Pyloric Stenosis

Pyloric Stenosis

Q. 1 Regarding  congenital  hypertrophic  pyloric stenosis of infancy which is not correct?
 A It most commonly occurs in first born male child
 B It usually presents at 3 to 4 weeks of life
 C Feeding is followed by bilious vomiting
 D Baby is hungry soon after vomiting
Q. 1 Regarding  congenital  hypertrophic  pyloric stenosis of infancy which is not correct?
 A It most commonly occurs in first born male child
 B It usually presents at 3 to 4 weeks of life
 C Feeding is followed by bilious vomiting
 D Baby is hungry soon after vomiting
Ans. C

Explanation:

Pyloric stenosis is associated with non-bilious vomiting.

Important points

1. Males (especially first born) are affected approximately four times as often as females.

2. The incidence of pyloric stenosis is increased in infants with type B and O blood groups.

3. Pyloric stenosis is associated with other congenital defects like tracheoesophageal fistula.

4. Exogenous administration of prostaglandin E to maintain patency of the ductus arteriosus is associated with pyloric stenosis.

5. Due to continuous vomiting, a progressive loss of fluid, hydrogen ion and chloride, leads to a hypochloremic metabolic alkalosis.

6. The diagnosis is traditionally established by palpating the pyloric mass.

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

8. Ultrasound examination confirms the diagnosis in the majority of cases.

9. Barium studies reveal an elongated pyloric channel, 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”.

10. The surgical procedure of choice is the Ramstedt pyloromyotomy.


Q. 2

Metabolic abnormalities associated with Congenital Pyloric Stenosis in the early phase include all EXCEPT:

 A Hypokalemia
 B

Aciduria

 C Hypochloremia
 D

None of these

Ans. B

Explanation:

Aciduria REF: Bailey and love 25th edition page 79, Fundamentals of Pediatric Surgery by Peter Mattei Page 341, Clinical cases in surgery – Michelle Li, Stephanie C. Lin, Aaron B. Caughey Page 104

“The classical electrolyte abnormality associated withpyloric stenosis is hypokalemic, hypochloremic metabolic alkalosis, presumably due to ongoing gastric acid loss from vomiting. Paradoxical acidurea is sometimes noted (with progressive Hypokalemia and hypovolemia) because the kidney excretes protons in exchange of sodium in an attempt to conserve fluid”

NOTE: Paradoxical aciduria is seen only after body stores of Sodium & Potassium get depleted. Initially there is alkaliuria, not aciduria.


Q. 3 A 3 week old patient presenting with vomiting and failure to thrive is found to have pyloric stenosis. What should be the next step of management?
 A

its emergency so do pyloromyotomy immediately

 B

fluid resuscitation may be delayed

 C

correction of electrolyte disturbances

 D

Cardiopulmonary resuscitation

Ans. C

Explanation:

Correction of electrolyte disturbances [Ref. Schwartz 9/e p1425 (8/e, p1486; 7/e, p1728); Rudolph’s Pediatrics, 21st Edition Chapter 17; Bailey & love 25/e p78 (24/e p1033; 23/e, p 899)]

Pyloric stenosis is never a surgical emergency although dehydration and electrolyte abnormalities may present a medical emergency

Fluid resuscitation and correction of electrolyte abnormalities and metabolic alkalosis is essential before surgery. Surgery done is Fredet-Ranzstedt pyloromyotomy. (In it the pyloric mass is split without cutting the mucosa)

More about Hypertrophic Pyloric Stenosis (HPS)

  • The musculature of the pylorus and adjacent antrum is grossly hypertrophied, the hypertrophy being maximum in the pylorus itself.
  • It occurs in approximately 3 in 1000 live birth and is the most common surgical cause of vomitting in infancy.
  • M > F (4 I)*
  • Characterstically the first born male child is affected.
  • The conditon is most commonly seen at 4 wks after birth ranging from the 3rd wk to on rare occasion, the 7th. However, 20% of infants are symptomatic from birth, and most are symptomatic within the first 2 months after birth.
  • Non-bilious vomiting, becoming increasingly projectile, occurs over several days to weeks. Eventually the infant will develop a nearly complete obstruction by the second to fourth week of life and will not be able to hold down even clear liquids. This invariaby proceeds to severe dehydration if not tlt.
  • These infants develop a metabolic alkalosis with severe depletion of potassium and chloride ions.
  • ” The diagnosis of pyloric stenosis usually can he made on physical examination by palpation of the typical ‘olive’ in the right upper quadrant and the presence of visible gastric waves on the abdomen.
  • When the olive cannot be palpated, ultrasound in experienced hands will diagnose the condition accurately in 95 percent of pts.” – Schwartz.
  • Also know
  • Administration of erythromycin in early infancy has been linked to the subsequent development of HPS.
  • Jaundice may be seen in HPS, although cause is not clear.

Q. 4

Congenital hypertrophic pyloric stenosis associated with

 A

Hypokalemic acidosis

 B

Hypokalemic alkalosis

 C

Hyperkalemic acidosis

 D

Hyperkalemic alkalosis

Ans. B

Explanation:Hypokalemic alkalosis [Ref. Schwartz 9/e p1426 (8/e, p1486; 7/e, p 1728); Maingot’s 10/e, p 2067; Bailey & love, 24/e, p 1033 (23/e, p 899)]

Repeat from Nov 09

  • Repetitive vomiting leads to hypochloremic, hypokalemic metabolic alkalosis. The urine is alkaline initially but eventually it becomes acidic (paradoxical aciduria)
  • Cause of paradoxical aciduria

– Initially the kidney compensate for metabolic alkalosis by reabsorbing hydrogen and chloride ions in exchange for sodium, potassium and bicarbonate. But gradually as the body stores of sodium and especially potassium become depleted, kidney starts retaining these ions in exchange of hydrogen ions. thus the urine becomes acidic and metabolic alkaline state is further aggravated.

Hypertrophic Pyloric Stenosis (HPS)

  • The musculature of the pylorus and adjacent antrum is grossly hypertrophied, the hypertrophy being maximum in the pylorus itself.
  • It occurs in approximately 3 in 1000 live birth and is the most common surgical cause of vomitting in infancy.
  • M > F (4 : 1)*
  • Characterstically the first born male child is affected.
  • The conditon is most commonly seen at 4 wks after birth ranging from the 3rd wk to on rare occasion, the 7th. However. 20% of infants are symptomatic from birth, and most are symptomatic within the first 2 months after birth.
  • Non-bilious vomiting, becoming increasingly projectile, occurs over several days to weeks. Eventually the infant will develop a nearly complete obstruction by the second to fourth week of life and will not be able to hold down even clear liquids. This invariaby proceeds to severe dehydration if not t/t.
  • These infants develop a metabolic alkalosis with severe depletion of potassium and chloride ions.
  • ” The diagnosis of pyloric stenosis usually can be made on physical examination by palpation of the typical ‘olive’ in the right upper quadrant and the presence of visible gastric waves on the abdomen.
  • When the olive cannot be palpated, ultrasound in experienced hands will diagnose the condition accurately in 95 percent of pts.” – Schwartz.
  • Treatment

– Pyloric stenosis is never a surgical emergency although dehydration and electrolyte

– abnormalities may present a medical emergency

– Fluid resuscitation and correction of electrolyte abnormalities and metabolic alkalosis is essential before surgery.

– Surgery : Fredet-Ramstedt pyloromyotomy. (In it the pyloric mass is split without cutting the mucosa)

  • Also know
  • Administration of erythromycin in early infancy has been linked to the subsequent development of HPS.
  • Jaundice may be seen in HPS, although cause is not clear.

