Surgery and proton radiation therapy for pediatric base of skull chordomas: Long-term clinical outcomes for 204 patients.

BACKGROUND: Data on clinical outcomes for base of skull (BOS) chordomas in the pediatric population is limited. We report patient outcomes after surgery and proton radiotherapy (PRT). METHODS: Pediatric patients with BOS chordomas were treated with PRT or combined proton/photon approach (proton-based; for most, 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Endpoints of interest were overall survival (OS), disease-specific survival, progression-free survival (PFS), freedom from local recurrence (LC), and freedom from distant failure (DC). RESULTS: Of 204 patients, median age at diagnosis was 11.1 years (range, 1-21). Chordoma location included 59% upper and/or middle clivus, 36% lower clivus, 4% craniocervical junction, and 1% nasal cavity. Fifteen (7%) received pre-RT chemotherapy. Forty-seven (23%) received PRT, and 157 (77%) received comboRT. Median total dose was 76.7 Gy (RBE) (range, 59.3-83.3). At a median follow-up of 10 years (interquartile range, 5-16 years), 56 recurred. Median OS and PFS were 26 and 25 years, with 5-, 10-, and 20-year OS and PFS rates of 84% and 74%, 78% and 69%, and 64% and 64%, respectively. Multivariable actuarial analyses showed poorly differentiated subtype, radiographical progression prior to RT, larger treatment volume, and lower clivus location to be prognostic factors for worse OS, PFS, and LC. RT was well tolerated at a median follow-up of 9 years (interquartile range, 4-16 years). Side effects included 166 patients (80%) with mild/moderate acute toxicities, 24 (12%) patients with late toxicities, and 4 (2%) who developed secondary radiation-related malignancies. CONCLUSION: This is the largest cohort of BOS chordomas in the literature, pediatric and/or adult. High-dose PRT following surgical resection is effective with low rates of late toxicity.

Anatomy of the Orbit.

Each orbit is a complex structure housing the globe, multiple cranial nerves, muscles, vascular structures, which support the visual sense. Many of these structures have been delineated in careful detail by anatomists but remain beyond the resolution of conventional imaging techniques. With the advances of higher resolution MR, surface coil usage, and thinner section computed tomographic images, the ability to resolve these small structures continues to improve, allowing radiologists to provide more detailed anatomic descriptions for preoperative and pretreatment planning.

Radiofrequency ablation and related ultrasound-guided ablation technologies for treatment of benign and malignant thyroid disease: An international multidisciplinary consensus statement of the American Head and Neck Society Endocrine Surgery Section with the Asia Pacific Society of Thyroid Surgery, Associazione Medici Endocrinologi, British Association of Endocrine and Thyroid Surgeons, European Thyroid Association, Italian Society of Endocrine Surgery Units, Korean Society of Thyroid Radiology, Latin American Thyroid Society, and Thyroid Nodules Therapies Association.

BACKGROUND: The use of ultrasound-guided ablation procedures to treat both benign and malignant thyroid conditions is gaining increasing interest. This document has been developed as an international interdisciplinary evidence-based statement with a primary focus on radiofrequency ablation and is intended to serve as a manual for best practice application of ablation technologies. METHODS: A comprehensive literature review was conducted to guide statement development and generation of best practice recommendations. Modified Delphi method was applied to assess whether statements met consensus among the entire author panel. RESULTS: A review of the current state of ultrasound-guided ablation procedures for the treatment of benign and malignant thyroid conditions is presented. Eighteen best practice recommendations in topic areas of preprocedural evaluation, technique, postprocedural management, efficacy, potential complications, and implementation are provided. CONCLUSIONS: As ultrasound-guided ablation procedures are increasingly utilized in benign and malignant thyroid disease, evidence-based and thoughtful application of best practices is warranted.

Does 3-T fetal MRI induce adverse acoustic effects in the neonate? A preliminary study comparing postnatal auditory test performance of fetuses scanned at 1.5 and 3 T.

