Radial club hand

1. Radial club handRadial club hand
Zameer aliZameer ali

2. •Congenital malformations of the hand
encompass a myriad of deformities,
all of which carry different
functional and cosmetic implications
for the patient and parents

3. •Classification by Swanson, Barsky,
and Enti for congenital deformity of
hand

5. Failure of formation
(arrest of development)
•Longitudinal deficiencies
•Transverse deficiencies

6. •Transverse deficiencies include those
deformities in which there is
complete absence of parts distal to
some point on the upper extremity,
producing amputation-like stumps
that allow further classification by
naming the level at which the
remaining stump terminates

7. LONGITUDINAL
DEFICIENCIES
•Longitudinal deficiencies include all failure-of-
formation anomalies that are not considered
transverse deficiencies. e.g phocomelia, radial ray
dysplasia, ulnar ray dysplasia, and central
dysplasia.
• In the Iowa study these deformities constituted
9.3% of reported malformations, compared with
the 7.1% incidence of transverse deficiencies.

8. Radial club hand
•Radial ray deficiencies include all
malformations with longitudinal
failure of formation of parts along
the preaxial or radial border of the
upper extremity: deficient or absent
thenar muscles, a shortened,
unstable, or absent thumb, and a
shortened or absent radius,
commonly referred to as radial
clubhand

9. EPIDEMIOLOGY
•Primary insult is to the apical ectodermal ridge
during critical limb development period.
(between 4
th
and 7
th
weeks)
•Mostly due to environmental factors.
–Compression
–Inflammatory processes
–Nutritional deficiency
–Irradiation
–Infection
–Medications (especially thalidomide)

10. •Results from a radial ray deficiency during
embryological development.
•Incidence ranges from 1 in 30,000 to 1 in 100,000.
•Ranges from 4.7% to 6.1% of all congenital
anomalies.
•Slightly more common in males than females and in
caucasians.
•Bilateral in 38 to 50 percent of cases.
•When unilateral, occurs twice as frequent on right
side.

11. •Deformity is radial deviation of
hand with a short forearm (50-75%
the length of normal forearm).
•Almost always present at birth.
•Prominent knob at distal end of ulna.

12. •No single definite implicating factors.
•Genetic studies have failed to show any
genetic basis except when deformity is
associated with a syndromal picture as in
Holt-Oram, Fanconi’s, and TAR syndrome
•Interestingly, one study found that twice
as many affected patients born during
summer months than winter months.

13. •Thumb may be absent or
severely deficient.
•Hand typically small.

14. •MCP joints with limited
flexion and some
hyperextensibility.
•May be flexion
contractures of PIP
joints.
•Elbow extension
contracture common as a
result of weak or absent
elbow flexors.

16. •The obvious deformity of a short forearm
and radially deviated hand is almost
invariably present at birth
•These conditions may occur as isolated
deficiencies, but more commonly they
occur to some degree in association with
each other.
•Radial club hand occurs in an estimated 1
per 100,000 live births
•

17. •when the deformity is unilateral, the
right side is more commonly
affected.
•Both sexes are equally affected.
• Complete radial absence is more
common than partial absence.

18. •In most cases of radial clubhand the cause
is unknown, and the deformities are
believed to occur sporadically
•In a study Wynne-Davies and Lamb found
a higher proportion of a first-degree
relative with minor congenital anomalies
than would be expected from a random
survey, which suggests a genetic
contribution

19. •The currently accepted and most
useful classification of congenital
radial dysplasias is a modification of
that proposed by Heikel

20. Heikel classification
•In type I (short distal
radius) the distal
radial physis is
present but is delayed
in appearance, the
proximal radial physis
is normal, the radius is
only slightly
shortened, and the
ulna is not bowed.

21. •In type II
(hypoplastic radius)
both distal and
proximal radial physes
are present but are
delayed in appearance,
which results in
moderate shortening
of the radius and
thickening and bowing
of the ulna.

22. Type III deformity
•(partial absence of
the radius) may be
proximal, middle, or
distal, with absence of
the distal third being
most common; the
carpus usually is
radially deviated and
unsupported, and the
ulna is thickened and
bowed.

23. The type IV pattern
•(total absence of the
radius) is the most
common, with radial
deviation of the
carpus, palmar and
proximal subluxation,
frequent
pseudoarticulation
with the radial border
of the distal ulna, and
a shortened and
bowed ulna.

