Congenital Dislocation of Radial Head Radial-Ulnar SynostosisRadial / Ulnar Club HandSyndactyly & Polydactyly

1. Congenital Dislocation of Radial Head
Radial-Ulnar Synostosis
Radial / Ulnar Club Hand
Syndactyly & Polydactyly
Presenter : Dr Yash Oza
Moderator : Dr Nitin Bhokare

2. • Congenital anomalies affect 6 per 10000 of newborns
• Out of which 75% have Upper extremity abnormalities.

3. Embryology of Upper Limb
• The upper and lower limbs begin
development in the 4th week of gestation.
• The limb buds are the precursor structures
of the limbs. Their formation begins in the
4th week.

4. • The limb buds first appear on the ventrolateral
body wall initially and extend ventrally. They
consist of a central core of undifferentiated
mesenchyme tipped with a layer of ectoderm,
the apical ectodermal ridge (AER).
• Elongation occurs through proliferation of the
underlying mesenchyme core

5. 1. AER – Apical Ectodermal ridge
• Acts as signaling center to guide underlying mesoderm to differentiate into
appropriate structure
• It is essential for proximo-distal limb development
• It is responsible for interdigital necrosis ,which separates the webbed hand.
2. ZPA - The zone of polarizing activity
• Signaling center for antero-posterior( Radio Ulnar) limb development
• Responsible for limb orientation
3. There is an another center called wingless-type (Wnt) signaling
center which is responsible for dorsalization of upper limb.
• All 3 function in coordination to ensure proper limb development.

6. 8 week after fertilization,
embryogenesis is complete &
all limb structures are present.
– majority of congenital
anomaly occurs during this
period.
Primary Ossification center are
present in all long bones by
12th week

7. Timeline :
Onset of development of arm bud :27 days 4th week
Well developed arm bud: 28-30 days
Elongation of arm bud: 34-36 days 5th week
Formation of hand paddle: 34-38 days
Onset of finger separation 38-40 days
full separation of fingers : 50-52 days 7th week

8. Classification of Congenital Anomalies of UL
• International federation of societies for surgery of the hand (IFSSH-1983)
1) FAILURE OF FORMATION OF PARTS:(Arrest of development)
a) transverse arrest : common levels are upper third of Forearm, wrist, metacarpal, phalangeal
b) Longitudinal arrest : phocomelia , club hand, cleft hand
2) FAILURE OF DIFFERENTIATION OF PARTS:
a) Soft tissue involvement
b) Skeletal involvement
Ex. radio ulnar synostosis ,camptodactyly ,arthrogryposis
3) DUPLICATION: polydactyly, mirror hand
4) OVERGROWTH: macrodactyly
5) UNDERGROWTH: thumb hypoplasia, madelungs deformity
6) CONGENITAL CONSTRICTION BAND SYNDROME
7) GENERALIZED ABNORMALITIES AND SYNDROME

9. Classification of Congenital Anomalies of UL
• International federation of societies for surgery of the hand (IFSSH-1983)
1) FAILURE OF FORMATION OF PARTS:(Arrest of development)
a) transverse arrest : common levels are upper third of Forearm, wrist, metacarpal, phalangeal
b) Longitudinal arrest : phocomelia , club hand, cleft hand

10. Classification of Congenital Anomalies of UL
• International federation of societies for surgery of the hand (IFSSH-1983)
2) FAILURE OF DIFFERENTIATION OF PARTS:
a) Soft tissue involvement
b) Skeletal involvement
Ex. radio ulnar synostosis ,camptodactyly ,arthrogryposis

11. Classification of Congenital Anomalies of UL
• International federation of societies for surgery of the hand (IFSSH-1983)
3) DUPLICATION: polydactyly, mirror hand
4) OVERGROWTH: macrodactyly

12. Classification of Congenital Anomalies of UL
• International federation of societies for surgery of the hand (IFSSH-1983)
5) UNDERGROWTH: thumb hypoplasia, madelung’s deformity
6) CONGENITAL CONSTRICTION BAND SYNDROME
7) GENERALIZED ABNORMALITIES AND SYNDROME

13. Club Hand
• Forearm dysplasia
• Longitudinal deficiency of Radius/Ulna resulting in
• deviation of wrist-hand towards Ulna/Radius
• Shortning of the forearm
• 2 types
1. Radial Club hand – d/t radial dysplasia
2. Ulnar Club Hand – d/t ulnar dysplasia

