ANESTHETIC MANAGEMENT FOR PATIENT WITH TRACHER COLLINS SYNDROME SCHEDULED FOR ELECTIVE CESAREAN DELIVERY

Abstract

Tracher Collins Syndrome is an autosomal dominant genetic disorder, as a result from mutation in TCOF1 gene. Other names for Treacher Collins Syndrome include Franceschetti- Zwalen-Klein syndrome, mandibulofacial dysostosis (MFD), zygo-auro-mandibular dysplasia and Treacher Collins Franceschetti syndrome. These changes cause deformities of the facial bones and occurrence at antimongoloid slant of the eyes, micrognathia and deformity of the ears. Complications may include breathing problems, seeing problems, cleft palate and hearing lost. Diagnosis of TCS is made through clinical evaluation, radiographic examination and molecular genetic analysis. Treatment is tailored to the specific needs of each individual by a multidisci- plinary craniofacial management team. We report a case of TCS with second twin pregnancy achieved with IVF. The first pregnancy ended with c-section (and death of the newborn after 23 days of delivery due to dysmorphia – TCS on the fetus). The patient was hospitalized at our clinic for evaluation and ending of pregnancy with elective section cesarean. The patient was managed successfully under regional anesthesia which is generally preferred technique to general anesthesia, in order to avoid potential complications associated to difficult airway management. The patient was left home on the fifth postoperative day with her two healthy children.