• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.1
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• pp.1-5
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• 2016

Robinow syndrome is skeletal dysplasia with both autosomal dominant and recessive inheritance patterns. It is characterized by short-limbed dwarfism, abnormalities in the head and face, as well as vertebral segmentation. A 2-year-7-month old boy with Robinow syndrome had visited Seoul National University Dental Hospital, for the evaluation of tooth palatal eruption on maxilla. He had micrognathia, delayed tooth eruption, cleft lip with bifid uvula. He also had an erupted mesiodens on the palatal side of maxillary primary incisors, which was tuberculated and 8mm in major diameter. The patient was scheduled for mesiodens extraction under general anesthesia. He was a young child with delayed development, so general anesthesia was inevitable. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. There were no postoperative complications related to anesthesia and dental treatment. Robinow syndrome patients have craniofacial dysmorphism and eruption disorders. Therefore, he requires regular check-ups as well as dental managements.