Podcast
Questions and Answers
Which of the following electrolyte imbalances is commonly associated with hypertrophic pyloric stenosis?
Which of the following electrolyte imbalances is commonly associated with hypertrophic pyloric stenosis?
- Hyponatremic hyperkalemic metabolic acidosis
- Hypochloremic hypokalemic metabolic alkalosis (correct)
- Hyperchloremic metabolic acidosis
- Hypernatremic metabolic acidosis
A 3-week-old male infant presents with non-bilious projectile vomiting after each feeding. Which of the following is the MOST likely diagnosis?
A 3-week-old male infant presents with non-bilious projectile vomiting after each feeding. Which of the following is the MOST likely diagnosis?
- Intussusception
- Gastroesophageal reflux disease (GERD)
- Hypertrophic pyloric stenosis (correct)
- Duodenal atresia
During the physical examination of an infant with suspected pyloric stenosis, what palpable finding is MOST indicative of this condition?
During the physical examination of an infant with suspected pyloric stenosis, what palpable finding is MOST indicative of this condition?
- Palpable 'olive' mass in the epigastrium (correct)
- Distended bladder
- Enlarged liver
- Diffuse abdominal tenderness
What seasonal variation is most associated with Hypertrophic Pyloric Stenosis?
What seasonal variation is most associated with Hypertrophic Pyloric Stenosis?
What is the MOST appropriate initial step in managing an infant diagnosed with hypertrophic pyloric stenosis?
What is the MOST appropriate initial step in managing an infant diagnosed with hypertrophic pyloric stenosis?
Which of the following radiological findings is MOST indicative of hypertrophic pyloric stenosis on an upper gastrointestinal contrast study?
Which of the following radiological findings is MOST indicative of hypertrophic pyloric stenosis on an upper gastrointestinal contrast study?
What is the primary goal of the Ramstedt pyloromyotomy procedure in the treatment of hypertrophic pyloric stenosis?
What is the primary goal of the Ramstedt pyloromyotomy procedure in the treatment of hypertrophic pyloric stenosis?
What is the expected male to female ratio of infants affected by Hypertrophic Pyloric Stenosis?
What is the expected male to female ratio of infants affected by Hypertrophic Pyloric Stenosis?
Besides dehydration, which of the following is a known complication of Hypertrophic Pyloric Stenosis?
Besides dehydration, which of the following is a known complication of Hypertrophic Pyloric Stenosis?
Apart from genetic and familial factors, what is an environmental etiological factor that can cause Hypertrophic Pyloric Stenosis?
Apart from genetic and familial factors, what is an environmental etiological factor that can cause Hypertrophic Pyloric Stenosis?
An infant presents with non-bilious projectile vomiting. Lab results show hypochloremic, hypokalemic metabolic alkalosis. Which of the following best explains the underlying pathophysiology leading to these electrolyte imbalances?
An infant presents with non-bilious projectile vomiting. Lab results show hypochloremic, hypokalemic metabolic alkalosis. Which of the following best explains the underlying pathophysiology leading to these electrolyte imbalances?
After initial resuscitation, an infant with pyloric stenosis is scheduled for a Ramstedt pyloromyotomy. What is the MOST important consideration for the surgeon during this procedure?
After initial resuscitation, an infant with pyloric stenosis is scheduled for a Ramstedt pyloromyotomy. What is the MOST important consideration for the surgeon during this procedure?
What would be the MOST appropriate initial fluid management strategy for an infant presenting with signs of dehydration due to hypertrophic pyloric stenosis?
What would be the MOST appropriate initial fluid management strategy for an infant presenting with signs of dehydration due to hypertrophic pyloric stenosis?
Which finding on an abdominal ultrasound is MOST consistent with the diagnosis of hypertrophic pyloric stenosis?
Which finding on an abdominal ultrasound is MOST consistent with the diagnosis of hypertrophic pyloric stenosis?
An infant with pyloric stenosis experiences persistent vomiting despite appropriate fluid resuscitation. Which of the following acid-base imbalances is MOST likely to develop?
An infant with pyloric stenosis experiences persistent vomiting despite appropriate fluid resuscitation. Which of the following acid-base imbalances is MOST likely to develop?
During the surgical correction of pyloric stenosis, what is the PRIMARY reason for carefully avoiding injury to the mucosa?
During the surgical correction of pyloric stenosis, what is the PRIMARY reason for carefully avoiding injury to the mucosa?
Besides hypertrophic pyloric stenosis, what other medical condition would be on the differential for bilious vomiting?
Besides hypertrophic pyloric stenosis, what other medical condition would be on the differential for bilious vomiting?
What is the aim of Potassium administration in the treatment of Hypertrophic Pyloric Stenosis?
What is the aim of Potassium administration in the treatment of Hypertrophic Pyloric Stenosis?
Besides the genetic or familial etiology, what other cause has been linked to Hypertrophic Pyloric Stenosis?
Besides the genetic or familial etiology, what other cause has been linked to Hypertrophic Pyloric Stenosis?
Gastric peristaltic waves that move from the left costal margin to the pylorus can be observed for infants with Hypertrophic Pyloric Stenosis. What does this observation suggest?
