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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 10.3171/jns.1985.62.2.0248 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 10

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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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Nandan Marathe, Laura-Nanna Lohkamp, and Michael G. Fehlings

S ince its initial description, the definition of Ehlers-Danlos syndrome (EDS) has notably changed. At present, it broadly refers to disorders of the connective tissue that are heritable and have similar features including joint hypermobility, dermal dysplasia, and vascular as well as internal organ fragility. 1 There has been no comprehensive review of spinal manifestations of EDS in recent literature. That has led to controversies in management protocols for this so-called orphan disease. The following narrative review sheds light on the causes

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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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Jake Jasinski, Doris Tong, Connor Hanson, and Teck Soo

Ehlers-Danlos syndrome (EDS) is an inherited connective tissue disorder due to collagen malformation. Patients diagnosed with EDS experience joint laxity that often leads to bony dislocations and musculoskeletal injuries. 1 As a result, patients diagnosed with EDS often develop spinal scoliotic deformities and high-grade spondylolisthesis. While the surgical management of appendicular skeletal manifestations of patients with EDS is well described, there is a lack of literature that describes the surgical management of high-grade spondylolisthesis in patients

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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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Petra M. Klinge, Abigail McElroy, John E. Donahue, Thomas Brinker, Ziya L. Gokaslan, and Michael D. Beland

–2 level. By applying that novel ultrasound technique, we confirmed previous MRI and myelography observations and found oscillatory motion, and in addition SC displacement, in response to head movement. 15 Hypermobile Ehlers-Danlos syndrome (EDS) is a genetic condition presenting with joint hypermobility. Although no genetic cause has been identified, given the clinical findings of joint hypermobility, mild skin hyperextensibility, and tissue fragility, impaired collagen modification has been suggested. 21 , 22 Patients with EDS frequently present with headache and

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

, Steinmann B , Tsipouras P , 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 10.1002/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O 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