Wednesday, July 8, 2009

Macrodactyly

Macrodactyly is an uncommon anomaly of the extremities.  It can affect both the fingers or toes which become abm=normally large due to overgrowth of the tissues composing the digit.  All the tissues are involved:   bone and soft tissue-particularly the nerves, fat and skin.  (photo credit)

Other names used for macrodactyly include megalodactyly, overgrowth, gigantism, localized hypertrophy, or macrodactylia fibrolipomatosis.

Hands are more commonly involved than feet. Most of the time (~90%) patients present with unilateral (one side affected) macrodactyly.  Often more than one digit is involved.   The most frequently involved digits of the hand are the index finger, followed by the long finger, thumb, ring, and little fingers.  Syndactyly may be present in 10% of patients.   Men are more often affected than women.

It is not known why macrodactyly occurs.  It does not appear to be an inherited anomaly, but there are some syndromes (ie Proteus Syndrome, Maffuci syndrome, and tuberous sclerosis) which may be associated with enlarged digits. There are some surgeons who believe that macrodactyly is a variant of neurofibromatosis. 

Macrodactyly may be either static or progressive.   The progressive type is more common than the static.

  • In static the enlarged digit (finger or toe) is present at birth and continues to grow at the same rate as the normal digits of the hand.  The involved digits are generally about 1.5  times the normal length and width of the normal digits.
  • In progressive the affected digits begin to grow soon after birth and continue growing faster than the rest of the hand.  The involved digit or digits can become enormous.

There is no medical treatment for this disorder.  It is treated by surgery.   In the hand, the indications for surgery can often be cosmetic in nature as the hand can functionally tolerate a digit with some increased width and length.  In the foot, the enlarged digit can make shoe fitting/wearing difficult.

Surgical treatment of macrodactyly is complex as multiple tissue layers are involved.  It typically will involve debulking, epiphyseal arrest, and shortening.  Multiple surgeries are the norm.

Soft tissue debulking:

  • This is done to help correct the width of the digit. This is often done at the same time as the epiphyseal arrest.   The affected fingers are approached volarly with Bruner-type incisions/flaps. The fat is removed from the skin and the tissues are debulked.
  • Care is taken to preserve the ulnar and radial digital neurovascular bundles. Sometimes the enlarged nerve branches will need to be sacrificed along with the enlarged subcutaneous tissues.
  • When a sufficient amount of tissue has been removed, the skin flaps are overlapped and excised, which allows for tension-free closure.  It needed, skin grafts using healthy skin will be done.
  • Debulking is often need to be done in staged procedures.

Shortening procedures:

  • This is done to help correct the length of the digit.  Shortening procedures usually involve either surgical excision (removal) of one of the phalanges of the finger or toe, or removal of a metacarpal (hand bone) or metatarsal (foot bone).  
  • Barsky and Tsuge originally introduced the two most described methods. Barsky’s technique involves removing the distal portion of the middle phalanx and proximal portion of the distal phalanx, thereby reducing the length of the finger while preserving the nail. Tsuge’s technique also preserves the nail by overlapping the dorsal portion of the distal phalanx with the volar portion of the middle phalanx.

Ray resection:

  • This may be done in progressive macrodactyly.  It involved the complete removal of the digit or digits.  It is also an option if there is excessive widening of the forefoot, where digital shortening and debulking procedure may not be effective.

Epiphyseal Arrest:

  • The timing of the this surgery is critical.  An attempt to “guess” the adult finger length is done by comparing the child’s digits with those of his/her parents.  When growth of the affected digits matches those of the parent, epiphyseal arrest can be performed.   This in effect will stop the bone growth of the digit.
  • The epiphyses of the proximal and distal phalanges  are the ones treated by disruption or removal.   The middle phalanx epiphyses is not treated to help preserve motion at the proximal interphalangeal joint.

Other surgical options include amputation and wedge osteotomies.  Amputation is reserved for patients with nonfunctioning digits or digits that are extremely difficult to correct.  Wedge osteotomies are performed in patients who have digits that are grossly deviated.

 

Complications of macrodactyly surgery include poor healing of flaps secondary to devascularization or undue tension, nerve injury or decreased sensation, infection, stiffness, bony nonunion or malunion, and failure of the epiphysiodesis.

 

 

REFERENCES

Wood VE. Macrodactyly. In: Green DP, Hotchkiss RN, eds. Operative Hand Surgery. 4th ed. New York, NY: Churchill Livingston; 1998:533-544.

