DEVELOPMENTAL

DYSPLASIA OF THE HIP

(DDH)

Audi Hidayatullah S
Definition

 DDH is a spectrum of disorders of development of

the hip that present in different forms at different
age
Spectrum instability
 Subluxatable
 Dislocated
 Subluxatable (reducible)
 Dislocatable (reducible)
 Irreducible
Epidemiology

Depends on geography :
Europe = 1 : 1000
Lapps & native america = 25-50 : 1000
Girls >> Boys = 7 : 1
Left hip more than right vertex presentation with
left occiput ant and left hip adjacent sacrum’s mother
Bilateral = 1 : 5 cases
Retrospective studies tend to report lower incidence
Screening programmes tend to report higher
incidence
Predisposing Factors

 Female Associate with relaxin

 Breech delivery
 First born
 Family history
 Oligohydramnions
 Other congenital abnormalities
Etiology
Genetic
1.Generelized joint laxity
2. Shallow acetabuum
Hormonal factor
Estrogen, progesteron & relaxin in the last few weeks of
pragnancy
Intrauterine malposition
Breech position with extended legs
Post natal
Carry the baby with legs together,hips & knee fully
extend
Patology

1. Muscle contracture : Iliopsoas, Adductor,

Hamstring, quadriceps
2. Capsul streching
3. Labrum acetabulare Limbus
4. Hour glass appearance
5. Fibrofatty pulvinar
6. Ligamentum teres
7. Ligamentum transversum
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Clinical Manifestations

 In the newborn ( up to 6 months )

 limitation of hip abduction
 Asymetrical of thigh / groin skinfolds
 uneven knee level  affected side shorter ( Galeazzi
sign )
 apparent shortening of an extremity
 Klisic sign +
Clinical Screening
 Baby must be relaxed
 Examiner must not use excessive force
1. Asymmetric Abduction
2. Barlow’s Sign: 3. Ortolani Sign: “Clunk of
“Clunk of Dislocation” Entry”
Provocative test Reduction maneuver
4. Galeazzi Sign
 “Asymmetrical skin crease”
“Galeazzi sign”
Klisic sign
Physical Exam Maneuvers

 A. Barlow Test
 dislocates an unstable hip
 stabilize pelvis with one hand, then flex and
adduct opposite hip with posterior pressure.
 dislocation is felt as a “clunk”
 release of posterior pressure  spontaneously
relocates femoral head.
 Physical Exam Maneuvers
(con’t.)
 B. Ortolani Test
 Reduces by gently a recently dislocated hip
 flex and abduct the affected thigh to lift femoral
head into acetabulum
 relocation “clunk”
Physical Exam Maneuvers (con’t)
Other Clinical Manifestations

 In older children ( age of 6 – 18 months )

 limping, toe walking, waddling (trendelenberg
gait)
 increased lumbar lordosis
 leg length discrepancy
 Decrease in ability to abduct
the dislocated hip    
 Klisic sign +
Older child

Trendelenburg sign & gait hyperlordosis

Diagnostic Imaging

 Ultrasound

 most useful during first four weeks of life

 More accurate than radiograph ( < 6 months )
 Non invasive
 visualization of cartilage
 Reduces the need for arthrograms
 Radiograph

 more helpful in the older children ( > 6 months )

 cheap, less operator dependent
 Shenton’s line ( DDH  should be broken )
 Horizontal Hilgenreiner’s & Perkin’s line
 The Acetabular Index ( A.I. )  > 30 degrees in DDH
 Metaphyseal Edge ( M.E.) angle
 Von Rosen view  to evaluate femoral head
reduction in child w/ susp. DDH Hip abducted,
Extend & internaly rotated
Classic line
Treatment
Treatment is based on  age

Aims :
1. concentric reduction
2. early acetabular development
3. Maintained until stable
4. Avoid avascular necrosis of the head
5. Correction of residual dysplasia

Neonate
Goal: maintain hip in flexed and abducted position to maintain
femoral head reduction and tighten ligamentous structures.
Pavlik harness or Frejka splint for 1-2 mos.
 Management Protocol
 1-6 months
 Pavlik harness for 3-4 weeks.
 Closed surgical reduction if harness fails.
 6-18 months.
 Closed or open surgical reduction
 Hip spica cast
 Goal : maintain reduction without damage femoral head
 Toddler ( 18 - 36 months )
 Open reduction with pelvic and/or femoral osteotomy
 3- 8 tahun
 Open reduction with pelvic and/or femoral osteotomy
Treatment

