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Pediatrics > Hershprug > Flashcards

Hershprug Flashcards

1
Q

If the neonate is vomitting after delivery the obstruction is (proximal or distal)

A

Proximal

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2
Q

What is the characteristic of hershspurge disease

A

Absence of ganglion cell

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3
Q

Males\females more likely to develop hershspurge disease:

A

Males

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4
Q

How does aganglionosis occur in hershprurge disease?

A

At 5th week of neonatal development, the migration of neural crest occur from the eosphagus craniocaudally to the anus.

If it cease at any point there will be aganglionosis.

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5
Q

What is the transitioning zone, the normal zone, abnormal zone in hershpsurge disease

A

Transition zone: area between normal and abnormal; abnormal number of ganglion “must be resected”
Normal: hypertrophy.
Abnormal: narrowing.

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6
Q

What are the different variations of hershpsurge

A

Rectal, Rectosegmoid (most common), long segment, total colon, extensive (involvement of small bowel)

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7
Q

How does hershspurge disease usually present

A
  • Delayed passage of meconium at 48 hours
  • constipation, distended abdomen
  • neonate won’t pass stool unless you put suppository or massage to overcome the narrowing.
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8
Q

What is enterocolitis

A

Constipation followed by Explosive diarrhea

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9
Q

In children with hershspurge disease, what additional features to expect

A

Anemia, caxhexia, anorexia.

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10
Q

What is the sign you’ll find in hershspurge disease when you put your finger in the rectum.

A

Exclusive evacuation of gas

“Blast”

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11
Q

How is eneterocolitis appearing in the x-ray?

A
  • intramural gas
  • thickning of bowel wall mucosa
  • dilated colon loop
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12
Q

What are the precaution to do before contrast enema

A

No suppository, no rectal wash, no PR examination at least 24 hours

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13
Q

Funneling in x-ray of child with constipation indicate

A

The transition zone for hershspurge disease

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14
Q

Delineation of 3 zones can be more clear in:

- neonates or older children?

A

Older children

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15
Q

Anorectal manometery finding in hershspurge?

A

.

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16
Q

What is the gold standard diagnosis of hershspurge?

A

Rectal biopsy

Thick nerve fibers, absent ganglion, +be ACE

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17
Q

DDx of delayed passage of meconium.

A

,

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18
Q

How to medically manage hershepurge?

A
  • insert NGT
  • rectal wash to evacuate the stool.
  • …
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19
Q

What is the surgical treatment of hershspurge diseasse

A

immediate pull-through

Swanson - soave - duhamel

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20
Q

Swanson method & drawback.

A

Remove the whole disease part and anastomose.

The nerve & sphincter may be affected

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21
Q

Soave method

A

Leave the muscle and nerve, pull the abnormal submucosa

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22
Q

Duhamel method

A

Donesn’t remove the diseased part, leave the anterior wall of the diseased as a reservoir “no contraction”, he pulls the normal behind it “contraction”

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23
Q

What is the aim of the pullthrough surgeries

A

Remove the diseased section of your child’s intestine and then pull the healthy portion of this organ down to the anus.

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24
Q

When to opt for the staged colostomy?

A
  • severe enterocolitis
  • ..
  • ..
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25
Q

Anorectal malformation, the septum divides the structure into anterior and posterior. These are.

A

Anterior: urogenital sinus - endoderm
Posterior: (hind gut) anorectal canal - ectoderm

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26
Q

What are the anorectal malformations in males?

A
  • Peineal fistula
  • anal stenosis
  • imperforated anus
  • rectouretheral fistula
  • rectovisical fistula
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27
Q

What are the anorectal malformations in females.

A
  • Perineal fistula
  • vestibular fistula
  • cloaca
  • anal stenosis
  • imperforated anus
28
Q

Single opening in the female “anorectal malformation” is called:

A

Cloaca

29
Q

Why do we have to wait 24hrs before adding the stoma in anorectal malformation?

A

.

30
Q

Pearl appearance in anorectal malformation is which type?

A

Perineal fistula

31
Q

Meconium ileum is associated with which disease?

A

Cystic fibrosis

32
Q

How to manage meconium plug and what is the outcome?

A

By evacuation, the baby will be fine

33
Q

In meconium plug and meconium atresia, how does the meconium appear?

