Hypertrophy of musculature of pyloric antrum , especially the circular muscle fibres Failure of pylorus to relax. Gastric outlet obstruction Duodenum is normal. Increased risk if newborn gets erythromycin or azithromycin in first 14 days after birth. INFANTILE HYPERTROPHIC PYLORIC STENOSIS
It is postulated that abnormal innervation of the muscular layer leads to failure of relaxation of the pyloric muscle, increased synthesis of growth factors, and subsequent hypertrophy, hyperplasia, and obstruction. Erythromycin has a prokinetic effect on gastric muscle contraction. ETIOLOGY
Common in first born males(4:1) Incidence: 4 in 1000 live births Familial link found. Seen between 3 rd and 6 th weeks of age , time taken by the hypertrophied muscle to cause complete obstruction CLINICAL FEATURES
Vomiting – forcible, projectile and non-bilious, may occur after every feed or only after some feed. Despite stomach distension, affected infants seem to have an insatiable appetite and may cry inconsolably. Constipation Dehydration Loss of weight SYMPTOMS
Visible gastric peristalsis Palpable lump of hypertrophied pylorus , better felt from left side, as a mobile, smooth, firm, olive like mass , with all borders made out, moves with respiration, with impaired resonance on percussion. Electrolyte imbalance( hyponatremic , hypokalemic , metabolic alkalosis with paradoxic aciduria ) SIGNS
VGP and mass is better seen and felt Vomiting is regurgitant Anorexia common In premature infants,
Biochemical assessment(pH>7.45, Cl - 3mEq/L) X ray Abdomen USG- doughnut sign, pylorus > 4 mm thickness,(normal<2mm) pyloric canal > 14 mm,(normal<10mm) cervix sign on long axis, target sign on short axis. INVESTIGATIONS
Barium meal, not usually done. If done, shows string sign/railroad track sign/double track sign with pyloric obstruction.
Correction of dehydration and electrolyte imbalance. Intravenous fluid therapy is begun with 0.45–0.9% saline, in 5–10% dextrose, with the addition of potassium chloride in concentrations of 30– 50mEq/L until the infant is rehydrated and the serum bicarbonate concentration is less than 30mEq/ dL , which implies that the alkalosis has been corrected. Most infants can be rehydrated within 24 hours TREATMENT
Surgery – Ramstedt’s operation- After laparotomy, hypertrophied muscle is cut out along the whole length adequately until mucosa bulges out. Muscosa not opened. Laparoscopic pyloromyotomy Oral feeds reintroduced in 8-12 hrs , post-op vomiting resolves in 24 hrs.
Postoperative pyrexia Gastroenteritis Electrolyte imbalance COMPLICATIONS OF SURGERY