444 Original article
Congenital tibial pseudarthrosis, changes in treatment
protocol
Lior Shabtai, Eli Ezra, Shlomo Wientroub and Eitan Segev
Ten children with congenital pseudarthrosis of the tibia were
treated with resection of pathologic bone, bone grafting,
intramedullary rodding, compression with circular frame,
simultaneous proximal tibia lengthening, and bone
morphologic proteins. Thirteen operations were performed
to achieve union. Four patients underwent simultaneous
lengthening and four patients received recombinant human
bone morphologic protein. Six children required
complementary operations for residual ankle and knee
valgus, and shortening. Union of the pseudarthrosis was
achieved in all cases with lengthening up to 5 cm. This
protocol is effective in achieving union of congenital
pseudarthrosis of tibia; attention should be paid to the ankle
Introduction
Congenital pseudarthrosis of tibia (CPT) is a rare condi-
tion, affecting one child in every 190 000 live births, with
unclear etiology and pathogenesis, and is mostly (55%)
associated with neurofibromatosis type I (NF-I) [1]. For
this reason, a complete clinical examination, specifically
neurological and dermatological, with full family history
anamnesis must be performed in a newborn with limb
anomalies.
CPT is characterized by progressive tibia and fibula
anterolateral bowing, pathological fractures, development
of tibia, and/or fibula pseudarthrosis. In addition, it may
rarely occur in other long bones. Normally, it is unilateral
and there is no predominance for sex or side [1,2].
Protection of the extremity with an orthosis until skeletal
maturity is recommended in all patients.
The pathology of CPT is still unknown. The main his-
topathological change observed in this lesion is a growth
of a highly cellular, fibromatosis-like tissue: there was no
difference between patients with or without NF-I on
comparing the pathological samples [3]. It seems that the
refracture rate decreases with age. This may be because
of a decrease in the activity of the pathologic tissue and
an increase in bone diameter with age [4].
The best age for treatment is after the age of 3 because of
the difficulty of stabilizing small bone fragments at a
younger age [5].
Many surgical techniques have been published in the
literature, such as free vascular fibular grafts [6–8], the
Ilizarov circular frame [9–11], intramedullary (IM) nails
with bone grafts [12–14], use of periosteal graft, or a
1060-152X Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
joint to achieve functionality of the limb. J Pediatr Orthop B
24:444–449 Copyright © 2015 Wolters Kluwer Health, Inc.
All rights reserved.
Journal of Pediatric Orthopaedics B 2015, 24:444–449
Keywords: ankle valgus, compression, congenital tibia pseudarthrosis,
fibular nonunion, leg-length discrepancy, recombinant bone morphologic
protein, union
The Department of Pediatric Orthopaedics, Dana Children’s Hospital, Tel Aviv
Medical Centre and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Correspondence to Lior Shabtai, MD, The Department of Pediatric Orthopaedics,
Dana Children’s Hospital, Tel Aviv Medical Center and Sackler Faculty of
Medicine, Tel Aviv University, 6 Weizmann Street, Tel Aviv 64239, Israel
Tel: + 972 52 7360 373; fax: + 972 36 9748 53; e-mail: lish3@hotmail.com
combination of these techniques [4,15]. All have been
used with variable degrees of success.
The challenge of achieving union combined with a high
rate of refractures and complications, such as diaphyseal
and joint deformities and leg-length discrepancy, have
led us to make changes in our treatment.
Our protocol includes resection of pathologic bone, bone
grafting, insertion of IM rodding, compression with a
circular frame, and simultaneous proximal tibia length-
ening. Since 2008, bone morphologic proteins (rBMPs)
have been added routinely to the nonunion site [16–19].
The aim of this study was to evaluate our results for the
treatment of CPT using the above protocol.
Patients and methods
A retrospective review of medical charts and radiographs
of all patients with CPT and NF-I was performed. Ten
children, six boys and four girls, with CPT and all with
NF-I were treated between 1986 and 2011 in our hos-
pital. Two children had been treated previously in other
hospitals, with persistent pseudoarthrosis. All cases were
unilateral. Six children had right-side involvement and
four children had left-side involvement.
Approval from the institutional review board was
obtained before commencement of the study. Each sur-
gical procedure was carried out using an image intensi-
fier. The surgical technique included resection of the
psuedoarthrosis, resection of the pathologic sclerotic
bone and the periosteal tissue, realignment with ante-
grade or retrograde rush pin, application of circular frame,
Ilizarov or Taylor spatial frame for stabilization, and
compression with optional proximal lengthening. The
DOI: 10.1097/BPB.0000000000000191

