Tuesday, September 27, 2011

Ulnar Longitudinal Deficiency: The Basics

What is Ulnar Longitudinal Deficiency (dysplasia)?

Ulnar Longitudinal Deficiency (ULD) is a lack of formation of the pinky side of the upper extremity.  It usually affects the forearm but can affect the hand, forearm, and upper arm.  It can affect bone, muscle, tendon, nerves, and blood vessels.  The severity is different in each affected child.

What specific abnormalities are seen?
Hand:  The entire hand may be affected.  The thumb can be affected and the webspace between the thumb and the index finger is often narrowed.  The hand can have syndactyly between other digits.  In more severe cases, a variable number of digits may be absent.  There can abnormal bony connections between the bones of the wrist and/ or bones of the hand (typically, the metacarpals).  Forearm:  The ulna is most commonly short.  It can be absent.  The radius bone may be affected as well but usually is normal.  Sometimes the radius is fused to the humerus (radioulnar synostosis). 

What are other names for ULD?
The most common other name is ulnar clubhand.  It is also sometimes called ulnar deficiency for short.

How does ULD happen?
The arm forms between 4 and 8 weeks of gestation, sometimes before a mom even knows she is pregnant.  By 8 weeks the arm is fully formed although obviously really small.  If there is some insult to the developing arm, part of it may not form normally.  There are different types of insults- some are genetic and some may be caused by outside influences (“environmental factors”).

Are there are medical problems associated with ULD?
The short answer is no.  The only associated medical abnormalities are to the musculoskeletal system.  Specifically, there can be abnormal development of the fibula (at the ankle) or even more rarely other bones in the lower extremity.  

Monday, September 26, 2011

Las Vegas and Potosi- What do these 2 cities have in common?

Recently, I took two hand surgery related trips.  First, the American Society for Surgery of the Hand was in Las Vegas at the beginning of September this year.  Not my favorite city but certainly a good place for a conference.  This meeting is always very educational and provides a wonderful forum for discussion on general hand issues.  There was some reporting/ discussion of congenital and pediatric hand issues including scientific papers and several lectures.  One of the papers was ours on pediatric and adolescent cubital tunnel syndrome.  Another reported the changing nature of scaphoid fractures in children; current fracture patterns are similar to adults with scaphoid waist fractures most common.  A third reported the syndromic relationships with fully formed extra ulnar digits.  And finally, there was a large series on outcomes after carpal wedge osteotomy for the arthrogrypotic wrist.

My next stop was a little more rural.  Last weekend, September 16-18, was our 4th annual Hand Camp at Camp Lakewood in Potosi, Missouri.  The camp for families and children with severe upper extremity anomalies is about 1.5 hours from Saint Louis.  There are physical activities including wall climb, archery, arts and crafts, and horseback riding with all children participating in each activity.  There are group sessions to allow frank discussion among parents and families.  Additionally, the junior counselors, teens who have successfully navigated the difficult social and functional issues, share their tips with the families and campers.  My favorite part of camp is watching kids succeed at tasks that seem daunting (think archery for a child with one arm) and also the group sessions where emotions can run high and lessons abound from the sharing of experiences.  Can’t wait for next year!