Treatment of macrodactyly;  Plast Reconstr Surg. 1967; 39:590-599; Tsuge K.

Congenital anomalies of the hand; Cohen M, ed. Mastery of Plastic and Reconstructive Surgery. New York, NY: Little, Brown and Company; 1994; Upton J, Hergrueter C.

Macrodactyly; Boston Children’s Hospital website

Macrodactyly; Wheeless’ Textbook of Orthopaedics

 

 

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14 comments:

3healthymonkeys said...

A minor point - Maffucci is with two c's. This is a nicely written and informative article. Thanks!

SeaSpray said...

Thank God for docs who can help people with these things.

That must be so difficult for them. I guess they adapt for use, but it is sad.

I've never heard of this before.

Carla Daniel said...

I have been looking for help for my son he was born with this and I have had the worst time in life trying to find someone who knew how to help him. He is now 24 and has an enormous left foot and would like surgery to heal this.
Can you help me?

rlbates said...

Carla, I think I would look for a plastic surgeon who does hand surgery or try your local/regional Children's Hosp (ortho or plastic department).

AJ said...

That made excellent reading. Thank you.

Aritul said...

Admittedly, many times I'm skeptical of the work plastic surgeons do. Then, I am reminded of problems such as these and am happy that medicine has advanced so that we have doctors who can fix problems such as these.

michelle said...

Thank you so much for your blog!I have a 14 year old daughter that has this condition in her left hand.It affects her index and palm.She also has a huge benign tumor in her wrist that is combinded with the nerve.She had surgery at the cleveland clinic three years ago.69 stitches from her wrist to the tip of her finger to debulk.The surgeon couldnt do anything about her wrist.She is a absolute wonderful,beautiful child.She has been a cheerleader for five years.She competes in competitions and is and avid tumbler on her squad.She has been experiencing some hand pain lately and we are starting to question another surgery.My question to you is do you think the digit will continue to grow throughout her life or does it stop at a certain age?Thank you so much and blessings michellefincham@blogspot.com

rlbates said...

Michele, I don't know how to answer that question. It would be best answered by someone who knows exactly what was done in the earlier surgery (so get those records for the new doc) and who can examine her hand now including current x-rays. Best to you both.

michelle said...

Thank you so very much!I do actually still have her x-rays and mri on a cd.Blessings michelle

Anonymous said...

Michelle, I hope all works out for your daughter. It takes an incredibly brave young lady to achieve so much while facing this sort of challenge. I admire your daughter. I have this condition in my left hand as well, and also had a benign tumor in my neck that had to be operated on. I went throughout high school hiding my problem because I was so ashamed. But now that I am a few years older, I realize...it could be worse. It could always be worse. For your daughter and I, we may not feel lucky. But to the person who has deformities all over their body that causes malfunction in everything or simply to the person who can't wear their shoes-your daughter and I are very lucky. Sometimes, it's easy to overlook the important things. I was operated on in Texas Children's Hospital and Shriners. Both are excellent hospitals, but I still have much work to be done. My doctor told me that around age 18 when my finger slowed growth, that I could get plastic surgery. But I haven't been back yet to get it worked on because I am in college now at age 20 and I'm a busy person. I have to take off college to have my operation. Anyways, good luck with everything. From a stranger to a stranger, God bless and I hope all good things happen for your courageous daughter.

Leebee said...

I have a 3yo old son with macrodactyly of his left foot (affecting his 2nd and 3rd toes). We have been advised to go with amputation although are reluctant to do so without attempting debulking first. The growth to his foot since birth has increased, but this appears consistent with the fact that he too has grown!
We are in Australia and willing to travel to find someone who may be able to help. We have been seeing an orthopaedic surgeon although I was thinking that a plastic surgeon would be more suitable.
Any suggestions?

rlbates said...

Not very familiar with physicians in Ausralia but am aware of a good plastic surgeon, Dr Jill Tomlison, at Hunter New England Area Health Services

Sarah said...

My 5 month old daughter was diagnosed with Macrodactyly. I am trying to determine if hers is static or progressive. Her enlarged toes have been staying about the same percentage larger than the other toes. Does that mean that the growth will remain static or could they all of a sudden start growing faster than the other digits?

rlbates said...

Sarah, it is impossible for me to predict whether your daughters toe growth remain static or suddenly increase. I would encourage you to establish a relationship with a pediatric plastic or orthopedic surgeon who has experience or interest in macrodactyly.