This document discusses radial club hand, which is a congenital musculoskeletal anomaly caused by failed development along the radial border of the upper extremity. It presents the embryology, classification systems, clinical features, treatment recommendations, and surgical techniques for radial club hand. Specifically, it describes the deficient muscles, skeletal abnormalities including absent radius, neurovascular anomalies, and treatment approaches such as splinting, casting, tendon transfers, centralization of the carpus, and bilobed flaps procedures.

1. Radial Club Hand
Dr Aiman Ali
Resident Orthopaedic Surgeon – Year 5
Dept of Orthopaedics & Trauma
Khyber Teaching Hospital Peshawar

2. Radial Club Hand
• Introduction :
• Malformations with failure of longitudinal
formation of parts along the radial border of
upper extremity
• Deficient thenar muscles
• Short / Absent thumb
• Short or absent Radius

3. Overview
• Epidemiology:
• 1 per 50,000 live births
• Bilateral in 50% cases
• Etiology:
• Unknown
• Genetic / Environmental factors
• Thalidomide use
• Disrupted development along the radioulnar axis
• 1/3rd associated with syndrome and 2/3rd have associated medical /
musculoskeletal anomaly

4. Embryology
• Upper Limb Bud – 4th to 8th week
• Mesodermal cells covered by
ectoderm
• Under the guidance of three
signaling structures
1. AER (Apical Ectodermal Ridge):
Proximo-distal Growth
2. ZPA (Zone of Polarizing Activity):
AP Growth
3. NRE (Non-Ridge ectoderm): Dorso-
Ventral Growth

5. Swanson Classification

6. Transverse Deficiency
• Complete absence of parts distal to some point on the upper
extremity: Amputation Like Stumps.
• Classified by naming the level at which the remaining stump terminates
• Upper third of forearm is the most common level

7. Longitudinal Deficiency
• All failure of formation anomalies that are not considered transverse
• eg Phocomelia, Radial longitudinal dysplasia, Ulnar longitudinal dysplasia

8. Heikel’s classification of RLD
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

9. 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.

10. • 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.

11. 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.

12. 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.

13. Clinical Features:

14. • Deformity:
• Radial deviation of hand with short forearm,
present at birth
• Thumb may be absent or deficient
• Hand small
• MCP Joints with hyperextension & limited
flexion
• Flexion contractures of PIP
• Elbow extension contracture

15. Clinical Manifestation: Musculoskeletal &
Neurovascular Abnormalities
• Skeletal Abnormalities:
• Scapula, Clavicle & Humerus are often reduced in size
• Ulna may be short, curved or thickened
• Total absence of Radius more frequent, but in partial deficiencies, proximal
portion of radius is often present
• Scaphoid and Trapezium absent in more than 50% cases
• Thumb including its metacarpal & Phalanges absent in 80% cases
• Capitate, Hammate, Triquetrum & four ulnar metacarpals & phalanges are
present in almost all cases

16. • The muscular anatomy always is variably deficient
• Muscles that frequently normal are the triceps, ECU, EDM, lumbricals,
interossei (except first dorsal interossei), and hypothenar muscles
• The long head of the biceps is mostly absent, and short head is hypoplastic.
• The brachioradialis is absent in nearly 50% of patients.
• The ECRL & ECRB are frequently absent or may be fused with the EDC.
• PT either absent or rudimentary & inserted into intermuscular septum
• PL deficient
• FDS & FDP deficient
• PQ, EPL, APL, FPL are absent

17. 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

18. Neurovascular Abnormalities
• Anomalous Pattern:
• Median nerve thickened & it runs just beneath the fascia on pre-axial border
• Ulnar Nerve normal
• Musculocutaneous nerve absent
• Radial nerve ends at lateral epicondyle
• Normal Brachial & Ulnar artery But Absent Radial Artery

19. Associated Syndromes
25% cases of RLD are associated with Cardiac, Hematopoietic, GI , Renal syndromes

20. Treatment Recommendations
• 0-6 Months:
• Splinting above the elbow
• Serial Casting
• 6-18 months:
• Splinting is less tolerated in this age
• Stretching program
• 2-3 years:
• Splinting and stretching not effective
• Consider Operative treatment
• Adolescents:
• Wrist Fusion

21. • Begins at Birth
• Stretching exercises aimed at lengthening
the contracted tissues on radial side
• Splinting / Casting as an adjunct to
stretching
Non Operative Treatment:

23. Operative Treatment
• Surgery is usually postponed till 2-3 years.
• Indications: persistent wrist deformity that limits function
• Goal: Wrist stability ,wrist alignment & forearm length
• Options available:
• Centralization of the carpus on the forearm.
• Thumb reconstruction
• transfer of the triceps to restore elbow flexion
• Radialization: Aligning the Ulna with 2nd MC
• Bilobed Flap: Soft tissue releases, ulnar osteotomy & temporary longitudinal wiring
of carpus with distal end of ulna in corrected position

24. Operative treatment
• Type I & Type II:
• Usually don’t require surgery – Treated conservatively
• If conservative management fails:
• release of the tight radial soft tissues and tendon transfer to support the realigned
position.
• Lengthening of Radius : Through Osteotomy & Ex-Fix / IEF
• Type III & Type IV:
• Centralization

25. Centralization:
• Sharpening of distal ulna to fit into a
surgically created carpal notch

26. Technique:
• 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

27. • Complications:
• growth arrest of the distal ulna,
• ankylosis of the wrist,
• recurrent instability of the wrist,
• damage to neural structures (particularly the anomalous median nerve).
• Contra-indications:
• Stiff Elbow
• Ulnohumeral synostosis

28. Other Options:
• Radialization: Aligning the Ulna with 2nd MC
• Modification of centralization to reduce recurrence.
• Combined with pre-op soft tissue distraction
• Carpus fixed with distal ulna
• Radial side tendons are transferred ulnarly
• Ulna lengthened with IEF
• Pollicization
• Opponensplasty
• Bilobed Flaps procedure
• Vascularized Epiphyseal transfer:
• metatarsophalangeal (MTP) joint unit is
transferred from the foot to the radial side of the wrist via
microsurgical techniques

29. Bilobed Flaps

30. • Thank you