Pyloric Stenosis And Other Congenital Anomalies Of Stomach

Question 1

T/F Hypertrophic pyloric stenosis (HPS) is rare in Asians and more common in whites

Answer 1

True

Question 2

HPS: Gender

Answer 2

M, especially firstborns

Question 3

The incidence of pyloric stenosis is increased in infants with ………blood groups

Answer 3

B and O

Question 4

Syndromes associated with pyloric stenosis….

Answer 4

Apert syndrome, 
Zellweger syndrome, 
trisomy 18, 
Smith-Lemli-Opitz syndrome, and 
Cornelia de Lange syndrome.

Question 5

Usages ……is associated with pyloric stenosis

Answer 5

Erythromycin

Highest risk if the medication is given within the f irst 2 wk of life

Question 6

Higher risk of pyloric stenosis is seen in female infants of mothers treated with ___ during pregnancy and breastfeeding

Answer 6

Macrolide antibiotic

Question 7

Reduced levels of this substance is implicated in the pathogenesis of pyloric stenosis….

Answer 7

Nitric oxide

Question 8

Initial symptoms of pyloric stenosis…

Answer 8

Non-bilious vomiting

Question 9

Characteristics of vomiting in pyloric stenosis…

Answer 9

The vomiting may or may not be projectile initially but is usually progressive, occurring immediately after a feeding.
Emesis might follow each feeding, or it may be intermittent.
The vomiting usually starts after 3 wk of age, but symptoms can develop as early as the first wk of life and as late as the 5th mo.
Approximately 20% have intermittent emesis from birth that then progresses to the classic picture.
After vomiting, the infant is hungry and wants to feed again

Question 10

Vomiting in infants with pyloric stenosis usually starts at what age

Answer 10

3 weeks of age, but can develop as early as a week and as late as 5th month

Question 11

Acid base abnormality seen in patients with pyloric stenosis with persistent vomiting

Answer 11

Hypochloremic metabolic alkalosis

Question 12

Hyperbilirubinemia is the most common clinical association of pyloric stenosis, also known as…

Answer 12

Icteropyloric syndrome.

Question 13

Icteropyloric syndrome. ……. ..

Answer 13

Unconjugated hyperbilirubinemia is more common than conjugated and usually resolves with surgical correction of the pyloric stenosis.

It may be associated with a decreased level of glucuronyl transferase as seen in approximately 5% of affected infants;
mutations in the bilirubin uridine diphosphate glucuronosyltransferase gene (UGT1A1) have also been implicated

Question 14

Other coexistent clinical diagnoses have been described, including………..

Answer 14

eosinophilic gastroenteritis, hiatal hernia, peptic ulcer, congenital nephrotic syndrome, congenital heart disease, and congenital hypothyroidism.

Question 15

Method of palpitation mass in pyloric stenosis…

Answer 15

The mass is firm, movable, approximately 2 cm in length, olive shaped, hard, best palpated from the left side, and located above and to the right of the umbilicus in the mid epigastrium beneath the liver’s edge.

The olive is easiest palpated after an episode of vomiting.

After feeding, there may be a visible gastric peristaltic wave that progresses across the abdomen

Question 16

USG features……..

Answer 16

Criteria for diagnosis include pyloric thickness 3-4 mm, an overall pyloric length 15-19 mm, and pyloric diameter of 10-14 mm (

Question 17

Signs in contrast studies ……

Answer 17

They demonstrate an elongated pyloric channel (string sign),

A bulge of the pyloric muscle into the antrum (shoulder sign), and

Parallel streaks of barium seen in the narrowed channel, producing a “double tract sign”

Question 18

The surgical procedure of choice is…….

Answer 18

Pyloromyotomy

Question 19

Ramstedt procedure ……..

Answer 19

Ramstedt procedure is performed through a short transverse skin incision.

The underlying pyloric mass is cut longitudinally to the layer of the submucosa, and the incision is closed.

Question 20

Etiology for postoperative vomiting in pyloric stenosis…..

Answer 20

Occurs in half the infants and is thought to be secondary to edema of the pylorus at the incision site

Question 21

Persistent vomiting in post op. suggests………

Answer 21

Persistent vomiting suggests an incomplete pyloromyotomy, gastritis, gastroesophageal reflux disease, or another cause of the obstruction.

Question 22

Treatment for infants with persistent vomiting secondary to incomplete pyloromyotomy.

Answer 22

Endoscopic balloon dilation

Question 23

Pyloric atresia has been associated……

Answer 23

Epidermolysis bullosa and
usually presents in early infancy.
The gender distribution is equal.