14. Radial Club Hand
• These includes all malformation with longitudinal failure of formation along the radial border of
upper limb.
• Incidence : 1 per 1 lakh ; 4.7 to 6.1% of all congenital anomalies
• Bilateral : 50% cases
• If Unilateral : Right > Left
• Male > Female
• Complete absence more common than partial absence
• May be associated with
• Absence/ deficiency of the thenar muscle
• Shortned/ absent thumb

15. Etiology
• In most of the cases the cause is unknown.
• These deformities are believed to occur sporadically.
• Although, Genetic and environmental factors have been suggested.
• Radial deficiency in association with fanconi anemia and
thrombocytopenia are are inherited as an autosomal recessive trial
• As a part of Holt oram syndrome in an autosomal dominant pattern.

16. RADIAL CLUB HAND CAN BE A PART OF SYNDROME:
1. Holt-Oram syndrome is an autosomal dominant disorder that includes an
absent radial bone in the arms, an atrial septal defect, and a first degree heart
block
2. The VACTERL association (also VATER association) refers to the non random co-
occurrence of birth
V- Vertebral anomalies
A-Anal atresia
C- Cardiovascular anomalies
T- Tracheoesophageal fistula
E - Esophageal atresia
R- Renal (Kidney) and/or radial anomalies
L- Limb defects
3. TAR Syndrome (thrombocytopenia with absent radius)
4. Fanconi anemia (FA) : congenital defects - ommonly short stature, abnormalities
of the skin, arms, head, eyes, kidneys, and ears, and developmental disabilities.

17. Clinical Presentation
• Usually Identified at birth by medical staff
• Come for Cosmetic problems
• Neglected patients: if deformity Is mild they
adapt to the deformity and do not seek
medical care
• Can present with other complaints which
may be a part of a syndrome like discussed
above

19. Examination finding
• deformity of hand with perpendicular
relationship between forearm and wrist .
• deformity is radial deviation of hand with a
short forearm
• prominent knob at distal end of ulna
• Thumb may be absent or severely deficient.
• Hand typically small
• Perform careful elbow examination: elbow
extension contractures common as a result of
week elbow flexors.

20. Xray Finding
• Partial or full radius may be absent
• Often thumb absent
• Bowing of ulna
• Carpal bone defects

21. Classification - HEIKEL CLASSIFICATION OF CLUB HAND
Type Distal Radius Proximal
Radius
Shortening Bowing of Ulna Fig
Short Distal
Radius
Present, but
delayed in
appearance
Slight Absent
Hypoplastic
Radius
Present, but
delayed in
appearance
Present, but
delayed in
appearance
Moderate Present
Partial
Absent
Radius
Absence of Prox / mid / distal
segment
Shortening
with carpal
radially
deviated
Present
Absent
Radius
Complete absence Shortening
with carpal
radially
deviated
Present

22. Modified Bayne & Klung Classification
Type Thumb Distal Radius Proximal Radius
N Hypoplasia/
Absent
Normal Normal
0 Hypoplasia/
Absent
Normal Normal/ R-U synostosis
/ D/L Radial Head
1 Hypoplasia/
Absent
>2mm shorter
than ulna
Normal/ R-U synostosis
/ D/L Radial Head
2 Hypoplasia/
Absent
Hypoplasia Hypoplasia
3 Hypoplasia/
Absent
Physis Absent Variable Hypoplasia
4 Hypoplasia/
Absent
Absent Absent

23. Management
• Type 1 and mild type 2: stretching and splinting
• Moderate type 2: radial lengthening
• Severe type 2: centralization of the carpus on the end of the ulna
• Type 3 and 4: centralization with tendon transfer

24. Conservative Management
• Immediately after birth, deformity often can be corrected passively and
early casting and splinting generally are recommended.
1. A light molded, short arm splint is applied along the radial side of
the forearm and is removed only for bathing and until the infant
begins to use the hands and then the splint are used only during
night.
2. Rioderm recommended application of a long arm corrective cast as
soon after birth as possible. The cast is applied in three stages
(similar to ponseti technique for club foot).the hand and wrist are
corrected first and last elbow is corrected as much as possible.
• There is no satisfactory conservative therapy for significant thumb
deformities associated with radial club hand.

