Introduction: Aim of this study is to evaluate the results of the treatment using the technique of dual cortical graft.

Material and Methods: Twenty-one patients (13 boys, 8 girls, age 3–8 years) were treated in period 1996–2008 in our institution. In seventeen patients, dual cortical graft harvested from tibia of parents was used for reconstruction of pseudarthrosis. NF type I was the cause of pseudarthrosis in 13 patients. After resection of the hamartomatous fibrous tissue, sclerotic bone ends and periosteum and reduction of angulations, the cortical grafts are placed on lateral and medial side of the tibia and are fixed thru the tibia proximally and distally with conventional screws. Cast immobilization follows for 9–12 weeks. In concomitant fibular pseudarthrosis, intra-medullary fixation and cancelous bone grafting treat the pseudarthrosis. In 11 patients, this technique represented the 1st surgical procedure, whereas in 6 cases (including 2 failed vascularised fibular transfers), 1–15 previous surgical procedures were used in treatment. Further surgical procedures followed for correction of the leg length discrepancy and deformity of the ankle.

Results: The fusion of non-union was achieved in 16 patients. 12 patients reached skeletal maturity (59%) whereas 4 patients (24%) did not finished the growth and are still in treatment. 10 patients use the protection brace and intramedullar nail was used in 2 patients to prevent refracture. One patient refused further treatment and amputation followed in another institution. Concerning functional outcome, 14 patients walk unlimited, 2 patients walk more then 1 hour and 1 patient walks less then 1 hour. 13 patients did not use the walking aid, 2 patients use 1 cane a 2 patients the crutches. The complication rate of this method is similar to the other methods, mainly due to the refractures. Only in one patient, the pseudarthrosis primarily fused after single procedure. In 3 patients, second procedure was necessary. In remaining 12 patients, 3–6 further surgical procedures were used for the healing of pseudarthrosis due the delayed-union of grafts or refracture of the tibia. In these cases, cancelous bone grafting and various techniques of fixation were used to achieve the union.

Discussion: and conclusion: The results of the dual cortical grafting technique are comparable to other effective methods like Ilizarov method and the method is useful also after previous failed procedures. The complication rate is similar to other methods. The prevention of the refracture by bracing or rodding is recommended in all patients. This surgical procedure should be delayed until 4 years of age.

Significance: The method of dual cortical grafting represents still effective method of treatment of CPT.

Aims: Congenital aplasia of the knee cruicate ligaments is considered to be a very rare anomaly of musculoskeletal system. It is frequently found in conjunction with other deformities. Proximal femoral focal deficiency (PFFD) is a congenital defect which is almost always accompanied by cruciate ligament defect.

The aim of our study was to determine the occurence of cruciate ligament aplasia arthroscopically.

Method: Arthroscopies of the knee joints were always performed as part of other primary operations for PFFD.

In our followed series consisting of 50 PFFD patients, 26 boys and 24 girls, five boys with PFFD classified respectively as Pappas III, VII, VII, VIII and IX and 3 girls with PFFD classified respectively as Pappas III, VII and VIII were examined arthroscopically. Deficiency of cruciate ligaments was also documented in a pateint with the diagnosis of isolated aplasia of the fibula.

Changes in the shape of intercondylar area of the distal femur, and aplasia or hypoplasia of the intercondylar ridge of proximal tibia in all PFFD patients were evaluated by an X-ray.

Conclusions: In all patients who underwent arthroscopy absence of both cruciate ligaments was proven.

Seriousness of the anomaly was inversely related to the degree of classification according to Pappas. None of our patients suffered from problems arising from knee instability. Based on our experience we recommend a reserved approach as far an indication for cruciate ligament reconstruction in PFFD patients is concerned.

Type IIb, so called mobiled pseudoarthrosis according to Paley classification, is characterized by congenital pseudoarthrosis of proximal femur with an isolated small and stiff femoral head. We are unable to create a moveable hip joint but appropriate length of the affected extremity can be reached by gradual lengthening. In previous classification it is known as Type Aitken C or Pappas III.

Type IIIa, with diaphysial deficiency of femur, corresponds to Type D according to Aitken or to Type I and II according to Pappas.

In Type IIIa, the knee joint is developed and functional with the ROM more than 45 degrees. In Type IIIb the knee joint is more or less stiff and functionally unuseable.

These three groups present the most severe congenital short femur deformities, but their occurrence is fortunately very seldom – less than 1 in 300 thousand live births. Among 41 cases of congenital short femur Pappas I–IV which were collected during 30 years from the Czech population of 10 million – Pappas I was seen in one case, Pappas II in five cases, Pappas III in 16 cases and Pappas IV in 19 cases. From the 16 cases of Pappas III deformity was found in three of them – stiffness of isolated femoral head was found and these three patients were added to this group.

Method of Treatment: In Type IIb we use complex treatment consisting of six consecutive steps:

Distraction of the distal part of femur up to acetabular level

Lengthening between 15 and 39 cm was reached.

In Type IIIa, ilio-femoral fusion (knee-for-hip procedure) was performed in five cases. The functional results are excellent. There was no need for Syme amputation or rotationplasty. The prerequisite is at least 60 degrees arc of motion in the knee joint. Severe restricted ROM in the knee joint may lead to pseudoarthrosis.

In Type IIIb (2 cases), the residual fragment of distal femur with unfunctional knee joint was stabilized in socket formed after pelvic osteotomy in the level of original acetabulum. The removal of telescopic proximo-distal movement stabilized the supportive function of the extremity.