BACKGROUND: Fetal MRI at 3 T is associated with increased acoustic noise relative to 1.5 T. OBJECTIVE: The goal of this study is to determine if there is an increased prevalence of congenital hearing loss in neonates who had a 3-T prenatal MR vs. those who had it at 1.5 T. MATERIALS AND METHODS: We retrospectively identified all subjects who had 3-T fetal MRI between 2012 and 2016 and also underwent universal neonatal hearing screening within 60 days of birth. Fetuses with incomplete hearing screening, magnetic resonance imaging (MRI) studies at both field strengths or fetuses affected by conditions associated with hearing loss were excluded. A random group of controls scanned at 1.5 T was identified. Five subjects had repeat same-strength MRIs (one at 3 T and four at 1.5 T). The pass/fail rate of the transient otoacoustic emissions test and auditory brainstem response test were compared using the Fisher exact test. A logistic regression was performed to assess the effects of other known risk factors for congenital hearing loss. RESULTS: Three hundred forty fetal MRI examinations were performed at 3 T, of which 62 met inclusion criteria. A control population of 1.5-T fetal MRI patients was created using the same exclusion criteria, with 62 patients randomly selected from the eligible population. The fail rates of transient otoacoustic emissions test for the 1.5-T and 3-T groups were 9.7% and 6.5%, respectively, and for the auditory brainstem response test were 3.2% and 1.6%, respectively. There was no significant difference in the fail rate of either test between groups (P=0.74 for transient otoacoustic emissions test, and P=0.8 for auditory brainstem response test). The median gestational age of the 3-T group was 30 weeks, 1 day, significantly higher (P<0.001) than the 1.5-T group (median gestational age: 20 weeks, 2 days). CONCLUSION: Our findings suggest that the increase in noise associated with 3 T does not increase the rate of clinically detectable hearing abnormalities.

Imaging of Tinnitus.

Although not all patients with tinnitus require imaging, patients with tinnitus and asymmetric hearing loss, additional neurologic findings, or pulsatile tinnitus should be evaluated with an appropriately tailored imaging study. Choice of imaging study should be guided by type of hearing loss and additional physical examination findings, such as middle ear lesion, presence of carotid bruit, or pulsatile tinnitus extinguished by jugular compression.

Noncontrast vestibular schwannoma surveillance imaging including an MR cisternographic sequence: is there a need for postcontrast imaging?

OBJECTIVE: The purpose of this study was to evaluate the use of a noncontrast MRI protocol that includes a cisternographic sequence (CISS/FIESTA/3D DRIVE) compared to a protocol that includes a gadolinium-enhanced sequence in order to determine whether a noncontrast approach could be utilized to follow vestibular schwannomas. METHODS: A total of 251 patients with vestibular schwannomas who underwent MRI of the temporal bones that included both cisternographic sequence and postcontrast T1 imaging between January 2000 and January 2016 for surveillance were included in this retrospective study. The size of the vestibular schwannomas was independently assessed on a noncontrast MR cisternographic sequence and compared to size measurements on a postcontrast sequence. The evaluation of intralesional cystic components (identified as T2 signal hyperintensity) and hemorrhagic components (identified with intrinsic T1 hyperintensity) on noncontrast MR sequences was compared to evaluation on postcontrast MR sequences to determine whether additional information could be derived from the postcontrast sequences. Additionally, any potentially clinically significant, incidentally detected findings on the postcontrast T1 sequences were documented and compared with the detection of these findings on the precontrast images. RESULTS: No significant difference in vestibular schwannoma size was found when comparing measurements made on the images obtained with the MR cisternographic sequence and those made on images obtained with the postcontrast sequence (p = 0.99). Noncontrast MR images were better (detection rate of 87%) than postcontrast images for detection of cystic components. Noncontrast MR images were also better for identifying hemorrhagic components. No additional clinically relevant information regarding the tumors was identified on the postcontrast sequences. CONCLUSIONS: Based on the results of this study, a noncontrast MR protocol that includes a cisternographic sequence would be sufficient for the accurate characterization of size and signal characteristics of vestibular schwannomas, obviating the need for gadolinium contrast administration for the routine surveillance of these lesions.

