[Extensive digital necrosis during dermatomyositis associated with MDA-5 antibodies]. / Nécroses digitales étendues au cours d'une dermatomyosite avec anticorps anti-MDA-5.

BACKGROUND: Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis. PATIENTS AND METHODS: A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea. Examination demonstrated skin lesions with edema on every digit associated with purpuric and cyanotic lesions, as well as erythematous papules on the helix and the elbows, and Gottron's papules. Systemic corticosteroid therapy was initiated. The immunoprecipitation results indicated the presence of anti-MDA-5 antibodies. Despite corticosteroid therapy, the patient's respiratory status gradually deteriorated towards pulmonary fibrosis and rapidly extensive necrosis appeared on all fingers and toes. Theses effects were resistant to cyclophosphamide and immunoglobulin but were stabilized by cyclosporine. DISCUSSION: Anti-MDA-5 antibodies are specific to DM and constitute a risk factor for severe interstitial lung disease (70% of cases) with a higher risk of mortality (40%). The cutaneous presentation of this DM is specific with palmar papules and mucocutaneous ulceration. Rapidly extensive digital necrosis has not been previously reported. No treatment has demonstrated superiority. CONCLUSION: We report the first case of DM with anti-MDA-5 antibodies involving interstitial lung disease and massive digital necrosis. Because of the pulmonary risk, in the presence of clinical lesions containing anti-MDA-5 DM, screening for these antibodies should be carried out.

Piezosurgical lateral suboccipital craniectomy and opening of the internal auditory canal in the rat.

AIM: Rotating burs (RB), routinely used in skull base and cerebellopontine angle (CPA) surgery for craniotomy and opening of the internal auditory canal (IAC) carry some risks for neurovascular tissue due to their rotating power. This paper describes the use of piezosurgery (PS), which selectively cuts bone with preservation of soft tissue, in lateral suboccipital craniectomy and opening of the IAC in the rat. METHODS: A lateral suboccipital craniectomy and opening of the bony IAC were performed with the Mectron® piezosurgical device under microsurgical conditions in the anesthezised rat. The piezosurgical device was evaluated with respect to practicability, safety, preciseness of craniectomy and IAC opening, and preservation of adjacent neurovascular tissue. The operation procedure is described in detail. RESULTS: The present work shows that PS allows easy, safe and precise bone cutting with no injury to neurovascular tissue, such as dura, transverse or sigmoid sinus, brain, and cranial nerves. No complications were noted during the procedure. Due to the absence of rotating power near neurovascular structures the drilling process was easy and comfortable for the surgeon. CONCLUSION: PS proved to be a safe, precise and easy to handle tool to perform suboccipital craniectomy and opening of IAC in the rat. Since PS makes the drilling process safer and more comfortable compared to a rotating bur it may be used instead of rotating burs in all scientific applications in animal models where a safe removal of bone near delicate nervous or soft tissue structures is essential.

[Therapy of posterior orbital tumors]. / Therapie posterior gelegener Orbitatumoren.

Tumors of the posterior orbit require different therapeutic modalities, depending on the histological entity. In the orbit all structures are in close relationship and the endocranium is in the direct proximity. This requires profound knowledge of topographic anatomy and high therapeutic precision. The surgical approach to the posterior orbit via a ventral intraorbital approach is strongly restricted due to the ocular bulb which consumes most space in the anterior orbit. Therefore if the bulb and vision are to be retained extraorbital surgical corridors are predominantly preferred. These are classified into extracranial and intracranial approaches. In detail, the former are medial transethmoidal orbitotomy, caudal transmaxillar orbitotomy and lateral orbitotomy. Frontolateral and frontotemporal orbitotomy as well as frontal, bifrontal and subfrontal orbitotomy are intracranial approaches. Apart from surgical methods there are several forms of radiotherapy which can be applied to orbital tumors under certain indications. Radiotherapy may be performed with external fractionated photon radiation or as stereotactic radiation, with heavy ions or protons or as brachytherapy. In this article various therapeutic interventions to the posterior orbit and the indications and potential side-effects are described.

[Surgical therapy in Graves' orbitopathy]. / Operative Therapie bei endokriner Orbitopathie.