24. Heikel's classification of radial
dysplasia
•A,Type I—short distal radius.
•B, Type II—hypoplastic radius.
•C, Type III—partial absence of
radius.
•D, Type IV—total absence of radius.

26. •Variable degrees
of thumb
deficiencies are
frequent with all
patterns OF
RADIAL CLUB
HAND

27. ASSOCIATED
SYNDROMES
•. Associated cardiac, hemopoietic,
gastrointestinal, and renal
abnormalities occur in approximately
25% of patients with radial clubhand
and may pose significant morbidity
and mortality risks. The most
frequently associated syndromes are
Holt-Oram syndrome,

28. Xray

29. ASOCIATED
SYNDROMES
•Fanconi anemia, thrombocytopenia– absent
radius TAR syndrome,
•VATER syndrome, which consists of
vertebral segmentation deficiencies, anal
atresia, tracheoesophageal fistula,
esophageal atresia, renal abnormalities,
and radial ray deficiencies.

30. ASSOCIATED
SYNDROMES
•In the Holt-Oram syndrome the
cardiac abnormality (most commonly
an atrial septal defect) requires
surgical correction before any upper
limb reconstruction measure is taken

31. HOLT ORAM
SYNDROME
•Cardiac defects most frequently seen
are ASD, VSD, tetralogy of Fallot,
mitral valve prolapse, PDA, total
anomalous pulmonary venous return.
•Congenital heart defects required
for diagnosis.

32. Associated syndrome
•Children with Fanconi anemia, a
pancytopenia of early childhood, have a
very poor prognosis, and death usually
occurs 2 to 3 years after onset of the
disease..

33. •Autosomal dominant
•Thumb always present
•A progressive pancytopenia
•May not progress until mid-childhood.
•Prognosis is poor.

34. Associated syndrome
•In TAR syndrome
•thrombocytopenia usually resolves by
the age of 4 to 5 years and, although
it may delay reconstruction, but is
not a contraindication to surgical
treatment

35. TAR SYNDROME
•Thumb is always present and radial
deficiency is bilateral.
•Autosomal recessive mode of
inheritance.
•Typically, prognosis is good and
platelet count improves to normal by
age 4 to 5

36. •Always check platelet count in child
with Radial clubhand and a thumb
prior to entertaining surgery.

37. Associated syndrome
•Approximately half of these patients also
have cardiac defects. Successful
treatment of the associated abnormalities
usually is possible, and upper extremity
reconstruction may be appropriate in
selected patients.

38. Associated syndrome
•Radial deficiency also is associated
with trisomy 13 and trisomy 18; these
children have multiple congenital
defects and mental deficiency that
may make reconstruction
inappropriate despite significant
deformity

39. Anatomical abnormalities of
congenital absence of the
radius
•The scapula, clavicle, and humerus
often are reduced in size,
•the ulna is characteristically short,
thick, and curved, with any radial
remnant.

40. Anatomical abnormalities
•Total absence of the radius is most
frequent, but in partial deficiencies
the proximal end of the radius is
present most often.

41. Anatomical abnormalities
•The scaphoid and trapezium are absent in
more than half of these patients; the
lunate, trapezoid, and pisiform are
deficient in 10%;
• the thumb, including the metacarpal and
its phalanges, is absent in more than 80%,
although a rudimentary thumb is not
uncommon.

42. Anatomical abnormalities
•The capitate, hamate, triquetrum,
and the ulnar four metacarpals and
phalanges are the only bones of the
upper extremity that are present and
free from deficiencies in nearly all
patients.

43. Anatomical abnormalities
•The muscular anatomy always is deficient,
although the deficiencies are highly
variable. Muscles that frequently are
normal are the triceps, extensor carpi
ulnaris, extensor digiti quinti proprius,
lumbricals, interossei (except for the first
dorsal interossei), and hypothenar muscles.

44. Anatomical abnormalities
•The long head of the biceps is almost
always absent, and the short head is
hypoplastic. The brachialis often is
deficient or absent as well

45. Anatomical abnormalities
•The brachioradialis is absent in nearly
50% of patients. The extensors carpi
radialis longus and brevis frequently are
both absent or may be fused with the
extensor digitorum communis.

