Download - 36452841 Hirchsprung Disease
-
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