Sunday, March 1, 2015

Syndactyly Outcomes and Challenges

Most children treated for syndactyly have an excellent outcome with normal finger motion and good maintenance of the reconstructed web space.  However, there are times with the ideal outcome does not happen.  A less than perfect outcome includes challenges like:
  •      prominent scarring,
  •      an inability to spread the fingers as widely as we would like,
  •      an inability to straighten the fingers fully,
  •      creeping of the web space (i.e., when the skin inches out towards the fingertips)
  •      rotation of the fingers
  •      deviation of the fingers
  •      nail irregularity
  •      discoloration of skin grafts (typically seen with groin skin grafts)

Here are some pictures of some of these challenges in syndactyly.

Syndactyly web creep between thumb and index finger.

Syndactyly web creep between index and long fingers.
Scarring after syndactyly reconstruction limiting finger extension.  Also not skin graft discoloration.

Rotation and deviation after complex syndactyly reconstruction.  Most notable in ring finger.

Here are some thoughts on risk factors and thoughts on these uncommon situations.

1.         The biggest risk factor is complex syndactyly- that is when the bones are joined together at the fingertip.  We know that these children often have rotation and deviation of the fingers as well as nail irregularity.  This was demonstrated in an article in the Journal of Hand Surgery in 2012. 
2.         Anytime there are more than two fingers involved, the risks increase.
3.         If the syndactyly is associated with a syndrome, the risks increase.
4.         There are technical risk factors for creeping of the skin or prominent scarring.  This means that the surgeon has some degree of control over these risks and an experienced surgeon can minimize these outcomes.  But, it is important to note that the surgeon can do everything “right” and sometimes a less than perfect outcome happens and additional surgery may be considered.
5.         We also believe that therapy after syndactyly reconstruction can be helpful to minimize problems with scarring.
6.         Finally, the nature of the syndactyly itself affects these outcomes.  Meaning some children and some types of syndactyly (they are not all the same, some are tighter, sometimes the skin is more pliable, etc.) increase risks.

The bottom line is that every child with syndactyly reconstruction will not have a perfect outcome and some will require a second operation.  We follow patients for at least several years after surgery and then intermittently until growth is done.  We know that scarred skin does not stretch as well as normal skin and the growing hand can experience problems with syndactyly scars.

Charles A. Goldfarb
My Bio at Washington University

Sunday, February 15, 2015

Radial Deficiency- Every Child is Unique

Every child with radial longitudinal deficiency is unique and therefore is treated individually. While there are general principles in treatment and these principles may be applied to most children, we begin care by working to understand each child and each family.

In a child with radial longitudinal deficiency presenting for evaluation, we consider many factors as we plan treatment.  First, the family's feeling about the anomaly and their desire for intervention is considered.  Each family comes from a different place and has different expectations, goals, and desires.  In some, there is a family history which will affect the understanding and perhaps provide the most realistic understanding as those families really know how radial longitudinal deficiency affects daily activities, daily tasks, and interactions with peers.

Other factors are considered as well.  In young children, we find that the time of diagnosis matters to the family's comfort with the diagnosis.  If a child is diagnosed by prenatal ultrasound, the family will have more time to grow to accept the diagnosis and perform their own research and come to some early conclusions about the role of the hand surgeon.  If not diagnosed before birth, more time may be necessary for the family to become comfortable with the diagnosis.  The age of the child at the time of the visit to the hand surgeon also matters (for similar reasons) as families can better understand potential function as the child ages and increases hand use.  Associated conditions matter including medical conditions (the kidneys and the heart).  In TAR syndrome, for example, the platelet count has to be considered before discussing surgery.

As I have written before, in children with radial longitudinal deficiency we have a general plan. Consider this child who is nearing two years of age and travels a great distance to come visit.   She has a severe angulation of the wrist with an absent radius (making this a Type IV radial deficiency) and an absent thumb (Type V thumb).   This is a quite common presentation in our clinic.

Radial longitudinal deficiency with marked deviation at the wrist.

Radial longitudinal deficiency with absent radius bone and deviation.
We also check a few other features.  First, we assess how correctable the wrist deviation posture is- passive stretching can help but the flexibility matters in considering surgery.  This child is mildly flexible but cannot be corrected all the way to neutral (i.e., we cannot straighten her wrist).

Gentle stretching shows limited correction of the wrist in radial deficiency.

And we also check the position of the wrist on the lateral (side) x- ray.  As is usually the case, the x- ray below confirms that the wrist is below (palmar) to the forearm.  This is part of our correction.

Radial longitudinal deficiency- lateral view showing palmar position of the carpus and hand compared to the forearm.
This child has limited correction and a stiff wrist.  He is planned for a 3- part reconstruction.  The initial treatment is to apply a fixator.  As I have previously blogged and blogged2, we often use a ringed fixator as it allows a 3-D correction before we centralize the wrist.  In some cases, however, we use a unilateral fixator which allows safe stretching of the wrist before the centralization procedure.
Application of a unilateral (one sided) fixator in radial deficiency.  The device allows gradual stretching which will make the centralization procedure easier and safer in the future.
This fixator can be placed on the ulnar side (as in picture) or the radial side- both are helpful and there are arguments as to which is best.  We plan to leave this fixator in place for about 8 weeks before the second stage is performed, the centralization procedure.  Then, approximately 6 months later, we will create a thumb by pollicizing the index finger.

