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Learning Task Day 2 Congenital Bone Disorder

SGDB9

Scenario I

A lecturer showed one of his medical student, a 4 months old female patient with

abnormalities in her left lower limb, shortening in her thigh, adducted and difference in skin fold

between her right and left thighs. Lecturer said this patient maybe having DDH (Development

Dysplasia of the Hip).

1. Where is the abnormality occured and why is it called DDH?

It occured in the hip joint, because there is a deformation on the hip joint.

2. What are the incidence, etiology and pathogenesis of DDH?

The incidence is 1 in 1000 newborn baby, hip dysplasia is considered to be amultifactorial

condition. Cause is unknown but common in breech position or large fetal size.

Congenital : Some studies suggest a hormonal link. Specifically the hormonerelaxin

has been indicated. A genetic factor is indicated by the trait running in families and

increased occurrence in some ethnic populations. A locus has been described on

chromosome 13.

Acquired : As an acquired condition it has often been linked to traditions of

swaddling infants or use of acradle board which locks the hip joint in an "adducted" position

(pulling the knees together tends to pull the heads of the femur bone out of the sockets or

acetabulae) for extended periods.

Further risk factors includebreech birth and firstborns. In breech position the femoral head

tends to get pushed out of the socket. A narrow uterus also facilitates hip joint dislocation

during fetal development and birth.

3.

How you diagnose and treat DDH patient based on age of the patient?

Diagnostic test for this patient (< 6 months old) :

Ultrasound (sonography): The preferred diagnostic tool in babies under 6 months of age,

ultrasound can detect a dysplastic hip (incorrect development of the socket) or a dislocated

hip (the femur is out of the socket)

Treatment for this patient (< 6 months old) :

The baby's thighbone is repositioned in the socket using a harness or similar device. The

method is usually successful. But if it is not, the doctor may have to anesthetize the baby

and move the thighbone into proper position, and then put the baby into a body cast (spica).

Scenario II

A normal male baby was born maternity clinic, with normal body weight, cried spontaneous

after examined by midwife. She found both of the babys legs bowed inwards. She has a daughter

studying in medical faculty. She asked her daughter about the abnormalities in this baby and how to

treat this baby appropriately.

1. What are the congenital abnormalities found lower limb?

Development Dysplasia of the Hip(DDH), Proximus Femur Focal Deviciency(PFFD), HabitualDislocation of Patella, Congenital Talipes Equinovarus (CTEV) or clubfoot.

http://en.wikipedia.org/wiki/Multifactorial_inheritancehttp://en.wikipedia.org/wiki/Relaxinhttp://en.wikipedia.org/wiki/Chromosome_13http://en.wikipedia.org/wiki/Swaddlinghttp://en.wikipedia.org/wiki/Cradle_boardhttp://en.wikipedia.org/wiki/Breech_birthhttp://en.wikipedia.org/wiki/Breech_birthhttp://en.wikipedia.org/wiki/Cradle_boardhttp://en.wikipedia.org/wiki/Swaddlinghttp://en.wikipedia.org/wiki/Chromosome_13http://en.wikipedia.org/wiki/Relaxinhttp://en.wikipedia.org/wiki/Multifactorial_inheritance

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Learning Task Day 2 Congenital Bone Disorder

SGDB9

2. What are the causes of bowed legs?

Bowed legs can be caused by deficiency of Vitamin D, genetical factor such as CTEV in

autosomal chromosome 18, growth abnormality such as abnormality of the growth plate at

the top of the shin bone (tibia) at the knee.

3. In this case what are deformities found in leg and ankle?

In this case, deformities leads to CTEV. CTEV deformities consisit of 4 elements :

equinus position of the heel : lateral talocalcaneal angle < 35o

varus position of the hindfoot : AP talocalcaneal angle < 20o

metatarsus adductus : adduction and varus deformity of the forefoot

talonavicular subluxation : medial subluxation of the navicular bone

4. How do you manage this baby and when do you start the treatment?

Management Current management of congenital talipes equinovarus is moving away from surgery

towards conservative treatment using Ponseti's regime of casting and manipulation.

Management will depend on the degree of rigidity, associated abnormalities and

secondary muscular changes.

Serial plaster cast is the main form of nonsurgical treatment with gentle manipulation of

the foot towards the corrected position before the cast is applied and changed every 1-2

weeks.

If clinical and X-ray correction are achieved by 3 months of age, then holding casts are

used for a further 3-6 months with orthoses/corrective shoes until the patient is walking

well.

Surgery If, despite conservative management, the hind foot remains in an equinus position, then

an operation is required to release the soft tissue responsible for shortening, e.g. release

of the tibialis posterior, abductor hallucis and Achilles tendons.

Complete soft-tissue release performed between 6 and 12 months achieves satisfactory

results in 80-90% of cases. A recent study found that results of surgery were better if

performed in the second, rather than the first, 6 months of life.7

The most common residual abnormality is dynamic pes varus and this is corrected with

centralisation of the tibialis anterior tendon.

Further corrective surgery may be required later in childhood. This may include wedge

excision of the calcaneocuboid bone, fusion of the midtarsal and subtalar joints, or calcaneal

osteotomy and talectomy.

Scenario III

A newborn baby found born with both of his legs bowed inwards.

1.

What are the congenital abnormalities found in this baby?

The congenital abnormalities found in this baby is congenital talipes equinovarus (CTEV).

2.

What are the radiologic examinations needed to be done for this patient?

We can use ultrasound to diagnose this patient.

http://www.patient.co.uk/doctor/Club-Foot.htm#ref7http://www.patient.co.uk/doctor/Club-Foot.htm#ref7http://www.patient.co.uk/doctor/Club-Foot.htm#ref7http://www.patient.co.uk/doctor/Club-Foot.htm#ref7

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Learning Task Day 2 Congenital Bone Disorder

SGDB9

3. What are the radiologic imaging are expected in this patient?

We expected that we could find the four elements of abnormality in CTEV to make the

diagnosis.

Learning Task

1. Definition of congenital abnormalities in musculoskeletal system.

Congenital abnormalities may be defined as: defects in the development of body form or

function that are present at the time of birth. (Salter 1999)

2.

Congenital abnormalities that common found.

Osteogenesis Imperfectia

3.

Signs and diagnostic criteria.

Ortolani sign, barlow provocation test, galeazzi sign, push pull manouver, trendelenburg

sign, orthopaedic check list.

4.

Management procedures.

gentle reduction, maintenance the flexion and abduction hip on Palvlik harness for about 4

months. Continous traction, adductor tenotomy, hip spica, Palliative and salvage operative

procedure (Salter Osteotomy) for 5 years above.

5. Consultation patient with congenital abnormalities.

Congenital abnormalities of musculo skeletal system need to be diagnosed early, for fast and

precise handling. Genetic Councelling necessary characteristic of hereditary disorders.