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"Congenital" means something about any individual which exists
before or from the time of their birth:-
What
are the different classifications of congenital hand deformities?
The classifications for hand deformities can vary. The classifications listed
below have been accepted by the American Society for Surgery of the Hand (ASSH).
Classifications may change as more knowledge is obtained regarding each of the
conditions. There are seven groups of deformities of the hand that will be
discussed, including the following:
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problems
in development of the parts
This occurs when parts of the body stop developing while the baby is in
the womb, causing either a complete absence of a part of the body, such as
the hand, or a missing structure, such as part of the arm bone. In the case
of the complete missing part, surgery is not indicated. These children may
be introduced to prosthetic devices early in their childhood. Types of this
classification include, but are not limited to:
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radial
clubhand
A radial clubhand is a deformity that involves all of the tissues on
the radial side (thumb side) of the forearm and hand. There may be
shortening of the bone, a small thumb, or absence of the thumb. This type
of birth deformity occurs in one out of every 55,000 births. Deformities
of the wrist are usually operated on around 6 months of age.
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ulnar
clubhand
An ulnar clubhand is less common than a radial clubhand. This
deformity may involve underdevelopment of the ulnar bone (the bone in the
forearm on the side of the little finger), or complete absence of the
bone.
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failure
of parts of the hand to separate
With this type of deformity, the parts of the hand, either the bones
and/or the tissues, fail to separate in the womb. The most common type of
this classification is syndactyly. Syndactyly is the most common congenital
hand deformity, in which two or more fingers are fused together. According
to some sources, this condition occurs in seven out of every 10,000 live
births. There is a familial tendency to develop this deformity. This
deformity usually involves both hands, and males are more often affected
than females. If the fingers are completely fused together, it is considered
complete. There are two types of syndactyly, including the following:
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simple
syndactyly
- involves fusion between only the tissues of the fingers.
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complex
syndactyly - involves fusion between the bones.
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Another
example of failure of the hand to separate is seen in contractures of the
hand. Contractures of the hand may also develop as a result of failure of the
cells to differentiate in utero. A contracture is an abnormal pulling forward
of the digits of the hand, usually caused by problems with the muscles or
skin. One of the common types of this classification includes congenital
triggering. Congenital triggering occurs when one of the digits is unable to
extend. It is usually seen in the thumb. It may take some time in the
child’s development before it is noted that the child can not extend the
thumb. About 33 percent of these cases improve on their own. Surgery is
usually not performed until the second year of life, but preferably before the
age of 3.
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duplications
of digits
Duplication of digits is also known as polydactyly. The little finger is the
finger that is most often affected. In the American-American population, the
incidence of duplication of digits is one in 300 live births. Thumb
duplications occur in .08 per 1,000 live births. There are three degrees of
polydactyly, including the following:
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type
1 polydactyly
- an extra digit is attached by skin and nerves.
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type
2 polydactyly
- an extra digit with normal parts is attached to the
bone or joint.
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type
3 polydactyly - an extra digit with normal parts is connected to an
extra normal metacarpal bone in the hand.
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undergrowth
of digits
Underdeveloped fingers or thumbs are associated with many congenital
hand deformities. Surgical treatment is not always required to correct these
deformities. Underdeveloped fingers may include the following:
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the
digit is small
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muscles
are missing
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bones
are underdeveloped or missing
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there
is complete absence of a digit
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overgrowth
of digits
Overgrowth of digits is also known a macrodactyly, which causes an
abnormally large digit. In this situation, the hand and the forearm may also
be involved. In this rare condition, all parts of the finger (or thumb) are
affected, however, in most cases, only one digit is involved (usually the
index finger). This condition is more commonly seen in males. Surgical
treatment of this condition is complex and the outcomes may be less than
desirable. Sometimes, amputation of the enlarged digit is recommended.
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congenital
constriction band syndrome
This occurs when a band forms around a digit (finger) or limb (arm),
causing problems that can affect blood flow and normal growth. Ring
constrictions are congenital (present at birth) and occur in one out of
every 15,000 live births. This condition may be associated with other
problems such as clubfoot, cleft lip, cleft palate, or other craniofacial
anomalies. The cause of the ring constrictions is unknown. Some theories
suggest that amniotic banding may lead to ring constrictions around a finger
or limb.
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With
this condition, there are four degrees of severity, including the following:
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simple
constrictions
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constrictions
that cause deformities beyond the constriction
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constrictions
that are associated with the fusion of parts of the finger
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constrictions
so severe that part of the finger will need to be amputated from the
process
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other
generalized problems with the skeletal system
These are a rare and complex group of problems.