25. Surgical Management
Plan for surgery
• Birth - 6 weeks : corrective casts
• 6 weeks - 3 months : alternate long arm cast
• 3-6 months: radial soft tissue release
• 1 year: first stage jess fixation and correction followed by
centralization of carpus on distal ulna with k wires.
• 1 to 3 years: centralization and subsequent tendon transfer
• 3-6 years: ulnar osteotomy and ulna lengthening
• 12-13 years: wrist stabilization by arthrodesis

26. Indications for surgery
• Without sufficient radial support
• With thumb and finger deformity
When contracture cannot be
corrected by plaster stretching
• Usually performed between 2-3
years of age
Contraindications for surgery
• Medical deficiency
• Associated anomalies not
compatible with long life
• Inadequate elbow flexion
• Mild deformity with adequate
radial support
• Older patients who have accepted
deformity and adjusted

27. PREOPERATIVE CORRECTIVE CAST
Start serial casting as soon as possible after birth
• Gently stretch the wrist as close to neutral as possible
• Apply an above elbow flexed cast in the maximally correct position
• Change cast weekly and Stretch the wrist further at each cast change
When the wrist reaches neutral apply a custom fabricated above
elbow splint and instruct the patient to stretch the wrist several times
each day Continue with splinting and stretching until centralization is
performed

28. External fixation & correction
• After application of Ex fix
• Start distraction on 4th post op day
• After correction, static phase for 3 weeks
• Removal of JESS followed by B/E POP for 2 weeks
• Followed by centralization surgery

30. CENTRALIZATION TECHNIQUES
• primary addresses radial angulatory deformity and may improve wrist
motion;
• this attempts to re-align the 3rd metacarpal at right angles to the
plane of the distal ulnar epiphyseal plate;
• in most cases centralization should be performed prior to one year of
age
• centralization may be performed along w/ radial soft tissue release
and as well as transfer of flexor and extensor carpi radialis tendons to
the ulnar side

31. CENTRALIZATION OF THE HAND USING
TRANSVERSE ULNAR INCISIONS
Manske et al. 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.

32. CENTRALIZATION OF THE HAND USING
TRANSVERSE ULNAR INCISIONS

33. CENTRALIZATION OF THE HAND WITH
REMOVAL OF THE DISTAL RADIAL ANLAGE
Watson et al. 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, K wire passed into ulna
to maintain position.

34. CENTRALIZATION OF THE HAND AND TENDON
TRANSFERS
Bora et al. centralization of hand and tendon
transfer.
A, Volar aspect of radial clubhand deformity
showing right-angle relationship of hand and
forearm and acute
angulation of extrinsic flexor tendons.
B, Volar aspect after centralization
and transfer of sublimis tendons of ring and
long
fingers.

35. CENTRALIZATION OF THE HAND WITH
REMOVAL OF THE DISTAL RADIAL ANLAGE
Watson et al. 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, K wire passed into ulna
to maintain position.

36. CENTRALIZATION WITH TRANSFER OF
THE FLEXOR CARPI ULNARIS
Bayne and Klug centralization of
radial clubhand.
A, Radial release and resection of
redundant soft tissue.
B, Centralization and pin fixation
with ulnar osteotomy.
C, Radial capsular release and
tendon transfer

37. CENTRALIZATION OF THE HAND WITH
REMOVAL OF THE DISTAL RADIAL ANLAGE
Buck-Gramcko centralization (radialization)
of
radial clubhand.
A, S-shaped incision.
B, Retrograde insertion of
Kirschner wire. ECR, extensor carpi radialis;
ECU, extensor carpi
ulnaris; FCR, flexor carpi radialis.

38. • Centralization is contraindicated in stiff elbow
• Late results shows variable success rates
• Wrist stiffness is another disadvantage ( as result of carpo-ulnar
fusion)

39. RADIALIZATION:
• the head of the ulna is brought under the radial carpal bones, and the
hand is fixed in ulnar deviation w/ a K wire;
• procedure includes release of tight radial soft tissue structures along
with possible tendon transfers to from the radial to ulnar side of the
wrist;
• worse results are seen with the more severe cases

40. ULNAR LENGTHENING FOR RADIAL DEFICITS
• may be considered following centralization if bowing is severe
• Wait for 5 to 7 days after first procedure before beginning to lengthen
ulna
• Then proceed at a rate of approximately 1 mm per day in three
increments
• Check the progress on radiograph and when lengthening begins can
be done weekly.
• Adjusting the lengthening rate depending on regeneration formation.
• The ulna can be usually be lengthened by 30 to 50%.
• remove the fixator and apply an above elbow cast

