Correction of forearm

deformities in congenital ulnar

club hand:
one bone forearm
Filippo M. Sns, Nunzio Catena
Microsurgery and Hand Surgery
Section, Orthopedics Unit, Gaslini
Childrens Hospital, Genoa, Italy

Ulnar club hand

Rare sporadic malformation, first description by Goller in
1698
Incidence: 1 new case every 300,000 live newborns
Partial or total absence of the ulna and of the ulnar rays
of the hand
Frequent association with muscoloscheletal anomalies
as lower limb deformities, scoliosis, mandible anomalies
Rarely associated with visceral anomalies
Goller DC: Abortus humani mostro. His Anta Misc Acad Nat Curios Norimb Decuria; 1698; 2:311
Blockey NJ, Lawne RJ. An unusual symmetrical distal limb deformities in siblings. J Bone Joint Surg 1963; 45-745
Langer LO Jr. Mesomelic dwarfism of hypoplastic ulna, fibula, mandible type. Radiology 1967; 89:654
Kuhne D, Lenz W, Peterson D, Schinenberg H. Defekt von femur und fibula mit amehe, phomelie oder ulnarer
stahldefekten der arme. Eir Syndrom Human Genetik 1967; 3:244
Makley JT, Heiple KG. Scoliosis associated with congenital deficiencies of the upper extremity. J Bone Joint Surg Am
1970; 52:279

Baynes classification (1982)

Hypoplasia of the ulna
Partial aplasia of the ulna with
bowing of the radius and
possible dislocation of the radial
head
Aplasia of the ulna with bowing
of the radius and dislocation of
the radial head
Aplasia of the ulna with fusion of
the radius with the humerus
Bayne LG: Ulnar club hand ulnar deficiency In Green DP: Operative Hand
Surgery. Vol. 1; New York Churchill Livingstone, 1982:245-257

Characteristics of deformities:

Ulnar anlage: Fibrous band attached to the

radius and ulnar portion of carpus

Cause of the bowing of the distal radius and

dislocation of radial head

Instability of forearm and elbow especially in

type II and III

Due to absence of the ulna, bowing of the radius

and dislocation of radiale head

Paradoxical hyperextension of the elbow (up to

+45)

Ogden JA, Watson HK, Bohne W. Ulnar dysmelia. J Bone Joint Surg Am 1976; Jun
58(4):467-475
Riodan DC. Congenital absence of radius or ulna. J Bone Joint Surg;1972;54B:381
Brody AS, Smith RJ. Deformities of the hand and wrist with ulnar deficiency. J Hand
Surg 1979; Jul 4(4): 304-315

Aim of the treatment

Creation of good stability for elbow and
forearm
Correction of the radius bowing and radial
head dislocation
Forearm lenghtening

The creation of one-bone forearm

allows the simultaneous
stabilization of forearm and elbow
1922: first description for post traumatic,
infective and neoplastic diseases
1952: Vitale described the first application in
children for ulnar diaphyseal defects
1965: Straub described the technique used
today for congenital ulnar club hand
Hey Groves E.W. On modern methods of treating fractures. Bristol John Wright
& Sons Ed. 2, 1922:320-323
Vitale C. Reconstructive surgery for defects in the shaft of the ulna in children.
J Bone Joint Surg Am 1952; 34:804-810
Straub LR. Congenital absence of the ulna. J Bone and Joint Sur Am 1965;
109:300-305

Indications

Bayne II grade
Elbow and forearm instability
Reduction of hand grasping
Anomalous forearm direction

Contraindications
Bayne III and IV grade
No patient and family compliance
Short proximal ulnar bone

Technique
dorsal access to the proximal
medium third of the forearm
preservation of the posterior
interosseous nerve
removal of the proximal
portion of the radius
fusion between the residual
proximal portion of the ulna
and the residual distal portion
of the radius
osteotomy is stabilized with
subcutaneous intramedullary
Kirschner wire
cast immobilization

Pearls
Cartilage and fibrous tissue must be
eliminated
Bone resection must be perfectly
congruent

Pitfalls
Risk of delayed consolidation, non union
or malunion in case of major age

Case series: 1996 2008

Gaslini Childrens Hospital
15 cases
Bayne I: 4
Bayne II: 9
Bayne III: 1
Bayne IV: 1
One bone forearm performed in 8 cases
Average age of 5.5 years (range 3-12 years)
All presented paradoxical hyperextension of the elbow
(average of +
37,5 - range + 25/ + 45)

Case series: 1996 2008

Gaslini Childrens Hospital
Associated musculoskeletal anomalies observed
Case 1
MP

Tranverse absence of forearm Hemimelia of the fibula

Case 2
MD

Cleft hand with bifid thumb

Case 3
RM

Absence of ring and small fingers - Hemimelia of the fibula

Surgical procedures preparatory to radio-ulnar fusion

Case 1 FN

Osteotomy of II metacarpal bone + Z plasty of first web

Case 2 EA

Ulnar anlage resection + Lenghtening of flexor ulnar carpi tendon by Z plasty

Case 3 MP

Ulnar anlage resection

Case 4
MD

Ulnar anlage resection + sindactyly division + IV/V metacarpal synostosis resection

Case 5 RF

Ulnar anlage resection+ sindactyly division

Case 6 RV

Ulnar anlage resection+ sindactyly division

Case 7 SN

Ulnar anlage resection

Case 8 ZN

Ulnar anlage resection

Results
Average follow-up was 5.3 years (range 1 to 13 years)
Union of the osteotomy was obtained in all cases
7 cases, union occurred in an average of 65 days (range 45-90
days)
1 case (surgery at 12 years of age) union was delayed, with
recovery after 5.5 months from surgery

After removal of the first plaster cast, kept on for an

average period of 35 days, a second cast was applied
(kept on for an average of 30 days) and subsequently an
orthopedic device was positioned (kept on at nights for
another 90 days)
Kirschner wires were removed on the average after
about 110 days from implantation
No infectious or vasculo-nervous complications or cases
of non-union occurred

Results
All cases showed improvement of forearm
functions and of grasping ability of the hand
(generally tridactyl)
The paradoxical
hyperextension disappeared
in all patients

Patient

Pre Op

Post Op

+ 45

II

+ 30

+5

III

+40

IV

+45

+5

+ 25

+5

VI

+ 45

VII

+ 35

+5

VIII

+ 35

In all the cases observed the formation of a

bone structure in continuity with the onebone forearm which reproduced the
morphology of the proximal metaepiphysis
of the radius without recreating a
radiohumeral joint surface

Additional surgical procedures

3 cases:resection of the newly formed radial
head due to its dislocation
1 case:one-bone lengthening with external
fixator, increasing forearm length by 3 cm
2 cases: bone lenghthening in progress

Case report n1

8 months

Case report n2

2 years

1 month

2 months

Case report n2

12 years

Case report n3




Case report n3

2 years

Conclusions
Early ulnar anlage resection
One-bone forearm procedure improves
malformed forearm functions
One-bone allows to have a larger and most
of all more stable substratus for future
procedures of skeletal lengthening
The regeneration of the proximal portion of
the removed radius should not be considered
as negative since it often contributes to a
greater elbow stability

Thank you for your attention

chirurgiamano@ospedale-gaslini.ge.it