Overview

Definition:
-Pyloric stenosis is a condition in infants characterized by the thickening of the pyloric sphincter muscle, leading to obstruction of gastric emptying
-Open pyloromyotomy is the surgical procedure to relieve this obstruction.
Epidemiology:
-It is one of the most common surgical emergencies in infancy, typically presenting between 3 to 12 weeks of age
-Incidence is estimated at 2-5 per 1000 live births, with a male predominance (4:1 to 5:1)
-First-born males are at higher risk
-There is a familial predisposition.
Clinical Significance:
-Untreated pyloric stenosis leads to severe dehydration, electrolyte imbalances, malnutrition, and can be life-threatening
-Prompt diagnosis and surgical intervention are crucial for a favorable outcome and preventing long-term complications.

Clinical Presentation

Symptoms:
-Non-bilious projectile vomiting, typically starting after the first week of life
-Emesis is forceful and can project food several feet
-Gradual weight loss or failure to gain weight
-Persistent hunger despite vomiting
-Dehydration signs: decreased urine output, dry mucous membranes, sunken fontanelle.
Signs:
-A palpable, firm, olive-shaped mass in the upper abdomen, usually to the right of the midline
-Visible gastric peristaltic waves progressing from left to right across the abdomen
-Signs of dehydration, including lethargy and decreased skin turgor.
Diagnostic Criteria:
-Diagnosis is primarily clinical, confirmed by imaging
-Classic presentation of projectile non-bilious vomiting in an infant with a palpable pyloric mass is highly suggestive.

Diagnostic Approach

History Taking:
-Detailed history of vomiting: onset, frequency, volume, character (bilious vs
-non-bilious), projectile nature
-Feeding history and weight gain pattern
-Urine output and stool frequency
-Family history of pyloric stenosis.
Physical Examination:
-Careful palpation of the abdomen for a pyloric mass (gastric olive)
-Observation for gastric peristalsis
-Assessment of hydration status: fontanelle, mucous membranes, skin turgor, capillary refill
-Vital signs monitoring.
Investigations:
-Abdominal ultrasound is the imaging modality of choice: measures pyloric muscle thickness (>3 mm) and channel length (>15 mm), and demonstrates delayed gastric emptying
-Upper GI series (barium swallow) can show a "string sign" or "shoulder sign" but is less preferred due to radiation and potential aspiration risk
-Electrolyte and blood gas analysis to assess for dehydration and metabolic alkalosis (hypochloremic, hypokalemic metabolic alkalosis is common).
Differential Diagnosis: Gastroesophageal reflux (GERD), milk allergy, overfeeding, intestinal obstruction (e.g., malrotation with volvulus), infectious gastroenteritis, neurological causes of vomiting.

Management

Initial Management:
-Fluid resuscitation and correction of electrolyte imbalances are paramount before surgery
-Intravenous fluids (e.g., normal saline with potassium supplementation) are administered
-Nasogastric tube may be inserted for gastric decompression if vomiting is severe.
Medical Management:
-Medical management is generally not indicated for established pyloric stenosis
-surgery is the definitive treatment
-However, correction of dehydration and metabolic derangements is essential prior to surgery.
Surgical Management:
-The standard treatment is a Ramstedt pyloromyotomy, an open surgical procedure
-Indications include confirmed diagnosis of pyloric stenosis with significant symptoms and signs of dehydration
-The technique involves incising the thickened pyloric muscle longitudinally, splitting it down to the mucosa without entering the lumen
-Historically, open surgery is performed via a circumumbilical or right upper quadrant incision.
Supportive Care:
-Postoperative care involves monitoring vital signs, pain control, and gradual reintroduction of oral feeds
-Intravenous fluids are continued until adequate oral intake is established
-Early feeding can usually commence within 4-6 hours postoperatively
-Close monitoring for signs of complications like wound infection, bleeding, or persistent vomiting.

Complications

Early Complications:
-Bleeding from the incision or within the abdominal cavity
-Wound infection
-Incomplete pyloromyotomy leading to persistent vomiting
-Perforation of the gastric mucosa during the procedure
-Injury to adjacent organs (e.g., pancreas).
Late Complications:
-Gastric outlet obstruction due to stricture or adhesions (rare)
-Chronic abdominal pain
-Increased risk of peptic ulcer disease in adulthood (debated).
Prevention Strategies:
-Meticulous surgical technique to avoid mucosal injury
-Proper wound care
-Aggressive fluid and electrolyte management pre- and post-operatively
-Early identification and management of any signs of complications.

Prognosis

Factors Affecting Prognosis:
-The overall prognosis for pyloric stenosis after pyloromyotomy is excellent
-Factors influencing recovery include the severity of preoperative dehydration and malnutrition, presence of any intraoperative complications, and speed of return of bowel function.
Outcomes:
-Most infants return to their birth weight and achieve normal feeding patterns within a few weeks
-Long-term outcomes are generally very good with minimal residual issues
-Recurrence of stenosis is extremely rare.
Follow Up:
-Routine follow-up is usually not required unless there are specific concerns or complications
-Parents should be advised on signs of feeding intolerance or post-operative complications
-Gradual increase in oral feeds is guided by the infant's tolerance.

Key Points

Exam Focus:
-Classic presentation: projectile non-bilious vomiting in a male infant, 3-12 weeks old
-Palpable "olive" and visible peristalsis
-Ultrasound findings: thickened pylorus
-Ramstedt pyloromyotomy is the definitive treatment
-Hypochloremic metabolic alkalosis.
Clinical Pearls:
-Always palpate for the pyloric mass carefully in infants with unexplained vomiting
-Differentiate from GERD by the projectile nature and presence of weight loss
-The procedure is a simple myotomy, not a resection.
Common Mistakes:
-Delaying diagnosis due to misattribution to simple reflux or overfeeding
-Inadequate correction of dehydration and electrolyte imbalance preoperatively
-Inadvertent mucosal perforation during pyloromyotomy.