|Radial deficiency patient. The fixator is about to be removed on the right and the wrist centralized. Note the difference between the wrists (the left side has not yet been treated).|
Monday, May 28, 2012
Friday, May 25, 2012
2) Cleft reconstruction in this case involves removal of extra skin between the fingers and tightening the space between the index finger and ring finger. We use part of the tendon sheath of the two fingers to stabilize these 2 fingers in a more aligned, narrowed posture.
3) Partial syndactyly. We utilized a dorsal flap to recreate the commissure between the fingers and then re- arranged the skin around this flap.
|Cleft hand before surgery|
|Cleft hand from palm view before surgery|
|Cleft hand with small space between thumb and index finger|
|Cleft hand immediately after surgery|
|Cleft hand after surgery|
|Cleft hand after surgery, palm view|
|Sprengel deformity at rest. Notice elevated shoulder on left.|
|Sprengel deformity with limited arm elevation.|
|Xray of Sprengel Deformity. Note bump in left shoulder compared to right. That is the high shoulder blade.|
Sunday, May 20, 2012
This operation allows easier use of the hands together. Compare the photographs of the children below. In the first two pictures (before surgery), the arm rotation position makes it difficult to use the hands. In the final picture (another child), one arm has been corrected and the other has not.
|Internally rotated arms in arthrogryposis. This is called reverse, pronated grasp.|
|Video game playing in child with arthrogryposis and internally rotated arm position.|
|After external rotation osteotomy on the child's right side and before surgery on the left. The wrist has also been corrected on the right (osteotomy) and the elbow released.|
Sunday, May 13, 2012
|Thumb deformity (clinodactyly) in Rubinstein Taybi.|
Saturday, May 12, 2012
Robinow syndrome is an exceedingly rare condition which may be transmitted as an autosomal dominant or recessive condition. Patients are short stature with the forearm segments classically most notably short (mesomelia). The fingers and toes are also short (brachydactyly) and may be curved as well (clinodactyly). There are classically spinal malformations, craniofacial anomalies, and abnormalities of the genitourinary system.
|Characteristic appearance of a patient with Robinow syndrome. Note also the short fingers and thumb.|
|Hand in Robinow. Note appearance of polydactyly|
of the thumb.
|Short fingers in Robinow syndrome.|
|Post surgery in Robinow syndrome. Additional bone and finger nail have been excised.|
The space between the thumb and index finger has also been widened.
Sunday, May 6, 2012
|Extra thumbs on both sides, quite different|
|Radial polydactyly on the right|
|Another difficult radial polydactyly|
Factors affecting appearance Factors affecting function
1) Radial Deviation 1) Length of forearm
2) Short length of forearm 2) Finger motion/ function
3) Presence/ status of thumb 3) Presence/ status of thumb
Other factors should be considered as well but I think these are less important. These include forearm motion- rotation (lacking especially in those with severe RLD) and wrist motion (generally limited and tending towards flexion with a lack of extension).
Some of these may be addressed with surgery although none are completely correctable.
1) Radial deviation (which may also affect function) may be addressed through a centralization procedure.
2) The forearm can theoretically be lengthened although I am not a strong believer in this operation (for patients with a diagnosis of RLD) as I believe forearm lengthening has limitations and the procedure may have numerous complications. I do believe that centralization "lengthens" the forearm by placing the hand and wrist back on the end of the forearm making the forearm longer.
3) Providing a thumb is clearly the "home run" surgery for children with RLD as it markedly improves both function and appearance.
4) Limited finger motion is difficult to address in RLD and in other congenital conditions.
In the photos below, I believe the short forearm in the most notable difference. The wrist has been centralized and is generally well aligned. The index finger has been pollicized into the position of a thumb and generally looks good although with careful assessment, it is noticed.
|RLD with a short forearm and a pollicized index finger as a thumb.|
|Another view of patient with radial deficiency|