Evaluation of Treatment of Congenital Pseudarthrosis of Tibia by IlizarovMethod

Background: Congenital pseudarthrosis of the tibia, a rare but well-known disorder, has been remarkably resistant to all types of therapy designed to promote healing. Successful treatment consists of the union of the pseudarthrosis and maintenance of that union without malunion, re-fracture, or excessive shortening (<2cm) of the leg. The principle of treatment of congenital pseudarthrosis of the tibia (CPT) with the Ilizarov method corrects all angular deformities and maximizes the cross-sectional area of the pseudarthrosis. Nineteen patients with a total of 19 CPT were treated using the Ilizarov apparatus. CPT is the most perplexing challenging pediatric orthopaedic problem especially when the patient of below years of age and has a history of the previous operations and tibia narrow and osteoporotic. Therefore, the purpose of this study was to evaluate the results of treatment of CPT by the Ilizarov method. Material & Methods:The prospective study was done from July 2008 to June 2010 at the National Institute of Traumatology and Orthopedic Rehabilitation, Dhaka. Various forces were used to treat the pseudarthrosis site including compression, distraction, open reduction, resection and shortening, resection and bone transport, and a temporary intramedullary k-wire given through calcaneum and talus into the tibial medullary cavity.Results:Lengthening was performed in all except one of the 19 patients. One patient had developed nonunion in both the pseudarthrosis site and proximal corticotomy site. The deformity was tried to correct in all cases. The union rate was 94.73% with one treatment. There were three early re-fracture. Eight patients. had a persistent residual deformity of ankle valgus from 5-9 degrees and five patients had residual angular deformity at the pseudarthrosis site from 5-10 degrees.Conclusions:One patient’s angulation degree required revision surgery, Ilizarov. All patients were given Previous sites, residual angular deformity, and natural history were considered predisposing factors for re-fracture. Two re-fractures united with months (range 10 months). This technique produced initial pseudarthrosis with the correction associated with deformity inequality. angulation and valgus.

Treatment strategy of congenital pseudarthrosis of the tibia in children / 中华实用儿科临床杂志

Congenital pseudarthrosis of the tibia(CPT) is one of the most challenging diseases confronting pediatric orthopedic surgeon.The goal of the treatment is to achieve primary union and minimize the incidence of refracture and residual deformities.An optimal surgical treatment for the management of CPT is currently unavailable.Combined surgery can obtain good results.However, duo to heterogeneity of the disease, variable patients require individualized treatment to get a better prognosis.

Long Term Results of Congenital Pseudarthrosis of the Clavicle: A Case Report of 22 Years Follow-up

Congenital pseudarthrosis of the clavicle is a rare condition, which rarely produces functional disabilities except for cosmetic problems. Surgical treatment involves autogenous iliac bone grafts and internal fixation. Few studies have reported long-term results of surgical treatment or morphological changes of the clavicle. We report a patient with a congenital pseudarthrosis of the clavicle, who demonstrated a near normal radiographic appearance of the clavicle and an excellent result 22 years after the operation performed at 4 years of age.

Ilizarov Treatment of Congenital Pseudarthrosis of the Tibia: A Multi-Targeted Approach Using the Ilizarov Technique

Congenital pseudarthrosis of the tibia (CPT) is one of the most challenging problems in pediatric orthopaedics. The treatment goals are osteosynthesis, stabilization of the ankle mortise by fibular stabilization, and lower limb-length equalization. Each of these goals is difficult to accomplish but regardless of the surgical options, the basic biological considerations are the same: pseudarthrosis resection, biological bone bridging of the defect by stable fixation, and the correction of any angular deformity. The Ilizarov method is certainly valuable for the treatment of CPT because it can address not only pseudarthrosis but also all complex deformities associated with this condition. Leg-length discrepancy can be managed by proximal tibial lengthening using distraction osteogenesis combined with or without contralateral epiphysiodesis. However, treatment of CPT is fraught with complications due to the complex nature of the disease, and failure is common. Residual challenges, such as refracture, growth disturbance, and poor foot and ankle function with stiffness, are frequent and perplexing. Refracture is the most common and serious complication after primary healing and might result in the re-establishment of pseudarthrosis. Therefore, an effective, safe and practical treatment method that minimizes the residual challenges after healing and accomplishes the multiple goals of treatment is needed. This review describes a multi-targeted approach for tackling these challenges, which utilizes the Ilizarov technique in atrophic-type CPT.

