Saturday, December 8, 2012

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

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