ORTHOPAEDIC & TRAUMATOLOGY TEXTBOOK

HASANUDDIN UNIVERSITY MAY 2014

BLOUNTS DISEASE

By:
Sri Hardianti C111 08 202
Nor Anisah bt Abu Hanipah C111 08 780
Muhammad Fadzhil Bin Amran C 111 09 841
Izzad bin Azlan C111 08 793
ReynaldoMailoa C111 09 131
Aditya Wisnu Pratama C111 09 296

Advisors:
dr. Jecky C.
dr. Rico A.
dr. Sebastian M.
dr. M. Horeb

Department of Orthopaedic & Traumatology
Faculty of Medicine
Hasanuddin University
Makassar
2014

TEXTBOOK
I) DEFINITION
Infantile tibia vara, first described by Erlacher in 1922, is best known as Blount's
disease after the classic description by Blount in 1937. Blount characterized the
deformity as an abrupt angulation just below the proximal physis, an irregular physeal
line, and a wedge-shaped epiphysis with a beak at the medial metaphysis. Apparent
lateral subluxation of the proximal end of the tibia is often present.
II) ETIOLOGY
Several authors have reported a familial occurrence of the conditionand one
report of infantile tibia vara in a family suggested that the disease may be inherited as
an autosomal dominant condition with variable penetrance.However, as noted by
Langenskild and Riska,

because the radiographic features of infantile tibia vara have
never been seen in patients younger than 1 year and rarely in patients younger than 2
years, the condition is considered a developmental disorder and not a congenital one.
Other studies have found no evidence of an inherited condition and have concluded
that the etiology is multifactorial.
III) ANATOMY
The tibia and fibula are the bones of the leg. The tibia articulates with the condyles
of the femur superiorly and the talus inferiorly and in doing so transmits the body's
weight. The fibula mainly functions as an attachment for muscles, but it is also
important for the stability of the ankle joint. The shafts (bodies) of the tibia and fibula
are connected by a dense interosseous membrane composed of strong oblique fibers.




A) Tibia
Tibia is located on the anteromedial side of the leg, nearly parallel to the fibula, the
tibia is the second largest bone in the body. The proximal end widens to form medial
and lateral condyles and there is tibial plateau, which articulate with the lateral and
medial condyles of the femur and the lateral and medial menisci intervening.
Separating the upper articular surfaces of the tibial condyles are anterior and posterior
intercondylar areas lying between these areas is the intercondylar eminence.
(3,4)

The shaft of the tibia is triangular in cross section, presenting three borders and
three surfaces. Its anterior and medial borders, with the medial surface between them,
are subcutaneous. At the junction of the anterior border with the upper end of the tibia
is the tuberosity, which receives the attachment of the ligamentum patellae. The
anterior border becomes rounded below, where it becomes continuous with the medial
malleolus. The lateral or interosseous border gives attachment to the interosseous
membrane. The lower end of the tibia is slightly expanded and on its inferior aspect
shows a saddle-shaped articular surface for the talus. The lower end is prolonged
downward medially to form the medial malleolus.
(3,4)


B. Fibula
The fibula is the slender lateral bone of the leg. It takes no part in the
articulation at the knee joint, but below it forms the lateral malleolus of the ankle joint.
It takes no part in the transmission of body weight, but it provides attachment for
muscles. The fibula has an expanded upper end, a shaft, and a lower end. The upper
end, or head, is surmounted by a styloid process. It possesses an articular surface for
articulation with the lateral condyle of the tibia. The shaft of the fibula is long and
slender. Typically, it has four borders and four surfaces. The medial or interosseous
border gives attachment to the interosseous membrane. The lower end of the fibula
forms the triangular lateral malleolus, which is subcutaneous. On the medial surface of
the lateral malleolus is a triangular articular facet for articulation with the lateral
aspect of the talus. Below and behind the articular facet is a depression called the
malleolar fossa.
(3,4)



Picture 1: Anatomy of tibia and fibula.
(5)


The anterior compartment, or dorsiflexor (extensor) compartment, is located
anterior to the interosseous membrane, between the lateral surface of the tibial shaft
and the medial surface of the fibular shaft, and anterior to the intermuscular septum
that connects them. The anterior compartment is bounded anteriorly by the deep fascia
of the leg and skin. The deep fascia of the leg overlying the anterior compartment is
dense superiorly, providing part of the proximal attachment of the muscle immediately
deep to it. The four muscles in the anterior compartment are the tibialis anterior,
extensor digitorum longus, extensor hallucis longus, and fibularis tertius. These
muscles pass and insert anterior to the transversely oriented axis of the ankle joint and,
therefore, are dorsiflexors of the ankle joint, elevating the forefoot and depressing the
heel. The long extensors also pass along and attach to the dorsal aspect of the digits
and are thus extensors of the toes.
(4)

The lateral compartment is the smallest (narrowest) of the leg
compartments, bounded by the lateral surface of the fibula, the anterior and
posterior intermuscular septa, and the deep fascia of the leg. The lateral
compartment contains the fibularis (peroneus) longus and brevis muscles.
(4)


Picture 2: Muscles of the cruris.
(5)


The posterior compartment (plantar flexor compartment, is the largest of
the three leg compartments. The posterior compartment and the calf muscles
within it are divided into superficial and deep subcompartments/muscle groups
by the transverse intermuscular septum. The tibial nerve and posterior tibial and
fibular vessels supply both parts of the posterior compartment but run in the
deep subcompartment deep (anterior) to the transverse intermuscular septum.
Muscles of the posterior compartment produce plantarflexion at the ankle,
inversion at the subtalar and transverse tarsal joints and flexion of the toes. The
superficial group of calf muscles are the gastrocnemius, soleus, and plantaris.
Thus, the muscles of the posterior compartment of the leg are popliteus, flexor
digitorum longus, flexor hallucis longus, and tibialis posterior.
(4)


Picture 3: Muscles of the cruris.
(5)


IV) PATHOPHYSIOLOGY
There are three types of tibia varum based on the age it begins:
infantile (less than three years old),
juvenile (occurs between four and 10 years), and
adolescent (11 years of age and older).
Physiologic tibia varum occurs between the ages of 15 months to three years.
There's no need for treatment for this normal stage of development. But it's not always
clear at this age if the tibia varum is physiologic (normal variation)
or pathologic(Blount's disease).


The general findings have included (1) islands of densely packed cartilage cells
displaying greater hypertrophy than expected from their position in the growth plate,
(2) islands of nearly acellular fibrocartilage, and (3) exceptionally large clusters of
capillary vessels.
The physeal cell columns become irregular and disordered in arrangement and
normal endochondral ossification is disrupted, both in the medial aspect of the
metaphysis and in the corresponding part of the physis.
The varus deformity progresses as long as ossification is defective and growth
continues laterally. In later stages of the deformity, an actual bony bridge may tether
medial growth, and the medial tibial plateau may appear to be deficient
posteromedially.
However, actual depression of the posteromedial tibial articular surface is
probably not present, at least at the outset of the deformity. The deficiency is
probably unossified abnormal fibrocartilage whose delay in ossification produces the
appearance of a defect and is directly related to the underlying histopathology.
Ligamentous laxity on the lateral side of the knee frequently develops in a neglected
or recurrent deformity.
V) RADIOLOGIC FINDING
Radiography is central to establishing the diagnosis of infantile tibia vara. A
standing anteroposterior view of the lower extremities from hip to ankle should be
obtained. The diagnosis is based on familiar radiographic changes in the proximal end
of the tibia: (1) a sharp varus angulation in the metaphysis, (2) a widened and irregular
physeal line medially, (3) a medially sloped and irregularly ossified epiphysis, and (4)
prominent beaking of the medial metaphysis with lucent cartilage islands within the
beak.
Unequivocal radiographic changes diagnostic of infantile tibia vara are rarely
observed before 18 months of age (the youngest published case wasradiographically
diagnosed at 17 months of age).

However, a normal knee radiograph in a toddler does
not rule out infantile tibia vara. As an aid to early identification of toddlers who are at
risk for the development of infantile tibia vara but who have no physeal or
metaphyseal changes, Levine and Drennan measured the tibial metaphyseal
diaphyseal angle (MDA), the angle created by the intersection of a line connecting the
most prominent medial portion of the proximal tibial metaphysis (the beak) and the
most prominent lateral point of the metaphysis with a line drawn perpendicular to the
long axis of the tibial diaphysis .
VI) CLASSIFICATION
In 1952, Langenskild classified infantile tibia vara according to the degree of
metaphysealepiphyseal changes seen on radiographs, with six stages varying with
advancing age. General prognostic guidelines were also provided. Restoration to
normal was common in stages I and II disease and possible in stages III and IV
disease, whereas stages V and VI disease were associated with recurrence and
permanent sequelae after treatment by mechanical realignment (osteotomy).
Although Langenskild's classification was primarily intended to be a
radiographic description of infantile tibia vara, prognostic implications have gradually
been derived from later studie. In 1964, Langenskild and Riska reported that a simple
osteotomy could cure the deformity in patients 8 years old or younger.In the few cases
in which simple osteotomy failed, inadequate surgical correction was implicated.
Radiographic stage progression of the deformity was thought to be a consequence of
skeletal maturation rather than an indication of progressive inhibition of medial
physeal growth and worsening of the condition. The premise that 8 years is the critical
age up to which the condition is surgically curable has undoubtedly resulted in a
certain complacency in treating young children, particularly those with demonstrable
stage progression. A number of investigators have reported difficulty applying the
Langenskild classification to predict outcome in their own patients.

1. Infantile Blounts Disease
Abnormal tibia vara
More common and usually affects both extremities
Classic presentation is in a child who is overweight and who begins walking
before 1 year of age; disease is associated with internal tibial torsion.
2. Adolescent Blounts Disease
Less severe than infantile forms and more often unilateral
The epiphysis appears relatively normal and does not have the beaking seen in
infantile forms.
The most characteristic radiographic finding is widening of the proximal
medial physeal plate.

VI) CLINICAL MANIFESTATION
Genu varum (bowed legs) normal in children less than 2 years old. Blounts
disease (tibia varum) is best divided into two distinct entities: invantile (0-4 years of
age) and adolescent (over 10 years of age). Infantile Blounts disease: more common
and usually affects both of extremities. It occurs more often in the overweight child
who begins walking at less than 1 year of age and is associated with internal tibial
torsion. Adolescent Blounts disease: less severe and more often unilateral. The child
walks with an outward thrust of the knee; in the worst cases there may be lateral
subluxation of the tibia.


