Gastric atresia
Gastric atresia is a rare congenital disorder characterized by a complete or partial obstruction of the stomach's lumen, preventing normal passage of stomach contents into the duodenum. This condition is a form of intestinal atresia, where a portion of the intestine is absent or closed. Gastric atresia is classified into three types based on the nature and location of the obstruction: Type I involves a membrane or web obstructing the gastric outlet, Type II is characterized by a fibrous cord between two blind-ending segments of the stomach, and Type III involves a complete separation of the proximal and distal ends of the stomach, often with a gap between them.
Etiology
The exact cause of gastric atresia remains unknown, but it is believed to result from a combination of genetic and environmental factors. Some theories suggest that it occurs due to a disruption in the normal development of the stomach during fetal growth, possibly due to vascular accidents or genetic mutations.
Symptoms
Infants with gastric atresia typically present with symptoms shortly after birth, including vomiting (which may be green due to the presence of bile), abdominal distension, and an inability to tolerate oral feeding. If not promptly diagnosed and treated, the condition can lead to severe dehydration, electrolyte imbalances, and failure to thrive.
Diagnosis
Diagnosis of gastric atresia often involves a combination of prenatal ultrasound findings and postnatal imaging studies. Prenatal ultrasounds may show polyhydramnios (excess amniotic fluid) due to the baby's inability to swallow and digest amniotic fluid properly. After birth, an X-ray of the abdomen typically shows a distended stomach with no gas beyond the obstruction. A contrast study, such as an upper gastrointestinal (GI) series, can help confirm the diagnosis by delineating the location and extent of the atresia.
Treatment
The primary treatment for gastric atresia is surgical intervention to remove the obstruction and restore continuity to the gastrointestinal tract. The specific type of surgery depends on the nature of the atresia. In cases where there is a simple membrane or web (Type I), a gastrotomy may be performed to excise the obstructing membrane. For more complex atresias (Types II and III), more extensive surgical reconstruction of the stomach may be necessary. Postoperative care is crucial and includes nutritional support, management of electrolyte imbalances, and monitoring for complications.
Prognosis
With timely surgical intervention and appropriate postoperative care, the prognosis for infants with gastric atresia is generally good. Most children are able to achieve normal stomach function and lead healthy lives. However, long-term outcomes can vary depending on the presence of associated anomalies, the type of atresia, and the occurrence of surgical complications.
Epidemiology
Gastric atresia is a rare condition, with an estimated incidence of 1 in 100,000 live births. It can occur in isolation or in association with other congenital anomalies, including those involving the heart, kidneys, and skeletal system.
NIH genetic and rare disease info
Gastric atresia is a rare disease.
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Rare diseases - Gastric atresia
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Contributors: Prab R. Tumpati, MD