Congenital Anomalies

Congenital anomalies of the stomach are infrequent. Depending on the degree of obstruction that occurs, they may present in the neonatal period or later.

Gastric atresia refers to the arrest of development of part of the stomach. It usually occurs in the antrum or the pylorus as a fibrous cord remnant or as a membrane with mucosal and submucosal layers. At birth, children with gastric atresia will have signs of gastric outlet obstruction. Treatment of gastric atresia requires surgical excision of membranes or joining the stomach and duodenum (gastroduodenostomy).

Microgastria refers to a small, tubular stomach associated with a megaesophagus histologically normal. It may occur in association with other anomalies. Microgastria results in decreased gastric acid secretion that hampers iron absorption and causes iron deficiency. Decreased production of intrinsic factor may also result in vitamin B12 deficiency. Frequent small-volume feeding or continuous-drip feeding is used as conservative management. Surgical intervention can enable normal growth and development.

Infantile hypertrophic pyloric stenosis is caused by hypertrophy of the circular muscle layer around the pyloric channel, which results in gastric outlet obstruction. The incidence in the US is approximately 3 per 1000 live births, but it can vary among ethnic groups (highest among whites, then black Americans, then Africans, and lowest among Asians). Pyloric stenosis is the most common reason for abdominal surgery in the first 6 months of life. This usually presents as progressive projectile vomiting after feeding, and is often apparent at the 3rd or 4th week of age. The vomitus is never stained with bile but can contain signs of blood. Anorexia, dehydration, and wasting may occur with time. Owing to chronic vomiting, a metabolic alkalosis develops. Two classic physical signs in pyloric stenosis are the palpable pyloric mass (olive) and visible peristaltic waves. The typical clinical presentation may be enough for diagnosis; however, in a few infants, contrast radiography or ultrasonography may be required. Initial treatment is to correct fluid and electrolyte imbalance but definitive treatment is surgery involving a longitudinal incision in the hypertrophied pylorus. Prognosis after surgery is excellent and the infant grows and develops normally.

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