Congenital Hypertrophic Pyloric Stenosis

Question 1

Definition

Answer 1

Progressive hypertrophy of circular muscles fibers of the pylorus with subsequent pyloric narrowing due to ganglionic cells immaturity

Question 2

Pathogenesis

Answer 2

Progressive vomiting results in:

• Loss of HCLp metabolic alkalosis
• Loss of potassium and chloride → hypokalemia and hypochloremia
• Loss of water and nutrientsp dehydration and failure to thrive

Question 3

Clinical picture

Answer 3

Incidence

• Males (especially first born) affected than females.
• Positive family history may exist.

Symptoms

1. Vomiting:

• Occurs shortly after feeding.
• Usually starts after the 2 nd – 3 th weeks of life (Rarely before or after)
• Initially, non-projectile then projectile
• Non bile stained.
• Baby is often hungry after vomiting.

2. Constipation; passage of small, infrequent stools

Question 4

Examination

Answer 4

Baby is marasmic & dehydrated

Palpable mass (olive tumor):

• In the right hypochondrium
• Mobile & non tender

Visible peristalsis from the left to the right

Question 5

Barium meal demonstrates:

Answer 5

Elongated narrow pyloric Canal “string sign”

Parallel streaks of barium seen in the narrowed channel “double tract sign”

Bulge of pyloric muscle into antrum “shoulder sign”

Markedly dilated stomach

Question 6

Abdominal ultrasound

Answer 6

Confirms diagnosis with 95% sensitivity Criteria for diagnosis:
Pyloric diameter → > 14 mm
Thickness—->4mm

Question 7

Treatment

Answer 7

1. Surgical: Ramstedt pyloro-myotomy

• Pre-operative p correct electrolytes disturbance and dehydration
• Post-operative p start small feeds p gradually increasing
• Efficiency :100% curative

2. Medical: Not efficient, it includes:

• Antispasmodic before feeds
• Small, thick, frequent feeds
• Keep upright for 1 hour after feeding

Question 8

Lab. Finding

Answer 8

Hypochloremic, hypokalemic metabolic alkalosis