Congenital Monocular Strabismus Fixus.

PURPOSE: To investigate the clinical characteristics and magnetic resonance imaging (MRI) findings of the extraocular muscle and ocular motor nerves in congenital monocular strabismus fixus. METHODS: The retrospective observational case series of three patients with congenital monocular strabismus fixus were reviewed between January 1, 2006, and December 31, 2016. Ophthalmologic examination and thin-sectioned MRI of the ocular motor nerve and the orbit were performed on the three patients. RESULTS: Three patients presented with unilateral non-progressive strabismus fixus with marked limitations of movement in all directions since birth. Of the three patients, one presented with esotropia, one with a large degree of exotropia and hypertropia, and one with an almost normal primary position. All three patients had normal ocular motor nerves, but adherences among the extraocular muscles, posterior Tenon's capsule, and the globe within the muscle cone on MRI. Two patients underwent strabismus surgery, but there were no postoperative improvements in the primary position and eye movements. CONCLUSIONS: Extensive adherences among the extraocular muscles, posterior Tenon's capsule, and globe may partially explain the cause of congenital monocular strabismus fixus and why strabismus surgery was ineffective. The findings further highlight the importance of MRI in detecting and characterizing atypical forms of strabismus. [J Pediatr Ophthalmol Strabismus. 2018;55(6):363-368.].

Clinical and histopathological features of adenomas of the ciliary pigment epithelium.

PURPOSE: Adenomas of the ciliary pigment epithelium (CPE) are rare benign tumours which have mainly to be differentiated from malignant ciliary body melanomas. Here we report on a consecutive series of patients with CPE adenomas and describe their characteristics. METHODS: The retrospective hospital-based case series study included all patients who were consecutively operated for CPE adenomas. RESULTS: Of the 110 patients treated for ciliary body tumours, five patients (4.5%) had a CPE adenoma. Mean age was 59.0 ± 9.9 years (range: 46-72 years). Mean tumour apical thickness was 6.6 ± 1.7 mm. Tumour colour was mostly homogenously brown to black, and the tumour surface was smooth. The tumour masses pushed the iris tissue forward without infiltrating iris or anterior chamber angle. Sonography revealed an irregular echogram with sharp lesion borders and signs of blood flow in Color Doppler flow imaging. Ultrasonographic biomicroscopy demonstrated medium-low internal reflectivity and acoustic attenuation. In magnetic resonance imaging (MRI), the tumours as compared to brain were hyperintense on T1-weighted images and hypointense on T2-weighted images. Tumour tissue consisted of cords and nests of pigment epithelium cells separated by septa of vascularized fibrous connective tissue, leading to a pseudo-glandular appearance. The melanin granules in the cytoplasm were large and mostly spherical in shape. In four patients, the tumours were hyperpigmented. Tumour cells were large with round or oval nuclei and clearly detectable nucleoli. CONCLUSIONS: These clinical characteristics of CPE adenomas, such as homogenous dark brown colour, smooth surface, iris dislocation and anterior chamber angle narrowing but no iris infiltration, segmental cataract, pigment dispersion, and, as compared to brain tissue, hypointensity and, as compared to extraocular muscles or lacrimal gland, hyperintensity on T2-weighted MRI images, may be helpful for the differentiation from ciliary body malignant melanomas.

Role of quantitative magnetic resonance imaging parameters in the evaluation of treatment response in malignant tumors.