46. Anatomical abnormalities
•The pronator teres often is absent
or rudimentary, inserting into the
intermuscular septum, and the
palmaris longus often is defective.
The flexor digitorum superficialis
usually is present and is abnormal
more frequently than is the flexor
digitorum profundus

47. Anatomical abnormalities
•The pronator quadratus, extensor
pollicis longus, abductor pollicis
longus, and flexor pollicis longus
muscles usually are absent

48. IN SUMMARY
•Preaxial musculature from lateral
epicondyle most severely affected.
•Radial wrist extensors (ECRL, ECRB and BR
either absent or severely deficient.
•Finger extensors usually present.
•Long head of biceps almost always absent.
Short head typically hypoplastic.
•Brachialis deficient or absent

49. Neurovascular
abnormality
•. The peripheral nerves generally have an
anomalous pattern, with the median nerve
being the most clinically significant. The
nerve is thicker than normal and runs along
the preaxial border of the forearm just
beneath the fascia.

50. Neurovascular
abnormality
•This nerve is at considerable risk
during radial dissections because it is
quite superficial

51. Neurovascular
abnormality
•The ulnar nerve characteristically is
normal according to most authors,
and the musculocutaneous nerve
usually is absent. The vascular
anatomy usually is represented by a
normal brachial artery, a normal ulnar
artery, a well-developed common
interosseous artery, and an absent
radial artery.

52. •Median nerve thickened and runs just
below fascia. At risk for injury
during surgical dissection along
concavity of deformity.
•Radial nerve typically ends at lateral
epicondyle after innervating triceps.
•Ulnar nerve normal.
•MC nerve absent.

53. •. The forearm is between 50% and
75% of the length of the
contralateral forearm, a ratio that
usually remains the same throughout
periods of growth

54. •. The thumb characteristically is
absent or severely deficient;

55. •Flexion contractures often occur in
the proximal interphalangeal joints.
Stiffness of the elbow in extension,
probably the result of weak elbow
flexors, frequently is associated with
a radial clubhand

56. •Most authors emphasize the elbow
extension contracture as an
extremely important consideration in
evaluating these patients for
reconstruction. Because of the radial
deviation of the hand, the child
usually can reach the mouth without
elbow flexion.

57. •. Lamb found that unilateral
involvement did not significantly
affect the activities of daily living,
but bilateral involvement reduced
activities by one third.

58. •Associated cardiac or hematological
problems may worsen the overall
prognosis.

59. Management of radial
club hand
•Operative treatment
•Non operative treatment

60. Non operative Non operative
managementmanagement
Radial club handRadial club hand

61. •Immediately after birth the radial
clubhand often can be corrected
passively, and early casting and
splinting generally are recommended .

62. •All treatment should begin at birth and
should consist of manipulation and serial
splinting/casting into corrected position.
•Should be done with a well-padded long
arm cast with arm gently placed into
flexion and hand in maximal correctable
position.
•Cast on two weeks, then off with 1 week
stretching/manipulation, then new cast x 2
weeks.

63. •A light, molded plastic, short arm
splint is applied along the radial side
of the forearm and is removed only
for bathing until the infant begins to
use the hands; then the splint is worn
only during sleep.

65. •. Riordan recommended applying a long arm
corrective cast as soon after birth as
possible. The cast is applied in three
stages by means of a technique similar to
that used for clubfoot casting (ponsetti
casting) The hand and wrist are corrected
first, and then the elbow is corrected as
much as possible

66. •Milford concluded that casting and
splinting in a child younger than 3
months of age often is impractical.
•

67. •Lamb reported that elbow extension
contracture can be improved by
splinting the hand and wrist in
neutral position; 20 of his 27
patients improved to 90 degrees. He
also cautioned that elbow flexion
never improves after centralization
procedures.

68. •There is no satisfactory conservative
therapy for the significant thumb
deformities associated with radial
clubhand

69. Operative TreatmentOperative Treatment
Radial club handRadial club hand

70. Operative Treatment
•centralization of the carpus on the
forearm,
• thumb reconstruction,
• and occasionally transfer of the
triceps to restore elbow flexion

71. •Although surgery may be postponed for 2
to 3 years with adequate splinting, there is
general agreement favoring operative
correction at 3 to 6 months of age in
children with inadequate radial support of
the carpus.
•Pollicization, when indicated, follows at 9
to 12 months of age if possible.

