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AA TRAITTRAIT TO ATO A TRAITORTRAITOR……
The enigma calledThe enigma called THALASSEMIATHALASSEMIA
-A STUDY TO EVALUATE THE ORO-FACIAL MANIFESTATIONS-A STUDY TO EVALUATE THE ORO-FACIAL MANIFESTATIONS
INDIAN DENTAL ACADEMYINDIAN DENTAL ACADEMY
Leader in continuing Dental EducationLeader in continuing Dental Education
www.indiandentalacademy.comwww.indiandentalacademy.com

What is thalassemia???What is thalassemia???
 Thalassemias are a heterogenous group ofThalassemias are a heterogenous group of
inherited disorders of hemoglobin synthesisinherited disorders of hemoglobin synthesis, of, of
one or more of the normal polypeptide chains;one or more of the normal polypeptide chains;
that leads to a decrease in or complete absencethat leads to a decrease in or complete absence
of the affected chainof the affected chain (chains).(chains).
 Thalassemias are defined according to the chainThalassemias are defined according to the chain
that is quanititatively reduced:that is quanititatively reduced:
αα thalassemia- deficiency ofthalassemia- deficiency of αα chainchain
ββ thalassemia- deficiency ofthalassemia- deficiency of ββ chainchain

www.indiandentalacademy.com
Indian Dental academy
• www.indiandentalacademy.com
• Leader continuing dental education
• Offer both online and offline dental
courses

What causes thalassemia?What causes thalassemia?

A study was conducted in the Dept. of Oral Medicine & Radiology,A study was conducted in the Dept. of Oral Medicine & Radiology,
Dr. D. Y. Patil Dental College & Hospital, Nerul, Navi MumbaiDr. D. Y. Patil Dental College & Hospital, Nerul, Navi Mumbai
AIM:AIM:
 To evaluate the oro- facialTo evaluate the oro- facial
manifestations in thalassemicmanifestations in thalassemic
patients.patients.
 To detect any correlation, if presentTo detect any correlation, if present
between the oro- facialbetween the oro- facial
manifestations and haematologicalmanifestations and haematological
picturepicture
 To find out if the oro- facialTo find out if the oro- facial
manifestations could aid in the earlymanifestations could aid in the early
diagnosis and if it could provide andiagnosis and if it could provide an
indication to the progress of theindication to the progress of the
disease.disease.

Materials and methods:Materials and methods:
 50 cases50 cases of thalassemia were takenof thalassemia were taken
from thefrom the Haematology DepartmentHaematology Department
of Lokmanya Tilak Medical Collegeof Lokmanya Tilak Medical College
&& Sion Hospital, MumbaiSion Hospital, Mumbai..
 Histories recorded:Histories recorded:
1.1. MedicalMedical
2.2. Past dentalPast dental
3.3. FamilyFamily
 Complete intraoral and extraoralComplete intraoral and extraoral
examinationexamination
 Systemic evaluation:Systemic evaluation:
1.1. Liver and spleen.Liver and spleen.
2.2. Cardiovascular system.Cardiovascular system.
 Complete haematologicalComplete haematological
investigationinvestigation
Thalassemia
minor, 28
Thalassemia
major, 72

Extra-oral examinationExtra-oral examination

Colour of skinColour of skin
50
31
43
21
0
5
10
15
20
25
30
35
40
45
50
Thalassemia major Thalassemia intermedia
Muddy yellow
Dark brown
•Deposition of excessive
iron in subcutaneous tissue
caused by the excessive
breakdown of abnormal
erythrocytes.
• Iron overloading due to
multiple blood transfusions

Bossing: Frontal and ParietalBossing: Frontal and Parietal
81
33
57
14
0
10
20
30
40
50
60
70
80
90
Frontal bossing
Parietal bossing
Bone marrow
hyperplasia leading
to enlargement of
the outline of the
flat bones of the
skull, including
frontal and parietal
bone

Hypertelorism and Depressed nasal bridgeHypertelorism and Depressed nasal bridge
72
70
78
76
66
68
70
72
74
76
78
Htpertelorism
Depressed nasal bridge
Relative to
depressed bridge
of the nose.
Relative to frontal bossing

Flaring of alaeFlaring of alae
39
29
0
5
10
15
20
25
30
35
40
Flaring of alae
• Air hunger caused by
chronic anemic
condition.
• Excessive exertion of
accessory respiratory
muscles to compensate
for reduced oxygen
•Accentuated by
depressed nasal bridge

