Recently, an 11-year-old boy who has been limping in his right foot for more than 3 years was diagnosed with right femoral varus deformity and polyostotic bone fiber dysplasia. After reviewing relevant literature and reports, the incidence of polyostosal bone fiber dysplasia is 1/1000000, and the carcinogenesis rate is 1%.
Three years ago, an 8-year-old boy, Su Su (not his real name), suffered a fracture of his right femur due to trauma and underwent surgery at a local hospital. Soon, Su Su had a thigh varus deformity, Su Su's condition has not improved in 3 years, the symptoms of claudication have gradually worsened, the pigmentation of café au lait spots on the skin surface has deepened, affecting physical and mental health, the situation has become worse, and it is difficult to treat in many hospitals, Su Su's parents immediately took Su Su to the Department of Orthopedics of Jiangxi Provincial Children's Hospital for help.
Further analysis showed that Su Su's right thigh was deformed, the right buttock was wider, and the symptoms of claudication were obvious, showing the deformity of "shepherd crutches", and he was diagnosed with right femoral varus deformity and polyosseous bone fiber dysplasia.
Preoperative diagram
Xiong Bin, director of the Department of Orthopedics of Jiangxi Provincial Children's Hospital, Lin Zhifeng, deputy director, and Jiang Qiqing, attending physician, discussed many times that because Susu is in a critical period of growth and development, the right bone is seriously affected, and surgical treatment should be taken immediately to remove the diseased tissue, correct the deformity, and reduce the risk of fracture.
Xiong Bin said that bone fibrodysplasia is a chronic disease caused by genetic mutations, which is a congenital non-genetic disease that occurs mostly in childhood and adolescence. Clinical manifestations vary depending on the site of involvement, with the proximal femur, tibia, ribs, and skull being the most common; Depending on the number of bones involved, the disease can be divided into monoostic and polyostic. Susu's case is polyostose bone fibrous dysplasia, which is easy to cause abnormal bone cell production, the formation of excessive fibrous connective tissue, brittle bones, and even cause skin pigment changes and endocrine abnormalities in the body.
During the operation, it can be seen that Su Su's periosteum in the upper femur is fibrous thickened, the lateral cortex of the femoral shaft is a "ground glass" fibrous change, the medial femoral cortex is thickened, the proximal femur varus deformity is serious, and the femoral trochanteric area is obviously curved and deformed.
Postoperative diagram
At present, the treatment team of Xiong Bin, Lin Zhifeng, and Jiang Qiqing has taken medication for Su Su's condition to help relieve pain, improve bone strength, adjust bone growth process, and guide Su Su to carry out rehabilitation exercises such as hip and knee joint function moderately.
Xiong Bin said that in the case of Su Su, regular re-examination is required after discharge, so that the healing of the osteotomy end and the maintenance of the deformity can be monitored in a timely manner.
orthopaedics
Xiong Bin: Director of the Department of Orthopedics, Chief Physician, specializes in the diagnosis and treatment of common diseases such as developmental hip dislocation, scoliosis caused by various reasons, muscular torticollis, stenosynovitis, polysyndactyly, knee varus, knee valgus, clubfoot and childhood fractures.
Visiting hours: [Honggutan Campus] Wednesday morning, Thursday morning special outpatient clinic
Wu Xinle: Deputy Director of the Department of General Surgery, Director of the Second Department of Orthopedics, Chief Physician, specializing in the diagnosis and treatment of common, frequent and difficult diseases in pediatric orthopedics, especially limb deformities such as clubfoot and hip dislocation; bone diseases such as necrosis of the femoral head; Diagnosis and treatment of trauma and its complications.
Visiting hours: [Honggutan Campus] Tuesday morning special outpatient clinic, Friday
Du Xiangping, chief physician, specializes in the diagnosis and treatment of various pediatric orthopedic diseases, especially in the diagnosis and treatment of children's fractures, trauma and its complications and sequelae, developmental hip dislocation, clubfoot and polydactyly and other limb deformities, scoliosis, avascular necrosis of the femoral head, bone and soft tissue tumors, bone infections and other diseases.
Visiting hours: [Honggutan Campus] special outpatient clinic on Monday afternoon, morning and afternoon on Thursday
Yi Shende: Deputy Chief Physician, specializing in the surgical and orthopedic treatment of complex fractures and dislocations of children's limbs, various complex bone diseases and limb tumors in children, and congenital malformations in children.
Visiting time: [Honggutan Campus] Tuesday
Lin Zhifeng: Deputy Director of the Department of Orthopedics, Deputy Chief Physician, good at the diagnosis and treatment of congenital and developmental diseases, trauma, infection, tumors and other diseases in pediatric orthopedics; He is especially good at the minimally invasive treatment of children's bone and joint trauma and the orthopedic treatment of posterior limb deformities. He has rich clinical experience in hip dislocation, avascular necrosis of the femoral head in children, spinal deformities, foot deformities, and knee sports medicine diseases.
Visiting hours: [Honggutan Campus] Wednesday and Thursday
Xiong Zhigang: Deputy Chief Physician, specializing in the treatment of pediatric trauma and various types of pediatric fractures, bone tumors, developmental hip dislocations, spinal deformities and limb deformities, etc., especially in the minimally invasive treatment of various pediatric fractures and the correction of children's limb deformities.
Visiting hours: [Honggutan Campus] Monday; 【East Lake Campus】Friday
Zeng Xiaohui: Deputy Chief Physician, has rich experience in the diagnosis and treatment of common pediatric orthopedic diseases: trauma, deformity, tumor, infection, etc., and has rich clinical experience in hip dislocation, avascular necrosis of the femoral head in children, spinal deformity, foot deformity, knee joint sports medicine diseases.
Visiting time: [Donghu Campus] Tuesday; 【Honggutan Campus】Thursday
Zou Jun: Deputy Chief Physician, good at pediatric fractures, congenital and acquired limb deformities, clubfoot in young children, various complex polysyndactyly (toe) treatments, and has rich experience in the diagnosis and treatment of developmental hip dislocation, femoral head necrosis, hip varus and other hip joint diseases, congenital, idiopathic, and neurofibromatosis resulting in scoliosis.
Visiting hours: [Honggutan Campus] Monday; 【East Lake Campus】Wednesday
Cai Jun: Deputy Chief Physician, has rich experience in the diagnosis and treatment of common pediatric orthopedic diseases: trauma, deformity, tumor, infection, etc., and has rich clinical experience in hip dislocation, avascular necrosis of the femoral head in children, spinal deformity, foot deformity, knee joint sports medicine diseases.
Visiting hours: [Donghu Campus] Monday; 【Honggutan Campus】Tuesday
Wu Min: Deputy Chief Physician, good at the diagnosis and treatment of children's limb fractures, post-fracture deformities and orthopedics, various congenital and developmental bone diseases, etc.; He has rich clinical experience in the minimally invasive treatment of fracture trauma in children, developmental hip dislocation, avascular necrosis of the femoral head in children and other diseases.
Visiting hours: Friday at Yangming Road Campus, Saturday and Sunday at Honggutan Campus.
Note: The visiting time is for reference only, please refer to the registration schedule on the hospital's WeChat platform for details.
Source: Jiangxi Provincial Children's Hospital
Correspondents: Jiang Qiqing, Xie Kantao, Luo Ying, Wei Meijuan
Editor: Huang Menglin
Reviewer: Chen Shirui
Issued: Daley Red
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