Congenital Diseases

Objective :
Define torticollis,talipes and congenital
dislocation of hip
State the investigation to diagnose the above
condition
State the treatment
Describe the nursing diagnosis and nursing
interventions
CONGENITAL
MUSCULAR
TORTICOLLIS
 A condition in which an infant
‘s neck muscle is shortened
causing neck to twist.

DEFINITION

Wryneck
 Etiology
Trauma around the time of birth:
-injury due to breech or difficult forceps
deliveries
-pressure on the sternocleidomastoid
(SCM) muscle  muscle become fibrous
and shorten
 Clinical manifestation
Painless mass on the neck appearing
during the first 2 month of life and
persistent tilt of the head to one side
Limited ability to turn head or move neck
(limited range of motion)
 Diagnostic test
1. X-rays – around neck to check fracture
2. CT scan and /or MRI – check
abnormalities in the soft tissue, such as
tumors
3. Electromyography(EMG) – to record the
electrical activity of skeletal muscle
4. Physical examination
 Treatment/management
• Physical therapy- to stretch and loosen the
muscle and improve the range of motion
a) Placing toys where the infant has to turn his
head to see them
b) Place the child in bed with the involved side
toward the wall so that they have to look the
opposite way to see you on side the crib
 Cont..
(c) Use of supplement stretching exercise:
Tubular Orthosis for Torticollis (TOT)
collar. Fitted by a physical therapist
 Pain relief- analgesic
 Muscle relaxant
 Surgery- open tenotomy (to lengthen the
short muscle)
 Complication
Neck deformity
Permanent neck stiffness
Congenital
Clubfoot
 Definition
What is congenital clubfoot?
– It is a condition in which 1 or both feet are twisted
into an abnormal position at birth.The condition is
also known as talipes.
What is a structural foot deformity?
– There is a deformity not only to the bones and
muscles, but also the ligaments and tendons.
 Etiology
The exact cause remains unclear.
Theories:
- Genetic Factors
- Utero Malalignment
 Sign & symptoms
the toes touch the inside of the ankle, or it may be only
vaguely apparent.
deformed talus
the Achilles tendon shortened
the calcaneus shortened and flattened.,
the calf muscles are shortened and underdeveloped,
soft-tissue contractures form at the site of the deformity.
The foot is tight in its deformed position and resists
manual efforts to push it into normal position.
Clubfoot is painless, except in elderly, arthritic patients.
In older children, clubfoot may be secondary to paralysis,
polio-myelitis, or cerebral palsy, in which case treatment
must include management of the underlying disease.
 Diagnostic Test
X-Ray
Ultra Sound
Genetic Testing
Physical Examination
Pictures of Clubfoot

Bilateral Clubfoot

Unilateral Clubfoot
 Treatment
Non –Operative treatment
 Manipulation to improve the alignment of the foot.
 Application of a long leg cast to maintain the new
alignment (Ponseti Method)
 Boots connected by a bar are used to maintain the
correction.
Surgery
 If surgery is going to performed, the optional time
in a child’s life is between 6 and 12 months of
age.
Casting (Ponseti method)

