Microgastria-limb reduction defect (MLRD) is a rare disorder of unknown etiology. Most of the cases of congenital microgastria reported in the literature are associated with other multiple congenital anomalies like; limb-reduction defects, asplenia, intestinal malrotation, hepatic symmetry, cardiopulmonary anomalies, central nervous system and renal anomalies, and laryngo-tracheo-bronchial clefts. Isolated congenital microgastria is an extremely rare condition. The microgastria-limb reduction association is believed to arise as a result of abnormal mesodermal development in the fourth or fifth week of embryonic life. All cases of microgastria associated with limb reduction defect have been sporadic with equal male: female distribution.
In the absence of other associated life-threatening congenital anomalies, microgastria can be managed successfully with early gastric augmentation leading to toleration of increasing amounts of oral feeding.
There is no evidence that demonstrate Mendelian inheritance in MLRD patients. The occurrence of twinning in three out of 13 cases suggested that the origin of the microgastria-limb reduction complex may be related to the process of twinning itself.