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PubMed1989

The preoperative use of extra-tissue expander for syndactyly.

To our knowledge, this is the first report of a method of stretching the interdigital skin of syndactyly by means of a pincer. The pincer, a U-shaped metal spring plate with a sponge cushion, is applied before the operation for about two months; pressure is placed against both sides of the interdigital fusion to expand the skin and to develop hollows. Use of the pincer facilitates a syndactyly operation by allowing defect coverage with a local flap only. Four patients successfully treated by this method are reported. The method has the great advantage of not requiring skin grafts, which often have unfavorable results both functionally and cosmetically.

Child↗
PubMed1999

Use of reverse triangular V-Y flaps to create a web space in syndactyly.

The authors describe a new technique for division in syndactyly. The web space is reconstructed using two reverse V-Y island triangular flaps. The flaps are raised both on the dorsal and the palmar aspect of the hand. This technique does not require the use of a skin graft. Fourteen syndactylies in 9 children-three incomplete (two congenital and one secondary to burn) and six complete-were treated using this technique. The results after a maximum 4-month follow-up and the advantages of the technique are discussed.

Adolescent↗
PubMed2000

A new technique for the treatment of syndactyly with osseous fusion of the distal phalanges.

In treating complex syndactyly of the hand, including bony union of varying degrees, how to cover the exposed bone surface is still disputable. The authors adopted a homemade device for the treatment of a case of syndactyly with terminal bony union. External tissue expander theory was applied to their device, and expansion of the cutaneous bridge was followed by division of the fused distal phalanges. Two months later the raw bone could be covered simply with the expanded local skin, and no skin graft was needed except for at the base of the affected fingers. This technique produces good cosmetic results of the fingertips and nail grooves without secondary deformities.

Humans↗
PubMed1996

Reconstruction of incomplete syndactyly of the toes using both dorsal and plantar flaps.

A new modified method for surgical correction of simple incomplete syndactyly of the toes has been devised. This procedure involves both a dorsal and a plantar skin flap and does not require a skin graft. Reconstruction of syndactyly using only skin flaps situated near the toes almost always results in skin deficiency. Use of a plantar flap from the sole provides sufficient skin for one toe and can be closed primarily. Fourteen cases involving 11 patients have been treated by this method, which is described herein.

Child, Preschool↗
PubMed1998

V-Y dorsal metacarpal flap: a new technique for the correction of syndactyly without skin graft.

A new technique for web reconstruction in congenital syndactyly is described. Twenty-one web spaces in 12 patients were treated using this method. The dorsal metacarpal flap was used as an island V-Y advancement to cover the newly created web space, thus avoiding skin graft in this space. A follow-up of 6 months to 2 years showed neither recurrence of the deformity nor web creep of any degree. The operation is rapid, easy, and reproducible. It is suggested that this technique can be used in all types of syndactyly.

Adolescent↗
PubMed2004

Cenani-Lenz syndactyly in a patient with features of Kabuki syndrome.

We report a 5 9/12-year-old male with Cenani-Lenz syndactyly (CLS). He presented with three malformed digits on each hand and bilateral 2,3 partial cutaneous toe syndactyly. The expression of this presumed autosomal recessive disorder is variable and has been confused with split hand foot malformation (SHFM). Not all CLS patients show the typical 'spoon hand' configuration originally described. Affected CLS patients show a disorganized appearance of the phalanges with some degree of syndactyly/oligodactyly, in addition to metacarpal and carpal fusions. Radioulnar synostosis may be present. Cases lacking radioulnar synostosis may show proximal dislocation of the radial heads. The distal limb anomalies seen in our patient resemble other reported CLS patients. Radiographic evaluation showed a disorganized appearance of the phalanges, a 'kissing delta phalanx' of one proximal phalanx, metacarpal fusions, carpal coalitions and posterior subluxation of the radial heads. Clinical examination revealed features suggestive of Kabuki syndrome including large, prominent ears, sparse, medially flared and arched eyebrows, wide palpebral fissures, long eyelashes, and oligodontia with flat head 'screwdriver-shaped' incisors, in addition to persistent finger and thumb-tip pads. The faces of CLS patients are rarely shown or described. However, some patients in published reports have large, prominent ears. A recent report suggested midfacial dysmorphism in two patients with CLS (). Although the findings of CLS with features of Kabuki syndrome in our patient may be coincidental, the faces of CLS patients need to be carefully evaluated and described to determine if there is a distinctive accompanying facial phenotype.

Bone and Bones↗
PubMed2004

Dorsal metacarpal island flap in syndactyly treatment.

