(Macrodactyly)

Very rare. Isolated gigantism of one or more fingers or toes. The phenomenon involves an increase in volume of all elements (bone  and soft tissue) of the enlarged area; there is often an important fatty infiltration at the palmar or plantar level. This exaggerated growth continues through puberty. It could be that this malformation is due to a mutation of the PIK3CA gene localized to the affected tissues (mosaicism).

Classifications:

• either according to the involved tissue (Flatt classification):

* type I: lipofibromatosis or true macrodactyly

* type II: associated with neurofibromatosis (local plexiform neurofibroma)

* type III: associated with a digital hyperostosis (rare)

* type IV: in the context of hemihypertrophy of the body

• either on the basis of the associated signs:

* syndromic

* non-syndromic

(1) real macrodactyly: Flatt type I

(2) pseudomacrodactyly: linked to a disease that increases the volume of the finger or toe: vascular malformation, enchondromatosis, amniotic bands etc...

Anesthetic implications:

none; according to the associated systemic disease or syndrome.

References:

-         Fitoussi F, Ilharrebode B, Jehanno P, Frajman P et al. 
Macrodactylie. 
Chirurgie de la main 2009 ; 28 : 129-37.

-         Babajanyan A, Nanan R, Liu A. 
Isolated gigantism of digits. 
J Pediatr 2014; 165:874.

Updated: October 2014