Q. 5

In a case of hypertrophic pyloric stenosis, the metabolic disturbance is

 A

Respiratory alkalosis

 B

Metabolic acidosis

 C

Metabolic alkalosis with paradoxical aciduria

 D

Metabolic alkalosis with alkaline urine

Ans. C

Explanation:

Metabolic alkalosis with paradoxical aciduria 1Ref Schwartz 9/e p1426 (8/e, p 1486): Maingot’s. 10/e, p 2067]

  • Repetitive vomiting leads to hypochloremic, hypokalemic metabolic alkalosis. The urine is alkaline initially but eventually it becomes acidic (paradoxical aciduria)
  • Cause of paradoxical aciduria

Initially the kidney compensate for metabolic alkalosis by reabsorbing hydrogen and chloride ions in exchange for sodium, potassium and bicarbonate. But gradually as the body stores of sodium and especially potassium become depleted, kidney starts retaining these ions in exchange of hydrogen ions. thus the urine becomes acidic and metabolic alkaline state is further aggravated.


Q. 6 Metabolic abnormality seen in congenital hypertrophic pyloric stenosis is
 A

Hypochloremic hypokalemic metabolic alkalosis

 B

Hyperchlonemic hypokalemic metabolic alkalosis

 C

Hypochloremic hypokalemic metabolic acidosis

 D

Hyperchloremic hypokalemic metabolic acidosis

Ans. A

Explanation:Hypochloremic hypokalemic metabolic alkalosis [Ref: Schwartz 9/e p1426 (8/e, p 1486); Maingot’s 10/e. p 2067 Bailey & love 25/e p78 (24/e p1033; 23/e p899)]

Repeated vomiting leads to hypochloremic, hypokalemic metabolic alkalosis. The urine is alkaline initially but eventually it becomes acidic (paradoxical aciduria)

  • Cause of paradoxical aciduria

Initially the kidney compensate for metabolic alkalosis by reabsorbing hydrogen and chloride ions in exchange for sodium, potassium and bicarbonate. But gradually as the body stores of sodium and especially potassium become depleted, kidney starts retaining these ions in exchange of hydrogen ions. thus the urine becomes acidic and metabolic alkaline state is further aggravated


Q. 7 Drug which can cause Hypertrophic Pyloric stenosis is:
 A Rifampicin
 B Erythromycin
 C Azithromycin
 D Ampicillin
Ans. B

Explanation:Erythromycin


Q. 8 In a case of hypertrophic pyloric stenosis, which of the following metabolic disturbance is found?

 A

Respiratory alkalosis

 B

Metabolic acidosis

 C

Metabolic alkalosis with paradoxical aciduria

 D

Metabolic alkalosis with alkaline urine

Ans. C

Explanation:

Due to vomiting of the gastric contents, infants develop a hypochloremic, hypokalemic metabolic alkalosis.

The urine pH is high initially but eventually drops because hydrogen ions are preferentially exchanged for sodium ions in the distal tubule of the kidney as the hypochloremia becomes severe.

Ref: Schwartz’s Principles of Surgery, 9th Edition, Chapter 39


Q. 9 What is true regarding congenital hypertrophic pyloric stenosis :

 A

More common in girls

 B

Hypochloremic alkalosis

 C

Hellers myotomy is the procedure of choice

 D

Most often manifests at birth

Ans. B

Explanation:

Hypochloremic alkalosis develops due to vomiting.

Ref: Text Book of Pediatrics By Nelson, 19th Edition, Page 1274; Bailey and Love’s Short Practice of Surgery, 22nd Edition, Page 674 and 23rd Edition, Page 899.


Q. 10

What is the following most characteristic feature of congenital hypertrophic pyloric stenosis?

 A

The patient is commonly marasmic

 B

It is associated with early loss of appetite

 C

It mostly affects the first born female child

 D

The pyloric tumour is best felt during feeding

Ans. D

Explanation:

Diagnosis of congenital hypertrophic pyloric stenosis can be made with a test feed, which produces a characteristic peristaltic wave which is seen to pass across the upper abdomen.

Palpation of the abdomen at that time reveals the lump.

Ref: Bailey and Love’s Short Practice of Surgery, 24th Edition, Page 1033.


Q. 11

A 3 week old baby presenting with difficulty in feeding, vomiting and failure to thrive is found to have pyloric stenosis. What should be the next immediate step of management?

 A

Its emergency so do pyloromyotomy immediately

 B

Fluid resuscitation may be delayed

 C

Correction of electrolyte disturbances

 D

Cardiopulmonary resuscitation

Ans. C

Explanation:

Pyloric stenosis is never a surgical emergency although dehydration and electrolyte abnormalities may present as medical emergency.

Treatment of dehydration and electrolyte imbalance is mandatory before surgical treatment, even if it takes 24–48 hours.

Fredet-Ramstedt pyloromyotomy is the treatment of choice and consist of incision down to the mucosa along the length of pylorus.


Q. 12

All of the following are FALSE in case of hypertrophic pyloric stenosis, EXCEPT:

 A

Symptomatic within one week

 B

Lump is always clinically palpable

 C

Treatment of choice is Finney’s pyloroplasty

 D

Ultrasonography is the diagnostic test

Ans. D

Explanation:

In hypertrophic pyloric stenosis, the musculature of the pylorus and adjacent antrum is grossly hypertrophied, the hypertrophy being maximum in the pylorus itself.

  • It occur in approximately 3 in 1000 live births and is the most common surgical cause of vomiting in infancy. Males are affected more commonly as compared to females and characteristically the first born male child is affected.
  • The condition is most commonly seen at 4 week after birth ranging from 3rd week to 7th week in rare occasions. Only 20% of infants are symptomatic from birth.
  • The diagnosis of pyloric stenosis usually can be made on clinical examination by palpation of the typical “olive” in the upper quadrant and the presence of visible gastric waves on the abdomen. In healthy infants when palpation is difficult, feeding can help in the diagnosis. After feeding peristalsis waves may be visible. After the infants vomits, the abdominal musculature is more relaxed and the mass is easier to palpate.
  • “When the olive cannot be palpated, USG can diagnose the condition accurately in 95% of patients”.
  • Surgical operation done for hypertrophic pyloric stenosis is Fredet- Ramstedt pyloromyotomy. (In it the pyloric mass is split without cutting the mucosa)

Ref: Schwartz 9/e, Page 1425; Bailey & Love 25/e, Page 78; Rudolph’s Pediatrics 21/e, Chapter 17


Q. 13

Clinical features of pyloric stenosis in infants are all, EXCEPT:

 A

Abdominal lump

 B

Constipation

 C

Weight Loss

 D

Metabolic acidosis

Ans. D

Explanation:

Metabolic alkalosis, not acidosis is seen in hypertrophic pyloric stenosis due to loss of hydrogen ions in repeated vomiting.
 

Ref: Schwartz 9/e, Page 1426 ; Maingot’s 10/e, Page 2067.


Q. 14

Congenital pyloric stenosis causes:

1. Bilious vomiting
2. Non- bilious vomiting
3. Projectile vomiting
4. Non projectile vomiting
5. Forceful
 A 1,2 & 4

 B

2,3,4 & 5

 C

1,3 & 4

 D

All are seen

Ans. B

Explanation:

The vomiting in pyloric stenosis may or may not be projectile, however non bilious vomiting after feed is the characteristic feature.
In about 10-15% of infants vomiting may contain blood.

This bleeding is due to reflux esophagitis, a common association with hypertrophic pyloric stenosis.

Also know:

Associated anomalies occur in 6-20% of patients.

These includes esophageal atresia, Hirschsprung’s disease, anorectal anomalies, and intestinal malformation.

Ref: Nelson 17/e, Page 1229.


