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Posttraumatic adrenal hemorrhage is a frequent finding after severe abdominal trauma and can have important clinical implications if it is bilateral. With the increased use of helical CT in the evaluation of trauma patients, posttraumatic adrenal hematoma is more frequently diagnosed. We present the CT findings of a unilateral posttraumatic adrenal hemorrhage where the diagnostic findings only appeared in the follow-up study. We think that mild enlargement of the adrenal gland in a trauma patient can be an early sign of an impending adrenal hemorrhage. [ABSTRACT FROM AUTHOR]

Discusses the clinical case of a boy with dextrocardia and dysphagia. Medical history of the patient; Abnormal location of the duodenojejunal junction; Diagnosis of Kartagener syndrome with oesophageal stricture and midgut malrotation; Causes of intestinal malrotation.

Changes in ventricular size, related to brain edema and hydrocephalus, as well as the extent of hemorrhage are associated with adverse outcomes in patients with subarachnoid hemorrhage (SAH). Frequently, these are measured manually using consecutive non-contrast computed tomography scans. Here, we developed a rule-based approach which incorporates both intensity and spatial normalization and utilizes user-defined thresholds and anatomical templates to segment both lateral ventricle (LV) and SAH blood volumes automatically from CT images. The algorithmic segmentations were evaluated against two expert neuroradiologists on representative slices from 20 admission scans from aneurysmal SAH patients. Previous methods have been developed to automate this time-consuming task, but they lack user feedback and are hard to implement due to large-scale data and complex design processes. Our results using automatic ventricular segmentation aligned well with expert reviewers with a median Dice coefficient of 0.81, AUC of 0.91, sensitivity of 81%, and precision of 84%. Automatic segmentation of SAH blood was most reliable near the base of the brain with a median Dice coefficient of 0.51, an AUC of 0.75, precision of 68%, and sensitivity of 50%. Ultimately, we developed a rule-based method that is easily adaptable through user feedback, generates spatially normalized segmentations that are comparable regardless of brain morphology or acquisition conditions, and automatically segments LV with good overall reliability and basal SAH blood with good precision. Our approach could benefit longitudinal studies in patients with SAH by streamlining assessment of edema and hydrocephalus progression, as well as blood resorption., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: This study aims to showcase the complementary nature of utilizing both histopathology and magnetic resonance imaging (MRI) in understanding the otologic pathophysiology of Meniere disease. In addition, it seeks to raise awareness of the value of preserving and curating historical temporal bone collections which continue to inform our understanding of otologic diseases. Methods: The essential anatomical feature of Meniere disease-the distended membranous labyrinth-is explored through a comparison of early temporal bone studies with contemporary MRI techniques. The histopathologic photomicrographs are of inner ear specimens from deceased patients with symptoms consistent with Meniere disease. The MRI sequences from living patients exhibiting classic Meniere disease symptoms during life are captured 4 hours post-administration of gadolinium. Results: Both histopathologic examination and MRI imaging reveal consistent distention of the saccule, utricle, and scala media in patients with Meniere disease. The study shows the histologic photomicrographs of actual Meniere patients compared to the MRIs and successfully demonstrates the correlation between postmortem histological findings and MRI evidence of distension in living patients. Conclusions: A corresponding distension of the membranous labyrinth is seen in both the histologic specimens and the Meniere MRIs. This correlation suggests the potential utility of utilizing MRI to aid in diagnosing atypical Meniere disease and distinguishing it from other disease processes, such as migraine equivalent vertigo. The integration of historical temporal bone studies with modern MRI techniques offers valuable insights into the pathophysiology of otologic diseases. In addition, it emphasizes the importance of preserving and curating historical temporal bone collections for continued research and medical education purposes. Previous studies of delayed MRIs did not use Meniere disease temporal bone histopathology images., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The pharynx is a complex muscular structure allowing breathing, swallowing, as well speech through common airspace. The normal imaging appearance of the pharynx and cervical esophagus can be challenging given the numerous interleaved surrounding muscles and numerous connections. This article presents the imaging anatomy of the pharynx and cervical esophagus and also discusses the clinical relevance of selected anatomical structures that have important significance in disease development and extension., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: High anterior septal deviation (HASD) is an underreported anatomic variant that can affect the decision to perform septoplasty for access during sinus surgery and ease of postoperative debridement. This study aims to 1) describe an objective method of assessing HASD, and 2) explore its prevalence and implications for performing septoplasty., Study Design: Retrospective cross-sectional study., Methods: Computed tomography scans from 2014 to 2020 were retrospectively reviewed. Two independent observers measured the following with respect to midline: distance to septum (SDD), distance to lateral nasal wall (LNW), and septal deviation angle (SDA)., Results: A total of 147 patients were included, with excellent interrater reliability across 99 patients (0.8-0.9). Mean measurements across all patients were SDD (2.77 mm ± 1.34), SDD/LNW (0.26 ± 0.12), and SDA (8.9° ± 4.0). Of 102 patients who underwent sinus surgery, 47 received septoplasty. Compared to the non-septoplasty cohort, the septoplasty cohort had a greater mean SDD (3.61 mm ± 1.48 vs. 2.27 mm ± 0.95; d = 1.10 [95% CI 0.67-1.51]), SDD/LNW (0.34 ± 0.13 vs. 0.21 ± 0.09; d = 1.18 [95% CI 0.76-1.60]), and SDA (11.1° ± 4.3 vs. 7.3° ± 3.4; d = 1.00 [95% CI 0.58-1.40]). Receiver operating characteristic cutoffs were SDD ≥2.43 mm, SDD/LNW ≥0.25, and SDA ≥7.6°, corresponding to a 49%-58% prevalence of HASD., Conclusion: HASD is relatively common and the methods described herein can reliably assess its dimensions. Measurements of SDD, SDD/LNW, and SDA exceeding cutoffs determined by this study may represent clinically significant deflections prompting consideration of septoplasty. These methods may aid in preoperative planning., Level of Evidence: 4 Laryngoscope, 132:1166-1171, 2022., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: X-linked deafness (XLD) is a rare disease, characterized by typical cochlear incomplete partition type 3 anomaly (IP-III). Accompanying hypothalamic anomalies were also recently described. The purpose of this study was to document the temporal bone and intracranial imaging findings in a series of patients with XLD with a review of the literature, to better understand this anomaly., Methods: The CT and MRI studied of 13 XLD patients were retrospectively evaluated. All structures of the otic capsule (OC) were subjectively and retrospectively assessed. The OC thickness and the size of the cochlea were measured and compared to the age-matched control group. Intracranial structures were also evaluated with specific attention to the hypothalamic region., Results: All cases had bilateral IP-III anomaly, bulbous internal auditory canals (IACs), absent bony modiolus with preserved interscalar septa, intact cochleovestibular, and facial nerves. OC thickness was decreased in all cases compared to the control group (p<0.001). In XLD patients, the cochlea had decreased transverse dimension and increased height compared to the control group (p< 0.001). Five patients (38.4%) had bilateral cystic structures adjacent to the vestibule and/or semicircular canals (SCCs). Hypothalamus was thickened or had a lobular appearance in all cases (subtle in one). Additionally, hamartoma-like appearance of the hypothalamus was present in half., Conclusion: XLD is a rare inner ear anomaly that is frequently associated with hypothalamic malformations. The OC thickness of IP-III patients appears to be decreased with accompanying decreased transverse dimension of the cochlea which could have implications in electrode selection during cochlear implantation. Cystic /diverticular lesions surrounding the vestibule and semicircular canals are also frequently seen but a rarely reported finding.

