients treated with twice-weekly silver impregnated (SI) nanocrystalline dressings for initial non-operative management of giant omphalocele (GO). METHODS: A retrospective review of patients with GO treated with SI dressings was undertaken. Clinical parameters, cost, and complications were recorded. RESULTS: Five patients with GO were treated with SI dressings between 2014 and 2016. Clinical characteristic (mean ± SD) included gestational age 36 ± 4 weeks, birth weight 2.6 ± 0.63 kg, GO size 10.2 ± 4.7 cm, ventilator days 7.5 ± 8.7 d, days in NICU 41 ± 20 d, days to full feeds, 30 ± 15 d, and LOS 62 ± 41 d. The average in-hospital cost of SI dressings was $110 CAD/week. This is comparable to daily silver sulfadiazine dressings ($109CAD/week) which were used historically. All patients were discharged with once- or twice-weekly dressing changes. No ruptures occurred. There was one mortality secondary to pulmonary sepsis. CONCLUSIONS: For initial non-operative management of GO, twice weekly SI nanocrystalline dressings is safe and effective. Use of SI dressings results in decreased handling of infants, reduced physician and nursing resource utilization, and favourable outcomes. LEVEL OF EVIDENCE: IV (Retrospective Case Series).

Understanding gastroschisis and its clinical management: where are we?

INTRODUCTION: Gastroschisis is the commonest developmental defect of the anterior abdominal wall in both developed and developing countries. The past 30 years have seen transformational improvements in outcome due to advances in neonatal intensive care and enhanced integration between the disciplines of maternal fetal medicine, neonatology and pediatric surgery. A review of gastroschisis, which emphasizes its epidemiology, multidisciplinary care strategies and contemporary outcomes is timely. Areas covered: This review discusses the current state of knowledge related to prevalence and causation, and postulated embryopathologic mechanisms contributing to the development of gastroschisis. Using relevant, current literature with an emphasis on high level evidence where it exists, we review modern techniques of prenatal diagnosis, pre and postnatal risk stratification, preferred timing and method of delivery, options for abdominal wall closure, nutritional management, and short and long term clinical and neurodevelopmental follow-up. Expert commentary: This section explores controversies in contemporary management which contribute to practice and cost variation and discusses the benefits of novel nutritional therapies and care standardization that target unnecessary practice variation and improve overall cost-effectiveness of gastroschisis care. The commentary concludes with a review of fertile areas of gastroschisis research, which represent opportunities for knowledge synthesis and further outcome improvement.

Mucous fistula refeeding in neonates with enterostomies.

BACKGROUND/PURPOSE: Neonates with intestinal pathology may require staged surgery with creation of an enterostomy and mucous fistula (MF). Refeeding (MFR) of ostomy output may minimize fluid and electrolyte losses and reduce dependence on parenteral nutrition (PN), though a paucity of evidence exists to support this practice. The purpose of this study was to assess the outcomes of infants undergoing MFR and document associated complications. METHODS: With REB approval, infants with intestinal failure undergoing MFR between January 2000 and December 2012 were identified. A chart review was conducted and relevant data were collected. Descriptive statistics were used. RESULTS: Twenty-three neonates underwent MFR. Mean gestational age and birth weight were 35weeks and 2416grams. Pathologies included intestinal atresia (n=12), necrotizing enterocolitis (n=5), meconium ileus (n=4), and other (n=6). Seven patients were able to wean from PN. Four patients had complications: 3 had perforation of the MF, 1 had bleeding. Four patients died, with one death directly attributable to MFR. CONCLUSIONS: In this cohort MF refeeding was associated with significant complications and ongoing PN dependence. With advances in intestinal rehabilitation and PN, the benefit of MF refeeding must be weighed against the potential complications.

Management of the open abdomen with the Abdominal Reapproximation Anchor dynamic fascial closure system.

BACKGROUND: With the increased use of damage control surgery and open abdomens, there are growing challenges in achieving primary fascial closure. The purpose of this study was to retrospectively review our experience using the Abdominal Reapproximation Anchor (ABRA; Canica Design Inc, Almonte, Ontario, Canada), a dynamic fascial closure system, to gain fascial apposition in complex abdominal surgical patients. METHODS: A retrospective review of patients who underwent placement of the ABRA device to aid in abdominal closure was undertaken. Details including age, sex, the reason for an open abdomen, the number of operations, the time to primary closure, the success rate of primary closure, and complications related to the use of the ABRA were analyzed. RESULTS: Between January 2006 and July 2011, 36 patient charts were identified. The average Acute Physiology and Chronic Health Evaluation II score was 21.9 ± 6.9. There was a mean of 3.1 ± 1.8 laparotomies before ABRA placement for each patient, and the duration of ABRA placement until removal was 10.4 ± 6.1 days. Complete fascial apposition was achieved in 83% of the patients across the entire study and in 91% of the patients in the final 2 years. Component separation was used in 17% of cases. The incisional hernia rate was 13% at 6 months and 11% at 12 months. CONCLUSIONS: Our use of the ABRA system resulted in an 83% fascial apposition rate, which further improved when experience was taken into account. The incisional hernia rate was acceptable in this complicated patient group. This technique is an excellent addition to a surgeon's armamentarium for complicated abdominal cases that require an open abdomen. Further prospective studies are planned to identify ideal candidates for this technique.

'It's something that I manage but it is not who I am': reflections on internalized stigma in individuals with bipolar disorder.

Bipolar disorder (BD) is a complex chronic condition associated with substantial costs, both at a personal and societal level. Growing research indicates that experiences with stigma may play a significant role in contributing to the distress, disability, and poor quality of life (QoL) often experienced in people with BD. Here, we present a sub-set of findings from a qualitative study of self-management strategies utilized by high functioning Canadian individuals with BD. Specifically, we describe a theme relating to participants' experiences and understandings of internalized stigma. Descriptive qualitative methods were used including purposeful sampling and thematic analysis. High functioning individuals with BD type I or II (N = 32) completed quantitative scales to assess symptoms, functioning and QoL, and participated in an individual interview or focus group to discuss the self-management strategies that they use to maintain or regain wellness. Thematic analysis identified several themes, including one relating to internalized stigma. Within this, four additional themes were identified: stigma expectations and experiences, sense of self/identity, judicious disclosure, and moving beyond internalised stigma. One of the more unique aspects of the study is that it involves a participant sample that is managing well with their illness, which differs from the norm in biomedical research that typically focuses on pathology, problems and dysfunction.