Nevenka B. Laban, Velibor B. Tasic, Dragan Danilovski, Momir Polenakovic and Zoran S. Gucev
Wildervanck syndrome (WS) combines features of Klippel-Feil syndrome (KFS), sixth nerve palsy, and deafness. This is a case of a 23 year old woman, diagnosed with KFS (a triad of short neck, low posterior hairline and restricted neck movements) at the age of 20 days. The manifestations of the WS in this patient are severe: she has torticollis, and an extremely severe scoliosis. In addition, she is short (-3 SD; parental target height + 0.8SD) and has mixed sensorineural and conductive deafness. She also has ptosis, strabismus and a high myopia. Radiologically, there are multiple coalitions of cervical vertebrae. Intelligence is unaffected (IQ 95), but deafness, strabismus and high myopia forced her early out of school. Karyotype is 46, XX. In brief, this is a patient with severe WS and additional anomalies. Short and/or reduced parental target height is a part of WS.
surgically treated cases. Indian J Orthop 2010; 44:35-41.
8. Di Silvestre M, Bakaloudis G, Lolli F, et al. Posterior fusion only for thoracic adolescent idiopathic scoliosis of more than 80 degrees: pedicle screws versus hybrid instrumentation. Eur Spine J 2008; 7:1336-1349.
9. Hamzaoglu A, Ozturk C, Aydogan M, et al. Posterior only pedicle screw instrumentation with intraoperative halo-femoral traction in the surgical treatment of severescoliosis (>100 degrees). Spine 2008;33:979-83.
10. Potaczek T, Jasiewicz B, Tęsiorowski
Zsuzsanna Szöke, András Suciu, Géza Jeszenszky and Piroska György
Truncus arteriosus (TA) or common arterial trunk is a rare malformation, accounting for 0.21 to 0.34% of congenital heart diseases, which, if left untreated, leads to increased mortality rates. The condition is characterized by the presence of a unique arterial trunk that overrides the interventricular septum. Despite an overall poor outcome, few subjects present in emergency settings with signs suggestive for pulmonary arterial hypoplasia and associated heart failure. We report the case of a 31-year-old female patient who had been previously diagnosed with pulmonary atresia and severe scoliosis as an infant, presenting in the emergency department with clinical sings of decompensated heart failure which were demonstrated to be attributable to the severe cyanogenic heart malformation and were reversible after initiation of appropriate therapeutic measures.
Yan Hong, Pascal Bruniaux, Xianyi Zeng, Kaixuan Liu, Antonela Curteza and Yan Chen
This research presented a novel method using 3D simulation methods to design customized garments for physically disabled people with scoliosis (PDPS). The proposed method is based on the virtual human model created from 3D scanning, permitting to simulate the consumer’s morphological shape with atypical physical deformations. Next, customized 2D and 3D virtual garment prototyping tools will be used to create products through interactions. The proposed 3D garment design method is based on the concept of knowledge-based design, using the design knowledge and process already applied to normal body shapes successfully. The characters of the PDPS and the relationship between human body and garment are considered in the prototyping process. As a visualized collaborative design process, the communication between designer and consumer is ensured, permitting to adapt the finished product to disabled people afflicted with severe scoliosis.
Z Gucev, G Kalcev, N Laban, Z Bozinovski, N Popovski, A Saveski, B Daskalov, D Plaseska-Karanfilska and V Tasic
long bones, severe platyspondyly and severescoliosis, are pointing towards the nonlethal autosomal recessive form of MD [ 1 , 6 ]. Nevertheless, some researchers have found a striking evolution of the skeletal anomalies with amelioration of the size of the long bones and significant improvement of the platyspondyly resulting in almost normal vertebral bodies at 15 years of age, was also reported [ 1 ]. On the contrary, Dai et al [ 7 ] found that the evolution of body proportion with age was not essential, while only the prominent joints were consistently found in