41. Ulnar Club Hand
• These includes all malformation with longitudinal failure of formation along the ulnar border of
upper limb.
• Incidence : 1 per 1 lakh ; 4.7 to 6.1% of all congenital anomalies
• Bilateral : 30% cases
• If Unilateral : Left > Right
• Male > Female
• Partial absence more common than complete absence
• May be associated with
• Anomalies of ulnar 2 digits ( 90% cases)
• Radial Head Dislocation ( 50% cases)
• Synostosis with humerus when part of ulna is present
• Radiohumeral synostosis

42. Etiology
• Cause is unknown
• Disruption of ZPA signaling (may have role)
• Most cases sporadic, except very few studies shows inheritance
• Ulnar deficiency occurs 1 week prior than in radial deficiency in embryonic
life
• Not associated with systemic disease like radial club hand
• But, may be associated with orthopedic problems
• PFFD
• Fibular deficiency
• Scoloiosis
• Phocomelia
• Multiple hand deformity

43. Clinical presentation
• Ulnar deviation of the hand
• Absent ulnar digits
• Syndactyly
• Elbow instability
• Elbow stiffness .
• Limited pronation, supination, or both
• Radial head subluxation or dislocation
• Deficient carpal bones
• Stable wrist
• Upper limb-length discrepancy

44. Classification
SWANSON'S CLASSIFICATION OF ULNAR
DEFICIENCY
• based on the embryologic origin and
morphological appearance

45. • Type 3- Complete
absence of the ulna
• Unstable elbow
• Straight radius
• Type 4 - Complete
absence of the ulna,
with a fibrocartilaginous
anlage attached to the
ulnar carpus
• Radiohumeral
synostosis at the elbow
• Bowed radius

46. • Type 1 - Ulnar shortening
(distally) with minor radial bow
• Hypoplasia of the ulna
• Proximal and distal epiphysis
present
• Hypoplastic or absent ulnar
digits
• Minimal radial bowing
• Type 2 - Significant ulnar
shortening with a
fibrocartilaginous anlage
attached to the ulnar carpus,
with significant radial bowing
• Partial aplasia of the ulna, distal
third
• Distal ulnar anlage
• Bowed radius with anlage acting
as a teacher
• Presence/absence of
progressive radial head
dislocation

47. Radiological findings
• 1) usually shows typical pattern of an absent distal ulna and a bowed
radius with an increased ulnar slope along its distal articular surface
• 2) check for the carpal bones: the pisiform and hamate usually are
absent. coalition of the other carpal bones are frequently present
• 3) thumb deformities
• 4) associated radial and hand deficiency
• 5) humeroradial synostosis

48. Treatment
For Ulnar deviation
Casting from birth
Long Arm cast
Gradual stretching of tight ulnar structures
(Mild cases will be corrected by 6 months age)

49. For Anlage
• Early presentation - Excise the anlage before the occurrence of radial
bowing o radial head dislocation, around age 6 months. The
procedure is performed through an ulnar incision along the wrist Care
is taken to protect the ulnar artery and nerve. Excision of the entire
anlage is not necessary; at least 50% should be excised. The wrist is
placed in maximal radial deviation in a long arm cast for a month.
Night splints are worn for up to 6 months to maintain the correction.
• Late presentation - Excise the entire anlage. Z-plasty of the skin and
selective tenotomies may be necessary.

50. • Radiohumeral synostosis - The anlage should be excised, as the
anlage tether can cause progressive bowing.
• Bowing of the radius - Radius corrective osteotomy,
• Forearm is in a less than useful rotation (it is better to wait until the child is
mature enough to make a decision about the appropriate position.)

51. • Radial head dislocation - Observe, resection only, or resection
with the creation of a single-bone forearm.
• Ulnar osteotomy has been performed with or without
shortening with open reduction and stabilization of the radial
head and plus/minus early radial head prosthetic
replacement.
• A single-bone forearm is created through a posterior
approach, protecting the posterior interosseous nerve. A
sufficient amount of proximal radius is excised to achieve
proper approximation and alignment with the ulna.
• An intramedullary nail or a plate is used for stabilizing the
fusion.

53. • Unstable elbow - Ulnohumeral or radiohumeral arthrodesis or elbow
ligamentous reconstruction
• Radiohumeral synostosis
• 6 Young children: Excise the ulnar anlage.
• Older children: Perform a distal humeral osteotomy to improve
forearm alignment.

