Wednesday, July 16, 2014

Best Scientific Publication on Birth Anomalies of the Upper Extremity

I have been fortunate to learn from a number of wonderful hand surgeons over my career.  One of the most impactful was Paul Manske, a hand surgeon and, more specifically, a congenital hand surgeon.  He spent the majority of his career here at Washington University School of Medicine.  I am not alone in this sentiment of appreciation and 4 of us have established an an annual award recognizing the "best" or most impactful publication in the field of upper extremity congenital/ pediatric anomalies.  Ann Van Heest, Michelle James, Relton McCarroll and I review the literature in our field to determine the winner of the award each year.  


 In 2011, the winner was Ann Nachemson's group for "Children with surgically corrected hand deformities and upper limb deficiencies: self concept and psychological well- being.  JHS 2011; 36E: 795- 801.  This manuscript provides insight into how children with deficiencies of varying severity identify with their anomalies.  The more severe group had a self concept score similar to a group of healthy children.  Children with milder deformities were found to have lower (worse) scores than those children with more severe deformities.  Nachemson Study
 
 In 2012, the winner was PP Kotwal, et al for "Comparison of surgical treatment and nonoperative management for radial longitudinal deficiency.  JHS Eur 2012; 37(2) 161-9.  This study evaluated two groups of children treated for radial deficiency and found superior outcomes in those treated with centralization (or radialization).  This study is important as it was the first to scientifically demonstrate the positive effect of centralization. Kotwal Study
 

Finally, we recently notified the authors Clement and Porter that "Forearm Deformity in Patients with Hereditary Multiple Exostoses: Factors Associated with Range of Motion and Radial Head Dislocation" in the Journal of Bone and Joint Surgery was the 2013 winner.  In a large group of patients, this study showed that distal radius exostoses were most common.  Additionally it confirmed that a shortened ulna was related to decreased forearm motion and radial head dislocations.  It suggested, therefore, that surgery should be considered in such patients. Clement Study

Tuesday, July 8, 2014

Type 2 Radial Longitudinal Deficiency

Bayne and Klug wrote what is now a classic scientific article on radial longitudinal deficiency in 1987 Classic Article.  This article described 4 types of radial longitudinal deficiency:

Type 1- Short distal radius
Type 2- Radius in miniature (short proximal and distal)
Type 3- Absent distal radius
Type 4- Absent entire radius

More recently, we added a Type 5- which is a Type 4 deficiency + an absence of the proximal humerus- Type 5 article and a James, McCarroll and Manske added the Type 0 and N Modified Classification.

Perhaps the least common type of radial longitudinal deficiency is the Type 2- Radius in Miniature. Because it is uncommon, treatment is not clearcut.  Here is one example of such a case.


Type 2 Radial Longitudinal Deficiency.  Note the very short radius bone.

In this patient, we lengthened the radius in an attempt to better balance the wrist on the forearm bones. Unfortunately, we did not obtain as much length as we hoped but the length we did obtain stood the test of time over the next 12+ years.  The wrist is balanced and the pollicization is doing well.

2 years after lengthening surgery for Type 2 Radial Longitudinal Deficiency

4 years after lengthening surgery for Type 2 Radial Longitudinal Deficiency

12 years after lengthening surgery for Type 2 Radial Longitudinal Deficiency.

Here are clinical pictures of the patient at 12 years after surgery for Type 2 Radial Longitudinal Deficiency.  There are several interesting features. First, the ulna has a big bow which makes it look like the elbow does not straighten fully (it does). Second, the wrist has remained relatively balanced- we will continue to follow him and with growth there may be a need to lengthen the bone a second time.  This time, we would plan an external fixator lengthening- this option was not possible due to the young age at the time of the first surgery.  There is also a pollicization which has helped function.  
Type 2 Radial Longitudinal Deficiency. 12 years after surgery.  Note relatively balanced wrist and pollicization.

Type 2 Radial Longitudinal Deficiency.  Elbow straight (it doesn't look straight as the ulna bone is curved (bowed).

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

Wednesday, July 2, 2014

Popular Press News Stories

I have attached a few links that I believe you might like to see.  These are stories relating to birth anomalies of the hand and upper extremities.  These stories were in the popular press.

World Cup Soccer  Interesting link which is also thought provoking.  Thanks to Terry Light.

Phantom Limb Pain  Link regarding birth anomalies and the brain.  Fascinating.

Robotic Prostheses  Technology advancement for prosthetics

More on Prosthetics  Summary of our 3D prosthetic work at Washington University.

Happy Reading.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu