Strange and Schafermeyer's Pediatric Emergency Medicine, Fourth Edition (Strange, Pediatric Emergency Medicine), 4th Ed.

CHAPTER 46. Pyloric Stenosis

Ronald I. Paul

HIGH-YIELD FACTS

• Pyloric stenosis presents most commonly at 5 to 6 weeks of age.

• Progressive nonbilious projectile vomiting is the most common presenting symptom.

• The typical electrolyte imbalance is hypochloremic metabolic alkalosis.

• Diagnosis is confirmed by ultrasound, and rarely by a fluoroscopic upper GI contrast study.

• Treatment is surgical pyloromyotomy.

Pyloric stenosis, also known as hypertrophic pyloric stenosis, is a condition affecting young infants in which the pylorus muscle becomes abnormally thickened, causing obstruction to gastric emptying. It is most often seen in the first few weeks of life. The most common age at presentation is between 5 and 6 weeks with a range of 2 to 31 weeks.1,2 Although premature infants may present later than term babies (6 weeks compared with 5 weeks), their presentation is actually earlier when measured from postmenstrual age (40 weeks vs. 45 weeks).2 Its incidence has been reported to be 3 per 1000 live births in the Western World. Lower incidence ranges have been reported for Asian children, with reports between 0.3 to 0.5 per 1000 infants in Taiwanese infants.3,4 A recent decline in pyloric stenosis has been reported both in the United States and several European countries concurrent with a declining incidence of sudden infant death syndrome, leading to some belief that recommended supine sleeping position may be responsible for both declines.5 A genetic predisposition has been noted with an increased incidence of siblings and a strong increased incidence in twins, both monozygotic and dizygotic.6,7 It is unclear if the increased incidence in twins is due to genetic or environmental causes. Genetic tests have identified a number of potential loci and further studies are ongoing.8,9 There is nearly a four-to fivefold male predominance and an increased incidence in premature infants.2 The highest incidence reported is in first-born infants, although this may be due to more families having only one child than two or three children.9,10 Environmental and mechanical factors have been implicated as potential causes. Infants that were breastfed exclusively have a much lower risk of developing pyloric stenosis compared with infants that have been bottle-fed or bottle-fed and breastfed.11 Very early exposure to erythromycin has been associated with a nearly eightfold increased risk of developing pyloric stenosis.12,13 Erythromycin is a motilin agonist, and thus it produces strong gastric and pyloric contractions. Infants exposed to erythromycin through breast milk may also be at risk for developing pyloric stenosis, but the data are not as conclusive.14 It is unclear if other macrolide antibiotics like azithromycin are also associated with increased risk, although there are case reports where infants treated with azithromycin have developed pyloric stenosis.15

SIGNS AND SYMPTOMS

The key to diagnosing pyloric stenosis remains a comprehensive history and physical examination, with confirmation by ultrasound. The diagnosis should be considered in any young infant with a history of repeated vomiting. The mean duration of symptoms prior to diagnosis is 8 days; however, it can range from 1 day to 13 weeks.1,2 A classic triad of projectile vomiting, palpable olive, and visible peristalsis on feeding has been described with pyloric stenosis.1,2 Projectile vomiting typically occurring immediately after feeding is reported in nearly all cases. Infants will not have anorexia and are often described as hungry. They generally appear well, depending on the level of hydration. It ranges from normal to severe dehydration depending upon the duration of symptoms. A palpable olive in the right upper quadrant used to be present in the majority of cases in earlier decades, but is uncommon now, present in only 13% in a recent study, likely due to earlier diagnosis.1 Peristaltic gastric waves may be seen in infants with advanced disease and dehydration but has recently been reported in only 25% of cases.2,16 Although the majority of infants have nonbilious vomiting, a small percentage of infants present with bilious emesis and hematemesis.17

DIAGNOSTIC EVALUATION

Electrolyte and blood gas measurements may show classic imbalances including hypochloremia and metabolic alkalosis. Similar to the disappearing palpable olive, recent studies demonstrate that laboratory abnormalities are less common than in the past, with hypochloremia now present in 23% and alkalosis present in 21% of patients with pyloric stenosis.1 Patients are generally depleted of total potassium as well, although the serum potassium level is usually normal.18

