Related Experiment Videos
Submucosal hypoganglionosis causing chronic idiopathic intestinal pseudo-obstruction
Georg B T Von Boyen1, Manfred Von der Ohe, Heinz-Jürgen Krammer
1Department of Medicine II (Gastroenterology), University Hospital of Heidelberg at Mannheim, Germany. georg.boyen@medizin.uni-ulm.de
Summary
A patient with chronic idiopathic intestinal pseudoobstruction experienced sepsis and endocarditis after total parenteral nutrition (TPN). Surgical biopsies revealed hypoganglionosis in the submucous plexus, a neurogenic defect.
Area of Science:
- Gastroenterology
- Neuropathology
- Surgical Pathology
Background:
- Chronic idiopathic intestinal pseudoobstruction (CIIPO) is a rare motility disorder.
- Neurogenic causes are suspected in CIIPO, often involving the enteric nervous system.
- Total parenteral nutrition (TPN) can lead to complications like sepsis and endocarditis.
Observation:
- A 39-year-old woman presented with recurrent intestinal obstruction symptoms.
- She developed sepsis and endocarditis during TPN, necessitating its cessation and surgical intervention.
- Surgical biopsies were obtained from the intestinal mucosa and submucosa.
Findings:
- Immunohistochemistry revealed enlarged nerve fiber strands in the submucosa.
- Analysis indicated a paucity of nerve cells within small ganglia, suggesting submucous hypoganglionosis.
- This contrasts with typical visceral neuropathies, which usually affect the myenteric plexus.
Implications:
- This case highlights a rare neurogenic defect in the submucous plexus causing intestinal pseudoobstruction.
- Submucous hypoganglionosis may be an underrecognized cause of CIIPO.
- Understanding plexus involvement is crucial for diagnosing and managing intestinal motility disorders.