A

Thick

34
Q

What is the most common chromosomal abnormalities associated with hershspurge disease?

A

Down syndrome

35
Q

Why is there a hypertrophied non-milenated nerves in the histopathology of hershspurge disease?

A

Because they try to overcome the obstruction

36
Q

You took a biopsy of hershspurge disease patient, what do you expect to see?

A
  • reduced ganglion cells in transition zone
  • abscence ganglion in myentric\submucosa of the diseased zone
  • hypertrophied non-mylinated nerve track of the healthy zone
37
Q

With progression of hershspurge disease, what could be the complications in neonates

A
  • perforation
  • peritonitis
  • enterocolitis
  • sepsis
38
Q

How to diagnose infants with hershspurge disease?

A

Infant with chronic constipation and inability to pass stool for 6-7 days
Unless the mother uses suppository

39
Q

How does enterocolitis usually develop in hershspurge disease?

A

Stagnation of stool will cause transmural inflammation.

40
Q

What are the male classification of anorectal malformation?

A
  • Anal stenosis
  • Recto-uretheral fistula & Recto-vesicular fistula
  • perenial fistula
  • imperforate anus without fistula
41
Q

What we will see in general in rectovesical fistula

A

Meconium through urine

42
Q

What is imperforate anus without fistula

A

No connection between rectum and other part of body

43
Q

What are the female classification of anorectal malformation?

A
  • preineal fistula
  • vestibular fistula
  • Cloaca
  • anal stenosis
  • imperforate anus without fistula
44
Q

What is cloaca?

A

Rectum + urethera

45
Q

What is perineal fistula?

A

Connection between rectum and perineum (But abnormal opening)

46
Q

What determines the prognosis of recto-vistibular fistula

A

Poor prognosis with flat buttock

47
Q

What is rectovistublar fistula?

A

3 openings within vestibule

48
Q

How does cloaca appear?

A

Single opening like tube

49
Q

If you see no anal opening, what will you do next and how will diagnose it?

A

I will wait 24 hours for the boy to pass meconium, there could be a perenial fistula

50
Q

Scrotal fistula is

A

An example of rectoperineal fistula

51
Q

Name low anomalies that are associated with good prognosis

A

1- bucket handle malformations
2- string of pearls
3- rectouretheral fistula

52
Q

What determines low anomaly

A

Lowr than the levator ani muscle

53
Q

what other associated anomalies with anorectal maformation?

A

VaCTERL

  • vertebra “spine sarcum”
  • anorectal
  • cardiac
  • throaco-eosphagel
  • Renal
  • lower extermity
54
Q

Iliac crest and last ossified bone, normal raito

A

0.77

55
Q

What is the initial management of a child with spine sacrum anomaly

A
  • NPO
  • IVF
  • NGT
  • AB
56
Q

What is the name of investigation of sacrum spine anomaly?

A

Cross table with pillow

57
Q

How to consider it as a high or low anomaly in sacral spine?

A

Las ossified bone and gas

more gas = high anomaly

58
Q

What is the most important classical surgical procedure in anorectal malformation?

A

Posterior saggital anorectoplast

59
Q

What is the principle method for treating anorectal maformation

A

Find the distal part of rectum > devide fistula > mobilize rectum into the muscle sling

60
Q

What is the post operative care for anorectal malformation

A

1- AB
2- foley’s
3- anal dilation to avoid formation of fibrosis

61
Q

What are the indications of colostomy?

A

High anomalies + clacoa

62
Q

What are the complications of stoma?

A

Prolapse - retraction - herniation

63
Q

What are the complication of stoma?

A

1- proximal: defect absorption, acidosis, renal defect
2- loop: leakage causing UTI
3- short: won’t be able to pull it
4- very high: recurrent infection due to disuse colon

64
Q

Between colostomy and posterior saggital anorectoplasty, what should you do?

A

Contrast on the stoma

It will show if there’s fistula

65
Q

What are the complication of posterior saggital anorectoplasty after stoma

A 
1- wound infection
2- anal stricture
3- recurrent rectouretheral fistula
4- fibrosis and retraction of vagina 
5- uretheral injury 
6- vas deferens

Pediatrics (41 decks)

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