Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly

8/18/2019 Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly

1/43

Approach toHepatosplenomega

ly24/8/2015


2/43

Case 1• MA, 2 years old boy

• NKMI, NKDA

• Presented with e!er or " wee#s asso$iated with$o%&h and r%nny nose or 1 wee#

• No abdo'inal distension noted

• (as bro%&ht to )P to see# treat'ent and$o'*leted one $o%rse o a'o+i$illin, howe!er

sy'*to's was not i'*ro!ed

• -%bse.%ently $hild was bro%&ht to Pri!ate os*ital

• nre'ar#able antenatal, birth and de!elo*'entalhistory


3/43

Physi$al e+a'inationAlert, *er%sion &ood, not ta$hy*noei$, 'ild

*allor, not a%ndi$e

P%lse rate 1"0 3e'* "

P 68/50

7%n&s and C- %nre'ar#able

Abdo'en Liver 3cm and spleen 2cm *al*ablebelow $ostal 'ar&in

Shotty cervical and inguinal lymph nodepalpable


4/43

Workingdiagnosis??

Investigation??


5/43

In!esti&ation doneDate 2!"!2#$ 3!"!2#$ %!"!2#$e'o&lobin 52 5.4 5.0

(C &'2 34.35 34.63

Platelet 25 31 28

9C $o%nt showed ly'*ho$yte *redo'inant


6/43

In!esti&ation doneDate 2!"!2#$ 3!"!2#$ %!"!2#$e'o&lobin 52 5.4 5.0

(C 2 34.35 34.63

Platelet 25 31 28

9C $o%nt showed ly'*ho$yte *redo'inant


7/43

Date 2!"!2#$ 3!"!2#$ %!"!2#$

Urea 5.2 5.5 4.1

Creatinine 34 31 35

Na 139 136 421

K 4.6 4.6 4.4

Cl

S.Bilirubin 2.1

Albumin 45

Globulin 32

AL 10

AL! 88

otal !rotein ##

L$% 6#4

Uri& A&i' 335

C(! 15

Blood C&S NG NG


8/43

Any other (urtherinvestigation?


9/43

• e* , e* C, I :1"/8/15; N<

• )*+ urgent, 7e%#o$ytosis with

ly'*ho$ytosis Presen$e o 32- blasts cellswhi$h are s'all in si=e with s$anty $yto*las'and in$ons$*i$%o%s n%$leoli s%&&esti!e oA$%te 7e%#e'ia :'ost li#ely A77;

•)eripheral blood Immunophenotypingwas sent showed precursor *.ALL


10/43

Case 2ID, 2 years old 'alay boys

NKMI, NKDA

*resented with 2 'onths history o abdo'inal *ain

asso$iated with ew e*isodes o *assin& loose stoolInter'ittent *ain whi$h *artially resol!ed ater abdo'inal

'assa&e

ro%&ht to Distri$t os* twi$e, was treated as &astritis

Mother $lai'ed she elt so'e 'ass while 'assa&in& theabdo'en

-%bse.%ently $hild was bro%&ht to Pri!ate )P

Child was reerred to ) as Pri!ate )P noti$e abdominalmass while *eror'in& *hysi$al e+a'ination


11/43

Physi$al >ndin&Alert, *er%sion &ood, war' *eri*hery, 'ild

*allor

P 6?/5, P


12/43

Workingdiagnosis??

Investigation??


13/43

Date 2/!&!$

ae'o&lobin 101 3(C 1?6

Platelet ??

0rea %'#

Creatinine 4"

Na 1"4

K 42

-ilir%bin 1

Alb%'in 4"A73 1"

A7P 182

3Protein 8"


14/43

Any (urtherinvestigation??