72. • contraindications to operative treatment
include severe associated anomalies not
compatible with long life,
• inadequate elbow flexion,
• mild deformity with adequate radial
support (type I and some type II
deformities),
• older patients who have accepted the
deformities and have adjusted accordingly

73. Centralization of Hand
•Centralization of the hand over the
distal ulna was first reported in 1893
by Sayre, who suggested sharpening
the distal end of the ulna to fit into a
surgically created carpal notch.

74. Techniques
•Centralization arthroplasty
technique, transverse ulnar
approach.
•A, Incision.
•B, Exposure of muscle,
tendon, and nerve.
•C, Capsular incision.
•D, Exposure of carpoulnar
junction and excision of
segment of carpal bones.
•E, Insertion of Kirschner
wire.
•F, Reattachment of extensor
carpi ulnaris tendon.

75. Techniques
•Centralization of radial clubhand.
•A, Z-plasties on radial and ulnar
sides of wrist.
•B, Incisions allow lengthening on
radial side. Ulnar incision takes up
skin redundancy, transposing it to
deficient radial side.
•C, Radial incision in wrist for
identification of median nerve.
•D, View from ulnar incision across
wrist to radial incision after
resection of all nonessential central
structures.
•E, Distal ulna seen through radial
incision at wrist.
•F, Kirschner wire passed through
lunate, capitate, and long finger
metacarpal.
•G, After centralization, Kirschner
wire passed into ulna to maintain
position

76. Techniques
•Centralization of radial clubhand.
•A, Z-plasties on radial and ulnar
sides of wrist.
•B, Incisions allow lengthening on
radial side. Ulnar incision takes up
skin redundancy, transposing it to
deficient radial side.
•C, Radial incision in wrist for
identification of median nerve.
•D, View from ulnar incision across
wrist to radial incision after
resection of all nonessential central
structures.
•E, Distal ulna seen through radial
incision at wrist.
•F, Kirschner wire passed through
lunate, capitate, and long finger
metacarpal.
•G, After centralization, Kirschner
wire passed into ulna to maintain
position

77. •Lidge modified this method by
leaving the ulnar epiphysis intact,
providing the forerunner of modern
centralization techniques.
• Other procedures have been
performed in an attempt to stabilize
the hand on the forearm.

78. •Bardenheuer in 1894 suggested splitting the
distal ulna longitudinally to allow the carpus to
become wedged between the two halves.
•Albee in 1919 attempted to create a radius with a
free tibial graft.

79. •Starr in 1945 and Riordan in 1955
used a nonvascularized fibular graft
to support the carpus, but fibular
growth did not continue and the
deformity recurred
•. DeLorme in 1969 suggested
intramedullary fixation of the carpus
on the ulna.

80. •Centralization has been shown to improve
function, particularly in bilateral involvement.
Bora et al. reported total active digital motion of
54% of normal after surgery, compared with 27%
in untreated patients. Forearm length was
functionally doubled, and the metacarpal-ulnar
angle averaged 35 degrees after surgery,
compared with 100 degrees in untreated patients.
•Tsuyuguchi et al., however, reported that only 6
of their 12 patients were satisfied with the
results despite obvious functional gains

81. •. Bayne and Klug reported that 52 of 53 patients
believed that cosmesis and function had been
improved by centralization.
•Good results had the following factors in common:
(1) all had adequate preoperative soft tissue
stretching; (2) surgical goals were obtained; (3)
there were no problems with postoperative
bracing; (4) most had less severe soft tissue
contractures; and (5) most were younger than 3
years of age at the time of centralization.

82. Complications of
centralization include
•growth arrest of the distal ulna,
•ankylosis of the wrist,
• recurrent instability of the wrist,
• damage to neural structures (particularly
the anomalous median nerve),
• vascular insufficiency of the hand,
•wound infection, necrosis of wound
margins, fracture of the ulna, and pin
migration and breakage.
•Major neurovascular complications are
rare.

83. AFTERTREATMENT.
•The hand is elevated for 24 to 48 hours.
The dressing is changed and sutures are
removed 2 weeks after surgery. A long arm
cast is applied and worn for an additional 4
weeks. The Kirschner wire is removed at 6
weeks, and a short arm cast is applied to
be worn for an additional 3 weeks. Night
splinting is continued until physeal closure
to avoid recurrence of radial deviation.

