Lecture Slides Presented at SIMS by Medical Unit SIMS Hospital

1. A diagnostic approach to
splenomegaly
Dr Nighat Majeed
Assistant Professor
Medical Unit II
SIMS/SHL Lahore.

3. Anatomy
• It lies within the left upper quadrant of the
peritoneal cavity.
• Abuts ribs 9-12, the stomach, the left kidney, the
splenic flexure of the colon, and the tail of the
pancreas.

4. Anatomy

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

6. Functions
• Immunosurveillance.
• Hematopoiesis.
• Clearance of microorganisms and particulate
antigens from the blood stream.
• Synthesis of immunoglobulin G (IgG), properdin.
Tuftsin.
• Removal of abnormal red blood cells.
• Embryonic hematopoiesis in certain diseases.

7. 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.

8. Splenomegaly
Splenomegaly definition by weight
• MILD; Spleens weighing 400-500 g.
Moderate; Spleen weighing 750-1000.
Massive; More than 1000 g to indicate massive
splenomegaly.

9. Symptoms and signs
• Abdominal pain/tiredness.
• Cold/flu/Sore throat.
• Early satiety due to splenic encroachment.
• Symptoms of anemia due to accompanying
cytopenia.
• Febrile illness (infectious).
• Pallor, dyspnea, bruising, and/or petechiae
(hemolytic process).

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

11. Symptoms and signs
• Palpable left upper quadrant abdominal
mass.
• Splenic rub.
• Lymphadenopathy.

12. 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).

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

14. 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.

15. Palpation of spleen
Palpation
• Right Lateral Decubitus.
Two-Handed.

16. Palpation of spleen
Supine
Hooking Maneuver of Middleton”
• Patient’s Fist under L CVA.
• Stand on left facing patient’s feet.
• Hook fingers over costal margin.
• A mass with notch in left
upper quadrant indicate
splenomegaly

17. 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.

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

20. Examination of the Spleen
Traube’s Space
• Supine position.
• 6th rib.
• Costal margin.
• Midaxillary line.
• Normal breathing.
• Splenomegaly = dullness

21. Percussion
Nixon’s Method
• Right Lateral.
• Decubitus.
• =8cm.
•

22. Percussion
• Castell’s Method
• Supine p
• Lowest intercostal
space
• Left anterior axillary
line
• Full inspiration and
• expiration
• Splenomegaly =
dullness

23. Palpation 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.

24. Principal causes of Splenomegaly
• Infection
1. Viral
2. Bacterial
3. Fungal
4. Rickettsial
5. Parasitic
• Hemolytic anemia
• Cardiac failure
• Trauma
• Neoplasia
• Portal hypertension
• Metabolic disorders
• Other

25. Principal causes of
Splenomegaly
Infection
• Bacterial (septicemia,endocarditic, cat scratch
disease, tularemia).
• Brucellosis.
• Tuberculosis.
• splenic abscess.
• Leptospirosis.
• Lyme disease.
• Syphilis.

26. Principal causes of
Splenomegaly
Infection
• In neonates,septicemia is most common.
Usual pathogens are group B Streptococcus and E.
coli.
• Enlarged spleen in infants, children, and
adolescents is due to acute viral
infection,especially with Epstein-Barr virus or
cytomegalovirus.

27. Principal causes of
Splenomegaly
Infection
• Fungal(histoplasmosis, candidiasis).
• Rickettsial (Rocky Mountain spotted fever).
• Parasitic (malaria, toxoplasmosis, visceral larva
migrans,schistosomiasis).
• Kala-azar.
• Viral hepatitis.

28. Principal causes of
Splenomegaly
Haematological
• Haemolytic anaemias (eg Thalassaemia, red cell
defects, Sickle cell anaemia).
• Acute leukaemias, chronic leukaemias.
• Polycythaemia rubra vera.
• Macroglobulinaemia.
• Lymphoma (Hodgkin's disease and non-Hodgkin's
lymphoma).
• Essential thrombocythaemia.
• Myelofibrosis.

29. Principal causes of
Splenomegaly
Congestive splenomegaly
• Liver cirrhosis.
• Budd Chiari syndrome.
• Portal or splenic vein obstruction.
• Heart failure.

