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[Polydactyly of the foot].

Polydactyly may be preaxial or tibial (hallux-side), postaxial or ulnar (side of the little toe) and central (middle toes). The duplication may appear at the distal and medial phalanges or at the whole digit. The metatarsal bone may be part wise or completely duplicated, the accessory toes may share only one metatarsal. Surgical intervention may be indicated in shoe problems, for esthetic reasons or, especially in duplication of the metatarsales, because of secondary deviation of the toes and therefore shoe problems or plantar callosities. Preoperative analysis including x-ray is of great importance to achieve good functional and cosmetic results.

Adolescent↗

The Coffin-Siris syndrome. A report of four cases and review of the literature.

Four additional cases of the Coffin-Siris syndrome bring the number of reported cases to 16. This disorder is characterized by the absence or hypoplasia of the nails, especially those of the fifth fingers and toes, growth retardation and mental deficiency, microcephaly, coarse facial appearance, sparse scalp hair and lax joints. Feeding difficulties and respiratory problems are common in infancy. Absence or hypoplasia of the distal and middle phalanges, especially those of the fifth finger and toes and retarded bone maturation are the most common radiological features.

Abnormalities, Multiple↗

Intraosseous epidermoid inclusion cyst in a great toe. A case report and review of the literature.

Epidermoid inclusion cysts are benign lesions that occasionally occur in the distal phalanges of the fingers but are less frequently identified and underreported in the toes. We describe a 55-year-old man with a history of work-related trauma followed by painful expansion of his right great toe, resulting in great anxiety. Imaging studies revealed a radiolucent lesion in the distal phalanx of his right hallux. Clinical differential diagnoses included the possibility of an intramedullary inclusion cyst and other various radiolucent lesions. During surgery, a cystic lesion that contained creamy material was discovered. Frozen section diagnosis of the lesion was an intraosseous epidermoid inclusion cyst. The lesion was removed and the patient recovered uneventfully. Although it has been reported that an unduly large number of phalangeal cysts have been treated by amputation, the judicious use of intraoperative frozen sections can prevent this scenario.

Bone Cysts↗

Treatment of lateral ray polydactyly of the foot: focusing on the selection of the toe to be excised.

The purposes of this study were to evaluate the results of the operative treatment of lateral ray polydactyly and to consider appropriate surgical procedures, especially focusing on the selection of the toe, lateral toe or medial toe, to be resected. Twenty-two patients with lateral ray polydactyly foot (25 individual feet) at an average of 71 months' follow-up were included in this study. Cases were classified morphologically into three types on the basis of Hirase's configuration. In addition, these types were divided into two subtypes, metatarsal and phalangeal, on the basis of radiographic evaluation of the level of duplication. The clinical evaluations of the reconstructed toe were performed, and these results were investigated according to their morphologic classification and excised toe group. The distinctive problem of medial toe excision is valgus deformity. Eight of 25 toes retained persistent valgus deformity, and all of these cases were in the medial toe excision group. On the other hand, a distinctive problem in lateral toe excision is postoperative pain. Two patients suffered from postoperative pain in phalangeal type cases in the lateral toe excision group, and the remaining medial toe had a medial protuberant middle phalanx. The pain occurred at that protuberant point. Based on their experiences, the authors created an algorithm for selection of the toe to be excised. In metatarsal type cases, from a functional perspective, the toe that has a radiographically dominant metatarsus should be retained. On the other hand, in phalangeal type cases, the authors give priority to shape rather than function, and they excise the morphologically smaller toe independent of the condition of the phalanx as viewed on radiography. If the medial toe and the lateral toe are approximately the same size, the authors excise the lateral toe to avoid valgus deformity. When the lateral toe has severe valgus deformity that seems unlikely to be correctable intraoperatively, the lateral toe should be considered for excision even if it is larger than the medial toe.

Adolescent↗

Free thin osteo-onychocutaneous flaps from the big toe for reconstruction of the distal phalanx of the fingers.

Free osteo-onychocutaneous flaps from the big toe have frequently been used for the reconstruction of distal phalangeal loss of the fingers. The thickness of the fatty tissue, however, makes these flaps bulky. We believe this disadvantage can be overcome with a free thin osteo-onychocutaneous flap from the big toe and consider this thin composite flap to be a superior method of reconstruction for the treatment of distal phalangeal loss.

Adolescent↗

Deformities of the smaller toes and surgical treatment.

According to the anatomical changes, deformities of the smaller toes often are referred to as hammer toe, club toe, and claw toe. In this article a new deformity, which often appears as inflammatory arthritis, is described. In this toe deformation, which the author calls zig-zag deformity, there are contractures at the metatarsophalangeal joints, and at the proximal and distal interphalangeal joints. Between January 1983 and June 1985, 70 patients with small-toe deformities were treated surgically: 64 by partial phalangeal resection and 6 by tendon transfer with the Girdlestone technique. The results are presented in this article.

