Eosinophilic Myenteric Ganglionitis with Degenerative Leiomyopathy

Abstract

Background: Chronic intestinal pseudo-obstruction (CIPO) is an umbrella term for a range of different conditions characterized by repetitive episodes or continuous symptoms and signs of bowel obstruction, including radiographic evidence of dilated intestines and air-fluid levels, due to impaired propulsion in the absence of an anatomical occluding lesion. It is a diagnostic challenge and can mimic Hirschsprung's disease. Clinical Description: A 10-month-old boy presented with a history of recurrent episodes of constipation since the age of 6.5 months. The first two had resolved with symptomatic treatment. The third had been associated with bilious vomiting and required exploratory laparotomy. He was referred to us when there was no symptomatic improvement. The child underwent extensive workup that included a review of earlier investigations (contrast-enhanced computerized tomography abdomen, barium enema, and sigmoid biopsy) as well as upper gastrointestinal endoscopy, workup for secondary CIPO, esophageal and antroduodenal manometry, genetic studies, for primary CIPO. A laparotomy with concurrent adhesionolysis, appendectomy, gastrostomy, and ileostomy was undertaken, which included full-thickness biopsies at multiple sites. This revealed both degenerative leiomyopathy and eosinophilic myenteric ganglionitis (EMG). Known associations of CIPO, an underactive bladder, and sinus arrhythmias were also detected. Management: The infant was provided with supportive therapy. A trial of steroids was given for the EMG. The child had multiple bad prognostic factors and also protracted multiple nosocomial infections. He succumbed to his illness and complications after 40 days of hospitalization. Conclusion: The combination of EMG and degenerative leiomyopathy has not been reported in CIPO before.