Purpose: Large endolymphatic duct and sac (LEDS) is one of the most common imaging abnormalities in congenital sensorineural hearing loss and is frequently seen with coexistent cochlear anomalies, especially incomplete partition type II. However, MRI findings of accompanying cochlear and especially modiolar dysplasias may be subtle. The purpose of this study is to evaluate the imaging findings of LEDS with high-resolution imaging at 3 T and correlate with the audiological data., Methods: 3 T temporal bone MRIs of 54 ears in 30 LEDS patients were retrospectively evaluated. The cochlear dysmorphism and modiolar deficiency were assessed qualitatively and quantitatively. The severity of LEDS anomaly, the signal changes within the LEDS and cochlea were also noted. The imaging findings were correlated to the audiological data., Results: The cochlea was abnormal in 77.8% of the ears with an isolated modiolar deficiency in 11.1%. Cochlea and modiolus were completely normal in 11.1% of the ears. In 63% of the ears X-distance was increased. T2 hypointensity within LEDS and cochlea were detected in 42.6%, and 7.4% of the ears, respectively. The median diameters of LEDS were higher in ears with severe to profound HL than ears with normal to moderate HL (p < 0.05). The X-distance, presence of T2 hypointensity within LEDS, and diameters of modiolus did not show statistical correlation with the audiographic data., Conclusion: High-resolution 3 T imaging of patients with LEDS anomaly revealed a spectrum of cochlear anomalies, but up to 11.1% of the ears had no underlying anomaly despite severe (endolymphatic duct/sac) dilatation and/or profound HL., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Clival infiltration is frequently seen in nasopharyngeal carcinoma (NPC) and the resultant bone marrow signal changes (BMSC) can persist even after complete tumor response to the radiation therapy (RT). The differentiation of those residual BMSC from recurrent/persistent disease may be challenging. We performed serial analysis of the clival BMSC after RT, to define an expected temporal evolution of those signal changes during the follow-up., Materials and Methods: Serial MRI studies of 50 NPC patients (with or without initial clival infiltration) who had undergone RT were retrospectively examined. Abnormal clival BMSC and contrast enhancement (CE) were evaluated on each follow-up scan. Duration of BMSC/CE was correlated with the degree of baseline clival involvement (BCID), RT dose, and primary mass volume (PMV)., Results: Clival BMSC persisted without any evidence of recurrence, for a mean of 66.5 (max. 137) months (with accompanying CE for up to 125 months) in 26 patients with clival infiltration at diagnosis. Duration of BMSC and CE showed statistical correlations with PMW (p < 0.05), but not with RT dose or BCID. The rate of recurrence in clivus was 14%. New clival lesions that occurred within the first 12 months after RT (in six patients) did not develop recurrence suggesting radiation osteitis (12%)., Conclusion: After RT, residual clival medullary signal change/enhancement is seen in most NPC patients and can persist even years without recurrence.

