Universität zu Köln

Warnecke, T., Schmitz, J., Kerkhoff, S. and Hinkelbein, J. (2018). Anesthesia in patients with NBIA. Neurodegeneration with brain iron accumulation. Anaesthesist, 67 (11). S. 871 - 878. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1432-055X

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Abstract

BackgroundNeurodegeneration with brain iron accumulation (NBIA) forms agroup of rare hereditary diseases with rapid neurodegenerative progression due to an abnormal accumulation of iron in the basal ganglia. This causes extrapyramidal symptoms as well as dystonia and mental retardation. The most common form of NBIA is pantothenate kinase-associated neurodegeneration (PKAN, formerly Hallervorden-Spatz syndrome). There are multiple anesthesiological challenges with great implications for the clinical routine, particularly regarding the preparation for general anesthesia and the premedication visits. As with other orphan diseases, the available recommendations are mainly based on case reports.Objective and methodsThis article gives ashort overview of complications associated with NBIA pertaining to general anesthesia. This includes anesthesia-relevant clinical symptoms and perioperative management. The published literature and case reports (available on PubMed) were reviewed to extract aset of recommendations.ResultsSo far only afew reports have included the anesthesia management of NBIA patients. Most of them refer to PKAN as the predominant type (50% of cases). Recommendations were found on www.orphananesthesia.eu and consensus guidelines on PKAN in general. In particular, dystonia-related restrictions in the maxillofacial area can complicate airway management and cause difficulties with respect to intubation. Furthermore, local or regional anesthesia as the sole anesthesia technique is not eligible/viable due to the reduced compliance of the patient. Special attention should be paid to atimely premedication visit and evaluation to ensure sufficient time to safely plan and prepare the anesthetic procedure.ConclusionThe handling of NBIA patients requires good preparation, including an interdisciplinary team and customized time management. In principle, both general anesthesia as abalanced method and total intravenous anesthesia (TIVA) seem to be possible/viable options. The main focus is on airway management. Even after brief sedation in the context of diagnostic measures, the patient should be monitored for longer than usual.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Warnecke, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmitz, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kerkhoff, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hinkelbein, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-167860
DOI:	10.1007/s00101-018-0488-4
Journal or Publication Title:	Anaesthesist
Volume:	67
Number:	11
Page Range:	S. 871 - 878
Date:	2018
Publisher:	SPRINGER HEIDELBERG
Place of Publication:	HEIDELBERG
ISSN:	1432-055X
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language HALLERVORDEN-SPATZ-DISEASE; MANAGEMENT; DYSTONIA; CHILD; STIMULATION Multiple languages Anesthesiology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/16786