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PubMed1977

Surgical treatment of simple syndactyly.

During the period 1963--74 a total of 20 operations for simple syndactyly were performed on 34 fingers. A dorsal rectangular flap and thick split skin grafts were regularly used except in 2 cases where full thickness grafts were used for covering the skin defects. There were no secondary operations. The period of observation after 13 operations was 4 years or more. It is concluded that the dorsal rectangular flap technique combined with thick split skin grafts give satisfactory results.

Child↗
PubMed1993

Bilateral syndactyly. A unique case with surgical correction.

Cases must be treated on an individual basis, appreciating the complexity of the syndactyly, considering the patient compliance, and understanding the goals of the surgical correction. Extensive discussion with the patient and parents must occur to clarify the goals of the surgery, the expectations, and possible complications. Careful preoperative planning and incision design is paramount in obtaining satisfactory results. Skin grafting may be required, either full-thickness from a variety of donor sites or split-thickness grafting as in one case study reported. Adjunctively, manipulation and stretching of the web space for 2 to 3 months preoperatively may be helpful to achieve more laxity of the soft tissues. The choice of suture material is of particular concern when dealing with a small child. It is usually wise to use an absorbable suture material for skin closure in a small child to prevent undue emotional stress to the child or even further anesthesia upon suture removal. Vascular compromise caused by soft tissue tension in not an infrequent occurrence. As with any surgery that addresses largely cosmetic deformities, there is no substitute for exact prior planning, meticulous technique, and surgeon experience to optimize results.

Child, Preschool↗
PubMed1999

Sturge-Weber syndrome with bilateral congenital syndactyly: a previously undescribed association.

The Sturge-Weber syndrome consists of unilateral port-wine haemangioma of the face which may be associated with an ipsilateral intracranial haemangioma and choroidal angioma. The common derivation of the meningeal, choroidal and facial vessels may explain a congenital malformation of all three areas. I report the case of a child with typical Sturge-Weber syndrome who had a previously undescribed association with bilateral congenital syndactyly.

Child, Preschool↗
PubMed1999

Anterior chamber eye anomalies, redundant skin and syndactyly--a new syndrome associated with breakpoints at 2q37.2 and 7q36.3.

We report a 34-year-old female with a de novo balanced reciprocal translocation involving 2q37.2 and 7q36.3. She has a unique combination of multiple congenital malformations that include redundant skin, complete tissue syndactyly of the hands and feet, hirsutism, polycystic ovaries and bilateral anterior chamber eye anomalies. Her son has inherited the unbalanced product (46,XY,der(2) t(2;7)(q37.2;q36.3). He has a similar clinical picture with additional features including complex congenital heart disease, post axial polydactyly, hypotonia and global developmental delay. The breakpoints may indicate the location of the gene(s) responsible for this unique combination of features.

Abnormalities, Multiple↗
PubMed2000

A variant of Cenani-Lenz type syndactyly.

Cenani-Lenz syndrome is a form of complete syndactyly resembling the spoon hand in Apert syndrome. It is often associated with disorganization of phalanges, the fusion of metacarpals and mesomelic shortness of the arm. We describe an additional case of Cenani-Lenz type of syndactylism in a 16 month-old girl who also has rib and vertebral abnormalities and mixed type hearing loss, that have not been seen before in a single patient.

Abnormalities, Multiple↗
PubMed1999

Two sibs with microcephaly, hygroma colli, renal dysplasia, and cutaneous syndactyly: a new lethal MCA syndrome?

We report two sibs of Turkish descent with multiple congenital anomalies including severe microcephaly, hygroma colli, cystic renal dysplasia, and bilateral cutaneous syndactyly of toes IV-V. In addition, the second sib presented with bilateral fusion of the eyelids, a bicornuate uterus, and clitoromegaly. The parents are first cousins, which suggests autosomal recessive inheritance. In reviewing previously published reports, several cases were found with cerebral, renal, and digital anomalies as the main features. Several of the additional symptoms present in the second sib were suggestive of Fraser syndrome, but the severe microcephaly in both sibs is unusual. The differential diagnosis is discussed, including the possibility of an entirely new entity in the broad spectrum of syndromes with cerebral, renal, and digital anomalies.

Abnormalities, Multiple↗
PubMed2001

[Surgical treatment of hand syndactyly in Apert syndrome].

