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The Bone & Joint Journal
Vol. 95-B, Issue 8 | Pages 1027 - 1034
1 Aug 2013
Khan T Joseph B

Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 8 | Pages 1134 - 1139
1 Aug 2011
Schindeler A Birke O Yu NYC Morse A Ruys A Baldock PA Little DG

Congenital pseudarthrosis of the tibia is an uncommon manifestation of neurofibromatosis type 1 (NF1), but one that remains difficult to treat due to anabolic deficiency and catabolic excess. Bone grafting and more recently recombinant human bone morphogenetic proteins (rhBMPs) have been identified as pro-anabolic stimuli with the potential to improve the outcome after surgery. As an additional pharmaceutical intervention, we describe the combined use of rhBMP-2 and the bisphosphonate zoledronic acid in a mouse model of NF1-deficient fracture repair.

Fractures were generated in the distal tibiae of neurofibromatosis type 1-deficient (Nf1+/−) mice and control mice. Fractures were open and featured periosteal stripping. All mice received 10 μg rhBMP-2 delivered in a carboxymethylcellulose carrier around the fracture as an anabolic stimulus. Bisphosphonate-treated mice also received five doses of 0.02 mg/kg zoledronic acid given by intraperitoneal injection.

When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in Nf1+/− mice remained ununited at three weeks compared with 7% of controls (p < 0.001). Systemic post-operative administration of zoledronic acid halved the rate of ununited fractures to 37.5% (p < 0.07).

These data support the concept that preventing bone loss in combination with anabolic stimulation may improve the outcome following surgical treatment for children with congenital pseudarthoris of the tibia and NF1.


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 12 | Pages 1627 - 1630
1 Dec 2008
Shiha AE Khalifa ARH Assaghir YM Kenawey MO

We present two children with massive defects of the tibia and an associated active infection who were treated by medial transport of the fibula using the Ilizarov device. The first child had chronic discharging osteomyelitis which affected the whole tibial shaft. The second had sustained bilateral grade-IIIB open tibial fractures in a motor-car accident. The first child was followed up for three years and the second for two years. Both achieved solid union between the proximal and distal stumps of the tibia and the fibula, with hypertrophy of the fibula. The first child had a normal range of movement at the knee, ankle and foot but there was shortening of 1.5 cm. The second had persistent anterior angulation at the proximal tibiofibular junction and the ankle was stiff in equinus.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 8 | Pages 1076 - 1081
1 Aug 2006
Vallamshetla VRP Mughal E O’Hara JN

Difficulties posed in managing developmental dysplasia of the hip diagnosed late include a high-placed femoral head, contracted soft tissues and a dysplastic acetabulum. A combination of open reduction with femoral shortening of untreated congenital dislocations is a well-established practice. Femoral shortening prevents excessive pressure on the located femoral head which can cause avascular necrosis. Instability due to a coexisting dysplastic shallow acetabulum is common, and so a pelvic osteotomy is performed to achieve a stable and concentric hip reduction. We retrospectively reviewed 15 patients (18 hips) presenting with developmental dysplasia of the hip aged four years and above who were treated by a one-stage combined procedure performed by the senior author. The mean age at operation was five years and nine months (4 years to 11 years). The mean follow-up was six years ten months (2 years and 8 months to 8 years and 8 months). All patients were followed up clinically and radiologically in accordance with McKay’s criteria and the modified Severin classification. According to the McKay criteria, 12 hips were rated excellent and six were good. All but one had a full range of movement. Eight had a limb-length discrepancy of about 1 cm. All were Trendelenburg negative. The modified Severin classification demonstrated four hips of grade IA, six of grade IB, and eight of grade II. One patient had avascular necrosis and one an early subluxation requiring revision.

One-stage correction of congenital dislocation of the hip in an older child is a safe and effective treatment with good results in the short to medium term.