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What Is
Syndactyly/Polydactyly?
There are many types of congenital hand deformities, the most common being
polydactyly and syndactyly. The syndactyly deformity occurs 1 in
2,000
births, and the Polydactyly, 1 in every 1,000 but both are usually hereditary,
therefore increasing the chances in isolated families. These names are derived from the ancient Greek, dactylos meaning finger and the prefix describes what is
wrong with the appendage: poly meaning many and syn
meaning together. You may have heard it called, web/webbed
fingers, webbed feet, or webbed toes.
With Syndactyly, (see
photo) the fusion, or webbing, of two digits or
phalanges (fingers, toes) is due to failure of separation during embryological development, usually between
the sixth and eighth weeks of intrauterine life. It can occur in hands and/or
feet and although it is more common in males, it can occur in females as
well. In 1/3 of these cases, there is another member of the family with the same condition.
Digits can be partially fused or fused along the entire
length of it's mass. The fusion can be simple (simple syndactyly) with the digits connected only by skin, or it can be complicated with shared bones, nerves, vessels,
and/or nails. Obviously, the complicated syndactyly will take several hours to
correct rather than a simple syndactyly correction. X-rays will be taken to
determine the difficulty of the operation as well as the appropriate technique
to be used.
Polydactyly (see
photo) is the occurrence of extra digits (fingers or toes). Polydactyly can
take the form of a barely discernible, undeveloped finger or toe to a fully developed extra,
functioning digit. Amazingly, polydactyly and syndactyly can be present
simultaneously. This deformity is known as polysyndactyly, or extra
joined fingers (or toes). This is a more difficult condition to correct due to length
of operation and time under anesthesia in children. Causes
& Symptoms of Syndactyly/Polydactyly
During pregnancy, and as the fetus is forming the appendages, the
cells in the area between the digits, are programmed to die
off. In persons with syndactyly or
polydactyly, these cells do not die off and the flesh (or bones, etc.)
remain connected or conjoined where they would normally become separate
appendages. In medically advanced civilizations the discovery of syndactyly/polydactyly
can be performed while the child is in utero by way of an ultrasound.
When a child is born, tests are performed with the presence of
polydactyly/syndactyly to determine if these deformities are symptomatic of
possible underlying disorders, conditions, problematic illnesses or simply
genetic. Blue Cross-Blue Shield has best explained it more than I ever
could therefore I will quote an article. Polydactyly is symptomatic of disorders
such as:
"...both Meckel and
Laurence-Moon-Biedl syndrome...Patau's syndrome, asphyxiating thoracic dystrophy, hereditary spherocytic hemolytic
anemia, Moebius syndrome, VACTERL association, and Klippel-Trenaunay syndrome." (Blue
Shield of California, Inc) "Syndactyly is a characteristic of Apert syndrome, Poland syndrome,
Jarcho-Levin syndrome, oral-facial-digital syndrome, Pfeiffer syndrome, and Edwards syndrome. Syndactyly may also occur with Gordon syndrome, Fraser syndrome, Greig cephalopolysyndactyly, phenylketonuria, Saethre-Chotzen syndrome, Russell-Silver syndrome, and triploidy." (Blue
Shield of California, Inc) "In some isolated cases of polydactyly or syndactyly, it is not possible to determine the
cause of the deformity. Some cases might be due to a genetic defect and sometimes there is too little information to
determine a specific, genetic cause. Some cases of polydactyly or syndactyly might be due
to external factors like exposure to toxins or womb anomalies such as tumors,
pressure, etc.. "When genetic reasons are present - this is normally caused by an autosomal dominant gene. This means that the gene is
not linked to a specific gender and males and females are equally likely to inherit the trait. This also means that since the gene is
dominant, children who have only one parent with the trait have a 50% chance of inheriting it."
Also "...people in the same family carrying the same gene can have different degrees of polydactyly or syndactyly. Polydactyly and syndactyly are also possible outcomes of a large number of rare inherited and developmental disorders. One or both of them can be present in over 100 different disorders where they are minor features compared to other characteristics of these diseases."
(Blue Shield of California, Inc)
Polydactyly & Syndactyly - mylifepath.com
California Blue Shield
Ratio per Birth:
Although if this deformity runs
in the family it my seem as though the ratio is higher than it actually is but
is actually isolated to a few families. Males will sometimes carry the gene more
often than females resulting in an increase of the presence of these deformities
in the male child. These deformities should be nipped in the bud so
to speak, as to prevent possible growth disturbances of the digits.
