Point of view 211

Congenital tibial dysplasia (congenital pseudoarthrosis of the tibia): an atypical variation

Wallace B. Lehman, Amr Atef Abdelgawad and Debra A. Sala
Journal of Pediatric Orthopaedics B 2009, 18:211213 Center for Children, NYU Hospital for Joint Diseases, New York, USA Correspondence to Professor Wallace B. Lehman, MD, Chief, Emeritus, Department of Pediatric Orthopaedic Surgery, NYU Hospital for Joint Diseases, 301 E. 17th Street, New York, NY 10003, USA Tel: + 1 212 598 6403; fax: + 1 212 598 6084; e-mail: wallace.lehman@med.nyu.edu

The results of the treatment of congenital tibial dysplasia (CTD) or pseudoarthrosis of the tibia, which is one of the most difficult conditions encountered by pediatric orthopedists, are rarely uniformly satisfactory. A considerable percentage of cases will fail to achieve union [14]. Even in those patients who obtain union, most will still need protective bracing and sometimes walking aids, which has a negative impact on their function and quality of life. The affected child along with his or her family will suffer the burden of multiple surgeries, repeated hospitalizations, long periods of external fixators and casts together with social deprivation and financial losses. With longer follow-up, functional results degenerate with some patients who initially had good results subsequently requiring amputation for persistent nonunion after refracture through the site of the lesion. In addition, other problems, including limb length discrepancy, arthritic painful ankle joint, valgus ankle and procurvatum of the tibia, become more evident with extended followup and affect functional results [5]. Several different classification systems for CTD [69] have been reported. Currently, the most commonly used one is the Crawford classification system [810], which divides CTD into the following four types: Type I (nondysplastic): anterolateral bow with increased cortical density and narrow medullary canal. Type II (dysplastic): Type IIA: failure of tubulation and widened medullary canal. Type IIB: cystic prefracture or canal enlargement from previous fracture. Type IIC: frank pseudoarthrosis with narrowing of fragment ends. Long-term follow-up has shown that types IIA and IIC were associated with the worst functional results. Boyd [7] found that patients with type II in his classification, which is equivalent to Crawfords Type IIC, had the poorest prognosis. Even with the relatively high union rates of 90% for type IIA and 62% for type IIC reported in the EPOS Multicentric Study [11], the functional results were very poor with 38 and 11% of the patients, respectively, being full weight bearing without bracing,
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90 and 75% ambulating without a walking aid, 81 and 37% walking for an unlimited distance, and 0 and 0% participating in unrestricted sports activities. This study showed that the union rate, which is the usual parameter measured, is not a good indicator of the functional results for patients. For example, a 62% union rate in type IIC was accompanied by only 11% of the patients ambulating full weight bearing without bracing and 0% engaging in sports activities. In contrast, the children who had amputations had very good functional results, as they were able to walk without any aids for unlimited distances and participate in sports. When compared with children who had amputations, the functional outcome of the children who had surgeries to achieve union in these two types was worse than the children who had amputations [11]. Gait analysis also showed that children, who had early onset CTD, even when they had achieved union, had no functional advantages over an amputee [12]. We reviewed our CTD cases that failed to achieve or maintain union to identify common characteristics. Our six cases had failed different modalities of treatment including excision of pseudoarthrosis, bone graft, intramedullary nail, Ilizarov external fixator, vascularized graft, and electrical stimulation. Four of the patients never achieved union and the other two refractured after achieving union [13]. These cases fell into type IIC of the Crawford classification system with all of the following additional characteristics: Affecting the distal one-third of the tibia. Affecting the distal one-third of the fibula. Deformed leg. Onset within the first 2 years of life. The early detection of these cases would enable the pediatric orthopedist to avoid performing multiple surgeries and to discuss the advantages of early amputation with the family. As the identification of these patients would serve to determine the direction of their treatment, we propose adding these cases to the Crawford classification system as an atypical variation of type IIC and referring to it as type IID (Fig. 1). In our experience, these type IID cases are very resistant to achieving union. They require multiple surgeries,
DOI: 10.1097/BPB.0b013e32832fbec6