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Treatment
for congenital hand
deformities:
Specific treatment for congenital hand deformities will be determined by your
child’s physician based on:
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your
child's age, overall health, and medical history
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extent
of the condition
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cause
of the condition
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your
child's tolerance for specific medications, procedures, or therapies
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expectations
for the course of the condition
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your
opinion or preference
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Treatment
may include:
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limb
manipulation and stretching
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splinting
of the affected limbs
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tendon
transfers
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external
appliances (to help realign misshapen digits or hands)
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physical
therapy (to help increase the strength and function of the hand)
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correction
of contractures
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skin
grafts - involves replacing or attaching skin to a part of the hand that is
missing skin or has been removed during a procedure.
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prosthetics
- may be used when surgery is not an option, or in addition to surgical
correction.
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Congenital
Hand disorders
CLASSIFICATION:
I. Failure of formation of parts (arrest of development)
A. Transverse deficiencies
1. Amputations: arm, forearm, wrist, hand, digits
B· Longitudinal deficiencies
i. Phocomelia: complete, proximal, distal
2· Radial deficiencies (radial
club hand)
3. Central deficiencies (cleft
hand)
4. Ulnar deficiencies (ulnar
club hand)
5. Hypoplastic digits
II. Failure of differentiation (separation) of parts
A. Synostosis: elbow, forearm, wrist, metacarpals, phalanges
B. Radial head dislocation
C. Symphalangism
D. Syndactyly
1. Simple
2. Complex
3. Associated syndrome
E. Contracture
i. Soft tissue
a) Arthrogryposis
b) Pterygium cubitaIe
c) Trigger digit
d) Absent extensor tendons
e) Hypoplastic
thumb
f) Thumb-clutched hand
g) Camptodactyly
h) Windblown hand
ii. Skeletal
a) Clinodactyly
b) Kirner's
deformity
c) Delta bone
childhood
Upper extremity problems
EMBRYOLOGY
of the Upper Limb
Weeks 4 to 6
| The critical APICAL ECTODERMAL RIDGE exerts an inductive influence on the
limb mesenchyme that promotes growth & development of the limbs.
Factors play a vital role in this process.
| | The upper extremity, with pronated forearms
begins to rotate externally.
| Developmental disturbances
during this period gives rise to major congenital malformations. Early
suppresion of limb development causes AMELIA (complete abscence of a limb);
Late suppression causes MEROMELIA (partial abscence). |
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Weeks 7 to Birth
| By week 7 the ten finger rays appear and
continue to differentiate till week 12 - 13 when the hands appear.
| The fingers develop as a result of cell death
in the tissue between the digits (failure of this causes syndactyly). |
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INTERNATIONAL CLASSIFICATION
(FDD.OUC)
| Failure of Formation
| Transverse Arrest / Aplasia
 | Amputations: arm, forearm, wrist,
hand, digits |
| Longitudinal Arrest
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| Failure of Differentiation
| A. Synostosis: elbow, forearm, wrist,
metacarpals, phalanges
| B. Radial head dislocation
| C. Symphalangism
| D. Syndactyly
| Simple
| Complex
| Associated syndrome |
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| E. Contracture
| i. Soft tissue
| ii. Skeletal
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| Duplication (polydactyly)
| Overgrowth
| Undergrowth
| Constriction
bands
| Generalised skeletal abnormalities
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SHOULDER
Sprengel Shoulder
| Associated with Klippel-Feil syndrome,
kidney disease, scoliosis, and diastematomyelia.
| Undescended scapula often associated with hypoplasia
of the affected side. Shoulder movements, especially abduction
may be restricted.
| Treatment only required if shoulder function
is impaired or the deformity is particularly unsightly. Surgery involves
releasing the vertebroscapular muscles from the spine, excising the
supraspinous part of the scapula along with the omovertebral bar. The
scapula is then repositioned by tightening the lower muscles (Woodward
Procedure). Surgery is best performed before the age of 6 years. |
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| Left Sprengel
Shoulder |
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Bilateral
Sprengel Shoulders |
Congenital pseudoarthrosis of the clavicle
| Caused by failure of union of the medial and
lateral ossification centres of the clavicle and may be related to pressure
from pulsations of the underlying subclavian artery. Every reported
unilateral case has been on the right side, except when dextrocardia
is present. |
| Surgical treatment only indicated for
unacceptable cosmetic deformity or functional symptoms such as mobility of
the fragments or winging of the scapula.