Charles A. Goldfarb
My Bio at Washington University

Sunday, February 1, 2015

Nora Lesion

A Nora Lesion is also knows as a bizarre parosteal osteochondromatous proliferation.  This tumor is a bony outgrowth on the outside of the bone, typically found in the fingers and off of the phalangeal bones.  The sexes are affected equally.  There is an occasional history of trauma but this may not be truly relevant.   This is a benign lesion (meaning it does not spread to other parts of the body) but it does have a high risk of coming back to the same spot after excision (i.e., local recurrence); it can recur in 50% of patients or more.  

This uncommon tumor is often seen in patients in their 20s and 30s but can be seen in adolescents (or younger).  It may be mistaken for a osteochondroma- please see two other mentions- post or 2nd post. It can also be confused with an osteosarcoma (parosteal variety).

Most patients present with a large bump but the size and rapid growth are both concerning and the bump can interfere with function.  Finger motion may be decreased.

Here is the case of a male in his 20s with the presentation of this bump over the previous 2 months.  There is no pain and despite the large size of the mass, his hand function was good.  

Clinical picture of index finger mass.  A Nora Lesion.

Radiographs were taken and an aggressive appearing mass is identified.

Nora lesion, view 1.

Nora Lesion, view 2.
Nora Lesion, AP view.

The patient was electively treated with surgical excision of the mass.  The pathology was confirmed as a Nora Lesion.   He will be followed closely over the next several years with clinical checks and intermittent x-rays.  Function is back to normal and he was back to work full duty by 2 weeks.

After removal of the Nora Lesion, radiographs show near normal bone.

Side view after Nora Lesion excision.

Charles A. Goldfarb
My Bio at Washington University

Monday, January 19, 2015

Ulnar Sided Cleft Hand

I have previously posted on cleft hand with a number of discussions.  More to come on this topic in the future as well.  However, I wanted to share a less common type of cleft hand, the ulnar cleft hand. Dr Tonkin from Sydney Australia has written about this unusual condition in the Journal of Hand Surgery.  In this case series, he reports on 3 children and describes ulnar cleft hand and reconstructive options.  Often the pinky needs to be re- aligned and stabilized.  The cleft can also be narrowed although, as with the more typical cleft hand, is not necessarily a function improvement.  It can be, however, an important appearance issue.

I have recently cared for a delightful family with adopted children from China- one has bilateral ulnar cleft hand.  As makes sense with adopted children, the family is taking a watch and see approach rather than jumping into surgery.  I think this is great.  Obviously, we want to really understand the child's function before even considering surgery.  And, to this family, the appearance not such an important factor.

In ulnar cleft hand, there is usually a good thumb and at least one good finger.  That is the case below with this ulnar cleft hand.  However, in Dr Tonkin's article, some kids had four digits and some had 5.

Notice the really deep cleft on the pinky side of the hand.  This is the case for both hands.  Note also the great thumb- index web space.   There is good alignment of the thumb.  The child has excellent pinch and large object grasp.  The pinky is not particularly useful on either side with the ulnar cleft hand.

Ulnar cleft hand, bilateral.

Ulnar cleft hand, left.  See deep cleft.

Right ulnar cleft hand.
x-ray of ulnar cleft hand.

x-ray of ulnar cleft hand, right side.
Charles A. Goldfarb, MD
My Bio at Washington University

Sunday, January 11, 2015

The Extra Thumb

An extra thumb, or radial polydactyly, can represent a difficult problem in congenital hand surgery.  The difficulty is that each child with an extra thumb is somewhat different.  There are certainly patterns (as we see with the Flatt Classification system) but not every thumb fits this classification system.  Zuidam and all (under the leadership of Steven Hovius in Rotterdam, The Netherlands) added to our understanding of radial polydactyly with these additional thoughts on the Topic.

The goals for reconstruction of the extra thumb are well accepted- a stable, straight thumb that functions well and looks good.  Patients don't like crooked thumbs (and nor do we) and no one likes thumbs that are not stable either (see our Publication). A thumb without stability is weak for pinch.  The surgeon's job is more difficult when the joints are not straight and the bones are not straight.  These situations require a more complex reconstruction of bone, joint, tendon, and muscle insertion.  Here is one such case of a Type IV extra thumb- which means the duplication is at the level of MCP joint with two proximal and distal phalanges (as you will see below).   In this case we had to narrow the metacarpal bone, reconstruction the ligament, and reconstruct the tendon insertion but did not have to cut and re- align the bones.

Radial polydactyly, type IV.  Not the deviation of both thumbs.

