Assessment of the anterior superior alveolar nerve and its impact on surgery of the lateral nasal wall.

BACKGROUND: The anterior superior alveolar nerve (ASAN) plays a major role in innervation of the lateral nasal wall. Its damage during nasal surgery can cause dental paraesthesia and numbness around the upper lip. METHODOLOGY: Retrospective evaluation of the computed tomographic (CT) scans of 50 consecutive patients analysing 100 sides. We measured the mean distance from the shoulder of the inferior turbinate to the descending portion of the anterior superior alveolar nerve, to the anterior superior alveolar canal and the anterior-posterior distance between the 'shoulder' of the inferior turbinate and the pyriform aperture. RESULTS: The mean distance from the shoulder of the inferior turbinate to the descending portion of the anterior superior alveolar nerve was 6.4 ± 2.33 mm, with no difference between sides The mean relative height of the shoulder in relation to the anterior superior alveolar nerve canal was 4.78 ± 2.31mm with no significant difference between the two sides. The anterior-posterior distance between the 'shoulder' of inferior turbinate and the pyriform aperture was 6.96± 2.28mm, with no significant difference between the two sides. CONCLUSIONS: We found the anterior superior alveolar nerve to be a constant landmark in the lateral nasal wall. Therefore, the course of the ASAN should be assessed on a CT scan when a surgical approach through the pyriform aperture or anterior medial wall of the maxillary sinus is planned.

Asymmetry in Gaze-Holding Impairment in Acute Unilateral Ischemic Cerebellar Lesions Critically Depends on the Involvement of the Caudal Vermis and the Dentate Nucleus.

Stabilizing the eyes in space when looking at a target is provided by a brainstem/cerebellar gaze-holding network, including the flocculus/paraflocculus complex (non-human primate studies) and the caudal vermis, biventer, and inferior semilunar lobule (human studies). Previous research suggests that acute lateralized cerebellar lesions preferentially lead to gaze-evoked nystagmus (GEN) on ipsilesional gaze. Here, we further characterize the effect of unilateral cerebellar lesions on gaze-holding and hypothesize that the side-specific magnitude of gaze-holding impairment depends on the lesion location. Nine patients (age range = 31-62 years) with acute/subacute (≤ 10 days old) MRI-confirmed unilateral cerebellar stroke were included. Horizontal gaze holding was quantified while looking at a slowly moving (0.5°/s) flashing target (gaze angle = ±40°). Asymmetry in eye-drift velocity was calculated and compared with the different MRI patterns of cerebellar lesions. Individual peak eye-drift velocities (range = 1.7-8.8°/s) occurred at the most eccentric eye positions (gaze angle = 28-38°). We found significantly asymmetric eye-drift velocity (EDV) in eight out of nine patients. The four patients with MRI-confirmed involvement of the caudal vermis and the dentate nucleus all presented with ipsilesionally-predominant EDV, while in the five patients with lesions restricted to the cerebellar hemisphere, EDV was stronger on contralesional gaze in three out of four found with an asymmetric EDV. Involvement of the caudal vermis and the dentate nucleus is critical for determining the directional GEN asymmetry in unilateral cerebellar lesions. Thus, our findings support the occurrence of GEN without floccular/parafloccular lesions and suggest that the EDV asymmetry in relation to the side of the lesion provides information about the involvement of specific structures.

Intermittent intracranial condylar dislocation with minimal functional sequelae.

Degenerative changes of the temporomandibular joint (TMJ) present with a broad spectrum of morphological alterations. However, erosions leading to a glenoid fossa defect and condylar impingement of the temporal lobe are a rare finding. A 77-year-old female patient presented with limited mouth opening and pre-auricular pain during mastication on the left side. She denied any neurological dysfunction. Her medical history included poliomyelitis, multiple cancers, and osteonecrosis of the left tibial plateau. Computed tomography revealed advanced degeneration of both TMJs. On the left side, a glenoid fossa fragment was elevated towards the left middle cranial fossa. Real-time dynamic magnetic resonance imaging (MRI) showed repetitive intracranial condylar dislocation during mouth closure. She declined surgery and received instructions for self-management. At the 12-month follow-up, she reported resolution of the pain and normal masticatory function. A control MRI showed a stable radiographic appearance. This report illustrates that intermittent dislocation of the mandibular condyle into the middle cranial fossa can be successfully managed conservatively. The self-limiting nature of the TMJ degenerative joint disease, patient preference, and the patient's general health status require consideration when advising patients on the therapeutic strategy.

