KELAINAN NEUROMUSKULAR

dr. Jufri Latief, Sp.B.,Sp.OT

BAGIAN ORTOPEDI DAN TRAUMATOLOGI

FAKULTAS KEDOKTERAN UMI
MAKASSAR

KELAINAN NEUROMUSKULAR

Paralisis Otak ( Cerebral Palsy )

Degenerasi Spinoserebelar ( Friedreich Ataksia)
Atropi Muskulus Peroneal (Charcot-Marie Tooth)
Lesi Medula Spinalis (Spina Bifida, Poliomielitis)
Peripheral Neuropathy
Duchene Muscular Dystrophy

Cerebral Palsy
Cerebral palsy occurs in approximately
0.25% of all ive births. In the majority of
cases, there is no known cause. A nonprogressive disorder of the brain causes
impairment of motor function. Mental
handicap is common. Note this 6-year-old
boy with right spastic hemiplegia. He has
equinus of the right foot.
This 2-year-old girl has spastic diplegia. She
has the characteristic scissoring of the legs
due to adductor spasticity. Physiotherapy to
stretch the spastic muscles is done initially.
Intramuscular botulinum toxin injection to
reduce the spasticity has gained
importance. The effects last for a few
months and the injection can be repeated.

Cerebral Palsy
This 2-year-old child has athetoid cerebral
palsy. It is caused by an extrapyramidal
lesion and is distinguished by purposeless
writhing movements. Joint contractures
are uncommon as the child is frequently
moving.

An 8-year-old child with spastic

quadriplegia. All 4 limbs are involved and
he is unable to walk. He is mentally
retarded and totally dependent on parental
care at home. He has windswept
deformities of the hips, flexion deformities
of the knees, right elbow and both wrists.

Cerebral Palsy
Marked flexion contractures of the elbow
and wrist. This causes difficulty with
maintaining skin hygiene at the flexor
creases and wearing clothes. The
contracted tendons can either be released
or lengthened.

Contracture of the adductor and psoas

tendons causes the adduction and flexion
deformity of the hips.

Cerebral Palsy
The elbow flexion contracture in
this child is less marked 10 days
after release of the biceps and
fractional lengthening of the
brachioradialis
Adductor tenotomy in a 5-year-old
with spastic quadriplegia. The
procedure has reduced the subluxed
right hip. Hip subluxation proceding
to dislocation is common in spastic
quadriplegics due to adductor
contracture.

Cerebral Palsy
An 8-year-old boy with crouch gait due to
contracture of the hips, knees and ankle. This
posture impairs his gait.

His posture has improved 6 months after

simultaneous release of adductors, psoas,
hamstrings and lengthening of the Achilles
tendons. The deformity may recur as he grows
and physiotherapy to stretch the tendons is
continued.

Cerebral Palsy
Flexion deformity of the knee is
due to hamstring contracture.
The popliteal angle is
approximately 70 degrees.

In this 3-year-old, an extension

contracture of the knees has
occurred due to quadriceps
fibrosis.

Spina bifida
Spina bifida is caused by disturbance
in the development of the vertebral
arches. Thoraco-lumbar kyphosis in an
11 year-old-boy with spina bifida. The
kyphosis may occur due to a
combination of muscle weakness and
bony anomalies.

Skin ulceration over the apex of the

kyphus is common as a result of
pressure necrosis. Recurrent skin
ulceration is an indication for surgical
correction of the deformity.

Hip dislocation in spina bifida

The pelvic X-ray of a 4-year-old
girl with spina bifida. Both hips
appear normal. She had
weakness of the lower limbs but
was able to walk.
By the age of 8 years, the right
hip had dislocated as the right
lower limb was weaker
compared to the left . This had
occurred gradually.

Hip dislocation in spina bifida

She is able to stand and walk with support.
Even after an open reduction of the right hip,
the walking ability may not be improved.
Furthermore, there is a risk of the hip redislocating.

By the age of 11 years, she uses the wheel

chair when she is out of the house. The hips are
not painful.

