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HEPATO & SPLEENOMEGALY
Dr.Subash Arun
HEPATOMEGALY:
• Enlargement of liver is called hepatomegaly.
• Presence of a palpable liver does not always
represent hepatomegaly .
May be mistaken for
• displacement of the liver by lung pathologies.
• abdominal tumor
• spinal deformity
• The normal range for liver span at
–1 week of age - 4.5 to 5 cm.
–At 5 yrs of age- 6 to 8 cms
–12 years, boys - 7 to 9 cm
girls - 6 to 8cm
Procedure:
Surface markings :
Upper border:4 th ICS in MCL
Lower border:9 th ICS in MCL
Lateral border:6 th rib in MAL
Functions of liver:
• Synthesis-albumin
gluconeogenesis
coagulation factors
• Metabolism-sugar
proteins,fats
• Detoxification
• Storage-vitamin A(ito cells),B12 ,iron copper
• Protective-RES (kuppfer cells)
• Grades of hepatomegaly:
mild- <4cms below Rt subCostal Margin.
mod- 5-7cms “ “
mass- >7cms “ “
• Hepatomegaly- five mechanisms,
– Infections,
– Excessive storage,
– Infiltration,
– Congestion, and
– Obstruction.
Liver E/o:
1, Edge/margin:
sharp/iregular – cirrhosis
round with soft consistency- kwashiorkor.
2, Surface-
smooth-congestion
irregular- granular(portal cirrhosis)
nodular(post necrotic cirrhosis)
uneven- multiple hydatid cysts
multiple liver abscess
4, Consistency-
Soft- CCF,acute hepatitis,anaemia
Firm- Chronic active hepatitis, cirrhosis
wilsons, galactosemia
neonatal hepatitis.
Hard- CML,hepatoblastoma
Cystic- hemangioma
5, Tenderness:
is seen in acute enlargement of liver due to
stretching of Glisson’s capsule.
in c/o cirrhosis or malignancy it will be tender
when capsule is infiltrated .
localised tenderness is seen in c/o
abscess/infected cyst.
Causes of tender hepatomegaly:
Infective- viral hepatitis,
infectious mononucleosis
leptospirosis
amoebiasis
congestive- CCF
Budd-chiari synd
misc-hepatoma,hydatid cyst
6, Pulsations:
s/I A-V malformations
triscupid regurgitation(systolic)
tricuspid stenosis(diastolic)
aortic regurgitation(Rosenbach sign)
• Palpable findings of liver:
soft,smooth,tender,enlargedCCF
firm & nodular cirrhosis of liver
obst jaundice
nodular secondaries,hepatoma
Riedels lobe tongue like projection of rt lobe of
liver
• General e/o findings in c/o hepatomegaly:
Hairflag sign(kwashiorkor)
Headmicrocephaly(cong.rubella)
hydrocephalus(toxoplasmosis)
craniotabes(hypervitaminosis A)
Eyescataract (wilsons,galactosemia)
KF ring(wilsons)
Hazy cornea(hurler)
PALLOR –
• 1)Infections - Malaria, kala-azar, bacteremia
• 2)Haemolytic anaemia - Hereditary spherocytosis,
sickle cell anaemia, thalassaemia, autoimmune
haemolytic anaemia.
• 3)Nutritional - Iron deficiency anaemia.
• 4)Leukaemia and lymphomas
• Fever - Infection - Malaria, kala-azar, enteric fever,
malignancy
• Jaundice, anorexia, vomiting, haematemesis,
malena - liver disease especially cirrhosis with
portal hypertension
• Recurrent Jaundice - Liver disease, Hemolytic
anemia
• Dyspnoea / difficulty in feeding - cardiac causes
e.g. CCF
• Petechiae, purpura, ecchymosis, lymphadenopathy
etc. – Leukaemia
• Koilonychia, platynychia - Iron deficiency
• Mental retardation - Mucopolysaccharoidoses
Neckengorged veins,raised jvp(constrictive
pericarditis)
Chestspider naevi,gynecomastia(liver failure)
Skinscratch marks(cholestasis)
CNStremors & dystonia(wilsons ds)
Mental retardation(glycogen storage ds)
Skeletalrickets,cystinosis,tyrosinemia.
Delayed development - Carbohydrate / Lipid
storage disorders
Abdomen E/o:
– Firm consistency liver with sharp edge - Cirrhosis,
constrictive pericarditis
– Just palpable soft spleen - Enteric fever, infective
endocarditis, etc.
– Ascites - Suggests cirrhosis with portal hypertension,
malignancy, TB
HISTORY:
• Age at onset
• Sex
• Fever, jaundice
• Acute illness, dyspnea, fatigue, diarrhea, vomiting
• Signs of malignancy- proptosis, subcutaneous nodules
• Travel history – endemic diseases
• Developmental milestones
• Nutrition history (neonatal formula)
• Medical history: umbilical catheter, weight loss, failure to
thrive, bleeding, bruising, Pruritis, pallor, heart disease ,
rashes, joint pain.
• Family history: Early cholecystectomy, gallstones, anemias,
ethnic heritage, liver disease, maternal HBV, HCV
Age
• Neonates and first few months of life - e.g.
Haemolytic anaemias (Thalassaemia major),
storage disorders
• Any age - Malaria, kala azar, sepsis, enteric
fever, etc.
INVESTIGATIONS
• Complete haemogram - Infections, anaemia
• Peripheral smear -
– Leukaemia (Blast cells)
– Thalassaemia (hypochromia, nucleated RBC's, target cells)
– Sickle cell anaemia (sickling on treatment with 2% sodium
metabisulphite)
– Parasitic diseases (Eosinophilia)
• ESR - Elevated in inflammatory diseases
• Reticulocyte count- High in haemolytic anaemia
Liver Function Test
• Serum proteins - Low in kwashiorkor
• SGOT/SGPT - Raised in hepatitis & hepatic necrosis
• Alkaline phosphatase - Elevated in hepatobiliary
obstruction & liver abscess
• Bilirubin (total, direct) - Haemolytic anaemias
MISCELLANEOUS TESTS
• Raised alpha foeto protein- Hepatoblastoma
• Hbs Ag - Hepatitis B
• High prothrombin time - Liver parenchymal dysfunction
• High sweat chlorides - Cystic fibrosis
• Wilson's disease - Low ceruloplasmin
• Liver scan - To differentiate biliary atresia from neonatal
hepatitis
• Urine and stool examination - In case of jaundice
• USG abdomen - Cirrhosis with portal hypertension,
Ascites, Tumors & cysts
• Liver biopsy- Pathological diagnosis
• Chest X-ray - ECG, echocardiography if cardiac
cause suspected
• Haemolytic profile in suspected haemolytic
anaemia
• Blood culture, Widal, Mantoux test - as required
Approach to child with hepatomegaly
Approach to neonate with hepatomegaly
TREATMENT STRATEGIES
• Therapy is directed at treatment of underlying disease
• Infections
–Consider interferon for hepatitis B
–Consider interferon and ribavarin for hepatitis C
• Metabolic disease
–Metabolism consultation
–Often requires specific restricted formulas
• Cholestasis
–Ursodeoxycholic acid
–Supplemental fat soluble vitamins A, D, E, K
•Immune suppression for autoimmune hepatitis
•Chemotherapy – Histiocytosis, leukemia, lymphoma
•Surgical treatment
•Kasai portoenterostomy for biliary atresia has better
outcome if done before 60 days of age
CASE HISTORY
• A three years old first order female
child
• Born out of 2nd degree consanguineous
marriage
presented with chief complaint of
• Distension of abdomen since 4months of age.
