© Borgis - Postępy Nauk Medycznych 6/2012, s. 523-529
*Milud Shadi, Paweł Koczewski
Kompleksowe leczenie wrodzonego stawu rzekomego goleni z zastosowaniem przeszczepu okostnej i stabilizacji śródszpikowej
Complex treatment of congenital pseudarthrosis of the tibia with periosteal grafting and intramedullary fixation
Department of Pediatric Orthopaedics and Traumatology, University of Medical Sciences, Poznań
Head of Department: Prof. Andrzej Szulc, MD, PhD
Streszczenie
Wstęp. W ostatnich latach w leczeniu wrodzonego stawu rzekomego piszczeli (WSRP) pojawiły się nowe metody, takie jak zastosowanie przeszczepu okostnej, białek morfogenicznych (BMP) czy bifosfonianów. W naszym ośrodku od 2006 r. wprowadziliśmy technikę operacyjną złożoną z: ograniczonej resekcji stawu rzekomego z szerokim usunięciem zmienionej okostnej, stabilizacji śródszpikowej oraz obłożeniem przeszczepami z kości gąbczastej i okostnej pobranymi z talerza kości biodrowej.
Materiał i metody. Materiał obejmuje 7 pacjentów ze średnią wieku 5,6 lat. 6 chorych prezentowało objawy neurofibromatozy typu I. Według klasyfikacji El-Rosasy-Paley typ I WSRP występował u 2 chorych, typ II u 5. Według klasyfikacji Crawford wszyscy należeli do typu IIC. Konfiguracja stawu skokowo-goleniowego była oceniona na radiogramach w projekcji a-p, a do oceny proksymalnej migracji strzałki zastosowano skalę Malhotra skrócenia strzałki. Skrócenie kończyny wahało się od 1 do 10 cm (średnia 4,7). Średnia okresu obserwacji wynosiła 3 lata i 1 miesiąc (od 2,1 do 4,6).
Wyniki. Pierwotny zrost WSRP uzyskano u 6 chorych w okresie od 4 do 6 miesięcy (średnia 4,5). U najmłodszego chorego brak zrostu po 13 miesiącach wymagał reoperacji z zastosowaniem tej samej techniki.
Wnioski. Wygojenie wrodzonego stawu rzekomego piszczeli może być uzyskane i utrzymane po zastosowaniu kompleksowego leczenia złożonego z ograniczonej resekcji kostnej, szerokiej resekcji patologicznej okostnej, stabilizacji śródszpikowej piszczeli i strzałki połączonej z przeszczepami z kości gąbczastej i okostnej.
Summary
Introduction. In the last years to improve the outcomes of congenital pseudarthrosis of the tibia (CPT) treatment, new procedures were added to the standard: periosteal grafting, bone morphogenic protein (BMP) and bisphosphonate. In this study we analyze results of CPT treatment with periosteal graft combined with intramedullary nailing.
Materials and methods. Retrospective study of medical records and the radiographs of 7 patients treated because of CPT with resection of the hamartomatous periosteum, splitting of proximal tibial end or pseudarthrosis site resection, IM fixation, periosteal and cancellous bone grafting from the iliac bone was made. Mean age at the surgery was 5.6 years. Six patients had NF-1 and one patient – idiopathic CPT. LLD ranged 1.0-10.0 cm (average 4.7). The anteroposterior preoperative scan of all patients were studied for assessments of ankle joint mortise, where proximal migration of the fibula was evaluated according to Malhotra scale for fibular shortening. The patients were classified according to El-Rosasy-Paley classification: 2 patients as type I, and 5 patients as type II. All of them were type IIC according to Crawford classification. The average follow-up after the index operation was 3.1 years (range 2.1-4.6).
Results. Primary pseudarthrosis union of the tibia was achieved in 6 patients, at a mean time of four and half months (range 4-6 months). Failure to obtain bone union after 13 months in youngest patient needs reoperation with the same technique.
Conclusions. Primary union of CPT in most of the cases can be obtained and maintained with limited pseudarthrosis resection, periosteal and cancellous bone grafting and intramedullary rod fixation.
Introduction
Congenital pseudarthrosis of the tibia (CPT) remains one of the most challenging pediatric problems in management and prognosis. It is uncommon disorder where the incidence is 1 per 140 000 to 190 000 patients. The diagnosis usually is made early under two years of age, while very rarely occur in older than eleven years of age. CPT has heterogeneous clinical picture. It may present as established pseudarthrosis in early age or only anterolateral bowing of the tibia that can fracture spontaneously and presents picture of real pseudarthrosis. The same situation may be initiated by osteotomy at the bowing site (1).
