3 - Paeds - Gastro - Encopresis and Hirschsprung's

Question 1

Encopresis - why are stools retained? why is this a vicious cycle? What does this cycle lead to?

Answer 1

retained to prevent pain of defecation, but lose water in colon so become more painful
cycle -> colon distends, loss of sensation to defecate, rectal sphincter distends - stools forced out if there is overloading

Question 2

Encopresis - define

Answer 2

repeated involuntary faecal soiling not caused by organic defect or illness

Question 3

Encopresis - causes?

Answer 3

constipation

psychological - never toilet trained, toilet phobia, IBS, manipulative soiling

Question 4

Encopresis - how to manage

Answer 4

GP > paeds GEnterologist

psych and parental help in training child, reward good behaviour, online info and support groups

Question 5

Hirschsprung Disease - what is it? from where to where?

Answer 5

narrow contracted large bowel section due to absence of ganglion cells from myenteric and mucosal plexus
Rectum -> variable distance proximally (75% confined to rectosigmoid, 10% whole colon)

Question 6

Hirschsprung Disease - Neonatal presentation? what develops a little later?

Answer 6

present with intestinal obstruction/failure to pass meconium in first 24h

abdo distension and later bile stained vomit develop

Question 7

Hirschsprung Disease - neonatal - what examination may you do? what will it show?

Answer 7

DRE

narrow segment, gush of liquid stool/flatus on withdrawal

Question 8

Hirschsprung Disease - Infants - present with? mortality rate? Sx?

Answer 8

severe, life threatening Hirschsprung enterocolitis in first few wks, often due to C diff - 25-30% mortality

Sx - abdo pain, fever, foul smelling/bloody diarrhoea, vomiting

Question 9

Hirschsprung Disease - what can an infant presentation become?

Answer 9

Can > sepsis, transmural intestinal necrosis and perforation

Question 10

Hirschsprung Disease - childhood presentation?

Answer 10

chronic profound constipation with abdo distension but NO soiling

growth failure may also be present

Question 11

Hirschsprung Disease - diagnosis

-demonstrate absence of? presence of? by doing what? what to show length of segment?

Answer 11

absence of ganglionic cells and presence of large ACh-ase +ve nerve trunks on SUCTION RECTAL BIOPSY

manometry/Ba studies to show length

Question 12

Hirschsprung Disease - MGMT - surgical? for enterocolitis?

Answer 12

surgical - colostomy + anastomosing of normal bowel to anus

for enterocolitis use broad spec IV ABx and fluids immediately and monitor closely