• No h/o:
– Jaundice, edema
– Change in bowel pattern , weight loss
• Past h/o:at 4 months of age child developed
convulsions-
fever
vomiting,
–Altered sensorium
–Breathlessness
– Subsequently she had 8-10 admissions for
severe metabolic acidosis, with hypoglycaemia
• Birth history:
–Full term, normal delivered
• Development history :
–Sat without support at the age of 1 year
–Walked unassisted at the age of 2 years
• On examination:
–Weight : 14 kg; Height: 84 cm (< 3rd
percentile)
–Doll like face, protuberant abdomen
–No pallor, cyanosis, clubbing,
lymphadenopathy, icterus
–P/A: huge hepatomegaly almost reaching
right lower quadrant; no splenomegaly
–CNS: Normal muscle tone and power,
normal deep tendon reflexes
–Other systems: NAD
–Fundus : NAD
• Attending paediatrician may have following
questions:
• Differential diagnosis?
• Is this is a routine chronic liver disease?
• Am I dealing with GSD or fatty oxidation
disorder where we get hypoglycaemia,
Hepatomegaly, and metabolic acidosis
• How will I explain acidosis?
• What is my diagnosis here?
• How should I investigate this case further?
APPROACH TO A CHILD WITH
HEPATOMEGALY
Let me examine him fully before I can say
that this person is dead !!
First be sure it is
hepatomegaly and
not a pushed down
liver !!!!
Always assess
Liver span
Consistency
Surface
APPROACH TO A CHILD WITH
HEPATOMEGALY
In this particular case one may just
consider SIZE of the liver which was
huge.
• Very limited causes of huge
hepatomegaly at this age.
• Most likely is some kind of storage
disorder; GSD, LSD or stretching a little
bit FAOD.
APPROACH TO A CHILD WITH
HEPATOMEGALY
• Presence of hypoglycemia and severe
metabolic acidosis will further reduce
the differential diagnosis to GSD and
FAOD
APPROACH TO A CHILD WITH
HEPATOMEGALY
• On the other hand, if size of the liver is
moderate or mild, differential diagnoses
could be altogether different.
• Since there could be many causes to
consider; good history and physical
examinaton are very essential
–Keep in mind that Wilson’s disease
could have an acute presentation.
–Chronic liver disease may have acute
decompensation
RULE OF THUMB ???
• Huge hepatomegaly with preserved liver
functions suggests
–storage disorder; at any age; or
–Reticuloendothelial hyperplasia
INVESTIGATIONS
• Remember!!
• Good history, aided by meticulous
examination will give clue to the
underlying cause, more than any single
investigation
Let me see if I can
find out what is
wrong with you!!
Liver biopsy showing mosaic
pattern, prominent cell
membranes and nuclear
hyperglycogenation (HE stain);
Distended hepatocytes without
fibrosis
FINAL DIAGNOSIS
GLYCOGEN STORAGE DISORDER
TYPE 1
A diagnostic approach to
splenomegaly
SPLEENOMEGALY
• It refers to enlargement of spleen beyond its normal
size.
• A spleen is said to be significantly enlarged if it is
palpable atleast 1cm below costal margin in a child
more than 6months of age.
• In 30% of newborns & 15% of infants <6months
palpable spleen is a normal variant.
Anatomy
• It lies within the left upper quadrant of the
peritoneal cavity.
• Abuts ribs 9-11, the stomach, the left kidney, the
splenic flexure of the colon, and the tail of the
pancreas.
Anatomy
• Normal Spleen
• Autopsy: <250g.
• Radioisotope Scintiscan: 12cm long x 7cm wide.
• Ultrasound: 11cm cephalocaudad diameter.
• ~3% of healthy people have splenomegaly.
Splenomegaly
Poulin et al defined splenomegaly on the
basis of size of spleen
• Moderate; if the largest dimension is 11-20 cm.
• severe; if the largest dimension is greater than
20 cm.
Splenomegaly
Splenomegaly definition by weight
• MILD; Spleens weighing 400-500 g.
Moderate; Spleen weighing 750-1000g.
Massive; More than 1000 g to indicate massive
splenomegaly.
Functions of spleen:
• Reservior for platelets,monocytes,FVIII etc.
• Haematopoiesis in fetus.
• Repairs and destruction of RBC’s by culling &
pitting.
PITTINGremoval of inclusion bodies (heinz
bodies,howell jolly bodies) without destroying
RBC’s.
CULLINGremoval of damaged/old RBC’s from
circulation.
• Immune function: IgM ,properidin,tuftsin are
produced by spleen.
prevention of inf. By capsulated org.(H.influ etc)
role in phagocytosis.
Grading of spleenomegaly:
Grade 1-normal,not palpable even on deep inspiration.
Grade 2-palpable just below costal margin usually on
deep inspiration.
Grade 3-palpable below costal margin but not projected
beyond a horizontal line half way b/w costal margin and
umblicus.the projection need to be ascertained along a
line dropped vertically from the left nipple.
Grade 4- lowest palpable point approaching the
umblical level but not below a line drawn
horizontally through umblicus.
Grade 5-lowest palpable point below umblical level
but not projected beyond a horizontal line situated
halfway b/w umblicus and symphysis pubis.
Grade 6-lowest palpable point beyond lower limit of
grade 5.
Grading according to size of spleen below LCM:
MILD palpable <3cms below LCM
MODERATE 4-7 below LCM
SEVERE >7cms below LCM.
Clinical E/o :
Size & Degree:
• it usually enlarges towards RIF.