Surgical therapy of Graves' orbitopathy comprises orbital decompression as well as strabismus and lid surgery. The former is primarily carried out during active disease, the latter during inactive disease. Orbital decompression abates increased intraorbital pressure and is thus applicable against dysthyroid optic neuropathy and also reduces exophthalmos. The choice of a specific procedure depends mainly on the experience of the respective center. In this article, the pterional transcranial, transnasal transethmoidal, transconjunctival and swinging eyelid approaches are presented. Eye muscle recession relieves the abnormal tension of fibrotic muscles and thus corrects diplopia. Compared to normal strabismus surgery, the dose-response relationship is increased. Lid lengthening surgery is applied to counter upper or lower lid retraction. If several of these operations are necessary the order is chosen in such a way that downstream procedures cannot change specific results of upstream operations.

[Clinical relevance of leukocyte differential in patients with marked leukocytosis in the emergency room]. / Signification d'une hyperleucocytose marquée et de la formule sanguine dans les situations d'urgence.

PURPOSE: We analyzed the characteristics of the leukocyte differential and the clinical outcome in patients admitted in an emergency department with marked leukocytosis greater than 20×10(9)G/L. METHODS: We studied a case series of consecutive patients admitted in an emergency department. The medical records were retrospectively reviewed after patient discharge. Three groups were defined: patients with infectious disorders (group I), noninfectious disorders (group II), and trauma (group III). Admission in intensive care unit (ICU), consciousness impairment or death defined the subgroup S of high severity. RESULTS: Groups I, II and III comprised, respectively, 150, 95 and 86 patients. The group I presented with higher temperature and neutrophilia (22,2±4.9 vs 20.9±4.0 and 21.1±3.9×10(9)G/L; P<0.001), and more profound eosinopenia (0.058±0.094 versus 0.098±0.170 and 0.092±0.104×10(9)G/L; P<0.001) and lymphopenia (1.16±0.98 vs 1.53±1.04 and 1.73±1.10×10(9)G/L; P<0.001) than the two other groups. Both neutrophilia and lymphopenia were independent predictors of infection by multivariate analysis. Frequencies of admission in ICU were, respectively, 8.7%, 40% and 43% (P<0.001). Leukocyte and neutrophil counts were significantly higher and basophil count significantly lower in subgroup S. Overall, 13.6% of the patients died and were characterized by basopenia. CONCLUSION: Marked leukocytosis indicated severe illness. Lymphopenia, eosinopenia and temperature were significant predictors of infection. A more severe clinical course was correlated with higher neutrophilia and basopenia.

[Nuchal swelling]. / Nuchale Schwellung.

In the unusual case of a 68-year-old woman with one-sided painless lateral neck swelling, the ENT examination showed a firm nuchal mass (4 × 4 cm) on the right side with no other pathological findings. Angio-MRI confirmed a solid, sharply demarcated tumor with arterial hyperperfusion. Core needle aspiration biopsy was performed, revealing well-circumscribed tufts showing the typical "cannonball" aspect. After preoperative embolisation we performed extirpation of the mass. Histological examination showed an acquired tufted angioma. Clinical as well as radiological follow-up examination detected neither local relapse nor metastases.

Minimally invasive removal of an extradural intraradicular lumbar schwannoma.

OBJECTIVE: Surgical exposure of peripheral lumbar schwannomas often requires extensive muscle mobilization which results in significant postoperative pain. We describe an alternative minimally invasive surgical technique for the treatment of such lesions. METHODS: We report the case of a patient with an extradural intraradicular schwannoma of the L4 root that was treated with a minimally invasive transmuscular approach by using tubular retractors. RESULTS: Muscle trauma and intraoperative blood loss were negligible. The postoperative course was uneventfull. There was no additional morbidity associated with the approach. The patient was discharged 72 h after the procedure. CONCLUSIONS: In selected cases extradural intraradicular lumbar schwannomas can be treated adequately with a minimally invasive transmuscular approach.

The clinico-surgico-pathological spectrum of myxopapillary ependymomas--report of four unusal cases and review of the literature.

According to the WHO grading system, myxopapillary ependymomas are assigned to WHO Grade I. However, the clinico-pathological spectrum might be very heterogenous. Herein, we report 4 cases exhibiting lumbar tumor masses, 1 causing muscular atrophy over a 30-year period, 3 displaying clinical history of persisting lumbar pain for only several weeks. All tumors were crooked with dura and spinal roots resulting in incomplete resection in three cases. On histological examination, two tumors were almost acellular and showed polycyclic hyaline and fibrotic extracellular matrix leading to differential diagnoses of chordoma, meningioma, fibrolipoma and ependymoma. Finally, together with the immunohistochemical investigations, electron microscopy led to the diagnosis of myxopapillary ependymoma, WHO Grade I, with massive degenerative changes. The other 2 cases presented with the typical neuropathology of myxopapillary ependymomas but showed local recurrence within 1 and 13 years throughout the whole neuraxis, and in 1 case additional metastases of the 3rd ventricle. Although the morphological feature of these myxopapillary ependymomas was benign, the presented cases showed that the biological behavior of myxopapillary tumors might differ greatly and that these tumors present a serious operative and diagnostic challenge. Myxopapillary ependymomas occur most often in the lumbosacral region. Due to the anatomic complexity of the cauda equina, a complete resection can be technically challenging in this region. However, a gross total resection at the primary surgery is the most predictive factor for the outcome.