Foot Deformities↗

Triggering of the lesser toes at a previously undescribed distal pulley system.

We have treated five patients with metatarsalgia and triggering of the lesser toes. This paper describes a mechanism of triggering that involves a previously undescribed flexor pulley system in the toes that is very similar to the pulley system in the fingers. The specific anatomy of the toe flexor pulleys is described based upon the dissections of 50 fresh-frozen cadaver toes. These pulleys were composed of transverse to obliquely oriented fibrous bands within the flexor sheaths from the metatarsal heads to the distal phalanges. We found a system of pulleys in the lesser toes and the great toe, analogous to that found in the hand. Trigger toe is a rare entity and is only briefly described in the literature. We believe it is more common than is now recognized and may be an important underdiagnosed cause of metatarsalgia. The diagnosis is made when active plantarflexion causes the toes to catch in flexion and the patient is then unable to extend them. Nonsurgical therapy consisting of steroid injection, NSAIDs, or changing footwear may be effective, otherwise surgical release of the A1 pulley may be required.

Adult↗

Congenital sensori-neural deafness associated with onycho-osteo dystrophy and mental retardation (D.O.O.R. syndrome).

A characteristic syndrome is described in which congenital sensori-neural deafness is associated not only with onychodystrophy but also with congenital bony anomalies the most characteristic of which are tri-phalangeal thumbs, bi-phalangeal digits of hands and feet, and dystrophic terminal phalanges of some of the fingers and toes. In addition, there is mental retardation and the dermatoglyphics are characterized by the presence of 10 arches and elevation of the atd angles. The syndrome is inherited as an autosomal recessive. It is suggested that this entity be named the D.O.O.R. Syndrome because of the deafness, onychodystrophy, osteodystrophy and retardation. A similar syndrome without retardation as described by Goodman et al. (1969) appears to be inherited as an autosomal dominant.

Abnormalities, Multiple↗

Brachydactylia with symphalangism, probably autosomal recessive.

Association, in one patient, of the following malformations: brachydactylia of all segments but terminal phalanges; proximal symphalangism of many fingers and toes; abnormalities of carpal and tarsal bones; partial duplication of both big toes; mild hypertelorism. Genetic transmission seems to be recessive autosomal.

Abnormalities, Multiple↗

Antley-Bixler syndrome: a disorder characterized by congenital synostosis of the elbow joint and the cranial suture.

The Antley-Bixler syndrome is a rare disorder characterized by craniosynostosis, midface hypoplasia, radiohumeral synostosis, joint contractures, arachnodactyly, and femoral bowing and fractures. We report four cases with this disorder, all of which had craniosynostosis, midface hypoplasia with characteristic facial appearance, and contractures of bilateral elbow joints. However, femoral bowing, fractures, and arachnodactyly were not seen in our patients. In addition, proximal phalanges of the thumb and the great toe showed deformity of the delta phalanx in two cases. Characteristic features in these cases were the synostotic deformity of the elbow joint; three had radioulnahumeral synostosis, and one had radioulnar synostosis. Therefore, our cases indicated that various synostotic patterns of the elbow joints may exist in this syndrome. It is reasonable to propose that characteristic craniofacial appearance associated with the synostosis of the elbow joints of various forms should be considered minimal diagnostic criteria of the Antley-Bixler syndrome.

Child, Preschool↗

Bony anomalies in a patient with reticulate acropigmentation of Kitamura.

Reticulate acropigmentation of Kitamura [RAPK] is a rare pigmentary genodermatosis. This is a case report of a 23-year-old Saudi female who started manifesting the disorder in the 2nd decade. The patient had bony abnormalities in the form of absence of terminal phalanges of the 2nd, 3rd and 4th toes. These bone anomalies are extremely rare, and the association has never been described before in the literature.

Acrodermatitis↗

Lethal course of X-linked dominant chondrodysplasia punctata in a male newborn.

We report a newborn with some manifestations of chondrodysplasia punctata. Additional abnormalities were hydrocephalus, bilateral syndactyly of the fourth and fifth fingers and toes, absence of the middle phalanx of all toes, hypoplasia of the second and third phalanges of all fingers and cryptorchidism. This observation suggests that we are possibly dealing with a rare male case of X-linked dominant chondrodysplasia punctata.

Abnormalities, Multiple↗

Reconstruction of the thumb by free skin flap of the big toe.

