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Mahmoud G. Nagib, Robert E. Maxwell and Shelley N. Chou

, Sprengel's deformity 1st admission: atlanto-occipital assimilation, atlantoaxial joint hypermobility; C3–4 fusion obstruction at the foramen magnum on myelography 2nd admission: myelographic defect at the cervicomedullary junction due to upward migration of odontoid process 1st op: craniectomy with C-1 laminectomy & fusion of occipital bone remains to C-2 & C-3 2nd op: transoral resection of odontoid process suboccipital & neck pain resolved following 2nd op; free of symptoms for over 1 yr 7 26, M acute onset of suboccipital headaches, lt facial, shoulder

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William E. Stehbens

observations of Pope, et al. , 66 and the extreme variability of joint hypermobility in children 92 support the possibility of the existence of formes frustes of hereditary connective tissue disorders and perhaps of as yet undetected metabolic disorders of connective tissue proteins. It is likely that the biochemistry of human connective tissues is as individualistic as human scent, fingerprints, and topography of the superficial veins of the upper limb, and the possible correlation of type III collagen deficiency with severe atherosclerosis warrants investigation. 72

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

Other features of Ehlers-Danlos syndrome Type IV include easy bruising, very thin and fragile skin with little or no hyperelasticity, papyraceous or keloid scar formation, joint-hypermobility usually limited to the digits, mitral valve prolapse, pneumothorax, and varicose veins. 33, 40, 41 A characteristic facies with prominent eyes, pinched nose, thin lips, and lobeless ears may be observed. 40, 41, 48 Literature Review A total of 16 patients with probable or definite Ehlers-Danlos syndrome Type IV and spontaneous carotid-cavernous fistulae were identified in

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

has been associated with the main vascular findings in our patient, that is, spontaneous carotid—jugular fistula 5 and carotid dissection. 33 In addition, mitral valve prolapse, abnormal scarring, and easy bruisibility are prominent features of this syndrome. 1, 33 Spinal (but not intracranial) arachnoid cysts have also been reported in this syndrome. 25 Our patient, however, did not have the characteristic thin and fragile skin or any joint hypermobility. Nevertheless, we thought it important to exclude a type III collagen disorder, the basic defect in Ehlers

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Wouter I. Schievink, Fredric B. Meyer, John L. D. Atkinson and Bahram Mokri

) thoracic epidural blood patch 2) transient benefit  diabetes mellitus  (Type I  (twice) 3) resolution of sympotoms  or II ‡ ) 3) surgical exploration 7 24, M headache, nausea, emesis, T-11 & T-12 meningeal ligation diverticula resolution of symptoms unusual body habitus  rt CNs V&VII pareses  diverticula  (ht 206 cm; wt 90 kg)  (6 wks)  (Type I ‡ )  & joint hypermobility 8 45, M headache, visual blurring cervicothoracic 1) oral steroids 1) transient benefit

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Iris Schrijver, Wouter I. Schievink, Maurice Godfrey, Fredric B. Meyer and Uta Francke

syndrome Case No. Age (yrs), Sex Location of CSF Leak Clinical Manifestations 1 24, M T-11 & T-12 tall stature (height 206 cm [>95th percentile],  meningeal  weight 90 kg), joint hypermobility  diverticula 2 22, F T-11 meningeal tall stature (height 176 cm [<95th percentile],  diverticulum  weight 50 kg), high arched palate,  mild joint hypermobility 3 40, F C-7 * tall stature (height 172 cm [90th percentile],  weight 65 kg), long & slender

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Wouter I. Schievink, M. Marcel Maya and Mary Riedinger

the initial and the recurrent leaks. In all of these three patients, a generalized connective tissue disorder was suspected (one patient had Ehlers—Danlos syndrome Type II and two patients had isolated joint hypermobility). In comparison, on the basis of physical examination, a generalized connective tissue disorder is suspected in approximately two thirds of patients with spontaneous spinal CSF leaks (unpublished data). Discussion Recurrent spontaneous spinal CSF leaks have only rarely been reported, and the risk of a recurrence—a major concern to both

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Andreas Jödicke, Andreas Hahn, Lars D. Berthold, Wolfram Scharbrodt and Dieter-Karsten Böker

: dolichocephaly, high and prominent forehead, downslanting palpebral fissures, strabismus, maxillary hypoplasia, high and narrow palate, micrognathia, and low-set ears. As in our case, skeletal abnormalities include arachnodactyly, campodactyly, flat foot, a deformity of the chest, scoliosis, and joint hypermobility. Hypotonia, developmental delay, minimal subcutaneous fat, and cryptorchidism are also characteristics indicative of SGS. 8 In general, vertebral anomalies are seen in fewer than one third of patients suffering from SGS. 8 Malformations of the CCJ have been

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Stéphanie Puget, Charles Kondageski, Alison Wray, Nathalie Boddaert, Thomas Roujeau, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose

the craniocervical junction showing tonsillar herniation. Lower: Posttreatment image (obtained 2 weeks after placement of the second blood patch) demonstrating improvement of the abnormality. On examination, the child was found to have the characteristic marfanoid appearance and arachnodactyly. She was taller and thinner than her parents; her height was 1.7 m and she weighed 30 kg. Her limbs were disproportionately long compared with her trunk. Scoliosis of 20° and joint hyper-mobility were noted. She appeared unwell, was obviously lethargic, and clinically

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

H ereditary disorders of connective tissue include a continuum of conditions caused by defects of extracellular matrix elements such as collagens, elastin, or fibrillin. 27 The EDSs constitute a distinct category within the HDCT group and are responsible for a wide variety of systemic manifestations including joint hypermobility, recurrent joint dislocations, skin laxity and fragility, poor wound healing, widened atrophic scars, tendon and muscle rupture, kyphoscoliosis, uterine rupture, large hernias, vascular fragility, easy bruising, venous varices