[Primary meningioma of the optical nerve sheet in infancy as initial presentation of neurofibromatosis type 2]. / Primäres Meningeom des Nervus opticus im Säuglingsalter als Erstmanifestation einer Neurofibromatose Typ 2.

Intraorbital meningiomas are rare tumors, making up less than 4% of all intraorbital tumors. Intraorbital meningiomas of childhood are curiosities with only few documented cases. We present the case of an 8­month-old male infant, presenting with strabismus and nystagmus. Magnetic resonance imaging showed a long segment thickening of the optical nerve and an intraocular tumor. The tumor was suspicious for retinal dysplasia and enucleation of the eye was performed to exclude malignancy. Histological examination revealed a meningothelial meningioma (WHO grade I), extending along the optical nerve and into the eye accompanied by retinal dysplasia and epiretinal membranes. Meningiomas of childhood, retinal dysplasia, and epiretinal membranes are regularly associated with neurofibromatosis type 2. Subsequent genetic analysis led to the final diagnosis. This case documents a very unusual early beginning of a neurofibromatosis type 2.

Diagnostic value of diffusion-weighted imaging (DWI) for the assessment of the small bowel in patients with inflammatory bowel disease.

AIM: To evaluate diffusion-weighted imaging (DWI) compared to standard magnetic resonance imaging (sMRI) in the assessment of inflammatory lesions of the small bowel. MATERIALS AND METHODS: Two readers retrospectively analysed MRI images of the small bowel including DWI followed by capsule endoscopy (CE) and ileocolonoscopy (ICS) in 30 consecutive patients with a suspected or established diagnosis of inflammatory bowel disease. Small bowel CE and the combination of CE + ICS were used as the standards of reference. Inflammatory lesions of the small bowel detected at endoscopy were compared with the findings of (1) sMRI alone (MRI without DWI), (2) DWI alone, and (3) sMRI in combination with DWI (sMRI + DWI). The sensitivity, specificity, and accuracy were calculated for all three readouts. The results of the three readouts were compared with each other. RESULTS: Using CE + ICS as the standard of reference, the mean sensitivity and specificity for the detection of inflammatory lesions of the small bowel at sMRI were 55.2% and 99.5%, at DWI 60% and 99%, and at sMRI + DWI 70% and 99%. Interobserver agreement between the two readers was very good (k=0.87-0.95). Two lesions in different patients were only detected at DWI. CONCLUSION: DWI of the small bowel not only allowed for the detection of inflammatory lesions with high accuracy, but also enabled the identification of additional lesions that were not found using sMRI alone.

[Intraocular Tumors other than Retinoblastoma in Children]. / Intraokulare Raumforderungen im Kindesalter ohne Retinoblastom.

The diagnosis of an intraocular mass in children can be challenging as invasive procedures are not allowed, in particular if a retinoblastoma may be present. In eyes with a unilateral tumour mass and loss of function enucleation with subsequent histopathological processing might be the only option to establish a diagnosis and to exclude a malignant tumour. The present paper deals with intraocular tumours other than retinoblastoma in children, with a special focus on the correlation of modern imaging techniques and histopathological findings.

Association of aneurysms and variation of the A1 segment.

BACKGROUND AND PURPOSE: Previous studies have described a correlation between variants of the circle of Willis and pathological findings, such as cerebrovascular diseases. Moreover, anatomic variations of the anterior cerebral artery (ACA) seem to correspond to the prevalence of aneurysms in the anterior communicating artery (ACoA). The aim of this study was to assess the prevalence of aneurysms in patients with anatomical/morphological variations of the circle of Willis. METHODS: We retrospectively analyzed 223 patients who underwent cerebral angiography between January 2002 and December 2010 for aneurysm of the ACoA. Diagnostic imaging was reviewed and statistically evaluated to detect circle of Willis anomalies, aneurysm size, and rupture. 204 patients with an unrelated diagnosis served as the control group. RESULTS: Variations of the A1 segment occurred significantly more frequently in the aneurysm group than in the control group. Mean aneurysm size in patients with grades I and III hypoplasia or aplasia was 6.58 mm whereas in patients with grade II hypoplasia it was 7.76 mm. CONCLUSIONS: We found that variations in the A1 segment of the ACAs are correlated with a higher prevalence of ACoA aneurysms compared with patients with a symmetric circle of Willis.

