Congenital skeletal malformations

Maria Carmela L. Domocmat, RN, MSN
Intructor
Northen Luzon Adventist College 1

a congenital deformity in which the foot is
twisted out of shape or position;
Aka: clubfoot

Dorland's Medical Dictionary for Health Consumers. © 2007 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

Maria Carmela L. Domocmat, RN, MSN 2

dorsiflexion - t. calca´neus
plantar flexion - t. equi´nus
abducted and everted -t.val´gus or flatfoot
abducted and inverted - t. va´rus
various combinations
t. calcaneoval´gus
t. calcaneova´rus
t. equinoval´gus
t. equinova´rus Dorland's Medical Dictionary for Health Consumers. © 2007 by Saunders, an
imprint of Elsevier, Inc. All rights reserved.


t. calcaneoval´gus
the foot is turned outwards with the toes pointing
upwards
t. calcaneova´rus
the foot points inwards and up
t. equinoval´gus
the foot points outwards and down
t. equinova´rus
most common type
foot is fixed in plantar flexion (downward) and
deviated medially (inward)


http://img.tfd.com/dorland/thumbs/talipes.jpg


http://www.abdn.ac.uk/~gen155/graphics/clubfoot.jpeg

http://www.fpnotebook.com/_media/Ortho
PedsFootCF.jpg


http://1.bp.blogspot.com/_IZV_l47MkXQ/TRpGEJogmHI/AAAAAAAAAGw/X1VQqO
DtJG4/s1600/child_foot_clubfoot_intro01.jpg


o The true etiology of congenital clubfoot is
unknown
o Extrinsic associations include
Teratogenic agents (eg, sodium aminopterin)
Oligohydramnios
Congenital constriction rings


o Genetic associations include
o mendelian inheritance (eg, diastrophic dwarfism;
o autosomal recessive pattern of clubfoot inheritance).
o Cytogenetic abnormalities (eg, congenital talipes
equinovarus [CTEV]) can be seen in syndromes
involving chromosomal deletion.


o Talipes may be positional or structural.
Positional talipes is caused by abnormal pressures
compressing the foot while it's developing, as a result
of its position in the womb.
Structural talipes is a more complex condition and
probably caused by a combination of factors, such as
a genetic predisposition

http://www.bbc.co.uk/health/physical_health/conditions/talipes2.shtml


o deformity is readily apparent at birth
o can be detected antenatally during the routine
development ultrasound scan around 20 weeks.
o X-rays may be needed to confirm diagnosis.


o treatment is most successful when started
early in infancy because delay causes muscles
and bones of legs to develop abnormally,
with shortening of tendons


gentle, manipulation of foot with casting
done every few days for 1 to 2 weeks then at 1- to 2-week
intervals
Ponseti’s Method of treatment


involves serial manipulation and plaster casting of the
clubfoot.
The ligaments and tendons of the foot are gently
stretched with weekly, gently manipulations.
A plaster cast is then applied after each weekly
sessions to retain the degree of correction obtained
and to soften the ligaments. Thereby, the displaced
bones are gradually brought into the correct
alignment.
Four to five long leg (from the toes to the hip) are
applied with the knee at a right angle.

LONG LEG CAST DENNIS BROWN SPLINT

http://www2.massgeneral.org/ORTHO/BabyCast.gif http://www2.massgeneral.org/ORTHO/DennisBrownBrace.gif


Making A Difference: Caring For Clubfoot at
the Sinai Hospital of Baltimore at
http://www.youtube.com/watch?v=Rmkrrvw
MH4A&feature=player_embedded#!


done if nonsurgical treatment not effective
tight ligaments released
tendons lengthened or transplanted
Other surgical treatments
- circumferential release: "cincinati incision"
- Goldner four quadrant approach:
- medial release
- posterior release
- posteromedial release
- tendon transfers

extended medical supervision is required
bcoz there is a tendency for this deformity to recur
(considered cured when the child is able to wear
normal shoes and walk properly)
care emphasizes muscle reeducation (by
manipulation) and proper walking


heels and soles of braces or shoes
prescribed following correction must be
kept in repair
corrective shoes may have sole and heel lifts
on lateral border to maintain proper
positioning


• Approximately 50-60% of club feet in newborns
can be corrected non-operatively.
• About 20% of infants requiring surgery need
further surgery at a later stage.


