ABSTRACT: INTRODUCTION Spondyloepiphyseal Dysplasia Congenita syndrome (SDCs) is a rare autosomal dominant disorder of vertebral and epiphyseal centers resulting in disproportionate dwarfism.

METHODS : CASE REPORT A 16yr old, 20kg, male with enlarged tonsils was posted for elective tonsillectomy. The preanaesthetic evaluation of revealed that patient had been diagnosed with SDCs at 10yrs of age during evaluation of limb deformity. Patient was disproportionately dwarf (75cm) with prominent forehead, depressed nasal bridge, wide set eyes, hypo-plastic maxilla, prominent mandible, relatively large tongue with 3cm mouth opening, MMS 3, enlarged tonsils, short neck and restricted neck movements. He had pigeon chest and thoracic kyphoscoliosis. Patient’s other cardiorespiratory and neurological parameters were normal. Routine investigations were normal. Roentogenogram of cervical spine and limbs showed features of SDCs. With standard monitors and difficult airway cart, general anaesthesia was administered to this unpremedicated patient with fentanyl, propofol, vecuronium. With minimal neck extension, laryngoscopy performed with Macintosh 2 blade revealed a cranially placed larynx with Cormack Lehane grade 2 view and very narrow oropharyngeal space. Trachea was intubated with 6mm oral RAE tube during which subglottic narrowing was noticed. Anaesthesia was maintained with oxygen, N2O and isoflurane with supplemental vecuronium and fentanyl with IPPV. Patient was positioned with minimal neck extension during surgical period. Intraoperative period was uneventful. At end of surgery, neuromuscular blockade was adequately reversed and trachea was extubated when the patient was fully awake. Patient was monitored in PACU with supplemental oxygen with an uneventful postoperative period.

RESULTS : not applicable

CONCLUSION : Anaesthetic consideration of SDCs will be discussed in detail.