Staged reconstruction for adult complete Treacher Collins syndrome.

The Journal of craniofacial surgery 2009 Vol.20(5) p. 1433-8

Zhang Z, Niu F, Tang X, Yu B, Liu J, Gui L

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Abstract

Treacher Collins syndrome is a congenital craniofacial malformation affecting the structures derived from the first and second brachial arches. For the complete form, the deformities can be severe; both the orbital-zygomatic region and mandible as well as the soft tissues should be reconstructed. To explore the surgical management of this kind of deformities, we proposed our protocol for the staged reconstruction: (1) upper-facial reconstruction with specially designed outer calvarial table, (2) mandibular lengthening by distraction osteogenesis technique and orthognathic surgery to correct the birdlike facial appearance and anterior open bite after distraction, and (3) lipofilling for the correction of residual depressive deformities. Two adult patients have undergone this protocol, and the results were satisfactory. Even though many authors advised using pedicled bone flap for the reconstruction of the orbit and zygomatic complex in young patient because free bone graft has the tendency of bone resorption and multiple onlay bone graft may need to be added in later stage, for adult patient, however, the reconstructed bone framework using outer cranial table can well maintain the bony facial contour of the orbital zygomatic region. The residual depressive deformities in the temporal and cheek regions can be treated with lipofilling. Compared with the technique of mandibular advancement osteotomy, bilateral mandibular lengthening by gradual distraction is relatively safe and can effectively elongate the mandible and the surrounding soft tissues. After the distraction, the open bite can be closed with subapical osteotomy, and the chin projection can be further added by advancement genioplasty.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
해부 mandible 하악골 dict 2
시술 genioplasty 턱끝성형술 dict 1
시술 flap 피판재건술 dict 1
시술 orthognathic surgery 안면윤곽술 dict 1
해부 soft tissues scispacy 1
해부 calvarial scispacy 1
해부 anterior scispacy 1
해부 lipofilling scispacy 1
해부 orbit scispacy 1
해부 bone scispacy 1
해부 cheek scispacy 1
해부 mandibular scispacy 1
해부 chin scispacy 1
합병증 lipofilling scispacy 1
질환 Treacher Collins syndrome C0242387
Mandibulofacial Dysostosis
scispacy 1
질환 congenital craniofacial malformation scispacy 1
질환 bite C0005658
bite injury
scispacy 1
질환 depressive deformities scispacy 1
기타 brachial arches scispacy 1
기타 orbital-zygomatic scispacy 1
기타 patients scispacy 1
기타 patient scispacy 1
기타 bone graft scispacy 1
기타 orbital zygomatic scispacy 1
기타 bilateral mandibular scispacy 1

MeSH Terms

Adipose Tissue; Adolescent; Bone Transplantation; Cheek; Chin; Facial Bones; Humans; Male; Mandible; Mandibulofacial Dysostosis; Open Bite; Orbit; Orthognathic Surgical Procedures; Osteogenesis, Distraction; Osteotomy; Plastic Surgery Procedures; Temporal Bone; Tissue and Organ Harvesting; Zygoma

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