Experience of surgical treatment on craniometaphyseal dysplasia
10.3760/cma.j.cnZHZXWKZZ-2018-0610-00269
- VernacularTitle:颅骨干骺端发育不良的手术治疗
- Author:
Yingnan GENG
1
;
Jie YUAN
;
Zheyuan YU
;
Liang XU
;
Huichuan DUAN
;
Min WEI
Author Information
1. 上海交通大学医学院附属第九人民医院整复外科 200011
- Keywords:
Skull deformity;
Bone resorption;
Craniometaphyseal dysplasia
- From:
Chinese Journal of Plastic Surgery
2020;36(6):655-659
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To retrospect the treatment and clinical effect of craniometaphyseal dysplasia(CMD), and summarize the experience of cranial reconstruction and nasal deformity correction in the management of this case.Methods:From June 2004 to June 2018, three male CMD patients of Shanghai Ninth People’s Hospital who received treatment. One patient was 1 year-old child who received drug therapy. A 5 year-old boy was treated with nasal cavity expansion and orbital hypertelorism for plastic and reconstructive surgery. The 7 year-old patient received cranial reconstruction and nasal deformity correction. Moreover, surgical treatments from 1967 to 2017 in the literatures on CMD were reviewed.Results:Indicators (Ca, ATP, PTH) in the laboratory of the first child returned to normal after medication treatment, and there was no obvious abnormality in the following 2 months. Nasal cavity of the second case was enlarged and function of the nose was improved, and interorbital distance was reduced by 16-17 mm. However, cranial facial deformation was not ameliorated obviously. For the third patient, scaphocephaly deformity was significantly improved. Skull thickness decreased from 3cm to 1-2 cm, the anteroposterior diameter of the skull was shortened up to 6 cm. The immediate review of dd dimer was 4.25 mg/L, FDP was 20.6 μg/ml, which was significantly higher than preoperative tests (dd dimer 0.98 mg/L, FDP 7.24 μg/ml). Two weeks after surgery, the patient received skull debridement due to ineffective anti-infective treatment. Ten months later, the child was admitted to the hospital because of infection. CT scan showed bone resorption, and we treated him with skull debridement and cranioplasty. Following 16 months, the patient was in a stable condition without complications until now.Conclusions:Drug therapy has a potential role in CMD treatment. However, surgery is the only effective management of it, although there will be a high risk and many complications, and the patients need repeated operations.