Denis Browne splint

 Living with Bilateral
Clubfoot
Physical restrictions
– No running
– No jumping
– Can’t stand on toes
Pain
Accepting the handicap
 Review
Clubfoot is a structural foot deformity
present at birth.
It’s cause is unknown.
1 out of every 1000 people are born with
clubfeet.
Treatment not only includes surgery, but
manipulation through the Ponseti
Method.
Congenital Hip
Dislocation
(CHD)
 Anatomy
In hip dislocation,the ball at the top of the
thightbone (femoral head) does not sit
securely in the socket (acetabulum) of the
hip joint.Surrounding ligaments may also
be loose and strectched.The ball may be
loose in the socket / completely outside of
it.
 Causes
Unknown
Maternal hormones
Ligamentous laxity
Hip instability
Certain mechanical factors
 Symptoms
“clicking” sound
Less flexibility (when he starts to walk,he’ll
probably limp,walk on his toes@ “waddle”
like a duck)
Leg will look shorter than the other
one,skin folds in the thighs will appear
uneven
 Risk factor
Female
First born babies
Racial differences
Family history – the condition increase the
risk in a manner suggestive of
multifactorial inheritance
 Cont…
Breech presentation – in which knees are
extanded is a higher risk than with knees
flexed.
Oligohydramnios
Neuromuscular disorders (cerebral
palsy,meningomyelocele,arthrogryposis)
 Diagnosis test
Ultrasound
X-ray
Ct scan & MRI – arthrography
The Barlow test – examiner attempts to
push the ball of the hip rearward out of the
socket
 Cont…
The Ortolani test – maneuver to reduce a recently
dislocated hip
 1-2 months – if positive, examiner feels a “clunk” as it
pops back in place
 3-6 months – galeazzi sign (examined in lying supine
with the hip & knees flex).Asymmetry of the gluteal
thigh or labral skin folds,decreased abduction on the
affected side,standing or walking with external rotation
of the affected leg & leg length inequalify
Ortolani’s and Barlow’s test
 Management
Traction (older children)
Surgery (arthroplasty)
The Pavlik Harness
-teach the parents on how to remove the
harness before and how to replice the hip
again before using the harness.
-assess the skin under the straps daily for
irritation or redness
 cont…
Spica cast
-teach parents how to do neurovascular
assessment (monitor temperature and
circulation in the toes) to prevent circulatory
compression.
Hip spica
 Nursing management
Care of child with cast or splint
1.Assess the ‘5P’ every 1 to 2 hours.
-pain -paresthesias
-pallor -paresis
-diminished distal pulses
2.Assess nail beds for capillary refill,if nails are
too thick or discolored,assess the skin around
the nail to decreased tissue perfusion
 Cont..
3.Elevated the site to prevent edema and
swelling
4.Change a child position every 2 hours to
prevent pressure sore.
5.Provide skin care to maintain hygiene and
promote comfortable to a child.
 Complication

Re-dislocation
Stiffness
Blood loss
Avascular necrosis
 Cont…
Extreme abduction
Long term complication;
 Osteoarthritis
 Pain
 Abnormal gait
 Unequal leg length
 Decreased agility
1. Deficient knowledge related to lack
of information about condition
Encourage parents to ask any doubt
about the procedure to reduces anxiety.
Explain that this communication may
initially seem awkward but in time it will
feel appropriate
Evaluate the family readiness to receive
explanation regarding the child treatment
and care
 Cont…
Provide information about sources and support
group
Spent time with the child and family
-therapeutic relationship
Advice parents to bring a child for follow up as
prescribed to prevent any complication
Instruct parents to report and seek medical help
if child experiences any abnormalities to
prevent complication
2.Impaired physical mobility related
to musculoskeletal impairment
Describe the different types of fractures
and treatment including casting, surgery,
and traction therapy.
Discuss nursing care of the child with a
cast or traction set-up.
Discuss the nursing care,
pathophysiology, signs and symptoms,
diagnostic evaluation, and therapeutic
management: e.g., Juvenile Rheumatoid
Arthritis, Osteomyelitis, Legg-Calve-
Perthes, Torticollis,Slipped Femoral
Capital Epiphysis, Club Feet, and Sports
Injuries.
3.Delayed growth and development
related to effects of physical
disability
Provide adequate nutrition to maintain
proper developments
Participate actively in physiotherapy
session
4.Risk for impaired skin integrity related to
specify: physical immobilization by cast
internal factors of altered circulation,
sensation by cast pressure
Inspect skin,tissue and mucus membrane
routinely.
Assess nutrition status,provide balanced
diet;protein,vitamin and mineral.
Do not try to scratch under a cast with a sharp
object to prevent injury to the child.
Do not get a plaster cast wet to prevent infection
occur.
Avoid a cast from get dirty to maintain good
hygiene
5.Risk for infection related to surgery
 Assess the specific signs of infection eg:
any discharge, odor and poor healing
 Maintain aseptic technique eg :during
dressing
 Monitor vital signs such as temperature
4hours
 Provide drink a plenty of fluid to prevent
dehydration
 Cont…
 Administer antibiotic as prescribed
 Ensure adequate nutrition to promote
wound healing
 Monitor intake and output to maintain
hydration
Appendix
 Reference
John Ebnezar: Textbook of orthopaedics
3rd edition
http://en.wikipedia.org/wiki/Congenital_deform