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Failure of hallux MP preservation surgery for rheumatoid arthritis.

Eight patients underwent surgery on 15 feet for rheumatoid forefoot problems. Thirteen of the 15 feet that were operated upon had an attempt to preserve the hallux metatarsophalangeal joint while resectional arthroplasty was performed on the lesser MP joints. All of the 13 feet that had the MP joint preserved had a well-preserved joint space preoperatively and no active signs of inflammation at the time of this procedure. Eight feet underwent a distal Chevron osteotomy to realign the great toe, two feet underwent an IP fusion as only the IP joint had evidence of erosive changes, and one foot underwent a combination of a Chevron osteotomy and a proximal phalangeal osteotomy (Akin procedure). Two patients had no surgery on their first ray as it was well aligned with no evidence of erosive changes. Of the 13 feet that did not have a fusion performed, 11 had development of a valgus deformity or inflammatory erosions. The average time to failure was 24 months (range, six to 36 months). The Chevron/Akin procedure remained successful at 18 months and one of the IP fusions was successful at six years after surgery. Although patients with rheumatoid forefoot disease may on occasion have a well-preserved hallux MP joint with minimal or no deformity and no active inflammation, with severe lesser toe involvement, most of these patients will fail a surgical procedure which does not involve fusion of the hallux MP joint.

Arthritis, Rheumatoid↗

Functional aspects of Neandertal pedal remains.

The fossil foot remains of the Neandertals (immediate predecessors of anatomically modern humans in Europe and western Asia between about 100,000 and 35,000 to 40,000 years ago) indicate an overall pedal configuration for these prehistoric humans that was largely indistinguishable from that of modern humans. They had fully adducted halluces, longitudinal and transverse pedal arches, compact posterior tarsal regions, slight valgus deviation of the hallux, and abbreviation of the lateral toes. Their pedal remains differed from those of modern humans only in their tendency to be more robust and their relatively short proximal hallucial phalanges and associated elongated distal hallucial phalanges. The former is related to their habitual levels of physical activity, which were greater than those of modern humans, and the latter is a pleiotropic effect of a biomechanical adaptation in the pollex for strength during manipulation.

Adaptation, Biological↗

A unique point mutation in the fibroblast growth factor receptor 3 gene (FGFR3) defines a new craniosynostosis syndrome.

The underlying basis of many forms of syndromic craniosynostosis has been defined on a molecular level. However, many patients with familial or sporadic craniosynostosis do not have the classical findings of those craniosynostosis syndromes. Here we present 61 individuals from 20 unrelated families where coronal synostosis is due to an amino acid substitution (Pro250Arg) that results from a single point mutation in the fibroblast growth factor receptor 3 gene on chromosome 4p. In this instance, a new clinical syndrome is being defined on the basis of the molecular finding. In addition to the skull findings, some patients had abnormalities on radiographs of hands and feet, including thimble-like middle phalanges, coned epiphyses, and carpal and tarsal fusions. Brachydactyly was seen in some cases; none had clinically significant syndactyly or deviation of the great toe. Sensorineural hearing loss was present in some, and developmental delay was seen in a minority. While the radiological findings of hands and feet can be very helpful in diagnosing this syndrome, it is not in all cases clearly distinguishable on a clinical basis from other craniosynostosis syndromes. Therefore, this mutation should be tested for in patients with coronal synostosis.

Adult↗

Sarcoidosis presenting as nail dystrophy.

A 45-year-old woman was referred to the dermatology clinic for assessment of "refractory onychogryphosis." She had a 3-year history of lesions involving distal phalanges of the first and third of her left foot. Initially she described periungual erythema and swelling. Three weeks later she noted a whitish growth and thickening of her third toenail. X-ray films of the digit were reported as normal. Several months later the same changes occurred in her great toe. These lesions were asymptomatic. There was no history of trauma. Numerous fungal cultures were negative. No light microscopic examinations were undertaken. She had a trial of both topical and systemic terbinafine of 3-months duration with no clinical improvement. Several clinical opinions were obtained from two dermatologists, a surgeon, and a chiropodist. Past medical history of note was significant for tubal ligation, cervical cancer, and chronic sinusitis. The latter condition in retrospect was thought to be secondary to sarcoidosis. Physical examination revealed periungual violaceous discolouration of the first and third toes of the left foot. There was evidence of significant nail changes including dystrophy, onycholysis, and hyperkeratosis (Fig. 1). The fingernails were normal. There were no other skin abnormalities. A punch biopsy of the tip of the third toe showed granulomatous inflammation. There was evidence of hyperkeratosis, exocytosis, and a dense infiltrate composed of collections of histiocytes and a few giant cells forming granulomas (Fig. 2). Repeat x-ray films of the foot showed soft tissue swelling of the first and third digits. There was bony resorption in the distal phalanges with a lacey trabecular pattern compatible with sarcoidosis (Fig. 3). Chest x-ray films revealed marked hilar adenopathy. The patient was sent to a respirologist who concurred with the diagnosis of sarcoidosis. Further investigations included a low serum calcium of 2.07 mmol/L, serum ACE of 70 U/L (upper limit of normal is 75), Wintrobe erythrocyte sedimentation rate (ESR) of 10 mm per hour, thyroid stimulating hormone concentration of 0.65 mU/L, and a urinary calcium excretion rate that was elevated at 7.3 mmol/day. Pulmonary function tests were unremarkable. The patient was initially treated with clobetasol under occlusion and intralesional triamcinolone with minimal improvement. She was subsequently started on prednisone, 15 mg per os daily because of the lung and bone involvement with significant improvement noted in the toe lesions with diminution of both the swelling and violaceous discolouration.

Administration, Topical↗

The foot in Apert syndrome.

Foot deformity in Apert syndrome is very characteristic and predictable. Progressive synostosis occurs on what is presumably an unsegmented cartilaginous mass. The first ray shortens with medial deviation of the great toe, secondary to growth abnormality and a delta phalanx. The two phalanx digits characteristically go on to fusion, with maintenance of minimal motion at the metatarsal phalangeal joints. The midfoot and hindfoot progress to characteristic fusion in a supinated position. There is prominence of the fifth metatarsal with callosities under the fifth and third metatarsal heads in all patients. Orthotic and surgical management of these conditions is necessary to ensure maximal function in the symptomatic Apert patient.

Acrocephalosyndactylia↗

[Failure of Dwyer's procedure in internal pes cavus in children. Physiopathological considerations and therapeutic deductions].

The authors have performed 34 Dwyer's calcaneal osteotomies in children with pes cavus confined to the medial arch in non-paralytic lesions (poliomyelitis and spina bifida were excluded). In 12 cases it was associated with osteotomy of the 1st metatarsal or with plantar release. No arthrodeses were performed in these 34 cases. After an average follow-up of five years the results were doubtful: in 24 instances the deformity was the same or worse. In 21 cases a secondary operation was necessary. The authors considered that the Dwyer's procedure corrects only the varus of the heel which is a secondary deformity. They believe that, in pes cavus, there is a dynamic clawing of the toes in the swing phase of gait. This produces secondary deformities. In the sagittal plane there is a synergic or paralytic imbalance at the metatarso-phalangeal level leading to vertical displacement of the 1st metatarsal and deepening of the medial arch. In the frontal plane, this vertical displacement leads to an irreducible pronation of the forefoot with secondary varus of the heel. In the horizontal plane a lateral rotation of the talus results in varus of the calcaneum.

Adolescent↗