LOCALIZED GIGANTISM ( MACRODYSTROPHIA LIPOMATOSA )

 

•Localized gigantism refers to focal enlargement of part of the body, and is always pathological.

Broadly localized gigantism can be divided into two forms :

§static: the region is overgrown but proceeds to grow in proportion to the rest of the body

§progressive: the enlarged region grows faster than the rest of the body

A number of conditions can result in localized gigantism, including:

q neurofibromatosis type 1

q Klippel-Trenaunay-Weber syndrome

q lymphangiomatosis

q haemangiomatosis

q macrodystrophia lipomatosa

q proteus syndrome

 

•Macrodystrophia lipomatosais a rare form of localized gigantism, and many terms have been used interchangeably, with resulting confusion. They include macrodactyly, megalodactyly, digital gigantism, macromelia, partial acromegaly, macrosomia and limited gigantism.

•Macrodystrophia lipomatosa is essentially analogous to static localized gigantism

•This condition typically presents with painless enlargement of the 2nd or 3rd digit of the hand or foot.  It is usually unilateral but may involve adjacent digits in the distribution of the median or plantar nerves. Occasionally it involves the entire limb.

CLINICAL FEATURES:

This condition typically presents with painless enlargement of the 2nd or 3rd digit of the hand or foot.  It is usually unilateral but may involve adjacent digits in the distribution of the median or plantar nerves. Occasionally it involves the entire limb. 

•Recognized associations include:

•syndactyly

•clinodactyly

•polydactyly

 

PLAIN RADIOGRAPHS:

•Plain films usually suffice and demonstrate splayed, lengthened and broadened phalanges with endosteal and periosteal bone deposition.

•The overlying soft tissues are markedly overgrown, especially in the volar and distal aspects. Within the soft tissues, focal lucent areas representing fat may be seen, which is characteristic.

 

  TREATMENT:

•Although macrodystrophia lipomatosa is considered a progressive form of macrodactyly (i.e. the growth of the affected parts is faster than the rest of the body) growth halts at puberty