Web space reconstruction is an important component of syndactyly treatment. There are several methods for a skin graft-free reconstruction. Previously, the dorsal metacarpal island flap based on a direct cutaneous branch of the dorsal metacarpal artery has been used as an island V-Y advancement for web defects in syndactyly. In this study, dorsal metacarpal artery flap was raised similarly as an island but was used instead as a transposition flap in a series of 19 web defects. Early results of a median (range) follow-up of 3 (1-5) years reveal neither recurrence of the deformity nor web creeping. Such utilization of the flap enables a better use of the skin territory proximal to the pedicle and a more comfortable reconstruction of the web space.

Adolescent↗
PubMed2005

Temporal skin grafts following straight incision for syndactyly correction.

For syndactyly correction, combined use of zigzag incision and full-thickness free skin grafts and many modifications have been reported. However, postoperative scars produced by this method on the dorsum and palm are conspicuous, and pigmentation of the grafts can be distressing for patients. In syndactyly correction, not only functional but also esthetic improvement is required. Therefore, we devised a new method, consisting of a straight incision and free skin grafts that are serially excised afterward. The scars after the serial excision are placed on the midlateral lines. Although this method necessitates at least 2 operations, the final scars are minimal, and functionally and cosmetically good results are obtained.

Humans↗
PubMed1977

A family with syndactyly type II (synpolydactyly).

Syndactyly Type II is reported in eight members of a family in four generations. Affected individuals show two distinctive patterns of variation in the expression of the gene. Distortion of dermatoglyphic patterns is associated with the severe but not the mild manifestation of the malformation. The diagnostic significance of minimal features of the condition is discussed. Linkage data suggest that loci for Syndactyly II and for blood-group antigens ABO, MNSs, P, Rh and Kell are not closely linked.

Adult↗
PubMed1984

Renal agenesis as a diagnostic feature of the cryptophthalmos-syndactyly syndrome.

Four families with the cryptophthalmos-syndactyly syndrome are reported. Nine affected patients died in perinatal period. Autopsy, performed in 6 cases, revealed renal agenesis (bilateral in 3 and unilateral in 3 cases). These observations together with recent literature data suggest that renal malformations (agenesis or severe hypoplasia) are one of the most common features of the cryptophthalmos-syndactyly syndrome and may serve as one of diagnostic criteria for this entity.

Abnormalities, Multiple↗
PubMedDate not supplied

Ectrodactyly, soft-tissue syndactyly, and nodulocystic acne: coincidence or association?

We report severe nodulocystic acne in a 21-year-old man associated with ectrodactyly of the right foot and soft-tissue syndactyly of the third and fourth left fingers, and the first to fourth left toes. His acne was resistant to conventional topical (clindamycin phosphate, erythromycin, tretinoin, peeling agents) and systemic (tetracycline, erythromycin) antiacne medications. Moderate improvement was achieved with systemic isotretinoin. Apart from presenting this case, we imply the disparity of the clinical characteristics of our case and those of Apert syndrome, a rare congenital condition with craniofacial anomalies, symmetric syndactyly of the digits, and acneiform eruption. We discuss the possible explanation for the association of acne lesions and bone deformities based on recent reports of mutations of fibroblast growth factor receptor 2 in the great majority of patients with this syndrome, as well as current experimental data on the involvement of the keratinocyte growth factor in the process of hair follicle growth, development, and differentiation.

Acne Vulgaris↗
PubMed1986

Type II syndactyly or synpolydactyly.

A new family with syndactyly type II or synpolydactyly is described with 16 affected members in six generations. No other major skeletal or extraskeletal malformations were present, but the association with minor local anomalies may be a common feature. Various metacarpal or metatarsal abnormalities may be part of this type of syndactyly. The family pedigree confirms the autosomal dominant mode of inheritance with incomplete penetrance and the frequent occurrence of non-manifesting heterozygotes resulting in 'skipped generations'.

Adult↗
PubMed1995

A large Turkish kindred with syndactyly type II (synpolydactyly). 2. Homozygous phenotype?

Syndactyly type II (synpolydactyly (SPD)) is an autosomal dominant condition with typical abnormalities of the distal parts of both upper and lower limbs. We report here a previously undescribed phenotypic feature of people with severe hand and foot deformities who were born to two affected parents. This is the first example of SPD subjects manifesting a very distinctive phenotype, suggesting that they must be homozygous for this condition. The typical characteristic clinical features in these subjects are as follows: (1) short hands with wrinkled fatty skin and short feet; (2) complete soft tissue syndactyly involving all four limbs; (3) polydactyly of the preaxial, mesoaxial, and postaxial digits of the hands; (4) loss of the normal tubular shape of the carpal, metacarpal, and phalangeal bones, so as to give polygonal structures; (5) loss of the typical structure of the cuboid and all three cuneiform bones while the talus calcaneus and navicular bones remain intact; (6) large bony islands instead of metatarsals, most probably because of cuboid-metatarsal and cuneiform-metatarsal fusions; and (7) severe middle phalangeal hypoplasia/aplasia as well as fusion of some phalangeal structures that are associated with the loss of normal phalangeal pattern. We report seven subjects with this phenotype from three different branches of a very large SPD pedigree exhibiting the same phenotype with minimal variation. In mice, the Polysyndactyly (Ps) mutation shows a pattern of synpolydactyly very similar to that of human SPD, suggesting that they may well be homologous mutations. A molecular genetic study is currently under way to determine the chromosomal location of the SPD locus in humans and to identify the corresponding homologous region in mice.