Anterior maxillary wall and lacrimal duct relationship - CT analysis for prelacrimal access to the maxillary sinus.

BACKGROUND: The distance between the anterior wall of the maxillary sinus and the nasolacrimal duct shows a large individual variation. METHODOLOGY: To evaluate the feasibility of accessing the maxillary sinus through a prelacrimal window access (PLWA), a series of 100 paranasal CT scans from adult patients was analysed retrospectively. The distance between the anterior maxillary wall and the anterior border of the lacrimal duct (= prelacrimal window) were measured in 200 sides. RESULTS: A distance of more the 7 mm was found in 12.5% maxillary sinuses and would enable straight forward PLWA. A distance between smaller than 7mm and larger than 3mm was present in 56.5% of sinuses, and would make surgical access more demanding. In 31.5% of maxillary sinuses the distance was smaller than or equal to 3mm and in these patients this approach would be difficult without transecting the nasolacrimal duct. CONCLUSION: Only in 12.5% of sinuses a prelacrimal endoscopic access is readily feasible, while in 56.5% temporary tear sac dislocation is required and in 31.5% lacrimal sac dislocation is always needed along with a significant amount of bone removal to enable PLWA.

[Palsy of CVI caused by ecchordosis physaliphora]. / N.-VI-Parese durch Ecchordosis physaliphora.

We report a case of symptomatic ecchordosis physaliphora (EP) in a 34-year-old woman who presented with progressive diplopia due to palsy of the left sixth cranial nerve. Repeated magnetic resonance imaging (MRI) disclosed typical characteristics of a congenital EP lesion with compression of the left abducens nerve presumably because of a secondary herniation of the arachnoid mater. We performed an augmenting combined recess resect procedure on the left eye. No progression of the lesion was observed over a period of 5 years. For differential diagnostics an EP has to be distinguished from skull base tumors, such as chordoma and chondrosarcoma.

Anterior spreader flap technique: A new minimally invasive method for stabilising and widening the nasal valve.

BACKGROUND: The 'anterior spreader flap' is a new minimally invasive technique, for the treatment of nasal valve insufficiency or stenosis. The aim of this study was to present our experience with a series of patients with nasal valve dysfunction corrected by the minimally invasive spreader flap technique. METHODOLOGY: We performed a retrospective review of our patients with nasal valve malfunction who underwent the anterior spreader flap between June 2010 and June 2013. The patients had to judge their symptoms of nasal obstruction pre- and 12 months post-operatively by the Nasal Obstruction and Septoplasty Effectiveness (NOSE) quality-of-life assessment scale. The difference between the pre- and post-operative evaluations of each group and between the groups was calculated, and it was statistically analysed. RESULTS: Forty-three patients were treated. In seven patients, the anterior spreader flap was performed as a single procedure (group F), in 15 patients the anterior spreader flap was combined with a septoplasty and a turbinoplasty (group FST), in 10 patients with a turbinoplasty (group FT) and in 11 with a septoplasty (group FS). All patients reported significant post-operative improvement in nasal breathing (p < 0.05). The mean improvement (diffNOSE) of all patients was 54.2 points. The 'FS' group and the 'FST' group showed best post-operative results, but there were no significant differences between the groups (p > 0.8). CONCLUSIONS: The anterior spreader flap is an effective and safe method for minimally invasive improvement of nasal breathing in patients with nasal valve dysfunction.

Brain metastases in gastro-oesophageal adenocarcinoma: insights into the role of the human epidermal growth factor receptor 2 (HER2).