Diagnosis of pyloric stenosis can be confirmed with either ultrasound or a fluoroscopic upper GI contrast study.19 Ultrasound is the diagnostic imaging procedure of choice. There is a range in the literature for criteria for diagnosis by ultrasound, which includes a pylorus muscle thickness of 3 to 4 mm or higher, and a pyloric length of 15 to 19 mm or higher2022 (Fig. 46-1). Additional criteria include no visualized flow of gastric content through a dilated pylorus. Muscle thickness and length have shown correlation with age and weight. Patients that are smaller and younger may have a clinical diagnosis and not meet minimal diagnostic criteria for ultrasound diagnosis.23 Point-of-care ultrasound by emergency physicians and surgeons have been reported and have shown success in confirming the diagnosis.2426 Because the development of pyloric stenosis is a progressive process, an initial negative ultrasound may need to be repeated later if symptoms persist.27

image

FIGURE 46-1. Ultrasound image of thickened pylorus muscle with length measured at 15 mm.

Although no longer the standard method for diagnosing pyloric stenosis in most centers, upper GI contrast fluoroscopy is still used by some without access to skilled ultrasonographers, or when the study is done to evaluate for other diagnoses. The diagnosis is confirmed by observing contrast propelled through a narrowed and obstructed pyloric channel.19 Two classic findings with an upper GI contrast fluoroscopy have been described including a string sign caused by the hypertrophic pylorus muscle and extension of the pylorus muscle into the stomach resembling an appearance of shoulders18,19 (Fig. 46-2). To accurately see the contrast leaving the stomach, the infant should have nothing to eat for several hours prior to the study or have gastric contents removed by an orogastric tube. Upper GI contrast fluoroscopy is considered accurate, albeit more invasive than an ultrasound with the potential for vomiting and aspiration. It involves protracted fluoroscopy time and therefore more radiation than an ultrasound. An Upper GI series can provide additional information if other diagnoses are being considered such as gastroesophageal reflux, duodenal web, or malrotation.

image

FIGURE 46-2. Upper GI image of thickened pylorus muscle demonstrating “string sign.”

MANAGEMENT

Initial management includes correction of fluid and electrolyte abnormalities if present, and referral to a pediatric surgeon. Surgical repair, pyloromyotomy,18 was first developed by Conrad Ramstedt in 1911 and remains the treatment of choice.28 Recently, laparoscopic pyloromyotomy has been instituted at some institutions and may provide clinical benefits including reduced time for initiation of feeding.29 With either procedure, infant feeding is usually begun within several hours.30

REFERENCES

1. Glatstein M, Carbell G, Boddu SK, Bernardini A, Scolnik D. The changing clinical presentation of hypertrophic pyloric stenosis: the experience of a large, tertiary care pediatric hospital. Clin Pediatr.2011;50:192.

2. Gotley LM, Blanch A, Kimble R, Frawley K, Acworth JP. Pyloric stenosis: a retrospective study of an Australian population. Emerg Med Austalas. 2009;21:401.

3. Leong MM, Chen SC, Hseieh CD, et al. Epidemiological features of infantile hypertrophic stenosis in Taiwanese children a nation-wide analysis of cases during 1997-2007. PLoS One. 2011;6:e19404.

4. Tiao MM, Tsai SS, Kuo HW, Yang CY. Epidemiological features of infantile hypertrophic pyloric stenosis in Taiwan; a national study 1996–2004. J Gastroenterol Hepatol. 2011;26:78.

5. de Laffolie J, Turial S, Heckmann M, Zimmer KP, Schier F. Decline in infantile hypertrophic pyloric stenosis in Germany in 2000-2008. Pediatrics. 2012;129:e901.

6. Krogh C, Fischer TK, Skotte L, et al. Familial aggregation and heritability of pyloric stenosis. JAMA. 2010;303:2393.

7. Yang G, Brisseau G, Uanchar NL. Infantile hypertrophic pyloric stenosis: an association in twins? Paediatr Child Health. 2008;13:383.