 1-6 months
 Pavlik harness for 3-4 weeks.
 Success rate of 85-95%
 If the dislocation persist after 4 weeks  discontinue
the harness and need Closed or open reduction
Summary

 DDH is an evolving process

 Proper serial exams are imperative to prevent
deformity
 Know clicks from clunks
 Earlier treatment=better outcomes
CTEV
CONGENITAL TALIPES
EQUINOVARUS

AUDI HIDAYATULLAH S
HISTORY

 commonly known as clubfoot

 has been a recognized deformity since the
time of the ancient Egyptians and was
described independently by Hippocrates and
the Aztecs.
 the underlying deformity consists of
 a hind foot in equinus (plantar-flexed)
 varus (inverted).
 a cavus (abnormally high arch)
 adductus component to the midfoot
 Classification

 Postural
Postural or positional talipes can be passively fully
corrected or even overcorrected
 Fixed
1.Flexible – correctable with non-operative treatment
2.Resistant - surgery
Epidemiology

 incidence of CTEV varies widely with race and

geography.
 In Japan, the disease affects approximately 0.5:1,000
live births;
 in Caucasians, the incidence is 1.2:1,000 live births;
 in natives of the South Pacific, the incidence is nearly
7:1,000 live births.
 All populations show a consistent 2:1 male
predominance, with bilateral disease affecting
approximately 50%.
Aetiology

 The true aetiology of congenital club foot is

unknown.
 Most infants who have clubfoot have no
identifiable genetic/syndromal/ extrinsic
cause
 Associations:
 Extrinsic causes:
 Teratogenic agents eg sodium aminopterin
 Oligohydramnios
 Congenital constriction bands/rings
 Genetic causes:
 Mendelian inheritance: eg Diastrophic dwarfism-
 Cytogenetic abnormalities- CTEV can be seen in
syndromes involving chromosomal deletion.
 Multifactorial inheritance
Pathogenesis

 its cause remains unclear, and innumerable

theories have attempted to explain its
origins.
Signs

 affected foot is in equinus and varus.

 There is a crease of varying depth over the
medial midfoot wherein the foot appears to be
folded on itself
 there is some degree of rigidity that may be
severe.
 The calf on the affected side is smaller than that
on the normal side, a difference that persists
even after correction of the deformity.
Pathology
 Bone
 Femur, tibia and fibula
 the entire lower limb can be shorter
 fibular shortening most common

 Talus : all relationships of the talus are abnormal-

including:
 anterior extrusion of the body of the talus
 medial and plantar deviation of the neck of the talus
Pathology, cont
 Os calcis
 medial rotation, equinus

 Navicular
 medial subluxation

 Cuboid
 medial subluxation

 Forefoot
 adducted and supinated, severe cases have
cavus also
 Muscle
 Atrophy of the leg especially in peroneal group -
number of fibres is normal , fibres are smaller in
size
 Triceps surae, Tib post, FDL,FHL are contracted
Other soft tissues

 Tendon sheaths
 frequently thickened, esp. about Tib post and perinea

 Joint capsules
 resistant CTEV - contractures of ankle, subtalar, talonavicular,
calcaneocuboid jts

 Ligaments
 Resistant CTEV - contractures of calcaneofibular + talofibular ligs, deltoid
lig, long and short plantar ligs, spring lig, long plantar lig. (bifurcate lig )

 Fascia
 Contracture of fascial planes and of plantar fascia
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Treatment

 Aims
 Correct deformity early
 Correct deformity fully
 Hold the correction until growth stops
Non operative treatment
Serial casting
 2 weekly changes for 3 months
 40-60% will be corrected
   Splintage
 Either with single boot or Denis Brown splints (for bilateral cases)
 Splintage begins at 2 - 3 days post birth
 Order of correction
 Forefoot adduction
 Forefoot supination
 Equinus
 NB. Attempts to correct equinus first may break the foot producing a
rocker bottom foot Force must never be used
Ponseti method

