This blog is about macrodactyly, a rare condition affecting the feet and the hands.
It is still under costruction, but if you know information that could be helpful, please, contact us:
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Words in italics are explained in the K.I.S.S. Section (still under construction and continuosly being updated)
A few hints about the condition
Macro means larger than what is considered normal; pedal macrodactyly is a condition where the toe on a foot is growing more quickly than the other normally growing toes. It is a rare congenital disease, which means that you are born with it, although it doesn’t seem to run in families. Because the toe is larger than the others, it has extra fibrous fatty tissue on the bottom (the plantar part of the toe, causing it to curl upwards, the dorsal or top.)
Macrodactyly falls into one of two types: static or progressive, the most common type. Patients with static macrodactyly have larger toes (or toe) when they are born. It then continues to grow proportionally to the other toes. The other type, progressive, the toe grows faster than it should, given the growth pattern of the child. The condition seems to affect slightly more boys than girls, and it can occur on one foot only, on both feet, and it can be symmetrical or asymmetrical. Macrodacytly isn’t necessarily associated with any other type of deformity, although it can be found in people with some types of syndromes, such as Proteus syndrome, Banayan-Riley-Ruvalcabe, Maffuccin, Ollier’s disease, and Milroy’s disease.
Kelikian described a rarer type, which he named hyperostic. In that type, para-articular calcifications develop, which limit significantly the ability of the affected articulations, and its appearance seems to start later and it does not stop with the closing of the epiphyses.
The index digit is affected more frequently and, in decreasing order of frequency, the middle, the thumb, the fourth and the fifth digits follow. More than one digit are affected 2-3 times more frequently than one digit alone.
True macrodactyly must be distinguished from other forms of enlarged digits that are a result of tumours, inflammation and other specific tissue dysplasias such as hemangiomas, arterovenous shunts, congenital lymphedema, lipomas etc.
The confirmation of macrodactyly, obviously, is made radiographically.
Although the cause of macrocactyly isn’t known, there are theories, which include one hypothesis of a defect in a cell that causes certain areas of the body to become supersensitive to growth. Another theory is that it may be caused by an interaction of genetic and environmental factors. There may be hemangiomas, which are benign (noncancerous) tumors made of a mass of blood vessels. These may also cause enlargement of the toes. Another cause could be damage in the nerves, causing rapid overgrowth of the area.
When the affected toes of children are examined, it is seen that the subcutaneous fat, the fat just below the skin, looks like adult fat, not a child’s. As well, all the elements of the toe are larger (the tendons, nerves, blood vessels, fat, nails, skin and the bones in the toe. The exception is the bone in the foot that goes to the affected toe is not larger than normal. The skin on the affected toe is thicker than normal and the toe feels rubbery and soft.
Treatment for the disorder depends on several issues, particularly, if the toe or toes are causing pain or disability.
If surgery needs to be done, the surgeon could destroy the growth part of the bone, along with removing as much as the excess tissue as is possible. This part, however, called defatting, is done in a two-step process. The first step involves reducing the thickness on the convex side of the toe by 10 to 20 percent. The second step involves doing the same to the other side, and shortening the bone and removing excess skin.
For the repairing of the length, shortening osteotomies are recommended. Several shortening methods have been descibed. Barsky and Tsuge recommend the removal of a large portion of bone on both sides of the distal phalango-phalangeal articulation and arthrodesis.
In children, in order to arrest further enlargement of the digit, epiphysiodesis of all of the epiphyses of the affected digit is recommended. This operation, however, does not restrain the enlargement in width of the digit. Keeping in mind that macrodactyly is caused by an abnormality of the nerve, operations on the digital nerves are recommended, such as the stripping of the nerves from their branches or the removal of a part of the nerve and end-to-end anastomosis.
Amputation could be considered, but this is not common and is usually only used as a last resort. As well, amputations of the smaller toes do not seem to have as big an effect on the foot as with the larger toes, so this is also taken into consideration.
Management is generally difficult. Probably multiple operations would be required, and the ultimate outcome would be potentially not satisfactory. Both the surgeon and the patient should know this and should be prepared.