Tuesday, March 27, 2012

Camptodactyly



Camptodactyly is the position of flexion of the proximal interphalangeal (PIP) joint. Translated from Greek, camptodactyly means “bent finger.”  It may be first noted in the newborn, as a congenital camptodactyly, or it may present or progress in adolescence.   There are a number of anatomical structures that have been described as “causing” camptodactyly including abnormal muscle and tendon insertions.  Additionally, camptodactyly may results from weak muscle extension power at the PIP joint or may be a part of a larger syndrome such as arthrogryposis.

There are no easy answers for camptodactyly but treatment starts with therapy.  Extension splinting at night (a static or resting splint) and more aggressive splinting during the day (dynamic or static progressive splinting) may be helpful.   Surgery does not provide an easy answer and the results may be disappointing to both surgeon and patient.  The position of the joint can usually be improved but almost never can it be made normal.  Surgery can also help with therapy by taking a joint that had been difficult to splint and making splinting possible.  Surgery usually consists of releasing any abnormal structures that may be limiting PIP joint extension, possibly release the tight joint itself, and possibly moving tendons to increase the strength of extension of the PIP joint.    One of the risks of surgery that worries the surgeon is the loss of ability to fully bend the finger.

Camptodactyly of small finger PIP joint.  Adolescent type.
Patient is attempting to straighten small finger. 

World Symposium on Congenital Malformation

Wow!  I had to write to share my recent experience at the World Symposium on Congenital Malformation of the Hand and Upper Limb.  This meeting is held once every three years and this year was in the United States in Dallas Texas, hosted by Marybeth Ezaki and Scott Oishi and the Texas Scottish Rite Hospital.  The meeting was an amazing 3 day event with discussion of all things involving the congenital upper extremity.  The participants included visitors from 6 continents and more than 30 countries!

I was fortunate to be on the program committee (although the hosts did the majority of the work)- the talks were on multiple fascinating topics ranging from surgical treatment to incidence to the psychology of children with these anomalies.  I shared our experience on outcomes of treatment for complex syndactyly (that is syndactyly with bony involvement).

On a related note, we presented the first annual Manske Award (named in honor of my former partner, Paul Manske) for the Best Congenital Manuscript of the year, 2011.  The winner was Ann Nachemson and colleagues from Gothenburg Sweden for their paper entitled "Children with surgically corrected hand deformities and upper limb deficiencies: self-concept and psychological well-being .  J Hand Surg European, 2011. 36: 795–801.  This paper demonstrated that children with congenital hand anomalies have self concept and pyschological well being which is comparable to unaffected children.  Interestingly, those children with severe deformities actually score better than those children with milder anomalies.  Congratulation to Ann and colleagues for a great contribution to our understanding.

Tuesday, March 6, 2012

External Fixator- Piece of Cake

video
There are two different types of fixators that we use in the care of kids with upper extremity differences.  One is a unilateral frame and is just on one side of the bone.  It usually has 4 pins sticking into the bone and a rail outside the skin.  It is useful for lengthening bone- done with a painless turning of a dial 3-4 times/ day.

The patient above (Lola) had a fixator for several months and, as you can, she did great with it.  As Lola mentions, there is no pain wearing the fixator or "turning" the dial to lengthen.  She mentions wearing a splint- we use a splint to provide a little extra protection while we are lengthening the bone.  The length of time the fixator is on the arm differs from child to child depending on the bone we are lengthening (we can lengthen the humerus, radius, ulna, metacarpals, and phalanges) and the desired length.

The other type of frame is a "circular frame" and, as it sounds, it goes around the extremity and gives great control and allows very precise correction of bony and soft tissue abnormalities.  We use this type of frame in radial deficiency before we perform a centralization procedure.  It helps to stretch the soft tissues and makes the centralization procedure more straightforward to perform and, we believe, more effective.

Circular frame for radial deficiency prior to centralization