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[A complex malformation of the first and second branchial arch and of the first bran-hial arch and of the first branchial cleft. Dysostosis temporo-spheno-facialis (author's transl)].

A complex malformation of the first and second branchial arches and of the first branchial cleft is described; there was also involvement of the axial skeleton and the limbs. The syndrome has been described by us as dysostosis temporo-sphenofacialis and could not be classified with any previously described entity. The reason for this was due to the imcomplete description of previously published cases, but also the large number of possible combinations of changes in various organs which can occur. In order to understand the condition more clearly, the aetiology and pathogenesis of abnormalities of the face and skull have been considered in detail. Adequate and accurate radiographic examination is considered as a pre-requisite to surgery, which can be cosmetic or improve hearing, and which may have good results.

Abnormalities, Multiple↗

[Changes in the cervical spine in juvenile rheumatoid arthritis (author's transl)].

Changes in the cervical spine in juvenile rheumatoid arthritis can be shown by A.P., lateral and functional views with maximal flexion and extension, and particularly by tomography. The latter is essential for proper evaluation of the atlanto-occipital and atlanto-axial joints and of the odontoid. Atlanto-axial subluxation was found in eight out of eighty-four patients. Destruction of the odontoid was found in twelve out of fifty-three patients examined by tomography. The earliest tomographic changes at the atlanto-occipital joint consists of isolated joint narrowing, erosions and fusion and were observed in sixty-four out of sixty-six patients examined. Similar findings at the atlanto-axial joint were observed in thirty-seven out of sixty-six cases. The severity of the changes correlated with seropositivity and duration of the disease. The most marked changes were found with a prolonged history and a positive Rose-Waaler reaction.

Arthritis, Juvenile↗

Enhancement of rat sciatic nerve regeneration by fibronectin and laminin through a silicone chamber.

In this study, the authors examined the effects of fibronectin and laminin on sciatic nerve regeneration in rats. Sixty-eight Sprague-Dawley rats underwent bilateral sciatic nerve transections and silicone tubulizations, with a 10-mm gap between the proximal and distal nerve stumps. Thirty rats (n=30) received 10 microg of fibronectin injection into the right sciatic nerve chamber, while saline was injected into the left nerve chamber, serving as the control. Another 30 rats (n=30) were given 6 microg of laminin injection into the right nerve chambers and saline into the left chambers. At 1, 3, and 4 months postoperatively, electrophysiologic and histologic examinations, including nerve morphometry, were performed. Eight additional rats, receiving fibronectin (n=4) and laminin (n=4) injections, were used for horseradish peroxidase (HRP) tracing at 3 months postoperatively. Results from the study showed that fibronectin- and laminin-treated groups had significantly higher motor nerve conduction velocity and evoked muscle action potential amplitude of the anterior tibial muscle than the control group (p<0.01). Nerve diameter and the number of myelinated axons from the groups receiving fibronectin and laminin applications were greater than the controls (p<0.01). Also, a greater number of HRP-labeled motor neurons were found in the ventral horns and dorsal root ganglia of the fibronectin- and laminin-treatment groups compared to the controls. The authors conclude that local applications of fibronectin and laminin into the nerve chambers can significantly improve axonal regeneration and maturation of injured rat sciatic nerves.

Action Potentials↗

[Indications and technic of transoral dens resection in craniocervical dysplasia].

Compressive lesions of the brain stem caused by ventrally or dorsally located bony malformations of the posterior skull base and the upper cervical spine show diffuse neurological signs and symptoms. They can imitate multiple sclerosis, cerebellar tumours, Ménière's disease and "Ménière-like" otological symptoms. Therefore, the otologist is involved in diagnosis and therapy of cranio-cervical dysplasia. At present dysplasia is differentiated into an anterior and posterior form of the disease. The most common lesion in the anterior form is brain stem compression by the high located dens axis. Therefore, transoral surgical removal of the dislocated odontoid process is the logical form of treatment and should be performed in patients with severe complaints. The technique used for the transoral resection of the odontoid process is described in detail. In 7 patients having the anterior type of the cranio-cervical dysplasia we performed a transoral resection of the elevated odontoid process. The results obtained were convincing with all patients. No intraoperative or postoperative complications were seen. Hearing impairment was not changed, neurological deficits and particularly vertigo improved. Follow-up examinations did not show any disturbances of head movements or remarkable dislocations of the kraniozervikal joints.

