i

Hirschsprung's disease

.".

, ~ 'U1DIIl'lY1!ll1l1l(ll "~,,n'llll'lllt !II. ~...., I

"UlDQU11nDtl1l11l9l1 nlfll'll11l1l1J1I11lfl1• : ••••••• 1 \

1l1lll:1Il'lYlDlIlllfl4)l"li,mnDlYIJ~ 1,,11

Definitions

l~q,h::a~riI. Unff01mmJ11tl1lflnfl1~ln~fl111J'IlM h 1l~1'ltflil~'I'lfl~Ll9ilhu~ 'l~

2. Unfln1J1ffl1J11tlflnmtlii ~'I'ltl1nfftll'l'l'llfl~hfl~l 'lnl il~'I'lfl~Ll9ifi1LU~'l~

~ ~ A". 1 .t'l"3. \lnnn1Jlffl1Jl1tlfl1i1l1t1tl~ classIfication 'Ilfl~ 1f1\l 11

4. Unffn1Jlffl1Jl1tll'l\'m11ii~litl hll~l 'tfltl~'I'lIHLl9ifi1Iii~'~

5. Unfln1J1ff11J11mi~9l11~l1Hlgn'll110111,.,111hll111 'ltfl il~'I'l()~Ll9ithlii~'~

6. UOfln1J1ff11J11mLtll'IHllm19l11~l1Hlgn'll110111"'11Jl'f1~l'tfl t1~'I'lfl~ll9ifi1IU~'~. . .7. UOflO1Jlff11J11tl1JflmL\l1Y11~m19111~fl'\lI\;1JI9i1J111'"11J~il1t1Yiff~fftll1Irl\l1'f1il Ll9iU~hi

ff11J11tl~\lU\l'~LlU\lfl\l~10m1l;' Barium enema ,~

8. UnilO1J1ff11J11tll'l\'m1~11ll1n1J1~il1t1~lrl\lhflrl',~~ ~ •• Yo 1.t,J A .1 ,.

9. \l nnn1J1ff11J11tl1Jflnmfll1::Ll'll1 n'llfl\l'llfl~ 1f1\l1lfJl~~::Ln~'IJ\l Llll::ff11J11tl ,.,m 1~Llll

1n1J1l\llij'fl~~\l 'l~

o!I ••• oJ"" •.•Hirschsprong's disease mfl aganglionic megacolon ,.,1J1t1mtll1::111Jm1'l~~\l'IJfl~•

~lMl,.,tJiLl1J1J partial ,.,;u complete ~11Jn1Jiim1'1l1~"'1t1',j'llU~ ganglion cells '\li\l• •

n~l1Jl\Jfl'llfl~~1'Itiri1\ltlll11J9im\ 1Ll,.,U~'l~9i\l9i~Ll9ifi11ii91

Pathology'I'l1J~1'tiri1\l~\ljj'IJ\l191l,.,tJi~\l HU~"'\lllrtu~\Jlnjj hypertrophy 'lJu~n~l1Jl1iu iim1

1J11J'Ilfl~lil'l n til 'ltiri1utll'llt1ii'll\l191t1n9iri1\l9ifJL~fl1J~::1i1\lLi'I\llt1m1t1 l~tlnl1 transitional

zone ~~ri1\lii'~::Yl1J ganglion cells u~l!utlol1t1n9i iitlOl1 Hypoganglionic segment

ii11t1n\l'l'l1Jfl111JFl~t1n9i~11J5-21% 'I'l1JDown's syndrome '~t1'::1J1W 5 %

2 .', ,

Classifica~on,of'Hlrschsprung's'dise'as~"; lllj~~1'I'U5 mill

I. Ultrashort segment Hirschsprung's disease

2. Short segment Hirschsprung's disease

3, Classical Hirschsprung's disease

4. Long segment Hirschsprung's disease

5. Total colonic aganglionosis

rectum u~m;ujjm1

A~:::llm~ spasm U'I1'U

i~tHnV9l1 Lrl!liimmillfl11ll~'U~'U'l'II2. internal sphincter••• .•...•.. J od ,

UU~f1f11ll10~'UI~V011 sphincter achalasia

Pathophysiology

ffim9J~'I'h'lH'liif1m~llf19l'U~ll~lhi~ii 2 ff1'Utill'