15/43

3%'o%r 'ar#er:26//2015;

A+) B1??00 ater dil%tion:I%/'7;

0S abdomen :"0//2015; showed lar&e 'ass at theri&ht ilia$ ossa re&ion e+tendin& to the e*i&astri$re&ion, li#ely li!er in ori&in

r&ent 11 thora4!abdomen:?/8/2015; showedlar&e li!er 'ass 'ost li#ely a he*atoblasto'a with

*%l'onary nod%les

-%bse.%ently $hild was reerred to P) or %rther'ana&e'ent


16/43

Approach to Hepatomegaly5Splenomegaly andHepatosplenomegaly


17/43

7i!er -it%ated at ri&ht hy*o$hondria$ re&ion

Mo!es down with ins*iration

nable to &et abo!enlar&ed to


18/43

e*ato'e&aly• 3he *resen$e o a *al*able li!er does not

always indi$ate he*ato'e&aly

•

In &eneral, a li!er ed&e greater than 3'$ cmin ne6borns and greater than 2 cm inchildren below the ri&ht $ostal 'ar&ins%&&ests enlar&e'ent

•7ormal 8ange o( Liver span1 wee# o a&e is 45 to 5 $'

At 12 years, the nor'al !al%e or boys is to8 $' and or &irls is ? to ?5 $'


19/43

Pitalls in he*ato'e&aly• 3he liver can be displaced in(eriorly by the

dia*hra&' or thora$i$ or&ans, &i!in& thei'*ression o he*ato'e&aly

• Children who ha!e orthopedic abnormalitiess%$h as narrow $hest walls or *e$t%s e+$a!at%'

$an erroneo%sly a**ear to ha!e he*ato'e&aly

• A nor'al !ariant o the ri&ht lobe o the li!er,$alled a 8iedel lobe, 'ay e+tend ar below theri&ht $ostal 'ar&in and be $on%sed as*atholo&i$ he*ati$ enlar&e'ent


20/43

Ca%ses o he*ato'e&aly9echanism• Ina''ation

• In>ltrati!e

• Ina**ro*riate stora&e

• as$%lar $on&estion• illiary obstr%$tion

9neumonic :SHI8;

•Str%$t%ral, Stora&e/'etaboli$

• Hae'atolo&i$al, Heart

• Ine$tion, ina''atory, in>ltrati!e

• 8eti$%loendothelial, 8he%'atolo&i$al

• %'or, ra%'a


21/43

9echanism Disease

Ina''ation •In(ections


22/43

9echanism Disease

Ina**ro*riate -tora&e •LipidE &a%$herGs ds, Nie'annPi$#

•1arbohydrateE&ly$o&en stora&eds, hereditary r%$toseintoleran$e, &ala$tose'ia,M%$o*olysa$$haridoses

•)rotein!amino acidE

tyrosinae'ia, %rea $y$le dee$t

•9ineralE wilson ds,hae'o$hro'atosis

•lectrolyte transportE $ysti$

>brosis

•7utritionE total *arenteraln%trition

•)roteaseE al*haE1Eantitry*sin

de>$ien$y


23/43

9echanism Disease

In>ltrati!e •)rimary neoplastic tumorse*atoblasto'a/e*ato$ell%lar$ar$ino'a

•)rimary non.neoplastictumorse'an&io'a,he'an&ioendothelio'a,terato'a, o$al nod%lar

hy*er*lasia

•9etastatic or disseminatedtumorsE7e%#e'ia, ly'*ho'a,ne%roblasto'a, histio$ytosis

•1ystsEParasiti$ $yst, $holedo$hal$yst, *oly$ysti$ li!er disease

•Hemophagocytic syndromes

•4tramedullaryhematopoiesisEthalase'ia,


24/43

9echanism Disease

as$%lar Con&estion •SuprahepaticCon&esti!e heart ail%re


25/43


26/43


27/43


28/43

-*leen• 7o$ated at let hy*o$hondria$ re&ion

• Mo!e down with ins*iration

•

nable to &et abo!e• s%ally not *al*able

• -'ooth, re&%lar 'ar&in

• Presen$e o s*leni$ not$h

• 3ra%beGs s*a$e H 6, 10 and 11th rib s*a$es

• nlar&ed towards


29/43

-*leno'e&alyNor'al -*leen is *al*able below the let

$ostal 'ar&in in nearly oneEthird o neonates

owe!er, a splenic edge (elt more than 2cm below the ribs de>nitely is an abnor'al>ndin&


