Anaesthesia Recommendations for Patients with Loeys-Dietz Syndrome
Journal Title: Progressing Aspects in Pediatrics and Neonatology - Year 2018, Vol 1, Issue 4
Abstract
Loeys-Dietz Syndrome is a otosomal dominant connective tissue disorder which has characteristic triad with a) Arterial tortuosity, aneurysms or dissections; b) Hypertelorism; and c) Bifid uvula or cleft palate. Although, arterial tortuosity is observed mostly in the head and neck vessels, it can occur other vessels as well. The clinical findings for musculoskelatal system involvement can be arachnodactyly, joint laxity, pectus deformities, scoliosis, dolichosternomelia, talipes equinovarus, comptodactyly, and cervical spine instability. Cardiovascular findings can be aortic diltation and disection, bicuspid aortic valve, and congenital heart disease such as atrial septal defect, patent ductus arteriosis. Central nervous system anomalies such as Chiari malformation and hydrocephalus can be observed. However, the life treatining complications that determine the prognosis are mostly cardiovascular system related findings like aortic root aneurysms, arterial tortuosity, aneurysyms of other vessels, patent ductus arteriosis, and atrial septal defect.Patients can have surgeries (emegency or elective) under anesthesia for cardiovascular (aortic aneurysm repairment, aortic valve replasman), orthopeadic (musculoskeletal system anomalie repairment), neurosurgical (for vascular, craniosynostosis, chari malformation and hydrocephalus), ophtalmic (for cataracts, strabismus, amblyopia, and exotropia), and general (for inguinal, umblical and hiatal hernia repairment, splenic or bowel rupture).
Authors and Affiliations
Aysun Postaci, İsmail Aytaç
Keywords
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- EP ID EP580436
- DOI 10.32474/PAPN.2018.01.000116
- Views 54
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