\. '11iii propulsive peristalsis ~ll~liliffff1'U~i~jj ganglion cells Lf1V'l't'lJ"hliliff

ff1'U aganglionic

on llf19l'U~ll~ih i ff!l1~11'l'IIUUU complete L';'U 'l'Umill total colonic

.• ' 'l 'aganglionosis l1~!lllUU partial L'II'U 'U0lJll ultra-short segment Hirschsprung's

disease ~'UnU'l'tV1nfftl1'1't'hliliffff1'U~i~jj ganglion cells Vl1ll10UllVLl1V~'lf1

Incidence

Clinical pictures

!l1m~Ufff1~.iN'1 ~fflllnl1'1'tui~'U Hirschsprung's disease till

1. Delayed passage of meconium

Swenson lui'] 1973 'l'tUl1 94% ~!l~~':hv Hirschsprung's disease i~rilv

meconium 'l'U 24 i1Lll~m0l1li~fll't!lf1

2. Abdominal distension

3. Constipation

Swcnson 'l'tujj!l1m~~ll~r;jo'l'UIlfill'Umo 'lh:::mw 93%

,OlJlI long segment Hirscbsprung's disease

•

4. Vomiting

l'IiJ'~Untl~iiI117Q~~Ulll1::"'tl~V~1I10 ~ 1l~1

S. Dlarrbea

l'IU'~tb::1I1111 I 'u 3 1111::iifl1111nltity1l10Il'ln::lnUtTtyty111119itlUl11n~ii.>!.1. '" .••'" .t • .t1111::11'117O'lftlU'lU fltl enterocolitts 'lJ~IUU111111~111'9l1t1'11l'1UUtltl'l1tt~'ltl~

Hirschsprung's disease

Diagnosis

1. HIstory

1.1 --~10 '::19111delayed passage of meconium•

, ~tll111'llLnUfli'~fl711

2. Pbyslcal examinations•

• ,17WiHI11t111tlOhflfl1111H~t109i'llJ~L'fl9i1llltl::'I1111l1UO~ ~ .

• 9l71~'I11~'I111711UOl'IUI10'll1ll::IU111::fltl:-. ' .ibjjtl11tl,UhU sphincter ;;19l11UUL;11~ti11~fhutlI11t1;;9iUlgO1l~1fhul;11~. . ~ .

t;,Mfhu ~u;; 1i1~l'Itl~ IJJtlioibJJtltltlO1l1l'1Ul1iiQ ~~17::1l11110iiuI11~Ulll'1::tllI*•

tltlO1l1 11119ln~'I1 H'I111111UO~::ili11UI1O'll1ll::m111::llUUU 1U~i11t1 Hirschsprung' s

disease mill Total colonic agangiionosis

1 . d -lltl:: UOlJlIL~mL,mO~

3. Roentgenograpblc diagnosis

Plain abdominal films 1~tI..t1't1~::(htl 2 11111titl supine lltl:: upright l~tl~1111

'" ..• 1 ~. '11" ""11'm::~lt1'ltl~l1011 'lJ~~::111111HllltlO1flI117Q9lPlU'ltl~tllt'Ul'10 'lJ~ i'lllO duodenal obstruction,

jejunal obstruction lll'1::i1eal obstruction tltlO't1'~

Barium enema InUO"9ln~~iitl1:: lt1'll1l1l10 1111111tlhrI117iii~li'tI~i11t1

Hirschsprung's disease Lnuthull1qj'~ 1ll'1::V~1111117tlll,j~classification ,~ L~tltl1::lt1'llll

