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A 17-year-old man presented with episodic abdominal pain, distensionand vomiting. Esophageal manometry showed a reduced lower esophageal pressurewith massive reflux, gastric emptying of liquids was normal and migratingmyoelectric complexes were present on small bowel motility tracing. Full thicknesssurgical biopsies from the upper jejunum, mid-small bowel and ileum showed anormal villus pattern bur a reduction in the number of neurons in the myentericplexus, degeneration and shrinkage of some of the persisting neurons in the myentericplexus and swelling of the accompanying nerve fibres and Schwann cells. Thiswas interpreted to be compatible with intestinal neurogenic pseudo-obstruction. Heinitially responded to glucocorticosteroids with reduction in the frequency and severityof these symptoms, accompanied by weight gain and a 14 cm growth in height.The steroids were stopped after two years, but within weeks his pain and distensionreturned. He then developed symptoms of angioedema, with markedly reduced C1esterase inhibitor activity and reduced C4 Both the symptoms of ,mgioedema andpseudo-obstruction were well controlled with stanozolol. His younger brother laterdeveloped similar symptoms. also had C1 esterase inhibitor deficiency, and also respondedto stanozolol; an older brother had previously had similar symptoms whichwere lost over time. This suggests that C1 esterase inhibitor deficiency may producesymptoms indicative of intestinal obstruction

C-Prime Esterase Inhibitor Deficiency Presenting as Intestinal Pseudo-Obstruction and Angioedema