OBJECTIVE: To elaborate the role of quantitative magnetic resonance imaging (MRI) parameters in the evaluation of treatment response in malignant tumors. DATA SOURCES: Data cited in this review were obtained mainly from PubMed in English from 1999 to 2014, with keywords "dynamic contrast-enhanced (DCE)-MRI," "diffusion-weighted imaging (DWI)," "microcirculation," "apparent diffusion coefficient (ADC)," "treatment response" and "oncology." STUDY SELECTION: Articles regarding principles of DCE-MRI, principles of DWI, clinical applications as well as opportunity and aspiration were identified, retrieved and reviewed. RESULTS: A significant correlation between ADC values and treatment response was reported in most DWI studies. Most quantitative DCE-MRI studies showed a significant correlation between K trans values and treatment response. However, in different tumors and studies, both high and low pretreatment ADC or K trans values were found to be associated with response rate. Both DCE-MRI and DWI demonstrated changes in their parameters hours to days after treatment, showing a decrease in K trans or an increase in ADC associated with response in most cases. CONCLUSIONS: Combinations of quantitative MRI play an important role in the evaluation of treatment response of malignant tumors and hold promise for use as a cancer treatment response biomarker. However, validation is hampered by the lack of reproducibility and standardization. MRI acquisition protocols and quantitative image analysis approaches should be properly addressed prior to further testing the clinical use of quantitative MRI parameters in the assessment of treatments.

Improved performance in differentiating benign from malignant sinonasal tumors using diffusion-weighted combined with dynamic contrast-enhanced magnetic resonance imaging.

BACKGROUND: Differentiating benign from malignant sinonsal lesions is essential for treatment planning as well as determining the patient's prognosis, but the differentiation is often difficult in clinical practice. The study aimed to determine whether the combination of diffusion-weighted (DW) and dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) can improve the performance in differentiating benign from malignant sinonasal tumors. METHODS: This retrospective study included 197 consecutive patients with sinonasal tumors (116 malignant tumors and 81 benign tumors). All patients underwent both DW and DCE-MRI in a 3-T magnetic resonance scanner. Two different settings of b values (0,700 and 0,1000 s/mm 2 ) and two different strategies of region of interest (ROI) including whole slice (WS) and partial slice (PS) were used to calculate apparent diffusion coefficients (ADCs). A DW parameter with WS ADCs b0,1000 and two DCE-MRI parameters (time intensity curve [TIC] and time to peak enhancement [Tpeak]) were finally combined to use in differentiating the benign from the malignant tumors in this study. RESULTS: The mean ADCs of malignant sinonasal tumors (WS ADCs b0,1000 = 1.084 × 10-3 mm 2 /s) were significantly lower than those of benign tumors (WS ADCs b0,1000 = 1.617 × 10-3 mm 2 /s, P < 0.001). The accuracy using WS ADCs b0,1000 alone was 83.7% in differentiating the benign from the malignant tumors (85.3% sensitivity, 81.2% specificity, 86.4% positive predictive value [PPV], and 79.5% negative predictive value [NPV]). The accuracy using DCE with Tpeak and TIC alone was 72.1% (69.1% sensitivity, 74.1% specificity, 77.5% PPV, and 65.1% NPV). Using DW-MRI parameter was superior than using DCE parameters in differentiation between benign and malignant sinonasal tumors (P < 0.001). The accuracy was 87.3% (90.5% sensitivity, 82.7% specificity, 88.2% PPV, and 85.9% NPV) using DW-MRI combined with DCE-MRI, which was superior than that using DCE-MRI alone or using DW-MRI alone (both P < 0.001) in differentiating the benign from the malignant tumors. CONCLUSIONS: Diffusion-weighted combined with DCE-MRI can improve imaging performance in differentiating benign from malignant sinonasal tumors, which has the potential to improve diagnostic accuracy and to provide added value in the management for these tumors.

Magnetic resonance imaging findings in sporadic Möbius syndrome.