BACKGROUND: Gastro-oesophageal adenocarcinomas rarely metastasize to the central nervous system (CNS). The role of the human epidermal growth factor receptor 2 (HER2) in patients with these cancers and CNS involvement is presently unknown. PATIENTS AND METHODS: A multicentre registry was established to collect data from patients with gastro-oesophageal adenocarcinomas and CNS involvement both retrospectively and prospectively. Inclusion in the study required a predefined clinical data set, a central neuro-radiological or histopathological confirmation of metastatic CNS involvement and central assessment of HER2 by immunohistochemistry (IHC) and in situ hybridisation (ISH). In addition, expression of E-cadherin and DNA mismatch repair (MMR) proteins were assessed by IHC. RESULTS: One hundred patients fulfilled the inclusion criteria. The population's median age was 59 years (interquartile range: 54-68), of which 85 (85%) were male. Twenty-five patients were of Asian and 75 of Caucasian origin. HER2 status was positive in 36% (95% CI: 26.6-46.2) of cases. Median time from initial diagnosis to the development of brain metastases (BMets) or leptomeningeal carcinomatosis (LC) was 9.9 months (95% CI: 8.5-15.0). Median overall survival from diagnosis was 16.9 months (95% CI: 14.0-20.7) and was not related to the HER2 status. E-cadherin loss was observed in 9% of cases and loss of expression in at least one DNA MMR proteins in 6%. CONCLUSIONS: The proportion of a positive HER2 status in patients with gastro-oesophageal adenocarcinoma and CNS involvement was higher than expected. The impact of anti-HER2 therapies should be studied prospectively.

Gaze holding deficits discriminate early from late onset cerebellar degeneration.

The vestibulo-cerebellum calibrates the output of the inherently leaky brainstem neural velocity-to-position integrator to provide stable gaze holding. In healthy humans small-amplitude centrifugal nystagmus is present at extreme gaze-angles, with a non-linear relationship between eye-drift velocity and eye eccentricity. In cerebellar degeneration this calibration is impaired, resulting in pathological gaze-evoked nystagmus (GEN). For cerebellar dysfunction, increased eye drift may be present at any gaze angle (reflecting pure scaling of eye drift found in controls) or restricted to far-lateral gaze (reflecting changes in shape of the non-linear relationship) and resulting eyed-drift patterns could be related to specific disorders. We recorded horizontal eye positions in 21 patients with cerebellar neurodegeneration (gaze-angle = ±40°) and clinically confirmed GEN. Eye-drift velocity, linearity and symmetry of drift were determined. MR-images were assessed for cerebellar atrophy. In our patients, the relation between eye-drift velocity and gaze eccentricity was non-linear, yielding (compared to controls) significant GEN at gaze-eccentricities ≥20°. Pure scaling was most frequently observed (n = 10/18), followed by pure shape-changing (n = 4/18) and a mixed pattern (n = 4/18). Pure shape-changing patients were significantly (p = 0.001) younger at disease-onset compared to pure scaling patients. Atrophy centered around the superior/dorsal vermis, flocculus/paraflocculus and dentate nucleus and did not correlate with the specific drift behaviors observed. Eye drift in cerebellar degeneration varies in magnitude; however, it retains its non-linear properties. With different drift patterns being linked to age at disease-onset, we propose that the gaze-holding pattern (scaling vs. shape-changing) may discriminate early- from late-onset cerebellar degeneration. Whether this allows a distinction among specific cerebellar disorders remains to be determined.

Cervical emphysema: an unusual presentation of laryngeal cancer.

BACKGROUND: Acute respiratory decompensation can occur on a background of slowly progressive airway compromise, for example in laryngeal squamous cell cancer. Surgeons in ENT, together with anaesthetists, are often asked to evaluate airway risk and as yet there is no widely adopted standardised approach. CASE REPORT: This paper reports the case of an 82-year-old male, who presented with acute airway compromise due to both endolaryngeal obstruction from a squamous cell cancer and extralaryngeal compression from massive subcutaneous emphysema. RESULTS: Primary total laryngectomy was performed, but the patient declined adjuvant radiotherapy. He died a year later from a heart attack without evidence of recurrence. CONCLUSION: To the best of our knowledge, this is the first case report of acute airway compromise from extralaryngeal subcutaneous emphysema secondary to laryngeal cancer. Options for acute airway management are discussed.

Detection and grading of endolymphatic hydrops in Menière disease using MR imaging.

