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