Intestinal atresia

Classification according to ICD-10
Q41.-	Congenital absence (s), atresia and stenosis of the small intestine
Q42.-	Congenital absence, atresia and stenosis of the large intestine
ICD-10 online (WHO version 2019)

The intestinal atresia is a congenital malformation with one or more closures ( atresia ) of the lumen in the gastrointestinal tract , occurs more frequently in the area of ​​the duodenum and the rectum .

The first description comes from the year 1683 by GC Goeller (or written by Goelle), quoted from Marcel Bettex (ed.) And Christina Oetzmann v. Sochaczweski

Classification

Location of intestinal atresia: duodenum, jejunum and ileum.

Depending on the affected section of the intestine, a distinction can be made between:

• Pyloric atresia
• Duodenal atresia
• Small intestinal atresia
  • Jejunal atresia
  • Ileum atresia
  • Small intestine atresia type IIIb , synonyms: apple peel syndrome; Apple peel syndrome ; Jejunal atresia; Jejunoileal atresia; English Apple-peel syndrome; Apple peel small bowel syndrome; APSB
• Colon atresia
• Anal atresia
• Atresia multiplex congenita syndrome with multiple atresia

distribution

The frequency is given as 1 to 4 in 10,000 live births. Chromosome aberration is present in 20% . Other data speak of 1 in 400–5000 newborns.

Intestinal atresia make up about a third of all obstructions in the newborn.

In the context of syndromes

• Atresia multiplex congenita syndrome (Gastrointestinal Defects And Immunodeficiency Syndrome; GIDID)
• Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery
• Epidermolysis bullosa junctionalis with pyloric atresia (JEB-PA)
• Familial visceral myopathy
• Feingold Syndrome
• Martínez-Frías syndrome
• Mitchell-Riley Syndrome
• Umbilical cord ulcer - intestinal atresia
• Stromme syndrome
• prenatal volvulus

root cause

In 1955 Louw and Barnard suspected a disturbance of the mesenteric vessels (multiple ischemic infarcts) towards the end of pregnancy as the cause of the intestinal atresia, after an inadequate recanalization of the gastrointestinal tract had previously been assumed.

In 1988, Puri and Fujimoto were able to prove that the cause is not ischemic but rather a malformation of the gastrointestinal tract.

classification

The following classification is common:

• Type I (duodenal diaphragm) with a membrane that closes the lumen and normal muscle wall
• Type II (cord-shaped duodenal atresia) with a brief interruption by a cord of connective tissue
• Type III (complete duodenal atresia) with complete separation of the two ends of the duodenum

A classification modified by Grosfeld and co-workers is currently used most frequently:

• Type I (membrane)
• Type II (blind ending connected by strand)
• Type IIIa (blind ending without connection)
• Type IIIb (apple-peel deformity) (or apple-peel syndrome)
• Type IV (multiple atresia, string of sausages)

Clinical manifestations

Clinical criteria are:

• Manifestation in the newborn age
• Hydramnios
• Vomiting after childbirth or during the first few days of life
• Depending on the location, also circumscribed distended and / or sunken abdomen
• no meconium loss
• often also hyperbilirubinemia

In 6–14% of unborn babies with intestinal atresia of the upper gastrointestinal tract , umbilical cord ulceration develops ( umbilical cord ulcer - intestinal atresia ).

diagnosis

The diagnosis is based on prenatal diagnosis , sonography , clinical findings, an X-ray of the abdomen overview, administration of contrast medium for gastrointestinal passage and / or colonic enema .

Differential diagnostics

The following are to be distinguished:

• Malrotation with or without volvulus
• Meconium ileus
• Intestinal duplication
• internal hernia
• Aganglionosis
• adynamic ileus

therapy

The treatment is performed surgically .

Healing prospects

The prognosis is good, depending on the extent of the changes. The further distal the atresia the higher the survival rate . The mortality is, when Apple peel syndrome (71%) and if in addition a meconium ileus (65%), an increase in multiple atresia (57%) meconium (50%) or a gastroschisis (66%) are present.

history

The first attempt at an ileostomy was made in 1889 by the English surgeon J. Bland Sutton, the first successful operation by P. Fockens in 1911

literature

• D. Brodsky: Intestinal atresias. In: D. Brodsky, C. Martin: Neonatology Review. 2nd edition 2010.

