Congenital Hand Differences
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1) Syndactyly
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Syndactyly is a condition in which children are born with fused or webbed fingers. About half of children with syndactyly have it in both hands (bilateral). Most of the time, syndactyly affects the fingers. Sometimes it affects the toes, but not as often.
While any two or more fingers can be joined, syndactyly most often occurs between the middle and ring fingers.
Symptoms of syndactyly differ depending on the type your child has. There are three types of syndactyly: Simple, complex, and complicated.
- Simple syndactyly means that the fingers are joined by skin and soft tissue only.
- Complex syndactyly means that the underlying bones are also joined together.
- Complicated syndactyly means that there are extra bones, and the tendons and ligaments have developed abnormally.
During pregnancy, a baby’s hands form in the shape of a paddle and later split into separate fingers. This happens very early, around the sixth to eighth week of pregnancy. Syndactyly happens if two or more fingers do not separate during this time.
Syndactyly is often diagnosed at birth. Sometimes it is detected even earlier, on a prenatal ultrasound.
Your baby’s doctor may use x-rays to assess the underlying structure of your baby’s fingers and determine a course of treatment. They may also evaluate your baby’s arms, shoulders, chest, feet, head, and face to look for signs of other abnormalities.
Syndactyly is treated with surgery to separate the joined fingers. Your child will probably have this operation when they are between 1 and 2 years old.
During surgery, the skin is split evenly between the two fingers. Your child may need a skin graft or a skin substitute to cover the newly separated fingers. Skin grafts are usually taken from the elbow or wrist crease to minimize scarring.
Your child will wear a cast or bandage covering their hand, lower arm, and elbow for two to three weeks. This will keep their hand still and protect the healing skin. Once the cast comes off, they will wear a splint to keep the fingers apart for six weeks.
Your child’s doctor may recommend occupational therapy to reduce scarring, manage stiffness and swelling, and improve function.
2) Brachydactyly
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Brachydactyly is a shortening of the fingers and toes due to unusually short bones. This is an inherited condition, and in most cases does not present any problems for the person who has it. There are different types of brachydactyly, based on which bones are shortened. This condition can also be a symptom of other genetic disorders.
The signs of brachydactyly are usually present at birth, but it’s possible that shortened limbs become more obvious with growth and development. The main symptom of brachydactyly is fingers, toes, or both that are shorter than normal. Unless you have another condition associated with brachydactyly, you should not feel any pain or have any other symptoms.
Brachydactyly is an inherited condition, which makes genetics the main cause. If you have shortened fingers or toes, other members of your family most likely also have the condition. It is an autosomal dominant condition, which means you only need one parent with the gene to inherit the condition. It’s thought that two different mutations in a certain gene contribute to brachydactyly.
In some cases, it’s possible that brachydactyly is caused by exposure to medications that the mother takes during pregnancy. It may also be caused by blood flow problems to the hand and feet, especially in developing babies.
The different types of brachydactyly are categorized by the bones and digits affected.
Type A
Type A brachydactyly is the shortening of the middle phalanges.These are as follows:
- Type A1: The middle phalanges of all the fingers are shortened.
- Type A2: The index finger and sometimes the little finger are shortened.
- Type A3: Only the little finger is shortened.
Type B
Type B brachydactyly affects the ends of the index through little fingers. The last bone on each finger is shortened or completely missing.
Type C
Type C is rare and affects the index, middle, and little fingers.
Type D
Type D brachydactyly is considered to be common and affects only the thumbs.
Type E
Type E brachydactyly is a rare form if it is not accompanied by another disorder. It is characterized by shortened metacarpals and metatarsals.
A careful examination of the hands and feet by a doctor may be enough to diagnose brachydactyly. X-rays can also be used to see which bones are shortened and to diagnose the type of brachydactyly. In mild cases, an X-ray may be the only way to tell that the condition is present.
In a large majority of cases of brachydactyly, no treatment is necessary. If your condition is not a part of another syndrome, you should be healthy and will have no medical concerns related to your hands and feet.
In extreme and very rare cases, surgery may be used to treat brachydactyly.
Plastic surgery may be used for cosmetic purposes, or in rare cases, to improve functionality. Many who need surgery will have brachydactyly along with another condition. Surgery may include an osteomy, which cuts the bone. This can contribute to “gradual lengthening”Trusted Source of the shortened fingers.
