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Challenges in Evaluation, Management and Outcome of the Patients with Treacher Collins Syndrome

Bowornsilp Chowchuen MD, MBA*, Kamonwan Jenwitheesuk MD*, Prathana Chowchuen MD**, Palakorn Surakunprapha MD*

Affiliation : * Division of Plastic Surgery, Department of Surgery, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand ** Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand

Background : The challenges for management of Treacher Collins Syndrome (TCS) are the analysis of deformities and staged soft tissue and bony reconstruction.
Objective : To present clinical and radiologic findings, the evaluation methods and the concept of staged reconstruction for patients with TCS. Material and Method: The authors reviewed the clinical and radiologic findings, the evaluation methods, the staged reconstruction and early surgical outcome of three patients with TCS treated at Srinagarind Hospital, between 1994 and 2011.
Results : One patient underwent evaluation by CT scan for planning, reconstruction of the zygoma and orbit, correction of the lower eyelid coloboma, bilateral staged ear reconstruction with costal cartilage. At the age of 8 years, he had acceptable initial outcome with planned middle ear management and staged facial reconstruction. Palatoplasties of a cleft palate were performed in two patients,
Conclusion : TCS is a rare craniofacial deformity but poses challenges in evaluation, management and reconstructive surgery. The development of Craniofacial Center and interdisciplinary management is important for provision proper evaluation, initial management and longitudinal care, including appropriate timing for staged reconstruction of bone, ear and soft tissue reconstruction in patients with TCS. This treatment should be tailored and balanced with the functional and psychological needs of patient and his/her family. In developing countries, funding from other resources, including the not- for-profit foundations, is needed in order to increase patients’ accessibility, and improve the quality of the entire treatment program.

Keywords : Treacher Collins Syndrome, Challenges for evaluation, Interdisciplinary management and staged reconstruction, Developing country


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