Approach in children with Hepatosplenomegaly To summarize the key points: 1. A full examination including inspection, palpation, percussion and auscultation of the abdomen should be performed to evaluate for hepatosplenomegaly. 2. Common causes include infections, hematological disorders, vascular congestion, tumors and infiltrations, and storage disorders. 3. Initial investigations should include a complete blood count, liver function tests, ultrasound and further testing based on history and exam findings. 4. Treatment is directed at the underlying cause and may include antibiotics for infections, chemotherapy for tumors, or management of metabolic disorders.

1. Approach To A Child With
Hepatosplenomegaly
Dr. Virendra Kumar Gupta
MD Pediatrics,MIAP
Fellowship In pediatric
Gastroentero-Hepatology & Liver
Transplantation
Assistant Professor
Institute of Paediatric Gastroenterology
Nims University Jaipur

2. ABDOMEN
Midclavicular lines
Transpyloric
Trans tubercular line

3. ORDER OF EXAMINATION
• Inspection
• Palpation
• Percussion
• Auscultation

4. INSPECTION
– Shape of the abdomen and flanks
– Skin –scar ,striae ,prominent veins ,umbilicus ,
visible peristalsis
– Movements of abdominal wall with respiration
– Ask the patient to cough and look for hernial
orifices

5. 1. Ensure that your hands are warm
2. Stand on the patient’s right side
3. Help to position the patient
4. Ask whether the patient feels any pain before
you start
5. Begin with superficial examination
6. Move in a systematic manner through the
abdominal quadrants
7. Repeat palpation deeply.

6. LIGHT PALPATION

7. DEEP PALPATION

8. PERCUSSION

9. Hepatomegaly
APPROACH TO A CHILD WITH
HEPATOMEGALY
Let me examine him fully before I can say
that this person is dead !!

10. First be sure it is
hepatomegaly and
not a pushed down
liver !!!!
Always assess
Liver span
Consistency
Surface

11. OBJECTIVES
• Location and extent of liver
• Clinical presentation
• Inspection
• Palpation
• Percussion
• Auscultation
• Causes

12. LOCATION AND EXTENT OF LIVER
• Location
– Right hypochondric region
– Epigastric region
– Left hypochondric region
• Extent
– Upper border- 6th rib
– Inferior border- crosses
midline at the level of
transpyloric plane (at the
level of L1 vertebrae)

14. CLINICAL PRESENTATION
• Pain in right hypochondrium
• Jaundice
• Abdominal distension
• Abdominal discomfort
• Fever
• Malaise
• Breathlessness

15. INSPECTION
• Skin – engorged veins
• Position of umbilicus
• Movement with respiration
• Size, shape and extent of swelling

16. PALPATION OF THE LIVER
1. Flex the knee joint
2. Ask the patient to take a deep breath in
3. Start palpating in the right iliac fossa
4. Move hand progressively further up the abdomen
5. Try to feel the liver edge
6. Check for tha liver span.

17. PALPATION
Dipping method Hook method

18. FINDINGS TO BE NOTED IN PALPATION
• Local rise of temperature
• Tenderness
• Extent of enlargement below costal margin
• Character of the edge – sharp or rounded
• Surface – smooth, irregular or nodular
• Consistency – soft, firm or stony hard

19. PERCUSSION
• Mid axillary line
• 6th rib to costal margin
• Upper limit of liver
dullness is raised in
– Subphrenic abscess
– Hydatid cyst
• Hydatid thrill

20. AUSCULTATION
• Hepatic rub – Hepatocellular carcinoma
Liver abscess
• Arterial bruit – Malignancy
Acute alcoholic hepatitis

21. 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

22. NORMAL RANGE FOR LIVER SPAN
–1 week of age - 4.5 to 5 cm
–At 5 yrs of age- 6 to 8 cm
–12 years, boys - 7 to 9 cm
girls - 6 to 8cm

23. SURFACE MARKINGS :
Upper border:4 th ICS in MCL
Lower border:9 th ICS in MCL
Lateral border:6 th rib in MAL

24. GRADES OF HEPATOMEGALY
Mild- <4cms below Rt. Subcostal Margin.
Mod- 5-7cms “ “
Mass- >7cms “ “

25. HEPATOMEGALY- FIVE MECHANISMS
– Infections
– Excessive storage
– Infiltration
– Congestion
– Obstruction

26. 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

27. 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.

