Authors' objectives:

The objective of this report is to assess the different therapeutic possibilities for patients suffering from achondroplasia, as well as to assess the outcome of these therapeutic measures.

A global view of the role of limb-lengthening techniques, of its acceptability according to the context, and of its therapeutic results and adverse effects has been intended, as well as the psychological and social impact that a small height - far below the parameters that define normality statistically - may have on the individual suffering from it (and on his or her family).

Authors' recommendations: Achondroplastic persons, for the very characteristics of their development (dwarfism), may show during their lives several medical-surgical problems that need specialised treatment. These individuals, both in childhood and adolescence and in adult age (e.g., genetic counselling) need advice and supervision by professional teams specialised in the diagnosis and therapeutical of different forms of low height and other possible consequences of their particular development. To achieve this level of care, referential hospital centres should be promoted, with the necessary professional and technical capacity to offer the integral health care approach needed by these persons. Only in this type of centre could a limb-lengthening programme for low height individuals be implemented, using an accurately defined protocol of indications, preoperative assessment criteria, surgical technique, and monitoring/follow-up. Limb-lengthening to correct low height is a controversial procedure, even within the self help groups and associations for persons suffering from this disability, and this is reflected on the different web sites found on the Internet. Many cultural, familiar and individual's preferences factors are involved in the decision to operate. Limb-lengthening techniques are complex, painful, long procedures, subject to potential multiple complications. This makes it necessary to accurately assess the individual's psychological and emotional status, as well as his/her expectations before making the decision. Limb-lengthening to correct low height is planned at an age when the person is not (legally) qualified to decide by him/herself. However, this is not an obstacle for considering him/her competent, according to age and other criteria. Informed consent, the role as trustees of parents or guardians, and the very aims of the operation, may advise, in specific occasions, the intervention of the Ethical Health Care Committee of the centre where these procedures are to take place, to guarantee, in the extent possible, the respect of the ethical principles.

Authors' methods: Review

Project Status: Completed

URL for project: http://www.gencat.net/salut/depsan/units/aatrm/html/en/dir393/doc7921.ht ml

Year Published: 1999

English language abstract: An English language summary is available

Publication Type: Not Assigned

Country: Spain

Organisation Name: Agencia de Qualitat i Avaluacio Sanitries de Catalunya

Contact Address: Antoni Parada, CAHTA, Roc Boronat, 81-95 (2nd floor), 08005 Barcelona, Spain, Tel. +34 935 513 928, Fax: +34 935 517 510

Contact Name: direccio@aatrm.catsalut.net / aparada@aatrm.catsalut.net

Contact Email: direccio@aatrm.catsalut.net / aparada@aatrm.catsalut.net

Copyright: Catalan Agency for Health Technology Assessment and Research