2. CONGENITAL DISORDERS
Blount’s Disease
• Osteochondrodysplasia
• Hereditary multiple
exostosis
• Achondroplasia
• Hypochondroplasia
BLOUNT DISEASE is the most common found in
congenital disordes and the management still controversial
Dyschondrosteosis
Dysostosis
Dyschondroplasia
Maffucci’s syndrome
3. Blount’s disease
Introduction
• Also known as TIBIA VARA
• In 1937, Blount’s (Walter Putnam Blount (1900–1992), an
American pediatric orthopedic surgeon) article prompted the
recognition of this disorder
Blount Disease Author: Matthew J DeOrio,;
American Medical Association and Florida Medical Association 2010
4. • In 1952 : study expanded by Langenskiöld. He described a
progression of radiographic changes seen with the disease in the
Scandinavian population.
• Characterised Disordered endochondral ossification of the
medial part of the proximal tibial physis, resulting in
multiplanar deformities of the lower limb.
5. • Rarely happens defect at the lateral ephyfisis causing tibia vara
• Clinical features :
• Assymetrical growth
• Varus
• Procurvatum (apex anterior)
• Internal rotation with limb shortening in unilateral cases
Blount Disease Author: Matthew J DeOrio,;
American Medical Association and Florida Medical Association 2010
6. Types of Blount Disease
Early Onset
Infantile
i. onset <3 years
ii. occasional
resolution
iii. Bilateral
iv. usually
symmetric
Late Onset
(Thompson&Carter
Classification)
Juvenile
i. onset between
4-10 years
ii. Unilateral
iii.associated with
a physeal bridge
Adolescent
i. onset over 10
years
ii. same
as juvenile
Blount Disease Author: Matthew J DeOrio,;
American Medical Association and Florida Medical Association 2010
7. • < 1 % of all children born.
• Boys : Girls = equally.
• Adolescent : 2.5% in the population at
greatest risk.
• Frequency : other family members with
Blount disease
Blount Disease Author: Matthew J DeOrio,;
American Medical Association and Florida Medical Association 2010
8. RISK FACTORS
• Obesity
• Afro-american
lineage
• Walking at an
early age
• Family history,
9-43% have an
affected family
member
ETIOLOGIES
• Biomechanical
overload of the
proximal tibial
physis
• Inheritance
(limited)
• Early
Ambulation
• Developmental
factors
Blount Disease Author: Matthew J DeOrio,;
American Medical Association and Florida Medical Association 2010
9.
10.
11. Excessive compressive
forces on the proximal
medial metaphysis of the
tibia
Deformity caused
by intrinsic
alteration of bone
formation
Disruption in
normal
endochondral bone
formation
Damaged cartilage
ossifies more slowly.
Histologic sections of
cartilage in the
infantile form show
damaged cartilage
12. Infantile
Child start to walk early.
Painless.
Marked obesity
Bilateral : 80% of cases.
The metaphyseal : palpable
over the medial aspect of the
proximal tibial condyle.
Adolescents
Pain at the medial aspect of
the knee.
Overweight or obese.
Unilateral: 80% of cases
The involved leg sometimes is
shorter than the opposite leg
by as much as 2-3 cm.
13.
14. • Stage I : medial metaphyseal beaking.
• Stage II: sharp antero-medial depression.
• Stage III: deepening of the depresssion.
• Stage IV: developed step is filled in by the epiphysis and
growth plate is irreversibly.
• Stage V: A triangular fragment separates
• Stage VI: medial growth plate ossification.
Growth continues in the normal lateral part.
16. • Sharp Varus anglulation
at metaphysis
• Widened and irregular physeal line
medially
• Medially sloped and irregularly
ossified epiphysis
• Prominent Beaking of the medial
metaphysis
Jugesh Cheema-Blount Disease imaging
17. Adolescent Blount disease.
Coronal T1-weighted MRIs of
the left knee in an 11-year-old
boy show Blount disease
affecting the entire tibial
growth plate and the lateral
part of the distal femoral plate.
Signal intensity changes in the
marrow of the metaphysis and
epiphyseal flattening are
evident in the medial portion
of the tibia; this is the classic
depiction.
Jugesh Cheema-Blount Disease imaging
19. • Treatment depend on the age of the child and the severity
of the varus deformity.
• Operative treatment is not recommended for children
younger than 2 years.
• Orthotic treatment can be used when the deformity is
increasing.
• If the deformity persist or increases to stage III or
IV,osteotomy is required before the child is aged 4 years.
• Surgery is usually the course of action for patients > than
3 years old.
DeOrio MJ,Blount Disease Treatment&Management 2010.
20. Age 2-3 years
Metaphyseal / diaphyseal angle > 11o
Tibial / femoral angle > 15o
Metaphyseal / epiphyseal angle > 25-30o
KAFO is designed to provide rotational support,
23 hrs/day
50% progress
Takes 1 year to determine effectiveness
Ineffective in adolescents
22. • Hip-knee-ankle-foot orthosis (HKAFO)
• Knee-ankle-foot orthosis (KAFO).
• Bracing is used 23 h/day.