Endoscope-assisted retrosigmoid intradural suprameatal approach to the middle fossa: anatomical and surgical considerations.

INTRODUCTION: Lesions involving the posterior and middle fossa may be reached by several surgical approaches depending mostly on tumors' location and characteristics. The retrosigmoid intradural suprameatal approach has been used to remove tumors of the posterior fossa extending into Meckel's cave and the middle fossa. With large tumors, this approach may allow exposure of the oculomotor and optic nerves, the supraclinoid internal carotid and communicating posterior arteries. METHODS: Three formaldehyde-fixed specimens have been prepared on both sides using the conventional retrosigmoidal-suprameatal approach. The entire endoscopic preparations have been documented via a digital recording system, using a 5mm endoscope with a 25 degrees perspective. The authors describe the anatomical corridor and technique of the endoscope-assisted retrosigmoid intradural suprameatal approach to lesions that are located predominantly within the posterior fossa and supratentorial extension into Meckel's cave, sellar and parasellar region. CONCLUSIONS: The endoscope-assisted suprameatal approach provides exposure of the antero-medial middle fossa even in cases of skull base lesions that have not caused significant displacement of neurovascular structures.

Evaluation of surface and volume rendering in 3D-CT of facial fractures.

OBJECTIVES: Three-dimensional computed tomography (3D-CT) of facial fractures has been reported as beneficial using surface (SR) and volume rendering (VR). There are controversial statements concerning the preferable algorithm. The purpose of this study was to evaluate and compare SR and VR for clinical 3D-CT in facial fractures on an experimental basis. METHODS: Multislice CT was obtained in 22 patients with facial fractures using two data acquisition protocols. Five SR and VR post-processing protocols were applied. Five assessors independently evaluated the quality of visualization of the fracture gap and dislocated fragments as well as the overall image quality using a five-point rating scale. The potential benefit of the 3D-images for radiological diagnosis and presentation was evaluated. The influence of the data acquisition protocol was analysed. RESULTS: SR in general achieved better evaluation scores than VR at corresponding thresholds. Variation of evaluation scores for all criteria was found for SR and VR depending on the segmentation threshold. Apart from the overall image quality no significant influence of the data acquisition technique was found for the evaluated criteria. CONCLUSIONS: SR provided sufficient and time efficient means for 3D-visualization of facial fractures in this study. No diagnostic benefit of VR over SR was found.

Rosai-Dorfman disease involving the cranial base, paranasal sinuses and spinal cord.

Rosai-Dorfman disease is a lymphoproliferative disease that rarely involves the central nervous system. A 32-year-old patient with an extensive process involving the petroclival region, cavernous sinuses, suprasellar region, anterior cranial fossa, paranasal sinuses, nasal cavity and spinal cord is reported. Sinus histiocytosis with massive lymphadenopathy lesions may be dural-based and located in the skull base. As these lesions may clinically and radiologically mimic meningiomas as well as other disorders, Rosai-Dorfman disease should be included in the differential diagnosis of extensive lesions involving the intracranial and spinal compartments along with meningiomatosis and inflammatory diseases. The individual management of Rosai-Dorfman disease may be challenging due to diffuse involvement of neurovascular structures at the skull base.

[3D-Visualisation of the middle ear by computer-assisted post-processing of helical multi-slice CT data]. / 3D-Darstellung des Mittelohres mittels computergestützter Nachverarbeitung helikaler Mehrschicht-CT-Daten.