| Treatment consists of ORIF with bone
grafting. Union is often predictable unlike pseudoarthrosis of the
tibia. |
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Deltoid Fibrotic problems
| Short fibrous bands replace the deltoid muscle
causing abduction contractures at the shoulder with elevation and winging of
the scapula when the arms are adducted. |
ARM
| These include extra bones, absent bones,
hypoplastic bones and fusions. |
| Failure of Differentiation
| Failure of Formation - may be transverse
(Aplasia) or axial [Picture] |
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| Most of the defects are non-genetic but some
are of autosomal dominant inheritance. |
Amelia:
complete absence of a limb
Phocomelia:
almost complete absence (mere stub remaining) [Picture]
Ectromelia:
partial absence
Congenital
radio-ulnar synostosis 
| Union of the forearm bones usually proximally
placing the arm in a pronated position. Associated with DDH, CTEV,
chromosomal abnormalities and foetal alcohol syndrome. |
Two types
| 1 - Medullary canals are linked creating a
large radius with anterior bowing
| 2 - Proximal radial dislocation with less
extensive fusion (usually unilateral) |
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| Both types are difficult to treat. Osteotomies
for disabling pronation, but if bilateral leave the dominant arm and
osteotomise the other to 20-30° supination. |
Also see Elbow
Disorders
Congenital dislocation of the radial head
| Abnormally formed radial head on along radius
with a bowed ulna. Often associated with a soft tissue disorder. Capitellum
shape abnormal in congenital dislocation and normal in traumatic
dislocation. Radial head excision if pain develops otherwise leave alone. |
Congenital pseudoarthrosis of the forearm
| Rare disorder associated with
Neurofibromatosis. Refractory to treatment but vascularised bone grafts
may be useful. |
Congenital webbing of the elbow (Pterygium
cubitale)
| Broad skin web crossing the elbow causing a
flexion deformity and a pronated forearm. Surgery is difficult because it
sometimes requires vessel and nerve lengthening. |
Madelung’s deformity 
| Abnormal growth of the distal radial epiphysis
with premature fusion of the ulnar half of distal radial epiphysis.
| Can be post traumatic or congenital.
| First described by Madelung, who described a
painful wrist deformity in a young woman in 1878.
| Clinical - Presents in Adolescence; median
nerve irritation; wrist pain.
| Worsens with growth.
| May be associated with: Dysplasias, Turner
syndrome, Langer syndrome.
| Radiographic Findings:
| increased width between the distal radius
and ulna.
| relatively long ulna compared to radius
(positive ulnar variance).
| decreased carpal angle.
| triangularization of the distal radial
epiphysis.
| wedging of the carpus between the deformed
radius and the protruding ulna, with the lunate at the apex of the
wedge. |
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| Treatment includes ulnar shortening +/- dorsal
radial closing wedge osteotomy for severe cases
| See eHand
for Clinical Example |
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Radial Hemimelia
/ deficiency (Radial club hand)
| Absence or hypoplasia of pre-axial structures:
| radius
| radial carpus (scaphoid, trapezium,
trapezoid)
| Thumb - may be floppy (pouce flottante) or
absent |
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| May occur in association with visceral
anomalies (CVS/GI/GU), VATER or blood dyscrasias e.g. Fanconi’s syndrome.
| Most common in the right hand
| Bilateral in 50% of cases.
| Classified I-IV based on how much radius is
present |
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| Type |
Features |
Treatment |
| I |
Short |
address thumb |
| II |
hypoplastic |
individualised |
| III |
partially absent |
centralisation |
| IV |
totally absent |
centralisation |
| Treatment is difficult - mild cases -
strapping & manipulation; severe cases - correction of wrist deformity (Centralisation)
by fusing ulna to carpus or circular frame & provision of a thumb (Pollicisation)
using Buck-Gramko method (transfer, shortening & rotation of index
finger).
| Abnormal distribution of nerves & arteries
must be appreciated prior to surgery. |
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Ulnar Hemimelia
/ deficiency (post-axial)
| This is not associated with systemic disorders
as is the radial club hand but can be associated with other musculoskeletal
deformities, especially hand deformities.