30/43

Ca%ses o s*leno'e&aly• 9oderate or massive s*leno'e&aly:8$' or

'ore below let $ostal 'ar&in;

• Mne%'oni$ 1HI9)S

1, 1ardia$ and conne$ti!e tiss%e diseases

H Hae'atolo&i$al

I, Ine$tion, In%ry

9 9ali&nan$y) )ortal y*ertension

S stora&es diseases


31/43

Ca%ses o -*leno'e&alyModerate s*leno'e&aly9echanism Diseases

Ine$tion •*acterialE-%ba$%te ba$terialendo$arditis, -e*ti$ae'ia,-*leni$ abs$ess, 3y*hoid e!er,

r%$ellosis, 7e*tos*irosis, 3%ber$%losis, Cat s$rat$hdisease

•


32/43

9echanism 1auses

Ina''ation/disordered

i''%nore&%lation

-7, brosis•SuprahepaticE %dd Chiarisyndro'e

-*leni$ !ein thro'bosis


33/43

9echanism Disease

Mali&nan$y 7e%#e'ia, odinGs ly'*ho'a,Non odinGs ly'*ho'a

-tora&e disease 7i*idosesM%$o*olysa$$haridosesA'yloidoses

-tr%$t%ral e'ato'a-*leni$ $yst/*se%do$yste'an&io'a7y'*han&io'a


34/43

Massi!e -*leno'e&aly

9echanisms DiseasesIne$tions Chroni$ 'alaria

-$histoso'iasisis$eral lesh'ananiasis:#alaEa=ar;

+tra'ed%lary hae'ato*oiesis ereditary s*hero$ytosis 3halasae'ia Maor

Con&esti!e Non $irrhoti$ *ortal >brosis

Mali&nan$y NonEhodinGs ly'*ho'aMetastati$ diseaseCM7

-tora&e disease 7i*idoses disease


35/43

e*atos*leno'e&aly9echanism Diseasee'atolo&i$al 3halasae'ia,'yelo*rolierati!e

ds and ly'*ho*rolierati!e ds

Ine$tion , 3@brosis


36/43

I'*ortant historyNeonatesImportant history Associated diseases!problems

Prolon&ed a%ndi$e +trahe*ati$ biliary atresiaAl*haE1 antitry*sin de>$ien$y)ala$tose'ia

o'ittin& and diarhhea Metaboli$/stora&e disorderse*ti$ae'ia

9a'ily history o early inantdeath or he*ati$,ne%rode&enerati!e, or *sy$hiatri$

disease

Metaboli$ diseases

Antenatal history 3@brosis


37/43

Child

Important history )ossible diseases!problems9e!er Ine$ti!e $a%ses

9orei&n tra!ellin&, in&estion oshell>sh or dr%&s

iral he*atitisParasiti$ ine$tion

history o *oor wei&ht &ain,

!o'itin&, diarrhea, distin$ti!eodors, loss o de!elo*'ental'ilestones, $o'*le+ sei=%redisorder, or hy*otonia

Metaboli$ disorder

A$holi$ stool or dar# $olor %rine e*ati$ dys%n$tion

istory o blood trans%sion iral e*atitis or C, IDr%& history & N-AID-, Isonia=id,

*ro*ylthio%ra$il, ands%lona'ides

history o ina''atorybowel disease ori''%node>$ien$y

Pri'ary s$lerosin& $holan&itis


38/43

Asso$iated *hysi$al

>ndin&sAssociated physical =ndings )ossible problem!diseasesAne'ia •e'atolo&i$al

disorder/'ali&nan$y•Ine$tion

Fa%ndi$e •e'olyti$ ane'ia•e*ati$ dys%n$tion•illiary tree dys%n$tion

ye 9indin&sCatara$ts

KayserE9leis$her rin&sChorioretinitisPosterior e'bryoto+on

• 3@


39/43


40/43

In!esti&ation


41/43


42/43

hank you


43/43

Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly

Documents