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Pyloric Stenosis
Introduction
Pyloric stenosis, also known as infantile hypertrophic pyloric stenosis (IHPS), is an uncommon condition in infants characterized by abnormal thickening of the pylorus muscles in the stomach, leading to gastric outlet obstruction. Clinically, infants are well at birth. Then, at 3 to 6 weeks of age, they present with "projectile" vomiting, potentially leading to dehydration and weight loss.[1] The radiologist plays a central role in diagnosing this condition. The treatment is surgical.
Etiology
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Etiology
The exact etiology of IHPS is unknown. Some studies have shown that young infants treated with macrolide antibiotics had an increased incidence of IHPS. Postnatal exposure to erythromycin has also been associated with an increased risk for the development of pyloric stenosis. Other risk factors include bottle feeding, preterm birth, cesarean section delivery, and first-born infants (30% to 40% of cases). If the mother was a heavy smoker during pregnancy, the risk of hypertrophic pyloric stenosis could increase by 1.5. to 2.0 fold.[1]
Epidemiology
Pyloric stenosis is 2 to 5 in 1000 live births annually. It is more common in males; the male-to-female ratio is 4 to 1. There is a familial link, but the hereditary pattern is polygenic. Pyloric stenosis is more common in the white population. It is less commonly seen in Indian, Asian, and Black populations.[1][2] The incidence is 2.4 per 1000 in whites, 1.8 in Hispanics, 0.7 in blacks, and 0.6 in Asians.
Pathophysiology
The hallmark of pyloric stenosis is marked hypertrophy and hyperplasia of the pylorus's circular and longitudinal muscular layers.[3] This thickening leads to the narrowing of the lumen of the gastric antrum. The pyloric canal becomes lengthened. The muscles of the pylorus become thickened. The mucosa becomes edematous and thickened. When severe, the stomach becomes dilated secondary to gastric outlet obstruction. This obstruction induces immediate postprandial, nonbilious, projectile emesis.
History and Physical
Infants with pyloric stenosis classically present with projectile, non-bilious vomiting. Vomiting may be intermittent or occur after each feeding. Emesis should not be bilious. In about 60% to 80% of the infants with pyloric stenosis, a firm, non-tender, hard pylorus measuring 1 to 2 cm in diameter, classically described as an "olive," may be present in the right upper quadrant. The clinician may also observe reverse peristaltic waves. Infants may show dehydration. Signs of dehydration in infants are depressed fontanelles, dry mucous membranes, decreased tearing, poor skin turgor, and lethargy.[4] The classic electrolyte imbalance of pyloric stenosis is hypochloremic, hypokalemic metabolic alkalosis. The diagnosis of pyloric stenosis is being made earlier because of earlier use of sonography, so those laboratory abnormalities are now present in less than 50% of cases. Dehydration may cause either hypernatremia or hyponatremia; both can lead to prerenal renal failure. Elevated unconjugated bilirubin levels may be present.
Evaluation
Infants with severe vomiting caused by pyloric stenosis can develop profound hypochloremia and hypokalemia. The classic biochemical abnormality in hypertrophic pyloric stenosis is hypochloremic, hypokalemic metabolic alkalosis.[4] Ultrasonography has become the standard imaging technique for diagnosing hypertrophic pyloric stenosis. It is reliable, highly sensitive, highly specific, and easily performed. An experienced ultrasonographer is necessary to diagnose pyloric stenosis. A novice sonographer may have trouble locating the pylorus. A pyloric wall thickness of 3 mm or greater and a pyloric channel length of 15 mm or greater are abnormal and indicate pyloric stenosis. Ultrasound findings also include target signs and lack of gastric emptying. Barium upper can help to diagnose pyloric stenosis when ultrasonography is not diagnostic but is rarely necessary. It should demonstrate an elongated pylorus and can show marked delayed gastric emptying. Findings may include a string sign, double-track sign, or beak sign. Sometimes, upper endoscopy, performed by a pediatric gastroenterologist, can be used as an additional diagnostic tool in rare, select cases when the other imaging tests are nondiagnostic or when the infant presents with atypical clinical features. Abdominal X-ray lacks sensitivity and specificity significantly. Findings may include large stomach gas with the absence of gas in the colon secondary to gastric obstruction. A Caterpillar sign may be present, a radiological finding showing a dilated stomach appearance secondary to hypertrophied pylorus gastric contractions.