• it is measured as child takes a deep breath from a
point on LCM in MCL to the tip of the enlarged
spleen.
Margin:
• Splenic notch is felt on the Ant. border & has a
sharp margin.
• Diff from kidney where there is absence of notch &
margin is round
Spleen vs. Kidney
Spleen
• Splenic notch.
• Can cross midline.
• Can’t get above.
• Moves down on
inspiration.
• Not ballotable.
• Splenic rub.
Kidney
• No notch.
• Never cross the
midline.
• May get above.
• Doesn’t move with
respiration.
• Ballotable.
• No rub.
• Inspection
• Look in left
Hypochondrium.
Examination of the Spleen
Percussion of spleen
Normal
• Left midaxillary line 9th –11th intercostal space
width 4-7cm.
• Enlargement of splenic dullness: splenomegaly.
Percussion (3 methods):
• Percussion of Traube's Space boundaries –
Left anterior axillary line
6th rib
costal margin .
• This area should be resonant on percussion.
• Dullness indicates possible splenic enlargement
Percussion by Castell’s method :
• percuss in the lowest Left intercostal space in the
anterior axillary line (usually the 8th or 9th IC
space)
• this space should remain resonant during full
inspiration .
• dullness on full inspiration indicates possible
splenic enlargement (a positive Castell’s sign)
Percussion by Nixon’s method:
• place the patient in Right lateral decubitus
• begin percussion midway along the Left costal
margin proceed in a line perpendicular to the Left
costal margin
• if the upper limit of dullness extends >8 cm above
the Left costal margin, this indicates possible
splenomegaly
Palpation of spleen
• To palpate the spleen, the patient is in the supine
position with the knees flexed to decrease
abdominal muscle tone.
• Begin the examination by palpating the right lower
quadrant and move upward across the abdomen as
the patient.
Palpation (3 methods)
Method #1:
• begin palpation in the RLQ.
• direct the patient's breathing by telling them when
to take a deep breath and when to exhale while
proceeding diagonally towards the Left Upper
Quadrant (LUQ), try to palpate the spleen edge
during each inspiratory phase
Method #2:
• place the patient’s Left fist under their Left
posterior chest.
• With your Right hand, begin palpation in the RLQ.
• Direct the patient's breathing by telling them when
to take a deep breath and when to exhale while
proceeding diagonally towards the LUQ, try to
palpate the spleen edge during each inspiratory
phase
Method #3 –The Hooking maneuver of Middleton
(optional):
• Place the patient’s Left fist under their Left
posterior chest position yourself on the patient’s
Left side, facing the patient’s feet.
• Using both hands, curl your fingers under the
patient’s Left costal margin ask the patient to take a
long, deep breath à attempt to palpate the spleen
with your fingertips
Percussion of spleen
• Percussion is also used to delineate the size of
the spleen.
• Percussion is only approximately 60%
accurate in most studies, with palpation about
50% accurate.
Consistency:
soft 1, normal
2,septicemia
3,enteric fever
4,infectious mononucleosis
firm 1,cirrhosis
2,lymphoma
3,leukemia
4,chr.malaria
hard secondaries
Tenderness:
s/I infective endocarditis
splenic abscess
splenic infarction
Surface:
smooth congestive causes like portal HTN
irregularhydatid cyst
• Spleen moves downwards and medially during
inspiration.
• Fingers cannot be insinuated btw enlarged spleen
and LCM
• Spleenic rub is palpable in spleenitis
Mechanism of splenomegaly:
• Reactive Reticulo-endothelial hyperplasia
• Lymphoid hyperplasia
• Proliferation of lymphoma cells
• Infiltration by abnormal cells
• Extramedullary hemopoeisis
• Proliferation of macrophages d/t RBC
destruction
• Vascular congestion
Symptoms and signs
• Abdominal pain/tiredness.
• Early satiety due to splenic encroachment.
• Symptoms of anemia due to accompanying cytopenia.
• Febrile illness (infectious).
• Pallor, dyspnea, bruising, and/or petechiae (hemolytic
process).
Symptoms and signs
• History of liver disease (congestive).
• Weight loss, constitutional symptoms (neoplastic).
• Pancreatitis (splenic vein thrombosis).
• Alcoholism, hepatitis (cirrhosis).
Signs
• Palpable left upper quadrant abdominal mass.
• Splenic rub.
• Lymphadenopathy.
Symptoms and signs
• Signs of cirrhosis (eg, asterixis, jaundice, telangiectasias,
gynecomastia, caput medusa, ascites).
• Heart murmur (endocarditis, congestive failure).
• Jaundice (spherocytosis, cirrhosis).
• Petechiae (any cause of thrombocytopenia).
Causes of splenomegaly
• Infective
• Hyperplastic
• Congestive
• Infiltration
infective
• Acute & subacute- IMN, infective endocarditis,
severe pyogenic inf.
Viral hepatitis,CMV,AIDS
• Chronic - TB,syphilis,brucellosis
• Tropical splenomegaly
• Malaria,kala azar, trypanosomiasis
congestive
• Intra hepatic obst.portal hypertension
- cirrhosis,biliary cirrhosis,hemochromatosis
- primary sclerosing cholangitis
• Extra-hepatic portal hypertension
- venous malf,thrombosis,stenosis
- ext.occlusion of portal,splenic vein
• Chronic passive congestion of cardiac origin
hyperplastic
• Extramedullary hemopoeisis- myeloprolif.d/s
- marrow damage
- marrow infiltration
• Reticulo endothelial hyperplasia –(abn.RBC)
- sickle cell d/s,spherocytosis,Hbnopathies,
thalassemia major,PNH
infiltrative
• Malignant infiltration- CML,lymphoblastic
- lymhomas, MPD,
- angiosarcoma,tumors
- metastasis (melanoma)
• benign -
- storage d/s –Gaucher’s,Neiman-pick
- amyloidosis
- hurler’s syndrome,MPS
-
cysts,fibromas,hemangiomas,hamartomas
-Eosnophilic granulomas
Disordered immunoregulation
• Felty’s syndrome- RA+ splenomegaly+leucopenia
• Systemic lupus erythromatosis
• Collagen vascular diseases
• Sarcoidosis
• Immune thrombocytopenia
Splenomegaly in children
• Metastatic neuroblastoma.
• Infection.
• Autoimmune: juvenile rheumatoid arthritis.
• Haemolysis: hereditary spherocytosis, sickle cell anaemia,
Thalassaemia
• Neoplasia: ALL, Hodgkin disease and NHL, acute or chronic
myeloblastic leukemia, neuroblastoma.
