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Congenital Pyloric Stenosis
Congenital or Infantile Spasm of Pylorus

Presentation

[…] if the condition was present at the time of inpatient admission. [icd.codes]

USG is the investigation of choice- features present in the pyloric canal can be seen. [medicinembbs.blogspot.com]

This is one of the few cases of HPS present at birth, which was diagnosed and surgically treated early, and we suggest a congenital etiology in previously reported cases of HPS. [ncbi.nlm.nih.gov]

Malrotation can present at any age, including adulthood, but most commonly presents in the first month of life. Intrinsic duodenal obstruction is the result of either complete or partial obstruction. [basicmedicalkey.com]

Typical presentation is at 4-6 weeks of life but can present very earlier or congenitally. Here we present 3 cases of HPS which presented in the first day of life with co-existing malrotation, annular pancreas, and duodenal atresia. [afrjpaedsurg.org]

Entire Body System

  • Weight Loss

    Projectile vomiting (non bile-stained fluid) Weight loss, child becomes emaciated and dehydrated. Hypertrophied pylorus is palpated in the epigastrium (“olive”). It’s mobile, smooth, firm mass with well defined borders. [medicinembbs.blogspot.com]

    Depending on the duration of symptoms, patients may suffer significant weight loss, even falling below birth weight. [mdedge.com]

    The possible complications of Pyloric Stenosis are: Weight loss or problems with appropriate weight gain according to age: The child weighs much less than what is expected for a normal growing child Dehydration, which can be severe enough to cause multiple [dovemed.com]

    Weight problems. Pyloric stenosis can keep a baby from gaining weight, and sometimes can cause weight loss. [mayoclinic.org]

    Other symptoms include constant hunger, dehydration, and failure to gain or weight loss. What are pyloric stenosis care options? Surgery to widen the pylorus (pyloromyotomy) is the treatment of choice is the standard of care. [nicklauschildrens.org]

  • Irritable Infant

    This mass is palpable to an experienced examiner in up to 80% to 90% of cases, but may be difficult to appreciate in the face of a dilated stomach or irritable infant. [basicmedicalkey.com]

Gastrointestinal

  • Vomiting

    Two days later the baby was brought again to the family practitioner with a history that he has been vomiting almost every feed and the vomiting was projectile immediately following each feed. [medindia.net]

    After vomiting, the infant is hungry and wants to feed again. As vomiting continues, a progressive loss of fluid, hydrogen ion, and chloride leads to hypochloremic metabolic alkalosis. [clinicalgate.com]

    The history is usually as follows : An infant, healthy at birth, seems to progress satisfactorily for two, three, or five weeks, and then vomiting comes on. It does not matter what the food is, breast or artificial feeding, the vomiting persists. [chestofbooks.com]

    Abnormal or frequent vomiting, may suggest an incomplete myotomy or a missed perforation. [basicmedicalkey.com]

    Projectile vomiting (non bile-stained fluid) Weight loss, child becomes emaciated and dehydrated. Hypertrophied pylorus is palpated in the epigastrium (“olive”). It’s mobile, smooth, firm mass with well defined borders. [medicinembbs.blogspot.com]

  • Projectile Vomiting

    The features of projectile vomiting, a pyloric olive and visible peristalsis were studied and their clinical and diagnostic significance was compared with that of radiological investigations. [ncbi.nlm.nih.gov]

    Hypertrophic pyloric stenosis should be suspected in all infants in the first several months of life with projectile vomiting. [merckmanuals.com]

    Two days later the baby was brought again to the family practitioner with a history that he has been vomiting almost every feed and the vomiting was projectile immediately following each feed. [medindia.net]

    Projectile vomiting (non bile-stained fluid) Weight loss, child becomes emaciated and dehydrated. Hypertrophied pylorus is palpated in the epigastrium (“olive”). It’s mobile, smooth, firm mass with well defined borders. [medicinembbs.blogspot.com]

    But these babies don't have projectile vomiting. gastroenteritis (inflammation in the digestive tract that can be caused by viral or bacterial infection) also can cause vomiting and dehydration. [kidshealth.org]

  • Persistent Vomiting

    Due to the loss of hydrochloric acid in the gastric contents from persistent vomiting, patients are at risk of electrolyte imbalance, specifically the characteristic hypochloremic metabolic alkalosis. [radiopaedia.org]

    Blood tests will reveal low blood levels of potassium and chloride in association with an increased blood pH and high blood bicarbonate level due to loss of stomach acid (which contains hydrochloric acid ) from persistent vomiting. [en.wikipedia.org]

