Central Deficiency, Severe

Central deficiency (cleft hand) may present in many different ways.  Sometimes, there is just long (or middle) finger absence and other times more than one digit may be missing.  The thumb- index web space is often affected, ranging from a mild narrowing to a complete syndactyly/ merging of the space.

In this young patient, both the long finger and the ring finger are completely absent.  There is a large cleft between the index finger and the small finger.  Finally, the thumb and the index finger are merged.

The primary functional limitation for this child is related to the lack of a thumb- index web space.  This affects large object grasp (think: soda can) and tip-to-tip pinch (think: manipulating beads).  In some children the large space between the index and small fingers (the cleft) can return some of this functionality, but certainly not all function.

Central Deficiency (cleft hand) with merged thumb and index finger.

Xray of central deficiency (cleft hand) with missing long and ring fingers.

If both the thumb and index finger have independent function on examination (can be difficult to assess), separation can be accomplished with reconstruction of the thumb- index web space.  This provides a thumb and two fingers.  It is important to note that reconstruction is not appropriate in all children with a limited number of digits and a number of factors should be considered including the opposite hand (i.e., whether it is affected).  Some families will favor a surgical approach while others may not.

Amniotic Constriction Band

The cause of amniotic constriction band is uncertain.  Some believe it is a developmental issue (a "dysplasia" resulting from abnormal development of the structures) and others believe that it is a "deformity" that results from banding or pressure on previously  normal structures.  Either way, at the time of birth, a variety of differences can be noted.

Banding, or ringing, of a finger, forearm, or leg can occur.  This is usually a problem only with appearance but deeper bands can also affect function.  Amputations (possibly from extremely tight bands or direct pressure in utero) can also occur.  The amputations usually affect fingers (or toes) or parts of fingers (or toes).  Finally, syndactyly (abnormal joining of the fingers) can also occur.  This type of syndactyly differs from other forms as the fingertips are joined whereas there are fenestrations (or holes) between the fingers closer to the hand.  Sometimes the tips of multiple fingers are joined together.  It is common for more than one extremity to be affected.

In the pictures below, the thumb and small finger are normal.  The index, long, and ring fingers are short due to amputations.  The long and ring fingers are syndactylized (joined at tip) but there is a fenestration between the digits.

Central Deficiency

Central deficiency is also called cleft hand.  It typically includes a missing long finger (middle finger) and a narrow thumb- index web space.  Severity varies and some patients have loss of additional digits or a syndactyly of the ring and small fingers.  Like many congenital anomalies, central deficiency is considered from both a functional and an appearance perspective.  The most important consideration for function is the space between the thumb and the index finger.  This space is reconstructed with the skin of the cleft if the narrowing is severe or, in a more mild narrowing, with a local skin rearrangement (z- plasty).  The cleft is a notable appearance issue.  Sometimes the cleft is reconstructed with a soft tissue reconstruction alone and other times, the index finger (actually the index ray) is moved away from the thumb to both widen the thumb- index web space and narrow the cleft.

The thumb- index web space is not terribly narrow so was reconstructed with a local skin rearrangement while the cleft was narrowed.

Recurrent Syndactyly

This 8 year old male was surgically treated with syndactyly reconstruction utilizing skin grafts 6 years ago (in his hometown).  He did well although the skin between the fingers gradually crept distally towards the fingertips.  The skin growth limited the spread of the fingers.

He and his family requested surgical reconstruction.  Hoping to avoid the use of skin grafts, we utilized a "Box Flap" technique which provided a satisfactory outcome.  These photographs are pictures from the operating room.

Toe Polydactyly

Toe Polydactyly

I am a congenital hand surgeon and, therefore, treat children with birth abnormalities of the upper extremity.  However, because abnormalities of the feet, including extra toes and toe syndactyly, are similar to hand anomalies, I also treat children with some birth differences of the feet. 

Toe polydactyly (extra toes) is less common and also less of a problem than extra fingers.  In some instances, extra toes can be ignored.  However, if the extra toes affect the width of the foot, wearing shoes can be a problem.  If shoe wear is difficult, removal of the extra toes is recommended to narrow the foot.

This case demonstrates a relatively minor extra toe next to the 3^rd toe.  There is also a partial syndactyly between the great toe and the second toe.

This child has a more significant extra great toe on each side preventing shoe wear.  

This was treated prior to standing/ walking and by 12 months of age, the child was able to wear shoes.

More thoughts on Pollicization

The decision to proceed with pollicization is not easy.  It is most straightforward when the thumb is completely absent and is most difficult when the child has any thumb, as families very reasonably would like to keep and improve the small thumb rather than excise it and pollicize the index finger.  However, the majority of congenital hand surgeons (especially in the United States) believe that a pollicized index finger will look and function better than a reconstructed small (hypoplastic) thumb.   Importantly, this is not to say that all small thumbs should be excised- in reality most can be reconstructed and stabilized but need to have a stable base (CMC joint).

This case demonstrates a Type 3b hypoplastic thumb with an unstable CMC joint.  Therefore, we did not feel that reconstruction made sense and we recommended pollicization.

This pictures were taken one week after pollicization.  The bruising and discoloration typically improves over the next 2 weeks.

Radial polydactyly

An extra thumb has been labeled many different ways: ‘radial polydactyly, thumb polydactyly, split thumb, and duplicated thumb’.  My preferred name is radial polydactyly as it tells you exactly the issue.  The term ‘duplicated thumb’ is not ideal as the thumbs are both smaller than normal but ‘split thumb’ is not perfect either because the thumbs are usually more than 50% of the size of the opposite thumb.

Radial polydactyly is typically both a functional problem and an appearance issue.  Surgery is recommended.  However, surgery is not simply removing the extra thumb- it is a reconstructive procedure that can range from a moderately complex procedure to a very complex procedure.  There is no ideal time for surgery but typically surgeons choose to operate between 1 and 2 years of age.  Sometimes the thumbs are merged to make the best, combined thumb and sometimes the bigger, more mobile thumb is reconstructed to make it stable and straight.  Surgery can involve soft tissues only but often also involves bone surgery.  A second procedure is required in some patients as they get older.

In this case, an older child had the smaller, outer thumb removed with a stabilization and reconstruction of the larger thumb.  

Before Surgery

After Surgery