Camptodactyly
Updates to Article Attributes
Camptodactyly is a clinical or imaging descriptive term where there is a flexion contracture (usually congenital) classically at the proximal interphalangeal joint.
Epidemiology
Prevalence ~ 1%
1/3~1%; one-third unilateral, 2/3two-thirds bilateralref.
Clinical presentation
The age of presentation can vary from being detected in utero in an antenatal scan or as an obvious deformity after birth or in childhood.
Pathology
There is often thought to be an abnormal insertion of the lumbrical or flexor digitorum tendons into the hand.
Genetics
It can occur sporadically or with an autosomal dominant inheritance.
Associations
While it can occur in isolation, there are numerous associations with the presence of camptodactyly (especially if detected inon obstetric imaging):
- aneuploidy syndromes 3
- non
aneuploidy-aneuploidy syndromes - other conditions causing an arthrogryposis
Classification
-
type I
- isolated anomaly of the little finger, presents in infancy and affects males and females equally
- most common form
-
type II
- same clinical features as type I; presents in adolescence
- affects girls more often than boys
- from abnormal lumbrical insertion, abnormal flexor digitorum superficialis (FDS) origin or insertion
-
type III
- severe contractures, multiple digits involved, presents at birth
- usually associated with a syndrome
-
Kirner's Deformity
- specific deformity of small finger distal phalanx with volar-radial curvature (apex dorsal-ulnar)
- often affects preadolescent girls
- often bilateral
- usually no functional deficits
Radiographic features
The involved joint is flexed and often can'tcannot be straightened. The phalangeal head may be small and if the flexion contracture is severe enough, the phalangeal head may articulating with and erode the dorsal cortex of the neck of the proximal phalanx.
Classification:
Type I
• Isolated anomaly of little finger, presents in infancy and affects males and females equally • Most common form
Type II
• Same clinical features as Type I, presents in adolescence • Affects girls more often than boys
From abnormal lumbrical insertion, abnormal FDS origin or insertion
Type III
• Severe contractures, multiple digits involved, presents at birth • Usually associated with a syndrome
Kirner's Deformity
• Specific deformity of small finger distal phalanx with volar-radial curvature (apex dorsal-ulnar) • Often affects preadolescent girls • Often bilateral • Usually no functional deficits
Treatment and prognosis
- non
operative-operative: stretching or splinting - operative: lumbrical insertion excision +/- FDS release or transfer
-<p><strong>Camptodactyly</strong> is a clinical or imaging descriptive term where there is a flexion contracture (usually congenital) classically at the <a href="/articles/proximal-interphalangeal-joint">proximal interphalangeal joint</a>.</p><h4>Epidemiology</h4><p>Prevalence ~ 1%</p><p>1/3 unilateral, 2/3 bilateral</p><h4>Clinical presentation</h4><p>The age of presentation can vary from being detected in utero in an antenatal scan or as an obvious deformity after birth or in childhood.</p><h4>Pathology</h4><p>There is often thought to be an abnormal insertion of the lumbrical or flexor digitorum tendons into the hand.</p><h5>Genetics</h5><p>It can occur sporadically or with an autosomal dominant inheritance.</p><h5>Associations</h5><p>While it can occur in isolation, there are numerous associations with the presence of camptodactyly (especially if detected in obstetric imaging):</p><ul>- +<p><strong>Camptodactyly</strong> is a clinical or imaging descriptive term where there is a flexion contracture (usually congenital) classically at the <a href="/articles/proximal-interphalangeal-joint">proximal interphalangeal joint</a>.</p><h4>Epidemiology</h4><p>Prevalence ~1%; one-third unilateral, two-thirds bilateral <sup>ref</sup>.</p><h4>Clinical presentation</h4><p>The age of presentation can vary from being detected in utero in an antenatal scan or as an obvious deformity after birth or in childhood.</p><h4>Pathology</h4><p>There is often thought to be an abnormal insertion of the lumbrical or flexor digitorum tendons into the hand.</p><h5>Genetics</h5><p>It can occur sporadically or with an autosomal dominant inheritance.</p><h5>Associations</h5><p>While it can occur in isolation, there are numerous associations with the presence of camptodactyly (especially if detected on obstetric imaging):</p><ul>
-<li>non aneuploidy syndromes<ul>- +<li>non-aneuploidy syndromes<ul>
-</ul><h4>Radiographic features</h4><p>The involved joint is flexed and often can't be straightened. The phalangeal head may be small and if the flexion contracture is severe enough, the phalangeal head may articulating with and erode the dorsal cortex of the neck of the proximal phalanx.</p><h5>Classification:</h5><p>Type I</p><p> • Isolated anomaly of little finger, presents in infancy and affects males and females equally<br> • Most common form</p><p>Type II</p><p> • Same clinical features as Type I, presents in adolescence<br> • Affects girls more often than boys </p><p>From abnormal lumbrical insertion, abnormal FDS origin or insertion</p><p>Type III</p><p> • Severe contractures, multiple digits involved, presents at birth<br> • Usually associated with a syndrome </p><p>Kirner's Deformity</p><p> • Specific deformity of small finger distal phalanx with volar-radial curvature (apex dorsal-ulnar)<br> • Often affects preadolescent girls<br> • Often bilateral<br> • Usually no functional deficits</p><h4>Treatment and prognosis</h4><ul>-<li>non operative: stretching or splinting</li>- +</ul><h4>Classification</h4><ul>
- +<li>
- +<strong>type I</strong><ul>
- +<li>isolated anomaly of the little finger, presents in infancy and affects males and females equally</li>
- +<li>most common form</li>
- +</ul>
- +</li>
- +<li>
- +<strong>type II</strong><ul>
- +<li>same clinical features as type I; presents in adolescence</li>
- +<li>affects girls more often than boys </li>
- +<li>from abnormal <a title="Lumbrical muscles of the hand" href="/articles/lumbrical-muscles-of-the-hand">lumbrical</a> insertion, abnormal <a title="Flexor digitorum superficialis" href="/articles/flexor-digitorum-superficialis">flexor digitorum superficialis (FDS)</a> origin or insertion</li>
- +</ul>
- +</li>
- +<li>
- +<strong>type III</strong><ul>
- +<li>severe contractures, multiple digits involved, presents at birth</li>
- +<li>usually associated with a syndrome </li>
- +</ul>
- +</li>
- +<li>
- +<strong>Kirner's Deformity</strong><ul>
- +<li>specific deformity of small finger distal phalanx with volar-radial curvature (apex dorsal-ulnar)</li>
- +<li>often affects preadolescent girls</li>
- +<li>often bilateral</li>
- +<li>usually no functional deficits</li>
- +</ul>
- +</li>
- +</ul><h4>Radiographic features</h4><p>The involved joint is flexed and often cannot be straightened. The phalangeal head may be small and if the flexion contracture is severe enough, the phalangeal head may articulating with and erode the dorsal cortex of the neck of the proximal phalanx.</p><h4>Treatment and prognosis</h4><ul>
- +<li>non-operative: stretching or splinting</li>
-</ul><p> </p>- +</ul>
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