Radial Club Disease

Children born with wrist and forearms turning inwards could be having radial club hand deficiency.  Early correction of this hand deficiency is recommended and preferred for better outcomes.

Dr Ratnav Ratan offers to treat Radial Club Hand deficiency in newborn and growing children. Dr.Ratnav is one of the leading orthopaedist available in Gurgaon and NCR with expertise in handling bone and joint issues in children and sportspersons.

Incidence

Radial club hand is a rare form of abnormality. The incidence of radial club hand is 1 in 30,000 to 1 in 100,000 live births.

Associated syndromes of Radial Club Hand

Unilateral radial club hand is passed genetically and is a standalone condition. On the other hand, bilateral radial club hand is associated with other conditions or a part of the syndrome. It is a part of a collective syndrome especially Holt-Oram syndrome, TAR syndrome, Fanconi’s anaemia and VATER/VACTERL syndrome. It is likely that some of them have a genetic factor.

Radial Club Hand Deformity – Radial longitudinal deficiency

Radial Club Hand is a congenital condition that affects either one or both the forearms of the child. It also affects the soft tissues and flesh with an unbalanced arrangement of nerves and muscles, some of which could be missing.

It is also referred to as radial dysplasia or radius deficiency.

Anatomy of the hand w.r.t. radial club condition

The child’s forearm comprises of two major bones viz. ulna which is located on the outer side of the arm while the radius is located in the inner side of the arm. A child with radius deficiency will have a malformed radius and the wrist will bend inside i.e. towards the thumb side of the forearm.

Characteristics of Radial Club Hand

•          Shorter arm

•          Curved forearm

•          Stiff elbows and fingers

•          Small thumb or no thumb 

There are four types of Radial Club Hand

Type 1 – Short Radius

Least severe deficiency with the radius minimally short in length than the ulna.

Type 2 – Hypoplastic Radius

Moderately severe deficiency with the wrist bending inwards and ulna bowing outwards accompanied by underdeveloped thumb

Type 3 – Partial Absence of the Radius

Moderately severe deficiency with the extremely short radius in length, inward bending of wrist, and minimal support to the hand accompanied by underdeveloped thumb or its absence

Type 4 – Complete Absence of the Radius

Severe deficiency with the absence of the radius bone and extremely short forearm accompanied by underdeveloped thumb or its absence

Initial treatment of radial club hand at birth

After birth, a pediatric orthopedist suggests the corrective treatment approach. It is advisable to begin the corrective treatment shortly after birth. The treatment could be a combination of operative as well as non-operative procedures. 

Passive stretching of the tight radial structures is advised. The parents and the caregivers have to do this stretching every time the diaper is changed and also at bedtime.

Stretching of stiff elbow condition with limited mobility is also advised along with passive stretching. Serial casting is used to stretch the tight radials at a gradual pace.

The splint fabrication treatment is recommended when the forearm has grown in length to accommodate a splint.

Types of corrective surgeries

Ulnarization

Ulnarization is a surgical procedure performed to treat radial club hand or radial dysplasia. The orthopaedist makes a volar incision i.e. on the palm side to enable a proper view of the hand’s neurovascular structures and limit the dissection of the ulna. In this procedure, the head of the ulna i.e. one of the long bones of the forearm is converted into a fulcrum.  The transfer of the flexor carpi ulnaris (FCU), the flexible muscle of the forearm is done to balance the wrist. This transfer of the FCU to the top of the wrist enables the patient to experience stronger grip and better finger range of motion.

It focuses on giving a normal alignment to the wrist apart from correcting the deformity.  Ulnarization ensures no recurrence of the deformity as the ulna prevents the wrist to return to its deformed position.

Ulnarization is performed when the child in the age of twelve to eighteen months. It is also done when the child is in the age bracket of six to eight years.

Advantages of Ulnarization

The child can have a fully mobile wrist joint with no growth arrest or recurrences of the deformity. The other major advantages are

•          No or minimal complications

•          Better grip strength

•          Improved image of the hand

•          Fully aligned wrist

•          Improvement in performing daily tasks

•          Active wrist and flexion

Other corrective surgeries

Wrist surgery

Lengthening

Surgery is done to place a frame with pins on the hand which penetrate into the ulna and the hand. The pins are made apart gradually to stretch a tight wrist of the child and try to get the hands in a straight position.

Centralization

A surgery to reposition the wrist and enable the hand to rest in a straight position on the tip of the ulna. The entire correcting period could take up to a year or longer for the ulna to broaden and offer stability to the wrist.

Soft tissue centralization

This surgery improves the movement of the wrist by repositioning the skin, muscles, and tendons. Also, splinting is a requisite before and after a soft tissue centralization procedure.

Forearm surgery

Osteotomy is performed to reshape the forearm bones and reposition them in a straight line using pins. Once the bones are healed and in correct position, the

Thumb surgery

An opponensplasty is a surgical procedure done to reconstruct a thumb with mild deformity and strengthen its muscles. Also, a pollicisation procedure is recommended to correct a missing or an underdeveloped thumb. The surgeon may place the index finger in the thumb’s position.

Elbow surgery

This surgery is recommended to release soft tissues at the back of the stiff elbow. Elbow surgery can be done in combination with other corrective surgeries or a standalone procedure.  Elbow surgeries are rarely suggested or required.

What if the radial club hand deformity is not treated?

The child will display a decrease in functionality on account of the deformed state of the hand. Further, delay in corrective treatment makes its treatment even more challenging especially when the child grows into an adult.