A Literature Review of Pedal Macrodactyly

Abstract

Macrodactyly is a congenital disease where the digits affected increase in size faster than can be attributed to normal growth of unaffected digits. Its etiology is ambiguous and hereditary patterns do not play a role. The abnormality develops in one or more toes and involves thickening of soft tissues, bone and accumulation of fat. The accepted treatment is reduction of the fibro-fatty bulk via dissection and ablation; the major aim being reconstruction of a pain-free functioning foot. Complications with surgery include delayed wound healing and inadequate initial de-fatting which could require a more proximal amputation.

The use of the Tsuge procedure for pedal macrodactyly

“…Any procedure that eliminates the toenail may be considered a form of amputation, which worsens the cosmetic abnormality of the foot. Goals of surgery should be the establishment of normal digital length, reduction of excessive soft-tissue bulk, and, ideally, preservation of the toenail through a minimum number of operations [4,7,12,15,17]. As no two cases are identical, treatment must be on an individual-case basis.…”

“…Early reports noted the association with fibrofatty accumulation [1,3,6]. Association with a variety of neurogenic disorders has been described, especially neurofibromatosis [7,8]. In-utero disruption of growth-limiting factors has also been proposed as causative [3,7].…”

“…Pedal macrodactyly is often of the progressive variety [7,9,12]. There is generally a slight male majority [2,5].…”

“…Association with a variety of neurogenic disorders has been described, especially neurofibromatosis [7,8]. In-utero disruption of growth-limiting factors has also been proposed as causative [3,7]. Although macrodactyly is most commonly an isolated finding, there are many known associations with secondary macrodactyly or pseudomacrodactyly, such as acromegaly; fibrous hamartoma of infancy; Von Recklinghausen's disease; Proteus syndrome; Bannayan-Riley-Ruvalcaba syndrome; Maffucci syndrome; Ollier's disease; Milroy's disease; and KlippelTrénaunay-Weber syndrome, among others [7][8][9][10].…”

“…The primary goal of treatment is to produce a pain-free, functional foot that can accommodate normal shoes; in addition, the cosmetic appearance of the foot, and particularly the nail, is of great concern to the patient and family [4,7,15]. Treatment options include, but are not limited to, debulking/ ablation of fat and other hypertrophied tissues; epiphyseodesis or physeal resection; reduction syndactyly; assorted osteotomies (including phalangeal shortening, and interphalangeal and metatarsophalangeal joint resections); interphalangeal and metatarsophalangeal joint arthrodeses; phalangectomy (particularly middle phalangectomy); amputation (various levels); and ray resection; numerous combinations are also utilized [3,4,6,8,9,13,[15][16][17].…”