Colon atresia

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Classification according to ICD-10
Q42.9 Congenital absence, atresia and stenosis of the large intestine, part unspecified
ICD-10 online (WHO version 2019)

The Kolonatresie (Dickdarmatresie) is a very rare congenital malformation with (in sections) closure of the large intestine, so that neither gas nor meconium can be deducted that the newborn to distended abdomen leads. It is the rarest form of intestinal atresia .

The first description comes from the year 1673 by Jean Nicolas Binninger., Quoted from.

distribution

The frequency is stated to be around 1 in 20,000. Colon atresia make up about 4% of all intestinal atresia.

Familial forms are also rare.

In almost half of those affected, there is an association with other malformations such as gastroschisis , other intestinal atresia (up to 20%), Hirschsprung's disease or skeletal malformations.

root cause

Disturbances of the mesenteric vessels (multiple ischemic infarcts) towards the end of pregnancy or a malformation of the gastrointestinal tract and a defect in the area of fibroblast growth factor 10 (FGF10) are discussed as the cause.

Classification

The common classification is described under Intestinal Atresia # Classification .

distribution

According to M. Davenport, the atresia can be found in the following sections:

  • Ascending colon 28%
  • Flexura coli dextra 3%
  • Transverse colon 23%
  • Flexura coli sinistra 25%
  • Descending colon and sigmoid colon 20%

Clinical manifestations

Clinical criteria are:

diagnosis

In the X-ray image (abdomen overview) numerous air-filled intestinal loops, also with mirror images, can be shown. In the colonic enema , the microcolon below and the occlusion by the atresia can be seen.

therapy

Treatment is performed surgically by resecting the atresia and creating an anastomosis . There are various approaches and techniques for doing this. If the atresia is recognized and treated several days after birth, the mortality increases significantly.

Healing prospects

The prognosis is very good with early treatment if there are no further malformations or complications.

In animals

Colon atresia can also occur in animals, especially Holstein cattle .

literature

  • S. Kim, S. Yedlin, O. Idowu: Colonic atresia in monozygotic twins. In: American journal of medical genetics. Volume 91, Number 3, March 2000, pp. 204-206, PMID 10756344 .
  • H. Saha, D. Ghosh, T. Ghosh, S. Burman, K. Saha: Demographic Study and Management of Colonic Atresia: Single-Center Experience with Review of Literature. In: Journal of Indian Association of Pediatric Surgeons. Volume 23, number 4, 2018 Oct-Dec, pp. 206-211, doi : 10.4103 / jiaps.JIAPS_219_17 , PMID 30443116 , PMC 6182946 (free full text).
  • F. Canavese, S. Cavallaro, G. Freni, T. Bardini: Atresia of the Colon. In: European Journal of Pediatric Surgery. 33, 1981, p. 316, doi: 10.1055 / s-2008-1063138

Individual evidence

  1. ↑ Colon atresia. In: Orphanet (Rare Disease Database).
  2. ↑ JN Binninger: Observationum et Curationum Medianalium, Centuriae Quinque, observ. 81 , p. 22 (Montbelgardi: Hyppianis, 1763)
  3. ↑ John G. Raffensperger, MD (8 March 2012). Children's Surgery: A Worldwide History. McFarland. , 2012, p. 114. ISBN 978-0-7864-9048-6
  4. ↑ a b c medscape
  5. ↑ R. Benawra, BL Puppala, HH Mangurten, C. Booth, A. Bassuk: Familial occurrence of congenital colonic atresia. In: The Journal of pediatrics. Volume 99, Number 3, September 1981, pp. 435-436, doi: 10.1016 / s0022-3476 (81) 80340-x , PMID 7264804 .
  6. ^ DW Wilmore: Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. In: The Journal of pediatrics. Volume 80, Number 1, January 1972, pp. 88-95, doi: 10.1016 / s0022-3476 (72) 80459-1 , PMID 4552656 (review).
  7. ↑ a b c d 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
  8. ↑ TJ Fairbanks, RC Kanard, PM Del Moral, FG Sala, SP De Langhe, CA Lopez, JM Veltmaat, D. Warburton, KD Anderson, S. Bellusci, RC Burns: Colonic atresia without mesenteric vascular occlusion. The role of the fibroblast growth factor 10 signaling pathway. In: Journal of pediatric surgery. Volume 40, Number 2, February 2005, pp. 390-396, doi: 10.1016 / j.jpedsurg.2004.10.023 , PMID 15750935 .
  9. ^ M. Davenport, A. Bianchi, CM Doig, DC Gough: Colonic atresia: current results of treatment. In: Journal of the Royal College of Surgeons of Edinburgh. Volume 35, Number 1, February 1990, pp. 25-28, PMID 2342005 .
  10. ↑ W. Schuster, D. Färber: Pediatric radiology: imaging diagnostics. 2nd edition 1996, Springer, ISBN 3-540-15944-4
  11. ^ LK Dalla Vecchia, JL Grosfeld, KW West, FJ Rescorla, LR Scherer, SA Engum: Intestinal atresia and stenosis: a 25-year experience with 277 cases. In: Archives of surgery. Volume 133, Number 5, May 1998, pp. 490-496, doi: 10.1001 / archsurg.133.5.490 , PMID 9605910 (review).
  12. ↑ M. Syed, RD Shanks: Atresia coli inherited in Holstein cattle. In: Journal of dairy science. Volume 75, Number 4, April 1992, pp. 1105-1111, doi: 10.3168 / jds. 0022-0302 (92) 77855-2 , PMID 1578023 .

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