116 THE JOURNAL OF BONE AND JOINT SURGERY
CASE REPORT
Bone morphogenetic protein 7 in the
treatment of congenital pseudarthrosis of
the tibia
L. Fabeck,
D. Ghafil,
M. Gerroudj,
R. Baillon,
Ph. Delincé
From CHU Saint
Pierre, Brussels,
Belgium
L. Fabeck, MD, PhD,
Orthopaedic Surgeon,
Professor
D. Ghafil, MD, Orthopaedic
Surgeon
M. Gerroudj, MD, General
Practitioner
R. Baillon, MD,
Orthopaedic Surgeon
Ph. Delincé, MD,
Orthopaedic Surgeon,
Professor
Department of Orthopaedic
Surgery
CHU Saint Pierre, 322 rue
Haute, 1000 Brussels,
Belgium.
Correspondence should be
sent to Dr L. Fabeck; e-mail:
laurent_fabeck@
stpierre-bru.be
©2006 British Editorial
Society of Bone and
Joint Surgery
doi:10.1302/0301-620X.88B1.
16619 $2.00
J Bone Joint Surg [Br]
2006;88-B:116-18.
Received 15 April 2005;
Accepted after revision
6 September 2005
We describe a 13-year-old boy with atrophic tibial pseudarthrosis associated with
neurofibromatosis who had undergone nine unsuccessful operations. Eventually, union
was obtained by the use of bone morphogenetic protein 7 in conjunction with
intramedullary stabilisation and autologous bone graft.
Congenital pseudarthrosis of the tibia is a rare
disorder, usually appearing during the first two
years of life. Neurofibromatosis is present in
approximately 40% to 50% of patients with
pseudarthrosis and approximately 10% of
patients with neurofibromatosis develop pseud-
arthrosis of the tibia. Treatment is challenging
and often disappointing and unsatisfactory out-
comes may lead to amputation.
1
Operative treatment includes intramedul-
lary rodding and grafting, limb lengthening,
vascularised fibular transfer and the Ilizarov
techniques.
2-5
However, even after multiple
attempts to achieve bony union,
2-4
amputation
may be needed.
The osteogenic proteins are a class of natural
growth factors of the bone morphogenetic pro-
tein family (BMP) which stimulate bone regener-
ation.
6
A specific member of this family is BMP-
7, known as human recombinant osteogenic
protein number 1 (rh OP-1), which accelerates
bone repair compared with a control fracture
when injected into a fresh fracture model.
7
Case report
The patient was born by normal delivery after an
uneventful pregnancy. There was no family his-
tory of congenital problems. At the age of nine
months, ‘café-au-lait spots’ appeared on his back
and abdomen. At 14 months, there was anterior
bowing of the right leg and neurofibromatosis
was diagnosed. When he had reached 20 months
and began to walk, the leg was protected by an
ankle-foot orthosis with an anterior shell.
Fig. 1a
Radiographs showing the tibia pre-operatively a) lateral and b) anteroposterior views. The
boy had undergone nine unsuccessful surgical treatments.
Fig. 1b