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- passive dorsiflexion of each fifth finger
greater than 90°,
- passive apposition of each thumb to the flexor
surface of the forearm,
- hyperextension of each elbow greater than 10°,
- hyperextension of each knee greater than 10°,
- ability to place the palms on the floor with the
knees fully extended.
Figure 2 gives a proposition of algorithm for
diagnosis approach in childrenwith hyperlaxity.
Typical examples of these pathologies are:
1)
Ehlers-Danlos syndrome
(EDS) is a
heterogeneous group of connective tissue
disorders characterized by hyperextensibility,
delayed wound healing, joint hypermobility,
thin skin, easy bruising, tissue fragility,
“cigarette-paper” scarring over bony promi
nences, mitral valve prolapse, and other
findings. There are 6 main types of EDS but
EDS Type III is the main type associated with
joint or patellar dislocation. Regardless of
presentation as a chief concern or an incidental
finding, clinicians should be aware that the
prominent skin findings of EDS are cutaneous
signs of an important systemic disorder.
2) Joint hypermobility syndrome
(JHS) is an
emerging pathology. This heritable disorder of
the connective tissues is characterized by
hypermobility, often affecting multiple joints,
and musculoskeletal pains in the absence of
systemic inflammatory joint disease such as
rheumatoid arthritis. As yet, the gene defect
underlying this condition remains unknown,
and the diagnosis is based upon clinical signs
and symptoms. The phenotype is similar to
Ehlers-Danlos type III syndrome hypermobility
type (same condition?). Under recognized and
sometimes dismissed as a pathologic entity,
this condition has no definitive treatment and
therefore poses a management challenge. JHS
have impaired proprioception, so that specific
rehabilitation program leads not only to symp
tomatic improvement, but also to demonstrable
enhancement of objective parameters such as
proprioception [4].
Fig. 2: Proposition of algorithm for diagnosis approach in children with hyperlaxity (Chotel French pediatric
societies Paris 2010). Genetic connective tissue diseases are important to consider according to associated
symptoms such as: scoliosis, thoracic deformity, pes planus, multiple fractures history…