7/27/2019 36452841 Hirchsprung Disease

1/29

HIRCHSPRUNG DISEASE

A.k.a. Congenital Aganglionic

Megacolon

7/27/2019 36452841 Hirchsprung Disease

2/29

HIRCHSPRUNG DISEASE

Congenital anomaly that resultsinmechanicalobstruction frominadequatemotility of partoftheintestine.

absenceofganglioncells

-Myenteric plexus of Auerbach

-Submucosal plexus of Meissner

Theseganglioncells were formerly known asintramural ganglia ofthe parasympatheticnervous

system-classified aselementsofan independent entericnervous system (ENS)

7/27/2019 36452841 Hirchsprung Disease

3/29

HIRCHSPRUNG DISEASE

Four times morecommoninmales than in

females

-follows a familial(family unit) pattern in asmall number ofcases

80% (estimate)ofthecases are dueto

autosomal dominantgeneticmutations with

incomplete penetrance

-associated with down syndrome

7/27/2019 36452841 Hirchsprung Disease

4/29

HIRCHSPRUNG DISEASE

7/27/2019 36452841 Hirchsprung Disease

5/29

CLINICAL MANIFESTATIONS

7/27/2019 36452841 Hirchsprung Disease

6/29

CLINICAL MANIFESTATIONS

Newborn

-abdominal distention

-vomiting-constipation

-failure to pass the meconium within last

24-48 hours of life

7/27/2019 36452841 Hirchsprung Disease

7/29

CLINICAL MANIFESTATIONS

Neonates

-signsof acute abdominal obstruction

-relieved by rectal stimulationorenema

-vomiting

-delayed meconium passage

7/27/2019 36452841 Hirchsprung Disease

8/29

CLINICAL MANIFESTATIONS

Older children

-oftenhavechronicconstipation with

passage of ribbon like, foul smelling stooland abdominal distention

-haveevidenceof:

-previous GI dysfunction-Failuretothrive

-Chronicconstipation

7/27/2019 36452841 Hirchsprung Disease

9/29

DIAGNOSTIC EVALUATION

7/27/2019 36452841 Hirchsprung Disease

10/29

DIAGNOSTIC EVALUATION

Suspected Diagnosis

-(neonate)

-clinical signsofintestinal obstruction

-failure to pass meconium

-(infants and older children)

-medical history

-constipation

7/27/2019 36452841 Hirchsprung Disease

11/29

DIAGNOSTIC EVALUATION

Barium enema often demonstratesthetransition zone

-betweenthe dilated proximal (colon)megacolon andnarrow distak segmentmay not develop until the age

oftwomonthsor laterRectal biopsy

-surgically- toobtain a full-thicknessbiopsy specimen

-suctionbiopsy- for histologic evidenceofthebasic

ganglioncells

7/27/2019 36452841 Hirchsprung Disease

12/29

DIAGNOSTIC EVALUATION

Anorectal manometry

-a catheter with a balloon attached isinserted

intothe rectum-thetest recordsthe reflex pressure response

totheinternal anal sphincter to distentionof

balloon

-normal response: relaxationoftheinternal

sphincter

7/27/2019 36452841 Hirchsprung Disease

13/29

PATHOPHYSIOLOGY

7/27/2019 36452841 Hirchsprung Disease

14/29

PATHOPHYSIOLOGY

7/27/2019 36452841 Hirchsprung Disease

15/29

NURSING INTERVENTION

7/27/2019 36452841 Hirchsprung Disease

16/29

NURSING INTERVENTIONS

Help the parents adjust to the congenital

disorder

-fostering aninfant- parentbonding-preparethe parents for medical surgical

intervention

-assistingthemincaring for thecolostomyafter discharge

7/27/2019 36452841 Hirchsprung Disease

17/29

NURSING INTERVENTIONS

Post operative care

-monitor bowel sounds and passafe ofstool

-will indicate whencanoral feedingcanbeinitiated

Home care

-provideinstructions aboutcolostomy care

-skincare,emptying and changingtheostomy

surfaces, and monitoring for problems.

7/27/2019 36452841 Hirchsprung Disease

18/29

TREATMENT

7/27/2019 36452841 Hirchsprung Disease

19/29

TREATMENT

Pull-through Surgery

Hirschsprung's diseaseistreated withsurgery.Thesurgery iscalled a pull-through operation.T

here arethree common ways to do a pull-through, and they arecalled the Swenson,theSoave, and theDuhamel procedures. Eachisdone a little differently,but all involvetakingoutthe partoftheintestinethat doesn't work and

connectingthehealthy partthat's lefttotheanus. After pull-throughsurgery,thechild has aworkingintestine

7/27/2019 36452841 Hirchsprung Disease

20/29

TREATMENT

Before surgery: The diseased section is

the part of the intestine that doesn't

work.

7/27/2019 36452841 Hirchsprung Disease

21/29

TREATMENT

Step 1: The doctor removes the diseased

section.

7/27/2019 36452841 Hirchsprung Disease

22/29

TREATMENT

Step 2: The healthy section is attached to

the rectum or anus.

7/27/2019 36452841 Hirchsprung Disease

23/29

TREATMENT

Colostomy and Ileostomy

Often,the pull-throughcanbe done rightafter the diagnosis. However,children who

havebeen very sick may firstneed surgerycalled an ostomy. Thissurgery helpsthechildgethealthy beforehavingthe pull-through.Some doctors do anostomy inevery childbefore doingthe pull-through.

7/27/2019 36452841 Hirchsprung Disease

24/29

TREATMENT

Colostomy and Ileostomy

In anostomy,the doctor takes out the diseasedpart of the intestine. Thenthe doctor cuts a

small holeinthebaby's abdomen. Theholeiscalled a stoma. The doctor connectsthetop partoftheintestinetothestoma. Stool leavesthebody throughthestoma whilethebottom partof

theintestineheals. Stool goesinto a bag attachedtotheskin around thestoma. You will need toempty thisbagseveral times a day.

7/27/2019 36452841 Hirchsprung Disease

25/29

TREATMENT

Step 1: The doctor takes out most of the

diseased part of the intestine.

7/27/2019 36452841 Hirchsprung Disease

26/29

TREATMENT

Step 2: The doctor attaches the healthy

part of the intestine to the stoma (a hole

in the abdomen).

7/27/2019 36452841 Hirchsprung Disease

27/29

TREATMENT

Ifthe doctor removes the entire large

intestine and connects the small intestine to

the stoma, thesurgery iscalled anileostomy.

Ifthe doctor leaves partofthe largeintestine

and connectsthattothestoma,thesurgery is

called a colostomy.

7/27/2019 36452841 Hirchsprung Disease

28/29

TREATMENT

Later,the doctor will dothe pull-through. The

doctor disconnectstheintestine fromthe

stoma and attachesit just abovethe anus. The

stoma isn'tneeded any more,sothe doctor

either sewsitup duringsurgery or waits about

6 weekstomakesurethatthe pull-through

worked.

7/27/2019 36452841 Hirchsprung Disease

29/29

END :3