Q. 15

TRUE/FALSE about features of Pyloric stenosis:

1. Hypokalemic alkalosis
2. Peristalsis right to left
3. Commonly caused by carcinoma stomach
4. Retention vomiting is present

5. Commonly females are affected

 A

1,2,3 true & 4,5 false

 B

1,2,5 true & 3,4 false

 C

1 true 2,3,4,5 false

 D

All are true

Ans. C

Explanation:

Hypertrophic pyloric stenosis is more common in males. Male : female incidence is 4:1.

  • Peristalsis is seen from left to right across the upper abdomen.
  • Retention vomiting means vomiting due to any mechanical obstruction occurring usually hours after ingestion of a meal. In hypertrophic pyloric stenosis the vomiting usually occur 10 to 30 min after the feed.
Ref: Nelson 17/e, Page 1229

Q. 16 A 3 week old child with projectile postprandial vomiting is diagnosed of having hypertrophic pyloric stenosis. Congenital hypertrophic pyloric stenosis is associated with:

 A

Hypokalemic acidosis

 B

Hypokalemic alkalosis

 C

Hyperkalemic acidosis

 D

Hyperkalemic alkalosis

Ans. B

Explanation:

Laboratory Findings of congenital hypertrophic pyloric stenosis:

Hypochloremic alkalosis with potassium depletion are the classic metabolic findings, though low chloride may be seen.

Dehydration causes elevated hemoglobin and hematocrit. Mild unconjugated bilirubinemia occurs in 2%–5% of cases.
 
Ref: Sundaram S.S., Hoffenberg E.J., Kramer R.E., Sondheimer J.M., Furuta G.T. (2012). Chapter 21. Gastrointestinal Tract. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

Q. 17 Which of the following electrolyte abnormality can be seen in an infant with congenital hypertrophic pyloric stenosis?

 A

Hypokalemic acidosis

 B

Hypokalemic alkalosis

 C

Hyperkalemic acidosis

 D

Hyperkalemic alkalosis

Ans. B

Explanation:

Infants with HPS present with nonbilious vomiting that becomes increasingly projectile over the course of several days to weeks.

Infants with HPS develop a hypochloremic, hypokalemic metabolic alkalosis.

Dehydration causes elevated hemoglobin and hematocrit. Mild unconjugated bilirubinemia occurs in 2%–5% of cases.

The urine pH is high initially but eventually drops because hydrogen ions are preferentially exchanged for sodium ions in the distal tubule of the kidney as the hypochloremia becomes severe. 

 
Ref: Hackam D.J., Grikscheit T.C., Wang K.S., Newman K.D., Ford H.R. (2010). Chapter 39. Pediatric Surgery. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwartz’s Principles of Surgery, 9e.

 


Q. 18

Which of the following is the CORRECT statement in a child presented with hypertrophic pyloric stenosis?

 A

Symptomatic within one week

 B

Lump is always clinically palpable

 C

Treatment of choice is Finney’s pyloroplasty

 D

Ultrasonography is diagnostic test

Ans. D

Explanation:

Infants with hypertrophic pyloric stenosis (HPS) present with nonbilious vomiting that becomes increasingly projectile over the course of several days to weeks.

The diagnosis of pyloric stenosis usually can be made on physical examination by palpation of the typical “olive” in the right upper quadrant and the presence of visible gastric waves on the abdomen.

When the olive cannot be palpated, ultrasonography can diagnose the condition accurately in 95% of patients.

Criteria for ultrasonographic diagnosis include a channel length of >16 mm and pyloric thickness of >4 mm.

Ramstedt pyloromyotomy is the treatment of choice and consists of incision down to the mucosa along the pyloric length.

 
Ref: Hackam D.J., Grikscheit T.C., Wang K.S., Newman K.D., Ford H.R. (2010). Chapter 39. Pediatric Surgery. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwartz’s Principles of Surgery, 9e.

Q. 19 Hypertrophic pyloric stenosis presents after how many months of birth :

 A

One

 B

Two

 C

Three

 D

Six

Ans. A

Explanation:

Pyloric stenosis is the most common surgical disorder producing emesis in infancy.

It results from hypertrophy of the circular and longitudinal muscularis of the pylorus and the distal antrum of the stomach with progressive narrowing of the pyloric canal. The cause is not known.

The male-to-female incidence is 4:1.It presents 2-4 weeks after birth.

Typically, the affected infant is full term when born and feeds and grows well until 2–4 weeks after birth, at which time occasional regurgitation of some of the feedings occurs.
 
Ref :Albanese C.T., Sylvester K.G. (2010). Chapter 43. Pediatric Surgery. In G.M. Doherty (Ed), CURRENT Diagnosis & Treatment: Surgery, 13e.

Q. 20

Which is the MOST common biochemical abnormality seen in congenital pyloric stenosis?

 A

Hyperkalemic metabolic acidosis

 B

Hypokalemic metabolic acidosis

 C

Hyperkalemic metabolic alkalosis

 D

Hypokalemic metabolic alkalosis

Ans. D

Explanation:

Neonates with congenital pyloric stenosis develop projectile postprandial vomiting between 2 and 4 weeks of age but may start as late as 12 weeks.

Due to this vomiting they develop hypokalemic hypochloremic metabolic alkalosis. 

 
Congenital pyloric stenosis is more commonly found in males.

Projectile vomiting occurring in neonate is nonbilious.

On examination, the upper abdomen gets distended after feeding, and prominent gastric peristaltic waves can be seen from left to right.

An oval mass in the shape of an olive can be palpated in the right upper abdomen, especially after vomiting.

USG done in them shows a hypoechoic muscle ring greater than 4 mm thick with a hyperdense center and a pyloric channel length greater than 15 mm. 
 
Ref: CURRENT Diagnosis & Treatment: Pediatrics, 21e chapter 21.

Q. 21 Congenital hypertrophic pyloric stenosis is characterized by progressive gastric outlet obstruction. Incidence of congenital hypertrophic pyloric stenosis is highest in:

 A

First female baby

 B

First male baby

 C

Second male baby

 D

Second female baby

Ans. B

Explanation:

Hypertrophic pyloric stenosis (HPS) is postnatal muscular hypertrophy of the pylorus.

HPS occurs in approximately 1–8 per 1000 births and originally was believed to occur in first-born males between 3 and 6 weeks of age.

Infants with HPS present with nonbilious vomiting that becomes increasingly projectile over the course of several days to weeks.
Eventually, the infant develops almost complete gastric outlet obstruction. Infants with HPS develop a hypochloremic, hypokalemic metabolic alkalosis.
 
Ramstedt pyloromyotomy is the treatment of choice and consists of incision down to the mucosa along the pyloric length.
 
Ref: Sundaram S.S., Hoffenberg E.J., Kramer R.E., Sondheimer J.M., Furuta G.T. (2012). Chapter 21. Gastrointestinal Tract. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

 


Q. 22

Which of the following metabolic derangement is associated with congenital pyloric stenosis?

 A

Hypochloremic acidosis

 B

Hypochloremic alkalosis

 C

Hyperchloremic acidosis

 D

Hyperchloremic alkalosis

Ans. B

Explanation:

Hypochloremic metabolic alkalosis is the classic metabolic finding in congenital pyloric stenosis. Other metabolic abnormalities includes elevated hemoglobin and hematocrit due to dehydration. 

 

 

 

Congenital pyloric stenosis is commonly found in male children. Infants presents with projectile postprandial vomiting usually between 2 and 4 weeks of age but may start as late as 12 weeks. Vomiting is usually non bilious. On examination, the upper abdomen may be distended after feeding, and prominent peristaltic waves are seen from left to right. Ultrasonography shows a hypoechoic muscle ring greater than 4-mm thick with a hyperdense center and a pyloric channel length greater than 15 mm.

 

 

 

Ref: CURRENT Diagnosis & Treatment: Pediatrics, 21e chapter 21, By Shikha S. Sundaram, MD .

 


Q. 23

Drug that can cause hypertrophic pyloric stenosis is?

