MRI Evaluation of the Normal and Abnormal Endolymphatic Duct in the Pediatric Population: A Comparison with High-Resolution CT.

BACKGROUND AND PURPOSE: An enlarged vestibular aqueduct is the most commonly reported imaging abnormality in children with sensorineural hearing loss. MR imaging is often used to evaluate pediatric sensorineural hearing loss; however, there are no well-established size criteria on MR imaging to diagnose an enlarged endolymphatic duct. The first purpose of the study was to determine a range of normal endolymphatic duct sizes on MR imaging and compare it with that in high-resolution CT. The second purpose was to assess the sensitivity and specificity of MR imaging in diagnosing an enlarged endolymphatic duct in patients with an enlarged vestibular aqueduct on CT. MATERIALS AND METHODS: Endolymphatic duct midaperture measurements were analyzed in 52 patients with no history of sensorineural hearing loss. Comparison of CT and MR imaging was made in a second cohort of 41 patients with a normal midaperture width on CT. The sensitivity and specificity of MR imaging were then evaluated in a third cohort of 24 patients with a documented enlarged vestibular aqueduct on CT. RESULTS: In 94 ears, normal endolymphatic duct midaperture measurements ranged from 0 to 0.9 mm on MR imaging. A significant correlation (P <.001) and moderate agreement were found between CT and MR imaging in 81 ears with a normal vestibular aqueduct on CT. Twenty-four patients had bilateral (n = 14) or unilateral (n = 10) enlarged vestibular aqueducts on CT, and the sensitivity and specificity of MR imaging were 97% and 100%, respectively, for a diagnosis of an enlarged endolymphatic duct. CONCLUSIONS: MR imaging measurements of the normal endolymphatic duct are similar to those established for CT. MR imaging is a useful tool for the diagnosis of enlarged vestibular aqueduct.

Evaluation of the Normal Cochlear Second Interscalar Ridge Angle and Depth on 3D T2-Weighted Images: A Tool for the Diagnosis of Scala Communis and Incomplete Partition Type II.

BACKGROUND AND PURPOSE: Cochlear malformations may be be subtle on imaging studies. The purpose of this study was to evaluate the angle and depth of the lateral second interscalar ridge or notch in ears without sensorineural hearing loss (normal ears) and compare them with ears that have a documented incomplete type II partition malformation. MATERIALS AND METHODS: The second interscalar ridge notch angle and depth were measured on MR imaging in normal ears by a single experienced neuroradiologist. The images of normal and incomplete partition II malformation ears were then randomly mixed for 2 novice evaluators to measure both the second interscalar ridge notch angle and depth in a blinded manner. For the mixed group, interobserver agreement was calculated, normal and abnormal ear measurements were compared, and receiver operating characteristic curves were generated. RESULTS: The 94 normal ears had a mean second interscalar ridge angle of 80.86° ± 11.4° and depth of 0.54 ± 0.14 mm with the 98th percentile for an angle of 101° and a depth of 0.3 mm. In the mixed group, agreement between the 2 readers was excellent, with significant differences for angle and depth found between normal and incomplete partition type II ears for angle and depth on average (P < .001). Receiver operating characteristic cutoffs for delineating normal from abnormal ears were similar for both readers (depth, 0.31/0.34 mm; angle, 114°/104°). CONCLUSIONS: A measured angle of >114° and a depth of the second interscalar ridge notch of ≤0.31 mm suggest the diagnosis of incomplete partition type II malformation and scala communis. These measurements can be accurately made by novice readers.

MR Imaging of the Cervical Spine in Nonaccidental Trauma: A Tertiary Institution Experience.

BACKGROUND AND PURPOSE: Cervical MR imaging has demonstrated a utility for detecting soft tissue injury in nonaccidental trauma. The purpose of this study was to identify the incidence and types of cervical spine injury on MR imaging in nonaccidental trauma and to correlate cervical spine injury with parenchymal injury on brain MR imaging and findings on head CT. MATERIALS AND METHODS: A retrospective review of children diagnosed with nonaccidental trauma in a tertiary referral pediatric hospital over 8 years was performed. Inclusion criteria were children younger than 5 years of age, a confirmed diagnosis of nonaccidental trauma, and cervical spine MR imaging within 1 week of presentation. Brain and cervical spine MR imaging, head CT, cervical radiographs, and skeletal surveys were reviewed. RESULTS: There were 89 patients included in this study (48 males; mean age, 9.1 months [range, 1-59 months]). Cervical spine injury on MR imaging was found in 61 patients (69%). Ligamentous injury was seen in 60 patients (67%), with interspinous ligaments being most commonly involved. Abnormal capsular fluid (atlanto-occipital and atlantoaxial) was present in 28 patients (32%). Cervical spine injury on MR imaging was significantly associated with parenchymal restricted diffusion on brain MR imaging and parenchymal injury on head CT (P = .0004 and P = .0104, respectively). Children with restricted diffusion on brain MR imaging were 6.22 (point estimate) times more likely to have cervical spine injury on MR imaging. CONCLUSIONS: There is a high incidence of cervical spine injury in pediatric nonaccidental trauma. Positive findings may affect management and suggest a traumatic etiology.

