Gastroesophageal Reflux Disease and Foregut Dysmotility in Children with Intestinal Failure

Abstract

Gastrointestinal dysmotility is a common problem in a subgroup of children with intestinal failure (IF), including short bowel syndrome (SBS) and pediatric intestinal pseudo-obstruction (PIPO). It contributes significantly to the increased morbidity and decreased quality of life in this patient population. Impaired gastrointestinal (GI) motility in IF arises from either loss of GI function due to the primary disorder (e.g., neuropathic or myopathic disorder in the PIPO syndrome) and/or a critical reduction in gut mass. Abnormalities of the anatomy, enteric hormone secretion and neural supply in IF can result in rapid transit, ineffective antegrade peristalsis, delayed gastric emptying or gastroesophageal reflux. Understanding the underlying pathophysiologic mechanism(s) of the enteric dysmotility in IF helps us to plan an appropriate diagnostic workup and apply individually tailored nutritional and pharmacological management, which might ultimately lead to an overall improvement in the quality of life and increase in enteral tolerance. In this review, we have focused on the pathogenesis of GI dysmotility in children with IF, as well as the management and treatment options.

Nutrition and Intestinal Rehabilitation of Children With Short Bowel Syndrome: A Position Paper of the ESPGHAN Committee on Nutrition. Part 2: Long-Term Follow-Up on Home Parenteral Nutrition

“…However, there is no evidence to support routine use of endoscopy in the absence of symptoms of severe/refractory gastroesophageal reflux disease (GERD). A comprehensive review on GERD and foregut dysmotility in children with IF, even if not SBS specific, was recently published addressing etiology and possible therapeutic options (96).…”

Current Surgical Concepts and Indications in the Management of the Short Bowel State: A Call for the Use of Multidisciplinary Intestinal Rehabilitation Programs

“…At the time of first assessment, data for personalized management are collected (neonatal history, surgical history, nutritional status, status of the anatomy and physiology of the remaining bowel, and potential metabolic and/or central line complications) [19][20][21][22]. In fact, a 360-degree multidisciplinary evaluation of the patient state is key to orienting management.…”

Nutrition and Intestinal Rehabilitation of Children With Short Bowel Syndrome: A Position Paper of the ESPGHAN Committee on Nutrition. Part 1: From Intestinal Resection to Home Discharge

“…Rapid gastric emptying may contribute to fluid losses in children with SBS, with subsequent risk of dehydration. On the other hand more than 10% of children with SBS show delayed gastric emptying after intestinal surgical resection most probably due to altered fundic receptive relaxation, decreased antral contractility, and incoordination of gastric emptying and duodenal contractions (62).…”

“…Rapid gastric emptying may contribute to fluid losses in children with SBS, with subsequent risk of dehydration. On the other hand more than 10% of children with SBS show delayed gastric emptying after intestinal surgical resection most probably due to altered fundic receptive relaxation, decreased antral contractility, and incoordination of gastric emptying and duodenal contractions (62). Alteration in gut motor activity may be observed especially in cases of prenatal small bowel malformations (gastroschisis and/or intestinal atresias) or severe postnatal pathology such as extensive NEC (17,63).…”

“… Alteration in gut motor activity may be observed especially in cases of prenatal small bowel malformations (gastroschisis and/or intestinal atresias) or severe postnatal pathology such as extensive NEC (17,63). Gastrointestinal dysmotility may be caused by decrease in the interstitial cells of Cajal, damage of the smooth muscle cells due to small bowel ischemia, and neuropathic changes with reduction of intramuscular nerve fibers, which occurs in various degree according to the underlying cause of SBS (62). They contribute, together with repeated surgical procedures to impair motor activity leading to SIBO and complications (see below).…”