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The Journal of the American Academy of... Feb 2018Polydactyly is one of the most common congenital hand deformities managed by orthopaedic surgeons. It is most often found in isolation; however, rarely, it may be... (Review)
Review
Polydactyly is one of the most common congenital hand deformities managed by orthopaedic surgeons. It is most often found in isolation; however, rarely, it may be associated with genetic syndromes. Polydactyly is classified as postaxial, preaxial, or central depending on the radioulnar location of the duplicated digits. Postaxial polydactyly, which affects the ulnar side of the hand, is most common and is typically managed with excision or suture ligation of the supernumerary digit. Preaxial polydactyly, which affects the thumb or radial side of the hand, often requires reconstructive techniques to ensure a functional, stable thumb. Central polydactyly is much less common, and reconstruction can be challenging.
Topics: Fingers; Hand Deformities, Congenital; Humans; Orthopedic Procedures; Polydactyly; Plastic Surgery Procedures
PubMed: 29309292
DOI: 10.5435/JAAOS-D-16-00139 -
The Journal of the American Academy of... May 2021Pediatric foot polydactyly presents in a wide variety of deformities from single extra digits attached only by a narrow soft-tissue connection to complex central foot... (Review)
Review
Pediatric foot polydactyly presents in a wide variety of deformities from single extra digits attached only by a narrow soft-tissue connection to complex central foot duplications with tarsal bone duplication. The goal of surgical reconstruction is a stable, mobile, pain-free foot with five cosmetically appealing toes that allows for normal footwear and painless ambulation. This review covers the incidence, genetics and embryology, classification, presentation and natural history, physical examination, radiographic assessment, and surgical intervention for all types of foot polydactyly.
Topics: Child; Foot; Foot Deformities, Congenital; Humans; Polydactyly; Toes; Walking
PubMed: 33443388
DOI: 10.5435/JAAOS-D-20-00983 -
Medicine Dec 2022This study collects what is known about the inheritance underpinnings of syndromic and non-syndromic polydactylies and highlights dactyly presentations with unknown... (Review)
Review
OBJECTIVE
This study collects what is known about the inheritance underpinnings of syndromic and non-syndromic polydactylies and highlights dactyly presentations with unknown genetic roots. This review summarizes the current information and genetics-enhanced understanding of polydactyly.
BACKGROUND
There is a frequency of 0.37 to 1.2 per 1000 live births for polydactyly, which is also known as hyperdactyly. It is characterized by the presence of extra fingers. Polydactyly is caused by a failure in limb development, specifically the patterning of the developing limb bud. The phenotypic and genetic variability of polydactyly makes its etiology difficult to understand. Pre-axial polydactyly, central polydactyly (axial), and postaxial polydactyly are all examples of non-syndromic polydactyly (ulnar). An autosomal dominant disorder with varying penetrance that is mostly passed down via limb development patterning abnormalities.
METHOD
A comprehensive search of MEDLINE/PubMed and other databases was followed by an evaluation of the relevant papers, with a particular focus on those published between 2000 and 2022.
RESULTS
Of 747 published article related to Polydactyly from MEDLINE/PubMed search, 43 were from the last 10 years and were the focus of this review.
CONCLUSION
Polydactyly is one of the most frequent congenital hand malformations. PAP is more common than PPD, whereas central polydactyly is very uncommon.
Topics: Humans; Polydactyly; Fingers; Toes
PubMed: 36550802
DOI: 10.1097/MD.0000000000032060 -
Neonatal Network : NN 2016Polydactyly, also known as hyperdactyly, is a common congenital limb defect, which can present with various morphologic phenotypes. Apart from cosmetic and functional... (Review)
Review
Polydactyly, also known as hyperdactyly, is a common congenital limb defect, which can present with various morphologic phenotypes. Apart from cosmetic and functional impairments, it can be the first indication of an underlying syndrome in the newborn. Usually, it follows an autosomal dominant pattern of inheritance with defects occurring in the anteroposterior patterning of limb development. Although many mutations have been discovered, teratogens have also been implicated in leading to this anomaly, thus making it of multifactorial origin. There are three polydactyly subtypes (radial, ulnar, and central), and treatment options depend on the underlying feature.
Topics: Fingers; Genetic Markers; Humans; Infant, Newborn; Mutation; Polydactyly; Syndrome
PubMed: 27194607
DOI: 10.1891/0730-0832.35.3.135 -
JBJS Reviews May 2022» Radial polydactyly is one of the most common congenital anomalies of the hand, with an incidence of 0.08 to 1.40 per 1,000 live births; it requires surgical treatment...
» Radial polydactyly is one of the most common congenital anomalies of the hand, with an incidence of 0.08 to 1.40 per 1,000 live births; it requires surgical treatment early in life.» Polydactyly occurs during weeks 5, 6, 7, and 8 of embryogenesis, principally due to misregulation of the sonic hedgehog protein within the developing limb bud.» The Flatt classification system of preaxial polydactyly (types I to VII), as published by Wassel, categorizes preaxial polydactyly based on osseous abnormalities, but it has substantial limitations.» For improved function and appearance, preaxial polydactyly ideally requires surgical intervention at an early age (between 1 and 2 years of age) before the development of fine motor skills.
