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Pyloric Stenosis

BASICS

  • Definition: Acquired narrowing of the pyloric canal due to progressive hypertrophy of the pyloric muscle, leading to gastric outflow obstruction.

EPIDEMIOLOGY

  • Age: Presents most commonly between 2–8 weeks (median 6 weeks)
  • Most common surgical condition in the first year of life
  • Incidence: 1.56 per 1,000 live births; higher in first-born males, Caucasians, and Hispanic children

ETIOLOGY & PATHOPHYSIOLOGY

  • Impaired relaxation β†’ muscle hypertrophy β†’ outflow obstruction
  • Gastric distension β†’ projectile, postprandial, nonbilious vomiting

RISK FACTORS

  • Maternal smoking
  • C-section delivery
  • Prematurity
  • Formula feeding
  • Postnatal macrolide antibiotics (esp. in first 2 weeks)
  • Multiple gestation (esp. monozygotic twins)

PREVENTION

  • Breast milk (contains vasoactive intestinal peptides) may help prevent pylorospasm

COMMON ASSOCIATED CONDITIONS

  • Hiatal and inguinal hernias

DIAGNOSIS

History

  • Nonbilious projectile vomiting after feeding, increasing in frequency/severity
  • Hunger, irritability, weight loss
  • Median symptom duration: 10 days

Physical Exam

  • Firm "olive-like" mass in right upper quadrant
  • Visible peristalsis after feeding
  • Late: dehydration (dry mucosa, poor turgor), tachycardia, irritability
  • Rare: jaundice (elevated indirect bilirubin from starvation)

Differential Diagnosis

  • Inappropriate feeding
  • GERD
  • Gastritis
  • Adrenal crisis
  • Antral/gastric web

Diagnostics

  • Labs: Hypokalemic, hypochloremic, metabolic alkalosis
  • Pyloric ultrasound (study of choice):
  • Muscle thickness β‰₯3 mm, length β‰₯15 mm, diameter β‰₯11 mm, volume β‰₯12 mL
  • Key factor: muscle thickness
  • Upper GI series: Rarely needed; "string sign" or "double-tract sign" if performed

TREATMENT

General Measures

  • Medical emergency, not surgical emergency
  • Restore hydration and correct electrolytes pre-op (esp. alkalosis)

Definitive Treatment

  • Surgical pyloromyotomy (Ramstedt procedure): Curative
  • Laparoscopic approach: less pain, shorter stay, faster recovery, fewer complications

Medical Therapy (Alternative)

  • Atropine (for those who cannot undergo surgery):
  • Oral: start 0.05 mg/kg/day, up to 0.1 mg/kg/day
  • IV: start 0.1 mg/kg/day, increase until vomiting ceases, then convert to oral at double the IV dose
  • Slower resolution (vomiting usually stops in 7 days; pyloric muscle normalizes in 5–15 months)
  • Lower success rates and longer hospital stay vs. surgery

ADMISSION & INPATIENT CONSIDERATIONS

  • Fluid resuscitation: β‰₯1 20 mL/kg bolus, D5 Β½ NS + 20 mEq KCl, 1.5–2Γ— maintenance fluids
  • Correct electrolytes before anesthesia (alkalosis can reduce inspiratory drive/post-op apnea)

ONGOING CARE

  • Discharge after tolerating 2–3 full feeds
  • Monitor for pain, emesis, apnea
  • If persistent emesis after 1–2 weeks: upper GI to rule out incomplete pyloromyotomy or duodenal leak

DIET

  • Ad-lib feeding after pyloromyotomy is recommended (decreases length of stay)
  • Early feeding = more emesis, but does not affect LOS

PATIENT EDUCATION

  • Caregivers: Signs of dehydration, infection, expected postoperative emesis

PROGNOSIS

  • Surgery is curative

COMPLICATIONS

  • Dehydration, failure to thrive, jaundice
  • Chronic abdominal pain, functional GI disorders post-op
  • Incomplete pyloromyotomy, mucosal perforation

ICD-10 Codes

  • Q40.0 Congenital hypertrophic pyloric stenosis

CLINICAL PEARLS

  • Most common surgical condition in 1st year of life
  • Presents 1–5 months, with projectile vomiting and a palpable RUQ mass
  • Abdominal US is diagnostic study of choice
  • Laparoscopic pyloromyotomy is preferred and curative; medical management with atropine is effective for select cases