SURGICAL MANAGEMENT OF CHILDREN WITH OSTEOGENESIS IMPERFECTA USING THE FASSIER-DUVAL INTRAMEDULLARY NAIL

Abstract

Osteogenesis imperfecta is a heterogeneous group of collagen type 1 defects. The resulting fragile bone with increased risk of fractures and deformity is the primary orthopaedic challenge. Surgical treatment is aimed at reducing the risk of fracture, correcting deformity and improving ambulatory status.

Management of the growing child with extensible intramedullary device was introduced in the 1960’s by Bailey and Dubow. Since then a number of different nails have been used. The current report presents our experience with the Fassier-Duval intramedullary nail in the first ten patients.

Materials and Method: The first ten children who had been operated between 2005 and 2008 with the Fassier-Duval (FD) nail were reviewed. The indication for surgery in all patients was to stabilise the long bones of the lower extremity to promote mobility. Correction of the axis of the long bones was obtained with wedged osteotomies. There where 7 girls and 3 boys.

Median follow-up was 2 years and 3 months (Range 1 – 4 years and 3 month).

Results: 22 FD nails were inserted in 13 operations in 10 children. 8 FD nails (4 operations) were primary FD nail insertions. The mean age at insertion was 2 years. 14 FD nails (9 operations) were exchanges of other devices. In this group the mean age at insertion of the FD nail was 6 years. In both groups mean admission was 4,5 days.

5 operations were reoperations due to complications. All of these were in femora. 5 patients had a fracture despite the nail. 3 were reoperated due to bending or perforation of the nail. 2 were treated conservatively. All 5 healed uneventfully. 1 patient was reoperated 3 times. First due to migration of the nail, secondly failure of the nail to elongate and thirdly because of a fracture with bending of the nail. There were no infections, neurological- or vascular damage.

At follow-up 6 patients were walking without any aid. 2 were mobilised with aids. 2 were mobilised in wheelchair.

No radiographic evidence of growth arrest has been noted secondary to the crossing of the epiphysealplate by the nails.

Discussion: Our result supports the reported benefits of extensible intramedullary device in children suffering from osteogenesis imperfecta. Minimizing the crippeling effects of OI is aimed at reducing the risk of fracture, correcting deformity and improving ambulatory status. There are also complications with the Fassier-Duval nail. However these are less than reported with other extensible intramedullary nails. Indeed one could in some cases argue that a bended nail or a fracture after nail insertion is a consequence of the better mobility on the way to a better quality of life.