Etiologies

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  • Hirschsprungs -- very long segment Hirschsprung's disease (lack of neurons to drive peristalsis) in the large and small intestine.
  • Jejunal Atresia -- When child is born with portions of the small bowel missing starting at the Jejunum. The missing segment can include some or most of the the large bowel also. This syndrome is usually caused by events in the early stages of the embryo's development (eight to ten weeks). Either the bowel can twist around its self or there can be a blood clot in the superior mesenteric artery. In both instances the blood supply to the bowel is cut off and the bowel dies. Many times when this bowel dies it is then reabsorbed by the body, this explains why the intestines are not there when the child is born. What is typically found at birth is a dead end coming off the stomach that is dilated due to back up of fluids and there is also a dead end coming off the rectum that is very skinny due to lack of use.
  • NEC --Necrotizing Enterocolitis.
  • Midgut Volvulus -- Twisting of the intestine that causes loss of blood flow in the bowel
  • Microvillous Inclusion Disease -- Also known as Congenital Enteropathy, Microvillous (or Microvillus) Atrophy, or Davidson's Disease. The microvilli that give the gut the surface area needed to absorb enough nutrients to thrive are included, or encapsulated, in vacuoles (bubbles) in the tissue just below the surface, instead of presenting ON the surface of the intestines. The gross length of intestine is there, so a barium study will appear normal, but the fractal surface area created by a normal villiated gut is not there, causing the baby to "dump," lose weight, get acidosis (and the commensurate electrolyte imbalance), and generally fail to thrive. Only histological staining of biopsy for the microvilli can diagnose this very rare congenital disease.

Note that it is possible to have meconium ileus without cystic fibrosis, for example, due to immaturity of intestinal ganglion cells. This rare, temporary condition may be initially misdiagnosed as Hirschsprungs or cystic fibrosis.