• imperfect development of hip –can affect
femoral head, acetabulum, or both
• head of femur does not lie deep enough within
the acetabulum and slips out on movement
• occurs in females 7 times more often than males


o acetabular dysplasia
mildest form
femoral head remains in acetabulum
o subluxation
most common form
femoral head partially displaced
o dislocation
femoral head not in contact with acetabulum
displaced posteriorly and superiorly


o limitation in abduction of leg on affected
side
o asymmetry of gluteal, popliteal, and thigh
folds
o Waddling gait and lordosis when child
begins to walk


With child in a
supine position,
the right knee
on the side of
the subluxation
appears lower
than the left
because of
malposition of
the femur head.


infant on a supine position.
Doctor abducts the hips by moving the bent
hips and knees apart.
If the hip feels like it can be pushed out the
back of the socket, this is considered
abnormal.
This is called a positive Barlow's Test and is a
sign of instability in the hip.


As the hip is abducted further, the doctor
might feel the ball portion (the femoral head)
slide forward as it slips back into the socket.
Or audible click when abducting and
externally rotating hip on affected side:


directed toward enlarging and deepening the
acetabulum by placing the head of femur within the
acetabulum and applying constant pressure
proper positioning: legs slightly flexed and abducted
Surgical Ix


o proper positioning: legs slightly flexed and
abducted
Pavlik harness
Frejka pillow: a pillow splint that maintains
abduction of legs
Bryant’s traction
Spica cast
Closed reduction


Hip abduction splint
holds the hips in an
abduction position,
forcing the femur
head into the
acetabulum.


http://www.orthopediatrics.com/binary/org/ORTHOPEDIATRICS/images/hi
pimages/child_hip_devel_dysp_treatment01.jpg


http://www.lpch.org/media/image
s/conditions/ei_0239.gif


A hip abduction cast for correction of
subluxation of the hip.


http://isakssonsgummifabrik.com/pics/babyfront.jpg


open reduction with casting
derotational osteotomy
Pelvic osteotomies


femur is cut and rotated to make it easier to
keep the femoral head inside the acetabulum.
When this procedure is done, the soft tissues
loosen up and the forces of the muscles tend to
keep the femoral head reduced.
Once again, the child is put in a spica cast for
several months while the bone heals.
A CT scan may be used to confirm successful
reduction before removing the cast.

for children older than 18 months which may require
additional surgery to change the acetabulum
(socket) in addition to the femur (thighbone)
The problem has been present longer and the anatomy has grown
more distorted over the longer period of time.


Several different types of osteotomies are used to
tilt the acetabulum in a more horizontal angle to the
floor. By doing this, the femoral head is less likely to
slide up and out of the socket with weightbearing.
Types : Steele osteotomy; Salter osteotomy;
Pemberton osteotomy


This can stop the femoral head from sliding
up and out of the socket.
Over time this shelf of bone above the
acetabulum remodels and forms a deeper
acetabulum.
the bone of the pelvis just above the
acetabulum is cut to allow the bone to slide
out and form a new roof over the hip joint.


uses a bone graft placed just above the hip
joint to create a new, wider roof, or shelf over
the acetabulum.
This keeps the femoral head from sliding up
and out of the socket and, as it heals, makes a
larger weightbearing surface to spread out
the weight that needs to be transferred from
the femoral head to the acetabulum and
pelvis.

not as common
the entire acetabulum is cut free of the pelvis
and moved or dialed at the best angle and
then allowed to heal in that position.


o Same with other clients with cast and
braces; pre- and post-op care
o Transportation and positioning
use wagon or stroller with back flat or mechanic’s
creeper
protect child from falling when positioned
never pick up child by the bar between the legs of
cast (use two people to provide adequate body
support if necessary)


A patient's guide to developmental dysplasia of the hip in children
retrieved on September 4, 2011 at
http://www.orthopediatrics.com/docs/Guides/dysplasia.html
Massachusets General Hospital. Pediatric orthopaedic ailments:
Clubfoot. Retrieved on September 4, 2011 at
http://www2.massgeneral.org/ORTHO/ClubFoot.htm
Saxton, Nugent, and Pelikan. (2006). Mosby’s comprehensive
review of nursing [18th ed]. St. Louis: Mosby
Talipes Equinovarus. Retrieved on September 4, 2011 at
http://www.patient.co.uk/doctor/Club-Foot.htm
Wheeless’ Textbook of Orthopaedics. Talipes
equinovarus/Clubfoot Retrieved on September 4, 2011 at
http://www.wheelessonline.com/ortho/talipes_equinovarus_clubf
oot


Congenital skeletal malformations

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