Gastric peristaltic waves that move from the left costal margin to the pylorus can be observed for infants with Hypertrophic Pyloric Stenosis. What does this observation suggest?
In the context of hypertrophic pyloric stenosis, what is the rationale behind administering intravenous fluids containing potassium chloride after initial resuscitation with normal saline?
In the context of hypertrophic pyloric stenosis, what is the rationale behind administering intravenous fluids containing potassium chloride after initial resuscitation with normal saline?
An infant with hypertrophic pyloric stenosis presents with a palpable 'olive' mass and visible peristaltic waves. Which statement BEST integrates this clinical presentation with the underlying pathophysiology?
An infant with hypertrophic pyloric stenosis presents with a palpable 'olive' mass and visible peristaltic waves. Which statement BEST integrates this clinical presentation with the underlying pathophysiology?
What is the MOST critical difference in the presentation of emesis between pyloric stenosis and other causes of vomiting in infants, and how does this difference inform diagnostic considerations?
What is the MOST critical difference in the presentation of emesis between pyloric stenosis and other causes of vomiting in infants, and how does this difference inform diagnostic considerations?
After successful surgical correction of pyloric stenosis via pyloromyotomy, an infant continues to exhibit forceful vomiting. What is the MOST likely reason for the persistence of this symptom?
After successful surgical correction of pyloric stenosis via pyloromyotomy, an infant continues to exhibit forceful vomiting. What is the MOST likely reason for the persistence of this symptom?
An infant diagnosed with hypertrophic pyloric stenosis has undergone initial resuscitation. What is the clinical rationale for delaying surgical intervention until the infant demonstrates adequate urine output and normalized electrolyte levels?
An infant diagnosed with hypertrophic pyloric stenosis has undergone initial resuscitation. What is the clinical rationale for delaying surgical intervention until the infant demonstrates adequate urine output and normalized electrolyte levels?
Which of the following statements BEST explains the increased risk of hypertrophic pyloric stenosis in first-born male infants, considering genetic and epigenetic factors?
Which of the following statements BEST explains the increased risk of hypertrophic pyloric stenosis in first-born male infants, considering genetic and epigenetic factors?
In managing an infant with hypertrophic pyloric stenosis-induced metabolic alkalosis, what compensatory mechanisms should the clinician anticipate, and how do these mechanisms influence management strategies?
In managing an infant with hypertrophic pyloric stenosis-induced metabolic alkalosis, what compensatory mechanisms should the clinician anticipate, and how do these mechanisms influence management strategies?
While assessing an infant with suspected pyloric stenosis using abdominal ultrasound, the sonographer struggles to visualize the pylorus adequately. Which of the following maneuvers is MOST likely to improve visualization and diagnostic accuracy?
While assessing an infant with suspected pyloric stenosis using abdominal ultrasound, the sonographer struggles to visualize the pylorus adequately. Which of the following maneuvers is MOST likely to improve visualization and diagnostic accuracy?
An infant with confirmed hypertrophic pyloric stenosis is being prepared for Ramstedt pyloromyotomy. Which of the following pre-operative findings would MOST strongly suggest the presence of a concurrent metabolic derangement that requires further investigation beyond typical pyloric stenosis-related electrolyte abnormalities?
An infant with confirmed hypertrophic pyloric stenosis is being prepared for Ramstedt pyloromyotomy. Which of the following pre-operative findings would MOST strongly suggest the presence of a concurrent metabolic derangement that requires further investigation beyond typical pyloric stenosis-related electrolyte abnormalities?
Following a Ramstedt pyloromyotomy, an infant exhibits persistent irritability and feeding intolerance, alongside signs of abdominal distension. What potential complication should be HIGHEST on the list of differential diagnoses?
Following a Ramstedt pyloromyotomy, an infant exhibits persistent irritability and feeding intolerance, alongside signs of abdominal distension. What potential complication should be HIGHEST on the list of differential diagnoses?
Flashcards
Hypertrophic Pyloric Stenosis
Hypertrophic Pyloric Stenosis
A condition marked by the thickening of the pylorus muscle, leading to gastric outlet obstruction.
Non-bilious Projectile Vomiting
Non-bilious Projectile Vomiting
Vomiting that's forceful but doesn't contain bile, often occurring 2-4 weeks after birth.
Hypocholoraemic Hypokalaemic Alkalosis
Hypocholoraemic Hypokalaemic Alkalosis
A condition where the body's acid-base balance is disturbed due to loss of stomach acid.
