ABSTRACT
Objective@#To investigate the clinicopathological characteristics, imaging manifestations, genetic manifestations, diagnosis and treatment of juvenile hyaline fibromatosis.@*Methods @# A case of juvenile hyaline fibromatosis was reported, and the patient's clinical manifestations, imaging examinations, histopathological examinations, genetic changes and treatment were summarized and analyzed.@*Results @#Juvenile hyaloid fibromatosis is more common in infants and children. This patient had typical clinical and pathological manifestations, including posterior occipital masses, skin and subcutaneous nodules, gum hyperplasia, joint contractures, and joint osteolytic lesions. The histopathological lesions were characterized by the proliferation of spindle cells in the tissue accompanied by a large amount of amorphous transparent matrix. Genetic testing was performed to confirm an ANTXR2 gene mutation, consistent with the known genetic changes of juvenile hyaline fibromatosis. The 6-month follow-up of the patient showed that there was no obvious recurrence after resection of the gum and facial mass. In addition to surgery, the treatment of this disease requires multidisciplinary symptomatic treatment combined with rehabilitation and supportive treatment to achieve a better prognostic effect.@*Conclusion@# Juvenile hyaline fibromatosis is a rare nonneoplastic autosomal recessive genetic disease. Mutations in the ANTXR2 gene lead to disorders of collagen synthesis and metabolism in the tissues and further cause subcutaneous nodules, gingival hyperplasia, joint contractures and bone dissolution.
ABSTRACT
Objective @#To explore the effect of negative pressure sealing drainage on the treatment of maxillofacial-neck-mediastinal infection in multiple spaces.@*Methods@# Vacuum sealing drainage (VSD) was applied in five patients with maxillofacial-neck-mediastinal infection caused by odontogenic infection accompanied by diabetes or renal failure and other systemic diseases. After extensive debridement, a negative pressure drainage sponge was placed in the pus cavity and then the wound was closed. Continuous negative pressure drainage was continued after the operation. At the same time, multidisciplinary consultation was applied to control basic diseases and, strengthen anti-inflammatory responses, and nutrition and other systemic treatments were applied.@*Results@#Four patients underwent continuous negative pressure drainage and successful removal of the negative pressure sponge after inflammatory symptoms subsided. One patient′s inflammatory symptoms became more serious after the operation, and we performed another operation to change the placement of the negative pressure sponge. All 5 patients underwent VSD with negative pressure sponge replacement ranging from 1 to 3 times during treatment. After multidisciplinary consultation, they were all cured and discharged from the hospital.@*Conclusion@#For infection of the mediastinum, maxillofacial region and neck, local treatment and systemic treatment are emphasized, as well as the treatment of infected lesions and basic diseases. Negative pressure closure and drainage technology promotes the alleviation of inflammation, and multidisciplinary combined treatment is beneficial for the control of basic diseases.