Post-processing of CT-data allows non-invasive 3D-Visualisation of the middle ear for diagnosis and surgical planning. In this study different post-processing techniques and the clinical application of a 3D-postprocessing algorithm in a large number of patients are presented. 20 normal patients, 6 dissected temporal bones and 213 patients with suspected middle ear pathology were examined using a low-dosage Multi-Slice CT protocol. Virtual endoscopic views of the middle ear and 3D-images of the ossicles were generated using a standardised algorithm. Evaluation of the image quality was performed. The virtual views of the dissected temporal bones were compared to real views. In 32 patients high-quality 3D-models of the individual anatomical structures were generated and displayed using different visualisation techniques. The standardised and evaluated method enabled visualisation of the normal middle ear anatomy. Assessment of different pathologies, especially malformation, trauma, implants and postoperative alterations, was facilitated. The high-quality 3D-models allowed precise imaging of the anatomical structures. 3D-Visualisation of the middle ear using CT-data is beneficial for radiological diagnosis and surgical planning in cases of complex middle ear pathology as a complementary examination technique.

[Multisegmental image fusion of the spine]. / Mehrsegmentale Bildfusion an der Wirbelsäule.

Fusion of medical images is a technique that permits the correlation of homologous anatomical structures in different imaging modalities on the basis of a spatial transformation of the data sets. CT and MRI of the spine provide complementary information of possible relevance for diagnostic and therapeutic decisions. Methods enabling a multisegmental CT-MRI fusion of the spine were developed. These solve the problem of altered spatial relationships of the individual anatomical structures due to differing patient positioning in successive data acquisitions. Routine clinical CT and MRI data of a thoracic section of the spine were obtained and transferred to a PC-workstation. Following segmentation of the CT-data, landmarks for each individual vertebra were defined in the CT and MRI data. For each individual vertebra the algorithm we developed then carried out a rigid registration of the CT information to the MR data. The fused data sets were presented as colour-coded images or on the basis of dynamic variation of transparency. To assess registration precision, fiducial registration errors (FRE) and target registration errors (TRE) were calculated. The algorithm permitted multi-segmental image fusion of the spine. The average time required for defining the landmarks was 22 seconds per landmark for CT, and 34 seconds per landmark for MR. The average FRE was 1.53 mm. The TRE for the vertebrae was less than 2 mm. The colour-coded images were particularly suitable for assessing the contours of the anatomical structures, whereas dynamic variation of the transparency of overlapping CT images enabled a better overall assessment of the spatial relationship of the anatomical structures. The algorithm permits precise multi-segmental fusion of CT and MR of the spine, which was not possible using current fusion-algorithms due to variations in the spatial orientation of the anatomical structures caused by different positioning of the axial skeleton in successive examinations.

[Development of a universal interface for the exchange of medical image data using magneto-optical media]. / Entwicklung einer universellen Schnittstellenumgebung zur Ubertragung medizinischer Bilddaten mit magnetooptischen Medien.

Digital images generated by medical imaging form the basis for radiological diagnosis and surgical planning. Despite the advent of the DICOM 3.0 standard for medical image communication, widespread application of the existing information is often limited by incompatibility of the data formatting used by different equipment generations, and the manufacturer-specific standards employed. An exchange interface based on magneto optical discs has been developed to retrieve and present medical image data regardless of the technological hardware and the specific formats used. Specially adapted routines to retrieve the data first had to be developed. A modular program structure was used to allow flexibility in the implementation of further routines and other exchange media. Over 20,000 CT and MRI images including header information obtained from different General Electric and Siemens scanners were extracted successfully from MO discs. The image data were used for follow up and surgical planning and were transferred to a PAC-server. The interface proved reliable and easy to use. Support for further proprietary formats is currently being developed. The present exchange interface permits reliable retrieval of digital images for diagnostic and surgical planning purposes, regardless of the hardware generation and manufacturer-specific formats.

[Three-dimensional-segmentation of the spine with specially adapted tools]. / 3-D-Segmentierung der Wirbelsäule mit speziell angepassten Werkzeugen.

Segmented 3-D data of the spine form the basis for various modern clinical applications. Among these, multisegmental image fusion, image registration and finite element modeling for biomechanical analysis are promising innovative tools capable of facilitating treatment decisions and optimization of individual therapy in the future. However, the complex anatomy of the spine and the often extensive degenerative deformation presenting in clinical practice, generally limit the application of fully automated segmentation. A newly developed software system is presented that meets the complex requirements for image segmentation of the spine through the use of specially adapted interactive tools that take account of its axial skeletal structure. Furthermore, a standardized protocol is introduced that combines the newly developed interactive tools (rotation transformation, warped dissection plane) and standard segmentation tools to permit rapid and accurate segmentation. To date, the software environment presented herein has been applied with success to the segmentation of cervical, thoracic and lumbar spine.