1/10. New type of synpolydactyly of hands and feet in two unrelated males.An 18-year-old Japanese boy and a 10-year-old Chinese boy both had nearly complete cutaneous syndactyly of the fingers and toes, six diphalangeal fingers on each hand, six toes on each foot (except the right foot of patient 2), and short, deformed, and on occasion partially fused metacarpals and metatarsals. Neither had other malformations and were of normal intelligence. The accessory toes in patient 1 were mesoaxial, each situated between the hallux and the third toe, whereas those in patient 2 were postaxial. In view of these findings, the disorder in 2 individuals is likely to represent a hitherto undescribed type of nonsyndromic synpolydactyly.- - - - - - - - - - ranking = 1keywords = hallux (Clic here for more details about this article) |
2/10. Anterolateral tibial bowing and duplication of the hallux: a rare but distinct entity with good prognosis.Congenital anterolateral bowing of the lower leg usually is a symptom of (impending) fracture because of congenital pseudarthrosis. This article reports a case of anterolateral bowing of the lower leg that is characterized by spontaneous correction and combination with duplication of the hallux. A review of the literature showed 10 comparable cases. This article is the first report on the magnetic imaging of this entity and describes its differentiation from congenital pseudarthrosis.- - - - - - - - - - ranking = 5keywords = hallux (Clic here for more details about this article) |
3/10. De novo GLI3 mutation in acrocallosal syndrome: broadening the phenotypic spectrum of GLI3 defects and overlap with murine models.acrocallosal syndrome (ACS) is characterised by postaxial polydactyly, hallux duplication, macrocephaly, and absence of the corpus callosum, usually with severe developmental delay. The condition overlaps with Greig cephalopolysyndactyly syndrome (GCPS), an autosomal dominant disorder that results from mutations in the GLI3 gene. Here we report a child with agenesis of the corpus callosum and severe retardation, both cardinal features of ACS and rare in GCPS, who has a mutation in GLI3. Since others have excluded GLI3 in ACS, we suggest that ACS may represent a heterogeneous group of disorders that, in some cases, may result from a mutation in GLI3 and represent a severe, allelic form of GCPS. The finding is important for counselling families with suspected ACS.- - - - - - - - - - ranking = 1keywords = hallux (Clic here for more details about this article) |
4/10. Pathomorphology and treatment of congenital anterolateral bowing of the tibia associated with duplication of the hallux.Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient.- - - - - - - - - - ranking = 4keywords = hallux (Clic here for more details about this article) |
5/10. rubinstein-taybi syndrome (RTS) with postaxial polydactyly of the foot: 4-year follow-up until improvement of dysbasia.rubinstein-taybi syndrome (RTS), also known as 'broad thumbs syndrome' or 'broad thumb-hallux syndrome', is a malformation syndrome characterized by the triad of broad thumbs or first toes, a peculiar facial expression called 'comical face' and mental retardation. Although various malformations are combined with the triad, polydactyly is rare. We treated a male patient with RTS complicated by postaxial polydactyly of the foot. His clinical course was different from typical patients with polydactyly, especially in the aspect of walking development. Osteoplasty-combined surgery, which was ideal for anatomical reconstruction, was performed on the patient at 2 years and 11 months of age. A 4-year follow-up period was required until there was an improvement of dysbasia.- - - - - - - - - - ranking = 1keywords = hallux (Clic here for more details about this article) |
6/10. Femoral hypoplasia-unusual facies syndrome with bifid hallux, absent tibia, and macrophallus: a report of a Bedouin baby.A male Bedouin baby with the clinical profile of femoral hypoplasia-unusual facies syndrome is described. The phenotype includes bilateral asymmetrical lower limb hypoplasia/aplasia with short remnants of both femora, absent right tibia, bifid right big toe, dysmorphic facies, thoracic/pelvic abnormalities, macrophallus, and bilateral cryptorchidism. This report re-emphasises the previously described rare association of femoral hypoplasia-unusual facies syndrome with preaxial polydactyly and suggests that the clinical spectrum of the syndrome could be stretched further to accommodate other unusual traits, for example, macrophallus and absent tibia.- - - - - - - - - - ranking = 4keywords = hallux (Clic here for more details about this article) |