Adult↗

[Transaxial spongiosa-plasty and ventral, temporary atlanto-axial fixation for therapy of dens pseudarthrosis].

PURPOSE: According to literature there is a pseudarthrosis rate of about 1% up to 64% depending on the treatment of the dens fracture (16). Generally the treatment of dens pseudarthrosis consists of the fusion of the joint C1/C2 with or without dens resection. Now, a method is presented whereby, on the one hand the pseudarthrosis is treated, while on the other hand the anatomical structures and physiological function of the joint C1/ C2 are restored. METHOD: The operation consists of a gradual outboring of the base of the dens and the dens axis and filling with autologous cancellous bone. Then follows a lateral, temporary transarticular screw fixation of C1/C2 which guarantees an immobilisation of the filled out dens. A halo-body-jacket is then applied. The removal of the screws of the temporary fixation follows three months post operatively after X-ray control. Then physiotherapy of the cervical spine follows. RESULTS: During 7/93 and 7/97 this operation was carried out on 11 patients. In 9 cases compression screw osteosynthesis was primarily conducted and in 2 cases conservative therapy had preceded. The X-ray follow ups showed on an average of 14 month a stable bony fusion in 10 patients, the clinical follow up examinations on an average of 14 month a normal function. CONCLUSION: The operation presented is indicated in case of dens pseudarthrosis because this accomplished a definite bony fusion without disturbance of the function of the joint C1/C2 in patients not older than 60 years. Disadvantages are to be found in the intraoperatively high X-ray radiation and the 3 month immobilisation.

Adolescent↗

Coronally oriented vertical fracture of the axis body: surgical treatment of a rare condition.

The neurosurgical management in a rare case of vertical axis fracture is presented along with discussion of the supposed pathogenetic mechanisms and the biomechanics underlying this type of cervical spine injury. Comprehensive neuroradiological investigation prior to surgery clearly demonstrated the dislocation of the anterior part of the axis body with concomitant C 2/C 3-disk injury. Therefore, the unstable fracture had to be managed by a one-time combined ventrodorsal approach using anterior C 2-C 3 locking plate fusion and C 2 bilateral dorsal transpedicular screw fixation. No operative morbidity resulted from this procedure, and stable bony fusion was achieved with minimal restriction of head mobility and with minor residual complaints. This case illustrates the variable biomechanical response of the upper cervical spine to trauma and the advantages of a non-standard surgical approach for internal fixation of the injured cervical spine.

Adult↗

[Fractures of the axis organ in ankylosing spondylitis (author's transl)].

Fractures of the axis organ in ankylosing spondylitis display special features resulting from the peculiar underlying osteopathy. Depending on the localisation und extent of the vertebral ossification process, the mechanical load situations are different from case to case. Besides the disease-specific metaplastic-productive development, it is also possible that the peculiar destructive-absorptive component dominates in the overall pattern of the fracture, leading to false interpretations such as "non-typical fracture", "persistent pseudoarthrosis", specific spondylitis, stress fracture, suspected tumour, etc. Eight observations by the author demonstrate the risks, problems, differential diagnostic difficulties and the experience collected from observations of the course of the disease: four fractures of the cervical vertebral column (following mild traumas) were mainly flexion, luxation (compression) fractures at C 5/6 with partly extensive spondylodiscitic destruction, conservative treatment being sufficient in the absence of severe neurological symptoms (with one exception). A transvertebral fracture (after trauma) had occurred at the eleventh thoracic vertebral body with extremely protracted healing. Two stress fractures in which healing tendency was absent, were seen in a lumbar vertebral column sigment with spondylo-arthritically stiffened vertebral arch components, being probably a post-traumatic spondylolisthesis in segment L 5/S 1.