Syndactyly
in Burn Victims
Syndactyly can also occur in victims
of fires as the intense heat will literally melt the skin and fuse the epidermis
and dermis of the phalanges, or fingers/toes. The burn victims' syndactyly case
will essentially be less invasive because bone fusion is not present in these
cases. The general same techniques (Z plasty) are utilized in post-burn
syndactyly as with congenital syndactyly although the lack of viable tissue for
grafting may complicate matters. Just as well, the lack of functionality or
cosmetic concerns are issues worth correcting. Frequently Asked Questions About
Syndactyly/Polydactyly
Coming Soon! Please Check Back! Are
You A Candidate For Syndactyly/Polydactyly Repair?
Syndactyly/Polydactyly can be diagnosed by external observation,
X-ray and fetal sonogram. Syndactyly/Polydactyly can be very obvious and
can thwart function of the hand and/or create unnecessary teasing in children
and awkwardness or embarrassment in adults. Usually a surgeon will perform
surgery anywhere from 18 months to 5 years of age, although some surgeons may choose to wait until 6
years of age. Possible
hindrance of social development should be considered regarding children. Many
children and adults are teased and made fun of regarding their "webbed hands and
feet" or extra fingers/toes. Kids can be cruel so consider this before putting off surgery until
a later date. Besides, growth disturbances are possible if the fingers or toes
are restricted from proper growth and function. Just remember, all surgeons are
different in what age they prefer to operate - please consult with several
surgeons. What
To Expect At Your Consultation
After checking a surgeon's
background and credentials, you will make an appointment for a consultation.
You
will meet with this surgeon and discuss your goals and you will disclose all
information regarding your health; if you smoke, what medications or vitamins
you presently take. This is very important. Visit the Aspirin
& Supplement List for more information. You will discuss your complaints
and concerns and discuss the various techniques and implants, if needed,
available to you. Your surgeon will explain the technique and incision
placements and methods that may be most appropriate for you and should discuss
the Risks associated with
syndactyly with you, as well. Complete disclosure of medical
history is necessary as well as if the cause is unknown, blood tests will be
performed, as well as X-rays to determine phalange (finger/toe) complications
such as fused bone, tendons, etc. You may also be tested for underlying
disorders or conditions if you have not been tested prior. The underlying
possible disorders an be VERY serious, possibly even bring upon death at an
earlier age. Your surgeon
will discuss with you your options, available techniques for your particular
case as well as the risks and complications of syndactyly/polydactyly repair.
You will also discuss the
available anesthesia that will be used for your procedure. Most Syndactyly/Polydactyly
procedures are performed under Light Sleep Sedation although some docs may use
General Anesthesia -- especially in small children. Either way, discuss this
beforehand as most people are hesitant to go under General Anesthesia. If you do
go under General, you make sure that the anesthesiologist is certified!
Please read the All About
Anesthesia Page -- the risks regarding going under are a whole
different ball game entirely. I personally prefer Light Sleep as the risks
are lowered when General is excluded. Do realize though that thousands of
patients safely go under General every day.
This surgery is not a one size
fits all type of thing. Knowledge is power -- so research before your
consultation. You will also discuss your pre-operative instructions and speak,
at length, about the recovery period instructions and what to expect in the
months ahead. You will be given prescriptions for antibiotics, pain relievers,
perhaps blood pressure medicines, anti-flammatory drugs and perhaps a box or
directions for gaining a box of Arnica montana.
This is stronger than the stuff
you can get at any heath food store. I swear by this stuff, I used it for all 4
of my surgeries and I know others who have used it and their bruising was
practically non-existent and their swelling, minimal. I also used a topical
formulation, called Boiron, that can be bought through Drugstore.com.
Ask your doctor about these two
products. Would like to know more on the benefits of Arnica
montana?
If you would like more information on Consultations or a list of
questions to ask your surgeon please visit the Consultation
Help
Page
Preparing
For Your Surgery
You will be given a pre-operative information packet that explains everything
you should do and know before your surgery date. The packet should include
all the medications you should not take for up to 2 weeks before your surgery.
These medications will include, but are not limited to, aspirin containing
products. Would you like to view a typical Medication
& Supplements List? Also, your surgeon may or may not advise you of the
benefits of Arnica montana for swelling and bruising. Would you like to learn of
the benefits of Arnica
montana?
