Dr. Netscher

Poland Syndrome

Tue, 06 Dec 2005 15:40:21 GMT

Poland syndrome (sequence) is a unilateral upper extremity condition that is generally not inherited. It occurs more frequently in males and more commonly affects the right upper extremity. There is frequently absence of the pectoralis major muscle and, in females, the breast on the affected side may fail to develop or may be hypoplastic. The fingers are often foreshortened and may not flex at the proximal interphalangeal joints (middle joints of the fingers). Frequently all the fingers are also webbed (syndactyly).

Hypoplastic Thumb

Mon, 21 Nov 2005 21:24:50 GMT

The milder forms of hypoplastic thumb can be reconstructed by means of tendon transfers to restore function to the thumb.

However, in the more severe forms of hypoplastic thumbs in which the thumb is either very rudimentary or sometimes totally absent, are best reconstructed by transposing the index finger into the thumb position and by a process called pollicization of the index finger. In this manner, the index finger is foreshortened and swung into the thumb position with the webspace being recreated by rotation flaps. This three-fingered hand, now with a normally positioned thumb, generally functions very well and has a natural appearance.

Also, a hypoplastic thumb and radial deficiency may occur with certain blood disorders. In the thrombocytopenia absent radius syndrome (TAR syndrome) there is a deficiency of platelets and a resulting blood coagulation disorder. The thumb is generally well formed, but the radius is absent. Fanconi's aplastic anemia can also occur in association with thumb and radius deficiencies. In this case, the blood abnormality may become manifest only some years after birth.

Treatment Options

Mon, 21 Nov 2005 21:06:50 GMT

If multiple fingers are affected, then the webspace releases may need to be performed in a series of staged procedures. Young children readily tolerate having surgery performed bilaterally and so this speeds up the overall recovery and reduces the number of anesthetic procedures required where there are multiple bilateral digits involved. Generally speaking, a zigzag separation of the digits is performed as these zigzag incisions are said to reduce the amount of scarring. Skin grafts are nearly always required to fill in some remaining gaps when the webspaces are released (except for the mildest forms of syndactyly). These skin grafts are most frequently obtained from the groin crease of the child and that site is sewn back as a straight line resulting in a concealed scar precisely in the flexion crease of the hip. In children of color, the skin taken as a graft from the hip crease area and may be too dark to provide a good color match for the naturally pale palm skin. And so, an...

Acrosyndactyly

Mon, 21 Nov 2005 20:35:05 GMT

A more complex type of syndactyly is termed acrosyndactyly.

This is a form of constriction band syndrome (sequence) in which the fingers were once completely separated but then a band formed around the fingers during embryonic development resulting in a re-fusion of adjacent digits. This is still a poorly understood phenomenon. Multiple fingers may sometimes be involved. A telltale appearance of this problem is an every present sinus (fenestration) at the base of the web fingers. Principles of separation of these acrosyndactylous fingers are the same as for other types of syndactyly. Sometimes these fingers may be foreshortened because the band that formed around the digits may have been so constricting as to reduce the blood supply to the growing fingers.

Case Studies

Mon, 21 Nov 2005 20:16:29 GMT

Case 1

This patient with YATER association had an absent radius and absent thumb. The severely deviated hand was initially centralized on the wrist.

The thumb was reconstructed by pollicization of the index finger, a procedure that moves the index finger into the thumb position and recesses the index finger to the normal length of a thumb.

Subsequently, the only fortam bone (ulna) was straightened and lengthened.

Case 2

This patient was born with polysyndactyly. The thumb spare was webbed and the index finger was "triplicated". The thumb webspace was first separated and then the polysyndactyly of the index finger was corrected.

Frequently Asked Questions

Wed, 16 Nov 2005 19:15:34 GMT

1. Is my child's congenital condition inherited?

Most congenital (born with) hand conditions are not inherited. In fact, only about 20% can be passed from generation to generation. These include certain types of cleft hand, same forms of little finger polydactyly, and Apert syndrome. The more common congenital hand conditions, such as thumb polydactyly, finger syndactyly, and hypoplastic/absent thumb are not inherited.