 A

Tertacyclin

 B

Erythromycin

 C

Ampicillin

 D

Rifampicin

Ans. B

Explanation:

Ans. is ‘b’ i.e., Erythromycin

o Maternal and infant use of erythromycin and other macrolide antibiotics have been reported as risk factors for infantile hypertrophic pyloric stenosis (IHPS).


Q. 24 Congenital hypertrophic pyloric stenosis usually presents –

 A

Within 2 days after birth

 B

Around 1 week after birth

 C

Around 2 weeks after birth

 D

Around 2 months after birth

Ans. C

Explanation:

Ans. is ‘c’ i.e., Around 2 weeks after birth

  • The symptoms vomiting usually starts after 3 weeks of age but symptoms may develop as early as 1st week of life and as late as 5 months of life.  – Nelson

Congenital hypertrophic pyloric stenosis

  • Hypertrophic pyloric stenosis is the commonest surgical disorder of the stomach during infancy.
  • Pylorus is thickened and elongated and its lumen is narrowed due to hypertrophy of circular muscle fibers of pylorus.
  • More common in boys.

o More common in infants of blood group ‘B’ and ‘O’.

o Associated congenital defects are —> Tracheoesophageal fistula and hypoplasia or agenesis of inferior labial frenulum.

o May be associated with Turner syndrome, Trisomy 18.

o Symptoms are not present at birth.

The vomiting usually starts after 3 weeks of age.


Q. 25 The metabolic derrangement in congenital pyloric stenosis is –

 A

Hypochloremic alkalosis

 B

Hyperchloremic alkalosis

 C

Hyperchloremic acidosis

 D

Hypochloremic acidosis

Ans. A

Explanation:

Ans. is ‘a’ i.e., Hypochloremic alkalosis

Clinical manifestations of congenital hypertrophic pyloric stenosis

  • Pyloric stenosis is usually not present at birth.

o Nonbillous vomiting is the initial symptoms of pyloric stenosis.

  • The vomiting usually starts after 3 weeks of age, but symptoms may develop as early as the 1st week of life and as late as the 5 months of life.

o Emesis may follow each feeding, or it may be intermittent.

o After vomiting, the infant is hungry and wants to feed again.

  • Due to vomiting, there is progressive loss of —> Fluid, Hydrogen ion, Chlorides

This results in hypochloremic metabolic alkalosis.

o Serum potassium levels are usually maintained, but there may be a total body potassium deficit.

o Jaundice (unconjugated bilirubin) is associated with a decreased level of glucuronyl transferase in 5% of patients.


Q. 26 A 6 year old boy presenting with palpable abdominal mass in the epigastrium. The clinical diagnosis is (There is no bile in vomitus)-

 A

Duodenal Atresia

 B

Choledochal cyst

 C

Pyloric stenosis

 D

Oesophageal Atresia

Ans. C

Explanation:

Ans. is ‘c’ i.e., Pyloric stenosis

Diagnosis of congenital hypertrophic pyloric stenosis

o History of vomiting without bile (nonbilious vomiting).

o Physical examination

i)        Palpable mass in the epigastrium

ii)          After feeding, visible gastric peristatic wave that progresses across the abdomen.

  • USG confirm the diagnosis

 Criteria —> Pyloric thickness > 4 mm or an overall pyloric length > 14 mm.

o Contrast studies

i) Shoulder sign            A bulge of the pyloric muscle into the antrum.

ii) Double tract sign -> Parallel streaks of barium in the narrowed channel.

iii) Elongated pyloric channel.

  • So the boy is having pyloric stenosis. The only problem is age of boy i.e 6 yrs. The hypertrophic pyloric stenosis which we know of, makes its clinical presentation in early infancy (3rd to 6th weeks of life)

o But the problem in solved once you know that there is a similar disease in adults – Hypertrophic pyloric stenosis of adults. (Bailey & Love, 22th/e, p 675).


Q. 27 3 month infants with abdominal palpable mass & non bilious vomiting –

 A

Hypertrophic biliary stenosis

 B

Hypertrophic pyloric stenosis

 C

Tracheoesophageal fistula

 D

Duodenal atresia

Ans. B

Explanation:

Ans. is ‘b’ i.e., Hypertrophic pyloric stenosis

Hypertophic pyloric stenosis

  • Most common cause of nonbilious vomiting is Hypertrophic pyloric stenosis.
  • Male > female.
  • Vomiting starts with 3 week of age.
  • Palpable mass is seen in epigastric region.
  • Visible peristalsis is seen soon after feeding.
  • Confirmed by USG abdomen.
  • Contrast study shows :

Shoulder sign

Double tract sign.

  • Treatment surgery = Ramstedt procedure.

Q. 28 Regarding congenital hypertrophic pyloric stenosis all are correct except-

 A Weight loss

 B

Diarrhoea

 C

Visible peristalsis

 D

Remsted’s operation is done

Ans. B

Explanation:

Ans. is ‘b’ i.e., Diarrhea

Diarrhea does not occur in pyloric stenosis, there is vomiting.

Treatment of congenital hvpertrophic pyloric stenosis

o The surgical procedure of choice is Pyloromyotomy.

o The traditional Ramstedt procedure is performed through a short transverse skin incision.

o Other methods are —>  i) Atropine therapy         ii) Endoscopic ballon dilatation


Q. 29 A 10 days old neonate is posted for pyloric stenosis in surgery. The investigation report shows a serum calcium level of 6 mg/dL. What information whould you like to know before you supplement calcium to this neonate –

 A

Blood glucose

 B

Serum protein

 C

Serum bilirubin

 D

Oxygen saturation

Ans. B

Explanation:

Ans. is ‘b’ i.e., Serum protein

o Normal calcium level is 8.9-10.1 nig/dl (total calcium).

  • Slightly less than half of the total serum calcium exists in free or ionized form. Remainder is bound to protein (mostly albumin). Ionized calcium is relevant for cell fuction.

“There are few clinical situations in which the total calcium is not an adequate surrogate for the ionized Ca++ concentration. The most common and severe problem is the presence of hypoalbuminemia”.

o Each 1 gm/dl of albumin in the serum binds about 0.8 mg/dl of calcium.

o A low total calcium concentration may be normal in a patient with significant hypoalbuminemia.

o Now, it is clear from above that a low level of serum protein lowers the total plasma calcium but not the ionized calcium, So, before treating hypocalcemia, measure the serum protein level.


Q. 30 All correlates with USG findings of congenital pyloric stenosis except:

 A

> 95% accuracy

 B

Segment length >16mm

 C

Thickness >4mm

 D

High gastric residues

Ans. D

Explanation:

D i.e. High gastric residues

Ultrasonography is the investigation of choice to confirm diagnose of hypertrophic pyloric stenosis with accuracy > 95% (approching almost 100%)Q. USG visualizes thickened and elongated pyloric canalQ. USG criteria for diagnosis include >16 mm pyloric length and >4mm pyloric muscle wall thicknessQ. Gastric residues are low b/o recurrent emesisQ.

The hypertrophied muscle project into gastric antrum. There is a constant assocaition with hyperplasia of antral mucosa.

It is a common developmental condition (3 in 1000 live births), affecting boys more than girls (M:F = 4/5 :1)Q. There is a familial predisposition.

Affected infant usually presents between 2-6 weeks of age, with projectile non bilious vomiting (D/D include pylorospasm, hiatus hernia & preampullary duodenal stenosis). HPS is never seen beyond 3 months of age except in premature infants in whom enteral feeding has been started late.

– Despite the recurrent vomiting, child has a voracious appetite that leads to cycle of feeding & vomiting that invariably results in severe dehydration, hypochloremic­hypokalemic metabolic alkalosis with eventual decrease in urine PH

Diagnosis can be made clinically on the basis of history and palpation of an olive mass in the subhepatic region (right upper quadrant) and presence of visible gastric (antral peristaltic) wavesQ.

