Pyloric atresia

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Classification according to ICD-10
Q40.9 Congenital malformation of the upper digestive tract, unspecified
ICD-10 online (WHO version 2019)

The pyloric atresia is a very rare congenital malformation with a closure ( atresia ) of the stomach (Magenatresie) at the level of the gastric output ( pylorus ).

The first description comes from the year 1749 by Calder, after A. Şencan.

Spread and cause

The cause and frequency are unknown. Gastric atresia make up less than 1% of all intestinal atresia , the porter's cavity ( antrum pyloricum ) is affected more often than the pylorus itself. An intrauterine inflammation can also be the cause. Autosomal - recessive inheritance was partially described.

In the following syndromes , pyloric atresia can be a key feature:

Classification

The following classification is common:

  • Aplasia
  • Atresia
  • membrane

Clinical manifestations

Clinical criteria are:

diagnosis

The diagnosis results from the clinic, the sonography and / or the X-ray with only evidence of air in the stomach with the remaining abdomen completely evacuated .

Differential diagnosis

A high form of duodenal atresia , hypertrophic pyloric stenosis or pyloric duplication are to be distinguished.

therapy

The treatment consists firstly in a relief of the stomach by a gastric tube , by compensating for alkalosis, and subsequently by excision of end-to-end as gastroduodenostomy or in the present membrane as a pyloroplasty .

literature

  • KT Flynn-O'Brien, S. Rice-Townsend, DJ Ledbetter: Structural Anomalies of the Gastrointestinal Tract in: Avery's Diseases of the Newborn, 10th ed. 2018, pp. 1039-1053.e3
  • SV Parelkar, SP Kapadnis, BV Sanghvi, PB Joshi, D. Mundada, S. Shetty, SN Oak: Pyloric atresia - three cases and review of literature. In: African journal of pediatric surgery: AJPS. Volume 11, number 4, 2014 Oct-Dec, pp. 362-365, doi: 10.4103 / 0189-6725.143178 , PMID 25323191 (review).
  • R. Gupta, V. Soni, P. Mathur, RB Goyal: Congenital pyloric atresia and associated anomalies: a case series. In: Journal of neonatal surgery. Volume 2, number 4, 2013 Oct-Dec, p. 40, PMID 26023460 , PMC 4420289 (free full text).
  • A. Ksia, H. Zitouni, A. Zrig, R. Laamiri, F. Chioukh, E. Ayari, L. Sahnoun, K. Maazoun, I. Krichene, M. Mekki, M. Belghith, A. Nouri: Pyloric atresia : A report of ten patients. In: African journal of pediatric surgery: AJPS. Volume 10, number 2, 2013 Apr-Jun, pp. 192-194, doi: 10.4103 / 0189-6725.115054 , PMID 23860076 (review).
  • A. Weitzel, P. Göbel, D. Roesner: Two cases of pyloric atresia. In: Journal for Pediatric Surgery: organ of the German, Swiss and Austrian Society for Pediatric Surgery = Surgery in infancy and childhood. Volume 39, Number 6, December 1984, pp. 396-397, doi: 10.1055 / s-2008-1044254 , PMID 6524094 .

Individual evidence

  1. ↑ a b c d e f M. Lehner: Congenital gastric atresia . In: Marcel Bettex (Ed.), Max Grob (Ent.), D. Berger (Ed.), N. Genton, M. Stockmann: Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd, revised edition, Thieme, Stuttgart / New York 1982, p. 7.36, ISBN 3-13-338102-4
  2. ↑ A. Şencan, E. Mir, I. Karaca, C. Günşar, A. Şencan, K. Topçu: Pyloric atresia associated with multiple intestinal atresias and pylorocholedochal fistula. In: Journal of pediatric surgery. Volume 37, Number 8, August 2002, pp. 1223-1224, doi: 10.1053 / jpsu.2002.34482 , PMID 12149711 .
  3. ↑ a b c K. Helmke, MA Lassrich: Magen. In: W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. 2nd edition, Springer 1996, Vol. II, p. 452, ISBN 3-540-60224-0 .
  4. ^ JA Bar-Maor, S. Nissan, S. Nevo: Pyloric atresia. A hereditary congenital anomaly with autosomal recessive transmission. In: Journal of Medical Genetics . Volume 9, number 1, March 1972, pp. 70-72, doi: 10.1136 / jmg.9.1.70 , PMID 5025486 , PMC 1469205 (free full text).
  5. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  6. ↑ Multiple intestinal atresia. In: Orphanet (Rare Disease Database).
  7. ^ A. Zankl, D. Brooks, E. Boltshauser et al .: Natural history of twin disruption sequence. In: American Journal of Medical Genetics. 127A, 2004, p. 133, doi: 10.1002 / ajmg.a.20680 .
  8. ↑ Epidermolysis bullosa simplex with pyloric atresia. In: Orphanet (Rare Disease Database).
  9. ↑ junctional epidermolysis bullosa with pyloric atresia. In: Orphanet (Rare Disease Database).

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