2. INTRO
• Idiopathic HPS
• Infantile HPS
• Pediatric HPS
• Hirschsprung in 1888
• 3 /1000 babies
• Form of gastric outlet obstruction
• Affects gastrointestinal tract of an
infant (mainly 1st born male)
– Obstruction of the pyloric lumen
– Pyloric muscular dystrophy
• Forceful vomiting, dehydration and
salt and fluid imbalance
• Immediate treatment
3. SYMPTOMS
3 weeks of age
VOMITING
-1st symptom
-NON BILIOUS
-may be brown tinged with blood if gastritis develops
CHANGES IN STOOL
--fewer, smaller stool
-constipation or mucus stool
FAILURE TO GAIN WEIGHT & LETHARGY
-fluid and salt abnormalities
-dehydration
*less active, sunken “soft spot” on head, sunken
eyes, wrinkled skin
*less urine (4 to 6 hours between a wet diaper)
-increased stomach contractions after feeds. May make
ripples, or WAVES OF PERISTALSIS
Symptoms may be deceptive because the infant may seem
uncomfortable, but may not appear in great pain or look ill
4. CAUSES
• Unknown
• Abnormal muscle innvervation, breast feeding
and maternal stress in 3rd trimester
• Type B or O blood group
• May be genetic
• 3 out of 1000 births-firstborn male
• Whites of Northern European ancestry
• Less common in African Americans
• Rare in Asians
5. MANAGEMENT
• Fluid replacement
• Electrolyte abnormalities
to stabilize patient
• 1960s oral atropine,
surgery
• 1991 Pyloromyotomy
-short transverse
incision or laparoscopically
6.
7.
8. COMPLICATIONS
• Are rare, but include:
-bleeding
-hernia
-infection
• In rare cases , initial operation may not resolve vomiting
therefore, a reoperation may be necessary
• Small intestine may develop a leak and infant may become
seriously ill
• Low incidence of morbidity and mortality
• Most infants go home from the hospital within one or two days
after surgery
• Excellent results
• no increased risk of stomach or intestinal problems later in life
• The risk of dying from the procedure is extremely small and is
often related to other severe medical conditions.
9. CASE STUDY
• This is a 3 week old male infant who presents to the emergency department with a
chief complaint of vomiting x 3-4 days. His mother states that the vomiting has gotten
progressively worse and now seems to "shoot out of his mouth." The emesis always
occurs after feeding, sometimes vomiting the entire volume of his feed. The vomitus is
non-bilious and non-bloody. After vomiting, the infant remains hungry and is still eager
to feed. He is exclusively bottle fed with formula. There is no history of fever, URI
symptoms, or diarrhea. He is less active than normal. He is making fewer wet diapers
and less stool than usual. There is no history of trauma or recent travel. There are no ill
contacts.
• Exam: VS T 37.0, P 170, R 50, BP 80/50, O2 saturation 99% on RA. Length is 54 cm (50th
percentile) and weight is 3.6 kg (25th percentile; previously 50th) and head
circumference is 37 cm (50th). He is a well-developed, well-nourished male in no
distress. His skin is normal. HEENT exam is normal. His neck is supple. Heart
auscultation reveals tachycardia and a regular rhythm. Lungs are clear. His abdomen is
slightly distended with active bowel sounds. No hepatosplenomegaly is noted.
Attempting to palpate an olive mass is inconclusive. He has no inguinal hernias.
Genitalia are normal. Extremities are normal. Color, perfusion, and capillary refill are
good. Neurologic examination is normal.
• CBC is unremarkable. Electrolytes: Na 131, K 3.2, Cl 95, bicarb 30. An IV fluid infusion is
started. An abdominal series shows no obstruction, but the stomach is dilated.
10. CASE STUDY
Diagnosis:
• An ultrasound study confirms the diagnosis of
pyloric stenosis. The patient undergoes a
pyloromyotomy and recovers without
complications.