54. outcome
• Most children learn to cope functionally with their condition.
• One of the common complications that is seen in the lengthening
reconstruction treatment ulnar deficiencies of the forearm is the
tightening of the flexor tendons to the digits, which restricts hand
function.
• Dislocation of an unstable elbow is also a potential complication. This
can be avoided by protecting the elbow with a hinged fixator.

55. Radio-Ulnar Synostosis
• It is osseous union between radius and ulna.
• Anomaly of longitudinal segmentation / failure of diffentiation
• 90% bilateral
• Male > female

56. Embryology
• At 35 days the elbow is 3 connected cartilaginous anale.
• Longitudinal segmentation produces 3 separate bones – humerus,
radius and ulna
• If this fails it leads to bony synostosis
• Most commonly related to fetal alcohol syndrome

57. Association
skeletal anomalies
• DDH
• club feet
• missing or diminutive thumb
• coalescence of carpal bones
• Symphalangism
• dislocation of radius
associated syndromes
• 1. Aperts 2. Williams 3. Klinefelter's 4.Poland’s
5. Acrocephalopolysyndactyly ( Carpenter’s syndrome)

58. Clinical Presentation
• Average age abnormality observed - 2.5 years
• functional complaints
• 1. difficulty holding or using small objects
• 2. inability to dress themselves
• 3. backhanded position in holding
• 4. difficulty feeding themselves
• 5. inability to accept small objects
• 6. difficulty competing objects sports involving upper extremities

59. Clinical Presentation
• average fixed flexion contracture of 16 degrees
• forearm shortening
• decreased carrying angle
• hypermobility of wrist
• fixed pronation range from 15 to 150 degrees. 40% have less than 30
degrees of fixe pronation and 60 more than 60 degrees.
• It is important to measure the angle of pronation to the plane of palm
as compensatory rotation through carpus is increase these patients

60. Radiological Finding
• spectrum of anatomic variation
• range of synostosis from proximal fibrous union to total synostosis of
radius and ulna
• absence of/or radial head deformity
• radial head dislocation
• forearm shortening
• varying continuity of cancellous bone throughout the coalition
• radial shaft bowing

61. Classification
• Type 1:
proximal or true radioulnar synostosis in were the radius and
ulna are smoothly fused at the proximal borders for a variable distance
• Type 2:
radioulnar synostosis with congenital dislocation of the head of
the radius in which the fusion is just distal to the proximal radial
epiphysis

62. Modified (Cleary et al)
• Type I : synostosis does not involve bone, associated with reduced radial head.
• Type ll : visible osseous synostosis, associated with normal reduced radial
head.
• Type III : visible osseous synostosis with a hypoplastic and posteriorly
dislocated radial head.
• Type IV : short osseous synostosis with an anteriorly dislocated mushroom
shaped radial head.

63. Management
• Observation
• If No functional deficits.
• fixed pronation of 15 degrees or less
Surgical correction.
• functional loss of forearm axial rotation.
• fixed pronation of greater than 60 degrees (particularly with bilateral
deformities).
• Aim- 20 degrees of pronation in unilateral cases
In bilateral dominant arm 20 degrees and neutral for non dominant

64. • Methods
• Taking down the synostosis and interposition of soft tissue
• poor results
• narrowing of interosseous membrane
• loss of correction with refusion

65. Kelikian/Doumanian swivel
• resection of a piece of proximal radius and insertion of swivel
• simultaneous transfer of FCR, FCU or EDU for active supination
• failure secondary to loosening of swivel, swivel failure

66. Derotational osteotomy distal to synostosis site
• rotation of forearm to improved position distal
to osteotomy
• fixation was with K-wires, Steinman pins or
staples
• failure from loss of rotation secondary to tight
interosseous membrane
• Significant risk of vascular compromise
secondary to functional shortening of arteries
forearm
• the further the osteotomy from synostosis the
greater the vascular risk

67. Derotational osteotomy through the synostosis
mass
• osteotomy through the synostosis with rotation
to more functional position
• position held with pins, external fixator or
plaster until synostosis reforms.
• usual loss of 15 degrees of original correction
• bilateral cases should have one hand in slight
pronation and the other in neutral position

68. Derotational osteotomy with the Ilizarov Method
• 1. osteotomy through the synostosis
• 2. low risk of vascular injury because of slow rotational correction

