Sunday, March 15, 2015
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
- Duodenoduodenostomy
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