• Inherited diseases: Gaucher's disease and other storage
disorders.
Massive splenomegaly (>8cm
>1000gm)
• Myeloproliferative disorder
• Chronic malaria,kala-azar (trop. Splenomegaly)
• Storage disorders
• Thalassemia major
• Sarcoidosis
• Hairy cell leukemia
• Gaucher disease
• Diffuse splenic hemangiomatosis
Moderate splenomegaly(4-8cm)
• Cirrhosis
• Lymphomas‘
• Amyloid
• Splenic abscess,infarct
• Hemolytic anemias
• IMN
Mild splenomegaly (1-3cm)
• Acute infective conditons
• Acute malaria,tyhoid,kala-azar,septicemias
Special situations associated with
splenomegaly
• Fever- typhoid,malaria,kalaazar, infect.endocarditis,
leukemia,lymphoma
• Tender spleen- rupture,abscess,infarct
• a/c illness+ anemia- AIHA,leukemia
• Fever + LN- IMN,leukemia,lymhomas,SLE,sarcoid
• Anemia- hemolytic anemia,hemoglobinopathies
• Jaundice – cirrhosis,hemolytic anemia
• Pulsatile spleen- aneurysm
• High ESR- connective tissue disorder
• Leukopenia- felty’s syndrome,septicemia
Step-wise approach to splenomegaly
• History
• Physical examination
• Laboratory testings
• Imaging
• Specialised testing
history
• Age ,gender
• Race
• h/o recent infections like malaria
• Fever,weight loss,sweating (lymphomas,infections)
• Pruritis
• Abnormal bleeding/bruising
• Joint pain
• h/o alcholism
• h/o trauma
• h/o neonatal umblical sepsis
• Residence & travel abroad
History …..cont
• Jaundice
• High risk sexual behavior (AIDS)
• Past medical history
• Drugs
Physical examination
• Size of the spleen
• Hepatomegaly
• Lymphadenopathy
• Fever
• Icterus
• Bruising,petechiae
• Oral & supf.sepsis
• Stigmata of liver disease
• Stigmata of RA/SLE
• Splinter hemorrhage,retinal hemorrhage
• Cardiac murmurs
Lab investigations
• CBC
• Blood smear
• Retic count
• Blood C/S
• Serology (fungal,viral,parasitic)
• LFT
• Hb electropheresis/ coombs test
• Coag.profile
• Amylase/lipase
• AMA, Anti CCP,RA factor
• Bone marrow analysis
Hypersplenism
Criteria for a diagnosis of hypersplenism:
• anemia.
• Leukopenia.
• Thrombocytopenia.
• combinations thereof, plus cellular bone marrow,
splenomegaly, and improvement after splenectomy.
Approach to Splenomegaly
Depends on Pretest Probability
• Clinical Suspicion of Splenomegaly (>10%).
• Percuss first and if positive palpate.
• If percussion is negative and suspicious,
order an ultrasound.
• If percussion positive but palpation is
negative, order an ultrasound.
• Both percussion and palpation
positive = SPLENOMEGALY.
Diagnostic Approach
• CBC provides information about hematological,
infectious, and inflammatory processes.
• Finding of pancytopenia, Anemia, Leukopenia,
Thrombocytopenia may indicate bone marrow
dysfunction or portal hypertension with
hypersplenism.
Laboratory tests
Routine tests :
• CBC, platelet count, sedimentation rate.
• chemistry panel, febrile agglutinins, serum
haptoglobins, ANA test, Monospot test, serum protein
electrophoresis, tuberculin test.
• chest x-ray, EKG, and flat plate of the abdomen.
Diagnostic Approach
• Increased sedimentation rate suggests infectious,
inflammatory, or neoplastic process.
• Bacterial, fungal, and other cultures may be
performed with suspected infection.
Diagnostic Approach
• Bone marrow exam is useful in diagnosis of
histiocytoses, lysosomal storage disorders,
and some infections(e.g., disseminated
histoplasmosis).
Diagnostic Approach
• Liver function tests and abdominalU/S with Doppler
methods should be performed with suspected portal
hypertension.
• Abdominal U/S and CT locate and define extent of
splenic masses
If there is jaundice
• A hepatitis profile, red cell fragility test, and blood
smear for parasites should be done.
If there is fever.
• Serial blood cultures, leptospirosis antibody titer,
and smear for malarial parasites should be done.
Laboratory tests
Laboratory tests
If there is a petechial rash
• A coagulation profile should be done.
To rule out malignancies
• Lymph node biopsies and bone marrow
examinations may be necessary.
Imaging
• USG- sensitive & specific non-invasive
• CT scan – etiology of splenomegaly
- liver size,heterogenecity
- splenic mets, abscess,calcf.,cysts
- retro peritoneal LN
- craniocaudal ln > 10 cm
• Liver- spleen colloid scan- (RBC –Cr51,Tc99)
- hepatic steatosis,SOL,splenic functions
- PHT,colloid shift +
• MRI/ Doppler usg- portal/splenic vein thrombosis
- cavernomas
imaging
• MRI scan- liver hemangiomas
hemochromatosis
erlenmeyer flask sign(Gaucher)
• PET scan - Dx & staging of lymphomas
- determine metabolic cells in spleen
Imaging Studies
Splenoportography
o This modality is used to evaluate portal vein patency
and the distribution of collateral vessels before shunt
operations for cirrhosis.
o Findings can help identify the cause of idiopathic
splenomegaly, especially in children.
• Angiography: Angiographic findings are used to
differentiate splenic cysts from other splenic tumors.
Imaging Studies
• Liver-spleen colloid scanning
o Erythrocytes are labeled with chromium-51 (51 Cr) ,
mercury-197 (197 Hg), rubidium-81 (81 Rb), or
technetium-99m (99m Tc), and the cells are altered by
treatment with heat, antibody, chemicals, or metal
ions so that the spleen sequesters them after
infusion.
o A spleen length >14 cm is consider enlarged on liver-
spleen scan
Specialised testing
• Abd.fat pad aspiration-amyloidosis
• JAK-2 mutation
• Gene testing(bcr-abl ,C282Y)-leukemia
• Enzyme testing-storage
• Lymph node biopsy-infection,malignancy
• FNAB spleen
• Splenectomy-hyperspleenism
• Lung or skin biopsy
• Liver biopsy
summary
• Splenomegaly – major physical finding
• Step wise approach- history,physical exam
• Look for associated features
• Lab investigation & Imaging
• Search for etiology & treat
• References:
1,Gupte S. Differential Diagnosis in Pediatrics,5th edn
Nw Delhi: Jaypee 2008.