Background: Graves' ophthalmopathy is the most common extrathyroidal manifestation of Graves' disease. The objective of this study was to investigate the clinical ophthalmological and MRI findings in newly diagnosed Graves' ophthalmopathy., Methods: This study included 36 newly diagnosed Graves' disease patients and 23 control participants. Patients and control participants underwent detailed ophthalmologic examination. In addition, all subjects underwent orbital MRI examination; and sizes, cross-sectional areas, and signal intensities of extraocular muscles were also measured., Results: Based on MRI measurements, the mean exophthalmos in the left eye was significantly higher in the patient group when compared to those of controls (2.04 ± 0.29 vs. 1.85 ± 0.15 cm, p  = 0.003). The mean long diameter of inferior oblique muscle in both the right and left eyes were significantly shorter in patients when compared to those of controls ( p  = 0.001, p  = 0.002, resp.); however, the mean long diameter of superior oblique in the left eye was longer in patients than those of controls ( p  = 0.001). Patients had significantly higher superior oblique muscle signal intensity than those of controls in the right eye ( p  = 0.01). There was no significant difference for the other parameters between the patient and control groups., Conclusion: Our findings suggest that there is no obvious change in MRI examination despite clinical ophthalmological findings in patients with newly diagnosed Graves' ophthalmopathy. Unnecessary MRI examination should be avoided in this patient group due to unsatisfactory cost-effectiveness., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2021 Yakup Cevik et al.)

Background: Although casts or external splint materials are commonly applied on the external nose after rhinoplasty, their beneficial effects have not been previously demonstrated in controlled studies., Methods: A total of 40 adult patients who underwent primary rhinoplasty were included in this prospective study. The patients were randomized into 2 groups to use an external splint or not after surgery. Only surgical taping was applied to the nasal dorsum of the patients for whom external splints were not used. The periorbital edema and ecchymosis levels of the patients were recorded on the second and seventh days after surgery. Moreover, all patients had a computed tomography scan at the third postoperative week to measure the distance between the beginning of the osteotomy line on the right and left sides and the midline., Results: On the second and seventh postoperative days, the levels of periorbital edema and ecchymosis were significantly lower in the patients with taping than in the patients with splints (P < 0.05 for each). No statistically significant difference was observed in terms of the difference in the distance between the beginning of the osteotomy line on the right and left sides to the midline between the group with external splints and that with tape (P = 0.661)., Conclusions: External splinting may not be used in cases where osteotomy is not performed or in patients where the osteotomies are sufficiently stable. Thus, the disadvantages of external splinting are avoided, and at the same time, periorbital edema and ecchymosis are less common., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To share our experience in diagnosis of congenital central nervous system (CNS) abnormalities by fetal magnetic resonance imaging (MRI)., Study Design: This study consisted of 110 pregnancies. Neurosonography (NS) findings were compared with MRI results. Anomalies were categorized into 10 groups: 1) Corpus callosum (CC) and cavum septum pellucidum (CSP) anomalies, 2) Neural tube defects (NTD), 3) Posterior fossa anomalies (PFA), 4) Primary ventriculomegaly (PVM), 5) Microcephaly, 6) Macrocephaly, 7) Periventricular leukomalacia (PVL), 8) Craniosynostosis, 9) Intracranial hemorrhage (ICH) and 10) Lumbosacral teratoma. Demographic features, clinical characteristics and perinatal outcomes of the study subjects were evaluated., Results: Gestational weeks for NS and for MRI were 25.5 and 26.5 weeks, respectively. Fourteen (12.7%) pregnancies were terminated. PVM (n = 36, 32.7%), CC and CSP anomalies (n = 29, 26.3%), PFA (n = 11, 10%) and NTD (n = 11, 10%) were the most common fetal MRI indications. There were no statistically significant differences between the accuracy of fetal NS and fetal MRI for CC and CSP anomalies, NTDs, PFA and PVM (p = 0.09, 0.43, 0.45 and 0.23, respectively). However, fetal MRI was more accurate for the detection of normal anatomic findings in cases with suspected microcephaly, macrocephaly and craniosynostosis in NS when pooled together (p = 0.007). Furthermore, MRI also seemed to be advantageous in CC & CSP anomalies though it was not validated by statistical measures. No statistically significant difference was found for diagnostic performance of NS and MRI according to gestational week (p = 0.27)., Conclusion: Fetal MRI in addition to NS may improve diagnostic accuracy in pregnancies with congenital CNS abnormalities., Competing Interests: Declaration of Competing Interest The authors state that they have no conflict of interest in this study., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objective: At present, early detection of spinal osteomyelitis is a challenge. Patients may present with non-specific symptoms and diagnostic imaging studies may be obtained for seemingly unrelated complaints. Paraspinal fat stranding on body computed tomography (CT) as a sign of osteomyelitis is easily overlooked and has not been reported in the literature to our knowledge. The purpose of this study is to review findings on body CT that points to unsuspected spinal osteomyelitis., Material and Methods: A retrospective review of patients with spinal osteomyelitis who also had concomitant chest, abdominal, or pelvic CT scans between August 2013 and February 2017 yielded 10 patients who had confirmed osteomyelitis (ages between 51 and 75, mean age 64.8). Images and medical records were reviewed., Results: The age of the patients ranged from 51 to 75 years (median value, 64). All patients had multiple underlying medical illnesses, and half of them had a known preceding infection such as sepsis or urinary tract infection. At presentation, three patients had a fever and two patients had neurologic deficits. Seven out of eight patients had elevated C-reactive protein and erythrocyte sedimentation rate, five patients had leukocytosis, and four patients had positive blood cultures. Paravertebral fat stranding and endplate erosions were observed in 9 and 6 cases, respectively, on initial body CT for unrelated indications, and subsequent magnetic resonance imaging confirmed osteomyelitis discitis., Conclusion: Clinically significant, but initially unsuspected, spinal pathology such as osteomyelitis may present on body CT scans. Earlier diagnosis of spinal osteomyelitis can be made by performing a focused evaluation of the paraspinal soft tissues and including osteomyelitis in the differential diagnosis, particularly in high-risk patients., Competing Interests: There are no conflicts of interest., (© 2020 Published by Scientific Scholar on behalf of Journal of Clinical Imaging Science.)