Isolated intracranial Rosai-Dorfman disease mimicking meningioma.

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. It is typically characterized by painless cervical lymphadenopathy, fever and weight loss. Although extranodal involvement has been reported in diverse sites, intracranial presentation, particularly in the absence of nodal disease is uncommon. To the best of our knowledge, 48 patients with intracranial masses have been reported previously. A 31-year-old man was admitted to our clinic with a 4-month history of progressive headache. His medical history was unremarkable except for occasional fever. There were not any neurological deficit and weight loss. No lymphadenopathy (particularly bilateral cervical) and extranodal involvement in diverse sites were revealed by physical and radiological examinations. Routine hematological and biochemical studies were normal except for mild leukocytosis and elevated erythrocyte sedimentation rate. The patient underwent magnetic resonance imaging (MRI) testing that revealed an enhancing mass in the left temporal lobe. Preoperative diagnosis was meningioma. The patient underwent a left frontotemporal craniotomy with complete resection of the mass. Histopathology was compatible with RDD. Extranodal RDD is rarely found intracranially. Prognosis is benign especially in the absence of nodal disease. It is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis.

Dysembryoplastic neuroepithelial tumor in the left lateral ventricle.

Extracortical location of dysembryoplastic neuroepithelial tumor (DNT) is rare. We present a case of DNT localized in the left lateral ventricle of a 21-year-old female patient. Magnetic resonance imaging of the brain showed a round mass lesion in the floor of the anterior horn of the left lateral ventricle. The lesion was hypointense on T (1)-weighted images and slightly hyperintense on T (2)-weighted images without contrast enhancement. Total removal of the yellowish-white colored soft tumor was performed through a transcallosal approach. Histologically, the tumor was of simple type of DNT that was composed of small oligodendrogliocytes clustered tightly about perivascular spaces with neurons floating in mucoid pools showing an eosinophilic fibrillary matrix in the background. The recognition of DNT with an unusual location in lateral ventricle has therapeutic and prognostic significance since DNT is curable by surgical excision and radiation therapy is of no obvious benefit.

The effect of cranioplasty on cerebral hemodynamics: evaluation with transcranial Doppler sonography.

BACKGROUND: The purpose of cranioplasty is not only cosmetic repair but also neurological improvement. The effect of cranioplasty on the cerebral hemodynamics flow has not been investigated by ultrasonographic techniques. AIMS: To investigate changes of cerebral hemodynamics after cranioplasty in patients with cranial defect using transcranial Doppler sonography (TCDS). SETTING: The Departments of Neurosurgery and Radiology of a university hospital. DESIGN: A prospective clinical study. MATERIAL AND METHODS: We prospectively examined the cerebral hemodynamics with TCDS pre- and postoperatively in 18 patients with cranial defect who underwent cranioplasty. All postoperative studies were done between the 7th and 15th day after cranioplasty. The anterior cerebral artery was examined through the transtemporal and transorbital windows, the middle cerebral artery through the transtemporal window, and the posterior cerebral artery through the transforaminal window. Bilaterally, the peak systolic, end diastolic and mean blood flow velocities of these arteries were measured. STATISTICAL ANALYSIS: Wilcoxon matched-pairs signed-ranks test. RESULTS: Before cranioplasty all the velocities ipsilateral to the cranial defect were significantly low, while in the contralateral side they were near normal. Ipsilateral low cerebral blood flows increased and reached normal levels (P<0.05) after cranioplasty. During the follow-up, neurological improvement was observed. CONCLUSION: Cranioplasty is carried out not only for preserving normal appearances and physical barrier but also for neurological improvement. This should be explained by the normalization of cerebral hemodynamics.

Spinal epidural cavernous angioma: case report.

Epidural cavernous angiomas are apparently rare lesions. We present a case of paraplegia with acute onset secondary to spinal epidural cavernous angioma at levels C7 and T1. Magnetic resonance imaging gave the clinician the opportunity to diagnose the lesion preoperatively. In this article, we present and discuss this rare case with a review of the literature.

Hereditary sensory and autonomic neuropathy: review and a case report with dental implications.

Hereditary sensory and autonomic neuropathy (HSAN) is a rare syndrome which is seen in early childhood. Five different types are described. Absence of pain and self-mutilation are characteristic findings of this syndrome. Teeth in the oral cavity can cause damage to the oral tissues and tongue. When it is diagnosed, there should be co-operation between dentist and neurologist. Using an oral shield prevents the biting and, thus, traumatization of the tissues can be prevented. A case report which is diagnosed as HSAN type 4 is presented and information submitted about its treatment.