Manejo quirúrgico de la pseudoartrosis congénita de tibia y peroné: experiencia del Hospital Ortopédico Infantil de Caracas / Surgical management of congenital pseudarthrosis of tibia and fibula: experience of the Hospital Ortopédico Infantil de Caracas

El tratamiento de la Pseudoartrosis Congénita de Tibia y Peroné (PCTyP) ha sido diverso. Todas las técnicas de tratamiento tienen una cuota de complicaciones, pero es la re-fractura y la falta de unión las principales consecuencias que se desean evitar. El objetivo de esta investigación se basa en analizar las técnicas quirúrgicas empleadas en nuestro centro y determinar los resultados. Se realizó un estudio retrospectivo, donde se estudiaron 11 pacientes con esta enfermedad con un seguimiento promedio de 4,73 años (DS±4,56). Todos los pacientes en quienes se utilizó la técnica de Charnley-Williams lograron la consolidación, la protección del clavo funcionó hasta que el extremo proximal de la tibia queda desprotegido al crecer el hueso y se produjeron angulaciones en esta zona, usualmente en la tibia proximal. Con el uso del clavo endomedular telescópico de Fassier-Duval se logra la protección de todo el hueso, inclusive durante el crecimiento. Se concluye que la técnica de Charnley-Williams es un buen método para la resolución de esta enfermedad y con el uso del clavo endomedular de Fassier-Duval se evita la refractura y se logra la unión permanente a medida que continúa el crecimiento del hueso. La discrepancia de los miembros inferiores es debido a la afectación de la tibia que compromete su crecimiento normal.

Treatment of Congenital Pseudarthrosis of Tibia and Fibula (PCTyP) has been mixed. All treatment techniques have a share of complications, but is the re-fracture and nonunion of the main consequences are to be avoided. The objective of this research is based on analyzing the surgical techniques used in our center and determine the results. We performed a retrospective study, which studied 11 patients with this disease with a median follow-up of 4.73 years (SD ± 4.56). All patients who used the technique of Charnley-Williams achieved the consolidation, protection of key works until the proximal end of the tibia is unprotected to grow bone and angulation occurred in this area, usually in the proximal tibia. With the use of intramedullary nail-Duval telescopic Fassier protection is achieved across the bone, even during growth. We conclude that the technique of Charnley-Williams is a good way to solve this disease and the use of intramedullary nail-Duval Fassier refracture and prevents the joining is accomplished as bone growth continues. The discrepancy of the lower limbs is due to the involvement of the tibia which assumes normal growth.

Lenthening of Tibia in CPT by Ilizarov’s Method.

A case of Congenital Pseudarthrosis of Tibia (CPT) with 24 centimetres shortening in a young boy of eighteen years treated by Ilizarov’s method is presented. No features of neurofibromatosis like café-aulait spots or skin nodules were seen. Excision of Pseudarthrosis site, deformity correction and the lengthening was done by Ilizarov’s apparatus, through upper tibial metaphysis. The follow up of two years showed sound union. It gives credence to Ilizarov’s as being one of the best methods for treatment of CPT and large shortening.

Long-term Follow-up Results of the Free Vascularized Fibular Graft in Congenital Pseudarthrosis of the Tibia / 대한정형외과학회잡지

PURPOSE: To report the long-term follow-up results of a free vascularized fibular graft (FVFG) for the treatment of patients with congenital pseudarthrosis of the tibia (CPT). MATERIALS AND METHODS: Fourteen patients with CPT, who underwent FVFG and were followed-up for more than 4 years, were enrolled in this study. The average age at FVFG was 4.5 years (range, 1~10.6 years) with an average follow-up of 11.6 years (range, 4~26.6 years). In 11 cases, 24 additional procedures were performed due to the secondary problems, such as delayed union, nonunion, leg length discrepancy, and deformity of the tibia. The clinical and radiological data were reviewed to evaluate the outcomes of the FVFG. RESULTS: Bone union and hypertrophy of the fibular graft was observed in all cases. The average time of union in 7 cases, in whom bone union was achieved without the aid of any supplementary procedures, was 6.7 months (range, 3.5~11 months). At the last follow-up, the mean limb length discrepancy was 2.5 cm (range, 0~8 cm), and the average anterior apex and valgus angulation of the tibial shaft were 18.4degrees (range, 0~85degrees) and 9.9degrees (range, -5~34degrees), respectively. The mean ankle alignment was valgus 7.2degrees (range, -3~30degrees), and the mean range of motion of the ankle joint was dorsiflexion 17degrees and plantar flexion 30degrees. CONCLUSION: FVFG is an effective procedure in congenital pseudarthrosis of the tibia which has the advantages of allowing a more aggressive resection of the abnormal bone and scar tissue and as well as resolving the bone defect after resecting the lesion. This procedure is expected to produce a better clinical result if rigid internal and external fixation are performed.