2,Nelson text book of pediatrics - 20th edn .

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Hepato&spleenomegaly

  • 2. HEPATOMEGALY: • Enlargement of liver is called hepatomegaly. • Presence of a palpable liver does not always represent hepatomegaly . May be mistaken for • displacement of the liver by lung pathologies. • abdominal tumor • spinal deformity
  • 3. • The normal range for liver span at –1 week of age - 4.5 to 5 cm. –At 5 yrs of age- 6 to 8 cms –12 years, boys - 7 to 9 cm girls - 6 to 8cm Procedure:
  • 4. Surface markings : Upper border:4 th ICS in MCL Lower border:9 th ICS in MCL Lateral border:6 th rib in MAL
  • 5. Functions of liver: • Synthesis-albumin gluconeogenesis coagulation factors • Metabolism-sugar proteins,fats • Detoxification • Storage-vitamin A(ito cells),B12 ,iron copper • Protective-RES (kuppfer cells)
  • 6. • Grades of hepatomegaly: mild- <4cms below Rt subCostal Margin. mod- 5-7cms “ “ mass- >7cms “ “
  • 7. • Hepatomegaly- five mechanisms, – Infections, – Excessive storage, – Infiltration, – Congestion, and – Obstruction.
  • 8. Liver E/o: 1, Edge/margin: sharp/iregular – cirrhosis round with soft consistency- kwashiorkor. 2, Surface- smooth-congestion irregular- granular(portal cirrhosis) nodular(post necrotic cirrhosis) uneven- multiple hydatid cysts multiple liver abscess
  • 9. 4, Consistency- Soft- CCF,acute hepatitis,anaemia Firm- Chronic active hepatitis, cirrhosis wilsons, galactosemia neonatal hepatitis. Hard- CML,hepatoblastoma Cystic- hemangioma
  • 10. 5, Tenderness: is seen in acute enlargement of liver due to stretching of Glisson’s capsule. in c/o cirrhosis or malignancy it will be tender when capsule is infiltrated . localised tenderness is seen in c/o abscess/infected cyst.
  • 11. Causes of tender hepatomegaly: Infective- viral hepatitis, infectious mononucleosis leptospirosis amoebiasis congestive- CCF Budd-chiari synd misc-hepatoma,hydatid cyst
  • 12. 6, Pulsations: s/I A-V malformations triscupid regurgitation(systolic) tricuspid stenosis(diastolic) aortic regurgitation(Rosenbach sign)
  • 13. • Palpable findings of liver: soft,smooth,tender,enlargedCCF firm & nodular cirrhosis of liver obst jaundice nodular secondaries,hepatoma Riedels lobe tongue like projection of rt lobe of liver
  • 14. • General e/o findings in c/o hepatomegaly: Hairflag sign(kwashiorkor) Headmicrocephaly(cong.rubella) hydrocephalus(toxoplasmosis) craniotabes(hypervitaminosis A) Eyescataract (wilsons,galactosemia) KF ring(wilsons) Hazy cornea(hurler)
  • 15. PALLOR – • 1)Infections - Malaria, kala-azar, bacteremia • 2)Haemolytic anaemia - Hereditary spherocytosis, sickle cell anaemia, thalassaemia, autoimmune haemolytic anaemia. • 3)Nutritional - Iron deficiency anaemia. • 4)Leukaemia and lymphomas
  • 16. • Fever - Infection - Malaria, kala-azar, enteric fever, malignancy • Jaundice, anorexia, vomiting, haematemesis, malena - liver disease especially cirrhosis with portal hypertension • Recurrent Jaundice - Liver disease, Hemolytic anemia • Dyspnoea / difficulty in feeding - cardiac causes e.g. CCF
  • 17. • Petechiae, purpura, ecchymosis, lymphadenopathy etc. – Leukaemia • Koilonychia, platynychia - Iron deficiency • Mental retardation - Mucopolysaccharoidoses
  • 18. Neckengorged veins,raised jvp(constrictive pericarditis) Chestspider naevi,gynecomastia(liver failure) Skinscratch marks(cholestasis)
  • 19. CNStremors & dystonia(wilsons ds) Mental retardation(glycogen storage ds) Skeletalrickets,cystinosis,tyrosinemia. Delayed development - Carbohydrate / Lipid storage disorders
  • 20. Abdomen E/o: – Firm consistency liver with sharp edge - Cirrhosis, constrictive pericarditis – Just palpable soft spleen - Enteric fever, infective endocarditis, etc. – Ascites - Suggests cirrhosis with portal hypertension, malignancy, TB
  • 21. HISTORY: • Age at onset • Sex • Fever, jaundice • Acute illness, dyspnea, fatigue, diarrhea, vomiting • Signs of malignancy- proptosis, subcutaneous nodules • Travel history – endemic diseases • Developmental milestones • Nutrition history (neonatal formula) • Medical history: umbilical catheter, weight loss, failure to thrive, bleeding, bruising, Pruritis, pallor, heart disease , rashes, joint pain. • Family history: Early cholecystectomy, gallstones, anemias, ethnic heritage, liver disease, maternal HBV, HCV
  • 22. Age • Neonates and first few months of life - e.g. Haemolytic anaemias (Thalassaemia major), storage disorders • Any age - Malaria, kala azar, sepsis, enteric fever, etc.
