Pyloric stenosis: Clinical sciences

Last updated: March 25, 2024

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A 4-week-old boy was transferred from an outside hospital to a pediatric tertiary care center yesterday with a suspected diagnosis of pyloric stenosis based on history and physical exam. The prior emergency department physician reported that the patient was initially tachycardic and hypotensive, and his parents stated that he had not voided in at least 12 hours. IV access was obtained, IV fluid resuscitation was initiated, and his hemodynamics began to improve prior to transfer. After transfer to the pediatric hospital, an abdominal ultrasound was obtained and findings confirmed the diagnosis of pyloric stenosis. Today, his temperature is 36.8 °C (98.2 ºF), pulse is 140/min, blood pressure is 70/40 mmHg, and SpO2 is 100% on room air. Laboratory results from this morning are shown below. He has had 2 wet diapers in the last 12 hours, no bowel movements, and no further episodes of emesis. He remains nil per os (NPO) with weight-based maintenance IV fluids running. What is the best next step in management?

Laboratory Value    Result    Reference Range    
Serum Chemistry         
Sodium     136 mEq/L    133-140 mEq/L    
Potassium     4.1 mEq/L    4.1-5.8 mEq/L    
Chloride    95 mEq/L    96-106 mEq/L    
CO2     33 mEq/L    20-26 mEq/L    
Creatinine     0.31 mg/dL    0.1-0.4 mg/dL    
BUN    9 mg/dL    2-19 mg/dL    
Venous Blood Gas         
pH     7.48    7.34-7.44    
PCO2    47 mmHg    40-50 mmHg    
PO2    35 mmHg    25-47 mmHg    
HCO3    38.2 mmol/L    22-26 mmol/L    

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Pyloric stenosis occurs from hypertrophy of the circular and longitudinal muscle fibers of the pylorus, which acts as a muscular valve between the stomach and the duodenum. This most commonly presents between 2 and 6 weeks of age. Pyloric stenosis often leads to complete or near complete gastric outlet obstruction, which can present as forceful vomiting. Excessive vomiting can in turn cause further complications, such as dehydration and metabolic abnormalities like hypokalemic, hypochloremic metabolic alkalosis with paradoxical aciduria. Management of pyloric stenosis includes fluid resuscitation and correction of metabolic derangements, as well as surgical pyloromyotomy, which is considered curative.

Alright, if a patient presents with a chief concern suggesting pyloric stenosis, you should first perform an ABCDE assessment to determine whether your patient is unstable or stable. If the patient is unstable, stabilize the airway, breathing, and circulation. Next, obtain IV access and initiate IV fluids for resuscitation. Most infants will show signs of severe dehydration and severe electrolyte abnormalities that need to be corrected during resuscitation. Finally, make sure to continuously monitor vital signs and keep the patient NPO.

Okay, once you’ve done acute management, your next step is to obtain a focused history and physical exam, along with labs like a CBC and CMP. The history is typically obtained from your patient’s caregivers, who may report episodes of immediate, post-prandial, nonbilious, projectile vomiting, as well as fewer wet diapers, which suggests dehydration.

Here’s a clinical pearl for you! “Projectile” vomiting refers to vomiting so forcefully that stomach contents are launched across a long distance. Be sure to ask caregivers for details when taking the history, because they might describe vomiting as “projectile” when their infant is simply spitting up!

Now, on physical exam, you might find signs of severe dehydration, such as hypotension, tachycardia, dry mucous membranes, a sunken fontanelle, or delayed capillary refill. Additionally, you might feel an olive-like mass that’s palpable in the right upper quadrant of the abdomen. On laboratory analysis, CBC is typically normal, while CMP shows electrolyte abnormalities consistent with a hypokalemic, hypochloremic metabolic alkalosis, as well as unconjugated hyperbilirubinemia in some cases. These findings together should make you suspect pyloric stenosis in the setting of severe hypovolemia. Your next step is to get an abdominal ultrasound to confirm your diagnosis.

Okay, findings on ultrasound that suggest a hypertrophic pylorus include pyloric wall thickening of 3 mm or greater, or a “target sign,” which indicates concentric hypertrophy. If these findings are found on ultrasound, you can diagnose pyloric stenosis.

Now that you have made the diagnosis, you will proceed with management. For patients that present in an unstable fashion, they will require continued fluid resuscitation and electrolyte management, as well as emergent consultation of your surgery team for pyloromyotomy.

Sources

  1. "Pyloric Stenosis: Point of Care Quick References" Pediatric Care Online (2022)
  2. "Current management of pyloric stenosis" Seminars in Pediatric Surgery (2022)
  3. "A history of the surgical correction of pyloric stenosis" Journal of Pediatric Surgery (2021)
  4. "Contemporary management of pyloric stenosis" Seminars in Pediatric Surgery (2016)