 This method was developed by Ignacio Ponseti,

MD, of the University of Iowa. The premise of
the method is based on the cadaveric and
clinical observations of Dr. Ponseti.
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Operative treatment
 Indication
 when a plateau has been reached in non-operative treatment
 persisting deformity
 rockerbottom deformity
 rapid relapse after correction has stopped
 when the child is of sufficient size to enable anatomy to be recognised

 Treatment by age
 Less than 5 years correction can be achieved by soft tissue procedures
(Postero-medial release)
 More than 5 years requires bony reshaping, eg dorso-lateral wedge
excision of the calcaneo cuboid joint (Evans procedure) or osteotomy of
the calcaneum to correct varus
 More than 10 years lateral wedge tarsectomy or triple if the foot is mature
(salvage procedures)
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by Audi Hidayatullah S

OSTEOGENESIS IMPERFECTA
 Introduction

 ~ brittle bone disease, Lobstein disease, blue-sclera

syndrome, and fragile-bone disease
 A spectrum of clinical disorders that share the symptom
of abnormal bone fragility.
 Most OI patients have disorders of collagen production
that affect either the quantity or quality of collagen
produced.
 The phenotype of OI is quite variable (mild  severe)
 The most severe form of OI is fatal in the perinatal period.
 The orthopaedic surgeon may be involved in the
Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
operative
6th Edition. 2006 management
Lippincott of the fractures and deformities.
Williams & Wilkins
 History

 The earliest known case of osteogenesis

imperfecta is in a partially mummified infant's
skeleton from ancient Egypt now housed in
the British Museum in London.
 In 1835, Lobstein coined the term
osteogenesis imperfecta.

Smith R, Francis MJ, Houghton GR. The brittle bone syndrome.

In: Osteogenesis Imperfecta. London: Butterworth. 1983.
 Epidemioplogy

 A rare condition
 Estimated prevalence of 1/15,000 to 1/20,000 children
 No racial or ethnic predilection
 Age
 symptoms (ie, fractures) begin widely varies.

Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
 Clinical Presentation

 The hallmark of OI:

 brittle bones and the tendency to fracture
 recurrent fractures occurring in childhood particularly during the
preschool years.
 Bone pain (Chronic and unremitting, usually relates to old
fractures).
 Muscle weakness
 Ligamentous laxity and joint hypermobility
 Wormian bones are present in the skull ± 60% of patients.
 Blue or gray-blue sclerae
 Dentinogenesis imperfecta
Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
Wormian Bones
 Classification
(Sillence etc, 1979)
 Type I: Mild OI
 Type II: Fetal/ Lethal Perinatal OI
 Type III: Severe OI
 Type IV: Moderate OI

Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
 Type I: Mild OI

 Limb Deformity (-)

 Normal or low-normal height
 Bony fragility
 Multiple fractures during childhood.
 Fractures ↓ after puberty.
 Blue/ White sclerae
 50% percent have presenile deafness  typically in
the third decade of life
 IA: Normal Teeth
 IB: Dentinogenesis
Morrissy, Raymond Imperfecta
T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
Multiple Fracture
Blue Sclerae
 Type II: Lethal Perinatal
OI
 The most severe form of OI
 The baby die at or shortly after birth
 Born with crumpled femoral and crumpled ribs
accompanied by pulmonary hypoplasia  death
 Central nervous system malformations and hemorrhages
 Blue/ White sclera
 A small nose, micrognathia, or both.
 All patients have in utero fractures
 Diagnosed by prenatal ultrasonography :
 Short & broad limbs (low echogenicity and low shadowing)
 Not possible to distinguish between lethal type II OI and severe OI
Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
Bilateral Femoral fractures
in newborn
 Type III Severe OI

 The most severe of the survivable types.

 Relatively large skulls but undeveloped facial  characteristic triangular
appearance of the face.
 Sclerae pale blue at birth  normal by puberty.
 Dentinogenesis imperfecta
 Short stature
 Severe limb deformities (bowing and coxa vara)
 Multiple vertebral compression fractures  severe scoliosis, kyphosis, and rib
cage deformity.
 Respiratory complications secondary to kyphoscoliosis
 Often have multiple fractures when they are born, but do not have the severe
thoracic deformities seen in type II OI.

Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
 Radiographic characteristics :
 very osteopenic bones with deformity related to previous
fracturing.
 A characteristic popcorn appearance of the epiphysis and
metaphysis occurs in early childhood.
 The pedicles of the vertebrae are elongated. The vertebrae are
wedged and may assume a codfishlike biconcave morphology.
 Posterior rib fractures are seen.
 Basilar invagination of the skull
BASILAR INVAGINATION
Spinal Findings
Popcorn
Apperance
 Type IV: Moderate OI

 Short stature
 Bowing and vertebral fractures
 Most patients are ambulatory, some use walking
aids.
 A wide range of ages at the first fracture and
number of fractures in patients
 The sclerae typically white
 Subtype:
 IV A: Normal Teeth
 IV B: Dentinogenesis
Morrissy, Raymond T.; Weinstein, Stuart imperfecta
L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
Dentinogenesis
imperfecta
 Pathophisiology

 Deffect in Type I Collagen:

 Qualitative: abnormality collagen I molecule
 Quantitative: decreased production of normal
collagen I molecules
 Type I collagen fibers are found in the bones,
organ capsules, fascia, cornea, sclera,
tendons, meninges, teeth and dermis.

Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
Etiology of Osteogenesis Imperfecta

 Mutations in the loci that encode a left-

handed helix formed by intertwining of pro-
alpha 1 and pro-alpha 2 chains (type I
collagen fibers)
 Mutations within the collagen 1A1 gene found on
chromosome 7q
 Mutations in the collagen 1A2 gene found on
chromosome 17q.
Diagnosis

 The clinical approach  Gold standard

 No single laboratory test that distinguishes
individuals with OI from normal.
 Plain radiography.
 Dual energy x-ray absorptiometry (DEXA)
scanning: BMD lumbar and femoral bone low
 Skin biopsy (collagen study)
 DNA-based genetic test for OI (not currently
clinically useful)
Medical Treatment

 Bisphosphonates
 Used in severe OI.
 Widely used drugs based on the pyrophosphate
molecule  the only natural inhibitor of bone
resorption.
 The exact mechanism of the drug is unclear  its
primary action at osteoclast.
 The direct effect of the bisphosphonate : ↓resorption
and turnover of bone bone pain and fractures ↓ 
weight bearing and mobility ↑ further
strengthening of bone and muscle.
 Proper vitamin intake such as Calcium, Vitamin C, and
Vitamin D are essential for bone growth and repair.
 Anabolic agents: specifically human growth hormone 
stimulates increased bone turnover. Clinical research
required.
 Bone Marrow Transplantation (BMT)
 Casts
 Normal casts would actually harm patients with OI.
 Instead specialized casts are used due to the brittle nature of the
bones.
 Fiberglass offers a lighter and more comfortable solution for OI
patients.
 The main purpose of casts: Immobilize the broken limbs.
 Immobilization should be limited to prevent bone loss.
 Surgical Treatment

 The treatment of fractures in mild OI

minimize disuse osteoporosis.
 Severe OI requires specialized techniques.
 Children with severe OI : IM rodding of the
long bones best carried out in conjunction
with medical treatment using
bisphosphonates

Morrissy, Raymond T.; Weinstein, Stuart L. Lovell & Winter's Pediatric Orthopaedics,
6th Edition. 2006 Lippincott Williams & Wilkins
Extensible Rod
Complications

 Secondary osteoporosis
 Immobilization following fractures or surgery
 Decreased physical activity and weight bearing
with severe deformity
 Prevention of secondary osteoporosis is an
important concept when treating fractures,
planning surgery, or recommending general
care.
Prognosis

 The prognosis with regard to walking is much

more strongly influenced by the subtype of OI
than by the treatment
 Modern combinations of medical and surgical
treatment combined with rapid advances in
the understanding of the biology of the
disease may one day change this situation.
Living With Osteogenesis Imperfecta

 Patients must make frequent trips to the hospital to get casts for
their multiple broken bones.
 Braces, walkers and wheelchair use is common.
 A balanced healthy diet and exercise is important to keep the fragile
body as healthy as possible.
 Specialized dental care may be necessary to protect fragile teeth.
Thank You