Spina bifida with foot deformity

A seven-year-old girl with spina
bifida and left club foot deformity.
There is a neuropathic ulcer (arrow)
as a result of walking on the outer
aspect of her left foot. She was
treated by debridement of the ulcer
and a talectomy to correct the club
foot deformity.
Neuropathic ulcer of the left foot in
another 9-year-old girl with spina
bifida

KELAINAN METABOLIK
DAN ENDOKRIN
Penyakit Rakitis (Ricketsia)
Osteomalasia
Penyakit Skorbut
Osteoporosis
Hipotiroidisme Pada Bayi
(Kretinisme)

Gambar A.
Gambaran klinis penderita rakitis berupa
pembengkakan pergelangan tangan.
Gambar B. 1. Terlihat penurunan densitas tulang pada
daerah metafisis
sehingga daerah ini tampak lebih
radiolusen; 2. Gambaran radiologik penderita yang sama
setelah pemberian vitamin D.

BILATERAL BOWING TIBIA CAUSA

HYPOPHOSPHATEMIC RICKETS

Primary hyperparathyroidism
16-year-old girl with primary
hyperparathyroidism due to
parathyroid adenoma. She has
developed scoliosis and
deformities of the limbs. The
increased osteoclastic
resorption of bone causes
rickets and subsequent bone
deformities. The parathyroid
adenomas have been excised.
Wind-swept deformity of the
wrist. The left wrist is radial
deviated while the right wrist
is ulnar deviated.

Primary hyperparathyroidism
Knock-knee deformity due to the
valgus of both knees, more marked on
the left. Note the tilted joint line of the
left knee as the valgus is due to
deformity of the distal femur. She
subsequently underwent a
supracondylar osteotomy and plating
of the left femur to correct the
deformity.
Note the pelvic obliquity and bilateral
deformities of the femoral head in this
X-ray.

KELAINAN EPIFISIS
DAN LEMPENG EPIFISIS
(KELAINAN PERTUMBUHAN)

Slipped upper femoral epiphysis

This 13-year-old boy has a slipped upper
femoral epiphysis (SUFE) of the right hip. He
complained of right knee pain for the past
few weeks. Hip pain referred to the knee is
common in children. The hips must always
be examined in any child complaining of
knee pain. The cause is unknown but is
probably multifactorial. Children with a
known endocrine disorder are at risk of
developing a slip of the upper femoral
He has right hip pain for a few months.
epiphysis.
He is able to walk without support and
sit cross-legged

Slipped upper femoral epiphysis

External rotation of the right hip is only
slightly limited.

Markedly limited internal rotation of the

right hip as the femoral head is
posterior to the neck.

Slipped upper femoral epiphysis

The X-ray showed a minimal slip
(less than 1/3 femoral head
diameter displacement) of the right
hip. The sign is subtle and may be
missed. He was treated with a
single screw-fixation.
Another 14-year-old boy with a
moderate slip of the left hip
(between 1/3 and 1/2
displacement of the femoral
head).

Slipped upper femoral epiphysis

Fourteen-year-old girl internallyfixed with two screws for a left
slipped upper femoral epiphysis.

One year later, avascular necrosis

of the femoral head has occurred.
There is narrowing of the joint
space.

Slipped upper femoral epiphysis

At the age of 15 years and 9 months,
there is remodelling of the femoral
head.

At age 18 years, the femoral

head has healed with residual
deformity. The joint space is
narrowed. She is asymptomatic.

Perthes disease
Perthes disease of the right hip in a
nine-year-old boy. He presented with hip
and knee pain of a few months
duration. Traction is used to immobilise
the hip and reduce the pain. Perthes
disease is due to infartion of the femoral
head. The cause is unknown. The
susceptible child is usually a boy
between the ages of 4 and 9 years.
X-ray shows increased sclerosis
indicating avascular necrosis of the
right femoral head.

1 year later, the right femoral

head has fragmented. With
the collapse of the femoral
head, the Shentons line is
disrupted and the femoral
head has subluxed.

15-year-old boy with bilateral

Perthes disease. The
prognosis for Perthes disease
in children younger than 5
years of age is frequently
good as the potential for
healing and remodelling is
greater than the older child.
In the adolescents, the
prognosis is poor.

Thank You