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Hepatosplenomegaly in Pediatrics

Hevi Pediatric Hospital

sami aswad

on 7 February 2014

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Transcript of Hepatosplenomegaly in Pediatrics

(commonly abbreviated HSM) is non-specific medical sign, is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly), to an extent that can not be regarded normal variant.
Hepatosplenomegaly with Bleeding Manifestations
1. Infective:
• Viral hemorrhagic fever: dengue, septicemia
• Intrauterine infections
2. Hematological:
• Chronic ITP(splenomegaly unlikely in acute ITP)
• Thalassemia (petechi secondary to hypersplenism)
• Henoch-schonlein purpura
Approach to patients with Hepatosplenomegaly
1. Age: neonate v.s older children
2. Sex
3. Residence or Travel history – endemic diseases
4. Is there any hx of trauma?
5. Fever, malaise ?
6. Is there any history of drug or toxin ingestion? Is there any exposure to radiation?


1. Spleen: Anatomy, Function
2. Isolated Splenomegaly
3. Liver: Anatomy, Function
4. Isolated Hepatomegaly
5. Hepatosplenomegaly
6. Approach to Hepatosplenomegaly

Under Supervision of:
Dr. Ghazi
Prepared by:
Sami Ibrahim Aswad

Clinical Examination
Hepatosplenomegaly can therefore be due to various causes and to narrow down the DDx one should consider the associated symptom such as fever, anemia, jaundice ….
Hepatosplenomegaly with ascites

• Abdominal or disseminated T.B
• Cirrhosis with portal hypertension
• Malignancy
Thank You!
1. Clears microorganisms from blood
2. Synthesis of immunoglobulins
3. Destruction of abnormal RBCs
4. Embryonic and extramedullary hematopoiesis


Spleen is
palpable in:
30% of newborn
15% of children under the age of 6 months

is when the spleen is palpable more than
1 cm
below the left costal margin in a child over
6 months
of age

sually caused by systemic disease and not by primary splenic disease.

Because of exposure below the protective rib cage splenomegaly results in increased risk of splenic injury or rupture.

Degree of enlargement

Mild splenomegaly

spleen <3 cm below left costal margin
Lower border is in the left hypochondrial area

Moderate splenomegaly
3-7 cm
Lower border is reaching the left lumber areas

Massive splenomegaly
>7 cm
Lower border is reaching up to the umbilicus sometime may reach right iliac fossa

Causes of isolated splenomegaly

• Splenic abscess
• Splenic tumor
• Splenic vein thrombosis
• Hereditary spherocytosis
• Infective endocarditis

Functions Of Liver

In childhood the liver is normally soft and palpable 2 cm below right costal margin

The liver size is determined by measuring its vertical span and its span varies with age:

Apparent hepatomegaly:
in this case liver may be
palpable with normal vertical span
, it is found in following condition:
• Pneumothorax
• Bronchiolitis
• Emphysema
• Asthma
• Subdiaphragmatic abscess
• Pectus excavtum
• Spinal deformity
• Narrowed or flared costal margin
• Rickets
• Abdominal tumor

Hepatomegaly can be classified to:
1. Mild: <4 c.m below right costal margin or Lower border is in the right hypochondriac area
2. Moderate: 4-7 cm or Lower border is reaching the right lumbar area
3. Massif: >7 cm or Lower border is reaching the right iliac fossa

Isolated hepatomegaly:
• Viral hepatitis
• Malnutrition
• Hepatoblastoma
• Hepatocellular carcinoma
• Amebic abscess
• Hydated cyst
• Hypervetamenosis
• Benign tumor (hemangiomas ,teratomas)
• Ascariasis

Important causes of hepatosplenomegaly
1. Infections:
• Viral: hepatitis viruses, Epstein-barr virus (EBV), HIV, TORCH(other viruses, rubella, CMV, herpes virus)
• Bacterial: typhoid, TB, brucellosis
• Spirochetal: leptospirosis
• Protozoa: malaria, kala-azar, Toxoplasmosis
• Fungal: histoplasmosis