Objective: To evaluate spinal MRI features of mucopolysaccharidosis (MPS) VI and to assess the correlation with clinical findings., Methods: We retrospectively evaluated spinal MRI scans and clinical findings at the time of imaging in 14 patients (8 male, 6 female) with MPS VI. Craniometric measurements were performed and the images were assessed for bony anomalies, spinal stenosis and spinal cord compression. The degree of cervical cord compression was scored and correlated with neurological examination findings at the time of imaging. Vertebral alignment, structural changes in spinal ligaments and intervertebral discs were also assessed., Results: All patients had cervical stenosis due to bony stenosis and thickened retrodental tissue (median: 6.05 mm, range 3.3-8 mm). Retrodental tissue thickness was found to increase with age (p = 0.042). Compressive myelopathy was detected at upper cervical level in 11 (79%) and lower thoracic level in 2 patients (14%). Significant inverse correlation was found between cervical myelopathy scores and neurological strength scores. The most common bony changes were hypo/dysplastic odontoid; cervical platyspondyly with anterior inferior beaking; thoracic posterior end plate depressions and lumbar posterior scalloping. Kyphosis due to retrolisthesis of the beaked lumbar vertebrae and acute sacrococcygeal angulations were other remarkable findings., Conclusion: MRI is an essential component in evaluation of spinal involvement in MPS VI, and scanning of the entire spine is recommended to rule out thoracic cord compression. Advances in knowledge: This study provides a detailed description of spinal MRI findings in MPS VI and underlines the role of MRI in management of cord compression.

Objective: Autonomous cortisol secretion of adrenal incidentalomas (AIs) is associated with poor cardiovascular outcome. Because centripetal obesity is a cardiovascular risk factor, we aimed to investigate whether autonomous cortisol secretion is associated with increased visceral fat accumulation., Design: Retrospective cohort study., Patients: Patients with AIs who attended for follow-up between January 2014 and December 2016 were evaluated. Autonomous cortisol secretion was diagnosed when 1 mg overnight dexamethasone (post-DST) cortisol was >50 nmol/L at baseline and follow-up. Follow-up duration was 34 (12-105) months. Thirty patients with nonfunctioning AIs and 44 patients with autonomous cortisol secretion were included. Adrenalectomy was performed in five patients. Six patients with Cushing's syndrome were also recruited., Measurements: Hormonal evaluation and assessment of total (T), visceral (V) and subcutaneous (S) fat area by computed tomography and calculation of V:S and V:T ratios at baseline and follow-up., Results: V, V:S and V:T increased (P<.001 for each comparison, Wilcoxon signed rank test for repeated measures) in patients with autonomous cortisol secretion while did not change significantly in patients with nonfunctioning adenomas. Linear regression models including post-DST cortisol, gender, concomitant treatments and follow-up duration showed that both baseline and follow-up DST significantly predicted Δ(V:S) and Δ(V:T) (P<.01 for all models)., Conclusions: In patients with AIs, a post-DST cortisol >50 nmol/L at both baseline and follow-up, was associated with a significant increase in visceral fat after a follow-up duration of ~3 years. This may be of importance to explain the link between autonomous cortisol secretion and poor cardiovascular outcome., (© 2017 John Wiley & Sons Ltd.)