Ilizarov frame was used in the patients up to 2003. Since
then, the Taylor frame has been used in all procedures.
An iliac autogenous bone graft was used in all children.
Simultaneous lengthening was performed in children
older than 5 years of age. rBMP was used only in redo
procedures when the nonunion did not heal or when
second nonunion developed. Another indication was to
facilitate union of osteotomies performed to correct distal
secondary deformities. Four children underwent simul-
taneous lengthening and four children received rBMP.
Two children were treated by a vascularized fibular graft,
one as a primary procedure and one as a secondary pro-
cedure, in addition to the above protocol after a failure of
the Ilizarov and bone graft technique. In both patients,
the resected segment was very long and the vascularized
fibular graft was used to bridge the large defect created.
Patients were divided into two groups: those who
reached skeletal maturity and those who did not. Time
for frame removal was measured, the degree of length-
ening was determined, and the refracture rate was
examined. We also looked for residual ankle valgus and
fibular nonunion in addition to leg-length discrepancy
and other deformities around the knee.
Results
Union of the pseudoarthrosis was achieved in 100% of
cases, eight patients on the first attempt and two patients
on the second attempt. rBMP was added in four children
and all healed at the first procedure.
A total of 13 operations were performed to achieve union.
Six children required further operations: two children
following a fall after union was achieved; one child had
disease spread to the regenerated bone; and three chil-
dren required axial correction.
The mean follow-up period was 89.1 months (range,
45.4–184.6 months), the mean age at last operation was
10.5 years (range, 5–20 years), and at the last follow-up
was at 18.4 years (range, 11.9–28 years).
Four children underwent simultaneous lengthening,
between 4.5 and 5.0 cm. The frame was removed after a
mean period of 7.1 months (range 5–10 months). All
patients had pin-tract infections that were treated with
oral antibiotics.
Five children reached skeletal maturity: three out of the
five had residual ankle valgus deformity (19°, 27° and
33°) and limited range of motion; one patient was treated
with six axial external devices and one patient underwent
an operation with the Ilizarov external fixator after he fell
and broke his femur. He developed ankle valgus (lateral
distal tibial angle 54°) and leg-length discrepancy of 3 cm.
Three children had asymptomatic fibula nonunion and
two had a leg-length discrepancy between 3 and 5 cm at
the last follow-up (Fig. 1a–c).
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Surgical treatment for CPT Shabtai et al. 445
Skeletally immature patients: two out of five patients had
residual ankle valgus (27° and 36°); one developed knee
valgus (mPTA 104°) and underwent proximal medial tibia
epiphysiodesis with a tension blade plate (Fig. 2a–c).
Three of the five patients had fibula nonunion with a leg-
length discrepancy of 1, 2.5, and 9.0 cm, respectively.
Discussion
The treatment of CPT is a surgical challenge for both
achieving and maintaining union. In addition, other
associated deformities of length and angulations should
be addressed before and after skeletal maturity.
Numerous surgical techniques have been described in the
literature, with variable results and rates of refractures. The
main treatment options for CPT are conservative, either with
braces, vascularized fibular grafting, IM nailing with a bone
graft, or external fixation with a circular frame, and, in rare
cases, amputation. The choice of treatment depends on dif-
ferent factors such as the type of pseudarthrosis (atrophic,
normotrophic, or hypertrophic type) and the extent of the
bone defect [2]. Conservative treatment is usually indicated
when there are no fractures and there is no progression of the
deformity; otherwise, surgical treatment may be indicated.
Mc Farland [20] first described the use of the bypass grafting
technique using autograft tibia for mechanic support of the
bowing. Ofluoglu et al. [21] treated 10 prepseudarthrosis
patients with prophylactic bypass grafting with an allograft
fibula placed posteromedially in combination with bracing. At
the final follow-up, none of the patients had fracture or
pseudarthrosis, but in four of ten patients, the deformity
persisted and corrective osteotomy was performed.
Sakamoto et al. [7] published his experience after the
treatment of eight children with a vascularized fibular
graft. Bone consolidation was achieved in all cases after an
average term of 6.6 months. Only one patient had a stress
fracture 4 months after the operation and he was treated
with a brace until union was achieved. In a multicenter
series performed by Ohnishi et al. [22], 73 patients were
treated with different surgical methods. Twenty-six
patients were treated with a circular frame, 25 with vas-
cularized fibular grafting, seven with both a circular frame
and a vascularized fibular graft, six with IM rodding
combined with free bone graft, five with plating and
grafting, and four patients with alternative treatment
methods. The best results were obtained with the Ilizarov
technique combined with a vascularized fibular graft.
There were four refractures in the Ilizarov group and one
refracture in the vascularized fibular group. In this study,
we used a free vascularized fibular graft in two cases. One
child received the graft as a primary procedure, which
resulted in failure, and the pseudarthrosis persisted.
Union was achieved only after performing the above
protocol. The second child received the vascularized
fibular graft in combination with the above protocol.
446 Journal of Pediatric Orthopaedics B 2015, Vol 24 No 5

