Anaesthesiological considerations in patients with Sneddon's syndrome.

[sneddon syndrome]

Sneddon 's syndrome is a rare disease with strong gender prevalence of females . This syndrome is characterized by livedo racemosa and cerebrovascular lesions . Since no specific test is available , the clinical differentiation from other disorders with similar symptomatology may raise difficulties . The cerebral involvement includes strokes with cases of more than one ischaemic event having been reported . Associations with convulsions , heart valve disease , systemic hypertension , and renal impairment have been described . We report the case of a 5 -year -old boy who was anaesthesized for dental surgery . Due to the fact that 50 % of Sneddon 's syndrome patients develop mental retardation , even minor procedures require general anaesthesia . A review of the literature is added and specific anaesthesiological aspects of the perioperative care of Sneddon 's syndrome are discussed .

Diseases
Validation

Diseases presenting "mental retardation" symptom

achondroplasia

alexander disease

alpha-thalassemia

aniridia

aromatase deficiency

canavan disease

classical phenylketonuria

coats disease

cohen syndrome

cowden syndrome

cystinuria

dentin dysplasia

familial hypocalciuric hypercalcemia

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

kabuki syndrome

kallmann syndrome

lamellar ichthyosis

lymphangioleiomyomatosis

monosomy 21

phenylketonuria

primary hyperoxaluria type 1

proteus syndrome

pyruvate dehydrogenase deficiency

sneddon syndrome

triple a syndrome

wolf-hirschhorn syndrome

zellweger syndrome

This symptom has already been validated