30. Principal causes of
Splenomegaly
Connective tissue disorders
• Systemic lupus erythematosus.
• Felty's syndrome.
• Connective tissue diseases (juvenile rheumatoid
arthritis, systemic vacuities).

31. Principal causes of
Splenomegaly
Neoplasia
Benign splenic tumors
• Hemangioma.
• Lymphangioma.
• Hamartoma.
Malignancies
• Acute lymphoblastic leukemia
• Acute myeloid leukemia.
• Hodgkin disease.
• Non-Hodgkin lymphoma.

32. Principal causes of
Splenomegaly
Portal Hypertension
• Any cause of portal hypertension may cause
enlarged spleen.
• Major causes are liver disease (cirrhosis, hepatitis,
extra hepatic biliary atresia);cavernous
transformation of portal vessels; and portal or
splenic vein thrombosis.

33. Principal causes of
Splenomegaly
Metabolic Disorders
• Amino acid disorders (tyrosinemia)
• Carbohydrate disorders
(galactosemia,hereditary fructose intolerance)
• Mucopolysaccharidoses (Hurler and Hunter
syndromes)
• Lipidoses (Gaucher disease, Niemann-
Pickdisease, GM-1 gangliosidosis type I)
• Glycoprotein disorders (sialidosis type II,
fucosidosis).

34. Principal causes of
Splenomegaly
Metabolic Disorders
• Histiocytosis X.
• Amyloidosis.

35. Principal causes of
Splenomegaly
Other
• Splenic cysts/Haemangiomas.
• Inflammatory bowel disease.
• Sarcoidosis.
• Histiocytoses.
• Drug hypersensitivity reactions.

36. Causes of massive
splenomegaly
• Chronic myeloid leukaemia.
• Myelofibrosis, malaria (hyper-reactive
malarial splenomegaly).
• Leishmaniasis.
• 'Tropical splenomegaly' (idiopathic; Africa,
SE Asia).
• Gaucher's syndrome.

37. 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.

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

39. 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.

40. 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.

41. 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.

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

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

44. 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

45. 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

46. 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.

47. Laboratory tests
• A CT scan of the abdomen and radio nuclide scan
for liver and spleen size and ratio should be done.
• The assistance of a hematologist or infectious
disease expert should be sought.
• A surgeon may need to be consulted for an
exploratory laparotomy.

48. Imaging Studies
• Craniocaudal measurement: A craniocaudal
measurement of 11-13 cm is frequently used as the
upper limit of normal for splenic size in imaging
studies.
• Computed tomography (CT) scanning

49. 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.

50. 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

51. Procedures
• Splenectomy
• Splenic biopsy
• A liver biopsy,
• splenic aspiration and biopsy.
• Bone marrow biopsy may all be helpful in
diagnosing the reticuloendothelioses such as
Gaucher's disease.

52. S P L E N O M E G A L Y
A U T O IM M U N E D IS O R D E R ? A M Y L O ID O S IS
C C F /S A R C O ID O S IS
P O R T A L H Y P E R T E N S IO N
R B C fin d in g o f h a e m o ly tic a n e m ia s
le u c o c y to s is
T h ro b o c y to s is
P a n c y to p e n ia s
A b n o rm a l
A b s e n t
E B V
T o x o p la s m o s is
g a u c h e rs d is e a s e
A p p ro a c h to ly m p h a d e n o p a th y
if p re s e n t
ly m p h a d e n o p a th y
N o r m a l
p e rip h ra l b lo o d film
T y p e title h e re
H IS T O R Y A N D
P H Y S IC A L E X A M IN A T IO N
M a s s iv e s p le n o m e g a ly
b lo o d film
B o n e m a rro w

53. S P L E N O M E G A LY
O bserve
M ild asym ptom atic
lapro tom y
N egative
B one m arrow biopsy
C T abdom e n
E xplora tory lap rotom y
N egative
Liver biopsy
H epato m egaly w ith ab norm a l liver fu nction s
M oderate to m arke d
lym ph adeno pathy absen t nam e here
serolog ical stu dies n egative