Anupa mishra

An Approach to Splenomegaly

BY DR ANUPAGUIDED BY: DR MANOJ GHODA

DR REKHA H BHAVSARDR ROHIT R MODI

DR DEEPA A BANKERDR PALLAVI P DAGLI

A 7 years old male child born out of a non consanguinous marriage presented with the following complaints:

Sumit’s story!!!

An umbilical hernia since age of 6 months which was gradually increasing in size

Noisy breathing/ snoring at night

Swelling of small joints of fingers with restricted movements of both wrists and fingers

Coarse facies

Umbilical hernia and protuberant abdomen

Short stature

Short spade like hands

A huge hepatosplenomegaly Systolic murmurCNS: mild delay of milestonesOpthalmologic exam: normal

Systemic examination

Hb: 12gm% WBC count: 13000 Differential count:

71% neutrophils & 22% lymphocytes

PS-non specific ESR: 15 CRP - negative S.Ca2: 10.2

S. Mg2+: 2.5 S. phosphorus: 4.7 ALP : 287

Investigations

Chest Xray: scoliosis, oar shaped ribs

X ray of the wrist and hand: widening of metaphysis of wrist joints, short and widened phalanges..typical features of dysostosis multiplexa.

2 D echo: moderate MR, mild TR, Mild PAH, borderline LVH bicuspid aortic valve

Special investigations done:Liver biopsy: s/o some storage disorder

Urine for GAGs: positive( type???)

For definitive diagnosis:Enzyme assays on culture fibroblalsts or serum leukocytes

Now that was simple wasn’t it??

A book picture of MUCOPOLYSACCHARIDOSIS!!

But what if such was not the case??!!

Very often what we have is only a large spleen!!

What to do then??

Lets see!!

CONGESTIVE

HEMATOLOGICAL

PARASITIC INFECTIONS

CHRONIC INFECTIONS

AUTOIMMUNE CONDITIONS

STORAGE DISORDERS

Age?

Residence?

Community?

Ask the following questions...

Is thereFever? Duration?Pallor?Bleeding from any site?Rashes?Transfusion of blood or blood products?

Blood in vomitus?Jaundice?Convulsions?Involuntary movements or developmental delay?

Family history?Maternal story?

Facies and skull Pallor Jaundice Rashes/ petehiae/purpura/ echhymosis

Lymphadenopathy Eyes Anthropometry

On examination:

Distended abdomen?Dilated veins?Hernial sites?Ascites?

Now look for the size of the spleen...

Systemic examination

Mild splenomegaly:•Infections•CCF•Portal hypertension(early stage)•Acquired Hemolytic anaemias

Moderate splenomegaly:•Malaria•Kala Azar•HIV•Portal hypertension

Massive splenomegaly:•Leukemia •Kala Azar•Chronic hemoytic anaemias•Storage disorders

If not significant..

MalariaKala azarPortal hypertensionChronic hemolytc anemias

Liver enlarged???

CBC including retic countPeripheral smearLFTsXraysUSG abdomen

What investigations to send?

Upper GI ScopyHb elctrophoresisBone Marrow examinationUrine analysis

Specific investigations...

The Mathematics of Splenomegaly...

fever pallor PS parasite

Kala azar Malaria

Chronic fever

Lymph nodes

Anemia/bleeding

ESR

PS picture

Joint pains

malignancy

hemolytic

rashesAutoimmune

disorders

Chronic fever

murmur

Wt loss/mt/ cxray

Lymph nodes

ESR

tuberculosis

RHD/IE

Hemetemesis/jaundice/

edema

Portal hypertension

Small liver

Altered LFTs

USG/UGI scopy

Coarse facies

Skeletonabnormal

Storage disorde

r

Neurologicdeficits

Ophthal screen

To take a detailed clinical history.. Always examine from head to toe.. Do not jump to the spleen directly

Even the baseline investigations can give so many clues about the final diagnosis

Narrow down to the group of disorders that you are dealing with.

REMEMBER..

Thank you!!!