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Ileal atresia – malformation of the ileum, the lower part of the small intestine; Colon atresia – malformation of the colon; Malformations may also occur along multiple portions of the intestinal tract; for instance a malformation that occurs along or spans the length of the jejunum and the ileum is termed jejunoileal atresia. [5] [3]
The intestinal atresia is of the "apple-peel" type, an uncommon type in which the remaining portion of the intestine is found twisted around its main artery, and this usually affects the jejunum. [2] [3] Often, much of the bowel is missing in this form of atresia. [9] There can sometimes also be intestinal malrotation. [2] [3] [8] [11]
The small intestine forms loops (B2) and slides back into the abdomen (B3) during resolution of the hernia. Meanwhile, the cecum moves from the left to the right side, which represents the additional 180° counterclockwise rotation of the intestine (C, central view). [4] The exact cause of intestinal malrotation is unknown.
The majority of cases are small intestinal atresia, while colonic atresias are uncommon. [14] There have been two main etiologies proposed for intestinal atresia: the first is a lack of re-vacuolization of the solid cord stage of intestinal development, and the second is a late intrauterine mesenteric vascular accident.
Familial multiple intestinal atresia (FMIA) or familial intestinal polyatresia syndrome (FIPA) is an inherited disorder where atresia occurs at multiple locations throughout the small and large intestines. It presents at birth and the prognosis is very poor with almost all those diagnosed with this condition dying with one month.
This is most often required due to Crohn's disease in adults and necrotising enterocolitis in young children. [2] Other causes include damage to the small intestine from other means and being born with an abnormally short intestine. [1] It usually does not develop until less than 2 m (6.6 ft) of the normally 6.1 m (20 ft) small intestine remains.
Volvulus causes severe pain and progressive injury to the intestinal wall, with accumulation of gas and fluid in the portion of the bowel obstructed. [11] Ultimately, this can result in necrosis of the affected intestinal wall, acidosis, and death. This is known as a closed-loop obstruction because there exists an isolated ("closed") loop of bowel.
There is no standard treatment for TTC7A Deficiency at this time. Management of TTC7A deficiency currently entails bowel resection for any atresias, hematopoietic stem cell transplantation to correct the immunodeficiencies and immunosuppression to help alleviate bowel disease and immune disregulation.