28. 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

29. 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

30. 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

31. • 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

32. Hepatosplenomegaly - causes
• Infections
• Haematological disorders
• Vascular congestion
• Tumours and Infiltrations
• Storage disorders
• Miscellaneous causes

33. Infections
i) Acute infections -
a)Protozoal - Malaria, kala-azar, toxoplasmosis
b)Bacterial - Typhoid, sepsis
c)Spirochaetal - Leptospirosis
d)Viral -Infectious mononucleosis, cytomegalo virus

34. ii) Chronic infections -
a)Mycobacterial - Disseminated tuberculosis
b)Protozoal -Malaria,kala-azar,toxoplasmosis
c)Spirochaetal - Congenital syphilis
d)Viral - HIV, Rubella, herpes, cytomegalovirus infection
e)Bacterial - Brucellosis
f)Fungal - Histoplasmosis

35. Haematological disorders
i)Iron-deficiency anaemia
ii) Haemolytic disorders -
a)Thalassaemia
b)Hereditary spherocytosis
c)Sickle cell anaemia
d)Autoimmune haemolytic anaemias
e)Isoimmunization disorders - Rh and ABO
incompatibility

36. Vascular congestion
i)Congestive cardiac failure
ii)Constrictive pericarditis
iii)Cirrhosis -
a) Hepatitis
b) Chronic active hepatitis
c)Biliary atresia
d)Cystic fibrosis
e)Wilson's disease
f)Galactosemia
g)Alpha-l-antitrypsin deficiency
h)Haemosiderosis

37. Tumours and Infiltrations
i)Leukaemia - Acute lymphocytic leukaemia
ii)Lymphomas — Hodgkin's and non-Hodgkins lymphoma
iii)Metastatic disease - Neuroblastoma
iv)Histiocytosis X

38. Storage disorders
i) Lipid storage diseases -
a)Gaucher disease
b)Niemann-Pick disease
c)Gangliosidoses
d)Mucolipidoses
ii) Mucopolysaccharoidoses
a)Hurler's syndrome
b)Hunter's syndrome
iii) Glycogen storage disease - Type IV
iv) Amyloidosis

39. Miscellaneous causes
i) Serum sickness
ii) Connective tissue disorders
a)Juvenile rheumatoid arthritis
b)SLE
iii) Sarcoidosis

40. 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

41. •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

42. Approach in children with Hepatosplenomegaly
To summarize

43. Approach in neonates with Hepatosplenomegaly

44. THANK YOU
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45. SPLEENOMEGALY

46. SPLEENOMEGALY
• It refers to enlargement of spleen beyond its normal
size.
• A spleen is said to be significantly enlarged if it is
palpable at least 1cm below costal margin in a child
more than 6 months of age.
• In 30% of newborns & 15% of infants <6months
palpable spleen is a normal variant.

47. ANATOMY
• It lies within the left upper
quadrant of the peritoneal
cavity.
• Abuts ribs 9-11, stomach, left
kidney, splenic flexure of the
colon, and tail of the pancreas.

48. ANATOMY
Normal Spleen
• Autopsy: <250g.
• Radioisotope Scintiscan: 12cm long x 7cm wide.
• Ultrasound: 11cm cephalocaudad diameter.
• ~3% of healthy people have splenomegaly.

49. 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.

50. 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.

51. Grading of spleenomegaly:
Grade1-normal,not palpable even on deep
inspiration.
Grade2-palpable just below costal margin usually on
deep inspiration.
Grade3-palpable below costal margin but not
projected beyond a horizontal line half way b/w
costal margin and umblicus.

52. Grade4- lowest palpable point approaching the
umblical level but not below a line drawn
horizontally through umblicus.
Grade5- lowest palpable point below umblical level
but not projected beyond a horizontal line situated
halfway b/w umblicus and symphysis pubis.
Grade6- lowest palpable point beyond lower limit of
grade 5.

55. Grading according to size of spleen
below LCM:
MILD palpable <3cms below LCM
MODERATE 4-7 below LCM
SEVERE >7cms below LCM.

