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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_inheritance8/11/2019 LT day 2 musculo eV
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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#ref78/11/2019 LT day 2 musculo eV
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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.
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