BACKGROUND: Although neuroradiological findings of Möbius syndrome have been reported as a result of brain and brainstem abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of the cranial nerve (CN) and branches in the orbits. This study presents the MRI findings in patients with sporadic Möbius syndrome. METHODS: Prospectively, CNs were imaged in the cistern using head coils and three dimensional fast imaging employing steady-state acquisition (3D-FIESTA), yielding a 0.5 mm(2) resolution in planes of 0.8 mm thickness in seven patients with sporadic Möbius syndrome. The cavernous and intraorbital segment of the CN and the extraocular muscles (EOMs) were imaged with T1 weighting in all patients. The cavernous segment was imaged in coronal planes, while the intraorbit in quasicoronal planes were imaged using surface coils. Intraorbital resolution was 0.16 mm(2) within 2.0 mm thick planes. RESULTS: In the seven patients, the CN were absent or showed hypoplasia in the cistern, cavernous sinus, and orbit. Abducens (CN VI) and facial (CN VII) nerves were absent on the affected sides. Unilateral CN IX (glossopharyngeal nerve) in two cases displayed dysplasia. Branches from the inferior division of CN III were observed to innervate the lateral rectus (LR) bilaterally in three cases and unilaterally in one case, and had intimate continuity with the LR muscle in two cases bilaterally and two cases unilaterally. Hypoplasia of EOMs was shown in five cases. Dysplasia of the medulla on the left side was found in one patient. CONCLUSIONS: Direct imaging of CNs and EOMs by MRI is useful in diagnosis of Möbius syndrome. It can directly demonstrate the abnormalities of the CN and orbital structures. The absence or hypoplasia of CN VI and CN VII may be the most common radiologic features in sporadic Möbius syndrome, and hypoplasia of CN IX may be an associated feature. The abnormality of EOMs and aberrant innervations in the orbit should be observed, and may be important for the study of the etiology.

[Magnetic resonance imaging findings of orbital disease in neurofibromatosis type 1 patients].

OBJECTIVE: To explore the magnetic resonance imaging (MRI) features of neurofibromatosis type 1 (NF1) with orbital involvement. METHODS: We retrospectively reviewed the MRI findings of orbital abnormalities in 80 NF1 patients (86 orbits). The diagnosis was confirmed by the updated National Institute of Health (NIH) criteria. All of them underwent MRI scans of orbit or brain while 71 patients had contrast enhancement. RESULTS: The orbital abnormalities were documented in 80 patients. And 79 cases (85 orbits) had plexiform neurofibroma in orbit and/or adjacent regions. The orbital involvements extended to superficial temporal fossa (n = 61), infratemporal fossa (n = 31), cavernous sinus (n = 61) and pterygopalatine fossa (n = 51). There was mild-to-moderate enhancement in 71 orbits on contrast-enhanced MRI examinations. There were localized defects of orbital walls because of sphenoid bone dysplasia of greater wing (n = 79), sphenoid bone dysplasia of lesser wing (n = 72), augmentation (glaucoma) of eyeballs (n = 32) and optic nerve gliomas (n = 5). And 53 cases had hyperintense lesions on T2-weighted brain images. CONCLUSION: Orbital involvement is common for NF1. And MRI is a useful tool of evaluating the extent of involvements and other abnormalities in the NF1 patients.

Characteristic findings of malignant melanoma in the sinonasal cavity on magnetic resonance imaging.

BACKGROUND: Malignant sinonasal melanoma (MSM) is a rare tumor with a perplexing signal intensity due to variable histopathologic components. This study was undertaken to delineate its MR imaging features. METHODS: MR imaging findings of 10 patients (6 women and 4 men, mean age 61.3 years old) with pathologically confirmed MSM were retrospectively reviewed. The location, size, signal intensity, enhancement, and internal imaging characteristics of all tumors were evaluated. Signal intensity and degree of enhancement was graded in comparison with the gray matter and adjacent muscle uptake, respectively. RESULTS: There were 8 tumors that were pathologically confirmed to contain melanin. Compared to gray matter of the brain, 7 of them demonstrated hyperintensity on T1WI and 6 (6/7) showed hypointensity on T2WI. There was multiple linear, dark-signal intensity on T2WI within the mass in 9 of the 10 patients' tumors. Evaluated with gadolinium-enhanced imaging, all 10 patients showed moderate enhancement within the areas that were isointense in the lesion on pregadolinium T1WI. Moreover, some parts which displayed hyperintensity on T1WI within the tumors of 7 patients showed mild enhancement that was similar to muscle on a time-intensity curve (TIC). CONCLUSIONS: MSM shows characteristic MR signal intensity (hyperintensity on T1WI and the linear, low-signal intensity on T2WI), which may provide valuable information for clinical diagnosis. Together with conventional MRI, TIC may be useful for indicating pleomorphic patterns of MSM.

