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Akira Kurata, Hidehiro Oka, Taketomo Ohmomo, Hitoshi Ozawa, Sachio Suzuki, Kiyotaka Fujii, Shinichi Kan, Yoshio Miyasaka, and Harue Arai

digital subtraction angiography because of the good visualization and relative lack of invasiveness associated with this modality. References 1. Bergqvist D : Ehlers-Danlos type IV syndrome. A review from a vascular surgical point of view. Eur J Surg 162 : 163 – 170 , 1996 Bergqvist D: Ehlers-Danlos type IV syndrome. A review from a vascular surgical point of view. Eur J Surg 162: 163–170, 1996 2. Forlodou P , Kersaint-Gilly A , Pizzanelli J , et al : Ehlers-Danlos syndrome with a

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Andrew A. Kanner, Shimon Maimon, and Zvi H. Rappaport

, Spector RH, Braun IF, et al: Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 62: 248–256, 1985 2. Debrun GM , Aletich VA , Miller NR , et al : Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type IV. Surg Neurol 46 : 247 – 252 , 1996 Debrun GM, Aletich VA, Miller NR, et al: Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type IV. Surg Neurol 46: 247–252, 1996 3. Evans AJ , Jensen ME , Mathis MJ , et

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Wouter I. Schievink, David G. Piepgras, Franklin Earnest IV, and Hymie Gordon

previous cavernous sinus thrombosis. Ehlers-Danlos syndrome is a group of hereditary connective-tissue disorders first described in 1668 50 and is currently divided into nine types. 7 Ehlers-Danlos syndrome Type IV, the so-called “vascular type,” was first described as a distinct clinical entity in 1967. 3 The basic molecular defect of this condition is an abnormality of Type III collagen. 39, 41 This type of collagen is one of the three major fibrillar collagens and normally constitutes approximately 40% of blood vessel walls. 32 The gene for the alpha-1 chain of

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Boleslaw Lach, Sreelatha G. Nair, Neville A. Russell, and Brien G. Benoit

small fibers sized 140 to 250 Å, and marked increase in the number of large fibers sized 300 to 400 Å ( Fig. 5 ). Fig. 4. Electron micrograph showing collagen in the abdominal aorta of a control patient (upper) and of our patient with Type IV Ehlers-Danlos syndrome (EDSIV, lower ). Note the smaller size of the collagen fibers in the control section compared to the EDSIV section. × 80,000. Fig. 5. Block graph showing distribution of collagen fibers according to their size. Black blocks indicate fibers in the normal control subject, and white

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Arnold Schoolman and John J. Kepes

Ehlers-Danlos syndrome is a familial hereditary disorder characterized by fragility of the skin and other tissues, gastrointestinal diverticula, arterial aneurysms, and occular abnormalities. The clinical features of this syndrome were first pointed out by van Meek'ren, 18 who in 1682 described a 23-year-old man who could stretch the skin of his right pectoral area to his left ear. Two hundred years later, Ehlers 6 drew attention to loose-jointedness and subcutaneous hemorrhages, while Danlos 2 described subcutaneous tumors in these patients. Vascular

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Neurosurgical Forum To The Editor Katalin Hegedüs , M.D. University of Debrecen Debrecen, Hungary 499 500 I read with great interest the report by Lach, et al. , on Type III collagen deficiency in their case of spontaneous carotid-cavernous fistula and multiple arterial dissection associated with Type IV Ehlers-Danlos syndrome (Lach B, Nair SG, Russell NA, et al: Spontaneous carotid-cavernous fistula and multiple arterial dissections in Type IV Ehlers-Danlos syndrome. Case report. J Neurosurg 66: 462

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Thomas H. Milhorat, Paolo A. Bolognese, Misao Nishikawa, Nazli B. McDonnell, and Clair A. Francomano

, Wenstrup RJ : Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK) . Am J Med Genet 77 : 31 – 37 , 1998 3 Beighton PH : McKusick's Heritable Disorders of Connective Tissue ed 5 St. Louis , Mosby Co , 1999 . 189 – 253 4 Beighton PH , Horan FT : Dominant inheritance in familial generalised articular hypermobility . J Bone Joint Surg Br 52 : 145 – 147 , 1970 5 Braca J , Hornyak M , Murali R : Hemifacial spasm in a patient with Marfan syndrome and

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Kazuhisa Nagae, Ikuo Goto, Kazuo Ueda, and Yasuyuki Morotomi

intracranial aneurysms, 26 vertebral artery aneurysms, 3, 8 and spontaneous carotid cavernous fistula 15 associated with the Ehlers-Danlos syndrome. 25 Hardin 16 described carotid and abdominal aneurysms in two related patients with Marfan's syndrome. Moreover, many authors have reported intracranial aneurysms associated with polycystic kidneys, 4, 5, 9, 27 arteriovenous malformation, 23 and coarctation of the aorta. 1, 4, 31 These reports suggest that mesodermal abnormalities, and alterations in the elastica and adventitia in particular, may play some role in the

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H. Lee Finney, Theodore S. Roberts, and Robert E. Anderson

frequency of many undiagnosed cases of this disorder because of its variable penetrance. Ehlers-Danlos syndrome was described as early as 1682 by a Dutch surgeon. 9 His patient was a Spaniard who could pull his right pectoral skin to his left ear. In the history of medicine these are the “India rubber men” and much has been written about the extraordinary dilatability of the skin. The specific abnormality is not clear, but is most likely a defect in the normal “basket weave” pattern of collagen fibers. This is a rather rare disorder found almost exclusively in persons

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Wouter I. Schievink, David G. Piepgras, and Douglas A. Nichols

by spontaneous dissection. However, rupture of such extracranial aneurysms rarely if ever occurs. 36 Our patient has a previously undescribed constellation of findings including spontaneous carotid—jugular fistula, carotid dissection, multiple intracranial arachnoid cysts, hemifacial atrophy, mitral valve prolapse, easy bruisibility, and abnormal scarring, suggesting the presence of a generalized connective tissue disorder. Although this complex of findings appears unique there is some overlap with previously described syndromes. Ehlers—Danlos syndrome type IV