Evaluation of eustachian tube function with ventilation scintigraphy by using 133Xe gas.

The aim of this study was to evaluate Eustachian tube function by ventilation scintigraphy. In 13 patients with normal tube function and 16 with one-sided tube dysfunction, 50 MBq 133Xe was applied into the nasopharyngeal space through a tube inserted into the nasal cavity. Immediately after the insufflation, the patients were asked to perform three Valsalva manoeuvres. The visualization of the middle ear was possible in 10 of the 13 patients (77 per cent) with normal tube function. Decreased uptake of the middle-ear region in the side of dysfunction was demonstrated in 11 of the 16 patients (68.7 per cent) with one-sided tube dysfunction. Quantitative evaluation by region of interest technique revealed that by using percentage retention of activity and uptake rates, there were significant differences between the sides of dysfunction and sides of normal tube function (p < 0.05) and also between sides of dysfunction and both sides of controls (p < 0.05). There was no significant differences between normal sides of patients and both sides of controls (p > 0.05). As a conclusion, this method is non-invasive, easy- to perform and has a low radiation burden in the evaluation of Eustachian tube function. Although the number of patients is limited, the test seems to be applicable with a success rate of about 74 per cent.

Cervical spine injuries and management: experience with 235 patients in 10 years.

During the past 10-year period 235 patients with cervical injury were included in this study. In this paper we present our clinical experiences in patients with cervical spine injury treated surgically and conservatively and their outcome. Only few data exist on the treatment of cervical spine injuries. The principles of the management are still controversial. The 235 patients with cervical spine injury admitted to our department were assessed with Frankel's grading scale and treated surgically and conservatively according to the type and level of the injury; 172 patients were treated surgically, and 63 patients were managed conservatively. The neurological state of the patients and the treatment modality are summarized in Table 1 and Table 2. In the upper cervical injury, except type II odontoid fracture with a dislocation of more than 6 mm, conservative treatment modalities were performed. In the lower cervical injury, an anterior approach with discectomy and anterior fusion were performed if there was spinal cord compression anteriorly. Otherwise a posterior approach with decompression and a variety of posterior fusion techniques were used.

Haemangiopericytoma of the middle ear with benign histological features.

Haemangiopericytoma is a rare vascular tumour, particularly in the head and neck region. We described the first case of haemangiopericytoma arising from the middle ear in 1995. The present case is the second example of a primary middle-ear haemangiopericytoma with benign histological features. Clinical photography, computed tomography (CT) and magnetic resonance imaging (MRI) scans and histological photographs depict the findings.

Presentation and management of supratentorial and infratentorial arachnoid cysts. Review of 25 cases.

BACKGROUND: To discuss the presentation, diagnosis, management modalities and outcomes of the arachnoid cysts. EXPERIMENTAL DESIGN: Retrospective study with a mean follow-up period of 31 months (ranging between 9 months and 5 years). SETTING: Institutional practice (The Military Medical Faculty Hospital). Patients and the participants: 25 patients with arachnoid cysts treated surgically. INTERVENTION: Fenestration and drainage of the cyst into cisterns in 13 cases, cyst-peritoneal shunting in 8 cases and cyst excision in 4 cases. MEASURE: The arachnoid cysts were followed-up by Computed Tomography or/and Magnetic Resonance Imaging. RESULTS: The results are excellent in 21 cases, moderate in 2 cases and poor in 2 cases. CONCLUSIONS: Regardless of the procedure used for arachnoid cyst treatment, the outcomes are somewhat similar if the surgical indications and techniques are properly selected.

Foot drop following brain tumors: case reports.

Three cases with foot drop following brain tumors in patients suffering from parasagittal pathology are reported. Foot drop was the first complaint in the patients in our series. We generally notice foot drop as a sign of lumbar disc herniation or peroneal nerve lesions but rarely foot drop may also occur with brain lesions.

Cyst of the mesencephalon (neuroepithelial cyst?): review and case report.

A case of a mesencephalic region cyst is reported. Neuroepithelial cysts are rare benign lesions that may be asymptomatic or may appear as space-occupying mass lesion. The pathogenesis and principles of diagnosis and management of these rare lesions are reviewed.