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In general, there is rarely an indication for early surgery if
congenital anomalies of the upper extremity are noted at birth,
even though they can be quite distressing to the parents, who want
immediate answers and reassurance about their infant. Most care
for congenital conditions is nonoperative, and reconstructive procedures
are postponed until anesthetic risk is lessened after approximately
age 7 to 8 months of age. Larger size and maturation of airway and
critical organ systems makes surgical care more predictable.4
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Certain conditions necessitate a search for related conditions.
These include, in particular, the spectrum of “radial dysplasia.” Recognition of
a small thumb or other elements of radial dysplasia require evaluation
of the cardiac, spinal, renal, and hematological systems. The hand
anomaly may be the presenting finding in VACTERRL association, (Chapters 176 or 179)
Fanconi’s anemia, thrombocytopenia absent radius (TAR)
syndrome (see Chapter 176), and other conditions.
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Other congenital conditions such as syndactyly, polydactyly with
broad connection to the hand, and major morphologic limb malformation
can be referred to a hand surgeon at 3 to 6 months of age. The postaxial
polydactyly with a small, pedunculated base can be tied off or ligaclipped
by the primary pediatrician. If the base is too wide, surgical care
is best delayed until the child is 1 year of age.4
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Trigger thumbs and fingers are mislabeled as truly being congenital
because most occur in infancy and early childhood, and some will
spontaneously resolve. A trigger thumb appears as a flexed distal
joint of the thumb, usually locked in flexion. There is a characteristic
nodule at the flexor aspect of the metacarpophalangeal joint. There
is no need for radiographs or other imaging with this condition.
Treatment is by surgical release if the deformity is bothersome
and still present by age 3. Trigger fingers are more complicated
than trigger thumbs and require surgical care as well. There is
no place for corticosteroid injection in the treatment of trigger
thumbs or trigger fingers.
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Congenital contractures and angled digits should be referred
for diagnosis, splinting, and treatment. The hand deformity may
be the presenting complaint in a number of conditions, including
the myriad forms of arthrogryposis.
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Oligodactyly, or too few fingers, and missing parts of fingers
are common congenital upper limb malformations. Few of these are related
to amnion disruption sequence. The most common diagnosis is symbrachydactyly,
which has a broad spectrum of presentation. Other diagnoses include
ulnar dysplasia and clefting syndromes. Referral for diagnosis and
treatment can be delayed until after 3 to 6 months of age.
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Infants with enlarged parts of their hand or arm should be referred
early for evaluation and diagnosis. The differential diagnosis includes
tumorous conditions, malformations, and hamartomas, as well as the
various forms of gigantism and hemihypertrophy. Hemihypertrophy
has been reported to be associated with Wilms tumor, although this
is rare.
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Ganglion cysts are common benign masses found in children and
teenagers on flexor sheaths and in the wrist area. This type of
synovial cyst must be differentiated from a solid tumor. An otoscope
can be used to transilluminate the mass. If there is transillumination,
then the mass is likely a cyst. If transillumination is equivocal,
ultrasonography is the preferred imaging modality to confirm the
diagnosis. No treatment is usually needed. There is a very high spontaneous
resolution rate, and a very high recurrence rate if surgically excised,
so avoidance of surgery is usually recommended for this condition.
If the mass is solid, painful, or of great concern to the parents,
referral to the hand surgeon is appropriate.
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Inflammatory Conditions
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Juvenile chronic arthritis and other inflammatory and collagen
vascular conditions can occur in young children (Chapter 201). Psoriasis in particular can mimic chronic infection.
The pediatric rheumatologist should be involved in management of
children with these conditions.
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Madelung deformity is a progressive deformity of the wrist due
to abnormal growth of the ulnar and volar part of the distal radius.
It typically presents in adolescence and is more common in girls.
Recognition and surgical intervention before skeletal maturity may
prevent worsening of this deformity. Corrective procedures can restore alignment
in severe cases.
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Kirner deformity is a rare progressive hooking of the small fingernail.
It is sometimes painful and can be treated surgically if severe (Table 217-1).5,6
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