++
Congenital microgastria is a rare anomaly that is characterized
by failure of the primitive foregut to differentiate into a stomach
during weeks 4 to 8 of gestation, thus resulting in a small tubular
stomach usually associated with megaesophagus and incomplete gastric
rotation (Fig. 400-1). Isolated
microgastria is uncommon,1 with associated anomalies
such as asplenia, situs inversus, limb hypoplasia, intestinal malrotation, laryngotracheal
clefts, and cardiac abnormalities being common.2,3 Only
about 60 cases have been described.
++
++
Clinical Management
and Outcome
++
Typically, patients present with recurrent vomiting, failure
to thrive, and recurrent respiratory infections due to the reduced
gastric volume and associated megaesophagus. Patients will often
have persistent diarrhea due to dumping syndrome resulting from the
lack of gastric capacity. Diagnosis of microgastria is by upper GI
contrast studies that demonstrate the typical findings of a very small
stomach. When identified, screening for associated anomalies is
warranted.
++
The approach to management of microgastria is controversial. Case
reports describe successes and failures of medical and surgical management
approaches.4,5 Medical management was associated
with poor growth, delay in cognitive development, and dumping syndrome,
so that surgical therapy was recommended. However, improved technologies that
allow routine use of gastric or jejunal slow-drip feedings has been
reported to have successful outcomes.6 This approach
prevents gastroesophageal reflux and allows appropriate nutrition
for growth. Proton-pump inhibitor therapy may be beneficial since
it can reduce gastric fluid volumes. Small oral feedings may be
administered to allow appropriate development of feeding skills.
Parenteral supplementation should not be required if jejunal feeding
can be maintained. In children where drip feeds are tolerated gastric volume
may increase significantly over the first years of life, and the patient
can eventually be converted to bolus or ad lib feeds.
++
If medical management is unsuccessful, some sort of surgical
therapy can be attempted. There are only a few case reports with
acceptable outcomes following gastric augmentation procedures combined
with some form of fundoplication. The most common type of augmentation
performed is the Hunt-Lawrence pouch, which involves the creation
of a Roux-en-Y double-row jejunal reservoir.1 Early
follow-up results suggest improved gastric capacity and minimal
reflux with this technique, leading to an increase in oral intake
and improved growth. Long-term follow-up reports are limited, with
only 2 patients that are described in the literature that demonstrate
normal social and cognitive function.
++
Neonatal gastric perforation usually occurs during the first
week of life, caused by trauma, ischemia, or rarely, as a spontaneous event.7,8 The
overall reported incidence is 1 in 2900 births, and it is more common
in males. Traumatic perforations usually are attributed to gastric
intubation or gastric overdistention ...