Treatment / Management
First, medical treatment is necessary and usually consists of rehydration and correcting electrolyte imbalances. If no or mild signs of dehydration are evident, 5% dextrose with 0.25% NaCl and 2 meq KCl per 100 mL is given. If moderate or severe, higher IVF NaCl concentrations are recommended. Given the impact on potential hypoventilation, bicarbonate levels should be corrected and monitored. NG tube should be considered. Once the infant is rehydrated, surgery is the next step.[5] Treatment is surgical and is called pyloromyotomy. In this surgery, the pyloric muscle is divided down to the submucosa. The surgery can be performed open or laparoscopically, depending on the surgeon. The operation is curative and has very low morbidity.(B2)
Differential Diagnosis
Midgut volvulus is a twisting of malrotated bowel, which can present in the first month of life with bilious vomiting. An upper GI series shows the bowel's "corkscrew" appearance. An abdominal CT scan shows an inverted relationship between the superior mesenteric artery and the superior mesenteric vein.[5][6] Also in the differential diagnosis are gastroenteritis, acute renal failure, sepsis, hernia, colic, constipation, necrotizing enterocolitis, trauma, toxic megacolon, Hirschsprung disease, testicular torsion, appendicitis, and urinary tract infection.
Prognosis
The prognosis is excellent when diagnosed early. Surgery is curative. There is minimal mortality.[7]
Complications
A delayed diagnosis can lead to dehydration and hypovolemic shock.[8]
Postoperative and Rehabilitation Care
The only postoperative care is a continuation of intravenous fluids until they tolerate feeding. Feeding can begin 4 to 8 hours after recovery from anesthesia.[9] Up to 80% of patients continue to have some milder form of vomiting after surgery. If vomiting persists 5 days postoperatively, they would indicate further radiologic studies such as an upper GI series. Postoperatively, infants should be observed in the hospital for surgical complications such as incomplete pyloromyotomy, mucosal perforation, and bleeding. The infants may be discharged home when hydrated and tolerating feedings well.
Consultations
Consultation with a neonatal surgeon should begin as soon as the radiologist diagnoses.[10]
Deterrence and Patient Education
Healthcare professionals should educate parents about the strong risk of pyloric stenosis in the family. There is a nearly 200-fold increased risk among identical twins and a 20-fold increase among siblings. So, parents should be vigilant in identifying symptoms in their future offspring as early as possible.[11]
Pearls and Other Issues
Other causes of vomiting in infancy include midgut volvulus, a web of the gastric antrum, duodenal web, and annular pancreas.[12] There can be a degree of pylorospasm in infants, which is responsible for some delay in gastric emptying. In pylorospasm cases, the ultrasound shows a normal thickness of the pylorus muscle and a normal length of the pylorus channel.
Enhancing Healthcare Team Outcomes
Promptly diagnosing and treating infants with pyloric stenosis requires cooperation among several in-hospital medical professionals operating as an interprofessional healthcare team.[12] First, the emergency room physicians must assess the infant and begin intravenous fluids as needed. The emergency physicians request an emergent abdominal ultrasound. The radiologist should promptly diagnose using a stat pediatric ultrasound by a trained sonographer. A surgeon with expertise in neonatal pyloric stenosis surgery must be consulted as soon as the diagnosis is made. A pharmacist can help with electrolytes and other medication orders, and nurses administer these and provide an important backstop to prevent errors and report on patient response. This interprofessional approach leads to improved patient outcomes. Ultrasound is used much earlier to aid the diagnosis, and the classic signs of IHPS are less common. The mean age of presentation is getting significantly younger, and infants are not developing the physical signs or electrolyte abnormalities they were in the past.
References
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Level 2 (mid-level) evidenceAbdellatif M, Ghozy S, Kamel MG, Elawady SS, Ghorab MME, Attia AW, Le Huyen TT, Duy DTV, Hirayama K, Huy NT. Association between exposure to macrolides and the development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. European journal of pediatrics. 2019 Mar:178(3):301-314. doi: 10.1007/s00431-018-3287-7. Epub 2018 Nov 23 [PubMed PMID: 30470884]
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Level 3 (low-level) evidenceBašković M, Župančić B, Lesjak N, Vukasović I. Hypertrophic Pyloric Stenosis - Five-Year Retrospective Analysis. Acta medica Croatica : casopis Hravatske akademije medicinskih znanosti. 2016 Apr:70(2):103-6 [PubMed PMID: 28722837]
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Kapoor R, Kancherla V, Cao Y, Oleson J, Suhl J, Canfield MA, Druschel CM, Kirby RS, Meyer RE, Romitti PA. Prevalence and descriptive epidemiology of infantile hypertrophic pyloric stenosis in the United States: A multistate, population-based retrospective study, 1999-2010. Birth defects research. 2019 Feb 1:111(3):159-169. doi: 10.1002/bdr2.1439. Epub 2018 Dec 13 [PubMed PMID: 30549250]
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