[Study of variations in adult sphenoid sinus by multislice spiral computed tomography].

OBJECTIVE: Explore the anatomic variations of adult sphenoid sinus and delineate the precise relationship between sphenoid sinus and adjacent structures. METHODS: Using multi planner reformation (MPR), the images of 260 spiral computed tomography (CT) scans were reviewed through a doctor station. RESULTS: The sphenoid was divided into 6 types: no development, conchal, pre-sellar, half-sellar, full-sellar and post-sellar. The prevalence was 0.19%, 1.54%, 8.08%, 22.88%, 20.58% and 46.73% respectively. And there was no difference between left and right. The prevalence of accessory septa or bone spur inside sphenoid sinus was 51.5%. With the gasification spreading, the prevalence was rising. And there was statistical significance among them. The dorsum sella was divided into three types: I, II, III. And its prevalence was 48.08%, 25.19% and 26.73% respectively. The difference of sphenoethmoid distribution among the various type sinus had no statistical significance. The prevalence of vidian canal and foramen rotundum protrusion was 39.2% and 15.8% respectively. And all occurred in pterygoid process cells. Internal carotid artery (ICA) was divided into 4 types. And the prevalence of type 0 to 3 was 13.5%, 50.0%, 26.9% and 9.6% respectively. With the gasification spreading, the prevalence of types 2, 3 was rising. And there were statistical significance among them. The prevalence of type 0 to 4 CNII was 4.4%, 19.2%, 26.0%, 29.0% and 21.4% respectively. With the gasification spreading, the prevalence of type 3, 4 CNII was rising. And the difference had statistical significance. CONCLUSION: MPR and multislice spiral CT can facilitate a precise study of anatomic variations in adult sphenoid sinus and delineate the relationships between sphenoid sinus and adjacent structures. Caution must be exercised during sphenoid and trans-sphenoid surgery to minimize the risk of inadvertently injuring the adjacent structures.

[Comparison of sensitivity of audiological tests to identify otitis media with effusion in newborn infants].

OBJECTIVE: To investigate the sensitive factors which were used in routine audiological tests to find out otitis media with effusion (OME) in newborn infants. METHODS: Subjects of this study were 48 infants, including 31 males and 17 females, who failed in the universal newborn hearing screening. The age ranged from 1.5 to 12 months with the average age of 4.3 months. All subjects accepted temporal bone CT and routine audiological assessments, including air-conduction and bone-conduction auditory brainstem response (ABR), 40 Hz-auditory event related potential (40 Hz-AERP), distortion-product otoacoustic emission (DPOAE), acoustic reflex, tympanometries using 226 Hz and 1000 Hz probe tone. Nine factors were statistically analyzed using Kappa test, Univariate chi(2) test and multivariate condition Logistic stepwise regression analysis, which included the results of acoustic immittance, the air-conduction and bone-conduction ABR thresholds, the difference between air-conduction and bone-conduction ABR thresholds, the latency of ABR wave I, duration between ABR wave I and V, 40 Hz-AERP thresholds, amplitudes and thresholds of DPOAE, and acoustic reflex thresholds (ART). RESULTS: Seventy-seven ears were diagnosed with OME, and 19 ears were normal. CT scan of temporal bone was set as a comparative standard. Kappa test indicated that the results of tympanometry with 1000 Hz probe tone (Kappa = 0.745, P < 0.001), the air-conduction ABR threshold (Kappa = 0.453, P < 0.001), the latency of ABR wave I (Kappa = 0.430, P < 0.001), the threshold of 40 Hz-AERP (Kappa = 0.582, P < 0.001), and DPOAE (Kappa = 0.495, P < 0.001) had agreement with the results of temporal bone CT on evaluating the function of middle ear. Univariate analysis indicated that sensitive factors of middle ear function in newborn infants were tympanometry with 1000 Hz probe tone (P < 0.001), ART (P < 0.001), the air-conduction ABR threshold (P < 0.001), the difference between air-conduction and bone-conduction ABR thresholds (P < 0.001), the latency of ABR wave I (P < 0.001), the threshold of 40 Hz-AERP (P < 0.001) and DPOAE (P < 0.001). And multivariate conditional Logistic stepwise regression model showed that tympanometry with 1000 Hz probe tone (P < 0.001) and 40 Hz-AERP threshold (P = 0.004) can be substituted into Logistic stepwise regression equation. CONCLUSIONS: Tympanometry with 1000 Hz probe tone and are sensitive factors to find out OME in newborn infants. The air conduction ABR threshold, ABR wave I latency, 40 Hz-AERP threshold and DPOAE could reflect the middle ear function of newborn infants effectively.