2. Did I do something during pregnancy that caused my child's hand condition?

About 20% of congenital hand problems can be traced back to an environmental factor during pregnancy. Such factors include viral illnesses and certain medications. Most congenital hand conditions are not caused by environmental conditions. However, the best advice is to avoid smoking, alcohol and non-essential medications during pregnancy, especially during the first trimester.

3. When is the best age to undergo surgical of my child's congenital hand condition?

This varies, especially if there are other problems that also need to be addressed, such as cardiac, gastrointestinal, and craniojavial. However, as a general rule, the more common conditions such as thumb polydactyly, webbed fingers (syndactyly), and absent thumb are probably best surgically somewhere between 10 months and 14 months of age.

Polydactyly

Wed, 02 Nov 2005 23:19:18 GMT

Polydactyly is probably the most common congenital hand anomaly. Polydactyly may affect both fingers and toes. On the hand polydactyly may be preaxial (affecting the thumb) or postaxial (affecting the little finger) and least commonly may involve the central portion of the hand.

Symbrachydactyly & Short Digits

Mon, 31 Oct 2005 19:51:52 GMT

The largest group of anomalies associated with shortened fingers is called symbrachydactyly. In this anomaly, the entire upper extremity may be foreshortened and somewhat smaller than the opposite side, but the fingers are the most severely affected. All of the digits may be affected or only the thumb. The most common type is the monodactylous type in which there is a deficiency of all the fingers except the thumb. These digits are often represented by small nubbins which have a tiny residual nail. Treatments: A variety of potential surgical options are available for identical digital amputations from the constriction band syndrome and for symbrachydactyly when multiple digits are hypoplastic. One might chose to ablate some of the shorter digital segments and transpose portions to adjacent digits in order to lengthen them. While this results in fewer than five digits in one hand, it positions those remaining digits for more functional grasping prehension, and augments the size of the...

Testimonials

Tue, 25 Oct 2005 21:29:49 GMT

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Treatment Options for Polydactyly

Tue, 25 Oct 2005 21:28:14 GMT

The reconstruction does not simply involve an ablation of the smaller duplication portion. The shared articular surface must be reshaped, the bones realigned, and the collateral ligaments of the joint must be reconstructed to ensure the stability of that joint, and tendons must be realigned. If these general principles are not adhered to, the thumb will otherwise have an unstable metacarpal phalangeal joint, and significant residual deformity, and major functional problems. On occasion, because of joint surface malalignment (particularly when the duplication involves a shared joint in addition to the ligament and tendon readjustments) a corrective bony osteotomy may be considered. This procedure involves actually tacking the bone and resetting its angle and may be required at the initial correction of the polydactyly. This is done to avoid any long-term misalignment of the reconstructed thumb and to minimize the risk of a residual zigzag unstable deformity to that reconstruction. While it is important to meet with the child at a young age, within a month or two of birth, the actual reconstructive surgery for polydactyly (digital duplication) will generally be performed somewhere around 9 or 10 months of age.

Syndactyly Cases

Tue, 25 Oct 2005 21:26:32 GMT

Syndactyly may occur as an isolated birth problem and will generally involve the single webspace between the middle and ring fingers, although other fingers may be affected. Some cases of bilateral symmetric anomalies may be inherited. Certain syndromes may also be associated with syndactyly— the most common of which is Poland syndrome (sequence) in which foreshortened fingers are conjoined or Apert syndrome where there are bilaterally symmetric complex webbing of the fingers and toes. Poland syndrome is a unilateral upper extremity problem that is often associated with absent chest muscles, especially the pectoral muscles. Apert syndrome is an inherited anomaly that is associated with characteristic craniofacial anomalies that frequently also requires surgical correction. It is always best to seek hand surgery consultation for any of these problems involving hand or feet syndactyly as soon as possible so that questions can be answered, anxieties allayed, and a check can be made for other anomalies that may occasionally be present. However, in most circumstances, surgery is not usually started before the child is at least six month of age.