Objectives  To determine the use of diffusion-weighted imaging (DWI) in the pre- and posttreatment evaluation of skull base chordomas. Design  Retrospective study. Setting  Tertiary care university hospital. Participants  In total, 17 patients with histopathological diagnosis of chordoma who had magnetic resonance (MR) imaging and DWI were evaluated. Of them, 13 patients had posttreatment MR imaging including DWI. Main Outcome Measures  Three apparent diffusion coefficient (ADC) values were obtained from tumor, and an ADC value was measured from pons for the purpose of normalization. ADC values of the subtypes of chordomas (typical and chondroid chordomas) were compared. Results  Ten (59%) masses had increased signal on trace DWI at pretreatment evaluation. The mean ADC entire tumor /ADC pons was calculated as 1.55 ± 0.44. The mean ADC entire tumor values of typical and chondroid chordomas were 1.26 ± 0.29 × 10 -3 mm 2 /s and 0.99 ± 0.46 × 10 -3 mm 2 /s, respectively. There was no statistically significant difference between ADC values of the subtypes ( p  > 0.05). For posttreatment evaluation, DWI enabled detection of residual tumor in the majority (85%) of cases. Conclusions  DWI is useful in diagnosis and posttreatment evaluation of skull base chordomas. However, ADC values in our series did not distinguish the subtypes of chordomas.

Purpose: The aim of this study was to determine the correlation between acute invasive fungal rhinosinusitis (AIFRS) and underlying diseases, micro-organisms, presenting symptoms, extent of disease, radiologic findings, and outcomes and propose a new classification system., Materials and Methods: The data of 19 AIFRS cases were analyzed retrospectively. Magnetic resonance imaging and computed tomography were performed in all patients preoperatively. All patients underwent at least 1 surgical debridement., Results: Hematologic diseases were the most common (52%) underlying diseases. Patients with type 2 diabetes and those with multiple etiologies causing immunosuppression had the lowest survival. Aspergillus and Mucoraceae species were isolated in 9 patients but were not associated with poor prognosis. Headache and nasal discharge or crusting were the most common presenting symptoms. Premaxillary involvement was significantly correlated with poor prognosis (P = .001). Unilateral involvement was correlated with poor prognosis, although this finding was not significant (P = .111). The overall mortality rate was 61.2%. Patients with neutropenia that was corrected had 80% survival (P = .014). Cessation of corticosteroids and regulating blood glucose levels in patients with immunosuppression from corticosteroid use resulted in 75% survival., Conclusion: There is no single curative treatment for AIFRS. For a favorable prognosis, underlying conditions must be treated in addition to surgical debridement and antifungals., (Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: Articulation of rostrum of sphenoid bone with alae of vomer forms a schindylesis type of joint. The circumference of this joint, called sphenovomerine suture (SVS), is very important in establishing a reliable surgical field in the endoscopic transsphenoidal pituitary surgery. Because of its vital role in endoscopic transsphenoidal pituitary surgery, this radio-anatomical study was designed to establish the morphological properties of SVS., Methods: In this study, the authors examined SVS in 235 patients (121 females and 114 males) on the computed tomography images of the paranasal sinus and made 4 measurements to describe SVS., Results: The mean distance between superior margin of the upper labial philtrum and top of SVS was 6.66 ± 0.43 cm for females and 7.44 ± 0.54 cm for males. The distance between the top of SVS and dorsum sellae was 3.08 ± 0.33 cm for females and 3.19 ± 0.32 cm for males, the alae of vomer angle in the upper surface was 74.22 ± 20.06° for females and 74.23 ± 19.68° for males. The distance between the most lateral points of 2 alae of vomer was 0.99 ± 0.17 and 1.01 ± 0.19 cm for females and males, respectively., Conclusions: For an easy and successful operation, removal of the SVS is very important as it will provide a better view of the sellar base and make the management of the surgical instruments easier in the wider safe surgical field thus created.

Background: Inflammatory myofibroblastic tumor is a rare benign neoplasm and most commonly involves the lung but occurs in extrapulmonary locations., Objective: To present imaging findings in inflammatory myofibroblastic tumors in children based on a single-centre experience., Materials and Methods: We retrospectively reviewed CT and MRI findings of children diagnosed with inflammatory myofibroblastic tumor in a single institution., Results: We identified 15 children (range: 1-17 years) with inflammatory myofibroblastic tumor. The tumor was localized to the lung (n = 5), mediastinum (n = 3), trachea (n = 1), bronchus (n = 1), abdomen (n = 2) and orbit (n = 3). All the extraorbital tumors were solid masses with homogeneous or heterogeneous enhancement. Four lung tumors and one posterior mediastinal tumor contained calcification. Local recurrence following surgical removal occurred in two children with invasion of the esophagus and of the left atrium in one. Localized masses were seen in all children with orbital tumour. Two of these had episcleritis and perineuritis; one had episcleritis, tendonitis, perineuritis, myositis and dacryoadenitis., Conclusion: The locations and imaging features of inflammatory myofibroblastic tumors are variable.