Adult↗
PubMed1996

An autosomal dominant syndrome of renal and anogenital malformations with syndactyly.

We describe a family with autosomal dominant inheritance of anal anomalies, renal tract abnormalities, genital malformations, and syndactyly. These clinical manifestations do not clearly fall into any previously described syndrome. A mother and daughter had almost identical congenital malformations, short stature, and unusual facies. The proband was born with anal stenosis, a rectovaginal fistula, clitoral hypertrophy, a pelvic right kidney, and syndactyly of both feet. Her daughter had the same anal, clitoral, and foot anomalies, a solitary pelvic kidney, and no fistula. This family is likely to represent autosomal dominant inheritance of a new combination of malformations, which may overlap with the Townes-Brocks syndrome, but does not fall into a current diagnostic category.

Anal Canal↗
PubMed1998

Presence of the Apert canonical S252W FGFR2 mutation in a patient without severe syndactyly.

Apert syndrome, characterised by craniosynostosis, craniofacial anomalies, and symmetrical syndactyly of the digits (cutaneous and bony fusion), has been associated with two canonical mutations in the FGFR2 gene (S252W, P253R) in the great majority of cases. Since these two alterations have been observed exclusively among these patients, it has been suggested that the S252W and P253R changes may play an important role in the occurrence of syndactyly. In order to verify whether the mutations S252W and P253R could also cause a milder phenotype, without involvement of the limbs, we have screened 22 patients with clinical characteristics compatible with Crouzon or Pfeiffer syndrome for these two particular changes. Surprisingly, we identified a Pfeiffer-like patient with the mutation S252W, and therefore we have shown for the first time the occurrence of one of the canonical Apert mutations without severe abnormalities of the upper and lower extremities.

Acrocephalosyndactylia↗
PubMed2004

Dorsal pentagonal island flap: a technique of web reconstruction for syndactyly that facilitates direct closure.

Though many techniques have been described for the correction of syndactyly, current methods result in skin deficiency that requires skin grafting, especially at the finger bases. We discuss the technical aspects and document our experience with the dorsal pentagonal metacarpal island flap for reconstruction of the web commissure in a series of 12 patients (22 webs) with syndactyly of varying complexity. In appropriately selected cases, this technique can improve reconstruction of the web commissure, facilitating direct closure, minimising the need for skin grafts and offers the potential for continued growth. This reduces operative time significantly and simplifies post-operative wound care. In this series, all our cases healed primarily in two to three weeks with minimal donor site morbidity. There were no post-operative complications, although the prominent dorsal scar remains a concern. At an average follow-up of 33.7 months, no cases of contracture or web creep after correction were noted.

Adult↗
PubMed2006

The bovine aristaless-like homeobox 4 (ALX4) as a candidate gene for syndactyly.

The ALX4 (aristaless-like homeobox 4) gene encodes a paired-type homeodomain transcriptional activator and plays a major role in anterior-posterior pattern formation during limb development. Here, the cloning, genomic structure and expression of the bovine ortholog of the ALX4 gene are reported. The bovine ALX4 gene consists of four exons and is located on BTA15q28-->q29 in a region syntenic to HSA11p11.2. The transcribed ALX4 mRNA encodes a 397-amino-acid protein showing a paired-type homeodomain and a C-terminal stretch of amino acids known as the OAR- or aristaless domain. The predicted protein shares 92.5% identity to human and mouse ALX4 proteins and all three species share almost complete identity in the conserved domains. ALX4 expression was detected by reverse transcriptase polymerase chain reaction in bovine fetal limb bones. The ALX4 gene was evaluated as a candidate gene for bovine syndactyly which has been mapped on the telomeric region of cattle chromosome 15. Sequencing of the four exons with flanking sequences of the bovine ALX4 gene from a panel of 14 affected animals belonging to German Holstein, German Fleckvieh and crossbreds, and 27 unaffected individuals from German Holstein revealed five silent SNPs within the coding region out of eleven SNPs in total. Four SNPs were polymorphic in the affected animals, but in comparison to the genotyped unaffected individuals the genotype distribution showed no evidence for an association to the phenotype. Therefore our data indicate that the ALX4 gene can probably be excluded as candidate gene for bovine syndactyly in the examined animals.

Amino Acid Sequence↗