Anesthetic Management for a Patient with Rosai-Dorfman Disease, Cowden Syndrome, and Lhermitte-Duclos Disease: An Extremely Rare Disease Combination
نویسندگان
چکیده
Rosai-Dorfman disease (RDD) is a rare condition that causes massive lymphadenopathy, most commonly in the cervical area. Cowden syndrome (CS) hamartomas skin and mucosa predisposes individuals to various malignancies. Lhermitte-Duclos (LDD), or dysplastic cerebellar gangliocytoma, often associated with CS. A 41-year-old female all three conditions presented abnormal uterine bleeding endometrial intraepithelial neoplasia (EIN). Precautions should be considered when evaluating patients RDD CS preoperatively during airway management owing potential for multisystem involvement, anatomical distortion, difficult airways. The likelihood of having extremely rare.
منابع مشابه
Cowden disease with Lhermitte-Duclos disease: case report.
BACKGROUND We report a case and review the recent literature describing 36 patients with both Lhermitte-Duclos disease (LDD) and Cowden disease (CD). Lhermitte-Duclos disease, or dysplastic gangliocytoma, is a benign hamartomatous condition involving the cerebellum. The presenting symptoms are usually headaches, gait ataxia, and symptoms of lower cranial nerve involvement. Cowden disease is a r...
متن کاملLhermitte-Duclos disease: an extremely rare cerebellar tumor
A 42-year-old female presented with complaints of mild occipital headaches of severe months duration. There were no cutaneous lesions or significant family history suggesting any genetic disease. On examination, she did not have any papilloedema, dymetria, dysdiadokokinasia or ataxia. Her cranial nerve examination was normal. On non-enhanced computed tomogram (CT) showed a mildly hyperdense wel...
متن کاملLhermitte – Duclos Disease in a Young Adult: Rare Entity
Lhermitte - Duclos disease also called dysplastic gangliocytoma of cerebellum is an extremely rare cerebellar lesion which share features of both malformation and neoplasm. The usual presentation is of raised intracranial pressure along with cerebellar signs. We report a case of 23 year male who presented with headache & diplopia. MRI was suggestive of the diagnosis. Subtotal excision of th...
متن کاملCowden syndrome and Lhermitte-Duclos disease in a family: a single genetic syndrome with pleiotropy?
Cowden syndrome is an autosomal dominant condition of multiple hamartomas. Patients with this phakomatosis have an increased risk of breast cancer and thyroid tumours. Lhermitte-Duclos disease is usually a sporadic condition of cerebellar ganglion cell hypertrophy, ataxia, mental retardation, and self-limited seizure disorder. We describe a three generation family with Cowden syndrome and Lherm...
متن کاملLhermitte - duclos Disease :
Lhermitte-Duclos disease (LDD) is a benign neoplasm of posterior fossa, involving cerebellum. It is also known as dysplastic cerebellar gangliocytoma. It is not a true neoplasm but a hamartoma. It can be either isolated finding or associated with Cowden (multiple hamartoma syndrome). adults.
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ژورنال
عنوان ژورنال: Cureus
سال: 2023
ISSN: ['2168-8184']
DOI: https://doi.org/10.7759/cureus.44318