| Only if function is severely disturbed should
wrist stabilisation be performed. |
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Reduplication of the
ulna
| Ulna and carpus are reduplicated leaving 7/8
digits and no thumb. Treatment includes removing most abnormal digits and
pollicisation to create a 5-digit hand. |
Central Deficiency (cleft
hand / lobster claw hand / ectrodactyly)
| Central abscense of at least one digit
| other digits may fuse (syndactyly)
| Typical Deformity = Autosomal Dominant; often
bilateral; more common in boys & may involve the feet
| Atypical Deformity = sporadic; associated with
cardiac & GIT abnormalities |
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HAND DEFORMITIES
| Goals of surgery are to preserve or improve
hand function and appearance. Timing of surgery should be immediate if
limb/digit threatening e.g. constriction bands. Within the first year if
disorder has a tethering growth effect e.g. club hand, before 3 if
development patterns are affected e.g. pollicisation and delayed until past
4 years if co-operation is required e.g. tendon transfers. |
Digits
Syndactyly (joined
phalanges)
| Can be Simple (skin only) or Complex
(bone involvement).
| may be Complete or Incomplete:
(complete= entire length of digit is affected)
| Incidence = 1:2000 births
| commonest congenital abnormality of the hand
| Can be associated with many anomalies.
| Apert
Syndrome = acrocephalosyndactyly
| Release performed 18 months-5years.
| PreOp Planning:
| individual Allen's tests to each involved
digit
| radiographs to rule out tethering of digits
| one side of digit should be operated on at a
time, in order to avoid vascular insufficiency
| with multiple syndactyly attention is first
directed to border digits
| the usual strategy is to preserve as much
regional skin as possible on the radial side of the involved digits (to
maximize pinch sensation) and saving FTSG for ulnar side of the affected
digit |
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| Clinical
Images (eHand) |
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Polydactyly
(duplicated digits)
Of three types
| Extra soft tissue only
| Bone, tendon and cartilage
| Completely developed with its own metacarpal
(rare)
| Clinical
Images |
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Thumb
Duplication (pre-axial polydactyly)
| Incidence = 1:1000 births
| Look for Cardiac anomilies & Fanconi
anaemia.
| Wassel
classification: seven subgroups depending on level of
bifurcation.
- Bifid distal phalanx
- Duplicated distal phalanx
- Bifid prox phalanx
- Duplicated prox phalanx
- Bifid metacarpal
- Duplicated metacarpal
- Triphalangism (single MC, dublicated PP
& DP + additional phalanx at end of one)
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Brachydactyly (short digits) / undergrowth
Macrodactyly
/ overgrowth
| Involves enlargement of all the structures,
especially the nerves of one or more digits. Can be associated with
Neurofibromatosis. |
Deviated digits
Clinodactyly
| Skeletal abnormality causes deviation in the
lateral plane. Usually involves the little finger and is caused by a trapezoidal
middle phalanx. Surgical correction for cosmesis only. |
Camptodactyly
| Familial soft tissue abnormality with
deviation in the sagittal plane. Commonly involves the little finger causing a
flexion contracture at the PIPJ.
| May be ass. with Dupuytren's, Marfan's,
arthrogryposis or other genetic syndromes.
| Few good surgical procedures exist.
| 2 stage correction (with ex-fix applied at the
time of FDS – extensor transfer has good results. |
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Kirner’s Deformity
| In-curling of little finger DIPJ in prepubertal
girls. Usually requires no treatment |
Symphalangism (Stiff PIPJ’s)
| Secondary to congenital ankylosis of the joint.
Associated with Apert
Syndrome. Observe only. |
Delta phalanx
| Triangular phalanx and physis, usually of the
thumb and little finger. A delta bone results in a sideways curve of the
finger. Surgery only if significant deformity. |
Thumb anomalies (Hypoplastic
thumb)
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Blauth Grade
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Description
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Treatment
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I
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Short thumb, hypoplastic thenar muscles
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Augment intrinsics
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II
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Grade I + Adducted MPJ
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Soft tissue Z-plasty
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III
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Deficient metacarpal
Abducted thumb
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Augment/ bone graft/pollicisation
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IV
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Floating thumb
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Pollicisation
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V
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Absent thumb
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Pollicisation
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Congenital trigger thumb
| Congenital stenosis at A1 pulley. Often
bilateral. 30% may resolve spontaneously by 1 year. Attempt splinting
but aim to correct surgically by 3 years. |
Constriction
band/ring (Streeter’s Dysplasia)
| Commonly involves digits or toes (especially
centrally) but can occur more proximally
| Associated with syndactyly, club feet and
neurological abnormalities. Treatment is by Z-plasty. |
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Congenital amputation
| Due to either constriction bands or failure of
development, most commonly very short below elbow amputation |
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BRTS