Surgical recommendations in Ehlers-Danlos syndrome(s) need patient classification: the example of Ehlers-Danlos syndrome hypermobility type (a.k.a. joint hypermobility syndrome).

摘要

I read with great interest the work by Burcharth and Rosenberg [1] (Herlev, Denmark) on surgical aspects of Ehlers-Danlos syndrome(s) (EDSs). In this work, the authors review 53 previously published papers, summarize indications and complications by anatomic structure, and define some practical recommendations for improving outcomes of skin closure after surgical incisions. Their work is definitely of invaluable support in the daily practice of many professionals involved in the management of hereditary connective tissue disorders.However, the extreme clinical variability and genetic heterogeneity of EDSs represent a major limit to such generalizations. In fact, as stated by the authors themselves in the first paragraph of their paper, EDS actually groups together an increasing number of genetic disorders sharing various degrees of generalized joint hypermobility and some cutaneous and soft tissue features, but with differences in the spectrum of ancillary findings and the molecular basis. Such discordance impacts prognosis which diverges significantly among clinical subtypes.