69. Congenital dislocation of radial head
• Dislocation of radial head may be congenital or secondary to the
diminished growth of ulna.
• Mostly posterior ( 2/3rd cases) ; others are anterior or lateral
• Often unilateral

70. Clinical presentation
• The abnormality is usually not detected at birth but is diagnosed later on in
childhood when the elbow is examined following come minor injury
• Usually the elbows are asymptomatic
• A complaint of stiffness may be reason of visit to physician in some cases
• The ulna is bowed, the direction of convexity depending on the type of
dislocation
• Anterior dislocation ulnar bow is forward
• Posterior dislocation-backward
• Lateral dislocations the ulna will bend laterally
• In anterior dislocation the ROM is limited and the radial head may be palpated in
the cubital fossa,
• in posterior dislocation the elbow will not extend fully and the prominent radial
head may be palpated posteriorly,

71. Radiological Findings
• Radiographs In a normal elbow a line drawn through the longitudinal
axis of the radial shaft bisects the capitellum of the humerus. This
normal finding is absent in this condition. The head of the radius is
dome shaped on its superior surface.

72. • It is important to distinguish traumatic from congenital dislocation.
The types of injury that cause traumatic dislocation of the radial head
are - missed Monteggia fracture dislocations, fracture of the radial
neck, pulled elbow, and occasionally a primary traumatic dislocation
of the radial head with other associated injury.
• In the newborn and infant, arthrography of the elbow is helpful in the
definitive diagnosis of radial head dislocation.

73. Treatment
1. When the diagnosis is made in the newborn or young infant, closed
reduction may be attempted
The posteriorly dislocated radial head is reduced by supination of the
forearm and extension of the elbow,
The anteriorly dislocated radial head is reduced by flexion of the
elbow.
• Reduction is maintained in an above elbow cast for four to six weeks.
Closed reduction is often unsuccessful .

74. Treatment
2. In children up to three years of age open reduction should be carried
out.
• In the older child it will be impossible to reduce the radial head.
3. The dislocation is left alone until late adolescence, when if symptoms
warrant, the radial head is excised.

75. Deformities of fingers
• Polydactyly
• Syndactyly

76. Polydactyly
• Duplication of fingers
• It is of 3 types
• Pre axial > duplication of thumb more common in whites.
• Central > duplication of index, middle and ring fingers
• Post axial > duplication of small finger - most common in African

77. PREAXIAL POLYDACTYLY : THUMB
DUPLICATION (BIFID THUMB)
• Epidemiology
• Most common duplication pattern in Whites and Asians
• 1 in 3,000 births
• Usually unilateral. Cause unknown.
• Sporadic, with systemic problems

78. Classification
Wassel classification
• Type I : Partial duplication of The distal phalanx and a common epiphysis
• Type II: Complete duplication including epiphysis of the distal phalanx.
• Type III : Duplication of distal phalanx and bifurcation of proximal phalanx.
• Type IV: Complete duplication of distal and proximal phalanx
• Type V : Complete duplication of distal and proximal phalanx with bifurcation of
the metacarpal
• Type VI : Complete duplication of distal and proximal phalanx and metacarpal.
• Type VII : Variable degree of duplication with triphalangeal thumb
Type IV is most common (47%) type VII (20%). type II (15%)

79. Classification
Wassel classification
• Type I : Partial duplication of The distal
phalanx and a common epiphysis
• Type II: Complete duplication including
epiphysis of the distal phalanx.
• Type III : Duplication of distal phalanx and
bifurcation of proximal phalanx.
• Type IV: Complete duplication of distal and
proximal phalanx

80. Classification
Wassel classification
• Type V : Complete duplication of distal and
proximal phalanx with bifurcation of the
metacarpal
• Type VI : Complete duplication of distal and
proximal phalanx and metacarpal.
• Type VII : Variable degree of duplication
with triphalangeal thumb
Type IV is most common (47%) type VII
(20%). type II (15%)

81. Changes in hand
• A Typically there is some degree of
hypoplasia of both duplicates and commonly
radial duplicate is more Hypoplastic.
• There may be convergence or divergence of
duplication
• Ulnar innervated intrinsic muscles to the
thumb (adductor policies, deep head of
nexor polices brevis), typically insert on the
ulnar most thumb duplicate

82. Changes in hand
• Median innervated intrinsic muscles to the thumb (abductor policies,
superficial higad of the flexor pollicis brevis and opponens pollicis)
insert on the radial most thumb duplicate
• Extrinsic flexor and extrinsic tendons may be duplicated and usually
are eccentric.
• Collateral ligaments of the duplicated joints are shared
• Both radial and ulnar neurovascular bundle to the digits may be
completely duplicated or may be shared with small separate branches
that supply the individual digits.