Radial polydactyly, type IV.  Not the deviation of both thumbs.  Palm view.

Radial polydactyly, type IV.  Not the deviation of both thumbs.  X- ray.

The outside thumb, the radial thumb, is removed to allow the best thumb (which is generally but always the inside thumb).  
Planned incision for radial polydactyly surgery.

Planned incision for radial polydactyly surgery.

Surgery allows straightening and stabilization to a now straight thumb.

The green sutures indicate where the ligament and the tendon were reconstructed for radial polydactyly.

After radial polydactyly surgery.

After radial polydactyly surgery.  Note that we avoid straight skin incisions as they can tighten over time.
Charles A. Goldfarb, MD
My Bio at Washington University

Thursday, January 1, 2015

Thumb Deformity after Polydactyly Reconstruction

Radial polydactyly, an extra thumb, can be more challenging to treat than other types of extra digits.   The surgeon cannot simply remove the extra thumb as ligaments need to be recreated and often bones and tendons need to be re- aligned as well.  Nonetheless, if the surgeon can create a stable, well- aligned thumb, the patient will do well both functionally and from an appearance standpoint (i.e., it will look good).  The other challenge is addressing the size of the thumb- often the thumbs are smaller than the normal thumb on the other hand.  Again, surgery can often help the size of the thumb but does not make it exactly the same as the normal hand (but it does not need to be an exact sized thumb).  The goal is a straight and stable thumb- as we have shown in the scientific literature.

If a thumb is not straight after radial polydactyly reconstruction, the patient may have trouble with pinch activities and may not like the way the thumb looks.  The options to treat the thumb are limited and include:
- Addressing any soft tissue problems (i.e., scar bands)
- Cutting and re- aligning crooked bones
- Making joints stiff (fusion).

This is one example of a child with a crooked thumb years after her initial surgery for an extra thumb.  She has trouble using the thumb in school and does not like the way it looks (it is very noticeable to her friends).   The deformity is from 1) a scar band and 2) bony instability at the joint.  Both issues need to be corrected in order to provide a satisfactory outcome.  An excellent result would be expected with surgery.

Deformity after radial polydactyly reconstruction

Another view of deformity of the thumb after radial polydactyly reconstruction.

Notable scar band which is contributing to the abnormal position of the thumb after radial polydactyly reconstruction.

Charles A. Goldfarb, MD
My Bio at Washington University

Saturday, December 27, 2014

A Dictionary for Birth Anomalies of the Upper Extremity

As much as I try to write for the non- physician, there are still medical terms throughout my blog posts. So, in an effort to simplify the terms and explain some basic concepts in the field of congenital hand, I will provide a dictionary of sorts.  I hope this helps simplify the terms and ideas.

Polydactyly means extra digit.  Polydactyly can be preaxial or radial sided (thumb sided) or postaxial (or ulnar sided, pinky sided) polydactyly.  The most common type is postaxial polydactyly.  There are a few rare types including central polydactyly (middle of hand) and mirror hand (3 or 4 extra digits on the thumb side).

Syndactyly means joined digits.  Most commonly there is only skin connecting the 2 fingers. However, sometimes bone can connect the fingers.  Rarely, more than 2 digits are involved in syndactyly.  The long and ring fingers are most commonly joined.

Longitudinal Deficiency means limited development on one side of the arm or forearm.  The most common type of longitudinal deficiency is radial sided (thumb sided) which may affect the radius bone, the muscles of the forearm and the thumb.  Less common is ulnar longitudinal deficiency which affects the pinky side of the forearm and may affect finger development as well.

Radial and ulnar longitudinal deficiency can affect the hand alone or the hand and forearm.  Cleft hand has been also called central deficiency as it typically affects the middle digits of the hand (as well as the thumb web space).  Cleft hand does not affect the forearm.

Amniotic constriction band has been called more than 30 different terms in the medical literature. Other common terms for this condition (which causes bands, syndactyly, and amputations) in amniotic band syndrome or constriction band syndrome. (although it is not a syndrome)  This category is by far the most common diagnosis given by pediatricians but is often inaccurate.

Symbrachydactyly is a tough term because it literally means short, webbed fingers but it is a term used for a broader range of conditions.  This includes children with absent fingers with nubbins and some kids with an absent hand or forearm.  This diagnosis can be difficult to distinguish from transverse deficiency or congenital amputation.

While I have previously written about causes for less than five fingers, it may be worth repeating. There are five common causes for less than five fingers: radial deficiency, ulnar deficiency, cleft hand, amniotic constriction band, and symbrachydactyly.

Camptodactyly means bent finger, specifically a finger in which the first joint (the PIP joint) does not straighten fully.  It can be present at birth, it can be associated with arthrogryposis, or it can present in the early teenage years.  Clinodactyly is most commonly seen in the small finger with deviation of the finger towards the ring finger.  The other fingers and the thumb can also be affected.

Charles A. Goldfarb, MD
My Bio at Washington University