[Endoscopic transpterygoid intervention of meningoencephalocele within lateral recess of sphenoid].

OBJECTIVE: To report the results of endoscopic transpterygoid intervention of nine patients with meningoencephalocele and cerebrospinal fluid (CSF) leaks within lateral recess of sphenoid sinus (LRSS). The diagnosis, operative techniques and their related problems were discussed. METHODS: Nine hospitalized patients with meningoencephalocele and CSF leaks within lateral recess of sphenoid sinus (LRSS) were included in this paper. Six were male and 3 were female, aged from 27 to 56 years old. Two patients had the histories of endoscopic repair. The preoperative orientation of CSF leaks and meningoencephalocele depended on CT scan and MR cisternography. Endoscopic transpterygoid intervention and the repair of skull base defects were undertaken under general anesthesia. RESULTS: All the operations were successful. One patient had a postoperative intracranial hypertension and Hydrocephalus. Two patients had postoperative ipsilateral facial, upper lip and palatal hypesthesia. One of them had a xerophthalmia. All the symptoms above mentioned relieved gradually 6 months after operation. No recurrence was found during follow -up for 6 to 58 months (mean 25. 6 months). CONCLUSIONS: Endoscopic transpterygoid intervention for meningoencephalocele and CSF leaks within LRSS is a minimally invasive technique and a straightforward approach.

[Spiral computed tomographic analysis of frontal recess regions].

OBJECTIVE: The purpose of the study was to determine the prevalence of frontal recess cells in Chinese patients who did not have frontal sinus disease related symptoms. METHODS: Forty-nine Chinese patients without frontal sinus disease symptoms were undergone spiral computed tomography (CT). Then multiplanar reconstruction images were evaluated using a standard triplanar reconstruction protocol on a computer workstation. RESULTS: The prevalence of agge rnasi cell was 94% (92/98). Sixty-four uncinate processes (65%, 64/98) had one superior attachment for each uncinate process, the other thirty-four uncinate processes (35%, 34/98) had two superior attachments for each uncinate process. The uncinate process' single superior attachment into the surrounding structures was identified to have the following distribution: 53% (52/98) to the lamina papyracea, 9% (9/98) to the middle turbinate, 3% (3/98) to the skull base. Most of the uncinate process' two superior attachments were either into the lamina papyracea and the skull base (24%, 23/98) or into the lamina papyracea and the middle turbinate (10%, 10/98). Only one uncinate process (1%) superiorly attached to the skull base and the middle turbinate. The prevalence of recessus terminalis was 87% (85/98). Of all the frontal cells identified in 32 sides (33%) of frontal recesses, the prevalence of type I, type II, type II and type IV cells were 23% (23 sides), 2% (2 sides), 7% (7 sides) and 0% (0 side) respectively. Supra bullar cell, frontal bullar cell and interfrontal septal cell were identified in 30 sides (31%), 7 sides (7%) and 7 patients (14%) respectively. CONCLUSIONS: The result characterized normal frontal recess pneumatization in Chinese. That, together with the variations of the uncinate process' superior attachment emphasized the roles of agger nasi cell and the uncinate process in endoscopic frontal sinus surgery.

