INFANTILE (IDIOPATHIC/BENIGN) HYPERTROPHIC PYLORIC STENOSIS

• Not congenital (symptoms are not present at birth)
• More common in boys (4:1)
• Pylorus thickened, elongated and lumen narrowed due to hypertrophy of circular muscle
• Increased incidence in 0 and B groups
• Increased risk in Turner syndrome and Trisomy 18
• Erythromycin or azithromycin exposure, in the first 2 weeks of life – increased incidence

Clinical features

• The classical presentation is non-bilious, projectile vomiting between 2 – 8 weeks of age
• Persistent vomiting à dehydration, malnutrition, hypokalemic, hypochloraemic alkalosis and paradoxical aciduria
• Visible gastric peristaltic waves moving from left to right across the upper abdomen
• Palpation of the pyloric tumor (olive-shaped) in the epigastrium or right upper quadrant is pathognomic
• X-ray after barium meal shows string sign or double neck sign and shoulder sign

Diagnosis confirmed by USG

• Persistent pyloric muscle thickness more than 3 to 4 mm
• Pyloric canal length more than 15 to 18 mm in the presence of functional gastric outlet obstruction
• Pyloric thickness (serosa to serosa) of 15 mm or greater
• Target sign on transverse images of the pylorus
• Failure of the channel to open during a minimum of 15 minutes of scanning
• Retrograde or hyperperistaltic contractions
• Antral nipple sign (prolapse of redundant mucosa into the antrum, which creates a pseudomass)
• Double-track sign (redundant mucosa in the narrowed lumen, which creates 2 mucosal outlines)
• Ramstedt’s operation (pyloromyotomy) is the treatment of choice. The hypertrophied circular muscle fibres are cut longitudinally without damaging the mucosa

 Exam Important

Don’t Forget to Solve all the previous Year Question asked on INFANTILE (IDIOPATHIC/BENIGN) HYPERTROPHIC PYLORIC STENOSIS