ACEM Fellowship
Pyloric stenosis

Pyloric stenosis

Introduction

  • Rare in first week of life
  • Typically weeks 2-8 of age
  • Protracted vomiting and hungry
  • Idiopathic diffuse hypertrophy and hyperplasia of circular muscle fibres of pylorus leading to gastric outlet obstruction
  • 2-4/1000 live births in developed countries
  • 4-5x more common in males
  • Family history is a risk factor
  • First-born males are most at risk

Presentation

  • Initially regurgitation and non-projectile vomiting, then gradually vomiting becomes projectile (during or soon after feeding)
  • Can be intermittent or after every feed
  • Hungry++
  • Vomiting is non-bilious but can have blood streaks (in up to 10%)
  • Stool amounts can be very small
  • If late presentation, severely dehydrated, lethargic, malnourished and metabolically deranged hypokalaemic, hypochloraemic metabolic alkalosis
  • Always consider in previously refluxing/posseting baby whose regurgitation changes in character
  • Takes time for clinical and USS findings to develop, so do not rule out if symptoms persist

Examination

  • Gastric distension, visible peristalsis from LUQ to epigastrium and palpable olive in RUQ/midline
    • Need empty stomach and relaxed anterior abdominal wall for palpation
  • Palpation during test feed may reveal olive
  • Need USS to exclude diagnosis at any given point in time
  • Paradoxical aciduria (renal sodium conservation at expense of hydrogen ions)
  • With increasing dehydration, renal potassium losses are amplified in order to retain sodium and water

Imaging

  • USS diagnostic imaging of choice
  • Accuracy near 100%
  • Bull’s eye on cross-section
  • Pylorus muscle thickness >4mm and pyloric channel length >17mm have PPV >90%
    • Lower limits in infants <30 days old

DDx

  • GORD
  • UTI
  • Oesophageal achalasia
  • Hiatus hernia
  • Overfeeding
  • Adrenal insufficiency
    • Frequent vomiting but usually metabolic acidosis and elevated serum potassium and sodium
  • Metabolic disorders
    • Usually other clues i.e. seizures, coma, hypoglycaemia

Management

  • Resuscitation over 24-48 hours with N/saline + 5% dextrose
  • Replace potassium (20mmol/L KCl) and chloride (in N/S)
  • Ramstedt pyloromyotomy once rehydrated, bicarbonate in normal range and chloride >90mmol/L
    • If bicarb not corrected, risk of apnoea/hypoventilation post-operatively

Last Updated on November 20, 2021 by Andrew Crofton