The precise etiology is still unclear, although 50% to 55% of patients with CPT have neurofibromatosis-1 (NF-1), but CPT may develop even without symptoms of NF-1. The cause may be neurofibroma grows within bone cortex leads to fracture and interference of bone union although this theory is very rarely confirmed histologically (1) except cases of Green and Rudo (2). They concluded that in true NF-1 cases, the Schwann and fibroblast cells should be seen in soft tissue but in electronic microscope, usually only fibroblast was seen in the pseudarthrosis site.
Although the main pathology in CPT is still unknown, the recent studies showed that the basic pathology is hyperplasia of fibroblast and formation of dense fibrous tissue. This invasive fibromatosis is located in the periosteum and between the bony ends surrounding the tibia which causes compression, osteolysis and persistence of pseudarthrosis (3-7).
According to Hermanns-Sachweh et al (8), the most striking finding was thickened periosteum with accumulation of nerve cells surrounding small arteries, causing subtotal or complete obliteration and local hypoxia which interfere the osteogenesis effect of the periosteum. It results in impairments of vascularization, which leads to decreasing of osteogenic capabilities. As the basic pathology in CPT is located in surrounding soft tissue while the bone is involved secondarily, Aegerter speculated that if all tumour tissues were removed, the normal callus would form (4).
The aims of CPT treatment are to achieve and to maintain the union until the skeletal maturity and to obtain the functional lower extremity. Difficulties to achieve these goals, regardless to surgical technique were mainly biological in nature (due to poor healing ability of the dysplastic bone segment) or mechanical (technical difficulty to fix the short osteopenic bone segment without transfixing the joint).
The modern treatment option includes pseudarthrosis site resection, shortening, grafting, intramedullary fixation or rodding in addition to reconstruction procedures with Ilizarov devices or microvascular bone transfer (9).
All these operative techniques, including the Ilizarov method which is considered as a choice of treatment of CPT, don’t fully guarantee the successful treatment of pseudarthrosis.
To improve the treatment outcomes, in the past years, new procedures were added as the standard: periosteal grafting, bone morphogenetic protein (BMP) and bisphosphonate (10).
Recently, the periosteal grafting as the CPT treatment was described by El-Rosasy et al (6) and Weber (7). This technique depends on to the type of the pathology and includes: resection of the hamartomatous periosteum, splitting of proximal tibial end or pseudarthrosis site resection, intramedullary fixation, periosteal grafting from the iliac bone and Ilizarov frame fixation. Since 2006, we have used the similar technique without external fixation.
The aim of this study is to evaluate our method’s results and the advantages, including the primary union after the index operation without any supplementary procedures.
Materials and Methods
Retrospective study was made on seven CPT patients treated with the method described above between 2006 and 2009 in our center. The analysis was done on medical records and radiographies. The mean age at the surgery was 5.6 years (range 2.7-9.2). Four patients were boys and three were girls. The left side was involved in four patients and the right in three patients (tab. 1). Five patients had established fibular pseudarthrosis while in two patients the fibula was intact. Six patients had NF-1 and one patient had idiopathic CPT.
Table 1. Patient’s history.
| Case | Sex | Age (yr.) | Side | NF-1 | El-Rosasy type | Crawford type | No. of previous surgeries | LLD (cm) | Site of CPT | Fibular CP |
| 1 | M | 6.1 | L | Yes | II | IIC | 8 | 7 | Distal | Yes |
| 2 | F | 8.2 | L | No | II | IIC | 5 | 10 | Distal | Yes |
| 3 | M | 5.4 | R | Yes | II | IIC | 2 | 4 | Distal | Yes |
| 4 | M | 9.2 | L | Yes | II | IIC | 4 | 3 | Distal | Yes |
| 5 | F | 2.7 | R | Yes | I | IIC | 0 | 1 | Distal | Yes |
| 6 | F | 3.5 | L | Yes | I | IIC | 0 | 4 | Middle | No |
| 7 | M | 4.1 | R | Yes | I | IIC | 1 | 4 | Distal | No |
Only two patients had no previous surgery before the described index operation, while the other five patients had been treated unsuccessfully with several surgical operations (4 to 8 times). The eldest two patients (case 1 and 2) underwent different procedures elsewhere, including: plate and screws fixation, nailing with bone graft, Ilizarov device application (twice in the case 1). Intramedullary fixation with autogenous bone graft has been used four times in one patient (case 4) and two times in another (case 3), curettage and frozen bone graft had been used in the other one (case 7). In all patients, the pseudarthrosis was associated with limb length discrepancy ranges from 1.0 to 10.0 cm (average 4.7).
The anterioposterior preoperative scan of all patients were studied for assessments of ankle joint mortise, where proximal migration of the fibula was evaluated according to Malhotra scale for fibular shortening (11).
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Piśmiennictwo
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