Abstracts / Posters

A RARE CASE OF TREACHER COLLINS SYNDROME – CASE REPORT

Dr Varsha

Institute : Adichunchangiri institute of medical sciences. B.G.nagara. karnataka

ABSTRACT: Treacher Collins Syndrome,also known as mandibulofacial dysostosis is a rare autosomal dominant disorder characterized by craniofacial abnormalities and dysmorphic facies. We report a case of Treacher Collins Syndrome with pseudopterygium in right eye, posted for pterygium excision and amniotic membrane grafting who presented to us with micrognathia, high arched palate, repaired cleft lip and palate, mild MR, mild TR, managed with general anaesthesia

METHODS : A 7year old male child, 16kgs.pre anaesthetic evaluation was done. Mother was the informant Child had dysmorphic facial features. He had,low set ears, micrognathia, high arched palate, malaligned teeth with epulis in place of upper central incisors, previously operated scar for cleft lip and palate with short neck. Anticipating difficult airway, difficult airway cart kept ready and planed for general anaesthesia using inhalation induction sevoflurane.

RESULTS : A smooth induction and recovery with stable hemodynamics both intra and post operative period.

CONCLUSIONS : Treacher Collins Syndrome presents with the primary concern of airway management for the anaesthesiologist. As there is mandibular hypoplasia leading to micrognathism and retracted mandible, high arched palate, cleft lip and cleft palate, and microcephaly, there is always an expected risk of difficult intubation.Difficult airway cart with all different gadgets for securing airway should be kept ready.cricothyroid membrane puncture needle for emergency tracheostomy. All the emergency drugs should also be kept within reach to tide over untoward complications of hemodynamic instability, laryngeal or bronchospasm and aspiration.