212 Journal of Pediatric Orthopaedics B 2009, Vol 18 No 5

Fig. 1

avoid the need for multiple operations and hospitalizations, the pain from frequent surgeries, the familys economic loss from repeated hospitalizations, the psychological impact of the isolation experienced by the child because of limited mobility and frequent hospitalizations, the need for protective bracing for extended periods of time and the inability to participate in sports activity. The early identification of those patients who will probably not unite or will have recurrent fractures will allow the surgeon to offer amputation as a primary, or at least an early, option with better short-term and longterm results including near normal activity and sports participation [11,12]. In addition, when presented to the family at the onset of treatment rather than after many unsuccessful interventions, amputation will not be viewed as a failure, but as a reconstruction of the lower extremity. The child and his or her family will consider the prosthesis as a part of the childs body. Good functional results have been reported for patients who had an amputation for CTD [1619]. In a long-term study, Andersen [10] showed that patients with amputations had fewer functional problems, which were mainly related to the prosthesis, compared with the patients who had achieved union with their functional problems of limb length discrepancy, limitation of motion, painful ankle joint, and atrophy of the leg and foot. His two patients, who did not receive any surgical treatment before amputation, had a reasonable functional result and required only a short period of hospitalization. He suggested that bony union is not a good predictor of the outcome in this disease and that some of his patients who had achieved union might have benefited from early amputation. Either a below-knee amputation or a Syme disarticulation [20] can be performed. As a longer stump is better for accommodating a prosthesis, a childs Syme disarticulation at the ankle joint is the better option. If the pseudoarthrosis causes instability after a Syme disarticulation, an intramedullary rod can be used to stabilize the tibia [16,18,19]. When the amputation is performed in the diaphysis of the tibia, diaphyseal appositional bone growth may necessitate multiple stump revisions as the child grows. In addition, the skin in the area of the CTD is usually unhealthy and has multiple previous scars, which may cause problems with prosthesis fitting. Other authors have described a poor outcome for their patients with similar characteristics to our patients with type IID. In 2002, Kim and Weinstein [21] found that cases with early onset disease, distal pseudoarthrosis and cases associated with fibular pseudoarthrosis were not good candidates for bone grafting and intramedullary nailing as these cases had unsatisfactory results. Murray and Lovell [3] also reported that the prognosis for early onset was worse than for late onset of the disease.

Type IID (an atypical variation of Crawfords type IIC): pseudoarthrosis with affection of distal one-third of tibia and distal one-third of fibula, deformed leg, and onset within the first 2 years of life.

repeated bone grafting, and long periods of immobilization. Even when union is achieved, refracture usually follows within a short period of time. The results of early amputation in these cases are better than multiple surgeries which leave a scarred, deformed, painful, short leg that needs permanent protection by bracing and limits the childs participation in recreational activities. Twenty-five years ago, Morrissy and his associates [14,15] sought an answer to the following question with regard to children with CTD: Which of the patients in whom the limb had been retained would have been better off, both functionally and cosmetically with an amputation? The new proposed classification system helps to answer this question. In our modified Crawford classification, type IID (an atypical variation of type IIC) carries the worst prognosis. By providing a set of criteria for this variation, the pediatric orthopedist can predict, from the beginning, that a patient with this type will have a poor outcome if treated with multiple surgeries and better function if treated with an early amputation. This will enable the surgeon to help the child and his or her family

Congenital tibial dysplasia Lehman et al. 213

In conclusion, we think that the cases of CTD that can be classified as type IID, an atypical variation of type IIC that is characterized by pseudoarthrosis affecting the distal one-third of the tibia and fibula, a deformed leg, and onset within the first 2 years of life, can be better treated with amputation rather than multiple surgeries. In these cases, the functional results for patients with amputations are better than those for patients who had repeated surgeries. The family should be warned from the beginning that a stable union is unlikely, and therefore not a realistic goal. Amputation should be offered to the family as a primary, or at least an early option along with the explanation that the functional results of early amputation in these cases are better than that of multiple surgeries, even if union is achieved.

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