2. Hematological:
• Hemolytic anemia: thalassemia major, hereditary spherocytosis, autoimmune, isoimmunization (ABO and RH incompatibility)
• Malignancy: leukemia, lymphoma
• Idiopathic myelofibrosis

3. Congestion:
• Restrictive pericardial disease
• Budd-Chiari syndrome
• Veno-occlusive diseases
• Schistomiasis
• Billiary Artesia

4. Metabolic:
• Wilson disease
• alpha-1-antitrypsin deficiency

5. Storage disorders:
• Glycogen strage dis.
• Lipid storage dis: Gaucher disease, Niemann-Pick disease
• Mucopolysaccharidosis

6. Miscellaneous:
Hepatosplenomegaly with pallor

1. Infective:

• Malaria
• Kala azar
• Infective endocarditis
• Disseminated T.B

2. Hematological:
• Thalassemia
• Sickle cell anemia
• Hereditary spherocytosis
3. Metabolic:
• Wilson's disease
• Gaucher's disease

4. Malignancy:
• Leukemia
• Lymphoma
5. Cirrhosis with portal hypertension

6. Collagen vascular disease:
• Juvenile Rheumatoid arthritis
Hepatosplenomegaly with icterus

1. Infective:
• Hepatitis
• Disseminated TB(icterus is more due to anti-TB drugs)
• Malaria
2. Hematological:

• Thalassemia
• Hereditary spherocytosis

3. Metabolic:
• Galactosemia
• αalpha- 1 antitrypsin deficiency
4. malignancy:
• lymphoma
• leukemia
5. cirrhosis

6. drugs:
• Anti-TB
• Anti-convulsants
3. Malignancy
• Leukemia
• Lymphoma
4. Cirrhosis with hypersplenism
5. Metabolic:
• Gaucher's disease
• Nieman-pick disease
epatosplenomegaly with lymphadenopathy:
1. Infective:
• Disseminated T.B
• Toxoplasmosis
• Kala-azar
2. Hematological:
• Thalassemia
3. Malignancy:
• Leukemia
• Lymphoma
• Histocytosis
4. Drugs:
• Phenytoin
• Dapsone
5. Collagen vascular disorder:
Hepatosplenomegaly with fever:
• Infection:
malaria, kala-azar, enteric fever
• Malignancy:
Hepatosplenomegaly with FTT:
• Infection:
disseminated T.B, HIV ,in newborn (TORCH), malaria
• Storage disorder:
glycogen storage dis, Niemann-Pick disease
Hepatosplenomegaly with neurological manifestations:

• Metabolic disorder:
wislon's disease
• Storage disease:
glycogen storage dis, Niemann-Pick disease, gaucher
Hepatosplenomegaly with renal involvement:
• Metabolic disorder:
wislon's disease
• Storage disease:
glycogen storage dis
• Infection:
hepatits B
• CHF with polycystic kidney dis
• Hereditary fructose intolerance
• Leukemia
8. Has the child had any change in stool color?
9. Weight loss ?
10. SOB or cough ?
11. Loss of consciousness .. Seizures ?
12. Is there any hx of rash bleeding, bruising, Pruritis?!!
13. Were there any complications during pregnancy, delivery, and after delivery?
14. Is there any maternal history of hepatitis B or C, CMV, EBV or HIV?
15. Does the child have any preexisting liver diseases, lung diseases, or congenital heart diseases?
16. Nutrition history (neonatal formula)
17. Developmental milestones
18. Is the child on any medications right now?
19. Is there any family history of cystic fibrosis, alpha-1 antitrypsin deficiency, storage
diseases, liver diseases, heart diseases, autoimmune diseases or malignancy?

