CONGENITAL HYPERTROPHIC PYLORIC STENOSIS

• Hypertrophy of the musculature of pyloric antrum, especially the circular muscle fibres, causing failure of pylorus to relax. 
• Duodenum is normal.

Pathophysiology

• Progressive hypertrophy of the circular muscles in the pyloric sphincter.
• Not present at birth but occurs over 3 to 5 weeks.

Clinical Features

• Common in 1st born male child.
• Projectile vomiting (non bile-stained fluid)
• Weight loss, child becomes emaciated and dehydrated.
• Hypertrophied pylorus is palpated in the epigastrium (“olive”). It’s mobile, smooth, firm mass with  well defined borders.
• Visible gastric hyperperistalsis.

Visible gastric hyperperistalsis

Diagnosis

•      Diagnosis can be made with test feed .
•      USG is the investigation of choice- features present in the pyloric canal can be seen.

D/D

•      GERD
•      Feeding problems
•      Raised ICP
•      Duodenal atresia
•      Intestinal obstruction

T/T

At first metabolic abnormalities should be corrected (i.e. dehydration with low Na+,Cl, K+ and metabolic alkalosis) with i.v dextrose, NS and K+.

Surgery

• Ramstedt’s operation:
• Laparotomy is done
• Hypertrophied muscle is cut along the whole length until the mucosa bulges out. Mucosa should not be opened.

Duodenal Atresia

• Atresia is distal to entry of the common bile duct (occurs at the point of fusion between the foregut and midgut)
• Association with Down syndrome

• History of maternal polyhydramnios (cannot reabsorb amniotic fluid)
• Vomiting of bile-stained fluid at birth

     "Double bubble sign": air in stomach and air in proximal duodenum

•      Antenatal diagnosis can be made-USG

T/T

•      Duodenoduodenostomy