Maxillary hyperplasia andMaxillary hyperplasia and
Incompetence of lipsIncompetence of lips
67
77
21
50
0
20
40
60
80
100
120
140
160
Maxillary hyperplasia
Incompetence of lips
and proclined anteriors
prevent complete
closure of lips
is due to overgrowth
of its marrow in
response to
underlying anemia

Intra-oral examinationIntra-oral examination

Gingiva: Pigmentation, gingivitis andGingiva: Pigmentation, gingivitis and
recessionrecession
69
28
36
21
0
10
20
30
40
50
60
70
Pigmentation
Gingivitis and/or recession
•Maxillary hyperplasia
leading to
incompetence of lips
and hence mouth
breathing habit
•Poor oral hygiene
•Iron deposition in the
gingiva giving it a
brownish black color
•Racial pigmentation

Tongue: Pallor, Depapillation,Tongue: Pallor, Depapillation,
Pigmentation.Pigmentation.
81
41
31
36
14
7
0
10
20
30
40
50
60
70
80
90
Tha la sse mia ma jor Tha la sse mia int e r me dia
Pallor
Pigmentation
Depapillation
•PALLOR - chronic
anemic status
•DEPAPILLATION -
vitamin B complex
deficiency
Iron overload

Pallor of oral mucosaPallor of oral mucosa
78
14
0
10
20
30
40
50
60
70
80
Pallor of mucosa
Chronic
anemic
status of the
patients

Proclination of teeth and SpacingProclination of teeth and Spacing
47
53
21
7
0
10
20
30
40
50
60
Proclination of teeth
Spacing
Proclination-
Erythroid hyperplasia of
the marrow
Spacing-
•Maxillary
hyperplasia
•Proclination of
teeth

Persistence of mammelonsPersistence of mammelons
33
0
0
5
10
15
20
25
30
35
Persistence of mammelons
Increased overjet
because the maxillary
anteriors are
proclined or in
labioversion,
preventing its normal
wear

Systemic changesSystemic changes
88
82
12
0
10
20
30
40
50
60
70
80
90
Hepatomegaly Splenomegaly Cardiovascular
changes
Thalassemic patients

Relation between oro-facialRelation between oro-facial
manifestations and blood picturemanifestations and blood picture
100
88
65
33
00
20
40
60
80
100
120
Hb 0-3 Hb 4-6 Hb 7-12 Hb 10-12 Hb 13-15
Oro-facial manifestations

SummarySummary
 Oro-facial manifestations are seen inOro-facial manifestations are seen in
86% subjects with thalassemia major86% subjects with thalassemia major
andand 78% subjects with thalassemia78% subjects with thalassemia
intermedia.intermedia.
 There is aThere is a definite relationshipdefinite relationship
between the blood picture and thebetween the blood picture and the
oro-facial featuresoro-facial features, thus showing a, thus showing a
predictive value for the prognosis ofpredictive value for the prognosis of
the disease.the disease.
 46% subjects’ parents gave a history46% subjects’ parents gave a history
ofof co-sanguinous marriagesco-sanguinous marriages betweenbetween
carriers.carriers.

Radiographic changesRadiographic changes

Factors to be considered by a dentistFactors to be considered by a dentist
 MassiveMassive risk of infectionrisk of infection,,
followed by bacteremia afterfollowed by bacteremia after
splenectomy.splenectomy.
 HaemoglobinHaemoglobin levels arelevels are lowlow..
 Paients are at increasedPaients are at increased risk ofrisk of
viral hepatitis and AIDSviral hepatitis and AIDS,,
precautions to be takenprecautions to be taken..
 Cardiomegaly present in someCardiomegaly present in some
patients, precaution to bepatients, precaution to be
taken during surgicaltaken during surgical
proceduresprocedures

Dental treatment planningDental treatment planning
 Short appointments.Short appointments.
 Diagnosis of pain in the teethDiagnosis of pain in the teeth
and jaws may be difficult.and jaws may be difficult.
 Teeth with infected pulpsTeeth with infected pulps
should be extractedshould be extracted, without, without
attempts at pulp treatment.attempts at pulp treatment.
 Extractions are best carriedExtractions are best carried
out at the time of admission forout at the time of admission for
blood transfusionblood transfusion..
 General anesthesia should beGeneral anesthesia should be
avoided if possible.avoided if possible.

Science has taught usScience has taught us
nothing…..nothing…..
The more we know…The more we know…
The more interesting theThe more interesting the
surrounding worldsurrounding world
becomes …..becomes …..
And more meaningful,And more meaningful,
the surroundingthe surrounding
darknessdarkness

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