Chronic idiopathic intestinal pseudo-obstruction with malabsorption, a scleroderma-like disorder

Area of Science:

• Gastroenterology
• Internal Medicine
• Pathology

Background:

• Intestinal pseudo-obstruction presents as a functional blockage without a physical obstruction.
• It can lead to significant gastrointestinal symptoms like pain, vomiting, and malabsorption.
• Distinguishing the underlying cause is crucial for effective management.

Purpose of the Study:

• To present a case of intestinal pseudo-obstruction in a 76-year-old male.
• To investigate the underlying pathophysiology of the patient's symptoms.
• To explore potential causes, including smooth muscle dysfunction, neurological deficits, and fibrotic changes.

Main Methods:

• Clinical presentation review including history of vomiting, abdominal pain, and diarrhea.
• Diagnostic investigations including esophageal and small intestine motility studies.
• Autonomic nervous system testing and post-mortem histopathological examination of the bowel.

Main Results:

• The patient exhibited aperistalsis of the lower esophagus and an atonic small intestine.
• Marked malabsorption was attributed to bacterial overgrowth secondary to hypomotility.
• Autonomic testing was normal, and autopsy showed minimal bowel fibrosis and intact neural components.

Conclusions:

• The findings suggest smooth muscle dysfunction as the primary cause of intestinal pseudo-obstruction in this case.
• The condition mimicked scleroderma but lacked significant fibrosis or neural abnormalities.
• This case underscores the importance of considering primary smooth muscle disorders in pseudo-obstruction.