''11111n1~llH 'II1111i'O'lll~1tI

u •.. , I' !'I , •••DO\lIll::'I1U~Ulln11'11111::lu'llHlrscbsprung's disease 'II Barium enema f16

• Disproportion of intestine

l'IU.hiifl1111llPlO~W;U'\Itl~'lUl9ltil M fhu ~U~::1i1~l'Itl~ ll~fhutlI11t1~::9iu

llflU Sigmoid colon ii'lUl9l 111qjn-h Rectum

• Transitional zone ,i'

4

. ~,

'nUUnllw ::111011Vi'lH111V~m~tlIn ::l111HY1U1il~'nll~ IIn::t11U ~u Ilt1U• d

unllw::iljjt111mhfltyLIl'n1::lhl1;u Hirschsprung's disease 'I111i'l. ~\ .

• Jejunization of colon. ,-',:.. ..,

'11lJ~li 1i ~t11U.1'u9illihlll1 ti ~'li'lPiu Ldll~~lnjjn 1111U1Pi1~U~m mucosa

• Saw tooth appearance

'nu~li1M'mlit11ulIn1v~Il9i11l11ti~'li'lPiu LOi'l~ln abnormal deep contraction

waves ~1l~li1i~

• Delayed evacuation of Barium

tilv x'rays 'lill~Y111~l1U~~lm'hBarium enema 1l~1 24 .,t111J~

Barium r\'1~1l~1uli1'~1J1n

4. Rectal manometry

, d

~::'nU111J

'liliMIOi'lnnrl1v~u,11 internal

, .d'lum1m1~ii'lmt1111JU1J~U,r~Il~t1111JriU~Il~ internal sphincter lijmYi1Jt1111Jriu

1u rectum, .: .1

'Ut1ulln91lijllt1111J~Uiu rectum 1'n1J\l~~U internal sphincter ~::t1n1vri1n~ liliM

t1111Jriuiu9i1lmti~ internal sphincter ni'ln~ Iln::'l~~1'::~::fhun~1J1

'l~~11::'~ ll9iiu~il1V Hirschsprung's disease lrllll~1Jt1111Jriuiu rectum

~ d ~ .1 d •• i"" . ~ '"sphincter nnlJUUi'l11J1n~U lfli'l spasm 111 l1 11Jl"I'l1Jl1tl~U'l~~1,::n~1J1 i'I

rectal manometry iill,:: 1V'lfU1J1n L~Il'li1v1ii~liv~il1Vmi1J ultrashort segment

Hirschsprung's disease LYn1::'lll"l'l1J11tll~U lesion iu Barium enema ,~

5. Rectal biopsy

• Suctional biopsy 1Il'n1:: mucosa ~ll~ rectum Ll~1\i11J1i'l11~mmU''llli', , ,.:.: .1 i .,

acetylcholine esterase 'nlJ11 llJU 'll1JUI'n1J~UU aganglionic colon

• Rectal muscle biopsy 11'lum'1ii~liv~l\l'n1::~11i'l ll9ifillU"'l~ invasive ii~•

lnlln i~lrlIl1!1m,gu '1 'lltl11Jntl iMm11ii~liv'~I'lilUU

Differential diagnosis ill Infancy and childhood

1. Anal stenosis

2. Anal fissures

3. Neurologic defects; meningomyelocele, poliomyelitis

4. Cretinism and myxedema

5. Acquired megacolon

Neurotoxic damage; Chaga's disease

B, Avitaminosis

Tuberculosis

Ganglion cell hypoxia

Differential diagnosis ''" newborn

I. Ileal atresia

2. Meconium plug syndrome

3. Ileus

4. Anorectal malformation

5. Malrotation with midgut volvulus

Treatment

• Non-operatlve treatment_, ,......., v .• ~_'I i" .. v.. ~

~~U1::IHflLl'lIlUnutyM1LIll'l1::11\.nflll';lu1tl 1JOltllllllll1::1JlJl0l1"1l~1l~nJ

