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At least 235 records · Page 13Linked to original sources
PubMed1994

Use of silastic sheet in Apert's syndactyly.

Patients with Apert's syndrome usually have severe complex syndactyly affecting both hands. Surgical separation of the distal digital bony fusion often exposes bone and cartilage which can cause problems with graft take. A case is presented where the bare divided bone between two digits was kept apart with a silastic sheet. On its removal 4 weeks later, the previously bare bone was covered with a membrane. This was covered with a full thickness skin graft with a complete graft take.

Acrocephalosyndactylia↗
PubMed1994

Tissue expansion for the treatment of complete syndactyly of the first web.

Tissue expansion is a well-established technique for the management of soft tissue deficiencies. In congenital hand surgery the construction of an adequate first web is paramount. We used tissue expansion in four hands in three patients with complete complex syndactyly of the first web space. Two of these patients had Apert's syndrome and the other an isolated mitten hand anomaly. The expander is preferably placed early in life so that first web construction is completed in the first year. Tissue expander ports are left exposed. There have been no infections, flap or expander loss in our series.

Acrocephalosyndactylia↗
PubMed1996

Prenatal detection of the separation of the great toe, toe syndactyly, and large bilateral choroid plexus cysts in a fetus with trisomy 18.

Prenatal sonographic presentation of toe deformities is associated with fetal aneuploidies. This report presents a second-trimester fetus with large bilateral choroid plexus cysts, clenched hands, separation of the great toe, toe syndactyly, abnormal double maternal serum biochemical screening results, and trisomy 18. We suggest a careful ultrasound screening of the fetal limbs and other organs once a choroid plexus cyst has been identified. If abnormal sonographic findings are present, or if the results of the maternal serum biochemical screening are abnormal, karyotyping should be recommended.

Abnormalities, Multiple↗
PubMed1986

Early surgical intervention in Apert's syndactyly.

The hand deformities in Apert's syndrome are among the most complex congenital hand problems to reconstruct due to the skeletal malalignment, symphalangism, and progressive functional deficits with growth. Ten children with Apert's syndactyly underwent early surgical intervention (mean age 8.4 months) as part of a staged program of digital separation leading to completion of both hands by the age of 2 years. The incidence of major complications was low, rate of revision (18 percent) acceptable, and the aesthetic result very satisfactory. Long-term functional results tend to reflect the effect of progressive intrinsic disease despite preservation of skeletal alignment and digital form with growth. However, the aesthetic improvement and potential for greater functional gains at a young age support the concept of early surgical intervention in this syndrome.

Acrocephalosyndactylia↗
PubMed1997

Macrocephaly with cutis marmorata, haemangioma and syndactyly--a distinctive overgrowth syndrome.

We describe nine children with a similar pattern of features including macrocephaly and cutis marmorata telangiectatica congenita. All were large at birth and had a distinctive capillary haemangioma involving the philtrum and upper lip. The seven who survived all developed hydrocephalus and had developmental delay. Six developed body asymmetry and three had internal arteriovenous malformations. Syndactyly of the second and third toes and/or the third and fourth fingers or toes was commonly seen. All of the cases were sporadic. This condition is easily recognizable and should be considered in the differential diagnosis of patients presenting with overgrowth and macrocephaly.

Abnormalities, Multiple↗
PubMed1980

Craniosynostosis and syndactyly: expanding the 11q-- chromosomal deletion phenotype.

A patient with a partial deletion (q23 leads to qter) of the long arm of chromosome 11 presented with craniosynostosis and syndactyly. These characteristics, which have not been previously reported with 11q--, expand the phenotype of this syndrome and emphasise the need for chromosome analysis with banding techniques in multiple congenital anomaly syndromes, even if the patient could be classified as having a non-chromosomal syndrome.

Child, Preschool↗
PubMed1978

["Apert's foot" (in acrocephalo-syndactyly) (author's transl)].

The authors discuss foot abnormalities in Apert's syndrome (acrocephalo-syndactyly). Characteristic of these deformities are bilateral, mostly symmetrical syndactlys of the toes; incorrect hyperextension positions of the first toe with large toes in flexed positions; intermetatarsal synostoeses, above all between the first and second metatarsal bones; multiple synostoses in the tarsal region; bi- and monophalanges with pseudo-, delta- or arcuate epiphyses. A classification of Apert's foot is presented, using 68 examples from the literature and 12 cases treated by the authors. The calssification is analogous to the hand deformities in Apert's syndrome. Three types can be distinguished: In type I, toes II-IV are webbed together. However, the large and small toes are still well separated. Syndactilys of toes II-V are present in type II, and all toes have grown together in type III. The deformity can be accompanied by stress pain and difficulties in providing shoes. In this case surgical correction is indicated in childhood: the incorrect position of the large toe is eliminated. Good results can be achieved via osteotomies of the metatarsal bones and resections of the intermetatarsal bone bridges.

Abnormalities, Multiple↗
PubMed1979

Oligodactyly and multiple synostoses of the extremities: two cases in sibs. A variant of Cenani-Lenz syndactyly.

Two sibs with a rare phenotype of oligodactyly with metacarpal, carpal, radioulnar, and metatarsal synostoses and shortening of the forearms were born of healthy parents. Genetic counselling with regard to future children of one of these sibs was given on the assumption of an autosomal recessive inheritance, which is highly probable, judging from the other rare familial cases found in the literature.

Adult↗
PubMed1984

A fortuitous donor site for full-thickness skin grafts in the correction of syndactyly.

The ideal donor site for full-thickness skin grafts to the non hair-bearing areas of the fingers is the glabrous skin of other digits. The use of these sites is usually restricted because of the limited amount of skin available and the donor site morbidity. We were recently presented with a clinical situation in which one congenital abnormality of the fingers requiring full-thickness skin grafts for its correction was associated with another congenital abnormality of the digits: amputation of the supernumerary fingers and toes provided the ideal donor site for the full-thickness skin grafts needed in the hand.

Amputation, Surgical↗
PubMed1976

Syndactyly and split hand.

Careful analysis of clinical and radiological features of both webbing of normal fingers and typical split hand, suggests that these two types of malformations should not be classified separately. Study of human embryos and experimental malformations of the apical ectodermal ridge, supplies a resonable explanation for the close relation between webbing of normal fingers and typical split hand.

Female↗
PubMed1992

Nonadjacent syndactyly in the congenital constriction band syndrome.

Hand anomalies in the amniotic band sequence are frequently both complex and difficult to explain in terms of our conventional understanding of limb embryogenesis. A case manifesting pseudosyndactyly between nonadjacent fingers confirms the role of an in utero disturbance after the seventh week, in concert with close apposition between nearby but not adjacent parts.

Amniotic Band Syndrome↗