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Pediatric Pyloric Stenosis

  • Author: Jagvir Singh, MD; Chief Editor: Kirsten A Bechtel, MD  more...
Updated: Jul 27, 2015


Pyloric stenosis, also known as infantile hypertrophic pyloric stenosis (IHPS), is the most common cause of intestinal obstruction in infancy. IHPS occurs secondary to hypertrophy and hyperplasia of the muscular layers of the pylorus, causing a functional gastric outlet obstruction.

In 1717, Blair first reported autopsy findings of pyloric stenosis. Although the description of the signs and symptoms of infantile hypertrophic pyloric stenosis can be found in the 17th century, the clinical picture and pathology were not accurately described until 1887 by the Danish pediatrician, Hirschsprung. Prior to 1912, early successful surgical procedures included gastroenterostomy, pyloroplasty, and forcible dilatation via gastrostomy. In 1912, Ramstedt observed an uneventful recovery in a patient following pyloroplasty, where sutures used in reapproximating the seromuscular layer had disrupted. Following this observation, he began leaving the split muscle layer unsutured in all subsequent repairs. The Ramstedt pyloromyotomy remains the standard procedure for pyloric stenosis today.

According to Pandya and Heiss, current recommendations include ultrasonography for diagnosis, preoperative corrections of electrolytes, and use of minimally invasive treatment techniques.[1]



Marked hypertrophy and hyperplasia of the 2 (circular and longitudinal) muscular layers of the pylorus occurs, leading to narrowing of the gastric antrum. The pyloric canal becomes lengthened, and the whole pylorus becomes thickened. The mucosa is usually edematous and thickened. In advanced cases, the stomach becomes markedly dilated in response to near-complete obstruction.

The causes of infantile hypertrophic pyloric stenosis are multifactorial.[2] Both environmental factors and hereditary factors are believed to be contributory. Possible etiologic factors include deficiency of nitric oxide synthase containing neurons, abnormal myenteric plexus innervation, infantile hypergastrinemia, and exposure to macrolide antibiotics.

A 2013 study found that bottle-feeding may play a role in an increased risk of hypertrophic pyloric stenosis,[3] and another 2014 cohort study assessed that treatment of young infants with macrolide antibiotics was strongly associated with infantile hypertrophic pyloric stenosis (IHPS) and should therefore only be administered if potential treatment benefits outweigh the risk. Maternal use of macrolides during the first 2 weeks after birth was also associated with an increased risk of IHPS.[4]

A study evaluated the association between exposure to oral azithromycin and erythromycin and subsequent development of infantile hypertrophic pyloric stenosis (IHPS). The study concluded that ingestion of oral azithromycin and erythromycin places young infants at increased risk of developing IHPS. The authors further reported that this association is strongest if the exposure occurred in the first 2 weeks of life, although persists to a lesser degree in children between 2 and 6 weeks of age.[5, 6]

Nitric oxide has been demonstrated as a major inhibitory nonadrenergic, noncholinergic neurotransmitter in the GI tract, causing relaxation of smooth muscle of the myenteric plexus upon its release. Impairment of this neuronal nitric oxide synthase (nNOS) synthesis has been implicated in infantile hypertrophic pyloric stenosis, in addition to achalasia, diabetic gastroparesis, and Hirschsprung disease.

A 2013 study reported the possibility that low serum lipids could be a risk factor for IHPS. Further studies are needed to determine the significance of these findings.[7]

Rogers has suggested, that persisting duodenal hyperacidity, secondary due to a high parietal cell mass (PCM) and loss of gastrin control, produces pyloric stenosis from repeated pyloric contraction in response to hyperacidity.[8]

No specific pattern of inheritance exists. It is more common in first-born white males of northern European ancestry and more concordant in monozygotic than dizygotic twins. It also has predominance in children of affected parents (as many as 7%).

A nationwide study of nearly 2 million Danish children born between 1977 and 2008 shows strong evidence for familial aggregation and heritability of pyloric stenosis. Results of the study found a heritability rate of 87% in affected families, lending to the idea that familial aggregation may be explained by shared genes that affect responses to postnatal factors in causing pyloric stenosis.[9]




United States

The incidence of infantile hypertrophic pyloric stenosis is 2-4 per 1000 live births.


Death from infantile hypertrophic pyloric stenosis is rare and unexpected. The reported mortality rate is very low and usually results from delays in diagnosis with eventual dehydration and shock.


Infantile hypertrophic pyloric stenosis is more common in whites than Hispanics, blacks, or Asians. The incidence is 2.4 per 1000 live births in whites, 1.8 in Hispanics, 0.7 in blacks, and 0.6 in Asians. It is also less common amongst children of mixed race parents.


Infantile hypertrophic pyloric stenosis has a male-to-female predominance of 4:1, with 30% of patients with infantile hypertrophic pyloric stenosis being first-born males.


The usual age of presentation is approximately 3 weeks of life (1-18 wk). Approximately 95% of infantile hypertrophic pyloric stenosis cases are diagnosed in those aged 3-12 weeks. Infantile hypertrophic pyloric stenosis is rare in premature infants. In addition, premature infants have a delayed diagnosis secondary to low birth weight and atypical presentation.

Contributor Information and Disclosures

Jagvir Singh, MD Director, Division of Pediatric Emergency Medicine, Lutheran General Hospital of Park Ridge

Jagvir Singh, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.


Richard H Sinert, DO Professor of Emergency Medicine, Clinical Assistant Professor of Medicine, Research Director, State University of New York College of Medicine; Consulting Staff, Vice-Chair in Charge of Research, Department of Emergency Medicine, Kings County Hospital Center

Richard H Sinert, DO is a member of the following medical societies: American College of Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Grace M Young, MD Associate Professor, Department of Pediatrics, University of Maryland Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians

Disclosure: Nothing to disclose.

Chief Editor

Kirsten A Bechtel, MD Associate Professor of Pediatrics, Section of Pediatric Emergency Medicine, Yale University School of Medicine; Co-Director, Injury Free Coalition for Kids, Yale-New Haven Children's Hospital

Kirsten A Bechtel, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Additional Contributors

Garry Wilkes, MBBS, FACEM Director of Clinical Training (Simulation), Fiona Stanley Hospital; Clinical Associate Professor, University of Western Australia; Adjunct Associate Professor, Edith Cowan University, Western Australia

Disclosure: Nothing to disclose.


Dara A Kass, MD Clinical Assistant Instructor, Department of Emergency Medicine, State University of New York Downstate Medical Center, Kings County Hospital

Dara A Kass, MD is a member of the following medical societies: American College of Emergency Physicians, Emergency Medicine Residents Association, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

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Lateral view from an upper GI study demonstrates the double-track sign.
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