Eosinophilic myenteric ganglionitis as a cause of chronic intestinal pseudo-obstruction

Abstract

Chronic intestinal pseudo-obstruction (CIPO) represents a rare and severe condition characterized by failure of the intestinal tract to propel its contents normally [1, 2]. The condition presents with clinical features such as abdominal pain, vomiting, distended abdomen, constipation, and diarrhea [1, 2]. These symptoms resemble an intestinal mechanical obstruction in the absence of a demonstrable lesion occluding the gut. It is thought that this can result from disturbance of the intestinal motor function, supplied by the enteric nervous system (ENS). The neurons of the ENS are contained in two groups of ganglia: the myenteric (Auerbach's) and the submucosal (Meissner's) plexuses. The ENS has the unique ability to control most gut functions, such as regulating vascular tone, secretion/absorption, and gut motility [3, 4]. Given these important functions of the ENS, it is not surprising that damage to the ENS results in digestive disorders and reduced quality of life. Human and experimental evidence indicates that inflammation can occur in the ENS, resulting in severe intestinal motor impairment. Inflammation of the ENS has indeed been observed in some cases of CIPO [3, 5, 6]. Many efforts have been made to classify CIPO. Based on histological examination, CIPO may be categorized as primary, secondary, or idiopathic in nature [1]. Primary CIPO can be classified into three major categories of gastrointestinal neuromuscular disorders (GINMD): mesenchymopathies, myopathies, and neuropathies, depending on the predominant involvement of interstitial cells, smooth muscle cells, or enteric neurons, respectively [1, 7]. The enteric neuropathies underlying CIPO can be subdivided into two major entities: (a) degenerative neuropathies, without any evident inflammatory response and (b) inflammatory neuropathies, referred to as myenteric ganglionitis [3, 8]. Inflammation within the myenteric ganglia is a recognized primary cause of CIPO, but the mechanisms through which the dysfunction occurs and the mechanisms leading to enteric neuropathies remain poorly understood [3, 4]. In this case report, we present the first male neonate with eosinophilic myenteric ganglionitis underlying CIPO and report his successful recovery following steroid treatment.