Orthopedic treatment of osteochondrodysplasia

I. Overview Abnormal bone fiber hyperplasia is a common disease of benign bone injury, mostly seen in young people during development; the onset is early and the lesions develop slowly. The lesions can be solitary or multiple, but solitary lesions are more common. They are seen in the maxillofacial region, long bones, and ribs. The bone tissue at the lesion site shows abnormal fibroblast proliferation and poor bone calcification. Abnormal osteofibroblastic hyperplasia often occurs in combination with other syndromes or as a clinical manifestation of one of them. Recent basic research has shown that the pathogenesis is related to abnormalities in the GNAS-encoded alpha-G protein. mutations in the GNAS1 gene lead to abnormal alpha-G protein, elevated cAMP levels, and elevated IL-6 expression levels; and IL-6 directly leads to abnormal osteoblast differentiation and activation of osteoclasts. Abnormal maxillofacial bone fiber hyperplasia leads to facial morphological deformities and some patients develop functional abnormalities, such as visual and hearing impairment and abnormal occlusal function. Clinical treatment At present, the treatment of maxillofacial osteochondrodysplasia is still mainly surgical. The aim of surgical treatment is to restore the normal shape of the face as the main purpose. Surgery is divided into two types: conservative surgery and radical plastic treatment. 1.Conservative surgery: Most of the surgical procedures are based on conservative treatment, i.e., removal of part of the diseased bone tissue. 2, the fundamental treatment: to restore the normal facial shape for the purpose of removing the hypertrophic bone tissue, correcting the facial skew, restoring the normal jaw plane and occlusal relationship. With conservative surgical treatment, the patient’s facial shape may still have different degrees of asymmetry and deviated jaw deformity after surgery, and some patients have bloated soft tissues on the affected side. Therefore, this approach is more appropriate for patients with unstable disease. For the orthopedic treatment of this type of disease, personal experience is as follows: 1. For patients before the age of 24, we take conservative treatment because there is a possibility of continued proliferation of osteofibrodysplasia before puberty, and after performing major surgery such as osteotomy and orthognathic surgery, the final effect will change due to the development of the lesion, even if the treatment is good at that time, so we take conservative treatment and do complete treatment after the condition is stabilized. In addition, it should be noted that: 1. some patients have affected vision or other organ functions due to the compression of the optic nerve by the lesioned tissue, so the symptoms of compression should be relieved in time to restore organ functions. 2. children with severe facial deformation also need timely treatment to avoid difficulties in future treatment after severe deformation. 2, for patients after the age of 24, the condition is stable and we use radical plastic surgery treatment. If there are patients with severe deviated jaws and facial deviation, we use fundamental plastic treatment methods to perform osteotomy methods, reconstruction of occlusal relationship, facial contouring, and soft tissue forming surgery to obtain better clinical results. Generally speaking, there is a possibility of self-healing after the age of 24, in addition, the condition is relatively stable at this time, so the above treatment methods can be considered. 3.Medication The main purpose of medication for abnormal bone fiber hyperplasia is to inhibit the abnormal proliferation of bone fibers and promote the normal calcification of bone. These drugs include aromatase inhibitors, estrogen receptor blocking drugs, and osteoclast activity inhibitors such as sodium pamiphosphate. However, there are different problems with the therapeutic effect of each of these drugs. The long-term efficacy of pamiphosphate sodium was not significant and did not differ significantly from the control group. The effect of sex hormone antagonists and agonists is also not very obvious, such as testosterone and gonadotropin-releasing hormone agonists, etc. The side effects of some of these drugs have some effects on the development of patients. For example, the patients’ secondary sexual characteristics are poorly developed and abnormal endocrine levels occur.