Early Surgical Repair of Macrodactyly

Abstract

We report a case of macrodactyly of the foot in a 3-year-old girl. The standard treatment for this condition has been ablation. Studies of phalangeal resection, phalangeal amputation, syndactylization, digit shortening, tissue debulking, and osteotomy have shown varying results. In the case reported here, debulking combined with a shortening osteotomy was the treatment of choice. Although the pediatric patient may require additional surgeries because the deformity will continue to grow, early treatment has allowed this child the benefit of a functional, cosmetically appealing foot that can be fitted with normal footwear.

A Literature Review of Pedal Macrodactyly

“…In a case study by Lagoutaris, early treatment of macrodactyly is discussed and could be viewed as a better option. Most patients that came in for treatment for macrodactyly were found to be in their late teens or adults; 13 although the condition is rare, an earlier treatment would benefit the patient in the future. A pediatric patient will not only benefit from an aesthetically pleasing foot, which will allow him or her to avoid any emotional distress, but will also be privileged to be fitted with normal footwear.…”

The use of the Tsuge procedure for pedal macrodactyly

“…The first known series on pedal macrodactyly was published in 1925 by Feriz [1]. It is generally held that toe macrodactyly is less common than finger macrodactyly [2,3]. Because of this rarity, most reports on pedal macrodactyly include case reports or small case series [4].…”

“…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].…”

“…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]. Although each procedure has its respective indication, there is no consensus as to the best procedure for any given type of macrodactyly [4].…”