  • 23. INVESTIGATIONS • Complete haemogram - Infections, anaemia • Peripheral smear - – Leukaemia (Blast cells) – Thalassaemia (hypochromia, nucleated RBC's, target cells) – Sickle cell anaemia (sickling on treatment with 2% sodium metabisulphite) – Parasitic diseases (Eosinophilia) • ESR - Elevated in inflammatory diseases • Reticulocyte count- High in haemolytic anaemia
  • 24. Liver Function Test • Serum proteins - Low in kwashiorkor • SGOT/SGPT - Raised in hepatitis & hepatic necrosis • Alkaline phosphatase - Elevated in hepatobiliary obstruction & liver abscess • Bilirubin (total, direct) - Haemolytic anaemias
  • 25. MISCELLANEOUS TESTS • Raised alpha foeto protein- Hepatoblastoma • Hbs Ag - Hepatitis B • High prothrombin time - Liver parenchymal dysfunction • High sweat chlorides - Cystic fibrosis • Wilson's disease - Low ceruloplasmin • Liver scan - To differentiate biliary atresia from neonatal hepatitis • Urine and stool examination - In case of jaundice
  • 26. • USG abdomen - Cirrhosis with portal hypertension, Ascites, Tumors & cysts • Liver biopsy- Pathological diagnosis • Chest X-ray - ECG, echocardiography if cardiac cause suspected • Haemolytic profile in suspected haemolytic anaemia • Blood culture, Widal, Mantoux test - as required
  • 27. Approach to child with hepatomegaly
  • 28. Approach to neonate with hepatomegaly
  • 29. TREATMENT STRATEGIES • Therapy is directed at treatment of underlying disease • Infections –Consider interferon for hepatitis B –Consider interferon and ribavarin for hepatitis C • Metabolic disease –Metabolism consultation –Often requires specific restricted formulas • Cholestasis –Ursodeoxycholic acid –Supplemental fat soluble vitamins A, D, E, K
  • 30. •Immune suppression for autoimmune hepatitis •Chemotherapy – Histiocytosis, leukemia, lymphoma •Surgical treatment •Kasai portoenterostomy for biliary atresia has better outcome if done before 60 days of age
  • 31. CASE HISTORY • A three years old first order female child • Born out of 2nd degree consanguineous marriage
  • 32. presented with chief complaint of • Distension of abdomen since 4months of age. • No h/o: – Jaundice, edema – Change in bowel pattern , weight loss • Past h/o:at 4 months of age child developed convulsions- fever
  • 33. vomiting, –Altered sensorium –Breathlessness – Subsequently she had 8-10 admissions for severe metabolic acidosis, with hypoglycaemia • Birth history: –Full term, normal delivered
  • 34. • Development history : –Sat without support at the age of 1 year –Walked unassisted at the age of 2 years • On examination: –Weight : 14 kg; Height: 84 cm (< 3rd percentile) –Doll like face, protuberant abdomen –No pallor, cyanosis, clubbing, lymphadenopathy, icterus
  • 35. –P/A: huge hepatomegaly almost reaching right lower quadrant; no splenomegaly –CNS: Normal muscle tone and power, normal deep tendon reflexes –Other systems: NAD –Fundus : NAD
  • 36. • Attending paediatrician may have following questions: • Differential diagnosis? • Is this is a routine chronic liver disease? • Am I dealing with GSD or fatty oxidation disorder where we get hypoglycaemia, Hepatomegaly, and metabolic acidosis
  • 37. • How will I explain acidosis? • What is my diagnosis here? • How should I investigate this case further?
  • 38. APPROACH TO A CHILD WITH HEPATOMEGALY Let me examine him fully before I can say that this person is dead !!
  • 39. First be sure it is hepatomegaly and not a pushed down liver !!!! Always assess Liver span Consistency Surface
  • 40. APPROACH TO A CHILD WITH HEPATOMEGALY In this particular case one may just consider SIZE of the liver which was huge. • Very limited causes of huge hepatomegaly at this age. • Most likely is some kind of storage disorder; GSD, LSD or stretching a little bit FAOD.
  • 41. APPROACH TO A CHILD WITH HEPATOMEGALY • Presence of hypoglycemia and severe metabolic acidosis will further reduce the differential diagnosis to GSD and FAOD
  • 42. APPROACH TO A CHILD WITH HEPATOMEGALY • On the other hand, if size of the liver is moderate or mild, differential diagnoses could be altogether different. • Since there could be many causes to consider; good history and physical examinaton are very essential
  • 43. –Keep in mind that Wilson’s disease could have an acute presentation. –Chronic liver disease may have acute decompensation
  • 44. RULE OF THUMB ??? • Huge hepatomegaly with preserved liver functions suggests –storage disorder; at any age; or –Reticuloendothelial hyperplasia
  • 45. INVESTIGATIONS • Remember!! • Good history, aided by meticulous examination will give clue to the underlying cause, more than any single investigation Let me see if I can find out what is wrong with you!!
  • 46. Liver biopsy showing mosaic pattern, prominent cell membranes and nuclear hyperglycogenation (HE stain); Distended hepatocytes without fibrosis
  • 48. A diagnostic approach to splenomegaly
  • 49. SPLEENOMEGALY • It refers to enlargement of spleen beyond its normal size. • A spleen is said to be significantly enlarged if it is palpable atleast 1cm below costal margin in a child more than 6months of age. • In 30% of newborns & 15% of infants <6months palpable spleen is a normal variant.
  • 50. Anatomy • It lies within the left upper quadrant of the peritoneal cavity. • Abuts ribs 9-11, the stomach, the left kidney, the splenic flexure of the colon, and the tail of the pancreas.
  • 51. Anatomy • Normal Spleen • Autopsy: <250g. • Radioisotope Scintiscan: 12cm long x 7cm wide. • Ultrasound: 11cm cephalocaudad diameter. • ~3% of healthy people have splenomegaly.
  • 52. Splenomegaly Poulin et al defined splenomegaly on the basis of size of spleen • Moderate; if the largest dimension is 11-20 cm. • severe; if the largest dimension is greater than 20 cm.
  • 53. Splenomegaly Splenomegaly definition by weight • MILD; Spleens weighing 400-500 g. Moderate; Spleen weighing 750-1000g. Massive; More than 1000 g to indicate massive splenomegaly.
  • 54. Functions of spleen: • Reservior for platelets,monocytes,FVIII etc. • Haematopoiesis in fetus. • Repairs and destruction of RBC’s by culling & pitting. PITTINGremoval of inclusion bodies (heinz bodies,howell jolly bodies) without destroying RBC’s. CULLINGremoval of damaged/old RBC’s from circulation.
  • 55. • Immune function: IgM ,properidin,tuftsin are produced by spleen. prevention of inf. By capsulated org.(H.influ etc) role in phagocytosis.
  • 56. Grading of spleenomegaly: Grade 1-normal,not palpable even on deep inspiration. Grade 2-palpable just below costal margin usually on deep inspiration. Grade 3-palpable below costal margin but not projected beyond a horizontal line half way b/w costal margin and umblicus.the projection need to be ascertained along a line dropped vertically from the left nipple.
  • 57. Grade 4- lowest palpable point approaching the umblical level but not below a line drawn horizontally through umblicus. Grade 5-lowest palpable point below umblical level but not projected beyond a horizontal line situated halfway b/w umblicus and symphysis pubis. Grade 6-lowest palpable point beyond lower limit of grade 5.
  • 58.
  • 59.
  • 60. Grading according to size of spleen below LCM: MILD palpable <3cms below LCM MODERATE 4-7 below LCM SEVERE >7cms below LCM.
