Transcript LOWER EXTREMITY DEFORMATIONS

BIRTH DEFORMATIONS
INTRODUCTION
 Positional deformations: abnormalities
mechanically produced by alterations of the
normal fetal environment, which restrict
fetal movement and/or cause significant
fetal compression.
 Deformations of the extremities occur
frequently because fetal movement is
required for normal musculoskeletal
development.
Presentations: Deformations
 Craniofacial abnormalities
-scaphocephaly,
-plagiocephaly
-mandibular asymmetry, -flattened facies
-deviated nasal septum, -ear abn. & craniosynostosis
 Congenital musculartorticollis
 Congenital scoliosis
 DDH
 Lower extremity abnormalities (foot & leg)
-metatarsus adductus,
-internal tibial torsion
-positional calcaneovalgus feet-external tibial torsion
-positional clubfoot
-physiologic genuvarum
ETIOLOGY: DEFORMATIONS
-intrinsic factors: risk for other fetal abnormalities
-CNS disorder: primary neuromuscular Dz
resulting in decreased fetal movement.
-renal Dz resulting in decreased production of
amniotic fluid & oligohydramnios, which
increases the risk of fetal compression from
outside forces.
-extrinsic factors: generally otherwise healthy,
primarily due to factors that lead to fetal
crowding & restricted fetal movement.
Extrinsic Factors

Oligohydramnios: from leakage of amniotic fluid
Breech position: movement of the fetal legs are restricted due to
entrapment between the body of the fetus & the uterine wall,
the risk of deformations x10 folds.
 Abnormalities of the amniotic cavity:
- the presence of uterine tumors or deformities
(eg, bicornute uterus or septated uterus)
- Multiple fetuses or very large fetus

- Compression of the amniotic cavity affecting its size and shape
due to a small maternal pelvis and the size of neighboring
maternal organs
- During the last trimester the impact of external factors increases
as the fetus grows and the amniotic fluid decreases. As a result,
positional deformities are more common in term infants than PT
The assessment of deformities
in the NB
 a thorough PE: detect any
neurological or other musculoskeletal
abnormality that may have been an
intrinsic cause of the deformity.
Foot Bones
1.Fibula
2 Tibia
3.Tarsals
4.Metatarsals
5.Phalanges
Foot Bones
FOOT BONES:
1.Calcaneus
2.Talus
3.Navicular
4,5,6 = 3 Cuneiforms,
7.Cuboid,
8-12 = Metatarsal
13-17= Phalanges
(2sesamoid bones
underneath the head of 1st
metatarsal bone)
(total= 28 bones/foot)
LOWER EXTREMITY
DEFORMATIONS:
 Incidence= 4.2% (in 2,401 consecutive NB)
Common foot deformities:
-metatarsus adductus*: 76%, most common
-positional calcaneovalgus: 18%
-positional calcaneovarus (talipes equinovarus,clubfeet)
Common leg deformities:
-internal tibial torsion
-external tibial torsion
-physiologic genu varum: bow legs)
 >90% = normal feet, FU at 5-6 yrs. of age
1.Metatarsus adductus
PE: forefoot, adduction while the
hindfoot remains in a normal position, thus forming a
"C" shape (concavity of the medial aspect of the foot)
a deep medial crease generally present
-in infant~ the most common cause of in-toeing
-in walking child~ abnormal shoe wear
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Incidence: ~ 1-2 /1,000
similar
in PT & term infants
increased in
-twin
-F/H metatarsus adductus.
-in 1st born children (the increased molding
effect from the primigravida uterus and abd.wall)
Metatarsus adductus
 Two classification systems have been
used to evaluate the severity of this
condition
Evaluation of the severity:
2.Flexibility is determined
by whether adduction can
be corrected with
manipulation
 Two classification systems
have back
beento the
normal position.
used to evaluate
the severity of this
condition (show figure 1) [8] .
1.the heel bisector
no correlation with Px
Metatarsus adductus:severity
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1.the heel bisector. in which the severity of metatarsus
adductus is determined by the relationship of the toes to
the projected axis of the foot that bisects the heel and
normally extends through the second toe. The severity of
the condition increases as the heel axis moves more
laterally in relationship to the toes. However, the severity
based upon this classification schema does not correlate
with the prognosis.
2.the degree of flexibility of the forefoot. Flexibility is
based upon the ability to correct the metatarsus adduction
by providing lateral pressure on the forefoot over the first
metatarsal while firmly holding the heel in a neutral position
with the other hand. In infants with flexible metatarsus
adductus, spontaneous correction generally occurs.
Rx is reserved for those with rigid (severe) or moderately
inflexible metatarsus adductus.
Metatarsus adductus
 Recent data: no association between DDH
& metatarsus addusctus. (Hip exam. at
every WCC visit until 2 yrs. of age).
 X-rays:-generally not necessary.
