Pediatric Hypertrophic Pyloric Stenosis Clinical Presentation

  • Author: Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Mar 8, 2012
 

History

  • Typical presentation of an infant with hypertrophic pyloric stenosis (HPS) is onset of initially nonbloody, always nonbilious vomiting at 4-8 weeks. Although vomiting may initially be infrequent, over several days it becomes more predictable, occurring at nearly every feeding. Vomiting intensity also increases until pathognomonic projectile vomiting ensues. Slight hematemesis of either bright red flecks or a coffee-ground appearance is sometimes observed.
  • Patients are usually not ill-looking or febrile. The baby in the early stage of the disease remains hungry and sucks vigorously after episodes of vomiting.
  • Prolonged delay in diagnosis can lead to dehydration, poor weight gain, malnutrition, metabolic alterations, and lethargy.
  • Parents often report trying several different baby formulas because they (or their physicians) assume vomiting is due to intolerance.
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Physical

  • Careful physical examination provides a definitive diagnosis for most infants with hypertrophic pyloric stenosis. However, some of the classic signs that would lead to diagnosis may be absent due, in part, to the early diagnosis of hypertrophic pyloric stenosis.
  • An enlarged pylorus, classically described as an "olive," can be palpated in the right upper quadrant or epigastrium of the abdomen in 60-80% of infants.[3] In order to assess the pylorus, the patient must be calm and cooperative. A pacifier or small amount of dextrose water may help. If the stomach is distended, aspiration using a nasogastric tube is necessary. With the infant supine and the examiner on the child's left side, gently palpate the liver edge near the xiphoid process. Then displace the liver superiorly; downward palpation should reveal the pyloric olive just on or to the right of the midline. To be assured of the diagnosis, the physician should be able to roll the pylorus beneath the examining finger. The tumor (mass) is best felt after vomiting or during, or at the end of, feeding. The diagnosis is easily made if the presenting clinical features are typical, with projectile vomiting, visible peristalsis, and a palpable pyloric tumor.
  • When diagnosis is delayed, the infant may develop severe constipation associated with signs of dehydration, malnutrition, lethargy, and shock.
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Causes

Despite numerous hypotheses, the exact etiology of HPS is not fully understood. Genetic, extrinsic and hormonal factors have been implicated. In addition, abnormalities of various components of the pyloric muscle, such as smooth muscle cells, growth factors, extracellular matrix elements, nerve and ganglion cells, neurotransmitters, and interstitial cells of Cajal, have been reported. Recently, genetic studies have identified susceptibility loci and molecular studies have concluded that smooth muscle cells are not properly innervated in this condition.[4]

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Contributor Information and Disclosures
Author

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H  Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, Bushnaq Medical Centre, University of Jordan Faculty of Medicine, Jordan

Hisham Nazer, MB, BCh, FRCP, DCh, DTM&H is a member of the following medical societies: Royal College of Paediatrics and Child Health, Royal College of Physicians, Royal College of Surgeons in Ireland, Royal College of Surgeons of Edinburgh, and Royal Society of Tropical Medicine and Hygiene

Disclosure: Nothing to disclose.

Coauthor(s)

Dena Nazer, MD  Medical Director, Child Protection Center, Children's Hospital of Michigan; Assistant Professor, Wayne State University

Dena Nazer, MD is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, and Helfer Society

Disclosure: Nothing to disclose.

Daniel A Beals, MD  Attending Staff, Cornerstone Pediatric Surgery

Daniel A Beals, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Physicians, American Pediatric Surgical Association, American Society for Bioethics and Humanities, Kentucky Medical Association, Society for Fetal Urology, Society of Critical Care Medicine, and Southeastern Surgical Congress

Disclosure: Nothing to disclose.

Specialty Editor Board

Jeffrey J DuBois, MD  Chief of Children's Surgical Services, Division of Pediatric Surgery, Kaiser Permanente, Women and Children's Center, Roseville Medical Center

Jeffrey J DuBois, MD, is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and California Medical Association

Disclosure: Nothing to disclose.

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.

B UK Li, MD  Professor of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Director, Pediatric Fellowships and Gastroenterology Fellowship, Medical Director, Functional Gastrointestinal Disorders and Cyclic Vomiting Program, Medical College of Wisconsin; Attending Gastroenterologist, Children's Hospital of Wisconsin

B UK Li, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, State University of New York Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

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