BACKGROUND AND PURPOSE: Endolymphatic hydrops has been recognized as the underlying pathophysiology of Menière disease. We used 3T MR imaging to detect and grade endolymphatic hydrops in patients with Menière disease and to correlate MR imaging findings with the clinical severity. MATERIALS AND METHODS: MR images of the inner ear acquired by a 3D inversion recovery sequence 4 hours after intravenous contrast administration were retrospectively analyzed by 2 neuroradiologists blinded to the clinical presentation. Endolymphatic hydrops was classified as none, grade I, or grade II. Interobserver agreement was analyzed, and the presence of endolymphatic hydrops was correlated with the clinical diagnosis and the clinical Menière disease score. RESULTS: Of 53 patients, we identified endolymphatic hydrops in 90% on the clinically affected and in 22% on the clinically silent side. Interobserver agreement on detection and grading of endolymphatic hydrops was 0.97 for cochlear and 0.94 for vestibular hydrops. The average MR imaging grade of endolymphatic hydrops was 1.27 ± 0.66 for 55 clinically affected and 0.65 ± 0.58 for 10 clinically normal ears. The correlation between the presence of endolymphatic hydrops and Menière disease was 0.67. Endolymphatic hydrops was detected in 73% of ears with the clinical diagnosis of possible, 100% of probable, and 95% of definite Menière disease. CONCLUSIONS: MR imaging supports endolymphatic hydrops as a pathophysiologic hallmark of Menière disease. High interobserver agreement on the detection and grading of endolymphatic hydrops and the correlation of MR imaging findings with the clinical score recommend MR imaging as a reliable in vivo technique in patients with Menière disease. The significance of MR imaging detection of endolymphatic hydrops in an additional 22% of asymptomatic ears requires further study.

Tumor or hematoma? : An unusual case of an extradural lesion of the lumbar spine.

PURPOSE: Spinal epidural hematoma is a rare clinical entity. We present a case of atypical contrast enhancement pattern in a chronic epidural hematoma of the lumbar spine mimicking an extradural tumor. CASE REPORT: A 76-year-old man on treatment with oral anticoagulants presented with a 1-month history of lower back pain radiating into his right upper thigh accompanied by spinal claudication. Preoperative MRI showed a posterior epidural lesion compressing the cauda equina with almost homogeneous contrast enhancement. Surgery was performed under the presumptive diagnosis of spinal extradural neoplasm. Intraoperative and histological findings were consistent with a chronic spinal epidural hematoma. Postoperatively, the patient had instant relief of his symptoms. CONCLUSION: Chronic spinal epidural hematoma may resemble an extradural tumor, requiring surgery for histological confirmation and decompression.

Homozygous and compound heterozygous mutations in the ATP6V1B1 gene in patients with renal tubular acidosis and sensorineural hearing loss.

Distal renal tubular acidosis (dRTA) is characterized by the inability to excrete acid in the renal collecting ducts resulting in inappropriately alkaline urine and hyperchloremic (normal anion gap) metabolic acidosis in the context of a normal (or near-normal) glomerular filtration rate. Inborn dRTA can be due to autosomal dominant or recessive gene defects. Clinical symptoms vary from mild acidosis, incidental detection of kidney stones or renal tract calcification to severe findings such as failure to thrive, severe metabolic acidosis, and nephrocalcinosis. The majority of patients with recessive dRTA present with sensorineural hearing loss (SNHL). Few cases with abnormal widening of the vestibular aqueduct have been described with dRTA. Mutations in three different genes have been identified, namely SLC4A1, ATP6V1B1, and ATP6V0A4. Patients with mutations in the ATP6V1B1 proton pump subunit develop dRTA and in most of the cases sensorineural hearing loss early in childhood. We present two patients from two different and non-consanguineous families with dRTA and SNHL. Direct sequencing of the ATP6V1B1 gene revealed that one patient harbors two homozygous mutations and the other one is a compound heterozygous. To our knowledge, this is the first case in the literature describing homozygosity in the same dRTA gene on both alleles.

Postoperative changes in the superficial temporal artery and the external carotid artery duplex sonography after extra-intracranial bypass surgery in European Moyamoya disease.

