Lower limb lengthening in patients with disproportionate short stature with achondroplasia: a systematic review of the last 20 years

Abstract

Parents of children suffering from disproportionate short stature due to achondroplasia may wish to have surgical leg lengthening carried out for the child. The aim is not to increase height, but rather to achieve physiological proportions in the body. In a systematic review of the literature on the topic dating from the last 20 years, the surgical approaches used for this purpose were analyzed in accordance with the Preferred reporting items for systematic reviews and meta-analyses (PRISMA) criteria. Twelve studies show that to date, involvement of the child in decision-making at the start of treatment has been expected and that it is recommended from the age of 12. In highly heterogeneous patient groups, with varying factors involved and different techniques being used, lengthening (often by more than 10 cm) is described. High complication rates are reported, with many setbacks often requiring repeat surgery. Using PALEY’S multiplier method, the expected standing height, sitting height, and leg length can be predicted and an individualized treatment approach can be planned and operative procedures could be started in early childhood as PERETTI suggests. As the patients are unable to be involved in decision-making as young children, these data may provide a basis for offering differentiated advice to parents, who usually consult a pediatric orthopedist at a very early stage in the child’s life.

Implications for Rehabilitation

• Achondroplasia and congenital dysproportional short stature is a condition which restricts quality of life often through stigmatisation.

• Reconstructive surgery by the meaning of deformity correction is a technique available to this patients (Lower) limb lengthening is an operative treatment concepts which tries to better the ratio of disproportion and lengthens the legs not only for improvement of body height

• This systematic review of the literature of the last 20 years describes the most common operative concepts and their limitations

Keywords::

• Achondroplasia
• limb lengthening
• congenital short stature
• external fixator

Conflict of Interest: The authors declare that no conflicts of interest.