3) Clinodactyly
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Clinodactyly means that your child has a finger that curves to one side. It usually affects the little (pinky) finger but can affect other fingers as well. It is typically caused by abnormal growth and development of the small bones of the finger.
There are three bones in the finger. In many cases, children develop clinodactyly when the middle bone is shaped like a triangle. This misshapen bone is called a “delta phalanx.” A delta phalanx may have a curved growth plate or more than one growth plate. Therefore, as the hand grows, the finger becomes more curved.
Clinodactyly can be passed on from parent to child. It may also be part of a related condition, such as Down syndrome.
Your child’s doctor will diagnose clinodactyly after talking with you about your child’s medical history and doing a careful physical examination. Your child will also have an x-ray.
Treatment for clinodactyly depends on how curved your child’s finger is.
However, clinodactyly can increase as the hand and fingers grow. Therefore, it is important for your child to be followed closely by their doctor in case their finger becomes more curved over time.
If clinodactyly interferes with the use or movement of their hand, your child may need surgery.
4) Ulnar dimelia
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Ulnar dimelia or mirror hand syndrome is a rare congenital anomaly of the upper limb characterised by absence of the radial ray (including thumb), duplication of the ulna and duplication of the ulnar halves of the carpals, metacarpals and phalanges.
The embryology of mirror deformity was first described by Saunders and Gasseling, as ectopic expression of the zone of polarising activity cells or the sonic hedgehog gene which is responsible for limb patterning on an anteroposterior axis.
Hox genes which are responsible for positional information during embryogenesis is also considered to play secondary role. The aetiology suggested is probably failure of differentiation of a part of the ray rather than its pure duplication.
Al-Qattan and Al-Thunayan et al. have propesed a classification for mirror hand spectrum 5,6. The system divides the condition into five types as follows:
- type 1: there is ulnar dimelia showing two ulnae with absent radius
- type 2: an intermediate form having two ulnae and a radius
- type 3: also an intermediate form having mirror hand polydactyly with one radius and one ulna
- type 4: a syndromal mirror hand with fibular dimelia (Laurin Sandrow syndrome)
- type 5: multiple hands on the end of forearm
Ulna dimelia is treated with surgical reconstruction intervention7. Generally, it is recommended that the reconstruction surgery be performed before the age of two. It is useful for the parents to observe their child during playing to establish the best radial digit for pollicising.
5) Cleft Hand
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Cleft hand occurs when a child’s hand doesn’t develop properly during pregnancy. The condition can sometimes be detected on a routine prenatal ultrasound. After the baby is born, the deformity is visible.
Although cleft hand usually affects both hands, it can also occur in only one hand. Children born with cleft hand may also have cleft foot. All affected children, except those with very mild cases, need one or more surgeries, usually starting in their first year of life.
Not all cleft hands look the same. Typically, the center portion of the hand is missing a finger or fingers and there’s a V-shaped cleft in that space. Less often, clefts occur on the thumb side or the little finger side of the hand.
Cleft hand is congenital, meaning babies are born with the condition. It develops during pregnancy when the bones of the hand are forming. The exact cause is unknown, but scientists and doctors are learning more and more about the possible genetic causes (passed from parent to child).
Cleft hand is diagnosed by your child’s doctor after a careful physical examination. As part of the diagnosis, your child will have an x-ray to look for related problems with the bones of the fingers and hand.
Not all children need surgery for a cleft hand, particularly if the condition is not too severe and they have good use of their hand. However, if your child’s hand has significant functional or cosmetic problems, their doctor may recommend surgery.
There are many different surgical options to repair a cleft hand. The timing and sequence of procedures will vary from child to child. In general, the first procedure is usually done when a child is 1 year old.
After surgery, your child will wear a long-arm cast stabilized by pins for about four to six weeks.
6) Polydactyly
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In the past, small extra digits used to be “tied off.” This is no longer done because it causes pain for your child and will often leave a “bump.” An extra finger or toe still has a nerve, vein, and artery. When the extra digit is tied off, the body tries to regrow the nerve.
If this happens, a small bump can form where the extra digit was tied off. This is called a neuroma (noo ROW muh) (Picture 3). A neuroma can cause pain and a “stinging” sensation (similar to what you feel when your foot falls asleep).