    […] but do not increase morbidity and actually may decrease the time to hospital discharge Addition of an H2 receptor blocker sometimes can be beneficial Treat persistent vomiting expectantly because it usually resolves within 1-2 days Avoid the temptation [emedicine.medscape.com]

    Operative complications include mucosal perforation, continued postoperative bleeding (very rare), and persistent vomiting due to incomplete pyloromyotomy (rare). [patient.info]

    All patients had classic projectile,and persistent non-bilious vomiting. All the parents denied the familial genetic diseases and there were no use of macrolide antibiotics during pregnancy. [journals.plos.org]

  • Vomiting in Infancy

    Infantile or congenital hypertrophic pyloric stenosis is one of the most common surgical causes of vomiting in infancy. [sonoworld.com]

    Of course, clinically it is important to consider other causes of vomiting in infancy. A degree of pylorospasm is common in infancy and is responsible for some delay in gastric emptying. The pylorus, however, appears sonographically normal. [radiopaedia.org]

  • Gastropathy

    Diffuse esophageal spasm, GERD ) - Esophageal stricture Stomach Peptic (gastric) ulcer - Gastritis ( Atrophic, Ménétrier's disease ) - Gastroenteritis - Dyspepsia - Pyloric stenosis - Achlorhydria - Gastroparesis - Gastroptosis - Portal hypertensive gastropathy [wikidoc.org]

    Increased physiologic and therapeutic prostaglandin levels have been associated previously with reactive gastropathy and gastric outlet obstruction ( 24, 25, 26 ). [nature.com]

Psychiatrical

  • Distractibility

    Using this sign as a guide to completion of the myotomy avoids overvigorous distraction, which can lead to mucosal perforation. [basicmedicalkey.com]

  • Withdrawn

    B: The superficial incision is made using the sheathed arthroscopy knife with the sheath withdrawn. C: The myotomy is created by pulling with the arthroscopy knife blade sheathed. [basicmedicalkey.com]

Workup

[…] antrum After upper GI barium study, irrigating and removing any residual barium from the stomach is advisable to avoid aspiration Endoscopy Reserved for patients with atypical clinical signs when ultrasonography and UGI studies are nondiagnostic See Workup [emedicine.medscape.com]

Serum

  • Hyponatremia

    […] volume deficit → urinary excretion of K and H to preserve Na⁺ ⁺ ⁺ and water • Initial alkalotic urine becomes acidotic- Paradoxical aciduria • Hypochloremic hypokalemic metabolic alkalosis with paradoxical aciduria with secondary respiratory acidosis • Hyponatremia [slideshare.net]

Treatment

Learn about this topic in these articles: symptoms and treatment In atresia and stenosis Pyloric stenosis is a spasmodic narrowing of the opening between the stomach and the duodenum. [britannica.com]

The efficacy of surgical treatment is emphasized. [ncbi.nlm.nih.gov]

Treatment is surgical incision of the hypertrophied pyloric muscle. Click here for Patient Education [merckmanuals.com]

Cases are still being frequently overlooked, the proper treatment is not carried out, and the infants die. [chestofbooks.com]

Read on to know all about this highly discomforting condition and its causes, symptoms, diagnosis and treatment. [primehealthchannel.com]

Prognosis

[…] gastric emptying of fluid into duodenum Complications Hypochloremic metabolic alkalosis DDx Infantile pylorospasm Muscle thickness between 1.5 and 3 mm Variable caliber of antral narrowing Antral peristalsis Delayed gastric emptying Elongation of pylorus Prognosis [learningradiology.com]

Prognosis The prognosis of pyloric stenosis is very good for those that are diagnosed early and treated with surgery. Life expectancy of infants diagnosed with pyloric stenosis is the same as that of the average individual. [encyclopedia.com]

Prognosis Prognosis is excellent unless diagnosis is delayed and prolonged severe dehydration occurs. Mortality is rare after pyloromyotomy. Peters B, Oomen MW, Bakx R, et al ; Advances in infantile hypertrophic pyloric stenosis. [patient.info]

[…] hypertrophic pyloric stenosis; pyloric stenosis Overview Historical Perspective Classification Pathophysiology Causes Differentiating Pyloric stenosis from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications and Prognosis [wikidoc.org]

