ABSTRACT : Marfan syndrome is a multisystem connective tissue disorder, which as presented by Antoine Bernard Marfan in 1896. It is a condition with autosomal transmission, the defect commonly is in FBN1 gene. The skeletal and cardiovascular systems are primarily involved, the manifestations ranging from pectuscarinatum, kyphoscoliosis, dilatation of ascending aorta, mitral valve prolapse and spontaneous pneumothorax. The anaesthetic goals were carefully assess the patient preoperatively and to maintain stable intraoperative haemodynamic parameters to prevent acute cardiovascular or respiratory complications.

METHODS : A 25 year old male diagnosed to have a fracture mandible was referred for pre-anaesthetic evaluation. On examination, the arm span to height ratio was increased (1.05), he had a high arched palate, wrist and thumb signs were positive. Echocardiogram revealed minimal prolapse of the mitral valve. The anaesthetic goals were to prevent decrease in the preload and bradycardia and to maintain the systemic vascular resistance on the lower side. Premedication was done with Inj. glycopyrrolate 0.2 mg i.v.+ Inj.fentanyl 300mcg, induced with Inj. propofol 60mg + Inj. vecuronium 6 mg i.v., naso-tracheal intubation done with cuffed endotracheal tube size 7.5, maintained on O2, N2O, halothane & Inj. vecuronium, reversed with Inj neostigmine 2.5 mg+ Inj. glycopyrrolate 0.5 mg and extubated.

RESULTS : There were no intraoperative or postoperative complications

CONCLUSIONS : Marfan’s syndrome occurs in one in 4-6/10000. Recognition of these patients during pre-anaesthetic evaluation, optimising the cardiac status and skilful administration of anaesthesia are crucial to prevent deleterious cardiovascular complications.