Congenital Pseudarthrosis of the Clavicle: Report of 4 Cases Treated with Surgical Methods / 대한정형외과학회잡지

Congenital pseudarthrosis of the clavicle is a rare condition, and optimal treatment strategies are controversial because this benign condition rarely produces functional disabilities except for an unsightly lump and occasional mild weakness of muscle strength around the shoulder girdle. Nevertheless, its prominence increases with age and it can cause cosmetic problems by drooping and shortening of the shoulder. Thoracic outlet syndromes have been reported in a few patients who did not receive treatment. The authors here report on congenital pseudarthrosis of a unilateral clavicle in 3 patients and bilateral clavicles in 1 patient. All patients were treated with autogenous iliac bone grafts and internal fixation, with a complete union and cosmetically satisfying results.

Expressions of VEGF and TGF-? in periosteum in patients with congenital pseudarthrosis of tibia / 吉林大学学报(医学版)

Objective To approach the expressions of vascular endothelial growth factor(VEGF) and transforming growth factor-beta(TGF-?) in periosteum in patients with congenital pseudarthrosis of tibia(CPT) and elucidate the pathogenesis possibility.Methods The expressions of VEGF and TGF-? in 19 specimens from patients with CPT were detected by using immunohistochemical method.Ten normal periosteum from the healing site after tibia fracture were taken as negative group;15 fresh periosteum from the close tibia fractures were positive group.Results VEGF and TGF-? expressed in vascular endothelial cytoplasm of periosteum.The expression levels of VEGF and TGF-? of CPT were lower than those in positive group(P0.05).Conclusion The decreasing of expression levels of VEGF and TGF-? in periosteum may be involved in pathogenesis of CPT.

Congenital Pseudarthrosis of Ulna: A Case Report / 대한정형외과학회잡지

Congenital pseudarthrosis of the ulna is an extremely rare disease that is usually associated with neurofibromatosis. Various treatments have been introduced such as conservative treatment, nonvascularized bone graft, free vascularized fibular graft, one bone forearm procedure and the Ilizarov technique. However, it is difficult to obtain bone union. We report a case of pseudarthrosis of the distal ulna that was treated by a one bone forearm procedure.

Congenital Pseuldarthrosis of the Tibia / 대한정형외과학회잡지

Congenital pseudarthrosis of the tibia was accepted as one of the most difficult disease in respect of the frequency and treatment for the orthopaedists. Since April, 1984, we experienced 3 cases of congenital pseudarthrosis of the tibia, one 2 year-old boy involved left tibia only and the other 4 year-old female involved bilaterally, obtained solid bony union in all which were treated by massive surgical management that contained vascularized bone graft and electric stimulation. Several complications were faced such as refracture, valgus deformities of the ankle, skin irritation sign, angular deformities. But refracture was healed without delayed or nonunion by simple plaster cast immobilization. The vascularized bone graft snd electric stimulation is thought to be more improved methods for the treatment of congenital pseudarthrosis of the tibia. Althought solid bony union was obtained in congenital pseudarthrosis of the tibia, periodic follow-up and adequste management must be mandatory up to the end of the bony growth.

Treatment of Congenital Pseudarthrosis of the Tibia with Electrical Stimulation / 대한정형외과학회잡지

We performed a clinical study of the effect of electrical current on the congenital pseudarthrosis of the tibia from May, 1980 to January, 1984. Eight congenital pseudarthrosis were treated with direct current stimulation, six of which had received previous treatment at least more than one time in any manner and two had none. Four were failures in previous treatment with vascularized fibular graft due to the non-uoion at the proximal or distal sites of grafted bones. Six of eight congenital pseudarthrosis were treated with invasive type electrical stimulators, and the remaining two were treated with semi-invasive type. The average follow-up period was 26 months (range from 8 to 39 months). As a result satisfactory union has occured in seven out of eight cases. The mean time for union was 27 weeks (range from 15 weeks to 48 weeks). No serious complication was observed, but in our experience, the semi-invasive type, comparing to invasive type, was handicaped with minor complications such as pin tract infection and skin sloughing due to the irritation by anode pad, and needed frequent recharging to maintain adequate voltage. With above result, we think that the electrical stimulation has revealed to be one of the reliable methods in the treatment of surgery-resistant congenital pseudarthrosis.