Experience with 12 cases of thumb reconstruction is reported, utilizing a free skin flap from the big toe and remodelled iliac bone graft, to create metacarpal and phalangeal configuration. Results show that the reconstructed thumbs have good functional recovery, cosmetic appearance, and varying degrees of sensation. The procedure preserves the big toe of the donor foot and produces minimal impairment.

Amputation, Traumatic↗

Autosomal recessive mesoaxial synostotic syndactyly with phalangeal reduction maps to chromosome 17p13.3.

Previously we have described a novel and distinct form of non-syndromic osseous syndactyly segregating in an autosomal recessive pattern in a consanguineous Pakistani family. The limb findings include mesoaxial reduction of the fingers, synostoses of the third and fourth metacarpals with associated single phalanges, fifth finger clinodactyly, and preaxial webbing of toes. We identified another published report of this phenotype in a large, inbred Turkish family. In the present study we mapped the phenotype in the Pakistani and Turkish families to chromosome 17p13.3 (multipoint LOD score 5.1). The identification of a single locus for this complex limb malformation in two families with distinct ethnic backgrounds supports the hypothesis that this is a distinct form of syndactyly. Since this form of syndactyly is phenotypically distinct from the previously described eight types, we propose to name this phenotype mesoaxial synostotic syndactyly with phalangeal reduction (MSSD, type IX syndactyly, Malik-Percin type).

Abnormalities, Multiple↗

Bone growth is induced by nail transplantation in amputated proximal phalanges.

Mammals are able to regrow the tips of amputated fingers and toes. However, regrowth is limited to regions covered by, and is dependent upon, the presence of the nail organ. If the nail organ is responsible for bone growth in digit-tips, we reasoned that transplanted nail organ might also be able to induce outgrowth from other levels of the digit. Partial nail organ has been transplanted to amputated proximal phalanges of young rats. To date, six transplants have successfully produced outgrowth of nail. New bone growth, not seen in control amputated digits, was documented by x-ray and by alizarin red and calcein injections to be directed toward implanted nail organ. These results support an inductive role for nail organ epithelium in bone growth after amputation and provide encouragement for attempts to enhance a positive outcome after appendage amputation.

Amputation, Surgical↗

Reconstruction of the distal phalanx of the fingers by free toe-to-hand transfer.

Anatomical examination of the blood-vessels in the distal phalanges of the foot show that the arteries and veins of this region are thick enough to be anastomosed by microsurgical means. This makes it possible to perform free toe-to-hand transfer, reconstructing defects of the distal phalanx and suturing arteries and veins at the level of the middle or even distal phalanxes. Eleven distal parts of the fingers have been reconstructed. All the transfers were successful, and good cosmetic and functional results were obtained.

Adolescent↗

Operative repair of fourth and fifth toe corns.

METHODS: We report a retrospective review of 57 consecutive patients (72 feet) over a period of 20 years who had been treated operatively for either a lateral fifth toe corn or an interdigital corn of the fourth interdigital space more than two years previously. Of these, 51 patients (62 feet) returned for a follow-up evaluation at a minimum of two years (average of over seven years) which included a review of the interval history since the surgery, a physical examination, a radiographic evaluation, and assessment of the patient's satisfaction with the alignment and results of surgery. Treatment of 31 lateral fifth toe corns involved either a lateral condylectomy and flexor tenotomy or a complete condylectomy. Treatment of 31 interdigital corns comprised either a single condylectomy, double condylectomy of adjacent corns, or a complete condylectomy (hammertoe repair) of a symptomatic corn. Treatment in each case was dependent upon the severity of the deformity. RESULTS: There was found to be no significant difference in comparison of the two major groups (interdigital corns and lateral fifth toe corns) with the measurement of the relative length of the fourth and fifth metatarsals, toe malalignment, angulation of the fourth and fifth toes (MTP-4, MTP-5 angles), and the phalangeal-5 angle. Pain was relieved in 58 of 62 feet (93%) and subjective acceptable alignment was achieved in 54 of 62 feet (87%). At final follow-up 53 feet were rated by patients as excellent, seven as good, one as fair, and one as poor. Complications included numbness of the involved digit (six feet). There were two superficial infections. There were two cases of joint instability due to excessive bone resection. Joint stiffness was commonly observed (34/62 feet, 55%), but was not associated with diminished satisfaction at final follow-up. Mild asymptomatic recurrence of a callosity was noted in 10 feet and moderate or severe recurrence was noted in two feet. Dissatisfaction was associated with moderate or severe recurrence. CONCLUSION: In this retrospective study at an average of more than seven years, we achieved a high level of patient satisfaction treating both lateral fifth toe corns and interdigital corns with a partial and/or complete condylectomy, the choice depending upon the magnitude of the deformity and the callus, and the fixed nature of the lesser toe deformity.

Adult↗