Pyloric Stenosis-Kabera Rene

8/4/2019 Pyloric Stenosis-Kabera Rene

1/13

KABERA Ren,MD

PGY III Resident

Family and Community Medicine

National University of Rwanda

PYLORIC STENOSIS


2/13

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.


3/13

ANATOMY


4/13

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).


5/13

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.


6/13

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.


7/13

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


8/13

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.


9/13

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.


10/13

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.


11/13

DIFFERENTIAL DIAGNOSIS

Inexperienced or inappropriate feeding

Gastro esophageal reflux

Gastritis

Congenital adrenal hyperplasia

Pyloric diaphragm

Pylorospasm


12/13

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.


13/13

Thank you

Documents

Pyloric Stenosis-Kabera Rene