The anesthetic management of a patient with Cohen syndrome.

[cohen syndrome]

Cohen syndrome is a rare genetic disorder caused by autosomal recessive inheritance and is characterized by the following features : mental retardation , infantile hypotonia , micrognathia , narrow and high-arched palate , microcephaly , prominent upper central incisors , poor dentition , short stature , and truncal obesity . Some patients have strabismus , myopia , optic atrophy , and total blindness . A small number of cases present with heart defects or mitral valve prolapse . Only approximately 100 cases have been reported in the world literature . The administration of general anesthesia in patients with Cohen syndrome can be a challenge because most of these patients are mentally retarded and uncooperative and have facial malformations that may make intubation difficult . We present our experience with the anesthetic management of a patient with Cohen syndrome .

Diseases
Validation

Diseases presenting "small number" symptom

adrenal incidentaloma

allergic bronchopulmonary aspergillosis

aromatase deficiency

cadasil

carcinoma of the gallbladder

cohen syndrome

cushing syndrome

dedifferentiated liposarcoma

dentinogenesis imperfecta

dracunculiasis

erdheim-chester disease

focal myositis

gm1 gangliosidosis

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

junctional epidermolysis bullosa

kabuki syndrome

kindler syndrome

lamellar ichthyosis

legionellosis

liposarcoma

locked-in syndrome

malignant atrophic papulosis

oculocutaneous albinism

oligodontia

omenn syndrome

pleomorphic liposarcoma

pyruvate dehydrogenase deficiency

severe combined immunodeficiency

thoracic outlet syndrome

triple a syndrome

waldenström macroglobulinemia

well-differentiated liposarcoma

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

zellweger syndrome

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