clinical correlation of HSM

1. HEPATO & SPLEENOMEGALY
Dr.Subash Arun

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

47. FINAL DIAGNOSIS
GLYCOGEN STORAGE DISORDER
TYPE 1

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.

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

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

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 .