It is quite possible that you will have
preliminary blood work
performed. This is normally an extra out-of-pocket expense that the patient must
participate in to check your white and red blood cell count (complete blood
count, CBC) which ultimately alerts your surgeon to disease or disorders beforehand.
So many things to do... so little time. Surgery
will be here before you know it so visit the Preparing
For Surgery page and relax. This section contains, printer-friendly pre-op
lists, tips and advice as well as things you must do to prepare for your big
day.
How This Procedure Is Performed
After your consultation you will
decide which surgeon will be the one to perform your correction. The surgery is
most likely performed under General anesthesia for children and with either
General or IV Sedation with local for Adults. For syndactyly, the incision
lines will be marked, most likely the Z plasty, to prepare for the separation.
The Z Plasty (or zig zag) incision will separate the fingers so that the tissue
can be divided up equally and rearranged. The areas of skin will be sutures into
its new position and skin graphs taken from the groin will be used to fill in
the missing areas of skin. Although if your child is to be circumcised, it is
possible to wait for his hand repair if a skin graft is needed. *The skin from the
circumcision is a viable graft and the extra incision site is not needed. If it
is a complex case, there may be tendons to be re-suspended or attached. There
may be shared joints, bones, blood vessels and nerves as well as the visible
skin. That is why it is very important to consult with a hand specialist for
cases such as these. Hand function is a high priority and no skimping should
ever be practice. Although I don't have to say this as if it is a child
involved, parents are quite protective of their children. But, it never hurts to
warn others that a plastic surgeon must have a skilled background in
surgery and function of the hand.
*Below
is a reported case regarding viable penile skin graft in the male patient and
delayed circumcision:
"Summary: A boy who had simple syndactyly involving the third web space of the left hand presented for elective syndactyly repair. Circumcision had been delayed because of neonatal medical problems. Elective syndactyly repair and circumcision were performed in one operation at age 9 months. Penile foreskin was used as a full-thickness skin graft for the syndactyly repair. The foreskin provided a functional syndactyly repair with good aesthetic characteristics. This obviated the need for two separate operations and for an additional skin graft donor site. To our knowledge, this is the first reported case in which foreskin was used for the repair of syndactyly. In boys with syndactyly, the authors advocate that parents be informed of this reconstructive option. Should the parents consider it to be suitable, then elective circumcision should be delayed until the time of syndactyly repair so that foreskin may be used for the syndactyly repair.
Journal of Pediatric Surgery, S. D. Oates; A. K. Gosain VOL. 32, NO. 10: pp.1482-4"
If Polydactyly is present it may
be more in-depth than excising the extra digit. There maybe be shared joints and
vessels, nerves, bone etc. as in the Syndactyly correction. Polydactyly can be
as simple as snipping off the extra digit and suturing the wound. To a complex reconstruction
of the hand. As I said before please see a qualified hand surgeon to better your
chances of a good result. The
hand(s)/feet will be wrapped in a bulky, gauze dressing or hard cast
with the fingertips/toe tips sticking out, if possible. This way the tips can be
checked for proper circulation. The tips will appear cold and bluish or purple
if proper oxygen is not reaching the tissues.
The Road To
Recovery
Although one surgery is usually what is needed, it
is possible that an additional surgeon may be needed if the case was complex.
Usually you will be released on an out-patient basis although if this surgery is
performed on a child the anesthesiologist has full discretion over the release
of the child. Children are small and quite vulnerable to complications of
anesthesia so this is important to report any suspicious reactions
or behavior.
If polydactyly or syndactyly
are just cosmetic and not symptomatic of a condition or disorder, the outcome is
quite wonderful. If it is in fact symptomatic, the outcome will rely heavily on
the treatment of the disorder. Please take the possibility of a disorder
seriously. Many of the possible conditions and disorders are quite serious and
may even result in an early death. If your appendages were sharing a
fingernail it is quite possible that your fingernails will never look normal again so please consider the possibility as well in a
realistic expectation. There will be some swelling and
bruising so don't be alarmed. pain medications should alleviate any discomfort.
The bandages must remain for 2 to 3 weeks for proper healing and protection.
You
must keep the bandages clean and dry. If for a child, you must ascertain this as
well as children are more apt to soil or get their bandages wet. For smaller
children you must either explain to them and slip a large sock over the bandage
to protect it further. If you had a skin graft from the groin area, this area
may be unbandaged and exposed to water after 2 to 3 days with no need for
re-bandage. After about
10 to 14 days
you will have your post-operative exam and the wounds will me cleaned and the
function of he hand/toes will be sought. If everything looks up to par your surgeon
will instruct you to slowly try and move your hand/toes, although very gently.
