Spontaneous Carotid-Cavernous Fistula

Abstract

THE PROTEAN manifestations ofEhlers-Danlos syndrome, a rare disease of congenital nature involving body structures of mesodermal origin, are well known. Considered as a disturbance of the connective tissue, the abnormality concerns mainly the elastic and more particularly the collagen tissue throughout the body. This disorder is characterized by hyperelasticity of the skin, hyperextensibility of the joints, and histories of "easy bruising" with cutaneous hemorrhage and prolonged poorly controlled bleeding with surgical procedures. More dramatic manifestations of the disorder in other organ systems occur, eg, spontaneous rupture of the heart and lung, ectasia of the viscera, cardiac anomalies, diaphragmatic hernia, and gastrointestinal diverticula.Recently, Rubenstein and Cohen13 described as unique a patient with Ehlers-Danlos syndrome associated with multiple intracranial aneurysms and spontaneous subarachnoid hemorrhage.Aneurysm of the larger vessels (aorta) has been recorded by McKusick12 in this syndrome. There appears to be only one reference in the literature (other than that of Rubenstein and Cohen13) to a vascular malformation within the head associated with this condition.5 Although the authors indicate that a vascular malformation was present, surrounded by normal-appearing brain tissue, the pathology is poorly de¬ scribed and no definite conclusions can be drawn regarding the exact nature of the lesion. They imply that a disturbed elastic tissue structure was present in the surround¬ ing areas.Experience with two patients with this disorder, who developed spontaneous carot¬ id-cavernous fistula, prompted the report¬ ing of their histories as also unique. Report of CasesCase 1.·-A 24-year-old woman gave a history that while at a party on April 26, 1959 she was seized by left fronto-orbital-auricular pain and a constant rushing noise in the head. Two weeks later the left eyeball protruded and became red from a subconjunctival hemorrhage. At that time she en¬ tered another hospital. She was found to have a loud pulse-synchronous bruit over the left cheek and left fronto-temporal areas but heard best over the left orbit. Occlusion of the left cervical carotid ar¬ tery stopped the bruit but was followed by dizziness and syncope.Suspecting a left carotid-cavernous fistula left carotid angiography was unsuccessfully made. At that time trachéal compression developed from a hematoma at the site of artery puncture. She began to exude an alarming amount of blood from her mouth. When the endotracheal tube and mouth air¬ way could be removed, a bleeding path was present along the track of insertion of the airway. The right extremities (where she had been pinched to deter¬ mine motor function because of concern of left carotid compression by a clot) showed intracutaneous hemorrhages 5 to 6 cm in diameter. Upon fur¬ ther inspection other areas of bruising from manipulation were found about the body. After sev¬ eral days she was discharged from hospital.Although the eye hemorrhages subsided, proptosis and left fronto-...

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review

“…Death was reported for 20 (20.6%) patients [ 4 , 6 , 7 , 9 – 12 , 19 , 21 , 23 , 24 , 26 , 32 , 43 , 44 , 47 , 49 , 51 ], of which 14 (70.0%) died in the course of sCCF management [ 4 , 7 , 9 , 11 , 12 , 19 , 23 , 24 , 32 , 43 , 44 , 49 , 51 ]. COL3A1 variants were reported in 9 patients.…”

Obstetric Aspects of the Ehlers-Danlos Syndrome

“…A high incidence of miscarriage in a kindred with the syndrome was reported by Johnson and Falls (1949) and Bauer (1952) made a similar observation. Miscarriage has also been reported by Graf (1965) and a stillborn affected child with multiple congenital defects was mentioned by Tucker et al (I 963).…”

“…Certain gynaecological aspects of the syndrome are of importance. Menorrhagia in affected patients has been described by Papp and Paley (1966), Tucker et al (1963), Graf (1965), andTaylor (1967). The high incidence of uterine prolapse is an understandable feature of the syndrome, and not surprisingly, repair is not always successful.…”

Myocardial Infarction and Coronary Artery Dissection During Pregnancy Associated with Type IV Ehlers-Danlos Syndrome

“…One previous case of cardiac rupture of the anterior left ventricular wall leading to death in a non-pregnant female with EDS has been reported. 14 Some reports note that patients with EDS have a higher incidence of premature rupture of membranes, which may ultimately lead to preterm labor. 7 It has been suggested that patients with EDS may produce an amniotic membrane which consists of abnormal collagen allowing it to be more susceptible to premature rupture.…”