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of or relating to or inside the intestines; "intestinal disease" (同)enteric, enteral
an abnormal condition in which a normal opening or tube in the body (as the urethra) is closed or absent

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/10/21 23:37:40」(JST)

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Intestinal atresia is a malformation where there is a narrowing or absence of a portion of the intestine. This defect can either occur in the small or large intestine.

Types of intestinal atresia

The different types of intestinal atresia are named after their location:

Duodenal atresia - malformation of the duodenum, part of the intestine that empties from the stomach
Jejunal atresia - malformation of the jejunum, the second part of the intestine extending from the duodenum to the ileum
Ileal atresia - malformation of the ileum, the lower part of the small intestine
Colon atresia - malformation of the colon

Duodenal atresia has a strong association with Down syndrome.^[1] It is the most common type, followed by ileal atresia.

An inherited form - familial multiple intestinal atresia - has also been described. This disorder was first reported in 1971.^[2] It is due to a mutation in the gene TTC7A on short arm of chromosome 2 (2p16). It is inherited as an autosomal recessive gene and is usually fatal in infancy.

Cause

The most common cause of non-duodenal intestinal atresia is a vascular accident in utero that leads to decreased intestinal perfusion and ischemia of the respective segment of bowel. This leads to narrowing, or in the most severe cases, complete obliteration of the intestinal lumen.

In the case that the superior mesenteric artery, or another major intestinal artery, is occluded, large segments of bowel can be entirely underdeveloped. Classically, the affected area of bowel assumes a spiral configuration and is described to have an "apple peel" like appearance; this is accompanied by lack of a dorsal mesentery. Ileal atresia can also result as a complication of meconium ileus.

Diagnosis

Intestinal atresias are often discovered before birth: either during a routine sonogram which shows a dilated intestinal segment due to the blockage, or by the development of polyhydramnios (the buildup of too much amniotic fluid in the uterus). These abnormalities are indications that the fetus may have a bowel obstruction which a more detailed ultrasound study can confirm.

Some fetuses with bowel obstruction have abnormal chromosomes. An amniocentesis is recommended because it can determine not only the sex of the baby, but whether or not there is a problem with the chromosomes.

Treatment

Fetal and neonatal intestinal atresia are treated using laparotomy after birth. If the area affected is small, the surgeon may be able to remove the damaged portion and join the intestine back together. In instances where the narrowing is longer, or the area is damaged and cannot be used for period of time, a temporary stoma may be placed.

References

^ Le, Tao (2013). First Aid for the USMLE STEP. New York: Mc Graw Hill. p. 308. ISBN 978-0-07-180232-1.
^ Mishalany HG, Der Kaloustian VM (1971) Familial multiple-level intestinal atresia: report of two siblings. J Pediat 79: 124

External links

UCSF Fetal Treatment Center: Bowel Obstructions
Information about congenital malformations of the gastrointestinal tract in premature babies - The Hospital for Sick Children

UpToDate Contents

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1. 腸管閉鎖症 intestinal atresia
2. 消化管閉鎖症および閉塞の出生前診断 prenatal diagnosis of gastrointestinal atresia and obstruction
3. 新生児における壊死性腸炎の病理および病因 pathology and pathogenesis of necrotizing enterocolitis in newborns
4. 先天性巨大結腸症（ヒルシュスプルング病） congenital aganglionic megacolon hirschsprung disease
5. 悪心および嘔吐を呈する幼児または小児に対するアプローチ approach to the infant or child with nausea and vomiting

English Journal

Familial aggregation of "apple peel" intestinal atresia and cardiac left-sided obstructive lesions: A possible causal relationship with NOTCH1 gene mutations.

Digilio MC, Magliozzi M, Di Pede A, Valfrè L, Dentici ML, Auriti C, Marino B, Novelli A, Dallapiccola B.
American journal of medical genetics. Part A. 2019 May;().
"Apple peel" intestinal atresia is a rare form of small bowel atresia, in which the duodenum or proximal jejunum ends in a blind pouch and the distal small bowel wraps around its vascular supply, in a spiral resembling an apple peel. The etiology of "apple peel" intestinal atresia is presently unkno
PMID 31111652

A neonate with bilious emesis.

Lin ZL, Zhu JH.
BMJ (Clinical research ed.). 2019 May;365()l1351.
PMID 31092396

Alveolar capillary dysplasia with misalignment of the pulmonary veins and hypoplastic left heart sequence caused by an in frame deletion within FOXF1.

Bourque DK, Fonseca IC, Staines A, Teitelbaum R, Axford MM, Jobling R, Chiasson D, Chitayat D.
American journal of medical genetics. Part A. 2019 May;().
Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) is a rare, autosomal dominant disorder of interstitial lung development, leading to pulmonary hypertension, and death in infancy. Associated features include malformations of the heart, gastrointestinal tract, and genitou
PMID 31074124

Japanese Journal

症例報告異所性膵を認めた2箇所の高位空腸膜様閉鎖の1例

日本小児外科学会雑誌 = Journal of the Japanese Society of Pediatric Surgeons 52(5), 1087-1090, 2016-08
NAID 40020936907

異所性膵を認めた2箇所の高位空腸膜様閉鎖の1例

日本小児外科学会雑誌 52(5), 1087-1090, 2016
NAID 130005405210

生体部分肝移植後早期に妊娠出産し得た胆道閉鎖症の1例

日本小児外科学会雑誌 52(5), 1103-1107, 2016
NAID 130005405207

Related Pictures

★リンクテーブル★

リンク元	「腸閉鎖」「腸管閉鎖」
関連記事	「intestinal」「atresia」

「腸閉鎖」

Intestinal atresia
Classification and external resources
Specialty	medical genetics
ICD-10	Q41, Q42
ICD-9-CM	751.1 751.2
OMIM	223400 243600
DiseasesDB	31514 33000