It is best to have the extra toes or fingers surgically removed. If the extra digit is on the hand, the surgery is usually done before a child enters school since he or she will be using the hands more in school. If the extra digit is on the foot, surgery to remove it is done before your child starts to walk.
Call your child’s doctor if the extra finger or toe gets twisted and starts to turn blue or black. This sometimes will happen and means it may likely fall off on its own. Your child may experience some pain with this and will need some oral pain medicines.
Your child’s doctor may order an x-ray of the extra digit to help decide how to treat the polydactyly.
- Usually this procedure is done as an outpatient surgery. The child does not usually spend the night in the hospital.
- The surgery staff will call you the day before the procedure to give you instructions about what your child can eat and drink and tell you what time to arrive for surgery.
- Your child will have stitches to close the incision. Special glue will be put over the stitches. The stitches will dissolve and do not need to be removed.
- If your child has complex polydactyly, more extensive surgery is done.Complex polydactyly has a bone, tendon, and ligament in the extra digit. Your child may need a surgical pin in the hand or foot and may have to wear a cast.
7) Macrodactyly
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Macrodactyly is an uncommon condition in which a baby’s toes or fingers are abnormally large due to the overgrowth of the underlying bone and soft tissue. Macrodactyly is not cancerous. However, the larger fingers or toes (digits) may make it hard for your child to use the affected hand or foot for certain activities.
When macrodactyly is progressive, the affected digits grow faster than the rest of the hand. If your child has static macrodactyly, their affected fingers or toes will generally be about one-and-a-half times the length and width of the unaffected digits. If their condition is progressive, the involved fingers or toes can become enormous.
Macrodactyly can occur in association with other conditions and syndromes, including:
- neurofibromatosis
- vascular malformations
- multiple enchondromatosis
- tuberous sclerosis
- Maffucci syndrome
Your child’s doctor probably will see signs of macrodactyly soon after your child is born. However, if your child has the progressive kind, it may not be detected right away. Your child will have an x-ray and may have an MRI (magnetic resonance imaging) of their hand or foot to provide more information about the enlarged tissues.
The goal of treatment is to ensure your child’s hand has the most function and best appearance possible.
In mild cases, treatment may involve watching your child’s hand carefully over time.
In more severe cases, your child’s doctor may recommend surgery. Unfortunately, there is no simple procedure for thinning and shortening the affected fingers or toes since all parts of the digits (bones, tendons, nerves, blood vessels) are larger than normal.
8) Radial Club Hand
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Radial club hand is a congenital (present at birth) hand anomaly where the radius bone in the arm is missing or underdeveloped, causing the hand to be bent towards the body (radially deviated). It is also known as radial ray deficiency or anomaly. One in 75,000 children is born with radial club hand.This page explains about radial club hand, what causes it, how common it is, what functional problems your child might have and how it can be treated.
Radial club hand is classified into four types from mild to severe, but within each type the degree of severity can vary enormously. The four types are as follows:
- Type I – This is at the milder end of the spectrum, with the radius being only a little shorter than normal and the hand mildly radially deviated at the wrist.
- Type III – A large part of the radius is missing and the hand is moderately to severely radially deviated. The wrist lacks support and the ulna is thickened and often bowed. The forearm is short.
- Type IV – The radius is completely absent and the hand is severely radially deviated and the forearm is short. This is regarded as the most common type of radial club hand.
- In all types, the thumb may be smaller than usual (hypoplastic) or absent.
Your doctor will examine your child closely and look at x-rays of your child’s arm to work out the classification of radial club hand. This will influence the treatment options available.
The majority of cases appear ‘out of the blue’ (sporadically) with no known cause. Radial club hand can affect one arm (unilateral) or both arms (bilateral). The condition affects both arms in about half of children born with radial club hand. It occurs very early in pregnancy between day 28 and 52. In very rare circumstances, exposure to some substances during pregnancy, for instance, thalidomide, can cause problems with the radial bones. Radial club hand is only occasionally diagnosed during routine ultrasound scans before birth.