Spectrum of Clinical and Associated MR Imaging Findings in Children with Olfactory Anomalies.

BACKGROUND AND PURPOSE: The olfactory apparatus, consisting of the bulb and tract, is readily identifiable on MR imaging. Anomalous development of the olfactory apparatus may be the harbinger of anomalies of the secondary olfactory cortex and associated structures. We report a large single-site series of associated MR imaging findings in patients with olfactory anomalies. MATERIALS AND METHODS: A retrospective search of radiologic reports (2010 through 2014) was performed by using the keyword "olfactory"; MR imaging studies were reviewed for olfactory anomalies and intracranial and skull base malformations. Medical records were reviewed for clinical symptoms, neuroendocrine dysfunction, syndromic associations, and genetics. RESULTS: We identified 41 patients with olfactory anomalies (range, 0.03-18 years of age; M/F ratio, 19:22); olfactory anomalies were bilateral in 31 of 41 patients (76%) and absent olfactory bulbs and olfactory tracts were found in 56 of 82 (68%). Developmental delay was found in 24 (59%), and seizures, in 14 (34%). Pituitary dysfunction was present in 14 (34%), 8 had panhypopituitarism, and 2 had isolated hypogonadotropic hypogonadism. CNS anomalies, seen in 95% of patients, included hippocampal dysplasia in 26, cortical malformations in 15, malformed corpus callosum in 10, and optic pathway hypoplasia in 12. Infratentorial anomalies were seen in 15 (37%) patients and included an abnormal brain stem in 9 and an abnormal cerebellum in 3. Four patients had an abnormal membranous labyrinth. Genetic testing was performed in 23 (56%) and findings were abnormal in 11 (48%). CONCLUSIONS: Olfactory anomalies should prompt careful screening of the brain, skull base, and the pituitary gland for additional anomalies. Genetic testing should be considered.

High-resolution CT findings in children with a normal pinna or grade I microtia and unilateral mild stenosis of the external auditory canal.

SUMMARY: A subset of patients presents with unilateral conductive hearing loss, a normal pinna or grade I microtia, and mild external auditory canal stenosis. The physical findings of microtia and a small external canal are commonly absent or subtle in this group of patients, who are being commonly referred for imaging to evaluate isolated conductive hearing loss. We present a case series of patients with unilateral conductive hearing loss and characteristic ossicular abnormalities, commonly anterior fixation of the malleus. All patients had a significantly increased distance from the cochlear promontory to the handle of the malleus and an abnormal incudostapedial angle, indicative of an abnormal ossicular position and/or morphology. Successful surgical reconstruction of the ossicular chain was attempted and accomplished in 3 patients.

Hypomagnesemia secondary to cerebrospinal fluid losses in a patient with congenital hydrocephalus.

We describe a newborn infant with massive congenital hydrocephalus, presenting with hypomagnesemia secondary to magnesium losses through cerebrospinal fluid (CSF) aspirations. Hypomagnesemia due to CSF losses has not been described in pediatric literature.

Isolated cerebellar edema and obstructive hydrocephalus in a child with cerebral malaria.

An important complication of cerebral malaria is increased intracranial pressure which, when severe, is associated with high mortality or neurologic sequelae. We describe a 7-month-old child with cerebral malaria for whom cerebellar edema and obstructive hydrocephalus were initial radiologic findings. Despite significant hydrocephalus, the child had normal intracranial pressure during the course of the infection, and he recovered with minimal sequelae.

MR imaging of the corpus callosum in pediatric patients with neurofibromatosis type 1.

BACKGROUND AND PURPOSE: Many pediatric patients with neurofibromatosis type 1 (NF-1) have an apparent increased thickness of the corpus callosum (CC) on sagittal T1-weighted images compared with patients not affected by NF-1. In this study, we compared the surface area of the CC in children with NF-1 with that of healthy pediatric control subjects to determine if this was another common intracranial manifestation of NF-1. METHODS: Midsagittal T1-weighted MR images of 43 consecutive children with NF-1 and 43 age- and gender-matched healthy control subjects were reviewed retrospectively. The surface area of the CC and the midsagittal intracranial skull surface (MISS) area were measured five times each on all midsagittal images. A mean CC to mean midline intracranial surface area ratio (CC/MISS) was calculated for each. RESULTS: There is a statistically significant increase in the mean CC surface area in pediatric patients with NF-1 (680 mm2 +/- 98, range 509-974 mm2) compared with control subjects (573 mm2 +/- 83, range 404-797 mm2). The mean MISS is significantly increased in patients with NF-1 (16568 mm2 +/- 1161, range 14107-19394 mm2 vs 15402 mm2 +/- 1133, range 12951-17905 mm2 for control subjects). CC/MISS was also significantly increased in the patients with NF-1 relative to the control subjects (.0410 +/- .0043, range .0330-.0530 vs .0372 +/- .0043, range .0270-.0470 for control subjects). CONCLUSION: A larger midsagittal surface area of the CC is another intracranial manifestation of NF-1 that can be demonstrated by sagittal MR imaging. The etiology is unclear, but could be related to abnormal neurofibromin and Ras protein activity. Potential clinical relevance is discussed herein.

