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Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_II | Pages 333 - 334
1 May 2010
Dungl P Chomiak J Frydrychová M Ostadal M Adamec O
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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

Connection between head and diaphysis

First femoral lengthening

Lengthening of the tibia

Contralateral epiphysiodesis around the knee

Plastic surgery

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.


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_I | Pages 116 - 116
1 Mar 2006
Frydrychová M Dungl P Chomiak J Adamec O
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Purpose: To give the review of the foot deformities in the patients with myelomeningocele (MMC), their relationship to the level of the neurological lesion and therapeutic possibilities.

Material & Methods: Since 1998 till 2004 there were 20 patients with myelomeningocele treated in our clinic, in 19 patients was noted the foot deformity (total 34 feet). The deformity was unilateral in 4 patients, asymmetric in 2 patients (equinovarus + calcaneovalgus or planovalgus). The most frequent was equinovarus deformity (16 feet, 47%), next planovalgus deformity (9 feet, 26,5%), calcaneovalgus (7 feet, 20,6%) and equinovalgus (2 feet, 5,9%). In 28 feet the surgery was done. For the correction of the various deformities of the feet were used soft tissue releases or tendons transfer, in the older patient or after failed soft tissue release the bony operation was done (tarsal osteotomy, talo-calcaneal stabilisation, artrodesis).

Results: The aim of every type of surgery was to achieve the acceptable foot shape, with plantigrade step and possibility of weigh bearing, with minimal risk of the pressure necrosis. In the most patients the purpose was achieved, although some of them passed several surgeries. In 18 deformities only one surgery obtained the correction of the deformity, the but for the 10 feet subsequent surgery was required because of the residual or relapsing deformity. In 3 patients was noted the pressure necrosis, in 1 patient appeared the fracture of the distal tibia (epiphyseolysis) after the removal the postoperative plaster.

Conclusion: The orthopaedic care about the patients with MMC is only a part of the interdisciplinary approach of several specialists. The procedures for the correction of the neurogenic deformities of the feet, belong to the delicate surgery and is necessary very careful indication in relation with the type of deformity, which is dependent on the high of the lesion, and with the expectancy of walking. In the patients with good prognosis of walking is necessary to choose the procedure which guarantee weight-bearing and plantigrade step without the risk of ischemic skin defects.