Etiology

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

Peptic ulcer may be an etiologic factor in some instances; however, the presence of a small ulcer is difficult to demonstrate in the majority of clinical cases. © Lippincott-Raven Publishers. [journals.lww.com]

Its etiology is probably multifactorial as the pylorus seems sensitive to attack at certain times and in certain subjects. [springerplus.springeropen.com]

EPIDEMIOLOGY AND ETIOLOGY • Incidence: 1-2/1000 live births • Epidemiology: more in first born males M:F - 4-5:1 • Etiology: Unknown • Genetic- 11q14-22 and Xq23 • Familial • Gender • Ethnic origin- more in whites • As new technology and concepts have [slideshare.net]

This is one of the few cases of HPS present at birth, which was diagnosed and surgically treated early, and we suggest a congenital etiology in previously reported cases of HPS. [ncbi.nlm.nih.gov]

Epidemiology

EPIDEMIOLOGY AND ETIOLOGY • Incidence: 1-2/1000 live births • Epidemiology: more in first born males M:F - 4-5:1 • Etiology: Unknown • Genetic- 11q14-22 and Xq23 • Familial • Gender • Ethnic origin- more in whites • As new technology and concepts have [slideshare.net]

American Journal of Epidemiology. 176 (1): 24–31. doi : 10.1093/aje/kwr493. ISSN 0002-9262. ^ Webb, A. R.; Lari, J.; Dodge, J. (1983). "Infantile hypertrophic pyloric stenosis in South Glamorgan 1970-79". Arch. Dis. [en.wikipedia.org]

[2] Synonyms and Keywords: Congenital hypertrophic pyloric stenosis; infantile hypertrophic pyloric stenosis; pyloric stenosis Overview Historical Perspective Classification Pathophysiology Causes Differentiating Pyloric stenosis from other Diseases Epidemiology [wikidoc.org]

Schechter R, Torfs CP, Bateson TF (1997) The epidemiology of infantile hypertrophic pyloric stenosis. Paediatr Perinat Epidemiol 11: 407–427. View Article Google Scholar 8. [journals.plos.org]

The epidemiology of infantile hypertrophic pyloric stenosis. Paediatr Perinat Epidemiol 1997; 11 :407–27. 16. Svenningsson A, Svensson T, Akre O, Nordenskjöld A. [nature.com]

Pathophysiology

Pathophysiology Progressive hypertrophy of the circular muscles in the pyloric sphincter. Not present at birth but occurs over 3 to 5 weeks. Clinical Features Common in 1st born male child. [medicinembbs.blogspot.com]

PATHOPHYSIOLOGY • Vomiting → loss of H and Cl →⁺ ⁻ Hypochloremic hypokalemic metabolic alkalosis • Protracted vomiting → ECF volume deficit → urinary excretion of K and H to preserve Na⁺ ⁺ ⁺ and water • Initial alkalotic urine becomes acidotic- Paradoxical [slideshare.net]

M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D. [2] Synonyms and Keywords: Congenital hypertrophic pyloric stenosis; infantile hypertrophic pyloric stenosis; pyloric stenosis Overview Historical Perspective Classification Pathophysiology [wikidoc.org]

Likewise, our understanding of the etiology and pathophysiology of duodenal obstruction and malrotation has been largely stagnant. [basicmedicalkey.com]

Pathophysiology [ edit ] The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the duodenum. [en.wikipedia.org]

Prevention

Last updated May 6, 2018 BruceBlaus Pyloric Stenosis is the thickening of the muscle layer of the pyloric region resulting in the narrowing of the pyloric sphincter, which prevents the food from leaving the stomach. [dovemed.com]

| Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies Case Studies Case#1 v t e Digestive system - Digestive disease - Gastroenterology (primarily K20-K93, 530-579 ) Upper GI tract Esophagus Esophagitis ( Candidal [wikidoc.org]

Prevention It is not known how to prevent pyloric stenosis. Resources Books Behrman, Richard E., et al., Nelson Textbook of Pediatrics. Philadelphia: WB Saunders, 2000. Periodicals Yoshizawa J, et al. [medical-dictionary.thefreedictionary.com]

The muscles in this part of the stomach thicken, narrowing the opening of the pylorus and preventing food from moving from the stomach to the intestine. [cincinnatichildrens.org]

Centers for Disease Control and Prevention Intersex (Medical Encyclopedia) [ Read More ] Stomach Disorders Also called: Gastric disorders Your stomach is an organ between your esophagus and small intestine. It is where digestion of protein begins. [icdlist.com]

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