Extremely rare. The stomach is replaced by a short tubular structure. In accordance with its embryological origin (5th week of intrauterine life), this malformation is generally (92 % of cases) associated with other malformations such as:

-        asplenia (40 %)

-        limb abnormalities (37 %): radial or ulnar malformation, amelia [MIM 156 810]

-        urogenital malformation (35 %)

-        facial dysmorphism (31 %)

-        other digestive abnormalities (3 %): malrotation

-        cardiac anomaly (29 %)

-        intestinal malrotation (27 %)

-        cerebral abnormalities (20 %)

-        diaphragmatic hernia (12 %)

-        esophageal atresia (12 %)

-        llaryngeal cleft.

Treatment: adapted according to the severity of the associated malformations: either feeding by gastro- or jejunostomy, or enlargement gastroplasty (Hunt-Lawrence intervention)

Anesthetic implications: 

according to the associated malformations; risk of denutrition

References : 

-        Laurie DE, Wakeling EL.
Congenital microgastria in association with Pierre-Robin sequence.
Clin Dysmorphol 2008 ; 17 :143-4

-        Hattori K, Bvulani B, Numanoglu A, Cox SG, Millar AJW.
Total esophageal gastric dissociation for the failed antireflux procedure in a child with microgastria.
Eur J Ped Surg Rep 2016 ; 4 :6-9.

-        Ruczynski LIA, Botden SMBI, Daniels-Scharbatke HE, Schurink M, de Blauw I.
Treatment of congenital microgastria.
Eur J Pediatr Surg 2021 ; 31 :129-34.

Updated: July 2021