7/10. Concomitant syndactyly and polydactyly in a pediatric foot.syndactyly and polydactyly in a child may warrant surgical treatment to avert resultant emotional and psychologic problems. The authors present a unique case report of a bilateral polydactyly of the hallux and bilateral syndactyly of the second and third toes in a normal 6-year-old Hispanic female. The article will discuss the surgical management of these problems, using the skin harvested from resection of the extra hallux for the adjacent side of the lesser toes after desyndactyly.- - - - - - - - - - ranking = 2keywords = hallux (Clic here for more details about this article) |
8/10. Grebe syndrome: clinical and radiographic findings in affected individuals and heterozygous carriers.Grebe syndrome is a recessively inherited acromesomelic dysplasia. We studied, clinically and radiographically, 10 affected individuals, originating from Bahia, brazil. The phenotype is characterized by a normal axial skeleton and severely shortened and deformed limbs, with a proximo-distal gradient of severity. The humeri and femora were relatively normal, the radii/ulnae and tibiae/fibulae were short and deformed, carpal and tarsal bones were fused, and several metacarpal and metatarsal bones were absent. The proximal and middle phalanges of the fingers and toes were invariably absent, while the distal phalanges were present. Postaxial polydactyly was found in several affected individuals. Several joints of the carpus, tarsus, hand, and foot were absent. Heterozygotes presented with a variety of skeletal manifestations including polydactyly, brachydactyly, hallux valgus, and metatarsus adductus. Grebe syndrome is caused by a missense mutation in the gene encoding cartilage-derived morphogenetic protein-1.- - - - - - - - - - ranking = 1keywords = hallux (Clic here for more details about this article) |
9/10. Congenital tibial aplasia with preaxial polydactyly. A case report.An anatomical study of a left lower limb with congenital tibial aplasia and preaxial polydactyly amputated at 10 months of age was carried out. The tibia was replaced by a fibrous band (a band of connective tissue) and there were four cuneiforms, six metatarsal bones and seven toes. The second metatarsal bone showed characteristics of the hallux. An intermuscular septum which showed an orifice for the anterior tibial artery was found on the medial side of the leg and foot. All the muscles of the leg and foot were present except for the tibialis posterior muscle, which was replaced by two atypical muscles. No muscular attachments reached the fibrous band. Several intertendinous connection bands were found. Also, an accessory muscular belly split from the tibialis anterior tendon and attached to the common flexor tendinous sheet of the foot. An unusual motor branch of the deep peroneal nerve ran together with this belly to supply the intrinsic muscles of the hallux. We also observed other minor anomalies of the nerve pattern. The arterial pattern was complete, except for some arteries which showed both an anomalous origin and course. The findings of this study suggest that the development of the skeletal elements plays an important role in the differentiation of the muscles, tendons, arteries and nerves. We postulate that a dysmorphogenic event involving the development of the tibial field of the limb could give rise to both defective histodifferentiation of the tibia and defective programmed cell death in the pre-hallucial anlage. These anomalies would determine secondary adaptations of muscles, tendons, vessels and nerves of the limb.- - - - - - - - - - ranking = 2keywords = hallux (Clic here for more details about this article) |
10/10. A polydactylous human foot with 'double-dorsal' toes.A human polydactylous left foot with 9 toes, amputated from an 11-mo-old child, was examined by x-ray and magnetic resonance imaging and by gross dissection to identify the digits. The normal sequence of toes from medial to lateral is 1, 2, 3, 4, 5. Examination of the morphology of tendons and muscles suggested the toe sequence was 1, 2, 3/4, ?5, 2, 3/4, 3/4, 5. The 2 toes in the sequence that are underlined were displaced dorsally and were found to have 2 extensor tendons, no flexor tendons and nails that were conical and situated at their tips. These toes resembled those described as 'double-dorsal' and which develop in paws of mice in which a gene normally expressed ventrally is functionally inactivated (Loomis et al. 1996). Specification of toe formation occurs in leg buds early in embryonic development and later there is rotation of the limb so that the anterior (rostral) part comes to lie medially, i.e. the hallux which was anterior (rostral) now is on the inner (medial) side of the foot. A disruption in the patterning of this foot in both anteroposterior (rostral-caudal) and dorsoventral axes during development could be responsible.- - - - - - - - - - ranking = 1keywords = hallux (Clic here for more details about this article) |
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