-toddlers/older children with persistent
deformity to determine if another condition,
such as skewfoot (complex foot deformity:
medial deviation of the forefoot, lateral
translation of the midfoot & valgus hindfoot
Rx for metatarsus adductus
Over 90% resolve without Rx, ~ the severity&the flexibility
 Mild- can overcorrect into abduction with little effort..
Rx not necessary: spontaneously resolve over time,
Overcorrection with passive & active stretching may lead to
mild abduction, stretching exercise should not be performed
Moderate--will passively correct only to the neutral
position.
Passive stretching exercises & orthotic splint or corrective
recommended, unclear efficacy
Regular FU and casting if no improvement
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Severe or rigid- unable to be passively abducted to the
midline. In these cases, corrective casting is required.
Results are best with early Rx before 8 m. of age
Rx of metatarsus adductus
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Without Rx or non-surgical Rx: excellent results in all patients with mild
deformity without treatment and in 90% of the more severely affected
feet 31 patients (45 feet) with metatarsus adductus who were followed for a
mean of 32.5years. At initial presentation,12 patients (16 feet) with mild
deformity received no Rx, and 20 patients (29 feet) with moderate and
severe deformity were treated with serial manipulation and casting, one
patient had bilateral involvement with no treatment on one foot and
intervention on the other. No patient was treated with surgical correction.
Below-knee plaster casting: of 65 infants with moderate (37 feet) and
severe (48 feet) metatarsus adductus, corrected the deformity within 6-8
wk in all cases. At a mean FU of 4 yrs, correction was maintained in all
children with initial moderate deformity; and of the 44 feet with severe
deformity available for examination, 6 had a moderate deformity, 1 had a
severe deformity, and the remaining maintained the correction .
Surgical correction: controversial,may be an option for the older child
with persistent metatarsus adductus, however the reported incidences are
high for failure (~40%) & complications (~50%) . Complications include skin
slough and persistent pain with prominence of the tarsal metatarsal jts.
2.Positional calcaneovalgusfeet
 hyperdorsiflexion of the foot with the
abduction of the forefoot, which often results
in the forefoot resting on the anterior surface
of the lower leg.
External tibial torsion: a common asso.finding
 more common: in first-born children due to
the increased molding effects of the
primigravida uterus.
Positional calcaneovalgus
(talipes calcaneovalgus)
newborn infant at rest
Positional Calcaneovalgus feet
DDx:mandatory

DDx: more severe conditions
-Congenital vertical talus (rock bottom feet): rare condition, frequently
associated with neuromuscular & genetic disorders (eg, trisomy 13,14,15),
a fixed dislocation of the navicular dorsolaterally on the head of the talus.
The ability to correct (or partially correct) the deformity with gentle pressure
usually distinguishes the more flexible calcaneovalgus feet from the rigid
congenital vertical talus. X-rays, if PE is inconclusive.
-Paralytic calcaneus foot deformity is seen in infants with a neuromuscular
disorder (eg, myelodysplasia or polio), which results in the absence or
paralysis of the gastrocsoleus muscle. The PE to detect a motor deficit
should differentiate between paralytic & positional calcaneovalgus feet.
-Posteromedial bow of the tibia: both a calcaneovalgus foot & a bowed,
shortened tibia. PE: a leg-length discrepancy. Bowing of the tibia (X-rays)
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Rx: Most cases of positional calcaneovalgus feet: spontaneously resolve.
If the foot cannot be plantar-flexed below neutral, casting is indicated.
Surgical Rx is not required.
3. Positional “clubfoot”
(talipes equinovarus)
 involves both foot & lower extremity,
foot=excessively plantar flexed, with
forefoot swung medially and the
sole facing inward.
CLUB FEET:CLASSIFICATIONS
1.Congenital clubfoot:
the most common type, usually an isolated anomaly
2.Syndromic clubfoot: associated with intrinsic etiologies
of clubfeet: connective tissue, genetic, neuromuscular
disorder, or syndrome
3.Positional clubfoot: due to intrauterine crowding or
breech position, not a true club foot, as opposed to a
true clubfoot, it easily corrects to a normal position
with manipulation & will resolve over time.