Individual evidence

1. ↑ Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0 , keyword "intestinal atresia"
2. ↑ ^{a b c} emedicine, medscape
3. ↑ G. Goelle: Abortus humani monstrosi. In: Hist Anatom Misc Acad Nat Curios. Norimbergae, 1683
4. ↑ ^{a b} M. Bettex (ed.), M. Grob (introduction), D. Berger (arrangement), N. Genton, M. Stockmann: Kinderchirurgie. Diagnostics, indication, therapy, prognosis. 2nd, revised edition, Thieme, Stuttgart / New York 1982, ISBN 3-13-338102-4
5. ↑ ^{a b} Chr. Oetzmann v. Sochaczweski: The morphology of the enteric nervous system in small intestinal atresia: studies on an animal model. Dissertation to obtain the degree of Doctor of Medicine at the Medical Faculty of the University of Hamburg, 2011, [1]
6. ↑ ^{a b} S. B. Appel: Intestinal atresia - an overview work. Diploma thesis to obtain the academic degree of Doctor of Medicine (Dr. med.univ.) At the Medical University of Graz, 2017
7. ↑ ^{a b} Small intestine atresia. In: Orphanet (Rare Disease Database).
8. ↑ jejunal atresia.  In: Online Mendelian Inheritance in Man . (English)
9. ↑ Uptodate
10. ↑ PJ Lupo, JL Isenburg, JL Salemi, CT Mai, RF Liberman, MA Canfield, G. Copeland, S. Haight, S. Harpavat, AT Hoyt, CA Moore, WN Nembhard, HN Nguyen, RE Rutkowski, A. Steele, CJ Alverson, EB Stallings, RS Kirby: Population-based birth defects data in the United States, 2010-2014: A focus on gastrointestinal defects. In: Birth defects research. Volume 109, number 18, November 2017, pp. 1504–1514, doi: 10.1002 / bdr2.1145 , PMID 29152924 , PMC 5915361 (free full text).
11. ↑ KE Best, PW Tennant, MC Addor et al .: Epidemiology of small intestinal atresia in Europe: a register-based study. In: Archives of Disease in Childhood. Fetal and neonatal edition. Volume 97, number 5, September 2012, pp. F353-F358, doi: 10.1136 / fetalneonatal-2011-300631 , PMID 22933095 .
12. ^ ^{A b} T. R. Prasad, M. Bajpai: Intestinal atresia. In: Indian journal of pediatrics. Volume 67, Number 9, September 2000, pp. 671-678, doi: 10.1007 / bf02762182 , PMID 11028122 (review).
13. ↑ familial visceral myopathy. In: Orphanet (Rare Disease Database).
14. ↑ Umbilical Cord Ulcers - Intestinal Atresia. In: Orphanet (Rare Disease Database).
15. ^ JH Louw, CN Barnard: Congenital intestinal atresia; observations on its origin. In: The Lancet . Volume 269, Number 6899, November 1955, pp. 1065-1067, doi: 10.1016 / s0140-6736 (55) 92852-x , PMID 13272331 .
16. ^ P. Puri, T. Fujimoto: New observations on the pathogenesis of multiple intestinal atresias. In: Journal of pediatric surgery. Volume 23, Number 3, March 1988, pp. 221-225, doi: 10.1016 / s0022-3468 (88) 80726-7 , PMID 3357137 .
17. ^ JH Louw: Congenital intestinal atresia and severe stenosis in the newborn; a report on 79 consecutive cases. In: South African journal of clinical science. Suid-Afrikaanse tydskrif vir kliniese wetenskap. Volume 3, Number 3, September 1952, pp. 109-129, PMID 12995090 .
18. ↑ JL Grosfeld, TV Ballantine, R. Shoemaker: Operative mangement of intestinal atresia and stenosis based on pathologic findings. In: Journal of pediatric surgery. Volume 14, Number 3, June 1979, pp. 368-375, doi: 10.1016 / s0022-3468 (79) 80502-3 , PMID 480102 .
19. ↑ emedicine.medscape
20. ↑ Umbilical Cord Ulcers - Intestinal Atresia. In: Orphanet (Rare Disease Database).
21. ↑ J Bland Sutton: ileum Imperforate . In: Amer J med Sci Vol. 98, No. 5, 1889, p. 457
22. ↑ P. Fockens: A surgical case of congenital small intestinal atresia. In: Zbl. Chir. Vol. 38, 1911, p. 532

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