12
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Anesthetic approach to patient with Down syndrome and Hallervorden-Spatz disease who underwent tooth extraction

      letter

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Sir, Hallervorden-Spatz disease (HSD) is a rare neurological condition, caused by iron accumulation, which causes degeneration of the central nervous system. Blood and cerebrospinal fluid iron levels are normal. Accompanying Down syndrome (DS) is very rare.[1 2] We present our anesthetic approach with midazolam, metoclopramide, and ranitidine combination for tooth extractions of a mentally retarded, DS patient with HSD. We did not come across the use of this combination for HSD patients during the literature search. A 16-year-old male with DS, dystonia, dysarthria, gait disturbances, and sudden onset of psychotic symptoms with the diagnosis of HSD referred to Ankara University, Faculty of Dentistry, Oral and Maxillofacial Surgery Department (Turkey, 2015), for tooth extraction. General anesthesia was considered because of cooperation difficulty, agitation, and motion disorders. At preoperative examination, thyromental distance = 5 cm, sternomental distance = 10 cm, mandibulohyoid distance = 3 cm, Mallampati class was IV, atlanto-occipital joint extension was limited, and he had macroglossia. The patient was considered as a difficult intubation case. Oxygen saturation, heart beat rate, and noninvasive blood pressure were tracked. The patient with University of Michigan Sedation Scale (UMSS)-1 was oxygenized and was administered 2 mg of intravenous midazolam and metoclopramide intravenous 10 mg and ranitidine intravenous 50 mg to increase lower esophagus sphincter pressure and neutralize stomach acid. Anesthesia was maintained with 1 mg/kg of intravenous ketamine. Convenient mouth opening was achieved at UMSS 3. Extractions were carried out with no additional anesthetic agents or methods. After anesthesia termination, UMSS 1 and sufficient respiration was achieved in 4 min, general condition was good, and the patient was sent to service. In conclusion, treatment plans for HSD patients require general anesthesia when difficult intubation criteria and cooperation difficulty are present.[1 2] Sedoanalgesia under monitored anesthesia care, with titration of short-term anesthetics, and achieving UMSS 3 provides sufficient mouth opening for minor dental surgery procedures. Probable complications are prevented; mortality is decreased, fast recovery and early discharge is provided. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

          Related collections

          Most cited references2

          • Record: found
          • Abstract: found
          • Article: found
          Is Open Access

          Neurodegeneration with Brain Iron Accumulation: An Overview

          Objective Neurodegeneration with brain iron accumulation (NBIA) is a group of neurodegenerative disorder with deposition of iron in the brain (mainly Basal Ganglia) leading to a progressive Parkinsonism, spasticity, dystonia, retinal degeneration, optic atrophy often accompanied by psychiatric manifestations and cognitive decline. 8 of the 10 genetically defined NBIA types are inherited as autosomal recessive and the remaining two by autosomal dominant and X-linked dominant manner. Brain MRI findings are almost specific and show abnormal brain iron deposition in basal ganglia some other related anatomical locations. In some types of NBIA cerebellar atrophy is the major finding in MRI.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: found
            Is Open Access

            Hallervorden-Spatz disease

            Hallervorden-Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. Hallervorden and Spatz first described the disease, in 1922 as a form of familial brain degeneration characterized by iron deposition in the brain. Here we present four HSD cases with different clinical pictures.
              Bookmark

              Author and article information

              Journal
              J Res Med Sci
              J Res Med Sci
              JRMS
              Journal of Research in Medical Sciences : The Official Journal of Isfahan University of Medical Sciences
              Medknow Publications & Media Pvt Ltd (India )
              1735-1995
              1735-7136
              August 2015
              : 20
              : 8
              : 826
              Affiliations
              [1]Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Ankara University, Besevler, Ankara, Turkey
              Author notes
              Address for correspondence: Dr. Ayşe Hande Arpaci, Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Ankara University, Besevler, Ankara, Turkey. E-mail: handarpaci@ 123456yahoo.com
              Article
              JRMS-20-826
              10.4103/1735-1995.168412
              4652319
              33f8a93a-26be-425b-be47-32a5ca7af282
              Copyright: © 2015 Journal of Research in Medical Sciences

              This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

              History
              Categories
              Letter to Editor

              Medicine
              Medicine

              Comments

              Comment on this article