  • 61. Clinical E/o : Size & Degree: • it usually enlarges towards RIF. • it is measured as child takes a deep breath from a point on LCM in MCL to the tip of the enlarged spleen. Margin: • Splenic notch is felt on the Ant. border & has a sharp margin. • Diff from kidney where there is absence of notch & margin is round
  • 62. Spleen vs. Kidney Spleen • Splenic notch. • Can cross midline. • Can’t get above. • Moves down on inspiration. • Not ballotable. • Splenic rub. Kidney • No notch. • Never cross the midline. • May get above. • Doesn’t move with respiration. • Ballotable. • No rub.
  • 63. • Inspection • Look in left Hypochondrium. Examination of the Spleen
  • 64. Percussion of spleen Normal • Left midaxillary line 9th –11th intercostal space width 4-7cm. • Enlargement of splenic dullness: splenomegaly.
  • 65. Percussion (3 methods): • Percussion of Traube's Space boundaries – Left anterior axillary line 6th rib costal margin . • This area should be resonant on percussion. • Dullness indicates possible splenic enlargement
  • 66.
  • 67. Percussion by Castell’s method : • percuss in the lowest Left intercostal space in the anterior axillary line (usually the 8th or 9th IC space) • this space should remain resonant during full inspiration . • dullness on full inspiration indicates possible splenic enlargement (a positive Castell’s sign)
  • 68. Percussion by Nixon’s method: • place the patient in Right lateral decubitus • begin percussion midway along the Left costal margin proceed in a line perpendicular to the Left costal margin • if the upper limit of dullness extends >8 cm above the Left costal margin, this indicates possible splenomegaly
  • 69.
  • 70. Palpation of spleen • To palpate the spleen, the patient is in the supine position with the knees flexed to decrease abdominal muscle tone. • Begin the examination by palpating the right lower quadrant and move upward across the abdomen as the patient.
  • 71. Palpation (3 methods) Method #1: • begin palpation in the RLQ. • direct the patient's breathing by telling them when to take a deep breath and when to exhale while proceeding diagonally towards the Left Upper Quadrant (LUQ), try to palpate the spleen edge during each inspiratory phase
  • 72. Method #2: • place the patient’s Left fist under their Left posterior chest. • With your Right hand, begin palpation in the RLQ. • Direct the patient's breathing by telling them when to take a deep breath and when to exhale while proceeding diagonally towards the LUQ, try to palpate the spleen edge during each inspiratory phase
  • 73. Method #3 –The Hooking maneuver of Middleton (optional): • Place the patient’s Left fist under their Left posterior chest position yourself on the patient’s Left side, facing the patient’s feet. • Using both hands, curl your fingers under the patient’s Left costal margin ask the patient to take a long, deep breath à attempt to palpate the spleen with your fingertips
  • 74. Percussion of spleen • Percussion is also used to delineate the size of the spleen. • Percussion is only approximately 60% accurate in most studies, with palpation about 50% accurate.
  • 75. Consistency: soft 1, normal 2,septicemia 3,enteric fever 4,infectious mononucleosis firm 1,cirrhosis 2,lymphoma 3,leukemia 4,chr.malaria hard secondaries
  • 76. Tenderness: s/I infective endocarditis splenic abscess splenic infarction Surface: smooth congestive causes like portal HTN irregularhydatid cyst
  • 77. • Spleen moves downwards and medially during inspiration. • Fingers cannot be insinuated btw enlarged spleen and LCM • Spleenic rub is palpable in spleenitis
  • 78. Mechanism of splenomegaly: • Reactive Reticulo-endothelial hyperplasia • Lymphoid hyperplasia • Proliferation of lymphoma cells • Infiltration by abnormal cells • Extramedullary hemopoeisis • Proliferation of macrophages d/t RBC destruction • Vascular congestion
  • 79. Symptoms and signs • Abdominal pain/tiredness. • Early satiety due to splenic encroachment. • Symptoms of anemia due to accompanying cytopenia. • Febrile illness (infectious). • Pallor, dyspnea, bruising, and/or petechiae (hemolytic process).
  • 80. Symptoms and signs • History of liver disease (congestive). • Weight loss, constitutional symptoms (neoplastic). • Pancreatitis (splenic vein thrombosis). • Alcoholism, hepatitis (cirrhosis).
  • 81. Signs • Palpable left upper quadrant abdominal mass. • Splenic rub. • Lymphadenopathy.
  • 82. Symptoms and signs • Signs of cirrhosis (eg, asterixis, jaundice, telangiectasias, gynecomastia, caput medusa, ascites). • Heart murmur (endocarditis, congestive failure). • Jaundice (spherocytosis, cirrhosis). • Petechiae (any cause of thrombocytopenia).
  • 83. Causes of splenomegaly • Infective • Hyperplastic • Congestive • Infiltration
  • 84. infective • Acute & subacute- IMN, infective endocarditis, severe pyogenic inf. Viral hepatitis,CMV,AIDS • Chronic - TB,syphilis,brucellosis • Tropical splenomegaly • Malaria,kala azar, trypanosomiasis
  • 85. congestive • Intra hepatic obst.portal hypertension - cirrhosis,biliary cirrhosis,hemochromatosis - primary sclerosing cholangitis • Extra-hepatic portal hypertension - venous malf,thrombosis,stenosis - ext.occlusion of portal,splenic vein • Chronic passive congestion of cardiac origin
  • 86. hyperplastic • Extramedullary hemopoeisis- myeloprolif.d/s - marrow damage - marrow infiltration • Reticulo endothelial hyperplasia –(abn.RBC) - sickle cell d/s,spherocytosis,Hbnopathies, thalassemia major,PNH
  • 87. infiltrative • Malignant infiltration- CML,lymphoblastic - lymhomas, MPD, - angiosarcoma,tumors - metastasis (melanoma) • benign - - storage d/s –Gaucher’s,Neiman-pick - amyloidosis - hurler’s syndrome,MPS - cysts,fibromas,hemangiomas,hamartomas -Eosnophilic granulomas
  • 88. Disordered immunoregulation • Felty’s syndrome- RA+ splenomegaly+leucopenia • Systemic lupus erythromatosis • Collagen vascular diseases • Sarcoidosis • Immune thrombocytopenia
  • 89. Splenomegaly in children • Metastatic neuroblastoma. • Infection. • Autoimmune: juvenile rheumatoid arthritis. • Haemolysis: hereditary spherocytosis, sickle cell anaemia, Thalassaemia • Neoplasia: ALL, Hodgkin disease and NHL, acute or chronic myeloblastic leukemia, neuroblastoma. • Inherited diseases: Gaucher's disease and other storage disorders.