Diagnosis of the HPS can be established (confirmed) by either USG (method of choice)Q or barium study.

Pyloric signs include

  1. String sign, is passing of thin barium streak through narrowed & elongated pyloric canal. It is most specific sign.
  2. Pyloric canal is almost always curved upward posteriorly
  3. Double/triple track sign or double string sign is produced by barium caught between crowded mucosal folds in pyloric canal overlying the hypertrophied muscle & parallel lines may be seen.
  4. Diamond sign or twining recess is transient triangular tent like cleft/niche in midportion of pyloric canal with apex pointing inferiorly secondary to mucosl bulging between two seperated hypertrophied muscles on the greater curvature side of pyloric canal.
  5. Apple core lesion, pyloric segment looks like apple core with under cutting of distal antral & proximal duodenal bulb.

Antral signs include

  1. Pyloric teat sign is out pouching along lesser curvature b/o disruption of antral peristalsis.
  2. Shoulder sign is impression of hypertrophied muscle on distended gastric antrum.
  3. Antral beaking is noted as thick muscle narrows the barium column as it enters the pyloric canal.
  4. Olive pit sign is impression of pyloric muscle upon antrum seen as tiny amount of barium at orifice.
  5. Caterpillar sign is gastric hyperperistaltic waves.
  6. Kirklin mushroom sign is indentation of base of duodenal bulb.

Ultrasonography (USG)

It is the method of choice to directly visualize the HPS. The examination is typically performed with a high frequency linear transducer (>5MH2) (as the pylorus & duodenum are very superficial in an infant) with infant in right posterior oblique position (to move any fluid present in fundus into antral & pylorus region. The stomach should not be emptied prior to examination as this makes identification of antropyloric area difficult. If fluid is administered to make visualization better, it should be removed at the end of examination to prevent vomiting/aspiration. Features include)

  1. Doughnut appearance/Bull’s eye or target sign is hypoechoic (black) ring of hypertrophied pyloric muscle around echogenic (reflective) mucosa & submucosa on cross /transverse section images.
  2. Shoulder/cervix-sign is indentation of hypertrophied muscle on fluid filled gastric antrum on longitudinal section.
  3. Antral nipple sign is protrusion (evagination) of redundant pyloric mucosa into distended antrum.
  4. Double tract sign refers to fluid trapped in center of elongated pyloric canal is seen as two sonolucent streaks in center.
  5. Exaggerated peristattic waves & delayed gastric emptying of fluid into duodenum
  6. Elongated pylorus with thickened muscles (most specific) is indicated by Length > 15mm, muscle thickness >3mm and transverse serosa to serosa diameter >15mm is consistent with HPS. At least 2 values should be positive. A thickness pylorospasm is transient & mostly resolve in 30 minutes and there is considerable variation in measurement or image appearance with time during thickness. (GI imaging) Pyloric canal length  16-17min, muscle wall thickness 2 3-3.2mmQ, pyloric volume > 1.4cm3, pyloric transverse diameter 13mm with pyloric canal closed and length (mm) + 3.64x + 3.64 x thickness (mm) >25 (Wolfgang) Pyloric length >16mm & muscle thickness > 4mm (Swartz)

Q. 31 X-Ray feature of pyloric stenosis is

 A Single bubble appearance

 B

Double bubble appearance

 C

Triple bubble apperance

 D

Multiple air fluid levels

Ans. A

Explanation:

A i.e. Single bubble appearance


Q. 32

String sign is suggestive of:

 A

Toxic Megacolon

 B

Hypertrophic Pyloric stenosis (HPS)

 C

Ulcerative Colitis

 D

IBS

Ans. B

Explanation:

B i.e. Hypertrophic Pyloric Stenosis

Meconium ileus is small bowel obstruction secondary to sticky tenacious meconium d/ t deficiency of pancreatic secretions. It is the earliest clinical manifestation of cystic fibrosis. It usually presents with abdominal distension, bilious vomiting and failure to pass meconium within 48 hours. It has Bubbly/Froathy/Soap BubbleQ/Apple sauce appearanceQ d/t admixture of gas with meconium.

Barium meal (swallow) appearance of Oesophageal Disorders

Bird’s beak appearance (very

dilated oesophagus with

smooth narrowing at its lower

end) (barium meal)

Oesophageal

achlasia

Cock screw (undulated)

Diffuse

appearance d/t tertiary

oesophageal

contractions (barium meal)

spasm

Tiny flash shaped outpouchig

Intramural

arranged in clusters or

longitudinal rows (on barium

study).

pseudodiverticulosis

Shaggy (contour) appearance

Candida

with irregular plaque like filling

Oesophagitis

defects with intervening normal

(double contrast

mucosa (early), cobble stone or

snake skin appearance or

coalescent plaques (in late

disease) which correspond to

white plaques seen in

endoscopy; Foamy oesophagus

(tiny 1-3 mm bubble like

lucencies producing foamy

appearance).

barium)

Shelf like indentation from

Cricopharyngeal/

anterior wall or well localized

oesophageal web

circumferential narrowing (at

(when a/w Fe

C5/C6) with minimal proximal

dilatation and jet effect (on

deficiency anemia,

glossits &

barium swallow)

pharyngeal atrophy

it is k/a Plummer

 

Vinson Syndrome)

Lucent, tortuous, worm like

Esophageal varices

filling defectss which distort the

(dilated subepithelial

mucosal pattern (on barium

veins d/t increased

swallow); thickened longitudinal

collaterals). Uphill

esophageal folds with fusiform

varices (d/t portal

seperation (on mucosal spot films);

hypertension) are

beaded or serpiginous

confined to distal half

longitudinal filling defects (best

& down hill varices

seen on mucosal relief views of

(d/t SVC obstruction)

collapsed oesophagus in prone RAO

involve upper &

position with high density barium

middle part of

paste/suspension to increase

oesophagus

mucosal adherence).

Endoscopy is

* Extraluminal compression

investigation of

makes a shllow angle with wall

of oesophagus.

choice

Smooth intramural filling defect

Leiomyoma of

with characteristic features of

submucosal lesion i.e. smooth,

rounded, semilunar indentation that

makes a sharp angle with the wall

of oesophagus with intact

mucosa, splitting/narrowng the

barium column, & widening the

oesophageal diameter and

splaying longitudinal esophageal

folds.

esophagus

Annular apple core lesion with

Carcinoma

overhanging margins (or

irregular stricture with

Oesophagus

 
 

Radiological features

Disorders

Beak sign; Double track or Tram track

sign, Shoulder sign; String sign

Hypertrophic

pyloric stenosis

Seagull /Mercedez Benz/ Crow Feet Sign

Radiolucent gall

stone with gas

Multiple gas fluid level (step ladder

pattern)

Intestinal

obstruction

Cigar bundle appearance on X-ray

Winding highway railway tract on USG

Medusa Head coloniesQ on CT scan

Round worm

Pincer sign, Claw appearance sign( 2,

Coiled spring appearanceQ, Target sign,

Meniscus sign (on barium enema)

Intussception

Shark mouth appearance

Ileocecal valve

Lead pipe appearanceQ

Ulcerative colitis

String of KantorQ, Bull’s eye or Target

lesion, Ram’s horn stomach (narrow

antrum, wide pylorus with flared up

tubular antrum opening into corpus of

stomach)

Chron’s disease

(Regional ileitis)

Thumb printing signQ

Ischemic colitis

(hallmarkQ) also in

Amoebic &

Ulcerative colitis

Saw tooth appearanceQ (on barium enema)

Diverticula of colon

(Diverticulosis)

 

Apple core lesion, Napkin’s sign (on

barium enema)

Carcinoma colon

Bilious vomiting in 1st month (hallmark),

current jelly stools/ malena with double

Midgut volvulus

(torsion of gut

bubble sign (x-ray); clockwise whirlpool

sign (USG), apple-peel/twisted

ribbon/Corckscrew & spiral

appearance (barium study); whirl like

pattern (CT); barber pole sign (angio)

around SMA)

Whirl sign (CT); bird of prey sign

(tapered beak like distal aspect of twist

on barium enema); white stripe sign &

three line sign (respectively d/t

obliquely oriented center line/ outerwall

Sigmoid volvulus

& center-line of U shaped loop),

northern exposure/bent tyre tube sign

(greatly distended paralyzed loop with

fluid-fluid level arising from pelvis &

extending towards diaphragm), Coffee

bean sign (midline crease

corresponding to mesentry of largely

gas distended loop)- on plain X-ray.