Etiologies of clubfoot
Intrinsic
-Chromosomal: Trisomy18, Deletions of chromosomes 18q, 4p, 7q, 9q, 13q
-Connective tissue Arthrogryposis, Collagen defects, Joint synostosis
-Neurologic Anencephaly, Anterior motor horn cell deficiency,
Hydrancephaly, Holoprosencephaly, Myelomeningocele, Spina bifida
-Muscular MyopathyMyotonic dystrophy
-Skeletal dysplasia Campomelic dysplasia, Chondrodysplasia punctata
Diastrophic dysplasia, Ellis-van Creveld
-Syndromes Escobar syndrome, Hecht syndrome, Larsen syndrome,
Meckel-Gruber syndrome, Multiple pterygium, Pena Shokeir, Smith-LemliOpitz, Zellweger syndrome
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Extrinsic
-Amniotic bands or synechiae
-Early amniocentesis
-Intrauterine crowding
Fibroids, Multiple gestation, Oligohydramnios (Potter sequence)
-Malposition Breech
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ROTATIONAL DEFORMITIES OF THE
LEGS
 Rotational variations of the foot position to the leg
often occur in younger children due to in-uterine
positioning. In utero, the fetus's
 Hips: typically flexed, abducted, & externally rotated,
lower legs: internally rotated and the
knees: flexed. With intrauterine crowding and
mechanical restriction, this
position predisposes the fetus
to rotational deformities:
int. & ext. tibial torsion
and ext.rotation of the
hips, which may result in
physiologic genu varum.
4. Internal tibial torsion
-a normal rotational variant &
due to intrauterine positioning,
-the most common cause of in-toeing
-may also be associated with
metatarsus adductus & genuvarum
-2/3 = bilat.
-In unilat. cases: the lt. side is more
frequently affected.
TFA (The thigh-foot angle)
 the angular difference
between the axis of
the foot and thigh
when the patient is in
prone position with
knees flexed 90 degrees, &
foot & ankle neutral position
 The normal TFA = +10 to +15 degrees.
 Internal tibial torsion = negative TFA
Rx: Internal Tibial Torsion
 Spontaneous resolution of internal tibial torsion occurs
with ambulation and normal growth, so no
intervention is generally required. Improvement is
typically observed 6-12 mos. from the time the child
walks, and complete resolution is expected in most
children by 4 years of age.
 Surgical Rx is rarely indicated and is reserved for the
older child with marked functional or cosmetic
deformity (ie, thigh-foot angle is abnormally negative,
with values below -10degrees).
5. External tibial torsion
 -a normal rotational variant due to
intrauterine positioning
 -the most common cause of outtoeing in infants & young children
 -usually identified when the child
begins to walk.
 -often accompanied by positional
calcaneovalgus.
PE: External Tibial Torsion
The normal TFA= +10 to +15 degrees
.
External tibial torsion:
the TFA is abnormally positive
with values= +30 to +50 degrees
.
.
Rx: External Tibial Torsion
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This condition typically follows a similar course of internal tibial
torsion, with spontaneous resolution over time. However, disability
(eg, knee pain patellofemoral instability) is more common in
children with persistent external tibial torsion than in those with
internal tibial torsion. Increased external tibial torsion is also
associated with neuromuscular disorders including myelodysplasia
and polio. As a result, a careful neurologic examination should be
performed in patients with this finding.
As with other positional deformities, intervention is usually not
required as the majority of affected children will have spontaneous
resolution. Significant improvement does not occur until the child
has achieved independent ambulation. External tibial torsion is
more likely to persist through adolescence than internal tibial
torsion.
Surgical Rx is rarely indicated and is reserved for the older child
with marked functional or cosmetic deformity (ie, thigh-foot values
> +40 degrees).
6. Physiologic genu varum
(bowlegs)
 Common: due to intrauterine positioning, caused by a
combination of external rotation at the hip due to the
tight posterior hip capsule & internal tibial torsion,
typically identified in the older infant or toddler shortly
after the start of ambulation.
 Usually bilateral, the severity between the limbs may
vary. In-toeing is generally seen during ambulation. During
ambulation, the child may compensate for his in-toeing
by externally rotating the tibia to put the foot in a
neutral position. This causes further external rotation of
the femur and subsequent exaggeration of the deformity.
 Spontaneous resolution occurs between 18-22 mos. with
stretching of the hip capsule and resolution of the
internal tibial torsion.
Bowlegs:genu varum
A pathologic disorder: more likely if worsening during
infancy, unilateral, knee instability, or pain.
Pathologic causes: Blount's disease (tibia vara), rickets, and
fibrous dysplasia.
Hx, PE & full-length bilat. standing radiographs can DDx
physiologic vs. pathologic (requires Rx) genu varum
In Blount's dz, the most common
cause of pathologic genu varum,
the characteristic radiographic finding
is acute medial angulation (beaking)
of the proximal medial metaphysis
of the tibia.(may not be present until
2-3 y.of age.)
Genu valgum (Knock knees)
 Physiologic: self correction~ age4-6y.
 Intermalleolar distance:
normal = <2cm
severe = >10cm
 Pathologic:
-Metabolic bone dz (rickets, renal osteodystrophy)
-Skeletal dysplasia
-Post traumatic physeal arrest
-Tumor
-Infections: osteo.
Rx: Knock Knees
 Unlikely to correct if:
>15 cm & after 6 y. of age
 Surgical Rx in:
-skeletally immature= surgical Rx medial
tibial epiphyseal hemiepiphysiodesis
-skeletally mature = osteotomy at the
center of rotation of angulation & is
usually in the distal femur