  • 90. Massive splenomegaly (>8cm >1000gm) • Myeloproliferative disorder • Chronic malaria,kala-azar (trop. Splenomegaly) • Storage disorders • Thalassemia major • Sarcoidosis • Hairy cell leukemia • Gaucher disease • Diffuse splenic hemangiomatosis
  • 91. Moderate splenomegaly(4-8cm) • Cirrhosis • Lymphomas‘ • Amyloid • Splenic abscess,infarct • Hemolytic anemias • IMN
  • 92. Mild splenomegaly (1-3cm) • Acute infective conditons • Acute malaria,tyhoid,kala-azar,septicemias
  • 93. Special situations associated with splenomegaly • Fever- typhoid,malaria,kalaazar, infect.endocarditis, leukemia,lymphoma • Tender spleen- rupture,abscess,infarct • a/c illness+ anemia- AIHA,leukemia • Fever + LN- IMN,leukemia,lymhomas,SLE,sarcoid • Anemia- hemolytic anemia,hemoglobinopathies • Jaundice – cirrhosis,hemolytic anemia • Pulsatile spleen- aneurysm • High ESR- connective tissue disorder • Leukopenia- felty’s syndrome,septicemia
  • 94. Step-wise approach to splenomegaly • History • Physical examination • Laboratory testings • Imaging • Specialised testing
  • 95. history • Age ,gender • Race • h/o recent infections like malaria • Fever,weight loss,sweating (lymphomas,infections) • Pruritis • Abnormal bleeding/bruising • Joint pain • h/o alcholism • h/o trauma • h/o neonatal umblical sepsis • Residence & travel abroad
  • 96. History …..cont • Jaundice • High risk sexual behavior (AIDS) • Past medical history • Drugs
  • 97. Physical examination • Size of the spleen • Hepatomegaly • Lymphadenopathy • Fever • Icterus • Bruising,petechiae • Oral & supf.sepsis • Stigmata of liver disease • Stigmata of RA/SLE • Splinter hemorrhage,retinal hemorrhage • Cardiac murmurs
  • 98. Lab investigations • CBC • Blood smear • Retic count • Blood C/S • Serology (fungal,viral,parasitic) • LFT • Hb electropheresis/ coombs test • Coag.profile • Amylase/lipase • AMA, Anti CCP,RA factor • Bone marrow analysis
  • 99. Hypersplenism Criteria for a diagnosis of hypersplenism: • anemia. • Leukopenia. • Thrombocytopenia. • combinations thereof, plus cellular bone marrow, splenomegaly, and improvement after splenectomy.
  • 100. Approach to Splenomegaly Depends on Pretest Probability • Clinical Suspicion of Splenomegaly (>10%). • Percuss first and if positive palpate. • If percussion is negative and suspicious, order an ultrasound. • If percussion positive but palpation is negative, order an ultrasound. • Both percussion and palpation positive = SPLENOMEGALY.
  • 101. Diagnostic Approach • CBC provides information about hematological, infectious, and inflammatory processes. • Finding of pancytopenia, Anemia, Leukopenia, Thrombocytopenia may indicate bone marrow dysfunction or portal hypertension with hypersplenism.
  • 102. Laboratory tests Routine tests : • CBC, platelet count, sedimentation rate. • chemistry panel, febrile agglutinins, serum haptoglobins, ANA test, Monospot test, serum protein electrophoresis, tuberculin test. • chest x-ray, EKG, and flat plate of the abdomen.
  • 103. Diagnostic Approach • Increased sedimentation rate suggests infectious, inflammatory, or neoplastic process. • Bacterial, fungal, and other cultures may be performed with suspected infection.
  • 104. Diagnostic Approach • Bone marrow exam is useful in diagnosis of histiocytoses, lysosomal storage disorders, and some infections(e.g., disseminated histoplasmosis).
  • 105. Diagnostic Approach • Liver function tests and abdominalU/S with Doppler methods should be performed with suspected portal hypertension. • Abdominal U/S and CT locate and define extent of splenic masses
  • 106. If there is jaundice • A hepatitis profile, red cell fragility test, and blood smear for parasites should be done. If there is fever. • Serial blood cultures, leptospirosis antibody titer, and smear for malarial parasites should be done. Laboratory tests
  • 107. Laboratory tests If there is a petechial rash • A coagulation profile should be done. To rule out malignancies • Lymph node biopsies and bone marrow examinations may be necessary.
  • 108. Imaging • USG- sensitive & specific non-invasive • CT scan – etiology of splenomegaly - liver size,heterogenecity - splenic mets, abscess,calcf.,cysts - retro peritoneal LN - craniocaudal ln > 10 cm • Liver- spleen colloid scan- (RBC –Cr51,Tc99) - hepatic steatosis,SOL,splenic functions - PHT,colloid shift + • MRI/ Doppler usg- portal/splenic vein thrombosis - cavernomas
  • 109. imaging • MRI scan- liver hemangiomas hemochromatosis erlenmeyer flask sign(Gaucher) • PET scan - Dx & staging of lymphomas - determine metabolic cells in spleen
  • 110. Imaging Studies Splenoportography o This modality is used to evaluate portal vein patency and the distribution of collateral vessels before shunt operations for cirrhosis. o Findings can help identify the cause of idiopathic splenomegaly, especially in children. • Angiography: Angiographic findings are used to differentiate splenic cysts from other splenic tumors.
  • 111. Imaging Studies • Liver-spleen colloid scanning o Erythrocytes are labeled with chromium-51 (51 Cr) , mercury-197 (197 Hg), rubidium-81 (81 Rb), or technetium-99m (99m Tc), and the cells are altered by treatment with heat, antibody, chemicals, or metal ions so that the spleen sequesters them after infusion. o A spleen length >14 cm is consider enlarged on liver- spleen scan
  • 112. Specialised testing • Abd.fat pad aspiration-amyloidosis • JAK-2 mutation • Gene testing(bcr-abl ,C282Y)-leukemia • Enzyme testing-storage • Lymph node biopsy-infection,malignancy • FNAB spleen • Splenectomy-hyperspleenism • Lung or skin biopsy • Liver biopsy
  • 113. summary • Splenomegaly – major physical finding • Step wise approach- history,physical exam • Look for associated features • Lab investigation & Imaging • Search for etiology & treat
  • 114. • References: 1,Gupte S. Differential Diagnosis in Pediatrics,5th edn Nw Delhi: Jaypee 2008. 2,Nelson text book of pediatrics - 20th edn .