What is diffuse juvenile polyposis of infancy?

Updated: Jun 06, 2020
  • Author: Jaime Shalkow, MD, FACS; Chief Editor: Cameron K Tebbi, MD  more...
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Answer

Answer

Diffuse juvenile polyposis of infancy

This entity occurs within the first months of life and is not familial. Patients may present with diarrhea, rectal bleeding, intussusception, prolapse, bowel, protein-losing enteropathy, macrocephaly, clubbing of fingers and toes, and hypotonia. [10]

The entire GI tract is involved. One third of these patients have other congenital abnormalities such as Meckel diverticulum, malrotation, and heart lesions. [7]

Patients require total parenteral nutrition (TPN) and bowel rest, followed by selective resection.

Despite appropriate treatment, this disease is almost universally fatal; only 2 patients have been reported to survive after age 2 years. [10]


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