Radial dysplasia, also known as radial club hand or radial longitudinal deficiency, is a congenital difference occurring in a longitudinal direction resulting in radial deviation of the wrist and shortening of the forearm

1. RADIAL DYSPLASIA – Kids
Orthopedic

2. ABOUT THE DISEASE

3. Radial dysplasia, also known as radial
club hand or radial longitudinal
deficiency, is a congenital difference
occurring in a longitudinal direction
resulting in radial deviation of the wrist
and shortening of the forearm. It can
occur in different ways, from a minor
anomaly to complete absence of the
radius, radial side of the carpal bones
and thumb.

4. Hypoplasia of the distal humerus may be present as well and can lead to
stiffness of the elbow. Radial deviation of the wrist is caused by lack of
support to the carpus, the radial deviation may be reinforced if forearm
muscles are functioning poorly or have abnormal insertions.

5. The incidence is between 1:30,000 and 1:100,000 and it is more often a
sporadic mutation rather than an inherited condition. In case of an
inherited condition, several syndromes are known for an association with
radial dysplasia, such as the cardiovascular Holt-Oram syndrome, the
gastrointestinal VATER syndrome and the hematologic Fanconi anaemia
and TAR syndrome.
Other possible causes are an injury to the apical ectodermal ridge during
upper limb development, intrauterine compression, or maternal drug use
(thalidomide).

6. TYPES OF ABNORMALITIES

7. Clinical Features Of Radial Deficiency (Types II, III, And IV; See Presentation,
Classification) Are Dramatic, With Abnormalities Of The Entire Extremity.
The Scapula Is Often Small, And The Clavicle Is Often Shorter, With An
Increased Curvature. The Humerus May Or May Not Be Short, And
Deficiencies Of The Capitellum And Trochlea Are Common. Elbow Motion Is
Usually Diminished More In Flexion Than In Extension.
Bone And Joint Abnormalities

8. The forearm is always decreased in length, and the ulna is approximately
60% of the normal length at the time of birth. This discrepancy persists
throughout the growth period and into adulthood. True forearm rotation is
absent in patients with partial or complete aplasia of the radius.

9. Numerous muscular abnormalities are found throughout the upper
extremity. The deltoid or the pectoralis major can be hypoplastic, can be
partially absent, or can have an abnormal insertion. The biceps may be
absent or fused to the underlying brachialis.
Muscle and tendon abnormalities

10. The forearm demonstrates the most severe abnormalities, which may involve
any of the muscles that originate from or attach to the radius, including the
following:
 Extensor carpi radialis longus
 Extensor carpi radialis brevis
 Pronator teres
 Flexor carpi radialis
 Palmaris longus
 Flexor pollicis longus
 Pronator quadratus
 Supinator

11. The radial nerve usually terminates at the elbow, and the ulnar nerve is
normal. An enlarged median nerve substitutes for the absence of the radial
nerve and supplies a dorsal branch for dorsoradial sensibility. This
subcutaneous branch is positioned in the fold between the wrist and
forearm and must be protected during surgery.
The vascular anatomy demonstrates a normal brachial and ulnar artery. The
radial artery is often absent, and the interosseous arteries usually remain
patent.
Nerve and artery abnormalities

12. Radial deficiency is associated with numerous systemic conditions,
including Holt-Oram syndrome (cardiac septal defects); TAR syndrome;
Fanconi anemia (aplastic anemia); and VACTERL syndrome. In addition
to these conditions, a variety of associated musculoskeletal deformities
appear sporadically. These include cleft palate, clubfoot, kyphosis,
scoliosis, torticollis, and rib deformities.
Associated abnormalities

13. Classification of radial dysplasia is practised through different models. Some
only include the different deformities or absences of the radius, where
others also include anomalies of the thumb and carpal bones. The Bayne
and Klug classification discriminates four different types of radial dysplasia.
A fifth type was added by Goldfarb et al. describing a radial dysplasia with
the participation of the humerus. In this classification, only anomalies of the
radius and the humerus are taken into consideration.

14. James and colleagues expanded this classification by including deficiencies of
the carpal bones with a normal distal radius length as type 0 and isolated
thumb anomalies as type N.
 Type N: Isolated thumb anomaly
 Type 0: Deficiency of the carpal bones
 Type I: Short distal radius
 Type II: Hypoplastic radius in miniature
 Type III: Absent distal radius
 Type IV: Complete absent radius
 Type V: Complete absent radius and
manifestations in the proximal
humerus

15. Treatment

16. In cases of a minor deviation of the wrist, treatment by splinting and
stretching alone may be a sufficient approach in treating the radial
deviation in RD. Besides that, the parent can support this treatment by
performing passive exercises of the hand. This will help to stretch the
wrist and also possibly correct any extension contracture of the elbow.
Furthermore, splinting is used as a postoperative measure trying to
avoid a relapse of the radial deviation.
Splinting And Stretching

17. More severe types (Bayne type III en IV) of radial dysplasia can be treated
with surgical intervention. The main goal of centralization is to increase
hand function by positioning the hand over the distal ulna and stabilizing
the wrist in a straight position. Splinting or soft-tissue distraction may be
used preceding the centralization.
Centralization

18. In classic centralization, central portions of the carpus are removed to create
a notch for placement of the ulna. A different approach is to place the
metacarpal of the middle finger in line with the ulna with a fixation pin.

19. Buck-Gramcko described another operation technique, for treatment of
radial dysplasia, which is called radicalization. During realization the
metacarpal of the index finger is pinned onto the ulna and radial wrist
extensors are attached to the ulnar side of the wrist, causing overcorrection
or ulnar deviation. This overcorrection is believed to make relapse of radial
deviation less likely.
Radialization

20. The child has to perform stretching, splinting, and similar therapeutic
exercises for a specific period of time, if the doctor decides to treat his
radical club hand non-surgically. However, the orthopaedic must
examine the child’s hand consistently to assess the effectiveness of the
treatment plan.
Recovery

21. On the other hand, the recovery period will differ if the orthopaedic has to
perform surgery. Also, the surgery and post-operative care will vary
according to the type of radical club hand. However, the orthopaedic will
perform the surgery in phases and only after the child attains a specific
age.

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