Perthes disease is a childhood condition that affects the hip. It results from a temporary loss of blood supply to the femoral head. The key points are:
- It typically affects children between the ages of 4-10 years old and is more common in boys.
- The exact cause is unknown but theories involve vascular compromise to the femoral head.
- Presentation includes limping, groin or thigh pain that increases with activity. Imaging shows changes to the femoral head over different stages.
- Treatment depends on the stage and age of the child, ranging from non-operative bracing and casting to surgical procedures like osteotomies if containment is needed to prevent further deformity and damage.
2. PERTHE’S DISEASE
• FIRST DESCRIBED BY
LEGG (USA), AND
WALDENSTORM IN
1909, AND BY
PERTHES(GERMANY)
AND CALVE(FRANCE) IN
1910
• Also known as pseudo-
coxalgia, osteochondritis
deformans, coxa-
juveniles
3. Epidemiology
• Disorder of the hip in young children
• Usually ages 4-8yrs
• As early as 2yrs, as late as teens
• Boys:Girls= 4-5:1
• Bilateral 10-12%
4. Etiology
• Unknown
• Past theories: infection, inflammation, trauma,
congenital
• Most current theories involve vascular
compromise
.
5. Blood supply to femoral head
• Infants
1. Metaphyseal arteries .
2. Lat epiphyseal arteries
3. Lig teres – insignificant
• 4 mts – 4 years
1. Lat epiphyseal
2. Metaphyseal art. decrease in number
(due to appearance of growth plate).
3. ligamentem teres not well developed.
6. Blood supply to femoral head
• 4 yrs to 7 years
1. Epiphyseal plate forms a barrier to metaphyseal
vessels.
• Pre-adolescent
1. After 7 yrs arteries of lig teres become more
prominent and anastomose with the lateral
epiphyseal vessels.
2. so between age 4-7 head of femur is mainly
supplied by lateral epiphyseal vessels.
7.
8.
9. -- Susceptible child : delayed bone age
--haemarthosis due to trauma or haemophilia
-- septic arthitis of hip joint
-- Synovitis
Medical cause (gaucher’s disease, cretinism, rickettsial infection ,
caisson,s disease.)
10. Presentation
• Often insidious onset of painless
limp,increases by activity.
• May complain of pain in groin, thigh, knee
• Few relate trauma history
• Can have an acute onset
11. Physical Exam
• Decreased ROM,
especially abduction and
internal rotation
• Trendelenburg test often
positive
• Muscular atrophy of
thigh/buttock/calf
• Limb length discrepency
12. Imaging
• AP pelvis
• Frog leg and lateral
view
• ..bone scan -shows
decreased uptake
13.
14. Radiographic Stages
• Four Waldenstrom stages:
– 1) Ischaemia and bone death(stage of avascular
necrosis)
– 2) Fragmentation stage
– 3) Reossification stage
– 4)Remodelling
15. Stage of Avascular Necrosis
Ischemia
A part ( anterior) or whole of capital
femoral epiphysis is necrosed.
On X-ray –
– The ossific nucleus looks smaller
– Classically of Perthes’, looks
dense
– The articular cartilage remains
viable & becomes thicker than
normal
– increased joint space.
16.
17. Stage of REVASCULARIZATION / FRAGMENTATION
• Ingrowths of highly vascular & cellular connective tissue.
• Necrotic trabecular debris is resorbed & replaced by vascular
fibrous tissue the alternating areas of sclerosis and
fibrosis appear on X- ray as fragmentation of epiphysis.
• New immature bone laid on intact
necrosed trabeculae further increases
the density of ossific nucleus on
X-ray.
. cresent sign (curved subchondral
radiolucent line)
present
18.
19. The femoral head may extrude from acetabulum
at this stage.
Stage of REVASCULARIZATION / FRAGMENTATION (contd.)
20. Stage of Ossification / Healing
New bone starts forming and epiphyseal
density increases in the lucent portions of
the femoral head.
21. • Remodeling / Residual stage
This is the stage of remodeling and there is no
additional change in the density of the femoral
head.
Depending on the severity of disease, speed of
repair , the residual shape of the head may be
spherical( coxa magna, coxa breva)
or distorted.
22.
23. Modified Elizabeth Town classification
Stages
• I Sclerotic
• A: no loss of height
• B: loss of height
• II Fragmentation
• A: early
• B:late
• III Healing
• A: peripheral
• B:>1/3epiphysis
• IV Healed
• 220 days
• 240 days
• 255 days
24. Salter-Thompson Classification
• BASED ON EXTENT OF SUBCHONDRAL
FRACTURE-
• A> LESS THAN HALF OF FEMORAL HEAD
INVOLVED
• B> MORE THAN HALF OF THE FEMORAL HEAD
INVOLVED
25.
26. Herring Lateral Pillar Classification
• 3 groups:
–A) no lateral pillar
involvment
–B) >50% lat height intact
–C) <50% lat height intact
27.
28. Catterall classification
• Based on extent of epiphyseal involvement
and percentage of collapse as seen in x-ray
(both AP and Lateral view)
29.
30.
31.