Authors describe 6 cases (3 women, 3 men) affected by Apert syndrome (acrocephalosyndactyly). Clinical material has been documented since 1953. They concentrate on the description of individual clinical cases on the basis of objective and radiograph examination of patients. They describe surgical procedure of syndactyly between the thumb and 2nd-5th digits on both hands. Prior to 1998 they started with separation of digits at the age of 3-5 years. After 1998 they began with it as early as at the age of 3-6 months. For the reconstruction of lateral aspects of separated digits and for the creation of inter-digital space they use "zig-zag" incision and for covering the resulting skin defects they use skin transplants. During separation of 2nd-4th digits they extirpate all phalanges of 3rd digit and they leave metacarpal III in situ by which they create a three-digit hand with a relatively good functional result.

Acrocephalosyndactylia↗
PubMedDate not supplied

Familial microtia in four generations with variable expressivity and incomplete penetrance in association with type I syndactyly.

Familial microtia with external ear canal atresia and conductive deafness is rarely reported. Autosomal dominant and recessive inheritance have been suggested depending on various family reports. Cases with other malformations in addition to microtia have been described, although the microtia generally is an isolated finding. Here we report a family with microtia, external auditory canal atresia and conductive deafness in four generations. The mode of inheritance of the disease was autosomal dominant within this family. Also, variable expressivity, incomplete penetrance and generation skipping are evident in the pedigree. Association of microtia with type I syndactyly, which has never been reported previously, was present in the index case.

Child, Preschool↗
PubMed2004

[Epidemiological analysis of syndactyly in Chinese perinatals].

OBJECTIVE: To study the epidemiological features of syndactyly (SD) in Chinese perinatals. METHODS: Data were collected through Chinese Birth Defects Monitoring Network, a hospital-based congenital malformation registry system. From 1987 through 2001 (except 1994, 1995), all live or still births with 28 weeks of gestation or more born in participating hospitals were assessed within 7 days after delivery. RESULTS: Totally 2311 perinatals with SD were identified among 7 478 746 births, and 57.2% of them were in association with other anomalies. The overall prevalence rate of SD was 3.09/10 000, the rate of isolated SD and associated SD was 1.32/10 000, and 1.77/10 000 respectively. The prevalence rates in urban and rural areas, in male and female births were 3.22/10 000 and 2.79/10 000, 3.42/10 000 and 2.59/10 000 respectively. An increasing trend was found during that period. The perinatal fatality rate of SD was 20.7%, that of isolated form was 5.7%, while that of associated form was 31.9%. The proportion of SD occurring in right side was the same as that in left side, and the proportion of SD in upper limbs equaled to that in lower limbs. CONCLUSIONS: The prevalence rate of SD in Chinese perinatals was similar to that reported in foreign literatures. Associated form of SD was more frequently seen. The prevalence of SD in urban areas was higher than in rural areas. Male excess was identified in both isolated and associated forms of SD. No selective predominance was observed either by affected side or by affected limbs.

China↗
PubMed2004

[Case report: Syndactyly in German Holstein calves].

In 8 black and white German Holstein calves syndactyly was observed. The affected calves were born in different farms between 1995 und 2002. The number of feet affected varied from one, several to all four. All syndactylous calves could be traced back to a common ancestor, which was already identified as a carrier bull for the mulefoot gene. The pedigree was consistent with a monogenic autosomal recessive inheritance and variable expressivity.

Animals↗
PubMedDate not supplied

Macrodactyly, syndactyly, and localized gigantism of the forefoot. A case report.

On the basis of the observation of a rare case of lipomatous macrodactyly of the foot associated with syndactyly in a young child, the authors analyze the anatomopathologic and therapeutic aspects of the pathology and report the data found in the literature. This disease initially requires careful surveillance of its progression, and use of correct footwear. Surgery is indicated when deformity achieves unacceptable values, both functional and cosmetic, in early childhood.

Foot Deformities, Congenital↗
PubMed1990

Syndactyly.

Congenital syndactyly is associated with a wide spectrum of local anomalies that strongly influence the ultimate outcome of treatment. Surgical options, indications, timing, and prognosis are all determined by the preoperative degree of deformity. The surgeon must apply knowledge of anatomic variations and adhere to fundamental principles of technique, which are reviewed in detail.

Fingers↗
PubMed1989

A pedigree of cervical stenosis, brachydactyly, syndactyly, and hyperopia.

Cervical myelopathy due to developmental cervical canal stenosis occurred in a 13-year-old boy. The patient's father and aunt also had an abnormally small cervical canal, although both were asymptomatic. The patient and his family had many congenital anomalies including hereditary brachydactyly, syndactyly, and hyperopia. The association of these anomalies seems not to have been previously reported in the literature.

Abnormalities, Multiple↗