Transcript
8/4/2019 Pyloric Stenosis-Kabera Rene
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KABERA Ren,MD
PGY III Resident
Family and Community Medicine
National University of Rwanda
PYLORIC STENOSIS
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INTRODUCTION
Pyloric stenosis classically presents with the gradual onset
of nonbilious projectile vomiting after 3 weeks of age.
First described by Hirschsprung in 1888
Ramstedt described an operative procedure to alleviate the
condition in 1907 the procedure used to this day to treat
pyloric stenosis
20% of infants are symptomatic from birth, and most are
symptomatic within the first 2 months after birth.
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ANATOMY
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INTRODUCTION
Genetics: Multifactorial inheritance risk; recurrence risk 3-
9% if first degree relative affected 1/300-1/1000 live births
(male 1/150 live births; female 1/750 live births) .
Predominant age: Infancy; onset usually at 3-4 weeks of
age, rarely in the newborn period or as late as 5 months of
age.
Predominant sex: Male > Female (5:1).
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ETIOLOGY
Hypertrophy is after birth, leading to gastric outlet
obstruction.
The pathogenesis remains unclear but postnatal infusion of
gastrin produces an identical lesion in newborn puppies.
Hypergastrinemia may play an important etiologic role.
Gastrin levels are known to be elevated in the newborn.
Prostaglandin E2 infusion, which is used to maintain a
patent ductus arteriosus in certain cardiac anomalies, hasbeen linked to a higher incidence of infantile hypertrophic
pyloric stenosis.
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ETIOLOGY
Recent studies show a relative lack of nitric oxide (a
smooth muscle relaxant) synthase innervation.
Decreased density of interstitial cells of Cajal (ICC)
Increased synthesis of epidermal growth factor in the
hypertrophied muscle,
Primary underlying cause of the disorder is still unclear.
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SIGNS AND SYMPTOMS
Epigastric distention
Visible gastric peristalsis, sometimes retrograde
Palpable tumor (olive) in right upper quadrant
Diagnosis traditionally made by palpation of mass Firm,
movable, approx 2 cm in length, olive shaped and best
palpated from the left.
Mass located above and to the right of the umbilicus in themidepigastrum beneath the liver edge
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SIGNS AND SYMPTOMS
Jaundice: unconjugated hyperbilirubinemia may be
observed, which is thought to result from inadequate
glucose absorption and an inability to maintain glucuronyl
transferase activity. Diminished stools
Prolonged vomiting may lead to dehydration, weight loss,
and development of hypochloremic alkalosis. Hypokalemia
Intermittent, non-bilious, projectile vomiting of increasingfrequency and severity.
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DIAGNOSIS
CLINICAL:OLIVE
LABORATORY:
Early - evidence hypochloremic alkalosis, with low serum
chloride and high bicarbonate
Later - may have acidosis with low bicarbonate and low
potassium
Elevated unconjugated bilirubin level
PATHOLOGICAL FINDINGS: Concentric hypertrophy of pyloric muscle.
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DIAGNOSIS
IMAGING:
Upright plain film of abdomen may reveal dilated stomach
(filled with fluid and/or air) and relative lack of air in
intestines.
Ultrasound (first choice if available) shows thickened and
elongated pyloric muscle
Barium swallow (performed only when diagnosis is not
clinically clear) reveals strong gastric contractions andelongated, narrow pyloric canal (string sign); now rarely
performed if ultrasound available.
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DIFFERENTIAL DIAGNOSIS
Inexperienced or inappropriate feeding
Gastro esophageal reflux
Gastritis
Congenital adrenal hyperplasia
Pyloric diaphragm
Pylorospasm
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MANAGEMENT
In infants who present with dehydration, fluid resuscitation
begins before diagnostic procedures are initiated.
Correction of alkalosis and hypokalemia is essential before
treatment, which is non emergent and performed only afterthe patient is stabilized.
The Ramstedt pyloromyotomy is the surgical procedure of
choice, being curative and having mortality rates of 0.0 to
0.5% and an incidence of recurrence of 1%. A longitudinal incision divides the serosal muscle on the
anterior surface of the pylorus down to the submucosa.
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Thank you
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