 

Distension of small bowel & colon

Necrotizing

(loops wider than L1 vertebral body ±

enterocolitis

air fluid level in right lower quadrant is

(ischemic bowel

(1st & most common sign), loss of mosaic

disease of

multifaceted bowel gaa pattern, large bowels

may be impossible to differentiate from small

bowel, fixed bowel or persistent loop

sign, bowel wall thickening, thumb

printingQ, pneumatosis intestinalisQ

(black lines of intramural gas a/b white

lines of intestinal wall), bubbly

appearance of bowel wall, gas in portal

venous system (plain horizontal beam

cross table x-ray); zebra pattern; Y

pattern & ring pattern (on USG)

premature infants)

 

shouldering) on barium

 

Adynamic ileus pattern, nodular

Pseudomembra‑

oesophagogram; other features

 

haustral pattern (thumb printing –

nous colitis

may be irregular narrowed segment

 

transverse banding = markedly

(Clostridium

of lumen, diffuse nodular filling

 

thickened & distorted haustral folds

difficile diseas)

defects, irregular /smooth

 

most prominent in transvere colon) -on

 

tapering strictue, bulky

 

plain x-ray; accordion sign (oral

 

endophytic mass and fixed

 

contrast traps between distorted

 

varicoid appearance (i.e. b/o

 

thickened closely spaced transvere

 

submucosal spread of tumor, the

 

edematous folds of low atenuation; is

 

appearance may mimic that of

varices, however, unlike varices it

 

typical but seen only in severe cases),

target sign and rarely pneumatosis coli

 

is fixed & does not change in size

 

± portal vein gas – on CT; barium enema

 

& shape at fluoroscopy)

 

is contraindicated

 

 

Bowler’s hat sign, lace like/reticular

surface pattern (characteristic of villous

adenoma >> tubular adenoma)

Colonic polyp

 
 
 

Q. 33 All are false except one in case of hypertrophic pyloric stenosis –

 A Symptomatic within one week

 B

Lump is always clinically palpable

 C

T/t of choice is Finney’s pyloroplasty

 D

Ultrasonography is diagnostic test

Ans. D

Explanation:

Ans is ‘d’ ie Ultrasonography is diagnostic test

Hypertrophic Pyloric Stenosis (HPS)

  • The musculature of the pylorus and adjacent antrum is grossly hypertrophied, the hypertrophy being maximum in the pylorus itself.
  • It occurs in approximately 3 in 1000 live birth and is the most common surgical cause of vomitting in infancy.
  • M > F (4 : 1)*
  • Characterstically the first born male child is affected.
  • The conditon is most commonly seen at 4 wks after birth ranging from the 3rd wk to on rare occasion, the 7th. However, 20% of infants are symptomatic from birth, and most are symptomatic within the first 2 months after birth.
  • Non-bilious vomiting, becoming increasingly projectile, occurs over several days to weeks. Eventually the infant will develop a nearly complete obstruction by the second to fourth week of life and will not be able to hold down even clear liquids. This invariably proceeds to severe dehydration if not t/t.
  • These infants develop a metabolic alkalosis with severe depletion of potassium and chloride ions.
  • ” The diagnosis of pyloric stenosis usually can be made on physical examination by palpation of the typical ‘olive’ in the right upper quadrant and the presence of visible gastric waves on the abdomen.
  • When the olive cannot be palpated, ultrasound in experienced hands will diagnose the condition accurately in 95 percent of pts.” – Schwartz.

Treatment

  • Pyloric stenosis is never a surgical emergency although dehydration and electrolyte abnormalities may present a medical emergency
  • Fluid resuscitation and correction of electrolyte abnormalities and metabolic alkalosis is essential before surgery.
  • Surgery : Fredet-Ramstedt pyloromyotomy. (In it the pyloric mass is split without cutting the mucosa)

Also know

  • Administration of erythromycin in early infancy has been linked to the subsequent development of HPS.
  • Jaundice may be seen in HPS, although cause is not clear.

Q. 34 Which among the following is not a ultrasound feature of Congenital Hypertrophic Pyloric Stenosis?

 A 95% sensitivity by ultrasound

 B

Thickness of pylorus > 4mm

 C

Canal length > 16mm

 D

High gastric residue

Ans. D

Explanation:

Ans is ‘d’ i.e. High gastric residue 

Ultrasound is the investigation of choice. It can diagnose HPS accurately in 95% of patients. Criteria for ultrasound diagnosis of HPS:

a.     channel length of > 16mm

b.  pyloric thickness of > 4mm

“The diagnosis of pyloric stenosis usually can be made on physical examination by palpation of the typical “olive” in the right upper quadrant and the presence of visible gastric waves on the abdomen. When the olive cannot be palpated, ultrasonography can diagnose the condition accurately in 95% of patients. Criteria for ultrasonographic diagnosis include a channel length of >16 mm and pyloric thickness of >4 mm. “- Schwartz


Q. 35 What is the most characteristic of congenital hypertrophic pyloric stenosis

 A

Affects the first born female child

 B

The pyloric tumour is best felt during feeding

 C

The patient is commonly marasmic

 D

Loss of appetite occurs early

Ans. B

Explanation:

Ans. is ‘b’ i.e., The pyloric tumor is best felt during feeding

Option (a)

– HPS classically presents in a first born male child between 3 and 6 weeks of age. However, children outside this age range also are commonly seen, and the disease is not restricted to either males or first borne children.

Option (d)

–  After vomitting the child has voracious appetite, that leads to a cycle of feeding and vomiting.

Option (c)

“Greater awareness of pyloric stenosis has led to earlier identification of patients with fewer instances of chronic malnutrition and severe dehydration” -Nelson I7/e

Option (b)

The diagnosis of pyloric stenosis is usually made by palpation – of the typical olive shaped hard pyloric mass located in right upper quadrant. In healthy infants when palpation is difficult, feeding can help in the diagnosis. After feeding gastric peristalsis waves may be visible. After the infant vomits, the abdominal musculature is more relaxed and the mass is easier to palpate. Sedation can also be used to facilitate examination but is usually unnecessary. In cases of doubt ultrasound can confirm the diagnosis.


Q. 36 A robust male baby with vigorous feeding and immediate vomiting at 2 months of age. Most probable diagnosis is:

 A

Paralytic ileus

 B

Hirschsprung’s disease

 C

Brain tumor

 D

Congenital hypertrophic pyloric stenosis

Ans. D

Explanation:

Ans is ‘d’ i.e. Congenital hypertrophic pyloric stenosis 


Q. 37

In a case of hypertrophic pyloric stenosis, the metabolic disturbance is

 A

Respiratory alkalosis

 B

Metabolic acidosis

 C

Metabolic alkalosis with paradoxical aciduria

 D

Metabolic alkalosis with alkaline urine

Ans. C

Explanation:

Ans. is ‘c’ i.e., Metabolic alkalosis with paradoxical aciduria 

  • Repetitive vomiting leads to hypochloremic, hypokalemic metabolic alkalosis. The urine is alkaline initially but eventually it becomes acidic (paradoxical aciduria)
  • Cause of paradoxical aciduria

– Initially the kidney compensate for metabolic alkalosis by reabsorbing hydrogen and chloride ions in exchange for sodium, potassium and bicarbonate. But gradually as the body stores of sodium and especially potassium become depleted, kidney starts retaining these ions in exchange of hydrogen ions. thus the urine becomes acidic and metabolic alkaline state is further aggravated.