Fig. 1

(a) (b)
R

R
1at
R R

(c)

1at
R

(a) Anteroposterior and lateral radiograph views of an 18-year-old patient. Thirteen years after resection of CPT and fixation with an intramedullary rod,
bone graft, and Ilizarov device, there was recurrent distal pseudoarthrosis after a failed attempt to correct axial deformity. (b) Anteroposterior and
lateral radiograph views 7 months after proximal lengthening, distal compression with Ilizarov, bone graft, and rBMP. (c) Anteroposterior and lateral
radiograph views after union was achieved. There was also residual shortening with ankle stiffness. CPT, congenital pseudarthrosis of tibia; rBMP,
recombinant bone morphologic protein.

Dobbs et al. [12] reported their results after treatment of
CPT in 21 children with an IM rod and autologous bone
grafting. The primary union rate was 86%. After a mean
follow-up of 14.2 years, 12 patients (57%) experienced
refracture and five patients (24%) underwent amputation.
Horn et al. [23] used the Ilizarov method for the treatment
of 15 children. Primary healing was achieved in more than
half of the patients. However, refracture occurred in all
except one patient within 4 years and required multiple
surgeries to achieve permanent healing.
Another method of treatment was presented by Birke
et al. [24]. They treated two children with a Fassier–Duval
telescopic rod. Both children required several reopera-
tions for nonunion, shortening, and loss of fixation.
Johnston and Birch [25] compared IM fixation following
resection/shortening of the pseudarthrosis as opposed to
reconstruction with an Ilizarov external fixator following
resection in two apparently identical cases of CPT. The first
method resulted in normal functioning limb at maturity,
whereas the second method resulted in amputation. The
authors suggested that acute shortening and compression
might provide early weight bearing and bone union.
Choi et al. [26] described the advantages of the Ilizarov
technique for the treatment of CPT. The Ilizarov device
can address complex deformities associated with this
condition. It can be reapplied in the event of refracture,
address bone loss and leg-length discrepancy, and can be
combined with other surgical techniques. In another
study, Choi et al. [27] described the ‘4-in-1
Osteosynthesis’ technique in eight cases of atrophic type
of CPT in which the fibula was also involved. In this
technique, both the tibia and the fibula are fused toge-
ther in one mass. In his study, stabilization of the ankle
was achieved in all eight cases and no refracture occurred.
The Masquelet technique is another treatment option
for atrophic CPT in which the fibula is also involved.
This two-stage technique was originally described for the

