anupa mishra
TRANSCRIPT
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!!!