Q. 38

What is true regarding congenital hypertrophic pyloric stenosis –

 A

More common in gilrs

 B

Hypochloremic alkalosis

 C

Hellers myotomy is the procedure of choice

 D

Most often manifests at birth

Ans. B

Explanation:

Ans. is ‘b’ i.e., Hypochloremic alkalosis 

Heller’s myotomy is the surgery for Achlasia Cardia, not HPS.


Q. 39

Clinical features of pyloric stenosis in infants are All / Except

 A

Abdominal lump

 B

Constipation

 C

Weight loss

 D

Metabolic acidosis

Ans. D

Explanation:

Ans. is ‘d’ i.e. metabolic acidosis 

  • Metabolic alkalosis, not acidosis is seen in hypertrophic pyloric stenosis due to loss of hydrogen ions in repeated vomiting.
  • As the hypertrophic pyloric stenosis progresses, complete gastric outlet obstruction occurs and not even clear fludis are able to pass. This leads to constipation or passage of small green “starvation” stools.
  • Rapid weight loss is seen if the condition is not detected early and treated.

Q. 40 Congenital pyloric stenosis causes –

 A

Billious vomiting

 B

Non-billious vomiting

 C

Projectile vomiting

 D

b and c

Ans. D

Explanation:

Answer ‘b’ i.e. Non-bilious vomitting ; ‘c’ i.e. Projectile vomitting 

  • The vomiting may or may not be projectile, however non-bilious vomiting after feed is the characteristic feature.
  • In about 10-15% of infants vomitus may contain blood. This bleeding is due to reflux esophagitis, a common association with hypertrophic pyloric stenosis.
  • Also know
  • Associated anomalies occur in 6-20% of pts. This includes esophageal atresia, Hirshprung’s disease, anorectal anomalies, and intestinal malrotation.

Q. 41 Features of pyloric stenosis :

 A

Hypokalemic alkalosis

 B

Peristalsis right to left

 C

Commonly caused by carcinoma stomach

 D

Retention vomiting present

Ans. A

Explanation:

Ans. is ‘a’ i.e. hypokalemic alkalosis 

  • Gastric peristalsis is seen passing from left to right across the upper abdomen.
  • Retention vomiting means vomiting due to any mechanical obstruction occurring usually hours after ingestion of a meal. In hypertrophic pyloric stenosis the vomiting usually occurs 10 to 30 min. after the feed (Though in neglected undiagnosed cases the stomach becomes dilated and the vomiting becomes regurgitant in type occurring only once or twice every 24 hrs)

Q. 42 True about pyloric stenosis –

 A

Hypokalemia

 B

Hyponatremia

 C

Metabolic acidosis

 D

a and b

Ans. D

Explanation:

Answer ‘a’ i.e. Hypokalemia; ‘b’ i.e. Hyponatremia


Q. 43

Which is Not true regarding Hypertrophic pyloric stenosis –

 A

Ultrasound is not useful for diagnosis

 B

Usually presents between 2 – 8 weeks

 C

Common in males

 D

Dilated stomach with minimal gas

Ans. A

Explanation:

Ans is ‘a’ ie. U/S is not useful for diagnosis

  • Option A is absolutely wrong statement as U/S is the inv. of choice for hypertrophic pyloric stenosis.
  • Already explained in Previous question.

Q. 44

Congenital hypertrophic pyloric stenosis associated with

 A Hypokalemic acidosis

 B

Hypokalemic alkalosis

 C

Hyperkalemic acidosis

 D

Hyperkalemic alkalosis

Ans. B

Explanation:

Ans is ‘b’ i.e. Hypokalemic alkalosis 


Q. 45

A 3 week old patient presenting with vomiting and failure to thrive is found to have pyloric stenosis. What should be the next step of management?

 A

its emergency so do pyloromyotomy immediately

 B

fluid resuscitation may be delayed

 C

correction of electrolyte disturbances

 D

Cardiopulmonary resuscitation

Ans. C

Explanation:

Ans is ‘c’ i.e. Correction of electrolyte disturbances 

Pyloric stenosis is never a surgical emergency although dehydration and electrolyte abnormalities may present a medical emergency

Fluid resuscitation and correction of electrolyte abnormalities and metabolic alkalosis is essential before surgery. Surgery done is Fredet-Ramstedt pyloromyotomy. (In it the pyloric mass is split without cutting the mucosa)


Q. 46

Make the diagnosis of a 26 day old Infant presenting with recurrent nonbilious vomiting with costipation and loss of wt‑

 A Oesophageal atresia

 B

Choledochal cyst

 C

Ileal atresia

 D

Pyloric stenosis

Ans. D

Explanation:

Ans is ‘d’ ie Pyloric stenosis

  • This is a typical picture of pyloric stenosis

a)       Infant presenting in 4th week

b)       Non-bilious vomitting.

c)       With constipation and wt. loss.

  • Lets see other options.
  • Esophageal atresia

a)     Infant presents soon after birth with complaints of regurgitation of milk, saliva pours almost continuously from its mouth. Attempt to feed are met with ‘Spitting up’ or frank vomitting.

b)     Persistence in feeding may produce aspiration, choking and respiratory distress.

c)       Diagnosis can be confirmed by passing a rubber catheter into the esophagus through the mouth which will encounter an obstruction.

Thus point (a) helps in ruling out oesophageal atresia.

  • Choledochal cyst

a)       Presentation is not so early in infancy. Though its a congenital anomaly only 1/2 the cases present before 20yrs. of age and only 1/3 present in the 1st decade of life.

b)       Classical triad of symptoms —>            

i)        Obstructive jaundice *

ii)       Upper abdominal pain*

iii)     Fever*

(c) A palpable epigastric mass may be discovered.

  • Beal atresia

a)       Vomitting will be bilious

b)       Infant presents in the 1st week of life.


Q. 47

In congenital pyloric stenosis the defect usually lies in – antrum –

 A Nerve fibres

 B

Circular muscle fibres

 C

Longitudinal muscle fibres

 D

Mucosa

Ans. B

Explanation:

Ans. is ‘b’ i.e., Circular muscle fibres 


Q. 48

Hypochloremia, hypokalemia and alkalosis are seen in‑

 A

Congenital hypertrophic pyloric stenosis

 B

Hirschsprung’s disease.

 C

Esophageal atresia

 D

Jejunal atresia

Ans. A

Explanation:

Ans is ‘a’ i.e. Congenital hypertrophic pyloric stenosis

Biochemical abnormality in congenital hypertrophic pyloric stenosis is a regular feature of AIIMS and AI examinations it has been repeated several times.