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 Surgical treatment for CPT Shabtai et al. 447

Fig. 2

(a) Anteroposterior and lateral radiograph views of a 5-year-old boy. No previous surgical history. (b) Anteroposterior and lateral radiograph views
3 months after resection of the pseudoarthrosis, intramedullary rod, distal compression with Ilizarov, and rhBMP. (c) Anteroposterior and lateral
radiograph views after union was achieved. There was also 1 cm shortening and asymptomatic broken rush pin. rhBMP, recombinant human bone
morphologic protein.

treatment of an extensive diaphyseal bone defect.
Pannier et al. [28] treated five patients with this techni-
que and achieved bone union in all cases.
In a multicenter study carried out by the EPOS in 2000 in
340 patients, the Ilizarov technique was considered the best
method, yielding the highest rate of fusion (75.5%) and rate
of success in correction of the additional deformities [5].
They also recommended the use of prophylactic IM rodding
to prevent refracture. El-Rosasy and colleagues also recom-
mended the use of prophylactic IM rodding. In their study,
the refracture rate decreased from 68 to 29% in patients
treated with both IM rodding and external fixation [29].
In our study, refracture occurred in three patients (30%),
which is comparable with other studies. In two patients,
refracture occurred after a major trauma while the
patients were not wearing a brace. This emphasizes the
importance of the use of the brace to protect the limb
until skeletal maturity.
To optimize the union rate and to decrease the rate of
refracture, a combination of different surgical methods
should be used.
Thabet et al. [15] treated 20 patients, all with the appli-
cation of a combined approach including free periosteal

Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.

448 Journal of Pediatric Orthopaedics B 2015, Vol 24 No 5
grafting, bone grafting, and IM nailing of both the tibia
and fibula combined with Ilizarov fixation. Union was
achieved in all patients after the primary operation. Ten
refractures occurred in eight of the 20 patients.
In the last decade, rhBMP was used widely for the
treatment of CPT. Spiro et al. [16] combined rhBMP with
an IM rod, and the initial rate and time of union was
reduced. Lee et al. [17] treated five children with
rhBMP-7 and IM rod fixation, and found that adding
rhBMP-7 did not result in enhanced bone healing.
Birke et al. [30], in another series, treated seven patients
with a combination of BMP and bisphosphonate (BP) as
an adjuvant to surgical intervention and considered that
BP therapy may be useful in preserving the BMP-
induced bone formation by inhibiting the osteoclastic
bone loss.
Paley [4] treated 15 patients with a combination of
pharmacologic and surgical management. After a mean
follow-up of 2 years, union was achieved in all cases
without refracture. Johnston and Birch [25] recom-
mended the use of BMP as an adjuvant treatment for all
surgical cases of CPT.
Our protocol combines all of the methods of treatment
described (except for BP), including resection of patho-
logic bone and the surrounding periost, iliac bone graft-
ing, insertion of IM rod, compression with a circular
frame and simultaneous proximal lengthening in some
patients, and rhBMP as it has been added routinely
since 2008.
One limitation of this retrospective study is the small
number of patients. Fortunately, CPT is a rare condition.
More multicenter studies with larger numbers of patients
should be carried out to establish the true gold-standard
treatment for congenital pseudarthrosis of the tibia.
Conclusion
Excision of pseudoarthrosis, IM nailing, autogenous bone
grafting, rBMP, and compression with a circular frame are
very effective in achieving union in CPT. The fibular
pseudarthrosis needs to be addressed, bone grafted, and
stabilized with an IM rod whenever possible. It is pos-
sible to lengthen the tibia proximally simultaneously.
Special attention should be paid to the ankle joint to
prevent valgus deformity that may affect function.
A biological approach to the treatment of the pseudar-
throsis combined with a lengthening technique is a pro-
mising protocol to prevent amputation.
Acknowledgements
Conflicts of interest
There are no conflicts of interest.
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
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