Gastroenterology and Hepatology Board Review: Pearls of Wisdom, Third Edition

Section III STOMACH

CHAPTER 14. Gastric Congenital and Structural Abnormalities

Ashok Shah, MD and Rene Rivera, MD

Image What diagnosis should be considered in a newborn with postprandial nonbilious vomiting and an abdominal x-ray showing a distended stomach with an absence of air in the bowel?

Gastric atresia. This condition most commonly affects the antrum and pylorus. Other symptoms include respiratory distress and drooling. The treatment is surgical.

Image What condition during pregnancy is associated with gastric atresia?

Polyhydramnios is common in gastric atresia, microgastria, and gastric teratoma.

Image Gastric atresia can be associated with which of the following conditions?

a. Down’s syndrome.

b. Junctional epidermolysis bullosa (JEB).

c. All of the above.

d. None of the above.

c—Down’s syndrome and JEB are both associated with gastric atresia. Other associated anomalies include absent gallbladder, vaginal atresia, tracheoesophageal fistula, atrial septal defect, and gut malrotation.

Image True/False: The histology of the remnant stomach in microgastria is normal.

True. This condition is extremely rare and is usually associated with congenital cardiac abnormalities. Most patients die within weeks to months of birth.

Image True/False: Congenital hypertrophic pyloric stenosis may first become manifest as an adult.

True. However, most cases of adult hypertrophic pyloric stenosis are acquired and occur most often in the setting of chronic peptic ulcer disease or cancer.

Image True/False: The therapy of adult and infantile hypertrophic pyloric stenosis is the same.

False. In neonates, the procedure of choice is a surgical pyloromyotomy. In adults, surgical resection of the pylorus is generally performed in order to rule out a small focus of cancer within the hypertrophied muscle.

Image What classic physical finding may be found in infantile hypertrophic pyloric stenosis?

“Palpable olive” or “palpable pyloric mass” plus visible peristalsis is found in 70%–90% of affected children.

Image Which study has supplanted contrast radiography as the diagnostic study of choice for infantile hypertrophic pyloric stenosis?

Abdominal ultrasonography of the pylorus.

Image True/False: Gastric duplication occurs more commonly in women than in men.

True. Duplication cysts are rare anomalies that arise during early embryonic development. Sixty-five percent of gastric duplications occur in women. When symptomatic, most (80%) become symptomatic in infancy or childhood.

Image True/False: Duplication cysts most commonly involve the stomach.

False. Duplication cysts most commonly involve the small bowel but may also involve the stomach, esophagus, and colon. There are two types of duplication cysts: one that lies adjacent to the bowel and does not communicate with the lumen and another that does communicate with the lumen.

Image True/False: Gastric duplications are associated with gastric carcinoma.

True. Malignant transformation has been documented in gastric duplications. Consequently, surgical excision has been recommended. Nevertheless, as most are asymptomatic and discovered incidentally, an expectant approach is often taken.

Image Where are gastric diverticula most commonly located?

Over 75% are located on the posterior wall within 2 cm of the gastroesophageal junction.

Image Surgical treatment is recommended for distal gastric diverticula because of:

a. High risk of bleeding.

b. High risk of perforation.

c. Associated risk of gastric obstruction.

d. Associated risk of malignancy.

d. Surgical treatment by amputation, invagination, or segmental resection has been recommended.

Image True/False: Most gastric diverticula are acquired and symptomatic.

False. Most are thought to be congenital, are asymptomatic, and are discovered incidentally.

Image What congenital gastric tumor contains all three embryonic germ layers?

Gastric teratoma. These tumors are rarely found in the stomach, occur almost exclusively in males, and are usually found extragastrically, near the greater curvature of the stomach.

Image True/False: Gastric teratomas are usually associated with other congenital abnormalities.

False. In most cases, gastric teratomas are an isolated finding. The prognosis of these tumors is good. Surgical excision is the treatment of choice.

• • • SUGGESTED READINGS • • •

Semrin MG, Russo MA. “Chapter 47-Anatomy, Histology, Embryology, and Developmental Anomalies of the Stomach and Duodenum.” Sleisenger and Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 9th ed. Philadelphia, PA: Saunders; 2006:780-785.

Wyllie R. “Chapter 326 – Pyloric Stenosis and Congenital Anomalies of the Stomach. Kliegman: Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders; 2007:1555-1558.