anesthesia and airway management of a patient with sturge- weber syndrome associated with extreme ocular manifestations and giant facial hemangioma

نویسندگان

mahtab poor zamany nejat kermany shahid beheshti medical center

badiozaman radpay shahid beheshti medical center

چکیده

sturge-weber syndrome (sws) is a very rare congenital disorder that is manifested by facial capillary malformation (port wine stain) which may be associated with capillary – venous malformations affecting many parts such as the brain, eyes facial skin and mucosa and also airways. in this report we present an unusual 28 years old female case of sws that presented with signs and symptoms of sudden increased intra ocular pressure (exacerbation of glaucoma) and manifestations of facial and airway involvement. a discussion about anesthetic and airway management of such patient follow the case presentation.

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منابع مشابه

Ocular manifestations of Sturge–Weber syndrome: pathogenesis, diagnosis, and management

Sturge-Weber syndrome has been included in the group of phakomatoses that is characterized by hamartomas involving the brain, skin, and eyes. The characteristic facial port-wine stain, involving the first branch of the trigeminal nerve and the embryonic vasculature distribution in this area, leads to several ocular complications of the anterior segment and can involve the eyelids and conjunctiv...

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Sturge–Weber Syndrome with Choroidal Hemangioma

1. Weissman K, Wadskov S, Mikkelsen HI, Knudsen L, Christensen KC, Storgaard L. Acquired zinc deficiency dermatoses in man. Arch Dermatol 1978;114:1509‐11. 2. Joshi A, Ratnu KS, Joshi KC, Vyas PC. Trace elements in nephrotic syndrome and chronic renal failure. Indian J Nephrol 1993;3:48‐50. 3. Perrone L, Gialanella G, Giordano V, La Manna A, Moro R, Di Toro R. Impaired zinc metabolic status in ...

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Sturge-Weber-Syndrome with extreme ocular manifestation and rare association of upper airway angioma with anticipated difficult airway.

We report a rare case of an 18 year old girl with Sturge-Weber syndrome, she had extensive facial port wine stains, right bupthalmos and advanced glaucoma involving both eyes. She underwent right eye glaucoma drainage device surgery under general anaesthesia, and had a difficult intubation due to extensive angiomatous like soft tissue swelling at her upper airway. This report highlights the imp...

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periodontal manifestations and unusual radiographic features in a patient with sturge-weber syndrome: a case report

the sturge-weber syndrome or encephalotrigeminal angiomatosis is a rare neurological and congenital disorder with a frequency of 1 in 50,000 births. this syndrome is a nonhereditary developmental condition and is characterized by the presence of congenital capillary malformation and a hamartomatous vascular proliferation involving the face (port-wine stain or facial birthmark), sometimes skull ...

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[Management of childhood glaucoma associated with Sturge-Weber syndrome].

PURPOSE Sturge-Weber syndrome is a rare congenital disorder consisting of cutaneous, leptomeningeal and ocular vascular malformations. Glaucoma occurs in 30-70 % of patients. Treatment is challenging due to a high risk of severe complications such as expulsive haemorrhage, massive choroidal effusion and serous retinal detachment. Ocular findings and the occurrence of complications under differe...

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عنوان ژورنال:
archives of anesthesiology and critical care

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