Carotid-Cavernous Fistula Associated with Ehlers-Danlos Syndrome Type IV

A case of traumatic, direct, carotid cavernous fistula (CCF) associated with Ehlers — Danlos syndrome (EDS) Type IV is reported along with a review of the literature. Excluding the present case, three similar cases associated with EDS-TypeIV have already been reported by Gerard M. Debrun et Al1. Despite the risks associated with endovascular manipulation, the fistula was successfully closed by intravascular embolisation but the patient expired a few days later because of underlying disease-associated vascular and visceral complications.

[1]  N. Miller,et al.  Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type IV. , 1996, Surgical neurology.

[2]  W. Schievink,et al.  Neurovascular Manifestations of Heritable Connective Tissue Disorders: A Review , 1994, Stroke.

[3]  H. Ito,et al.  Balloon occlusion of a spontaneous carotid-cavernous fistula in Ehlers-Danlos syndrome type IV. , 1993, Surgical neurology.

[4]  F. Earnest,et al.  Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome Type IV. Case report. , 1991, Journal of neurosurgery.

[5]  P. Charvet,et al.  [Ehlers-Danlos syndrome and pregnancy. Apropos of a case]. , 1991, Journal de gynecologie, obstetrique et biologie de la reproduction.

[6]  R. Higashida,et al.  Treatment of carotid-cavernous fistulas associated with Ehlers-Danlos syndrome. , 1990, Neurosurgery.

[7]  S. Cassidy,et al.  Internal carotid artery aneurysm: a vascular manifestation of type IV Ehlers-Danlos syndrome. , 1989, Connecticut medicine.

[8]  D. Compston,et al.  Spontaneous carotid cavernous fistula in Ehlers Danlos syndrome. , 1988, Journal of neurology, neurosurgery, and psychiatry.

[9]  M. Kupersmith,et al.  Management of nontraumatic vascular shunts involving the cavernous sinus. , 1988, Ophthalmology.

[10]  B. Lach,et al.  Spontaneous carotid-cavernous fistula and multiple arterial dissections in type IV Ehlers-Danlos syndrome. Case report. , 1987, Journal of neurosurgery.

[11]  J. Miles,et al.  Spontaneous arterial perforation: the Ehlers-Danlos specter. , 1987, Journal of vascular surgery.

[12]  A. Peaceman,et al.  Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death. , 1987, Obstetrics and gynecology.

[13]  R. Pyeritz,et al.  Ehlers-Danlos syndrome IV due to a novel defect in type III procollagen. , 1984, American journal of medical genetics.

[14]  F. Pope,et al.  Molecular Abnormalities of Collagen: A Review , 1983, Journal of the Royal Society of Medicine.

[15]  J. Heckenlively,et al.  Spontaneous carotid-cavernous fistula and the Ehlers-Danlos syndromes. , 1983, Ophthalmology.

[16]  I. Eriksson,et al.  Vascular complications in the Ehlers-Danlos syndrome. , 1983, Acta chirurgica Scandinavica.

[17]  T. Krieg,et al.  Molecular Defects of Collagen Metabolism in the Ehlers‐Danlos Syndrome , 1981, International journal of dermatology.

[18]  D. Taylor,et al.  Ehlers-Danlos syndrome during pregnancy: a case report and review of the literature. , 1981, Obstetrical & gynecological survey.

[19]  F. Pope,et al.  EDS IV (Acrogeria): New Autosomal Dominant and Recessive Types 1 , 1980, Journal of the Royal Society of Medicine.

[20]  Charles B. Wilson,et al.  Current surgical management of neurologic disease , 1980 .

[21]  D. Hollister Heritable disorders of connective tissue: Ehlers-Danlos syndrome. , 1978, Pediatric clinics of North America.

[22]  Barabash Ap Vascular complications in the Ehlers-Danlos syndrome, with special reference to the "arterial type" or Sack's syndrome. , 1972 .

[23]  Victor A. McKusick,et al.  Heritable disorders of connective tissue , 1972 .

[24]  I. Julien,et al.  [Spontaneous carotido-cavernous fistula and Ehlers-Danlos diseases]. , 1971, La Presse medicale.

[25]  D. Boucaud,et al.  Fistule carotido-caverneuse spontanée et maladie d'Ehlers-Danlos. , 1971 .

[26]  Madsen Ph Carotid-cavernous fistulae. A study of 18 cases. , 1970 .

[27]  P. Beighton Obstetric aspects of the Ehlers-Danlos syndrome. , 1969, The Journal of obstetrics and gynaecology of the British Commonwealth.

[28]  J. Kepes,et al.  Bilateral spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome. Case report. , 1967, Journal of neurosurgery.

[29]  C. J. Graf Spontaneous carotid-cavernous fistula. Ehlers-Danlos syndrome and related conditions. , 1965, Archives of neurology.

[30]  M. K. Rubinstein,et al.  Ehlers‐Danlos syndrome associated with multiple intracranial aneurysms , 1964, Neurology.

[31]  V. McKusick Heritable disorders of connective tissue. VIII. Concluding comments. Introduction; fibrodysplasia ossificans progressiva; osteopoikilosis; Leri's pleonosteosis; Paget's disease of bone; other possible hereditary and generalized disorders of connective tissue; the future in the study of heritable dis , 1956, Journal of chronic diseases.

[32]  V. McKusick Heritable disorders of connective tissue. IV. The Ehlers-Danlos syndrome. , 1956, Journal of chronic diseases.