83. Treatment -Surgical
• goals of treatment
• to construct a thumb that is 80% of the size of the contralateral thumb
• resect smaller thumb (usually radial)
• preserve / reconstruct medial collateral structures in order to preserve pinch
function
• reconstruction of all components typically done in one procedure
• Timing of surgery 18 months but not later than 5 years

84. CORRECTION OF TYPES I AND II
BIFID THUMBS
• Bilhaut-Cloquet Technique
• technique
• involves removing central tissue and combining
both digits into one
• outcomes
• approximately 20% have late deformity
• problems include stiffness, angular and size
deformity, growth arrest, and nail deformities

85. CORRECTION OF TYPES I AND II
BIFID THUMBS
• Bilhaut-Cloquet Technique

86. CORRECTION OF TYPES III To VI BIFID THUMBS
• LAMB, MARKS, AND BAYNE
• indications
• usually favored approach for type III and IV
• type V and VI usually require more complex
transfer of intrinsics and collateral ligaments
• technique
• preserve skeleton and nail of one component
and augment with soft tissue from other digit
and ablation of lesser digit (radial digit most
commonly)

87. CORRECTION OF TYPE VII BIFID THUMBS
• In a Wassell type VII duplication (complete duplication), the radial
thumb should be removed. The remaining thumb may require web
reconstruction and metacarpal osteotomy to complete the
pollicization.

88. POSTAXIAL POLYDACTYLY:
• Duplication of the small finger
• It is the most common pattern of duplication in the black population
• Autosomal dominant inheritance

89. • Three types
• Type-1: Duplication of soft parts only
• Type -2: Partial duplication of the digit, including the osseous
structures
• Type-3: Duplication of the ray including the metacarpal

90. Treatment - Surgical
TYPE 1 : tie off in nursery or amputate before 1 year of age
TYPE 2 & 3
• operative
Excision of the extra digit
• technique
• preserve radial digit
• preserve or reconstruct collateral ligaments from ulnar digit remnant
• preserve muscles

91. CENTRAL POLYDACTYLY
• commonly associated with syndactyly
• Treatment
• osteotomy and ligament reconstructions
• Indications
• perform early to prevent angular growth deformities digit may lead to angular
deformity or impaired motion

92. SYNDACTYLY
• Most common congenital malformation of the limbs
• Epidemiology
• Incidence : 1 in 2,000 - 2,500 live births
• M > F
• BL in 50% of cases
• ray involvement >fig

93. Clinical Features
• Usually noted after birth
• In isolated - long / ring finger web space (57%) ringg / small finger
web space (27%) thumb index and index/long finger web space
• In syndromes - Thumb index and Index/long
• It is usually associated with symbrachydactyly, cleft hand,
synaptodactyly or amniotic disruption syndrome

94. Classification
Simple : only skin and salt tissue connections.
• Incomplete : web space extends to include the
finger tip (a)
• Complete : Web space occurs anywhere
between the normal commissure and the finger
lips (b)
Complex : Marked by skeletal anomaly (c)
Complicated : Accessory phalanges or
abnormal bones involved in fusion (d)

95. • The intervening skin usually is normal but
delicate in surface area
• The two nails may be completely separate or
the digits may share a common nail without
intervening eponychium
• Abnormally tight fascial bands usually are
present within the web
• There are anomalous sharings of
musculotendinous units, nerves and vessels
between joined digits.
• Complex pattern, may have interosseus
connections & Differentiation joints may be
incomplete

96. Treatment
• Timing of Surgery approximately 12 months of age.
• When it involves multiple web space - sequence and staging is required
• 1st procedure - thumb index and long/ring web space,
• 3 months later 2nd procedure - Index/long and ring/small.
• Only one side of the involved digit should be released at a time to avoid
vascular compromise of skin flaps or digits
• Technique includes:
• Commissure reconstruction
• Separation of digits.
• Resurfacing the digits

99. SYNDACTYLY RELEASE WITH DORSAL FLAP

100. SYNDACTYLY RELEASE WITH DORSAL FLAP

102. SYNDACTYLY RELEASE WITH MATCHING VOLAR
AND DORSAL PROXIMALLY BASED V-SHAPED FLAPS

103. Thank you