Pediatric Lower GI CIS - Thieman/Brandau Flashcards

1
Q

Case 3 day old M, no meconium pass, premature labor

  • fam hx - CF and TEF
  • lethargic, distended abdomen
  • fused 3rd 4th digits
A

imperforate anus

acute medical needs - before surgery - infection

other anomalies to look for - VACTERL association

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

genetic syndrome

A

well characterized constellation of major and minor abnormalities that occur together in predictable fashion presumably due to single underlying etiology

ex/ trisomy 21 and klinefelters

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

genetic sequence

A

group of related anomalies that generally stems from single initial major anomaly that alters development of other surrounding tissues and structures

ex/ potters sequence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

potters sequence

A

primary problem - renal agenesis

no amniotic fluid - face growth problems

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

genetic association

A

group of anomalies that occur more frequently together than would be expected by change but dont have predictable pattern of recognition or etiology

ex/ VACTERL assocation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

VACTERL association

A
vertebral - spinal US/xray
anal
cardiac - do echo
TEF - physical exam
renal - renal US
limb anomalies - physical exam/xray
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

vesicoureteral reflux

A

high percentage in kids with VACTERL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

necrotizing eneterocolitis

A

with obstruction - distends - cuts off blood supply

look for gas bubbles in bowel wall**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

wangenstein view

A

to see where gas is in pelvis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

types of imperforate anus

A

high type - GI and GU fistula

low type - approach perineal - anoplasty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

low imperforate anus

A

air down to perineum and cure with anoplasty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

embryo of imperforate anus

A

failure of coacal membrane to involute

separate GI and GU tracts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

female high type imperforate anus

A

protrudes high in vagina

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

male high type imperforate anus

A

protrudes in bladder/urethra

worry for infection**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

high vs. low fistula with imperforate anus

A

high - no external sphincter muscle development

hard to get through these muscles and nerves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

with imperforate anus

A

always a fistula

17
Q

Case 3 day old not passed meconium

  • bilious vomiting
  • fam hx - CF
  • distended abdomen, lethargic
  • palpable fullness RLQ
A

likely CF

18
Q

cystic fibrosis

A

pancreatic problems - hard time digesting things

auto recessive

most common fatal genetic disorders - even more in amish

baby taste salty

19
Q

CFTR mutations

A

over 1000 mutations have been found

delta508 is common one - 70%

20
Q

management of CF

A

multidisciplinary team approach

gastroenterologist, pulmonologist, nutritionist, social worker

21
Q

soap bubble on abdominal xray

A

meconium ileus

do enema - that can actually break up the viscous stool

-gastrografin hypertonic with muclolytic agent**

22
Q

gastrografin hypertonic enema wit mucloytic agent

A

works 60-70% with meconium ileus

so need surgeon around

23
Q

Case 3 day old M not passed meconium, bilious vomiting, distended abdomen, lethargic

  • low set ears and macroglossia
  • passage of large amounts of flatus and meconium with finger withdrawal
A

hirschsprung disease

24
Q

low set ears

A

numerous syndromes and deletions may cause this

25
Q

beckwith wiedeman syndrome

A

microcephaly
macroglossia
umbilical hernia

big infants at birth

seizure, cyanosis, hypoglycemia
feeding difficulty

malignancy risk - renal

26
Q

hirschsprung disease

A

complications - necrotizing enterocolitis**

more common in males

patho - failure of cephalocaudal migration of parasympathetic nerve ganglion

lower rectum to small bowel**

10% rectum - explosive finger**
75% sigmoid
10% entire colon
5% small bowel

27
Q

biopsy for hirschsprung

A

suction mucosa

look for the ganglion nerve cells - auerbachs plexus

28
Q

Case 8 month old male to ER

  • projectile bilious vomiting
  • okay after episodes
  • fam hx - pyloric stenosis
  • distended abdomen, lethargic
  • guarding RLQ
  • liquid dark red stool guaiac positive
A

intussusception**

currant jelly stool

29
Q

currant jelly stool

A

intussusception

30
Q

intussusception

A

lead point - could be meckel diverticulum, enlarged peyers patches

age range - 6 mo - 2 years

can be cured with barium enema - 70%

air also works - and no risk of barium with perforation

31
Q

intussusception meckels

A

often tx with surgery