Microgastria

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Classification according to ICD-10
Q40.2 Other specified congenital malformations of the stomach - micro gastric disease
ICD-10 online (WHO version 2019)

The Microgastria from ancient Greek γαστήρ gaster , German , stomach ' and ancient Greek μικρός Mikros , German , small, cramped' , is a very rare dysplasia due to a disturbed embryonic development with the result of a (substantially) to small stomach . Other malformations are almost always present.

A complete absence is called agastrie .

The first description of a Microgastria dates back to 1894 by the Parisian pathologist Maurice Dide (born June 3, 1873 in Paris, † March 26, 1944 Buchenwald concentration camp ), the clinical and radiological findings were in 1956 by the founder of the Pediatric Radiology John Caffey described .

The definition as a syndrome was made in 1971 by Reinhard D. Schulz and F. Niemann.

distribution

The frequency is given as less than 1 in 1,000,000.

A microgastria can be an essential feature of syndromes such as B. at:

root cause

The cause of this embryonic development disorder is unknown. The stomach arises together with the throat , larynx , esophagus and duodenum as a spindle-shaped extension of the foregut , from which the trachea and lungs also develop. Therefore, malformations of other organs are associated with micro gastric disease - albeit to a different extent. Due to the lack of or incomplete rotation of the foregut, there is often a malrotation , heterotaxia , also situs ambiguus , asplenia or an Ivemark symptom complex .

Clinical manifestations

Clinical criteria are:

  • Manifestation in infancy, in the newborn period, prenatal
  • Vomiting from birth because the stomach is too small and can hardly store and digest food.

diagnosis

The diagnosis is made by imaging sonography during the prenatal diagnosis as well as after the birth by means of X-ray diagnosis :

The gastric bladder is missing , the stomach is hardly or not at all in the gastrointestinal passage with X-ray contrast media. Often there is cardia insufficiency, the lower part of the esophagus is enlarged as a spare reservoir.

therapy

The treatment is surgically z. B. by Hunt-Lawrence-Pouch or by gastric augmentation .

literature

  • C. Filisetti, L. Maestri, M. Meroni, F. Marinoni, G. Riccipetitoni: Severe Dumping Syndrome in a 6-year-old Girl with Congenital Microgastria Treated by Hunt-Lawrence Pouch. In: European journal of pediatric surgery reports. Volume 5, number 1, January 2017, pp. E17 – e20, doi: 10.1055 / s-0037-1601305 , PMID 28361011 , PMC 5371510 (free full text).
  • P. Vasas, SS Mudan, CA Akle: Congenital microgastria with limb defect combined with megaduodenum: case report and review of literature. In: The Indian journal of surgery. Volume 73, Number 2, April 2011, pp. 122-124, doi: 10.1007 / s12262-010-0185-2 , PMID 22468061 , PMC 3077154 (free full text).
  • RC Cohen: Congenital microgastria - management with a Hunt-Lawrence jejunal reservoir pouch. In: Pediatric Surgery International. Volume 6, 1991, doi: 10.1007 / BF00176429 .
  • O. Hochberger, W. Swoboda: Congenital microgastria. In: Pediatric Radiology. Volume 2, 1974, p. 207, doi: 10.1007 / BF00972735 .

Individual evidence

  1. ↑ a b c microgastria, congenital. In: Orphanet (Rare Disease Database).
  2. ^ Maurice Dide: Sur en estomac d'adulte a type foetal. In: Bulletins de la Société anatomique de Paris. Volume 69, 1894, pp. 669-670 ( Gallica ).
  3. ^ John Caffey: Pediatric X-ray diagnostic. A textbook for students and practitioners of pediatrics, surgery and radiology. 3rd edition, 1956, pp. 486-487.
  4. ↑ Reinhard D. Schulz, F. Niemann: Kongenitale Mikrogastrie in connection with skeletal malformations — a new syndrome. In: Helvetica paediatrica acta. Volume 26, Number 2, June 1971, pp. 185-191, PMID 5139246 .
  5. ↑ a b P. Hernáiz Driever, G. Göhlich-councilor, R. King, K. Heller, H. Schmidt, RP tree, HJ Böhles: Congenital microgastria, growth hormone deficiency and diabetes insipidus. In: European Journal of Pediatrics. Volume 156, Number 1, January 1997, pp. 37-40, doi: 10.1007 / s004310050549 , PMID 9007489 .
  6. ↑ Micro gastric disease - shortening of the extremities. In: Orphanet (Rare Disease Database).
  7. ↑ J. Roberts, W. Torres-Martinez, E. Farrow, A. Stevens, P. Delk, KE White, DD Weaver: A case of Robin sequence, microgastria, radiohumeral synostosis, femoral deficiency, and other unusual findings: a newly recognized syndrome? In: American journal of medical genetics. Part A. Volume 164A, number 2, February 2014, pp. 287–290, doi: 10.1002 / ajmg.a.36273 , PMID 24311538 .
  8. ↑ a b c W. Schuster, D. Färber (Ed.): Children's radiology. Imaging diagnostics. 2nd edition, Springer 1996, ISBN 3-540-60224-0 .
  9. ^ P. Shepherd, N. Smeulders, AH Coleman, LS Chitty: Congenital microgastria. A rare cause of failure to visualize the fetal stomach. In: Prenatal diagnosis. Volume 31, Number 10, October 2011, pp. 1010-1012, doi: 10.1002 / pd.2821 , PMID 21744366 .
  10. ^ CJ Hunt: Construction of food pouch from segment of jejunum as substitute for stomach in total gastrectomy. In: AMA archives of surgery. Volume 64, number 5, May 1952, pp. 601-608, doi: 10.1001 / archsurg.1952.01260010619009 , PMID 14923080 .
  11. ^ W. Lawrence: Reservoir construction after total gastrectomy: an instructive case. In: Annals of surgery. Volume 155, February 1962, pp. 191-198, doi: 10.1097 / 00000658-196200000-00004 , PMID 14463011 , PMC 1466039 (free full text).
  12. ^ BJ Dicken, NM Novotny, FD Breckler, JP Yim, FJ Rescorla: Use of the Hunt-Lawrence pouch in congenital microgastria - a report of 2 cases. In: Journal of pediatric surgery. Volume 45, Number 11, November 2010, pp. 2238-2240, doi: 10.1016 / j.jpedsurg.2010.07.001 , PMID 21034951 .
  13. ^ VS Jones, RC Cohen: An eighteen year follow-up after surgery for congenital microgastria - case report and review of literature. In: Journal of pediatric surgery. Volume 42, Number 11, November 2007, pp. 1957–1960, doi: 10.1016 / j.jpedsurg.2007.07.054 , PMID 18022457 (Review)
  14. ↑ P. Menon, KL Rao, HP Cutinha, BR Thapa, B. Nagi: Gastric augmentation in isolated congenital microgastria. In: Journal of pediatric surgery. Volume 38, Number 10, October 2003, pp. E4-E6, doi: 10.1016 / s0022-3468 (03) 00518-9 , PMID 14577093 .

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