6-Year-Old Undergoes Toe to Hand Transfer

From the Baltimore Examiner:

Hector Salas endured nine hours of surgery Tuesday and emerged sporting a new ring finger.

The 6-year-old underwent a procedure that removed a toe from his foot and added a finger to one of his hands, which only had a thumb and “nubbins.”

Complications usually surface within the first five days following surgery, and the first 24 hours hold the most danger, said Dr. James Higgins, a surgeon who performed the operation at Union Memorial Hospital’s Curtis National Hand Center.

Helping Hands Foundation Aids Romanian Boy

The Capitol (Annapolis, Maryland) had an article recently about Paul Lacatos, a 17-month old Romanian boy who suffers from complex syndactyly along with other issues in his legs.

The Helping Hands Foundation helped bring Paul to Annapolis from Romania. The foundation has covered travel and medical costs along with finding a host family for Paul and his mother.

The article itself is comprehensive and thoughtful. It touches on almost every aspect of the story from potential environmental causes of the condition to the emotional bond that Paul's host family developed for him. Most importantly, it demonstrates how the generosity of these individuals has resulted in a tangible benefit for Paul.

New Diagnosis for Poland's Syndrome

On Wednesday we took our six-month-old daughter to see Dr. Steven Moran at Shriners Hospital in Minneapolis. He quickly diagnosed her with Poland's Syndrome. According to this overview by the National Organization for Rare Disorders:

Poland Syndrome is a rare condition that is evident at birth (congenital). Associated features may be extremely variable from case to case. However, it is classically characterized by absence (aplasia) of chest wall muscles on one side of the body (unilateral) and abnormally short, webbed fingers (symbrachydactyly) of the hand on the same side (ipsilateral).

In those with the condition, there is typically unilateral absence of the pectoralis minor and the sternal or breastbone portion of the pectoralis major. The pectoralis minor is a thin, triangular muscle of the upper chest wall; the pectoralis major is a large, fanlike muscle that covers most of the upper, front part of the chest.

Affected individuals may have variable associated features, such as underdevelopment or absence of one nipple (including the darkened area around the nipple [areola]) and/or patchy absence of hair under the arm (axilla). In females, there may be underdevelopment or absence (aplasia) of one breast and underlying (subcutaneous) tissues. In some cases, associated skeletal abnormalities may also be present, such as underdevelopment or absence of upper ribs; elevation of the shoulder blade (Sprengel deformity); and/or shortening of the arm, with underdevelopment of the forearm bones (i.e., ulna and radius).

Our daughter appears to have the classic symptoms of the disorder minus any skeletal abnormalities in her ribs and shoulder blade. I based this last comment on an x-ray she had at three-months-old that didn't appear to show anything abnormal. It is certain she's missing at least one of her pectoral muscles.

There is likely to be surgical procedures down the road that are both functional and cosmetic in nature. I'll cover more on the condition, treatment options, and related topics in subsequent posts.

As for our daughter's hand, Dr. Moran was as optimistic about her function and prognosis as the other specialists, orthapedists, hand surgeons and pediatricians we've met with.

Cleveland Clinic

I came across this overview from the Cleveland Clinic that includes a good straight-forward explanation of the most common congenital limb conditions, treatment options, and information for relevant staff at the Clinic. I dug around in the Cleveland Clinic site to try and find a page dedicated to the Clinic's approach and resources for treating limb differences. The closest I could come up with is the Pediatric Plastic Surgery Section and the Orthopedic Surgery Section.

I haven't read or heard anecdotally about the Clinic's reputation for pediatric plastic or orthopedic surgery, surgical rehabilitation or the specific experience its specialists have with limb differences. While it's always risky to make assumptions about an institutions ability to diagnose and treat rare conditions, given the Clinic's national reputation it's probably fair to assume that if your seeking initial information and treatment options you'll be well served. This is especially true if you need to seek treatment in the Cleveland area and don't have access to other treatment centers in the Midwest or East Coast.

Back to Curtis

Last week we took our daughter back to see Dr. Thomas Graham at Curtis National Hand Center. This was our first real appointment at Curtis. Our first visit was during the monthly clinic the Center offers for those seeking initial consultations for children with congenital hand differences.

As I previously detailed, our daughter has a small thumb and a pinky finger on her right hand. She is missing her index, middle and ring finger. The growth of the hand so far has allowed her to form a very functional pincher grip.

Overall the discussion was as positive as we could've hoped for. Dr. Graham was as optimistic about our daughter's hand function as he and the others physicians were during her initial clinic visit. It was Dr. Graham who first diagnosed her condition as "transverse failure to form." Meaning it is not connected to any other conditions and is very unlikely to have been passed on genetically. In his words, it was a "lighting strike."

The bottom-line from this appointment is that there is really nothing more to do in the short-term other than continue watching our daughter develop. We are seeing one more specialist at Shriners later this month. Provided his guidance doesn't dramatically differ from the 1/2 dozen others doctors we've seen, my wife and I have agreed that we've heard enough to be confident that our daughter is lucky to have the level of function she has and that there are no procedures we need to plan for in the near future.

2007 O&P Extremity Games

From the Extremity Games Press Release:

"The O&P Extremity Games is an extreme amateur sporting competition for individuals, 13 years and older, living with limb loss or limb difference. The event allows participants to demonstrate skill, persistence and passion while competing for cash and other prizes - proving There’s No Replacement for the Competitive Spirit. The inaugural 2006 O&P Extremity Games rocked with over 500 participating athletes, sponsors, spectators and volunteers. That number is expected to triple in 2007. "

The 2007 Extremity Games take place July 19 - 21 at the Orlando Watersports Complex.