General examination
: due to
- malaria, kala-azar, bacteremia
haemolytic anaemia
- hereditary spherocytosis, sickle cell anaemia, thalassaemia, autoimmune haemolytic anaemia
- iron deficiency
Leukemia and lymphoma
Jaundice -
Liver disease / haemolytic anaemia
Petechiae, purpura, ecchymosis,lymphadenopathy -
Koilonychia, platynychia -
Iron deficiency
Stigmata of liver disease.
Mental retardation-
Tender hepatomegaly
- Viral hepatitis, CCF, liver abscess, enteric fever
Firm consistency liver with sharp edge
- Cirrhosis, constrictive pericarditis
Just palpable soft spleen
- Enteric fever, infective endocarditis, etc.
- Suggests cirrhosis with portal hypertension, malignancy, TB
Systemic examination
Raised JVP
- CHF, constrictive pericarditis
may be present because of bone marrow infiltration.
WBC Count:
neutropenia or neutrophilia (infection or leukemia)
Reticulocyte count
- High in haemolytic anaemia
Peripheral smear -
(Blast cells)
(hypochromia, nucleated RBC's, target cells)
Sickle cell anaemia
(sickling on treatment with 2% sodium metabisulphite)
Parasitic diseases
Viral infection
(atypical lymphocyte)
Platelet count
: their may
due to:
decreased production
(bone marrow infiltration)
increased destruction
( immunologic causes, drug reactions, or viral infections)
sequestration or hypersplenism.

- Elevated in inflammatory diseases
Liver function tests:
Serum Albumin
- Low in kwashiorkor
- Raised in hepatitis & hepatic necrosis
Alkaline phosphatase
- Elevated in hepatobiliary obstruction & liver abscess
Bilirubin (total, direct)
- Haemolytic anaemias(indirect)
High prothrombin time
- Liver parenchymal dysfunction
Obtain viral-antibody titers:
to detect EBV, CMV, and HIV.

may reveal bacterial, fungal, or other infections.

B.M aspiration and biopsy:
when leukemia or lymphoma, is suspected.

Antinuclear antibodies ,anti-DNA & serum component:
when collagen vascular dis. Is suspected.

Miscellaneous tests
Raised alpha foeto protein
- Hepatoblastoma
Hbs Ag
- Hepatitis B
High sweat chlorides
- Cystic fibrosis
Low ceruloplasmin
-Wilson's disease
Urine and stool examination
- In case of jaundice
Liver biopsy
- Pathological diagnosis
Imaging Studies:
For any evidence of mediastinal or hilar adnopathy in (T.B or lymphoma)

ECG, echocardiography
if cardiac cause suspected
can confirm the presence of the enlarged spleen ,liver or space-occupying lesions (cyst or abscess), Ascites .

CT scanning and MRI:
Clarifying abnormalities in (size,shape,presence LAP), To differentiate biliary atresia from neonatal hepatitis
To Summarize
Therapy is directed at treatment of underlying disease
–Consider interferon for hepatitis B
–Consider interferon and ribaviron for hepatitis C

Metabolic disease
–Metabolism consultation
–Often requires specific restricted formulas

Immune suppression for
autoimmune hepatitis
Chemotherapy for,
Regarding liver:
treatment is correction of underling etiology.
some time when there is cyst, abscess or billiary disorder surgery may indicated.
Regarding spleen:
the primary goal is treatment of the underlying disease.
splenectomy may be the therapy of choice for symptoms or complications caused by the enlarged organ.
may be helpful in several medical conditions, including:
1- Congenital anemias:
(hereditary spherocytosis, elliptocytosis)
2- Autoimmune disorders
: immune thrombocytopenic purpura,
autoimmune hemolytic anemia
3- Thalassemia major:
splenectomy decreases transfusion requirements caused by hypersplenism.
4- Gaucher disease:

If splenectomy is performed, immunize at least 10 days prior
–Haemophilus influenzae
if under 5 years –Meningococcal vaccine

–Postsurgical penicillin prophylaxis required

According to the cause

A 23-month-old previously well child failed to waken normally from sleep.
Physical examination
revealed a well-developed child unresponsive to verbal, tactile, or painful stimuli in decerebrate posturing with a fixed and dilated left pupil. He appeared
with abundant
and moderate
. An MRI of his head revealed the above image. Dx?!!
Now back to the Case
Causes of massive splenomegaly
1. CML
2. Myelofibrosis
3. Malaria
4, Kala Azar
5. Lymophoma
6. Storage Disorders
1. Partha's Fundamentals of Pediatrics
2. A Practical Approach to Clinical Pediatrics
3. Nelsons text book of pediatrics, 19th edition.
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