Gastric Peristaltic Waves
Gastric Peristaltic Waves
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Palpable Pyloric Mass
Palpable Pyloric Mass
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Pyloric Stenosis Ultrasound Criteria
Pyloric Stenosis Ultrasound Criteria
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String Sign
String Sign
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Beak Sign
Beak Sign
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Pyloric Stenosis Treatment
Pyloric Stenosis Treatment
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Pyloromyotomy
Pyloromyotomy
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Hypertrophic Pyloric Stenosis (HPS)
Hypertrophic Pyloric Stenosis (HPS)
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Visible Gastric Peristalsis
Visible Gastric Peristalsis
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Hypochloremic, hypokalemic metabolic alkalosis
Hypochloremic, hypokalemic metabolic alkalosis
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Infant with pyloric stenosis
Infant with pyloric stenosis
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Arterial Blood Gas (ABG) abnormalities in HPS
Arterial Blood Gas (ABG) abnormalities in HPS
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Beak Sign on Upper GI Series
Beak Sign on Upper GI Series
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Ramstedt Pyloromyotomy
Ramstedt Pyloromyotomy
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Anatomic causes
Anatomic causes
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Levels as markers of adequate resuscitation
Levels as markers of adequate resuscitation
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HPS Seasonal Variation
HPS Seasonal Variation
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HPS and Constipation
HPS and Constipation
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HPS Vomiting type
HPS Vomiting type
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Typical onset age
Typical onset age
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HPS General Signs
HPS General Signs
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HPS vs Ethnic Origin
HPS vs Ethnic Origin
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HPS and Firstborn boys
HPS and Firstborn boys
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Environmental Etiology
Environmental Etiology
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Study Notes
- Hypertrophic pyloric stenosis is a condition marked by hypertrophy of the pylorus' circular muscle layers, causing constriction and obstruction of the gastric outlet.
- Described by Hirschsprung in 1888.
- Costs 5 LE.
Incidence
- Occurs in 1-4 per 1000 live births.
- Male to female ratio of 4:1.
- Increased risk in firstborn boys.
- Symptoms often show a seasonal peak in the spring.
- Leading surgical cause of emesis in infancy.
Etiology
- Can be idiopathic.
- Genetic factors are rarely autosomal dominant.
- May be familial.
- Ethnic origin is more common in whites, particularly Caucasians.
- Environmental factors include erythromycin or azithromycin exposure.
- Transpyloric feeding of premature babies is also a risk factor.
Pathophysiology
- Hypertrophied muscles lead to gastric outlet obstruction (GOO).
- GOO results in non-bilious projectile vomiting.
- Gastric fluid loss is also typical.
- Results in hypochloremic hypokalemic alkalosis.
- Leads to paradoxical aciduria.
Symptoms
- Typically appears 2-4 weeks after birth.
Manifestation
- Occasional regurgitation that becomes more frequent and forceful over several days.
- Vomiting is non-bilious.
- Shortly after vomiting, the infant acts starved and will feed vigorously.
- The infant experiences constipation with infrequent and firm stools.
- Progressive feeding intolerance and weight loss leading to failure to thrive.
Signs
- Dehydration.
- General signs include dry mucous membranes.
- Oliguria.
- Sunken eyes.
- Poor skin turgor.
- Jaundice in 2% of cases from starvation.
- Depressed fontanelles.
- Local signs include gastric peristaltic waves moving from left costal margin to the pylorus.
- Palpation reveals a pyloric tumor or olive mass in over 90% of cases.
Complications
- Dehydration.
- Hypokalemic hypochloremic metabolic alkalosis with paradoxical aciduria.
- Starvation.
- Gastritis and reflux esophagitis.
- Aspiration pneumonia.
Investigations
Laboratory
- Hypochloremic hypokalemic metabolic alkalosis.
- Paradoxical aciduria.
- Hyperbilirubinemia.
- Low serum levels of potassium and chloride.
- Increased blood pH and high blood bicarbonate level.
Radiological
- Abdominal ultrasound is the most sensitive and specific test.
- Muscle thickness greater than 4 mm or length of pylorus greater than 16 mm.
- String sign indicates a narrow pyloric channel.
- Bulge in the distal antrum with streak of barium pointing towards the pyloric channel.
- Beak sign is a bulge in the distal antrum with a barium streak pointing towards the pyloric canal.
- Barium may outline crowded mucosal folds as parallel lines.
Differential Diagnosis
Medical Causes
- Overfeeding.
- GERD.
- Gastroenteritis.
- Pylorospasm.
- Increased intracranial pressure.
- Metabolic disorders like adrenal insufficiency.
Anatomic causes
- Antral web.
- Foregut duplication cyst.
- Duodenal stenosis.
- Gastric tumors.
- Tumors causing extrinsic gastric compression.
Treatment
- Mainstay therapy is resuscitation.
- Followed by pyloromyotomy.
Preoperative
- Correct dehydration.
- Correct hypokalemic hyperchloremic alkalosis.
- Give a 10-20 mL/kg bolus of normal saline initially if electrolyte values are abnormal.
- Follow with D5/½NS containing 20-30 mEq/L of potassium chloride at 1.25-2 times the maintenance rate.
- Monitor serum carbon dioxide to be less than 30 mmol/L.
- Monitor chloride to be greater than 100 mmol/L.
- Monitor potassium to be between 4.5-6.5 mmol/L.
Operative
- Ramstedt pyloromyotomy.
- Approaches include a right upper quadrant transverse skin incision.
- Or a circumumbilical skin incision known as "Bianchi".
- Or via a laparoscopic approach.
- Pylorus is incised along its entire length.
- Spread widely, exposing but not breaching the underlying mucosa.
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