Macrodactyly of the foot is a rare but disabling condition. We present the results of surgery on 18 feet of 16 patients, who underwent ray amputation and were followed-up for more than two years at a mean of 80 months (25 to 198).

We radiologically measured the intermetatarsal width and forefoot area pre-operatively and at six weeks and two years after surgery. We also evaluated the clinical results using the Oxford Ankle Foot Questionnaire for children (OxAFQ-C) and the Questionnaire for Foot Macrodactyly.

The intermetatarsal width and forefoot area ratios were significantly decreased after surgery. The mean OxAFQ-C score was 42 (16 to 57) pre-operatively, improving to 47 (5 to 60) at two years post-operatively (p = 0.021). The mean questionnaire for Foot Macrodactyly score two years after surgery was 8 (6 to 10).

Ray amputation gave a measurable reduction in foot size with excellent functional results. For patients with metatarsal involvement, a motionless toe, or involvement of multiple digits, ray amputation is a clinically effective option which is acceptable to patients.

Cite this article: Bone Joint J 2015;97-B:1364–9.

Background: Proteus Syndrome is a rare, sporadic overgrowth disorder for which the underlying genetic defect remains unknown. Although the clinical course is well-described, there is no systematic histopathological description of the lesional pathology. Objective: To describe the histopathological features encountered in a series of patients with Proteus syndrome from a single centre. Patients/Methods: Patients with Proteus syndrome who had undergone therapeutic surgical resection or biopsy were identified from a database and the histopathological findings were reviewed, with particular reference to descriptive features of the underlying tissue abnormality. Results: There were 18 surgical specimens from nine patients, median age 4 (range 1–9) years, including four main categories; soft tissue swellings (lipomatous lesions), vascular anomalies (vascular malformation and haeman-gioma), macrodactyly (hamartomatous overgrowth) and others (sebaceous naevus and non-specific features). In all cases the clinical features of overgrowth were due to increased amounts of disorganised tissue, indicating a hamartomatous-type defect in which normal tissue constituents were present but with an abnormal distribution and architecture. Vascular malformations represented a prominent category of lesions, accounting for 50% of the specimens, predominantly comprising lymphatic and lymphovascular malformations. No malignancy or cytological atypia was identified in any case. Conclusions: The histopathological features of lesions resected from children with Proteus syndrome predominantly include hamartomatous mixed connective tissue lesions, benign neoplasms such as lipomata and lymphatic-rich vascular malformations

We report the results of the treatment of 23 patients with macrodactyly. Eighteen had a two-stage bulk-reducing (defatting) procedure; phalangectomy was used to shorten the digits. At a mean follow-up of nine years (2 to 12), two patients had been lost to follow-up, and three await a second-stage procedure. Good cosmetic correction was achieved in 12 patients, with satisfactory results in seven; two patients had poor results and required amputation

A case is reported of a young girl who presented with macrodactyly of the right middle finger and tumour masses on the palmar side of both interphalangeal joints. The lesions were fibrocartilaginous and appeared to be hyperplastic palmar plates. The macrodactyly and the hyperplasia of the palmar plates were attributed to trauma

Seven patients with macrodactyly in the foot are reported. None showed any stigmata of neurofibromatosis and all were found to have excessive accumulation of fibro-fatty tissue as the most striking pathological feature. It is suggested that this may represent the basic lesion in this condition. The literature is reviewed and attention is drawn to the differences between macrodactyly in the hand and in the foot

1. A patient with macrodactyly of the middle finger of the left hand was followed up from birth until the finger was amputated at the age of three. 2. The affected finger, besides showing gigantism at birth, grew at a faster rate than the normal fingers. The degree of bone maturation (as judged from the appearance and size of the phalangeal ossification centres) proceeded at a faster rate than the normal fingers. No vascular abnormality which could account for the gigantism was detected either radiologically or microscopically. The affected finger showed histological abnormalities of both bone and soft tissues

1. A case of local gigantism is described, with enlargement of the left thumb, the left ring finger, the right little finger, and the right second and third toes. 2. No other record can be found in the literature of an association of local gigantism of fingers of both hands with a similar condition of the toes of one foot, though there is no theoretical reason why macrodactyly should not affect the digits in any combination. 3. Theories regarding the etiology are discussed