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Pyloric Stenosis

Pyloric Stenosis is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Progressive narrowing of the pyloric canal, occurring in infancy
  • Synonym(s): infantile hypertrophic pyloric stenosis (IHPS)

Epidemiology

  • Predominant age: infancy
    • Onset usually at 3 to 6 weeks of age; rarely in the newborn period or as late as 5 months of age
  • Considered the most common condition requiring surgical intervention in the 1st year of life
  • A recent decline in incidence has been reported in a number of countries.
  • Predominant sex: male > female (4 to 5:1)

Incidence
In Caucasian population, 2 to 5:1,000 babies; less common in African American and Asian populations

Prevalence
National prevalence level is 1 to 2:1,000 infants, ranging from 0.5 to 4.21:1,000 live births.

Etiology and Pathophysiology

  • Abnormal relaxation of the pyloric muscles leads to hypertrophy.
  • Redundant mucosa fills the pyloric canal.
  • Gastric outflow is obstructed, leading to gastric distension and vomiting.
  • The exact cause remains unknown, but multiple genetic and environmental factors have been implicated.
  • Breast versus bottlefeeding—increased vasoactive intestinal peptide in breast milk may mediate pyloric relaxation and increase gastric emptying, whereas bottlefeeding may cause higher serum levels associated with pylorospasm (1)[C].

Genetics
Recent studies have identified linkage to chromosomes 3, 5, 11 and 19.

Risk Factors

  • 5 times increased risk with affected first-degree relative
  • Strong familial aggregation and >80% heritability
  • Multiple gestation—200-fold increased risk if monozygotic twin diagnosed and 20-fold increased risk if dizygotic twin diagnosed
  • Breastfeeding protective versus bottlefeeding where risk is increased
  • Postnatal macrolide use (i.e., erythromycin, azithromycin)—erythromycin agonist of motilin, which might cause continuous contraction of the pyloric muscle (1)[C]
  • A recent surveillance study of a population-based birth defects registry identified association between pyloric stenosis and the use of fluoxetine in the 1st trimester, even after adjustment for maternal age and smoking. The adjusted odds ratio was 9.8 (95% CI 1.5–62) (2)[B].

Commonly Associated Conditions

Associated anomalies present in ~4–7% of infants with pyloric stenosis

  • Hiatal and inguinal hernias (most commonly)
  • Other anomalies include the following:
    • Congenital heart disease
    • Esophageal atresia
    • Tracheoesophageal fistula
    • Renal abnormalities
    • Turner syndrome and trisomy 18
    • Cornelia de Lange syndrome
    • Smith-Lemli-Opitz syndrome
  • A common proposed genetic link between breast cancer, endometriosis, and pyloric stenosis has been observed in families.

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Citation

Stephens, Mark B., et al., editors. "Pyloric Stenosis." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116508/1.1/Pyloric_Stenosis.
Pyloric Stenosis. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116508/1.1/Pyloric_Stenosis. Accessed July 16, 2019.
Pyloric Stenosis. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116508/1.1/Pyloric_Stenosis
Pyloric Stenosis [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 July 16]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116508/1.1/Pyloric_Stenosis.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Pyloric Stenosis ID - 116508 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116508/1.1/Pyloric_Stenosis PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -