CONGENITAL TIBIA

HEMIMELIA
PRESENTATION LAYOUT

 Definitions.
 Inroduction.
 Cause.
 Classification.
 Clinical presentation.
 Treatment.
DEFINITION OF TERMS
 Congenital: Something, which is acquired from birth.

 Tibia: The shin bone, which is located below the knee.

 Hemimelia: The congenital absence of part of one or more limbs.

 TH: Tibia Hemimelia.

INTRODUCTION
 Tibial hemimelia is a condition in which
child is born with a tibia (shin bone) that
is shorter than normal or complete tibia
absent.
 A rare birth defect that causes a baby to
have a partially or completely missing
tibia.
 The incidence of tibial hemimelia has been estimated to
be 1 in 1 million live births, making it one of the rarest
congenital lower-limb deformities seen. It can be
unilateral or bilateral, (70% of unilateral cases and 30%
of cases reported bilateral).

 For some unknown reasons, Tibia Hemimelia seems to

favor the right side.

 Tibial hemimelia may also be present alongside

additional congenital deformities, such as congenital
femoral deficiency.
 A bifid femur (the top end of the thigh bone is split into two.
 Club foot(foot is turned inward).
 Absent of toes or too many toes.
 Affected individuals can also have issues with
the knee, such as missing ligaments that
supports the knee and thus, trouble straightening
the knee.

 Furthermore, the progression of deficiency of

the tibia in TH is from distal to proximal (ankle
to knee).
CAUSE

 The cause of Tibia Hemimelia is uncertain/

unknown, though some literatures trace the
cause to be genetic. However, it is still not
certain and has not been widely accepted
that the exact cause of Tibia hemimelia is
genetic.
CLASSIFICATION
There are many classifications of Tibia Hemimelia.
However, Jones classification is more widely accepted;

Type i: absent tibia; it’s sub categorized into:

 Type ia: with absent tibia and under development lower femoral

epiphysis.

 Type ib: with absent tibia but normal lower femoral epiphysis.

 Type ii: in which the tibia is distally deficient and well developed
proximally.

 Type iii: in which the tibia is proximally deficient and well ossified
distally.

 Type iv: characterized by shortening of the distal tibia, with distal

tibiofibular diastasis and normally developed proximal tibia.
CLINICAL
PRESENTATION
Tibial hemimelia presents as a shortened leg with knee and ankle deformities
bowed to variable degree. Varus in the ankle and knee flexion contracture in
the knee can occur.

 There is a deficiency of collateral ligaments as well and this leads to

instability of the joints due to lack of collateral ligaments.

 The patellar bone may be absent and so could be quadriceps muscle. When
present, their function is reduced.
 Thetibia is absent or under developed, but if present may
be palpated below the femur.

 Thefibula is intact and may be palpable as a distinct

bony prominence near the knee.

 Thefoot is in stiff equinovarus (congenital club foot)

especially of the hindfoot. Often, foot deformities such
as duplication of rays, shortening of the first metatarsal,
and loss of medial rays with multiplication of lateral rays
may be seen. In very mild deformities duplication of the
great toe may be the only clinical sign of tibial
hemimelia.
TEST.
 X-rays,from hips down to the feet to determine which bones
are present and which are missing soon as your child is
born.
 Magnetic resonance imaging(MRI) scans, to learn more
about your child’s knee and ankle joints.
TREATMENT/MANAGEMENT

 Non surgical treatment.

• Wearing a shoe lift.If the child’s foot can fit in a shoe ,a
shoe lift can help to even out a smaller difference in leg
length.
• Prosthetics.For a larger difference in leg length,an
artificial device can be fitted over the shorter limb to
allow the child’s foot to be flat on the floor.
 Surgical treatment.
• Limb reconstruction and lengthening,involves one or more
surgeries to repair the bones, muscles and joints that are
affected by hemimelia.This is followed by gradual
lengthening of the leg using an external fixator which is
worn until the lengthened bone is strong enough to support
the patient.
• Limb amputation,The best chance for a child to have an
active life often involves amputation of the affected
limb.After the limb is amputated,the prosthetist will fit
the child for a prosthetic limb.
THANK YOU