[Characteristics of computed tomography of allergic fungal sinusitis].

OBJECTIVE: To sum up the characteristics of computed tomography of allergic fungal sinusitis (AFS) and to compare the CT changes with the findings in the operation. The diagnostic role of CT scan was discussed. METHODS: The CT scans of 21 patients diagnosed as AFS were analyzed. The patients ranged from 15 to 50 years old,there were 17 males and 4 females. The CT was scanned with both bony and soft tissue windows. The preoperative examinations included nasal endoscopy, skin prick test, total serum IgE and nasal secretion smear. The findings in the operation were compared with the preoperative CT scans. Histopathology and fungal smear were done postoperatively. RESULTS: Endoscopy showed that all patients had polyps with wasfy yellow or inspissated white secretion. Nine cases (11 sides) showed yellow-to-brown material similar to peanut butter in the nasal cavities. Nasal CT scan demonstrated unilateral lesion in 10 cases (10 sides) and bilateral lesions in 11 cases (22 sides), who were all pansinus diseases. CT scan demonstrated a sheet areas of high-attenuation like "ground glass" within sinuses coupled with soft tissue image around them. Bone erosion of anterior skull base was encountered in 3 cases (4 sides). One case showed the intracranial extension. Twenty cases had conceived nasal endoscopic sinus surgeries. One case underwent endoscopic sinus surgery combined with coronal approach. The yellow-to-brown material (allergic mucin) was detected in the cavities of 17 cases. Four cases had green-to-brown secretion like mud. After follow-up of 6 months to 7 years, 14 cases were cured, 7 cases improved. Among them, 3 cases had to get revision surgeries because of recurrence 2 years after surgery. CONCLUSIONS: The characteristic of a sheet area of high-attenuation like " ground glass" within sinuses coupled with soft tissue image around them in computed tomography of nasal sinus, accompanied with invasive expansion or bone erosion, has a diagnostic significance for AFS.

[Anatomical and computed tomographic analysis of the interaction between uncinate process and agger nasi cells].

OBJECTIVE: To investigate the anatomical interaction between uncinate process and agger nasi cell to better understand the anatomy of the frontal sinus drainage pathway by endoscopy, spiral computed tomography (CT) and sectioning. METHODS: Twenty-one skeletal skulls (forty-two sides) and one cadaver head (two sides) were studied by spiral CT together with endoscopy and collodion embedded thin sectioning at coronal plane. The sections with the thickness of 100 microm were stained with hemotoxylin and eosin. RESULTS: Under endoscopy, a leaflet of bone to the middle turbinate, which is given off by uncinate process, forms the anterior insertion of the middle turbinate onto the lateral nasal wall. The middle portion of the uncinate process attached to the frontal process of the maxilla in all of the skeletal nasal cavities, as well as the lacrimal bone in 78.6% of the skeletal nasal cavities. On CT scans, the agger nasi cell is present in 90.5% of the skeletal nasal cavities. While the lateral wall of the agger nasi cell is formed by lacrimal bone, the medial wall of the agger nasi cell is formed by uncinate process. And the anterior wall is formed by the frontal process of the maxilla. The superior portion of the uncinate process forms the medial, posterior and top wall of the agger nasi cells. The superior portion of the uncinate extends into the frontal recess and may insert into lamina papyracea (33.3%), skull base (9.5%), middle turbinate, combination of these (57.2%). CONCLUSIONS: The agger nasi cell is the key that unlocks the frontal recess.