Adult↗

Early posterior acrylic fixation of traumatic odontoid fractures.

Nine cases of traumatic odontoid fractures had, as a primary treatment, a posterior surgical fixation (C1 + C2 + C3) by means of acrylate, without waiting for the unpredictable results of external systems of immobilisation. No immediate or late (follow-up: six months to seven yrs) neurological complications occurred. Solid union took place in all the cases but one. Two cases of wound infection were recorded. All the patients were actively mobilised on the second postoperative day without external immobilisation.

Acrylates↗

[Treatment of fresh fractures of the dens axis by compression screw osteosynthesis].

Today most fractures of the odontoid process are treated conservatively. In 32% a bony stabilization is not achieved. Therefore each third has to have a secondary operation, because the only life-threatening psuedoarthrosis is one in the odontoid process of the axis. For this reason the question is repeatedly raised, whether better results will be achieved by on early primary operation. The advantages of the compression-screw osteosynthesis are compared with the well-known operative procedures. The operative management is described. An illustrative case in regard to diagnostic operation and follow up study is described.

Axis, Cervical Vertebra↗

["Crowned" odontoid process and osteoarthrosis of the anterior atlantoaxial joint (author's transl)].

The so-called "peridentale aureole" ("crowned odontoid"), a horseshoe-like calcification around the odontoid process, can occasionally be shown on transbuccal views of the occipito-atlantoaxial region, but is commonly only seen on a.p. tomography in patients with osteoarthrosis of the anterior atlantoaxial joint. Tomographic examinations reveal that these irregular horseshoe-like calcifications around the odontoid peg represent mainly the osteophyte formation on the superior border of the anterior arch of the atlas. These calcifications are often surrounding a smaller calcification on the tip of the odontoid process. The peridentale aureole or crowned odontoid process is easily overlooked unless tomography is performed, and misinterpretations are possible if the radiologist is not familiar with this appearance of osteoarthrosis and some other joint diseases of the anterior atlantoaxial joint.

Aged↗

The course of atlanto-axial involvement and disc narrowing of the cervical spine in rheumatoid arthritis.

In 188 patients with rheumatoid arthritis 585 radiological examinations of the cervical spine were performed. Subluxation of C1-2 was found more often than expected; it was often the only radiological change in that segment, and its degree was not related to the duration of the disease. Lateral radiographs taken in full flexion were important for the diagnosis. Age over 40 years at the onset of RA, increased ESR at least on one occasion and seropositivity were related to more severe degrees of involvement. The presence of subcutaneous nodules, high titres of the Rose-Waaler reaction and the presence of antinuclear antibodies had no definite relation to the degree of involvement.

Adolescent↗

Missed cervical spine fracture-dislocations: the importance of clinical and radiographic assessment.

OBJECTIVE: To review the case of a patient who suffered a cervical spine fracture-dislocation missed at a hospital emergency department. CLINICAL FEATURES: A 77-year-old man involved in a motor vehicle accident was transported to a local emergency hospital where cervical spine x-ray films taken were reported as demonstrating no evidence of acute injury. The patient visited a chiropractic clinic 6 days later, where x-ray films were again obtained, finding that the patient sustained fractures of C5 and C6, as well as a bilateral facet dislocation at C5/C6. Computed tomography confirmed the fractures, and magnetic resonance imaging findings demonstrated cervical spinal cord compression and posterior spinal cord displacement. INTERVENTION AND OUTCOME: The patient was referred for preoperative medical evaluation. He underwent C5-6 closed reduction and anterior/posterior fusion surgery and was released without complication. Patient follow-up indicated full recovery with minimal neurologic symptoms. CONCLUSION: Cervical spine fracture-dislocations are often missed during standard radiographic examinations in emergency department settings. Chiropractors are encouraged to perform a comprehensive evaluation of patients presenting with cervical trauma even if they have had prior x-ray films reported as normal. Standard x-ray films taken at emergency department facilities are not entirely reliable for detecting or revealing cervical spine fracture-dislocations. This case stresses the importance of careful clinical assessment and imaging procedures on patients who have encountered cervical spine trauma.

Aged↗