INTRODUCTION: Despite Duplex ultrasonography being a noninvasive, easily repeatable, readily available and economical tool, this examination and its normal ranges are rarely described in Moyamoya disease (MMD). METHODS: Duplex ultrasonography examinations of the superficial temporal artery (STA) and external carotid artery (ECA) were performed preoperatively, postoperatively (within 30 days) and as follow-up exams (6 months postoperatively) after 32 cerebral revascularization procedures in 20 European patients with MMD. RESULTS: A significant higher mean diastolic flow of STA compared to preoperative values was found in postoperative (p<.000) and follow-up exams (p<.001) in Duplex ultrasonography. Postoperative and follow-up Duplex sonography of STA also showed a significantly higher mean systolic flow compared to preoperative values (p<.05 and p<.05). Also ECA showed significantly changes after bypass surgery (p<.05). CONCLUSION: Duplex ultrasonography of STA is a reliable and non-invasive tool to investigate hemodynamic changes after bypass surgery and to detect bypass patency in European patients with MMD.

[Aberrant internal carotid artery as a cause of pulsatile tinnitus: a difficult diagnosis in MRI?]. / Aberrierende A. carotis interna als Ursache eines pulssynchronen Tinnitus : Schwierige Diagnose in der MRT?

We present the case of a 43-year-old patient with sensorineural hearing loss and the finding of an aberrant internal carotid artery in the left tympanic cavity that was causing pulsatile tinnitus. The aberrant vessel was initially invisible on magnetic resonance imaging (MRI) and was confirmed by high-resolution computed tomography and MR angiography (MRA). Recognition of an aberrant course of an internal carotid artery often requires a combination of MRI and MRA to establish the diagnosis and rule out other differential diagnoses.

Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia.

PURPOSE: (a) To assess MR features in patients with Tolosa-Hunt syndrome (THS) and to (b) correlate MR findings with criteria derived from previously reported pathologic observations. METHODS: Fifteen patients with twenty episodes of painful ophthalmoplegia prospectively selected according to International Headache Society (IHS) standards underwent MR examinations focused on the cavernous sinus. Initial examinations in 20 and follow-up MR images in 17 episodes were retrospectively reviewed by 3 independent observers. RESULTS: The primary criteria: an enhancing soft tissue lesion within the cavernous sinus, increase in size and lateral bulging of the anterior cavernous sinus contour were consistently present in 15 initial episodes and in 5 recurrences (20/20). Agreement among observers was 100%. The secondary criteria: internal carotid artery narrowing in 7 patients, extension towards the superior orbital fissure in 13 and orbital apex involvement in 8 patients were unanimously agreed upon in 87.5%, 86.6% and 80%. Complete resolution of findings was observed on follow-up studies. CONCLUSION: In patients with THS the MR features conform to previously reported pathologic findings. MR features are evocative of THS when an increase in size and bulging of the dural contour of the anterior CS supplemented by carotid artery involvement and extension towards the orbit are present. Resolution of findings within 6 months is required to support the diagnosis.

Masticator space abscess derived from odontogenic infection: imaging manifestation and pathways of extension depicted by CT and MR in 30 patients.

Propagation of odontogenic masticator space abscesses is insufficiently understood. The purpose was to analyse pathways of spread in 30 patients with odontogenic masticator space abscess. The imaging findings in 30 patients (CT in 30, MR in 16 patients) were retrospectively analysed. CT and MR imaging depicted a masticator space abscess within: medial pterygoid muscle in 13 patients (43.3%), lateral masseter and/or pterygoid muscle in 14 (46.7%) and superficial temporal muscle in 3 patients (10%). In the lateral masticator space intra-spatial abscess extension occurred in 7 of 14 patients (50%). The sub-masseteric space provided a pathway in seven (70%). Extra-spatial extension involved the submandibular space only in 3 of 14 patients (21.4%). Medial masticator space abscesses exhibited extra-spatial spread only. Extension affected the parapharyngeal space and/or soft palate in 7 of 13 lesions (53.8%). MR imaging in comparison to CT increased the number of abscess locations from 18 to 23 (27.8%) and regions affected by a cellular infiltrate from 12 to 16 (33.3%). The sub-masseteric space served as a previously underestimated pathway for intra-spatial propagation of lateral masticator abscesses. Medial masticator space abscesses tend to display early extra-spatial parapharyngeal space and/or soft palate extension.

Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients.

BACKGROUND AND PURPOSE: Defects at the skull base leading to spontaneous CSF rhinorrhea are rare lesions. The purpose of our study was to correlate CT and MR findings regarding the location and content of CSF leaks in 27 patients with endoscopic sinus surgery observations. MATERIALS AND METHODS: Imaging studies in 27 patients with intermittent CSF rhinorrhea (CT in every patient including 10 examinations with intrathecal contrast, plain CT in 2 patients, and MR in 15 patients) were analyzed and were retrospectively blinded to intraoperative findings. RESULTS: CT depicted a small endoscopy-confirmed osseous defect in 3 different locations: 1) within the ethmoid in 15 instances (53.6% of defects) most commonly at the level of the anterior ethmoid artery (8/15); 2) adjacent to the inferolateral recess of the sphenoid sinus in 7 patients including one patient with bilateral lesions (8/28 defects, 28.6%); 3) within the midline sphenoid sinus in 5 of 28 instances (17.9%). Lateral sphenoid defects (3.5 +/- 0.80 mm) were larger than those in ethmoid (2.7 +/- 0.77 mm, P < or = 0.029) or midsphenoid location (2.4 +/- 0.65 mm, P < or = 0.026). With endoscopy proven arachnoid herniation in 24 instances as reference, MR was correct in 14 of 15 instances (93.3%), CT cisternography in 5 of 8 instances (62.5%). Plain CT in 1 patient was negative. CONCLUSION: In patients with a history of spontaneous CSF rhinorrhea, CT was required to detect osseous defects at specific sites of predilection. MR enabled differentiating the contents of herniated tissue and allowed identification of arachnoid tissue as a previously hardly recognized imaging finding.

The surgeon's view of the anterior ethmoid artery.

OBJECTIVES: To define the relationship of the anterior ethmoid artery to the frontal recess and secondly whether the degree of pneumatisation of the suprabullar recess/supraorbital cell correlates with the distance between the anterior ethmoid artery and the skull base thus making it more vulnerable to damage during surgery. METHOD: Thirty-four cadaver head sides were perfused with pink latex. All specimens had high-resolution computed tomography (CT) scans using bone windows in the axial, coronal and sagittal planes. The specimen's nasal septum was removed and the ethmoid sinuses dissected to locate the anterior ethmoid artery. Calipers were used to measure distance between the artery and the frontal recess and from the skull base. RESULTS: The anterior ethmoid artery was found in all the specimens and scans. The distance between the anterior ethmoid artery and the posterior wall of the frontal recess was 11 mm (range 6-15 mm). In all specimens, the artery was seen between the second and third lamella. The commonest location of the artery was in the suprabullar recess (85.3%). Supraorbital cells were seen in 16 specimens. The ethmoid sinuses were well pneumatised with a large supraorbital cell in 10 of these specimens and in these the artery was lying 3.7 mm (range 1-8 mm) away from the skull base. Six specimens had poor pneumatisation and a small supraorbital cell and in these the artery was found close to or with in the skull base. In specimens without a supraorbital cell, the artery lay at the skull base in all but one. CONCLUSIONS: The position of the anterior ethmoidal artery is very variable. The artery is found between the second and third lamella. When the ethmoid sinuses are more pneumatised and in particular when there is a supraorbital cell, the artery lies below the skull base. A good strategy is to identify the degree of pneumatisation of the ethmoid sinuses from CT scans preoperatively to see if the artery is at an increased risk of being damaged.

Endovascular treatment of cerebral vasospasm following aneurysmal subarachnoid hemorrhage.

Endovascular treatment by balloon angioplasty or intra-arterial papaverine infusion has been established as a valuable treatment option in patients with cerebral vasospasm refractory to maximal medical therapy. A summary of the indications, applications and limitations is provided for microcatheter guided selective papaverine infusion and transluminal balloon angioplasty in patients who sustain cerebral vasospasm following subarachnoid haemorrhage. Structured neuro-intensive and endovascular treatment of imminent vasospasm integrate papaverine administration and balloon angioplasty as complimentary rather than alternative techniques.