Congenital microgastria was first described in 1894 (Dide 1894) and is extremely rare with less than 50 cases reported to date (Sharma and Menon 2005). It is thought to be the result of an early gastric development arrest from the foregut between the 4th and

5th week of gestation; there is no fetal rotation and the greater and lesser curvatures fail to develop. The spleen arises in the dorsal mesogastrium by mesenchymal differentiation at around the same time, which may explain the frequent association of microgastria with asplenia or polysplenia.

Complete lack of gastric development or agas-tria is the most extreme form of microgastria (Schlesinger and Parker 2003).

Presenting symptoms of microgastria are recurrent postprandial vomiting, respiratory distress/ infection, failure to thrive and malnutrition. These are all consequences of the small stomach and the associated gastroesophageal reflux (Hoehner et al. 1994; Waasdorp et al. 2003).

The diagnosis is made by an upper GI study which will demonstrate an abnormally small tubular or saccular stomach in the midline (Kroes and FesteN 1998) (Fig. 3.4).

Megaesophagus is almost always associated with microgastria, which may be secondary to the gas-troesophageal reflux or caused by the esophagus taking over the reservoir capacity of the stomach (Mouiton et al. 1994).

There is a very high incidence of other associated anomalies with esophageal stenosis, such as a left-sided diaphragmatic hernia (Sharma and Menon

2005), right-sided diaphragmatic hernia (Velasco et al. 1990), growth hormone deficiency and diabetes insipidus (Herniaz Driever et al. 1997), intestinal malrotation, cardiopulmonary anomalies, central nervous system anomalies (hydrocephalus, arrhin-encephaly, microphthalmia) (Schlesinger and Parker 2003), renal anomalies (Kroes and FesteN 1998), laryngo-tracheo-bronchial clefts (Ryan et al. 1991; Samuel et al. 1997), limb reduction defects (Cunniff et al. 1993), Pierre Robin sequence and partial trismus (Giurgea et al. 2000). Isolated congenital microgastria is extremely rare.

The treatment of choice in patients with microgastria, if no other life-threatening anomalies are present, is gastric augmentation (Menon et al. 2003), classically a Hunt-Lawrence pouch (a double-lumen jejunal pouch anastomosed to the greater curvature of the stomach with a distal Roux-en-Y jejunos-tomy). Prolonged medical management of gastroesophageal reflux in these patients is not beneficial, since the stomach size will not increase significantly over time (Blank and Chisholm 1973). Operating at an early stage is indicated to allow normal growth and development. Life expectancy is determined mostly by the associated anomalies, being the most frequent cause of death (Mouiton et al. 1994).

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