• The orthotic is changed every 2 months
correct the bowlegged position.
http://www.orthopediatrics.com orthopediatrics Corp.
23. Conventional Orthotic Design
• Consists of a medial bar
• Thigh Cuff
• Foot plate
• Locked knee
• Medially directed force strap at the
knee joint (pulls the knee towards the
medial bar)
HKAFO / KAFO
http://www.orthopediatrics.com orthopediatrics Corp.
24. INFANTILE
• method
• tibial osteotomy
• tibia rotated
outward and
angled laterally to
give 6-10o of
valgus
• osteotomy of
fibula through
separate incision
• fixation with
external fixator or
internal fixation
or cast
ADOLESCENT
• method
• tibial osteotomy
• tibia rotated outward and
angled laterally to give 6-
10o of valgus
• osteotomy of fibula
through separate incision
• fixation with external
fixator or internal fixation
or cast
• +/- epiphysiodesis based
on age and leg length
discrepancy
absolute indications
– depression of the
tibial plateau
– impending closure
of the medial physis
of proximal tibia
– ligamentous laxity
of the knee
relative indications
– metaphyseal/diaphy
seal angle > 30o
– tibial/femoral angle
> 15o
INDICATIONS
25. 1) Wedge-shaped bone is removed from the medial side of the femur.
2) It's then inserted into the tibia to replace the broken bone.
3) Pins and screws used to hold everything in place.
http://www.orthopediatrics.com orthopediatrics Corp.
26. • A special circular wire frame on the outside of the leg with pins to
hold the device in place.
• Called a distraction osteogenesis..
http://www.orthopediatrics.com orthopediatrics Corp.
27. CANNULATED SCREW OPERATIVE BRIEF
1.Insert 1.6 mm pin 2. Apply contoured eight-
plate over pin.
3. Insert the epiphyseal
guide wire, followed by the
metaphyseal guide
wirecenter guide wire and
check position with
floroscope.
4.Drill the
epiphyseal hole,
followed by the
metaphyseal
hole.
5. Remove the
guide wires and
turn each screw
one to two more
times. Note the
position of the
screw proximal
and distal to the
physis
31. • Osteotomy + internal fixation: penyembuhan dalam 8 mingg.
• The cast: dilepas 5-6 minggu pasca operasi
• External fixators dan gangguan osteogenesis - gradual angular
manipulation is begun 1 minggu setelah external fiksasi dan
osteotomy. Correction : subsequent 2-3 weeks,
• The frames : removed 12 weeks postoperative
http://www.orthopediatrics.com orthopediatrics Corp.
32. • Kegagalan pengobatan
didefinisikan sebagai
kelainan varus residu yang
memerlukan osteotomi atau
keselarasan varus moderat
atau berat (sumbu mekanis
yang melewati zona -3 atau -
4, dengan penyimpangan
sumbu mekanik> 40 mm
medial ke pusat sendi lutut)
pada saat itu. Dari tindak
lanjut terakhir Schematic diagram showing the
zones of the mechanical axis.
L = lateral, and M = medial
SabharwalS-Blount disease-The journal of Bone and Joint Surgery
33. PROGNOSIS
• Bergantung pada usia pasien dan tingkat keparahan
deformitas pada saat intervensi
• Infeksi tibia vara memiliki prognosis yang baik dan
kekambuhan deformitas rendah bila diobati pada usia
muda dan stadium dini
• Namun, pasien yang lebih tua dengan kelainan bentuk
lanjut (yaitu, stadium IV-VI) berisiko mengalami
kekambuhan
34. KESIMPULAN
• Penyakit Blount adalah pertumbuhan yang jarang terjadi
yang ditandai dengan adanya osifikasi gangguan pada
aspek medial, jarang pada aspek lateral, pada tibialis
proksimal. Jenis Blount's: infantil, juvenil dan adolescen
• Pembedahan biasanya dilakukan untuk pasien lebih dari
3 tahun
Radiographic indices used in the evaluation of lower-extremity bowing in infants and young children. The mechanical tibiofemoral angle (A) is the angle between a line drawn from the center of the hip to the center of the knee and a line drawn from the center of the knee to the center of the ankle. The tibial metaphyseal-diaphyseal angle (B) is the angle between a line drawn through the most distal aspects of the medial and lateral beaks of the proximal tibial metaphysis and a line perpendicular to a line drawn along the lateral aspect of the tibial diaphysis. The femoral metaphyseal-diaphyseal angle (C) is created by a line drawn perpendicular to the anatomic axis of the femur and a line drawn parallel to the distal femoral physis. The epiphyseal-metaphyseal angle (D) is created by a line drawn through the proximal tibial physis, parallel to the base of the epiphyseal ossification center, and a line connecting the midpoint of the base of the epiphyseal ossification center with the most distal point on the medial beak of the proximal tibial metaphysis. The percentage deformity of the tibia, % DT (E), is calculated as the degree of tibial varus (the medial angle between the mechanical axis of the tibia and a line drawn parallel to the distal femoral condyles) divided by the total amount of limb varus (femoral varus [FV] + tibial varus [TV]). Femoral varus is represented by the medial angle between the mechanical axis of the femur and a line parallel to the distal femoral condyles.