Biomaterial Database

Giant intracranial aneurysm associated with Marfan's syndrome. Case report. 1976

L H Finney, and T S Roberts, and R E Anderson

The authors describe a giant intracranial internal carotid aneurysm in a patient with Marfan's syndrome. Treatment consisted of internal carotid ligation with subsequent thrombosis of the lesion. The patient's course was complicated by carotid arterial dissection and possible laminar thrombosis within the aneurysm increasing the size of the lesion. Three clinical syndromes, Marfan's, Ehlers-Danlos, and pseudoxanthoma esasticum, are discussed. The literature is reviewed with reference to the hazards of treatment of vascular pathology in patients with these connective tissue disorders.

UI	MeSH Term	Description	Entries
D008382	Marfan Syndrome	An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2.	Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D011561	Pseudoxanthoma Elasticum	An inherited disorder of connective tissue with extensive degeneration and calcification of ELASTIC TISSUE primarily in the skin, eye, and vasculature. At least two forms exist, autosomal recessive and autosomal dominant. This disorder is caused by mutations of one of the ATP-BINDING CASSETTE TRANSPORTERS. Patients are predisposed to MYOCARDIAL INFARCTION and GASTROINTESTINAL HEMORRHAGE.	Gronblad-Strandberg Syndrome,Groenblad-Strandberg Syndrome,Pseudoxanthoma Elasticum, Autosomal Dominant,Pseudoxanthoma Elasticum, Forme Fruste,Pseudoxanthoma Elasticum, Incomplete,Elasticum, Incomplete Pseudoxanthoma,Elasticums, Incomplete Pseudoxanthoma,Gronblad Strandberg Syndrome,Incomplete Pseudoxanthoma Elasticum,Incomplete Pseudoxanthoma Elasticums,Pseudoxanthoma Elasticums, Incomplete,Syndrome, Gronblad-Strandberg
D002343	Carotid Artery, Internal	Branch of the common carotid artery which supplies the anterior part of the brain, the eye and its appendages, the forehead and nose.	Arteries, Internal Carotid,Artery, Internal Carotid,Carotid Arteries, Internal,Internal Carotid Arteries,Internal Carotid Artery
D002532	Intracranial Aneurysm	Abnormal outpouching in the wall of intracranial blood vessels. Most common are the saccular (berry) aneurysms located at branch points in CIRCLE OF WILLIS at the base of the brain. Vessel rupture results in SUBARACHNOID HEMORRHAGE or INTRACRANIAL HEMORRHAGES. Giant aneurysms (>2.5 cm in diameter) may compress adjacent structures, including the OCULOMOTOR NERVE. (From Adams et al., Principles of Neurology, 6th ed, p841)	Aneurysm, Cerebral,Aneurysm, Intracranial,Basilar Artery Aneurysm,Berry Aneurysm,Brain Aneurysm,Cerebral Aneurysm,Giant Intracranial Aneurysm,Mycotic Aneurysm, Intracranial,Aneurysm, Anterior Cerebral Artery,Aneurysm, Anterior Communicating Artery,Aneurysm, Basilar Artery,Aneurysm, Middle Cerebral Artery,Aneurysm, Posterior Cerebral Artery,Aneurysm, Posterior Communicating Artery,Anterior Cerebral Artery Aneurysm,Anterior Communicating Artery Aneurysm,Middle Cerebral Artery Aneurysm,Posterior Cerebral Artery Aneurysm,Posterior Communicating Artery Aneurysm,Aneurysm, Berry,Aneurysm, Brain,Aneurysm, Giant Intracranial,Aneurysm, Intracranial Mycotic,Aneurysms, Basilar Artery,Aneurysms, Berry,Aneurysms, Brain,Aneurysms, Cerebral,Aneurysms, Giant Intracranial,Aneurysms, Intracranial,Aneurysms, Intracranial Mycotic,Artery Aneurysm, Basilar,Artery Aneurysms, Basilar,Basilar Artery Aneurysms,Berry Aneurysms,Brain Aneurysms,Cerebral Aneurysms,Giant Intracranial Aneurysms,Intracranial Aneurysm, Giant,Intracranial Aneurysms,Intracranial Aneurysms, Giant,Intracranial Mycotic Aneurysm,Intracranial Mycotic Aneurysms,Mycotic Aneurysms, Intracranial
D004535	Ehlers-Danlos Syndrome	A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability.	Cutis Elastica,Ehlers Danlos Disease,Ehlers-Danlos Disease,Danlos Disease, Ehlers,Disease, Ehlers Danlos,Disease, Ehlers-Danlos,Ehlers Danlos Syndrome,Syndrome, Ehlers-Danlos
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013514	Surgical Procedures, Operative	Operations carried out for the correction of deformities and defects, repair of injuries, and diagnosis and cure of certain diseases. (Taber, 18th ed.).	Surgical Procedures,Ghost Surgery,Operative Procedures,Operative Surgical Procedure,Operative Surgical Procedures,Procedure, Operative Surgical,Procedures, Operative Surgical,Surgery, Ghost,Surgical Procedure, Operative,Operative Procedure,Procedure, Operative,Procedure, Surgical,Procedures, Operative,Procedures, Surgical,Surgical Procedure