32. Catteral ‘head at risk sign’
Clinical
• Female sex
• Obese
• Age is on higher
side
• Progressive fixed
flexion and
adduction
deformity
Radiographic
1) lateral subluxation of the
femoral head from the
acetabulum,
2) speckled calcification
lateral to the capital
epiphysis,
3) diffuse metaphyseal
reaction (metaphyseal
cysts),
4) a horizontal physis
5) Gage sign
38. Prognosis
• 60% of kids do well without tx
• AGE is key prognostic factor:
– <6yo= good outcome regardless of tx
– 6-8yo= not always good results with just
containment
– >9yo= containment option is questionable, poorer
prognosis, significant residual defect
– --Flat femoral head incongruent with acetabulum=
worst prognosis
39. Treatment in onset to early
fragmentation stage
For age < 5 yr- treatment is not needed regardless of stage
except when there is femoral sublaxation--- containment is
needed
for age 5-8 yr--
When <50% of femoral epiphysis is necrotic-- 3 monthly follow
up with xray to detect early sublaxation
When >50% of femoral epiphysis is necrotic—containment is
done
40. Continued..
For age 8-12 yr containment is done as soon
as disease is diagnosed provided disease is
not in the late stage of fragmentation .
For age >12 yr- containment is useless,
treated as adult with osteonecrosis
41. continued
Treatment ( late fragmentation stage to
complete healing and reossification)---
Depending on the deformity of the femoral
head or hinged abduction
For hinged abduction- do valgus osteotomy
For management of the sequele –
reconstructive procedure
42.
43. Aims of Treatment
• CONTAINMENT( hold the femoral head in
acetabulum)
• Minimize enlargement of the femoral head
• Prevent or correct GT overgrowth
• Prevent secondary degenerative arthritis of
the hip
44. Treatment options
• Weight Relief
• Containment by bracing or casting
• Surgical Containment
• Greater trochanteric arrest
45. Non-operative Tx
• Improve ROM 1st
• Bracing:
• Removable abduction orthosis
• Pitrie cast
-Wean from brace when improved x-ray healing signs
• Check serial radiographs
– Q3-4 mos with ROM testing
• Orthotic treatment is discontinued when the disease enters
the reparative phase and healing is established
55. INDICATIONS- patients with a spherical femoral head, little or no
acetabular dysplasia (center-edge angle of at least 15 to 20
degrees),lateral overloading, and a valgus neck-shaft angle of more
than 135 degrees.
DISADVANTAGES
-varus angulation that may not correct with growth (especially in
an older child),
– further shortening of an already shortened extremity,
– the possibility of a gluteus lurch produced by decreasing the
length of the lever arm of the gluteal musculature,
– the possibility of nonunion of the osteotomy,
– requirement of a second operation to remove the internal
fixation
v
64. INNOMINATE OSTEOTOMY
• ADVANTAGE-Anterolateral coverage of the femoral head,
lengthening of the extremity (possibly shortened by the
avascular process), and avoidance of a second operation
for plate removal.
• DISADVANTAGES-1)inability sometimes to obtain proper
containment of the femoral head, especially in older
children;
• 2)an increase in acetabular and hip joint pressure that
may cause further avascular changes.
• 3)an increase in leg length on the operated side
compared with the normal side causing a relative
adduction of the hip and uncover the femoral head
• Eg.-Salter’s ostoeotomy
65. THE SHELF PROCEDURE (STAHELI)
IND.; -Severe dis ( CATTERALL-3/4) IN EARLY FRAG.
STAGE.
67. Salvage surgery for sequelae of Perthes’ds
• Aims of treatment
Relieve pain
Correct Trendelenburg gait
Minimize the risk of development of
degenerative arthritis
68. TREATMENT
Reconstructive procedures
• Valgus extension osteotomy
indication -hinge abduction of hip
• Cheilectomy
indication – malformed femoral head with lateral
protuberance Coxa plana
• Chiari osteotomy
indication – malformed femoral head with lateral
subluxation
• Trochanteric advancement
indication – premature capital femoral physeal arrest
• Greater trochanteric epiphysiodesis
indication – premature capital femoral physeal arrest
• Shelf augmentation procedure
indication – coxa magna & lack of acetabular
coverage
-incidence of positive family hx ranges from 1.6% to 20%, but no hard evidence of predisposition
-is more common in certain geographic areas (urban>rural)=nutritional?, later born children, strong association with ADHD (33%)
-Phemister- thought it was infectious but cx neg
-Axhausen- thought bacillary embolism with weak infection which healed quickly
-1975 Matsoukas showed association with prenatal rubella
-1973 Sanches, infarcted animal femoral heads, unable to produce typical histologic picture of LCPdz with one infarction, could do it with a second. Supported by Inoue using human histologic material
-must recognize thigh & knee pain as possible hip pathology
-pain usually mild and relieved by rest, often present late due to mild sx
-early decreased abduction due to synovitis/spasm, may become permanent after development of femoral head deformity
-adduction contracture due to long standing spasm
-atrophy due to disuse due to pain, shows long standing nature
-short limb due to head collapse= poor prognosis
-frog leg= better for crescent sign
-compare films with previous to determine change
-arthrography can show status of cartilage not shown on x-ray, check ROM to r/o hinging abduction
-hinging abduction due to large femoral head extruding laterally & hinging over
edge of acetabulum
-Catterral 1 and usually 2 do well without treatment
-abduction usually affected most of ROM
-use PT to regain abduction (overcome spasm) and internal rotation
-may require several weeks of abduction traction
-don’t start bracing until abd/int rot restored to normal
-arthrography pre-bracing to determine congruency throughout ROM
-head collapse is independent of weight bearing, not necessarily NWB
-hips braced in abd/ int rotation to transmit weight over wide area of acetabulum, prevents head collapse