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Results for “Hand Deformities”

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At least 703 records · Page 39Linked to original sources
PubMed1993

A morphological and family study on isolated terminal transverse type of congenital limb deficiency in Hungary, 1975-1984.

A population-based and validated data set of 195 cases with isolated terminal transverse-type congenital limb deficiency was evaluated in Hungary, 1975-1984. Terminal transverse types of congenital limb deficiency are not usually associated with non-limb defects, and typically only one limb is affected. Upper limb is more frequently affected than lower (9:1) in monomelic cases. The left side and females are affected more often in upper limbs while lower limb defects are evenly distributed between right and left sides and both sexes. Familial occurrence was not found.

Congenital Abnormalities↗
PubMed1974

Trisomy-8 syndrome.

Explore the source record for details and available documents.

Abnormalities, Multiple↗
PubMed1980

Monodactylous splithand-splitfoot. A malformation occurring in three distinct genetic types.

Monodactyly is a sign of at least 3 different types of autosomal dominant ectrodactyly. In the first type only the 1st and the 5th or only the 5th toes are present on both feet. The trait is fully expressed in all affected children of patients. No skipping of a generation has been observed. Both parents of several affected children may be normal, or one parent may show minor manifestations only. Single strand mutation is suggested as an explanation of these exceptional cases. Monodactyly is seen less frequently in the second type, ectrodactyly, ectodermal dysplasia and cleft lip and palate (the EEC syndrome), than in the first type. The limb defects are more variable. The third type of ectrodactyly shows extreme intrafamilial variability, comprising various degrees of ectrodactyly, monodactyly and adactyly, defects of the ulna and/or of the tibia. Minor manifestations often occur in affected children of patients. Skipping of a generation is not uncommon.

Abnormalities, Multiple↗
PubMed1984

[Plastic surgery reconstruction of late sequelae following burns of the hands].

Late consequences of burns of the hands can be divided into four types depending on their extent and the involvement of the underlying tissue structures. Of 88 hands operated on, 43% were Type I, 28% were type II, 25% were type III, and only 4% belonged to the mutilating type IV. Clinical examples of types I and III are shown. Type I requires operation on the skin only to restore full function of the hand, whereas type III also needs repeated surgical treatment of the dorsal apparatus, tendons, ligaments and joints to improve hand movement.

Adolescent↗
PubMed1994

Jackson-Weiss syndrome registered in four successive generations. The facies of Crouzon's syndrome with foot abnormalities.

A family with 15 individuals in four successive generations affected by Jackson-Weiss syndrome, craniosynostosis with Crouzon-variant-like phenotype and feet's abnormalities, is presented. An autosomal dominant inheritance pattern with complete penetrance, variable expressivity, and wide intrafamilial variation, more among, less within the same generation, was observed. Concerning the frequency and severity of complications, the evolution of craniofacial deformities seems to parallel those described with Crouzon syndrome, suggesting the similar evaluation and management.

Adult↗
PubMed1982

Constitutional narrowness of the lumbar spinal canal and cheiro-lumbar dysostosis.

The author reports a further case of cheirolumbar dysostosis (associated of Verbiest's constitutional narrowness of the lumbar canal and short and broad phalanges and metacarpals) and a case with multiple malformations including both the narrow lumbar canal and brachycheiry and also several other malformations of the bones. The author discusses the nosography of Verbiest's syndrome with regard to cheirolumbar dysostosis and to more complex and diffuse malformations.

Adult↗
PubMed1994

Post-axial hexodactily and single atrium: a new syndrome?

A 3 1/2 year old boy is presented with postaxial hexodactily in all extremities, a persisting large left vena cava superior (VCS) draining into the left part of a single atrium and a mild dextroversio cordis. All extra fingers and the lateral parts of the fused metatarsals were amputated. At age 10, the cardiovascular anomalies were surgically corrected successfully by separating the atrium with a pericardial patch and by draining the left VCS to the right atrium with a graft. The patient seems to represent a new syndrome, since the combination of postaxial hexodactily with single atrium had previously been observed by the author in another patient in 1970.

Angiocardiography↗
PubMed1987

Radiologic findings in the Proteus syndrome.

The radiological findings in two patients with the Proteus syndrome are described. Features in our two cases not previously mentioned or stressed include vertebral dysplasia and enlargement (megaspondylodysplasia), bilateral genu valgum, recurrent after surgery and intraabdominal and mesenteric lipomatosis. Emergency laparotomy was performed on the first patient who had a twisted necrotic portion of mesenteric fat. Macrodactyly, skeletal muscle atrophy and subcutaneous fat accumulation in the abdominal wall were present in both. In addition the second patient was mentally retarded and had frontal bony prominence of skull. Computed tomography was used for the specific diagnosis of the lipomatous tissues in both patients.

Bone and Bones↗
PubMed1987

The Proteus syndrome: the magnetic resonance and radiological features.

The Proteus syndrome is a recently delineated group of skeletal and mesodermal malformations. Its characteristics include hemihypertrophy and fatty/lymphangiomatous masses. This description outlines the imaging sequences available to the radiologist. It is mainly concerned with the use of magnetic resonance imaging. This shows the extent of the mesodermal malformation with particular reference to the extent of intra abdominal infiltration.

Child, Preschool↗