Purpose: Chordoma is a rare neoplasm that arises from embryonic notochordal remnants along the axial skeleton (i.e., clivus, sacrum) and the vertebral bodies. They comprise less than 1 % of CNS tumors and 1-4 % of all bone malignancies. It rarely affects children and adolescents (<5 %). Chordomas are locally aggressive and highly recurrent. Their management is challenging for clinicians., Methods: This retrospective study includes six pediatric patients with pathological evidence of clival chordoma. These cases were identified over a period of 15 years in a tertiary care institute., Results: There were two boy and four girls with a mean age of 10.6 years (range, 4-16 years). The chief complaint was due to cranial nerve palsy (or dysfunction), mostly affecting lower cranial nerves (66.6 %), followed by diplopia and headache. One patient had obstructive sleep apnea. All patients were operated and a total of 15 surgeries were performed (mean, 2.5). Tumor recurrence was observed in four patients (67 %). Two-year and 5-year progression-free survivals (PFS) were 67 and 33 %, respectively. None of the patients were lost either during the surgery or the follow-up period (6.9 years: 1-14 years)., Conclusions: Clival chordomas are challenging tumors in neurosurgical practice. A multidisciplinary approach is warranted in each patient. Today, the best management strategy seems to be surgical resection followed by radiotherapy. Chemotherapy should be considered in selective and preference basis. Sharing institutional experiences will provide future insights in prognosis of these rare tumors. Implementing newer surgical instruments, endoscope in particular, is encouraged in management of the clival chordomas.

Background and Purpose: Endochondral ossification of the otic capsule is a process that continues postnatally; hence, incomplete endochondral ossification is seen as pericochlear hypoattenuation on temporal bone CT scans of children. We determined the prevalence and extent of this entity in a large series and assessed its relation to age and underlying sensorineural hearing loss., Materials and Methods: Initially, temporal bone CTs of 40 children with sensorineural hearing loss were retrospectively assessed and compared with those of a control group scanned for non-sensorineural hearing loss reasons to assess any difference in the prevalence or extent of incomplete endochondral ossification. Then the CT scans of 510 children (age range, 17 days to 17 years) were retrospectively reviewed, and any observed endochondral ossification areas were classified as mild, moderate, or extensive, according to their extent., Results: Neither the presence nor degree of incomplete endochondral ossification had any significant correlation with the presence of sensorineural hearing loss (P = .08 and P = .1, respectively). Incomplete endochondral ossification was more frequently seen (62% of cases) than complete ossification. There was no statistically significant correlation between incomplete endochondral ossification and sex (P = .8), but an inverse correlation was found between the presence of incomplete endochondral ossification and increasing age (P < .001). Overall, mild incomplete endochondral ossification was the most frequent involvement pattern (44.4%)., Conclusions: The pericochlear hypoattenuation in the otic capsule representing incomplete endochondral ossification is a normal finding in children and can be seen as a marked curvilinear hypoattenuation at younger ages in the absence of any clinical disorder., (© 2015 by American Journal of Neuroradiology.)

Vanishing white matter disease is one of the most prevalent leukodystrophies in childhood. It is caused by mutations in any of the genes encoding the 5 subunits of the eukaryotic translation initiation factor 2B (eIF2B), EIF2B1 through EIF2B5. Phenotypic variation is wide and it may affect people of all ages. Here we present a child with vanishing white matter who had hepatomegaly and hypertriglyceridemia attacks along with neurologic deterioration episodes. He was found heterozygous for the 2 mutations c.817 A>C, p.Lys273Gln and c.939&#95;948del, p.Asp314ProfsX23 in the gene EIF2B2. Until today, this association was not defined in the literature.

Presentation of an interrupted aortic arch (IAA) in adulthood is extremely rare. Nonhemorrhagic stroke has not been reported previously in any adult with IAA. We, herein, describe a formerly asymptomatic 52-year-old male presenting with recurrent vertebrobasilar circulation ischemic strokes resulting from accelerated atherosclerotic arteriopathy secondary to IAA associated upper body hypertension. Surgical correction of IAA led to treatment of hypertension and cessation of ischemic attacks together with regression of collateral arterial networks as shown by computer tomography angiography., (Copyright © 2012 by the American Society of Neuroimaging.)