84. Centralization of Hand
and Tendon Transfers
•Bora et al. suggested that treatment be
started immediately after birth with
corrective casts to stretch the radial side
of the wrist. When the patient is between
6 to 12 months old the hand is centralized
surgically over the distal end of the ulna,
and tendon transfers are carried out 6 to
12 months later

85. •) three tendon transfers are performed 6 to 12
months after the centralization procedure.
Before attempting to transfer the flexor
digitorum sublimis tendons, test for function,
because in some instances the sublimis tendon is
nonfunctioning in one or more of the three ulnar
digits. Passively maintain the metacarpophalangeal
joints and the wrist joint in hyperextension and
the interphalangeal joints in extension, and
release one finger at a time.

86. •AFTERTREATMENT. A cast is
applied after the procedure and is
worn for 1 month; after this a night
splint is worn for at least 3 months.
Careful follow-up should be made to
observe for possible recurrence of
deformity. A night splint can be used
for several years.

87. Pollicization for
Reconstruction of Thumb
with Radial Clubhand

88. •Although the thumb frequently is absent
or severely deficient in radial dysplasia,
children usually are able to adapt to the
thumbless hand with ulnar-side-of-index-
to-radial-side-of-middle finger prehension
and finger-to-palm prehension after
centralization. Despite this adaptability,
overall function and self-care activities
are impaired and can be improved with
successful pollicization.

89. •Because normal as well as compensatory
prehensile patterns are firmly established within
the first year of life, it is desirable that surgical
reconstruction be performed early. Pollicization is
recommended for both unilateral and bilateral
cases. If a "floating" thumb deformity is present,
with inadequate musculotendinous and bony
elements, the remnant should be amputated
before pollicization to allow reconstruction of a
stable thumb..

90. •Gosettin 1949 was the first to report
replacement of the thumb with the index
finger, and the index finger continues to
be the preferred donor digit if it is not
too deficient. Despite reports of
successful single-stage toe-to-hand
transfers, in the congenitally deficient
thumb the index is preferred because the
appearance is more acceptable and there is
less donor site morbidity

91. •Buck-Gramcko reported that results were
better when pollicization was performed in
the first year of life; his youngest patient
was 11 weeks old. Side-to-side grip
between index and middle fingers,
particularly for smaller objects, persisted
in children whose reconstruction was
performed later in life

92. •Buck-Gramcko reported that results
were better when pollicization was
performed in the first year of life;

93. •Side-to-side grip between index and
middle fingers, particularly for
smaller objects, persisted in children
whose reconstruction was performed
later in life.

94. •The index finger must be rotated 160 degrees
and placed in 40 degrees of palmar abduction for
optimal function and appearance. Hyperextension
instability at the index metacarpophalangeal joint
is prevented by positioning the metacarpal head in
70 to 80 degrees of hyperextension before
fixation. The reattached intrinsic muscles are
important in the function of the thumb and in the
formation of a new thenar eminence for cosmesis

96. AFTERTREATMENT.
•The hand is immobilized for 3 weeks,
and then careful active motion is
begun.

97. Opponensplasty

98. •Abductor digiti minimi opponensplasty, as
described by Huber, may be appropriate for the
rare patient with only isolated thenar aplasia in
association with the radial clubhand or for
patients with weakness in apposition after
pollicization. Manske and McCarroll reported
improvement in appearance, dexterity, strength,
and usefulness of the thumb in 20 of 21 patients
with an average age at operation of 4 years and 9
months.

100. Triceps Transfer to
Restore Elbow Flexion

101. •An elbow stiff in extension is a contraindication
to centralization; rarely, however, a child may
have passive elbow flexion but minimal or no
active flexion because of complete absence of
elbow flexors. Menelaus reported that triceps
transfer restored elbow flexion in two patients
when performed 2 to 3 months after
centralization; both patients improved from a
preoperative passive range of motion of 0 to 45
degrees to a postoperative active range of motion
of 0 to 90 degrees

102. SUMMARY
•Always be mindful of associated syndromal
patterns and concomitant medical problems when
approaching radial clubhand deformity.
•Set reasonable treatment goals and counsel
families on reasonable expectations.
•Know that elbow flexion typically does not
improve after surgery, so it is imperative to
address this pre-op in order to maintain ADLs.

103. Thank You…