Your sutures will not be removed as they will fall out on their own,
do not pick at them! Your skin grafts and hands/feet may become very dry
so it is encouraged to moisturize them with a good moisturizer like Lubriderm,
Nivea (which I use) or Diporbase. If the wounds and grafts dry out they will
possibly split and bring you much pain and a possibly re-do. In
the weeks ahead report any foul smell, severe pain (you will have shooting pains
as the nerve sensitivity returns), redness, sign of infection, blackened skin
(other than bruising and the skin graft may appear darker than the rest of the
hand/toes), yellowing of the skin, yellowish cottage cheese excretion, severe
tenderness or anything else out of the ordinary. Do not cleanse with peroxide or
antiseptics as this could destroy the new fibroblasts (collagen or scar tissue)
that your skin need to heal itself. Peroxide can actually bubble away new skin. Risks &
Complications Associated With Syndactyly/Polydactyly
Repair
The risks of this procedure include
anesthesia complications (especially General), post-operative
difficulty breathing, and sore throat from intubation. Also hematoma, bleeding,
skin graft necrosis (death), circulation or pressure damage from the bandages or
hard cast infection. Please alert your surgeon to any of these
symptoms as well as as fever, chills, cyanosis (bluish cast) excessive swelling,
numbness in the fingers/toes or tingling such as when your foot falls
asleep.
The Least You Need To Know
-
There are many types of congenital hand deformities, the most common being
polydactyly and syndactyly. The syndactyly deformity occurs 1 in
2,000
births, and the Polydactyly, 1 in every 1,000 but both are usually hereditary,
therefore increasing the chances in isolated families.
-
With Syndactyly, the fusion, or
webbing, of two
digits or phalanges (fingers, toes) is due to failure of separation during embryological development, usually between
the sixth and eighth weeks of intrauterine life. It can occur in hands and/or
feet and although it is more common in males, it can occur in females as
well.
-
In 1/3 of these cases, there is another member of the family with the same condition.
-
Polydactyly is the occurrence of extra digits (fingers or toes).
Polydactyly can
take the form of a barely discernible, undeveloped finger or toe to a fully developed extra,
functioning digit.
-
Polydactyly and syndactyly can be present
simultaneously. This deformity is known as polysyndactyly, or extra
joined fingers (or toes).
-
Syndactyly can also occur in victims
of fires as the intense heat will literally melt the skin and fuse the
epidermis and dermis of the phalanges, or fingers/toes.
-
You may also be tested for
underlying disorders or conditions if you have not been tested prior. The
underlying possible disorders an be VERY serious, possibly even bring upon
death at an earlier age.
-
The surgery is most likely
performed under General anesthesia for children and with either General or
IV Sedation with local for Adults. For syndactyly, the incision lines
will be marked, most likely the Z plasty, to prepare for the separation.
The
Z Plasty (or zig zag) incision will separate the fingers so that the tissue
can be divided up equally and rearranged.
-
If Polydactyly is present it
may be more in-depth than excising the extra digit. There maybe be shared
joints and vessels, nerves, bone etc. as in the Syndactyly correction.
-
There are risks of this
operation, as with any other operation -- do not think you are immune. Please
read the risk section regarding the possible negative outcomes.
-
Please
read the All About Anesthesia Page - the risks regarding
going
under are a whole different ball game entirely.
-
The Costs can be quite
extensive, usually depending upon the anesthesia choice and the age of the patient.
Averaging in private practices/public hospitals: $3,000. to $6,000. for a
primary surgery without complications.
Related Links
& Information (all
links leading out of the site will launch a new window)
Birth:
Polydactyly Photo
Birth: Syndactyly Photo
Blow Up of Syndactyly of the Bone
Syndactyly Release Surgery - UK
Complete Simple Syndactyly of All Digits With Polydactyly : A Rare Case
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605.
(888) 663-4637
http://www.modimes.org/
NIH/National Institute of Child Health and Human
Development
9000 Rockville Pike, Building 31, Rm 2A32, MSC 2425
Bethesda MD 20892
(301) 496-5133
Fax: (301) 496-7101
Congenital Deformities of the Hand
http://www.indianahandcenter.com/
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