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Gastrointestinal (GI) motility disorders (GMDs) are represented by a spectrum of conditions that range from benign prevalent disorders (gastroesophageal reflux (GER) and childhood constipation) to more rare and severe entities (chronic intestinal pseudo-obstruction (CIP) and Hirschsprung’s disease). Altered GI motility adds considerable co-morbidity to structural anomalies such as intestinal atresia, stenosis, or gastroschisis. Pediatric GMDs are classified according to the results of GI motility testing. It is likely that with advanced methods of studying the brain–gut axis, classification of these disorders will eventually be based on pathophysiology. Within the pediatric population, GMDs are also known to be either congenital or acquired, depending on the presence or absence of symptoms at birth.1,2 Congenital disorders usually cause symptoms within the first 2 months of life and can be sporadic or familial. Acquired motility disorders present later in life and can be secondary to a variety of insults including infections and adverse reactions to medications.3 Within the pediatric population, GMDs account for up to 15% of all intestinal failures.

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Based on histopathology and patterns of motility abnormalities, traditionally the causes of GMDs are also classified as visceral neuropathy or visceral myopathy.3 Neuropathic disorders are more common, but myopathies are usually associated with more severe symptoms.2,3 The role of genetic mutations in visceral neuropathies or myopathies has not yet been thoroughly elucidated.

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Other possible causes of motility disorders include intrauterine ischemic insults, exposure to amniotic fluid,4 delayed maturation of either the enteric nervous system or the interstitial cells of Cajal,5 and disorder of the mitochondrial electron transport chain enzymes.6 Inflammation within the myenteric ganglia may cause severe progressive neuropathic CIP in conjunction with autoimmune disease and circulating antienteric neuronal antibodies.7 Mitochondrial myopathies are known to be associated with a variety of clinical syndromes including CIP.8 Patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) have GI dysmotility, peripheral neuropathy, and ophthalmoparesis, and muscle biopsy shows histological features of mitochondrial myopathy.1,9,10

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While treatment of motility disorders has improved significantly over the last two decades, treatment options remain relatively limited. Motility disorders produce nutritional and electrolyte deficiencies, chronic and recurrent vomiting, fecal incontinence, chronic and recurrent pain or discomfort, reduced independence in daily life, and reduced mobility. A wide range of clinical skills is often required to optimally treat these patients. Centers specializing in this care generally establish a multidisciplinary team approach. As part of the treatment, psychological and social support efforts are extremely necessary to achieve optimal outcomes.

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Given the number of pediatric GMDs and the space limitations of this chapter, only the following six motility disorders will be reviewed:

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  • esophageal achalasia (EA);
  • motility disorders following repair of congenital intestinal atresias;
  • motility disorders associated with gastroschisis;
  • CIP;
  • motility disorders following intestinal transplantation;
  • Hirschsprung’s disease.

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Definition and Epidemiology

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EA is a primary esophageal motility disorder characterized ...

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