Common Causes for Polydactyly

Tue, 25 Oct 2005 20:40:29 GMT

Most cases of thumb duplication are not inherited. However, little finger polydactyly, especially in people of color, is often inherited as an autosomal dominant trait.

Polydactyly Cases

Tue, 25 Oct 2005 20:39:33 GMT

The most common type of thumb polydactyly is the Wassel type IV anomaly where the duplication occurs at the level of the metacarpal phalangeal joint. Wassel classified thumb duplication based on the level of the duplicated elements. There is thus a shared joint.

Short & Absent Digits

Tue, 25 Oct 2005 20:37:15 GMT

Short or absent digits may have several possible causes. A thumb may be hypoplastic in that it is smaller than the opposite side thumb and has deficient muscles and ligament instability. In an extreme situation, a thumb may be totally absent. An absent thumb may occur in isolation as a congenital hand anomaly, or it may occur in association with radial dysplasia (formerly called radial clap hand). Radial dysplasia with absent thumb may be part of the VATER association of anomalies to include vertebral, anorectal atresia, tracheoesophageal fistula, and renal abnormalities.

Cleft Hand

Tue, 25 Oct 2005 20:34:05 GMT

Also, absent fingers may occur as the result of failure of an entire longitudinal portion of the hand and finger to develop. This results in a cleft hand. In some pages, the cleft hand may occur bilaterally and my also involve the feet, with a familial incident caused as the result of dominant genetic inheritance (Figure 73). Such a condition used to go by the name of lobster claw hand. The most common surgical treatment for correction of the cleft hand is to close down the cleft space, and transpose the flap of skin from the closed down space across into the thumb webspace to widen than interspace. Once again, the goal of such a reconstruction is to make the hand more functional for grasping and also of course to improve the appearance.

Syndactyly

Tue, 25 Oct 2005 19:47:23 GMT

Syndactyly or webbed fingers result from an embryonic failure of separation of the developing finger. It may involve only two adjacent digits or multiple fingers may be affected. It can be relatively simple involving a skin bridge only between two fingers or it may be a more complex problem with the bones of adjacent fingers being joined as well. Nerves and arteries, and even tendons to the adjacent involved fingers may also sometimes be shared in a complex manner. It may involve hands and/or feet.

What Is Polydactyly

Tue, 25 Oct 2005 19:06:50 GMT

Polydactyly is probably the most common congenital hand anomaly.

Common Causes
Most cases of thumb duplication are not inherited.

Polydactyly Treatment
The actual reconstructive surgery for polydactyly (digital duplication) will generally be performed somewhere around 9 or 10 months of age.

Learning About Syndactyly

Wed, 05 Oct 2005 14:46:06 GMT

Syndactyly Cases
Syndactyly may occur as an isolated birth problem...

Acrosyndactyly
The fingers were once completely separated, but then a band formed around the fingers...

Syndactyly Treatment
Syndactyly or webbed fingers result from an embryonic failure of separation of the developing finger...

About Hand Surgeon, Dr. Netscher

Wed, 28 Sep 2005 18:06:41 GMT

Houston hand surgeon Dr. Netscher works on deformities, as well as nerve and musculoskeletal injuries of the hand. He has a history of having excellent results which is dependent on the efforts of the physician-patient relationship. Learn More

More About Congenital Hand Deformities

Wed, 28 Sep 2005 16:36:56 GMT

Apert Syndrome and the Causes

Apert Syndrome is an inherited anomaly that is associated with characteristic craniofacial anomalies that frequently also require surgical correction. It is always best to seek hand surgery consultation for hand or feet syndactyly as soon as possible so that questions can be answered, anxieties allayed, and a check can be made for other anomalies that may occasionally be present with Apert Syndrome.