Topics: Humans; Infant; Child, Preschool; Polydactyly; Thumb
PubMed: 37545004
DOI: 10.2106/JBJS.RVW.21.00214 -
Bulletin of the Hospital For Joint... 2013Polydactyly of the hand is a difficult problem and poses a unique challenge for the hand surgeon. The embryology of limb development is complex, leading to a host of... (Review)
Review
Polydactyly of the hand is a difficult problem and poses a unique challenge for the hand surgeon. The embryology of limb development is complex, leading to a host of different phenotypes of polydactyly. Polydactyly can occur in any digit and is described as preaxial, postaxial, and central, based on location. Classification systems exist for each of these locations, which guide treatment options. Surgical treatment needs to address the aesthetic and functional aspect of hand reconstruction. Careful consideration and planning of surgical treatment individualized to each patient is required to obtain the best possible outcome.
Topics: Fingers; Humans; Orthopedic Procedures; Patient Selection; Polydactyly; Recovery of Function; Risk Factors; Treatment Outcome
PubMed: 24032579
DOI: No ID Found -
American Journal of Orthopedics (Belle... May 2015Polydactyly is considered either the most or second most (after syndactyly) common congenital hand abnormality. Polydactyly is not simply a duplication; the anatomy is... (Review)
Review
Polydactyly is considered either the most or second most (after syndactyly) common congenital hand abnormality. Polydactyly is not simply a duplication; the anatomy is abnormal with hypoplastic structures, abnormally contoured joints, and anomalous tendon and ligament insertions. There are many ways to classify polydactyly, and surgical options range from simple excision to complicated bone, ligament, and tendon realignments. The prevalence of polydactyly makes it important for orthopedic surgeons to understand the basic tenets of the abnormality.
Topics: Hand Deformities, Congenital; Humans; Polydactyly
PubMed: 25950541
DOI: No ID Found -
Clinical Genetics Nov 2017GLI3 mutations are known to be associated with nine syndromes/conditions in which polydactyly is a feature. In this review, the embryology, pathogenesis, and animal... (Review)
Review
GLI3 mutations are known to be associated with nine syndromes/conditions in which polydactyly is a feature. In this review, the embryology, pathogenesis, and animal models of GLI3-related polydactyly are discussed first. This is followed by a detailed review of the genotype-phenotype correlations. Based on our review of the literature and our clinical experiences, we recommend viewing GLI3-related syndromes/conditions as four separate entities; each characterized by a specific pattern of polydactyly. These four entities are: the preaxial polydactyly type IV-Greig-acrocallosal spectrum, postaxial polydactyly types A/B, Pallister-Hall syndrome (PHS), and oral-facial-digital overlap syndrome. We also provide illustrative clinical examples from our practice including a family with a novel GLI3 mutation causing PHS. The review also introduces the term 'Forme Fruste' preaxial polydactyly and gives several conclusions/recommendations including the recommendation to revise the current criteria for the clinical diagnosis of PHS.
Topics: Animals; Disease Models, Animal; Genetic Association Studies; Humans; Polydactyly; Syndrome; Zinc Finger Protein Gli3
PubMed: 28224613
DOI: 10.1111/cge.12952 -
Current Pediatric Reviews 2018Thumb duplication, also called radial or preaxial polydactyly, is classified as an alteration of the radio-ulnar axis in the formation and differentiation of the hand... (Review)
Review
BACKGROUND
Thumb duplication, also called radial or preaxial polydactyly, is classified as an alteration of the radio-ulnar axis in the formation and differentiation of the hand plate, according to the Oberg, Manske and Tonkin classification.
OBJECTIVE
Radial polydactyly is a common upper limb malformation. This paper is a comprehensive analysis of radial polydactyly, its genetic explanation, classification, surgical techniques, functional results, as well as present and future scientific evidence on this congenital pathology.
CONCLUSIONS
Radial polydactyly occurs most frequently as an isolated defect, but it may also occur in association with other abnormalities, or as part of a syndrome. The Wassel classification is the universal classification for thumb duplication. One of the limitations of it is the misclassification of the immature epiphysis. The Wassel classification not only refers to the pathoanatomy of the polydactyly but also guides the selection of various surgical techniques. Three well-defined surgical objectives should be ensured: thumb alignment, stability, and an acceptable final esthetic appearance. Tada score is a validated functional scale that takes into account the most common and limiting complications, namely clinodactyly and instability. Based on some recent strong research evidence, the JSSH assessment system provides the most reliable outcome of scores. More studies are required to provide evidence-based conclusions regarding the treatment of radial polydactyly.
Topics: Genetic Markers; Humans; Polydactyly; Plastic Surgery Procedures; Thumb; Treatment Outcome
PubMed: 29366421
DOI: 10.2174/1573396314666180124102012 -
Current Opinion in Pediatrics Feb 2020The purpose of this review is to describe various forms of hand polydactyly and their different treatment approaches. Hand polydactyly is commonly classified as ulnar... (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to describe various forms of hand polydactyly and their different treatment approaches. Hand polydactyly is commonly classified as ulnar (small finger) or radial (thumb). Polydactyly can be sporadic, genetic, and/or associated with syndromic conditions.
RECENT FINDINGS
Both ulnar and radial polydactyly can be surgically treated to optimize hand aesthetics and function. Timing of surgery is based on multiple factors, most notably including safety of anesthesia and socialization of the affected child. The pediatrician should be aware of potential associated conditions, such as chondroectodermal dysplasia or Ellis-van Creveld syndrome for ulnar polydactyly.
SUMMARY
Polydactyly is a common congenital hand difference and can be broadly be classified by radial or ulnar involvement. Polydactyly warrants hand surgical referral, as surgical treatment is often indicated. Pediatricians should be aware of treatment options, as well as of commonly associated anomalies and syndromes.
Topics: Child; Fingers; Hand Deformities, Congenital; Humans; Polydactyly; Thumb
PubMed: 31851054
DOI: 10.1097/MOP.0000000000000871