8. Panteli C. New insights into the pathogenesis of infantile pyloric stenosis. Pediatr Surg Int. 2009;25:1043.

9. MacMahon B. The continuing enigma of pyloric stenosis of infancy: a review. Epidemiology. 2006;17:195.

10. Delprat GD, Pflueger O. Pyloric stenosis not a disease of “first-born”. Calif Med. 1948;68:76.

11. Kroch C, Biggar RJ, Fischer TK, Lindholm M, Wohlfahrt J, Melbye M. Bottle-feeding and the risk of pyloric stenosis. Pediatrics. 2012;130:e943.

12. Cooper WO, Griffin MR, Arbogast P, Hickson GB, Gautam S, Ray WA. Very early exposure to erythromycin and infantile hypertrophic pyloric stenosis. Arch Pediatr Adolesc Med. 2002;156:647.

13. Mahreshwai N. Are young infants treated with erythromycin at risk for developing hypertrophic pyloric stenosis? Arch Dis Child. 2007;92:271.

14. Mahon BE, Rosenman MB, Kleiman MB. Maternal and infant use of erythromycin and other macrolide antibiotics as risk factors for infantile hypertrophic pyloric stenosis. J Pediatr. 2001;139:380.

15. Morrison W. Infantile hypertrophic pyloric stenosis in infants treated with azithromycin. Pediatr Infect Dis J. 2007;26:186.

16. Taylor ND, Cass DT, Holland AJ. Infantile hypertrophic pyloric stenosis: has anything changed? J Paediatr Child Health. 2012;2:1.

17. Piroutek MJ, Brown L, Thorp AW. Bilious vomiting does not rule out infantile hypertrophic pyloric stenosis. Clin Pediatr. 2012;51:214.

18. Pandya S, Heiss K. Pyloric stenosis in pediatric surgery; an evidence-based review. Surg Clin N Am. 2012;92:527.

19. Hernanz-Schulman M. Pyloric stenosis: role of imaging. Pediatr Radiol. 2009;39:134.

20. Iqbal CW, Rivard DC, Mortellaro VE, Sharp SW, St Peter SD. Evaluation of ultrasonographic parameters in the diagnosis or pyloric stenosis relative to patient age and size. J Pediatr Surg. 2012;47:1542.

21. Rohrschneider WK, Mittnacht H, Darge K, Träger J. Pyloric muscle in asymptomatic infants: sonographic evaluation and discrimination from idiopathic hypertrophic pyloric stenosis. Pediatr Radiol.1998;28:429.

22. Mollitt DL, Golladay ES, Williamson S, Seibert JJ, Sutterfield SL. Ultrasonography in the diagnosis of pyloric stenosis. South Med J. 1987;80:47.

23. Said M, Shaul DB, Radner G, Radner G, Sydorak RM, Applebaum H. Ultrasound measurements in hypertrophic pyloric stenosis: don’t let the numbers fool you. Perm J. 2012;16:25.

24. Malcom GE, Raio CC, Del Rios M, Blaivas M, Tsung JW. Feasibility of emergency physician diagnosis of hypertrophic pyloric stenosis using point-of-care ultrasound: a multi-center case series. J Emerg Med. 2009;37:283.

25. McVay MR, Copeland DR, McMahon LE, et al. Surgeon-performed ultrasound for diagnosis of pyloric stenosis is accurate, reproducible, and clinically valuable. J Pediatr Surg. 2009;44:169.

26. Sivitz AB, Tejani C, Cohen SG. Evaluation of hypertrophic pyloric stenosis by pediatric emergency physician sonography. Acad Emerg Med. 2013;20:646.

27. Keckler SJ, Ostlie DJ, Holcomb GW, St Peter SD. The progressive development of pyloric stenosis: a role for repeat ultrasound. Eur J Pediatr Surg. 2008;18:168.

28. Georgoula C, Gardiner M. Pyloric stenosis a 100 years after Ramstedt. Arch Dis Child. 2012;97:741.

29. Carrington EV, Hall NJ, Pacilli M, et al. Cost-effectiveness of laparoscopic versus open pyloromyotomy. J Surg Res. 2012;178:315.

30. Juang D, Adibe OO, Laituri CA, et al. Distribution of feeding styles after pyloromyotomy among pediatric surgical training programs in North America. Eur J Pediatr Surg. 2012;22:409.