Abstract

Introduction:

Pediatric intestinal pseudo-obstruction (PIPO) mimics mechanical obstruction in the absence of any lesion occluding the gut and is rarely reported in children.

Aim: 

To understand the etiology, clinical course, diagnostic strategies, and management of patients with pediatric intestinal pseudo-obstruction.

Case Report:

A 2-year 10-month-old child presented with constipation and intermittent gaseous abdominal distension since 6 months of life with recurrent urinary tract infection. CT of the abdomen with oral contrast showed dilated esophagus and over a distended stomach with air-fluid levels within along with fluid-filled mildly prominent small bowel loops are seen without any obstructive lesion with bilateral hydroureter. The genetic report showed mega cystitis microcolon intestinal hypoperistalsis syndrome (MMIHS) with a homozygous mutation on exon 32 in the MYLK gene (c.5264T>C). He had no response to domperidone, erythromycin, octreotide, and pyridostigmine. He responded partially to senna based laxative.

Conclusion: 

The need of the hour is to find out the appropriate treatment for this condition as none of the medications have proved to be effective in the management of this condition.