The level of hand function will depend on the severity of the radial club hand and whether one or both hands are affected. Radial club hand with wrist deviation and forearm shortening is often associated with thumb underdevelopment or absence, and stiffness of the finger joints with difficulty bending the joints. Corrective surgery aims to improve the appearance and function, but function is always affected for the child with a moderate to severe radial club hand. There will be reduced movement of the fingers with poor pinch and grasp, reduced strength and ability to position the hand, for example to turn the palm upwards.
There are various options for treatment and the doctor will explain which are most suitable for your child. This decision about suitable treatments is influenced by the severity of your child’s radial club hand and their general health including other symptoms.
Your child will be helped to maximise their functional potential through advice, therapeutic exercise and splinting. Non-surgical treatment with stretches and splints is usually required in all types of radial club hand.
A series of operations is often recommended to those children with type ll, III and IV radial club hand. Surgery tends to take place in phases. This enables your child to recover from one operation and post-operative care before moving on to the next. There are also some operations that are best done when a child is at a certain age. Your doctor may suggest the following operations:
This is an operation to remove the fixator and maintain the new position of the hand sitting on the end of the ulna bone. This will either be achieved by a tendon transfer for a radialisation operation where movement is preserved, or a tendon transfer and bone fixation for a centralisation operation, where most of the wrist movement is lost.
Most children with radial club hand have short forearms in the limbs affected. There are operations that can be used to lengthen the forearm but these are complex and rarely produce forearms of the same length. If surgery is possible, this tends to happen during mid to late adolescence.
9) Radioulnar Synostosis
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Radioulnar synostosis is a rare condition in which the two bones of the forearm the radius and the ulna are abnormally connected. This limits rotation of the arm. Radioulnar synostosis is usually congenital (something your child was born with). It can also occur as the result of a forearm fracture or trauma.
The information on this page focuses on congenital radioulnar synostosis.
Congenital radioulnar synostosis can be mild to severe. The telltale symptom is a limitation in your child’s ability to rotate their forearm from a palms-down (pronated) position to a palms-up (supinated) position. A child with radioulnar synostosis may also carry their elbow at an abnormal angle and their forearm may be short or bowed
In severe cases, the bones are connected and the forearm is fixed in one position, usually with the palm facing down to some degree. Depending on the position of the forearm and the flexibility of your child’s wrist, their activity may be limited in ways that are either subtle or noticeable.
A baby’s arms develop between the fifth and eighth week of pregnancy. In the early stages, the radius and ulna bones are connected. These bones later separate and become individual bones. If the radius and ulna do not fully separate, the baby will be born with radioulnar synostosis.
Most cases of congenital radioulnar synostosis happen for no known reason. About one in five children born with the condition have a family history of radioulnar synostosis. It can also be part of an underlying syndrome or developmental abnormality.
10) Scapholunate Ligament Injuries
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The scaphoid and lunate are two small bones in the wrist held tightly together by a band of tissue called the scapholunate ligament. When this ligament is injured the unstable wrist bones very gradually develop painful arthritis over months and years. This condition is usually seen in adults but can occasionally occur in younger people with severe wrist injuries like wrist dislocations. Scapholunate ligament injuries can eventually progress to a condition called SLAC wrist, which is a severe wrist osteoarthritis which is often painful and limits function.
Most scapholunate ligament injuries occur due to wrist trauma like falling on an outstretched hand, but occasionally no history of injury is recalled, or perhaps just a minor wrist sprain. Some cases of chronic scapholunate ligament injury may be due to repetitive heavy loading of the wrist as seen with obesity, or inflammatory conditions of the wrist that damage the ligament gradually over time.
- Pain (thumb side of the wrist)
- Swelling (back of wrist, and more on thumb side)
- Stiffness of the wrist
- Clicking, popping, grinding, or clunking sensations in the wrist
There is a wide range of treatment for scapholunate ligament injuries. Deciding which treatment is best for you is complex process and depends on many factors.
- Activity modifications
- NSAIDs
- Cast / Splinting
- Steroid injections
Complete tears of the scapholunate ligament due to recent wrist trauma are most likely to benefit from a surgical repair of the ligament. Once the tear is more the a few weeks old, the torn ligament becomes scarred and cannot be directly repaired. Reconstruction with tendon grafts can be considered but is not always successful. Once wrist arthritis is present, various reconstructive procedures can be attempted, or the wrist can be fused to help alleviate pain.
