Pyloric Stenosis 26/12/10 FANZCA Part II Notes CK Notes OHOA page 796 Pyloric Stenosis is a medial emergency that requires urgent fluid resuscitation and resolution of biochemical abnormalities. Definitive surgical treatment can then be undertaken to restore enteral nutrition. CLINICAL FEATURES - commoner in first born males 80% males 10% are premature projectile vomiting in neonate (not billous) 2-8 weeks dehydration weight loss hyperaldosteronism with paradoxical aciduria visible peristalsis and olive sized mass in epigastrium confirmed on US associated pathology: cleft palate, GORD PATHOPHYSIOLOGY AND BIOCHEMISTRY - develops: 1. 2. 3. 4. 5. 6. hypochloraemia metabolic alkalosis hyponatraemia hypokalaemia initially, alkaline urine -> later, acidic urine dehydration HYPOCHLORAEMIA - loss of chloride in vomitus METABOLIC ALKALOSIS - loss of H+ in vomitus - decreased secretion of pancreatic HCO3- increased HCO3- presented to distal tubule and eliminated producing an alkaline urine HYPONATRAEMIA - loss of Na+ in vomitus - decreased absorption of Na+ Jeremy Fernando (2010) - loss of Na+ in urine until kidney adjusts to increased HCO3- load - activation of rennin-AG-ALD system to off set this and restore Na+ and H2O HYPOKALAEMIA - K+ loss in vomitus - activation of rennin-AG-ALD system with produces loss of K+ in urine - with extreme K+ loss in urine -> it gets reabsorbed in distal tubule with loss of H+ worsening metabolic alkalosis and producing and acidic urine DEHYDRATION - in ability to absorb enteral fluid and vomiting - activation of rennin-AG-ALD system + ADH PARADOXICAL ACIDURIA - in order to prevent hypokalaemia MANAGEMENT Fluid resuscitation Fluid resuscitation determined by weight and degree of dehydration assessed clinically (tissue turgor, pulse, fontanelle, CR centrally, peripheral perfusion, respiratory rate) - IV boluses of normal saline or colloid (4% albumin) – 10-20mL/kg to restore circulating volume - maintenance @ 4mL/kg/hr with 5% dextrose with 0.45% normal saline and 20mmoL KCl - fluid therapy should be titrated to clinical variable including urine output (2mL/kg/hr) - need a lot of K+ once they pee Laboratory criteria by which patient is sufficiently resuscitated for surgery - ideally biochemical abnormalities would be normal before surgery however, variable associated with adequate resuscitation and resolution of metabolic alkalosis include: - serum Cl- of at least 105mmol/L serum HCO3- (normal) urinary Cl- of >20mmol/L urinary K+ urinary Na+ Intraoperative - operation = splitting of the pylorus muscle longitudinally down to the mucosa (myomectomy) - risk of pulmonary aspiration from gastric outflow obstruction - aspirate N/G and don’t remove as will help to decompress stomach from vigourous ventilation Jeremy Fernando (2010) - RSI or use of NDNMBD fentanyl 1mcg/kg paracetamol suppository 30-40mg/kg bupivacaine infiltration extubate awake and in left lateral position Postoperative - remove N/G feed within 6 hours give maintenance IVF until feeding established use apnoea alarm overnight Jeremy Fernando (2010)