Acromegaly's effect on voice is still indefinite. We aimed to define acoustic characteristics of patients with acromegaly. Cross-sectional case-control study was designed. Thirty-seven patients with acromegaly and 30 age- and sex-matched healthy controls were included. Fundamental frequency (F0) and measurements related to frequency, amplitude, noise and tremor of the obtained voice sample were analyzed using Multi-Dimensional Voice Program. Absolute jitter (Jita) and jitter percent (Jitt), shimmer in decibel and shimmer percent, noise to harmonic ratio and soft phonation index, fundamental frequency tremor frequency and frequency tremor intensity index represented the parameters related to frequency, amplitude, noise and tremor of the voice sample, respectively. Patients with acromegaly, especially the uncontrolled patients, exhibited significant differences in frequency perturbation measurements. Jitt of all patients and Jita of uncontrolled patients were significantly higher than that of control group (p = 0.044 and p = 0.043, respectively). Jitter which is a measure of frequency perturbation can be assumed as an indicator of hoarse and deepened voice. Jita of all patients and Jitt of uncontrolled patients were elevated, but not reaching a statistical significance. Controlled and active patients had similar analysis of acoustic parameters. In the correlation analysis, shimmer and IGF-1 (insulin like growth factor 1) was found to be positively correlated in all patients with acromegaly and in female patients. When the p value is adjusted according to Bonferroni correction regarding the use of ten parameters for acoustic analysis (so adjusted p is <0.005), all the statistically significant findings become insignificant. Considering the parameters test different properties of voice, it is reasonable to pay attention to the findings. Patients with acromegaly have increased frequency perturbations measures, but this increase is non-significant according to Bonferroni correction. This may be perceptually sensed as hoarse voice. Amplitude perturbations within the voice of the patients with acromegaly are positively correlated with IGF-1 levels, this correlation is also non-significant according to Bonferroni correction.

Proteus syndrome is a rare disorder with progressive asymmetrical and disproportionate overgrowth of various tissues of the body. The syndrome is characterized by a wide range of malformations, including craniofacial deformities. Extraoral examination revealed several of the classical craniofacial features of Proteus syndrome: pronounced hemifacial hypertrophy, macrodactyly and hyperostosis. Intraoral examination revealed a high arched palate and gingival hyperplasia. Other findings were unilateral enlargement of the tongue, alveolar growth and dilaceration of the roots of the teeth. There were severe degenerative changes and deformities in the left temporomandibular joint but the oversized condyle was asymptomatic; there was no pain, limitation and deviation at mouth opening. Treatment was not necessary owing to the asymptomatic situation but periodic follow-up with clinical and radiographic examination was considered. The aim of this article is to describe the radiographic manifestations of an asymptomatic condyle malformation and other craniofacial, oral and dental findings in a 33-year-old female patient with known Proteus syndrome.

Purpose of Review: To review the literature on early visual manifestations of subacute sclerosing panencephalitis (SSPE) with regard to two patients who had visual problems preceding the onset of neurological symptoms. One patient had cortical visual disturbances and the other had visual loss due to retinal pigment epithelial changes., Recent Findings: SSPE is a chronic encephalitis characterized by a history of measles infection and a progressive disease of the central nervous system that still occurs frequently in countries with insufficient measles immunization. Visual manifestations can occur as a result of involvement of the pathways that lead from the retina to the occipital cortex during the course of the disease, but are rare as a presenting sign. Fundus changes, especially macular retinitis and macular pigment disturbances, appear to be the most common ocular manifestations of SSPE., Summary: Ophthalmologists must be aware that SSPE can knock their door with ocular findings of SSPE, months or years before the onset of neurological symptoms.

Purpose: Meningiomas are mostly benign, however atypical or malignant subtypes with more aggressive clinical course and higher recurrence rates can also be seen. The purpose of this study was to determine whether histopathological subtypes of meningiomas could be assessed preoperatively using apparent diffusion coefficient (ADC) values., Materials and Methods: Conventional magnetic resonance (MR) and diffusion-weighted (DW) imaging of 177 adult patients with pathologically proven meningiomas were retrospectively evaluated. Tumor size and the degree of associated edema were noted. The signal intensity of the lesions on DW imaging was evaluated and graded. Mean ADC values were obtained as the mean of measurements from three regions of interests within the mass. ADC ratios of meningioma/contralateral normal appearing subcortical parietal white matter were also calculated., Results: The histopathological analysis revealed 135 benign, 37 atypical and 5 malignant lesions. With classification according to the subtype, the mean ADC values and ratios of benign meningiomas were as 0.99±0.12×10(-3) mm(2)/s and 1.22±0.07, respectively. ADC values for atypical and malignant groups were both 0.84±0.1×10(-3) mm(2)/s. The ADC ratios were 1.05±0.1 and 0.96±0.2 for atypical and malignant subtypes, respectively. There was no statistically significant difference between the mean ADC ratios of the three subtypes (ANOVA test; P≥0.05). Gender, age of the patients and tumor size showed no statistically significant difference between the different histological groups., Conclusion: DW MR imaging was not found to have any additional value in determining histological behaviour nor in differentiating histopathological subtypes of meningiomas., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Sarcoidosis is a chronic, multisystemic, non-caseating granulomatous disease of unknown etiology. Nasopharyngeal involvement is very rare in sarcoidosis. The objective of this report was to evaluate a rare involvement of sarcoidosis. This report includes a case of nasopharyngeal sarcoidosis. A 51-year-old female with nasopharyngeal sarcoidosis was treated as sarcoidosis, and she was better. Nasopharyngeal involvement is very rare in sarcoidosis but it must be kept in mind.