VII) TREATMENT
Untreated infantile tibia vara generally results in a nonresolving and sometimes
progressive varus deformity that produces joint deformity and growth retardation,
which can then be corrected only with complex surgical procedures. Even when such
surgery is performed, substantial articular disruption of both compartments of the knee
may have already occurred.Thus, once the radiographic diagnosis of infantile tibia
vara is certain, the orthopaedist should recommend treatment immediately because
patients treated in the early stages of the disease have a better prognosis. There is no
justification for simply observing a patient with an unequivocal diagnosis. Treatment
choices and prognosis depend greatly on the age of the patient at the time of diagnosis,
which should be the same age at which treatment is recommended.
Orthoses.
If the child is younger than 3 years of age and the lesion is no greater than
Langenskild stage II, orthotic treatment is recommended because 50% or more of
these patients can be successfully treated with braces, especially if they have only
unilateral involvement. There may be an inclination to brace patients before a true
Blount lesion is visible on radiographs, particularly when the MDA is suggestive of
varus progression. Thus, when evaluating the reported good outcomes from brace
treatment, one must realize that some patients probably had physiologic genu varum
rather than true infantile tibia vara. Nevertheless, orthotic treatment appears to affect
the natural history favorably The type of orthosis prescribed and the length of time
that the orthosis is worn during a 24-hour period vary. Raney and associates used a
knee-ankle-foot orthosis (KAFO) that produced a valgus force by three-point pressure
in 60 tibiae (38 patients), with lesions in 54 tibiae (90%) resolving without surgery.
Significant risks for failure included ligamentous instability, patient weight above the
90th percentile, and late initiation of bracing. Of the 54 tibial lesions that resolved, 27
were treated by full-time orthotic use, 23 by nighttime use only, and 4 by daytime use.
Three of the six tibiae requiring surgery had been treated with full-time orthotic use
and three with nighttime use only. Based on these findings, the authors conjectured
that nighttime-only bracing might be as efficacious as full-time bracing, although they
acknowledged that inherently one would expect daytime use (i.e., during weight
bearing) to be the most important factor in successful orthotic treatment. On the other
hand, Zionts and Shean reported daytime ambulatory bracing to be successful in
altering the natural history of tibia vara in patients younger than 3 years with
Langenskild stage I or II disease.
We have used conventional KAFOs, conventional hip-knee-ankle-foot orthoses
(HKAFOs), and elastic KAFOs in the treatment of infantile Blount's disease. Since
1987, the elastic Blount brace, a medial upright design that uses a wide elastic band
just distal to the knee joint, has been used almost exclusively because of its ease of
fabrication and smaller profile. With this orthosis, 65% of tibiae had successful
outcomes at an average follow-up of 5.9 years. However, corrective osteotomies for
one or both extremities were eventually required in 70% of patients with bilateral
involvement, as opposed to only 6% of patients with unilateral involvement. All of the
patients were instructed to use the brace during the day (i.e., during weight bearing).
Depending on the patient's physician, some patients were encouraged to use the brace
for 20 to 24 hours per day.
Treatment of Langenskild Stage II Lesions.
Surgical treatment in the early stages of the disease (stage II) is crucial to
achieve permanent and lasting correction and to avoid the sequelae of joint
incongruity, limb shortening, and persistent angulation. Patients with stage I or II
disease have a significantly lower incidence of repeat osteotomy than do those with
stage III disease.Surgical overcorrection of the mechanical axis to at least 5 degrees of
valgus by 4 years of age, along with lateral translation of the distal osteotomy
fragment, is believed to be optimal. Such overcorrection ensures that the supine
correction attained at surgery will be sufficient to translate the mechanical axis into the
lateral compartment of the knee once the patient begins bearing weight.
Overcorrection of the mechanical axis offsets the tendency of the knee to go back into
varus as a result of any sloping of the medial epiphyseal surface and relaxation of the
lateral ligaments.
Although Schoenecker and colleagues reported that correction to within 5
degrees of neutral alignment would prove adequate, most authors recommend
physiologic valgus or overcorrection. Based on the physeal inhibition phenomenon
proposed by Cook and associates, overcorrection to absolute valgus alignment is
required to reverse the excessive compressive forces medially and allow a
Langenskild II or III physis not already irreversibly damaged to respond to such
mechanical unloading.
Treatment of Langenskild Stage III Lesions.
Stage III lesions can respond to corrective osteotomy alone in patients older than 4
years. However, the longer the delay in surgery after 4 years of age, the greater the
risk for recurrence, which is not uncommon with stage III lesions. Thus, because of
the worsening prognosis, neither observation nor orthotic treatment is recommended
beyond this age, especially if the deformity exceeds 10 degrees of femorotibial varus.
Treatment of Langenskild Stages IV/V Lesions.
Lesions greater than stage III cannot be definitively corrected by simple mechanical
realignment because physiologic physeal arrest has already occurred by stage IV.Even
though no bony bridge can be visualized by tomographic methods in stage IV or V
lesions, physeal damage has progressed to the point where stages IV and V lesions
effectively act as medial physeal arrests.
Treatment of Langenskild Stage VI Lesions.
Treatment of stage VI lesions with established bony bridges must also be
individualized. Factors to be considered are patient age, the amount of skeletal growth
remaining, and the degree of deformity of the joint surface. If the patient has less than
2 years of growth remaining and a relatively normal joint surface, corrective
osteotomy with complete physeal closure is a practical means of obtaining and
maintaining correction. The osteotomy can performed through the physis so that the
mechanical correction is placed as close to the joint as possible and permanent physeal
closure occurs.
As previously mentioned, resection of the bony bridge with placement of
interposition material is appropriate in patients younger than 7 years. Unfortunately, a
patient with a stage VI lesion will probably be older than this age limit, when
epiphysiolysis is less predictable. Treatment options in patients with more than 2
years' growth remaining include completion of the lateral tibial epiphysiodesis,
angular correction, and lengthening, if indicated, usually during the same treatment
session.In patients requiring limb length equalization with or without correction of
deformity, correction by external fixation and distraction osteogenesis is an effective
and invaluable method for salvaging a potentially unsatisfactory extremity. Breaking
of a physeal bridge by asymmetric physeal distraction has been described as an
alternative approach to resection of the bony bridge in children near skeletal maturity.

References :
- Solomon, L, Warwick D.L, Nayagam,S. Apleys system of orthopaedics and
fractures. 9
th
editions. 2010.
- Miller M. D, Review of Orthopaedics . 5th edition. 2008.