  • The biochemical abnormalities seen are:

( I ) Hypokalemia               (3) Alkalosis and

(2) Hypochloremia              (4) Paradoxical aciduria


Q. 49 Condition which does not cause metabolic acidosis‑

 A

Renal failure

 B

Ureterosigmoidostomy

 C

Pancreatic or biliary fistula

 D

Pyloric stenosis

Ans. D

Explanation:

Ans. is ‘d’ i.e., Pyloric stenosis 


Q. 50

Hypochloremic alkalosis is a complication of ‑

 A

Congenital pyloric stenosis

 B

Vomiting

 C

Haematemesis

 D

a and b

Ans. D

Explanation:

Ans. is ‘a’ i.e. Congenital pyloric stenosis & ‘b’ i.e. Vomiting 


Q. 51

The commonest cause of metabolic alkalosis is ‑

 A

Cancer stomach

 B

Pyloric stenosis

 C

Small-bowel obstruction

 D

Diuretics

Ans. B

Explanation:

Ans. is ‘b’ i.e., Pyloric stenosis 


Q. 52

Ramsted’s operation is for –

 A

Duodenal atresia

 B

Volvulus of small intestine

 C

Anorectal malformation

 D

Congenital pyloric stenosis

Ans. D

Explanation:

Ans. is ‘d’ i.e., Congenital pyloric stenosis 


Q. 53

What is the most characteristic of congenital hypertrophic pyloric stenosis:

 A

Affects the first born female child

 B

The pyloric tumour is best felt during feeding

 C

The patient is commonly marasmic

 D

Loss of appetite occurs early

Ans. B

Explanation:

Answer is B (The pyloric tumor is best felt during feeding)

  • First born male child is characteristically most commonly affected
  • It is four times more common in males as in females .
  • The condition does not present at birth. It is most commonly seen 4 weeks after birth

Presentation

  • Vomiting is the presenting symptom (child vomits milk and no bile is presentQ)
  • Immediately after vomiting the child is hungry i.e. loss of appetite does not occur.
  • Weight loss is striking and rapidly the infant becomes emaciated and dehydrated.

However, greater awareness of pyloric stenosis has led to earlier identification of patients and hence with fewer incidences of chronic malnutrition and severe dehydration – Nelson 10th/1130

  • The diagnosis is usually made with a test feed : In this the baby is fed with the bottle by a nurse or mother and surgeon :

palpates the abdomen with a warm hand to detect the lump

– observes the characteristic peristatic waves pass across the upper abdomen.

  • Pathologically musculature of pylorus adjacent to antrum is grossly hypertrophied
  • Ultrasonographv is the investigation of choice
  • Hyperchloremic alkalosis is common and following diagnosis first concern is to correct metabolic abnormalities -child is rehydrated with dextrose saline & potassiumQ
  • Treatment of choice Ramsted’s operation – ‘Pyloromyotomy’

Q. 54 What is true regarding congenital hypertrophic pyloric stenosis :

 A More common in girls

 B

Hypochloremic alkalosis

 C

Hellers myotomy is the procedure of choice

 D

Most often manifests at birth

Ans. B

Explanation:

The answer is B (Hypochloremic alkalosis) :

The vomiting of hydrochloric acid results in hypochloremia and alkalosis i.e. hypochloremic alkalosis.

Characteristically firstborn male infant is most commonly affected (Males > Females) 

The onset of symptoms has its, peak incidence between the third & sixth weeks of life and it is rarely present at birth.

The most common presenting symptom is vomiting & accounts for the hypochloremic alkalosis.

The procedure of choice is a Halsted’s operation & not a Heller’s myotomy.

– Heller myotomy is the procedure of choice for achalasia cardia.


Q. 55 X-ray feature of pyloric stenosis is:           

March 2013 (c)

 A Single bubble appearance

 B

Double bubble appearance

 C

Triple bubble appearance

 D

Multiple bubble appearance

Ans. A

Explanation:

Ans. A i.e. Single bubble appearance


Q. 56

In a case of hypertrophic pyloric stenosis, the meta­bolic disturbance is:  

March 2004

 A

Respiratory alkalosis

 B

Metabolic acidosis

 C

Metabolic alkalosis with paradoxical aciduria

 D

Metabolic alkalosis with alkaline urine

Ans. C

Explanation:

Ans. C i.e. Metabolic alkalosis with paradoxical aciduria


Q. 57

Which of the following is correct regarding congenital hypertrophic pyloric stenosis:       

March 2004

 A

Presence of palpable lump

 B

Characteristically affects first born female infants

 C

Spurious diarrhoea is the presenting feature

 D

Bile will be present in the vomitus

Ans. A

Explanation:

Ans. A i.e. Presence of palpable lump


Q. 58

Regarding congenital hypertrophic pyloric stenosis, all are correct EXCEPT:        

September 2012

 A

Weight loss

 B

Diarrhoea

 C

Visible peristalsis

 D

Ramstedt’s operation done

Ans. B

Explanation:

Ans. B i.e. Diarrhoea

Congenital hypertrophic pyloric stenosis

  • Presents about 4 weeks after birth,
  • Weight loss,
  • Hypochloremic alkalosis,
  • Fluid of choice: Isotonic normal saline

Q. 59 Metabolic disturbance seen with pyloric stenosis:

March 2007, March 2013 (a, b, d, e)

 A Hypokalemic hyponatremic alkalosis

 B

Hyperkalemia

 C

Hyperchloremic acidosis

 D

Hypernatremia

Ans. A

Explanation:

Ans. A: Hypokalemic hyponatremic alkalosis


Q. 60

In pyloric stenosis, the following changes are seen:

 A

Hypokalemic hyponatremic alkalosis

 B

Hyperkalemia

 C

Acidosis with hyponatremic alkalosis

 D

Hyperchloremic acidosis

Ans. A

Explanation:

Ans. A: Hypokalemic hyponatremic alkalosis

  • The vomiting of hydrochloric acid results in hypochloremic alkalosis. Initially, the sodium and potassium may be relatively normal. However, as dehydration progresses, more profound metabolic abnormalities arise, partly related to renal dysfunction.
  • Initially, the urine has a low chloride and high bicarbonate content reflecting the primary metabolic abnormality.
  • This bicarbonate is excreted along with sodium, and so with time, the patient becomes hyponatremic and more profoundly dehydrated.
  • Resulting in the urine becoming paradoxically acidic and hypokalaemia ensues. Alkalosis leads to a lowering in the circulating ionized calcium, and tetany can occur.

Q. 61 Hypochloremic alkalosis is seen in:       

March 2009

 A

Achalasia cardia

 B

Congenital hypertrophic pyloric stenosis

 C

Ureterosigmoidostomy

 D

Diarrhea

Ans. B

Explanation:

Ans. B: Congenital hypertrophic pyloric stenosis

Blood tests in congenital pyloric stenosis reveals hypokalemic, hypochloremic metabolic alkalosis due to loss of gastric acid (which contain hydrochloric acid and potassium) via persistent vomiting; these findings can be seen with severe vomiting from any cause.

The potassium is decreased further by the body’s release of aldosterone, in an attempt to compensate for the hypovolaemia due to the severe vomiting.

Chronic hyperchloraemic metabolic acidosis occurs secondary to ureterosigmoidostomy.


Q. 62 X-ray feature of pyloric stenosis is:           

March 2013

 A Single bubble appearance

 B

Double bubble appearance

 C

Triple bubble appearance

 D

Multiple bubble appearance

Ans. A

Explanation:

Ans. A i.e. Single bubble appearance


Q. 63

String sign is seen in:

 A

Crohn’s disease

 B

TB of the ileocaecal region

 C

Idiopathic hypertrophic pyloric stenosis

 D

All of the above

Ans. D

Explanation:

Ans. D. All of the above

String sign in radiology seen-
– Crohn’s disease
– Tuberculosis
– Hypertrophic pyloric stenosis


Q. 64

Diagnose the condition based on the sign marked by an arrow in the photograph below ? 

 A

Stomach carcinoma.

 B

Ileocaecal TB.

 C

Ulcerative colitis.

 D

Hypertrophic pyloric stenosis.

Ans. D

Explanation:

The gastrointestinal string sign (string sign of Kantour) refers to the string-like appearance of a contrast-filled bowel loop caused by severe narrowing of a bowel loop.

Originally used to describe the reversible narrowing caused by spasms in Crohn disease, it is now used for any severe narrowing of the bowel lumen, including that seen in hypertrophic pyloric stenosis, gastrointestinal tuberculosis, carcinoid tumorand colon cancer.



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