56. 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

57. Spleenv/s. 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.

58. • Inspection
• Look in left
Hypochondrium.
Examination of the Spleen

59. 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.

60. Palpation (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

61. Method #2:
• Ask the patient to lie on their
right side.
• Support the rib cage with your
left hand
• And again ask the patient to
take deep breaths in moving
your right hand up towards
the left hypochondrium.
• Try to palpate the spleen edge
during each inspiratory phase

62. 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.

63. Percussion of spleen
Normal
• Left midaxillary line 9th –11th intercostal space
width 4-7cm.
• Enlargement of splenic dullness: splenomegaly.

64. 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. 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)

67. 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

68. Consistency:
soft 1, normal
2,septicemia
3,enteric fever
4,infectious mononucleosis
firm 1,cirrhosis
2,lymphoma
3,leukemia
4,chr.malaria
hard secondaries

69. Tenderness:
s/I infective endocarditis
splenic abscess
splenic infarction
Surface:
smooth congestive causes like portal HTN
irregularhydatid cyst

70. • Spleen moves downwards and medially during
inspiration.
• Fingers cannot be insinuated btw enlarged spleen
and LCM
• Spleenic rub is palpable in spleenitis

71. 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

72. 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).

73. Symptoms and signs
• History of liver disease (congestive).
• Weight loss, constitutional symptoms (neoplastic).
• Pancreatitis (splenic vein thrombosis).
• Alcoholism, hepatitis (cirrhosis).

74. Signs
• Palpable left upper quadrant abdominal mass.
• Splenic rub.
• Lymphadenopathy.

75. 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).

76. Causes of splenomegaly
• Infective
• Hyperplastic
• Congestive
• Infiltration

77. infective
• Acute & subacute- IMN, infective endocarditis,
severe pyogenic inf.
Viral hepatitis,CMV,AIDS
• Chronic - TB,syphilis,brucellosis
• Tropical splenomegaly
• Malaria,kala azar, trypanosomiasis

78. 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

79. hyperplastic
• Extramedullary hemopoeisis- myeloprolif.d/s
- marrow damage
- marrow infiltration
• Reticulo endothelial hyperplasia –(abn.RBC)
- sickle cell d/s,spherocytosis,Hbnopathies,
thalassemia major,PNH

80. 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

81. Disordered immunoregulation
• Felty’s syndrome- RA+ splenomegaly+leucopenia
• Systemic lupus erythromatosis
• Collagen vascular diseases
• Sarcoidosis
• Immune thrombocytopenia

82. 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.

83. 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

84. Moderate splenomegaly(4-8cm)
• Cirrhosis
• Lymphomas‘
• Amyloid
• Splenic abscess,infarct
• Hemolytic anemias
• IMN

85. Mild splenomegaly (1-3cm)
• Acute infective conditons
• Acute malaria,tyhoid,kala-azar,septicemias

86. 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

87. Step-wise approach to splenomegaly
• History
• Physical examination
• Laboratory testings
• Imaging
• Specialised testing

88. 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

89. History …..cont
• Jaundice
• High risk sexual behavior (AIDS)
• Past medical history
• Drugs

90. 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

91. 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

92. Hypersplenism
Criteria for a diagnosis of hypersplenism:
• anemia.
• Leukopenia.
• Thrombocytopenia.
• combinations thereof, plus cellular bone marrow,
splenomegaly, and improvement after splenectomy.

93. 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.

94. 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.

95. 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.

96. Diagnostic Approach
• Increased sedimentation rate suggests infectious,
inflammatory, or neoplastic process.
• Bacterial, fungal, and other cultures may be
performed with suspected infection.

97. Diagnostic Approach
• Bone marrow exam is useful in diagnosis of
histiocytoses, lysosomal storage disorders,
and some infections(e.g., disseminated
histoplasmosis).

98. 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

99. 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

100. 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.

101. 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

102. imaging
• MRI scan- liver hemangiomas
hemochromatosis
erlenmeyer flask sign(Gaucher)
• PET scan - Dx & staging of lymphomas
- determine metabolic cells in spleen

103. 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.

104. 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

105. 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

106. Approach in children with Hepatosplenomegaly
To summarize

107. Approach in neonates with Hepatosplenomegaly

108. THANK YOU
……………….WAKE UP