Midface anomalies in children.

A variety of congenital midface anomalies occur in children. High-resolution computed tomography (CT) and magnetic resonance (MR) imaging have proved helpful in determining the nature and extent of dysplasia, thereby facilitating treatment planning. A classification system has been developed that groups these anomalies into four categories based on embryogenesis and anatomic location. These categories comprise anomalies that are related to the nasal cavity, nasofrontal region, nasolacrimal apparatus, and craniofacial syndromes. CT is the imaging modality of choice in children with possible choanal atresia, pyriform aperture stenosis, or anomalies of the nasolacrimal duct (eg, nasolacrimal duct stenosis, dacryocystoceles). MR imaging is the modality of choice in patients with congenital midface masses (eg, dermoid and epidermoid cysts, nasal gliomas, encephaloceles) and craniofacial syndromes (eg, Apert syndrome, Crouzon syndrome, Treacher Collins syndrome). In many cases, however, both CT and MR imaging are required to adequately evaluate midface anomalies. Familiarity with the characteristic imaging features of these anomalies along with knowledge of midface embryogenesis and normal developmental anatomy is essential to prevent misinterpretation of anatomic variations that may simulate disease.

Imaging and clinical evaluation of isolated atresia of the oval window.

BACKGROUND AND PURPOSE: Congenital causes of hearing loss in children commonly are encountered, and imaging aids in diagnosis as well as presurgical evaluation. Atresia of the oval window not associated with atresia of the external auditory canal (EAC) is a rare cause of congenital hearing loss in children. We present the clinical and imaging findings in children with isolated oval-window atresia. METHODS: Atresia of the oval window was defined as the absence of the structure with the presence of a bony plate superimposed between the vestibule and middle ear. The bony plate is within the expected region of the oval window. Using a computerized database, nine patients with isolated oval-window atresia were found. All had been evaluated with high-resolution computed tomography (HRCT) and all had medical records available for review, including audiogram results. Imaging studies were interpreted by the consensus of two pediatric neuroradiologists. RESULTS: Atresia of the oval window was documented in all cases using HRCT criteria. The most common anomalies associated with oval-window atresia were inferomedial malposition of the facial nerve (n = 8), malformed incus (n = 6), and displaced stapes (n = 2). Four patients had symmetric bilateral involvement. Hearing tests were not specific, because conductive, sensorineural, and mixed patterns were found. CONCLUSION: Anomalies of the oval window should be sought in all patients with congenital hearing loss. Associated findings, such as facial nerve aberrancy and ossicular anomalies, are important in both diagnosis and surgical planning.

Lymphatic air embolism: a new hypothesis regarding the pathogenesis of neonatal systemic air embolism.

Objective. Neonatal systemic air embolism (NSAE) has been thought to result from introduction of air into the pulmonary veins through hypothesized alveolar-capillary fistula. The objective of this paper is to reassess the distribution of intravascular air visualized radiographically in this entity. Based on these data, an alternative theory for the pathogenesis of NSAE is proposed. Materials and methods. Four cases from our institutions and 21 additional reviewable published radiographs of NSAE were evaluated for the presence and location of intravascular air. Nonparametric statistical analysis was performed to determine if the predominance of intravascular air was venous or arterial in location, and to determine the presence or absence of pulmonary interstitial emphysema (PIE), pneumothorax, pneumomediastinum, and pneumopericardium. Results. Isolated systemic venous air was present significantly more often than isolated systemic arterial air (p < 0.0005). In addition, the presence of isolated right-sided cardiac air was found significantly more often than air within left heart chambers alone (p < 0.0005). PIE in patients with NSAE was found to be statistically more prevalent than pneumomediastinum or pneumopericardium, though similar in prevalence to pneumothorax. Conclusion. Our data support the hypothesis that intravascular air in NSAE is predominantly venous in location. PIE was found to be a very common associated finding in NSAE. Based on our current knowledge of pulmonary lymphatics, radiographic anatomy, and the lymphatic location of PIE, we propose that air within the pulmonary lymphatic system (PIE) gains access to the systemic venous system via lymphatic ducts, which results in the clinical entity NSAE. This readily explains the venous predominance of air in NSAE.