Classification and Developmental Biology of Congenital Anomalies of the Hand and Upper Extremity

Manske, Paul R.; Oberg, Kerby C.

doi:10.2106/jbjs.i.00008

Cited by 69 publications

(35 citation statements)

References 83 publications

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“…The fixed position of pronation observed in most cases was explained by Wilkie's studies [10], which showed that the forearm of the young embryo is usually in an intermediate pronation position and that the failure of segmentation makes it remain permanently in that position. In patients with unilateral synostosis such as the observation of our patient, shortening is observed in 32% of cases [11], which is very high compared the other series [4,9,12]. Functional disorders are not always correlated with the degree of fixed position of the forearm, as compensatory motion is observed with age.…”

Section: Discussionmentioning

confidence: 61%

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Surgical Treatment of Congenital Radioulnar Synostosis in Children: A Case Report

Bakkaly¹,

Ghordaf²,

Bouljrouf³

et al. 2017

Int J Surg Med

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Congenital radioulnar synostosis is a rare condition, only 400 cases have been reported in the world literature. Often bilateral, it can be recognised from birth by a clinical examination if it is attentive (examination of the prono-supination of the two elbows). It is in fact often discovered later, especially in unilateral forms, in children of school age. The proximal congenital radioulnar synostosis often results in functional, cosmetic limitations of the upper limb, especially in the bilateral forms. Rotational osteotomy through the synostosis site is the usual procedure. The techniques and sites envisaged for this surgery are numerous, with multiple difficulties, risks and possible complications. We propose the observation of an eight-year-old boy with an asymptomatic form of proximal radioulnar synostosis. The surgical treatment consisted of a resection of the synostosis with rotational transverse osteotomy of the two bones of the forearm completed by a plaster in a neutral position. Clinical results, evaluated at an average of twelve months postoperatively, were found to be satisfactorily on aesthetic and functional levels. The aim of our work is to highlight our straightforward and reliable surgical procedure of the osteotomy of the synostosis maintained by spindles, allowing the functional improvement of the movements of the forearm of our child and thus a healthy life.

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Section: Discussionmentioning

confidence: 61%

“…However, there are sporadic cases [4,5,6]. A failure of longitudinal segmentation occurring at around the seventh week of fetal development is the cause of the deformity, which is responsible for the persis-tence of a fibrous or osseous bridge between the radius and ulna, due to an abnormality of the path of the Posterior interosseous artery.…”

mentioning

confidence: 99%

Surgical Treatment of Congenital Radioulnar Synostosis in Children: A Case Report

Bakkaly¹,

Ghordaf²,

Bouljrouf³

et al. 2017

Int J Surg Med

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“…The ideal classification system for congenital anomalies of the hand "should provide a simple description of the appearance of the hand to enhance communication between hand surgeons, and at the same time incorporate the embryological mechanism, as well as dictate the surgical reconstruction and predict the prognosis" [18]. Unlike most other classifications, our documentation system provides a simple description of either the morphological or radiographic appearance or functional status of a child's hand to facilitate communication between physicians, but it does not attempt to imply any underlying embryological causation, although it does incorporate all the previous subclassification systems.…”

Section: Discussionmentioning

confidence: 99%

A New Documentation System for Congenital Absent Digits

Kaplan

2012

Hand (New York, N,Y.)

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Background Congenital absent digits continue to be described by many confusing terms and are currently classified in categories I, V, and VI of the International Federation of Societies for Surgery of the Hand classification and seven subclassification systems. Very few classification systems provide any logical basis for surgical reconstruction. The purpose of this study was to develop a simple alphanumerical documentation system to reproducibly describe the morphological or radiographic appearance of congenital absent digits and facilitate communication of these childrens' hand anomalies from one hand surgeon to another. Methods Dorsal and palmar photographs and PA radiographs of 235 hands in 204 children born with congenital absent digits over a 15-year period were analyzed to determine which digital rays were missing and their level of absence. Each hand can be described by three letters, R (radial), C (central), and U (ulnar), as well as numbers 1-5. The first letter and number designate which rays are missing and the second and third letters and numbers designate which rays remain present. Results There are 15 morphological phenotypes of congenital absent digits. The three most common phenotypes are U4R1 (a thumb but absence of all four fingers), R1U4 (absent thumb), and R5 (aplastic hand). Conclusions This new documentation system allows hand surgeons to describe the simple morphological or radiographic appearance of congenital absent digits; incorporates all the previous subclassification systems that have attempted to describe congenital absent digits in radial, central, and ulnar deficiencies, symbrachydactyly, and congenital constriction ring syndrome; and has subsequently allowed the development of an algorithm which predicts whether conventional or microsurgical reconstruction is indicated for each specific phenotype.

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“…[2][3][4][5]18 ULD is one-third to one-tenth less common than RLD. This anomaly is more common in males (3:2 ratio) and occurs bilaterally in about 25% of affected individuals.…”

Section: Etiology Of Uldmentioning

confidence: 99%

Combined Congenital Radial and Ulnar Longitudinal Deficiencies: Report of 2 Cases

Oberg

Harris²,

Wongworawat³

et al. 2009

The Journal of Hand Surgery

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Classification and Developmental Biology of Congenital Anomalies of the Hand and Upper Extremity

Abstract: "Classification and developmental biology of congenital anomalies of the hand and upper extremity

Cited by 69 publications

References 83 publications

Surgical Treatment of Congenital Radioulnar Synostosis in Children: A Case Report

Surgical Treatment of Congenital Radioulnar Synostosis in Children: A Case Report

A New Documentation System for Congenital Absent Digits

Combined Congenital Radial and Ulnar Longitudinal Deficiencies: Report of 2 Cases

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