U1::"lUlJlM11ilii~ \Jl~11tJllllliilJlfmlll1iitJu iii.,j un::9i~1411lu m::ul'fLiill~i~ ii~

h1'luliilJ~ decompression GI tract nllU 1~tlnnlr/ NG-tube, l;' rectal inig;tion un::ll1'

LV. fluid '\l1' antibiotics lU11tJ~~111'lU

• Operative-treatment

mnh colostomy

d'lu0l1i'mni"1fl1n 1~1l11~complication 11J'lh~m"~111Yh definitive surgery

indication lU0l1yh colostomy till

I. Failure to decompression GI tract

2. Failure to thrive

3. In" Enterocolitis

Definitive surgery iill~ 4 ii'\my '1 fill

a) Swenson's operation

b) Soave's operation

c) Duhamel's operation

d) Rehbein's operation

Complication

P~perative complications

I. enterocolitis

2. failure to thrive

3. peritonitis due to intestinal perforation

Early post-operative complications

1. Anastomotic insufficiency, anastomotic ulcers

2. Stenosis

3. Early post-operative ileus

4. Slipping back of the neorectum

5. Miscellaneous; wound infection. peritonitis, perianal abscess

Late complications

1. Constipation

incomplete resection

sphincter achalasia

scarred stricture

fecaloma

2. Incontinence

3. Enterocolitis I diarrhea

4. Enuresis

5. Fistula

6. Impotence

.'

6

.;.. ..

Conclusiond'lllm~tnn~~:amlJ1Jl;;V\JHnm~fh9ifl,.tJ~nmh9iflJ~ 4 U\J\J ~1UJ~

modification m~ ~~:: Lii!Jmi1Ih9ifliiitfl'! II ~llti\Jt1amLl'I'I16~~lm~ fh9ifl-hiif111Ui111 ity1ii

1~~~lIi1'd - ~d1fl Lla::"lln\J9l1':l 1V11Um1UlllU H"!J~l'Imll t'lll1l'1Ulnll!Jml'lV~ fl

il~t;'flty~ t'{fltfTl1i'\J~~ ~::l;,m1 i'n~11~f1rl'llfli>\'Ha~ ~1l~ll ~::~ !J~iimlUrm1u

111.1 ~ '" 'd" ~. L,.l ~lnV1n\J pathophysiology un:: anatomy "!J~ anorectum L1J\l!Jm~fl 1l~Il::fl1ulHnn~1

~i11v1lfl~Ha~ Ua::lnfl complication l!!JV;;t'{fl

*-------ReC.rences :

1. A. Scharli, Pathophysiology oCHirschsprung's disease in Alexander M.

Holschneider, Hirschsprung's disease, Thieme-Stratton, Inc., New York, 1982, 23-40

2. G. Kaiser and M. Bettex, Clinical generalities, in Alexander M. Holschneider, Hirschsprung's

disease, Thieme - Stratton, Inc., New York, 1982,43-53

3. BJ. Cremin, Diagnosis oC Hirschsprung's disease, in Alexander M. Holschneider,

Hirschsprung's disease, Thieme-Stratton, Inc., New York, 1982, 55-60

4. D. Helbig, Hirschsprung's disease in infancy and childhood, in Alexander M. Holschneider,

Hirschsprung's disease, Thieme-Stratton, Inc., New York, 1982, 93-101

5. H.B. Nixon, Hirschsprung's disease in the newborn, in Alexander M. Holschneider,

Hirschsprung's disease, Thieme-Stratton, Inc., New York, 1982, 103-113

6. William F. Sieber, Hirschsprung's disease, in Kenneth J. Welch, Judson Cr. Randolph, Mark

M. Ravitch, et ai, Pediatric surgery, Year book medical publishers, Inc., Chicago, 1986,

995-1019

7. Arvin I. Philippart, Hirscshprung's disease, in Keith W. Ashcraft, Thomas M. Holder,

Pediatric surgery, Harcourt Brace Jovanovich, Inc. Philadelphia, 1993,358-370

••••••••••••••

'J'