Tuesday, February 23, 2016

Surgery for Olliers Disease

Multiple enchondromatosis is also known as Olliers Disease.  In this disease, at least 2 cartilage tumors known as enchondromas grow in different bones.  Initially, we tend to watch the growths, but sometimes surgery is necessary.  I have previously blogged about Olliers as part of a post on MACRODACTYLY.  There are number of sites that provide good, basic information on Olliers Disease including WikipediaNORD, and OMIM.  The NORD site is perhaps the best.

The enchondromas in Olliers Disease are typically benign, meaning that they don't spread and are not malignant.  There is a risk, however, of transformation to "bad" cartilage tumors such as chondrosarcoma.  This is the reason that we keep a close eye on these tumors.  A related disease, Maffucci Syndrome, also has enchondromas but includes blood vessel growths called hemangiomas.  This can have a higher rate of malignancy.

The indications for surgery include pain, deformity (such as angling of the bone), or a break in the bone.  The reason problems develop is that the cartilage tumors can expand the bone and make it weak- this weakness can lead to a fracture.  In addition, the growing tumor can affect the growth plate leading to angulation of the bone.  It is interesting that adults can also develop these tumors but in these situation in adults, only 1 enchondroma appears.  

It takes several years at least for evidence of the enchondromas to appear in the child.  Sometimes the first sign is a broken bone through the weak area and sometimes the first sign is angulation of the bone.  If we believe that a child has Olliers, we perform a skeletal survey- that is we xray multiple different body areas.  Typically Olliers only affects one side of the body.

Here is one child with pain and deformity related to the enchondroma growths.  There are 3 growths in this child, 1 in the thumb and 2 in the index finger.
Olliers Disease.  Note the swelling of the index finger.  That is the expanded bone.


Another view of Olliers Disease in the hand.

Olliers Disease.  Note the disease in 2 bones of the index finger and 1 bone of the thumb.

In this child, due to pain and enlargement, some deformity, and decreased motion, we performed surgery to remove the cartilage tumor.  We filled the "holes" with bone graft.  This should heal over time.
Enchondroma as removed from the index finger.  This is soft cartilage growth.

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


Monday, February 15, 2016

Camptodactyly- bent finger- treatment with splinting

Camptodactyly is the Greek word for bent finger- specifically flexed so that straightening the finger at the middle joint is not possible.  I have blogged about camptodactyly several times before- read those posts HERE and HERE. 

Therapy is the first line treatment for camptodactyly.  And it usually works.  But therapy has its limits and if the finger is too far bent, therapy can not obtain the leverage for splinting.  Typically, fingers bent less than 60 degrees are treated with splinting at least to start.

Here are a few pictures of a 13 yo child with camptodactyly presenting to me for the first time.

Camptodactyly.  The ring finger on both hands is most effected.

Camptodactyly.  The ring finger is most effected.

Camptodactyly demonstrating an ability to make a fist. This ability is preserved.


There are different splinting techniques but I wanted to share our hand- based splint for camptodactyly of the central digits.  The fingers are held in a bent position (through the MP joint) which relaxes tight structures in the fingers such as the tendons.  The tan straps then put pressure on the tight PIP joints as a straightening force.

Camptodactyly hand based splint.  

Camptodactyly hand based splint.  

Camptodactyly hand based splint.  

Camptodactyly hand based splint.  


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




Monday, February 8, 2016

Separating joined fingers: A Case Demonstration of a New Strategy

Surgery to separate joined fingers has been largely unchanged for 40 years.  This post is meant to share case pictures and details on this new strategy. Syndactyly, or the abnormal joining of the fingers, is one of the most common birth anomalies of the hands and feet.  In our recent assessment of birth anomalies in NY State, the prevalence of syndactyly was approximately 1.3 in 10,000 live births (polydactyly was clearly the most common anomaly).

The treatment of syndactyly has been successful but with little change over the last 40 years.  I have previously blogged a few times on this topic HERE.  We understand the importance of the webspace (the 'commissure') and avoiding tension on the flaps. Skin grafts are often necessary in syndactyly reconstrution (although there are good graftless techniques- see previous posts).  I recently blogged HERE about a new technique that we have utilized- a skin graft substitute called hyaluronic acid scaffold.  It has been very successful in our hands and we hope to share our early term results soon.

This case demonstrates both the basic principles of syndactyly reconstruction and the use of the hyaluronic acid scaffold instead of skin grafts.  I will blog in the near future with some early postoperative results in another case.

Syndactyly of the middle and ring fingers.

Planned skin incision in syndactyly reconstruction.  Note the primary commissural flap and the zig zag flaps.

Syndactyly reconstruction from palmar approach.
Syndactyly reconstruction after suturing.
Syndactyly appearance from the palm after suturing.
Syndactyly after suturing with areas in need of coverage.

Syndactyly after suturing with areas in need of coverage.

Syndactyly reconstruction after placement of hyaluronic acid scaffolding (white)
Syndactyly reconstruction after placement of hyaluronic acid scaffolding (white)

Syndactyly reconstruction after placement of hyaluronic acid scaffolding (white)

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