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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
Anorectal malformation, the septum divides the structure into anterior and posterior. These are.
Anterior: urogenital sinus - endoderm Posterior: (hind gut) anorectal canal - ectoderm
26
What are the anorectal malformations in males?
- Peineal fistula - anal stenosis - imperforated anus - rectouretheral fistula - rectovisical fistula
27
What are the anorectal malformations in females.
- Perineal fistula - vestibular fistula - cloaca - anal stenosis - imperforated anus
28
Single opening in the female “anorectal malformation” is called:
Cloaca
29
Why do we have to wait 24hrs before adding the stoma in anorectal malformation?
.
30
Pearl appearance in anorectal malformation is which type?
Perineal fistula
31
Meconium ileum is associated with which disease?
Cystic fibrosis
32
How to manage meconium plug and what is the outcome?
By evacuation, the baby will be fine
33
In meconium plug and meconium atresia, how does the meconium appear?
Thick
34
What is the most common chromosomal abnormalities associated with hershspurge disease?
Down syndrome
35
Why is there a hypertrophied non-milenated nerves in the histopathology of hershspurge disease?
Because they try to overcome the obstruction
36
You took a biopsy of hershspurge disease patient, what do you expect to see?
- 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
With progression of hershspurge disease, what could be the complications in neonates
- perforation - peritonitis - enterocolitis - sepsis
38
How to diagnose infants with hershspurge disease?
Infant with chronic constipation and inability to pass stool for 6-7 days Unless the mother uses suppository
39
How does enterocolitis usually develop in hershspurge disease?
Stagnation of stool will cause transmural inflammation.
40
What are the male classification of anorectal malformation?
- Anal stenosis - Recto-uretheral fistula & Recto-vesicular fistula - perenial fistula - imperforate anus without fistula
41
What we will see in general in rectovesical fistula
Meconium through urine
42
What is imperforate anus without fistula
No connection between rectum and other part of body
43
What are the female classification of anorectal malformation?
- preineal fistula - vestibular fistula - Cloaca - anal stenosis - imperforate anus without fistula
44
What is cloaca?
Rectum + urethera
45
What is perineal fistula?
Connection between rectum and perineum (But abnormal opening)
46
What determines the prognosis of recto-vistibular fistula
Poor prognosis with flat buttock
47
What is rectovistublar fistula?
3 openings within vestibule
48
How does cloaca appear?
Single opening like tube
49
If you see no anal opening, what will you do next and how will diagnose it?
I will wait 24 hours for the boy to pass meconium, there could be a perenial fistula
50
Scrotal fistula is
An example of rectoperineal fistula
51
Name low anomalies that are associated with good prognosis
1- bucket handle malformations 2- string of pearls 3- rectouretheral fistula
52
What determines low anomaly
Lowr than the levator ani muscle
53
what other associated anomalies with anorectal maformation?
VaCTERL - vertebra “spine sarcum” - anorectal - cardiac - throaco-eosphagel - Renal - lower extermity
54
Iliac crest and last ossified bone, normal raito
0.77
55
What is the initial management of a child with spine sacrum anomaly
- NPO - IVF - NGT - AB
56
What is the name of investigation of sacrum spine anomaly?
Cross table with pillow
57
How to consider it as a high or low anomaly in sacral spine?
Las ossified bone and gas | more gas = high anomaly
58
What is the most important classical surgical procedure in anorectal malformation?
Posterior saggital anorectoplast
59
What is the principle method for treating anorectal maformation
Find the distal part of rectum > devide fistula > mobilize rectum into the muscle sling
60
What is the post operative care for anorectal malformation
1- AB 2- foley’s 3- anal dilation to avoid formation of fibrosis
61
What are the indications of colostomy?
High anomalies + clacoa
62
What are the complications of stoma?
Prolapse - retraction - herniation
63
What are the complication of stoma?
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
Between colostomy and posterior saggital anorectoplasty, what should you do?
Contrast on the stoma | It will show if there’s fistula
65
What are the complication of posterior saggital anorectoplasty after stoma
``` 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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