Context: The impact of acromegaly on the auditory system remains unknown., Objective: This study aimed to examine audiological symptoms and the structure and function of the auditory system in patients with acromegaly. DESIGN/SETTING AND PATIENTS:  The study included 44 patients with acromegaly. Pure tone audiometry, speech audiometry, tympanometry, otoacoustic emissions testing, computerized tomography of the temporal bone and magnetic resonance imaging of the ear were performed in all patients. The study also included 36 age- and sex-matched healthy controls. The patients with acromegaly were divided into three subgroups, as follows: controlled disease (n=13); partially controlled disease (n=16); uncontrolled disease (n = 15)., Results:   In all, 43% of the patients with acromegaly had hearing loss, and 20% had had an episode of otitis in at least one ear. Median pure tone average (PTA) in the patients with acromegaly was 12.5 dB (range: 2-72 dB), vs 8.3 dB (range: 0-20 dB) in the control group (P < 0.001). PTA did not differ significantly between the three patient subgroups. Audiometric tests showed various degrees of hearing loss in at least one ear in 21 patients (48%). Conductive, sensorineural and mixed type hearing loss in at least one ear was noted in 9%, 30% and 18% of the patients, respectively. Auditory imaging showed that 50% of the patients had temporomandibular joint degeneration (TMJD)., Conclusions:   Hearing loss is quite common in patients with acromegaly. Contrary to common belief, in this study, conductive hearing loss did not occur more frequently than other types. Based on PTA findings in the controls and patients with acromegaly, acromegaly caused hearing loss, but the level of disease activity had no effect on hearing. Middle ear pressure problems might be caused by increased perilymph because of growth hormone (GH)-related volume overload., (© 2012 Blackwell Publishing Ltd.)

Maxillofacial osteosarcoma constitutes a minor percentage of all the head and neck tumors. We describe a 10 year-old girl presenting with swelling and pain in left maxillary region and diagnosed as low grade osteosarcoma. The patient was operated and given a chemotherapy protocol consisted of Cisplatin and Doxorubicin. After six courses of chemotherapy the patient was in complete remission and she is well with no evidence of disease for five years. Since high local recurrence rates have been reported in craniofacial osteoarcoma and we know the deleterious side effects of radiation therapy in children, we believe that best management strategy for osteosarcomas in maxillofacial region in children is radical surgical excision and postoperative chemotherapy (Fig. 3, Ref. 11).

Introduction: Giant cavernous malformations (GCM) are very large, low-flow vascular malformations, which usually have atypical imaging features and are commonly misdiagnosed preoperatively as neoplasms or vascular malformations. These lesions have mostly been reported in children. As cavernomas show different features in children compared to adults, we evaluated the imaging features of pediatric GCMs in order to help in the preoperative diagnosis of these malformations., Methods: Brain MR studies of nine children (mean age of 4 years; 8 months-9 years) with biopsy-proven GCM were retrospectively evaluated. We defined GCMs as cavernomas of ≥4 cm. Lesions were evaluated regarding their size, location, signal characteristics, general appearance (uni/multilocular) as well as regarding the presence of mass effect, edema, and fluid-fluid levels and were classified according to the Mottolese classification of pediatric cavernomas., Results: Lesion locations were parietal (n = 5), frontal (n = 2), temporal, and intraventricular. Seven lesions were in the periventricular region (with five in the periatrial region). Six patients had T1 hyperintense multilobulated lesions with "bubbles of blood" appearance and three patients had heterogeneous lesions with reticular core. All lesions had mass effect, edema (marked in four cases), and peripheral hemosiderin rim. Fluid-fluid levels were also common (n = 7). Most of our lesions (six of nine) were classified as type IIIA, two as type IIIC, and one as type IA., Conclusion: In children, a GCM should be considered in case of very large hemorrhagic intra-axial mass with "bubbles of blood" multicystic appearance, surrounding hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect, especially in the periatrial location.

Background and Purpose: SBO is a life-threatening infection that may have radiologic features similar to those of the neoplastic processes. The purpose of this study was to evaluate the DWI findings in SBO to facilitate the differential diagnosis., Materials and Methods: The MR imaging findings of 9 patients with SBO were retrospectively evaluated and compared with MR imaging studies from 9 patients with NPC, 9 with lymphoma, and 9 with metastatic disease of the skull base. ADC measurements were performed from the ADC(ST) and the ADC(NST) in all 4 groups., Results: The mean ADC(ST) values were 1.26 ± 0.19 × 10(-3) mm(2)/s for SBO, 0.74 ± 0.18 × 10(-3) mm(2)/s for NPC, 0.59 ± 0.11 × 10(-3) mm(2)/s for lymphoma, and 0.99 ± 0.34 × 10(-3) mm(2)/s for metastatic disease, respectively. The mean ADC value of SBO was significantly higher than those of NPC and lymphoma (P < .0001). There was no significant difference for the comparison of SBO and metastatic lesions. When an ADC value equal to or higher than 1.08 × 10(-3) mm(2)/s was used to rule out lymphoma and NPC, the accuracy was 96%., Conclusions: Although SBO is a relatively rare condition, its differential diagnosis from neoplastic processes of the skull base is essential to start appropriate treatment promptly. ADC values may help to distinguish patients with SBO from those with malignant lesions.