Masquelet's procedure and bone morphogenetic protein in congenital pseudarthrosis of the tibia in children: a case series and meta-analysis

Abstract

Purpose: A type 2 recombinant human bone morphogenetic protein (rhBMP2) and Masquelet's procedure were used in three children presenting with congenital pseudarthrosis of the tibia (CPT). Recent studies on CPT suggested the presence in situ of pathologic tissues promoting pseudarthrosis. The authors hypothesized that large segmental resection of pseudarthrosis could improve prognosis of the CPT. Masquelet's procedure and rhBMP2 have been advocated for the treatment of long bone defect.

Method: The authors report three cases of CPT in children treated with Masquelet's procedure and application of rhBMP2. They analyzed all published cases of CPT similarly treated.

Results: In the present study, Masquelet's procedure did not improve the results in the treatment of CPT, but segmental bone reconstruction was possible. Bone healing was obtained in three out of the five applications of rhBMP2. In one case, the patient's parents asked for leg amputation. Analysis of the 33 published cases with the application of BMP in CPT points to a 62 % healing rate in this pathology.

Conclusion: The authors confirmed that segmental bone reconstruction is possible in CPT using Masquelet's procedure. In the literature, the success rate of the application of rhBMP in CPT appears to be lower than the healing rate usually reported without BMP. Nevertheless, the strict selection of patients, limited number of cases, and their heterogeneity make interpreting the results difficult. However, the theoretical risk which the children are exposed to during the use of BMP makes rigorous selection of the indications necessary. Finally, the interest of rhBMP2 application in Masquelet's procedure remained to be proven.

Keywords: Bone morphogenetic proteins; Child; Congenital pseudarthrosis; Induce membrane; Neurofibromatosis.

Fig. 1

Observation 1, Quentin: a Congenital bow of the tibia in neurofibromatosis type 1 (NF1), b fracture, orthopedic treatment with casting failed, c Masquelet’s procedure, d pseudarthrosis relapses, e first application of human recombinant bone morphogenetic protein 2 (rhBMP2), healing within 14 weeks (distal screws were removed early in order to protect the growth plate), f 1 year follow-up, fracture of the plate, g second application of rhBMP2 and bone grafting, h in situ infection with bone absorption, i radiological result when parents asked for amputation

Fig. 2

Observation 2, Loris: cystic congenital pseudarthrosis of the tibia (CPT), a 2 months old, b worse evolution despite orthopedic treatment, c first treatment with vascularized autogenous fibula graft and external frame, d pseudarthrosis relapses, e third pseudarthrosis despite performing a Masquelet’s procedure, f third treatment with the application of rhBMP2 leading to bone consolidation, g result in July 2008, h fracture in March 2010 with orthopedic treatment, i result at final follow-up in May 2011

Fig. 3

Observation 3, Alicia: a congenital bow of the tibia in NF1, b fracture at 3 years old, c, d first procedure (Masquelet’s) and application of rhBMP2, e 7 months follow-up, pseudarthrosis relapses, f second application of rhBMP2 and bone grafting, g pseudarthrosis relapses, h third procedure with intramedullary nailing (Fassier–Duval nail) and bone grafting (May 2008), i bone consolidation (October 2008), j, k result at final follow-up (May 2011)

Fig. 4

Results presented relative to the procedure used for stabilization after the application of bone morphogenetic protein (BMP)