Sporadic Visceral Myopathy: Full Thickness Rectal Biopsy to Clinch the Diagnosis

Chronic intestinal pseudo-obstruction (CIPO) may result fromdysfunctional intestinal nerves or muscles. Primary causesare uncommon and include familial and sporadic visceralmyopathies. Visceral myopathy may be characterized by CIPO,ureterohydronephrosis, and hepatobiliary dilation. The diagnosis ismade by the clinical history and characteristic findings of fibrousreplacement of the muscularis externa, vacuolar degeneration, andcytoplasmic inclusions in myocytes on a full thickness specimen ofaffected viscera. Few case reports highlight sporadic hollow visceralmyopathy (SHVM) in an adult involving the entire GI, biliary, andurinary tract, We present a unique case of abrupt onset of CIPO,biliary and urinary tract dilation in a 35-year-old Mexican woman,initially presumed to have lupus enterocolitis, and after failure torespond to immune modulating therapy, diagnosed by full thicknessrectal biopsy with SHVM, Early full thickness biopsy of affectedviscera in patients presenting with CIPO with associated biliary andurinary tract dilation should be considered. Our case demonstratesthe safety and adequacy of a full thickness rectal biopsy in making adiagnosis of SHVM.