Systematic approaches for reviewing neuro-imaging scans in ophthalmology.

Neuroimaging is an important tool in ophthalmology, but many ophthalmologists are uncomfortable evaluating actual scans. Unfortunately, exclusive reliance on a radiologist's report can lead to diagnostic and management errors. We outline a methodology for equipping ophthalmologists with the skills necessary to read neuroimaging studies with respect to specific clinical questions.

Imaging of orbital disorders.

Diseases of the orbit can be categorized in many ways, but in this chapter we shall group them according to etiology. Inflammatory diseases of the orbits may be infectious or noninfectious. Of the infections, orbital cellulitis is the most common and typically arises as a complication of acute sinusitis. Of the noninfectious, inflammatory conditions, thyroid orbitopathy is the most common and results in enlargement of the extraocular muscles and proliferation of the orbital fat. Idiopathic orbital inflammatory syndrome is another cause of inflammation in the orbit, which may mimic thyroid orbitopathy or even neoplasm, but typically presents with pain. Masses in the orbit may be benign or malignant and the differential diagnosis primarily depends on the location of the mass lesion, and on the age of the patient. Lacrimal gland tumors may be lymphomas or epithelial lesions of salivary origin. Extraocular muscle tumors may represent lymphoma or metastases. Tumors of the intraconal fat are often benign, typically hemangiomas or schwannomas. Finally, globe tumors may be retinoblastomas (in children), or choroidal melanomas or metastases in adults.

MR imaging findings of endophthalmitis.

Endophthalmitis is a sight-threatening ophthalmologic emergency. The clinical diagnosis is often challenging, and delayed diagnosis may exacerbate the poor visual prognosis. B-scan ultrasonography or spectral domain optical coherence tomography are imaging aids at the clinician's office. Cross-sectional imaging such as CT and particularly MRI can also help in the assessment of disease extent or complications. MR imaging findings are rarely described in the literature. Here, we discuss the spectrum of imaging findings of endophthalmitis and correlate them with key anatomic and pathophysiologic details of the globe. Early disease is often subtle on MR imaging with thick uveal enhancement, while advanced disease demonstrates retinal/choroidal detachment, vitreal exudates and peribulbar inflammation. Other noninfectious inflammatory diseases of the globe can show similar findings; however, MR diffusion-weighted images help identify infectious exudates and evaluate response to therapy. Knowledge of the spectrum of imaging findings of this disease is important for radiologists and help in the management decision process.

Laryngeal mask airway may result in false negative imaging for carotid medialization: A case report.

Chromosome 22q11.2 microdeletions result in multiple congenital abnormalities, including an increased risk of carotid medialization, which is an important consideration for preoperative planning in children with velopharyngeal insufficiency. Preoperative imaging of the neck vasculature is recommended. Here we describe a case in which a child had negative imaging studies despite the presence of a medialized carotid artery on physical examination, likely secondary to the supraglottic airway use during sedated imaging, which displaced the carotid laterally. The type of airway used should be a consideration for children undergoing sedated imaging prior to pharyngeal procedures.

Losing your voice: etiologies and imaging features of vocal fold paralysis.

Neurogenic compromise of vocal fold function exists along a continuum encompassing vocal cord hypomobility (paresis) to vocal fold immobility (paralysis) with varying degrees and patterns of reinnervation. Vocal fold paralysis (VFP) may result from injury to the vagus or the recurrent laryngeal nerves anywhere along their course from the brainstem to the larynx. In this article, we review the anatomy of the vagus and recurrent laryngeal nerves and examine the various etiologies of VFP. Selected cases are presented with discussion of key imaging features of VFP including radiologic findings specific to central vagal neuropathy and peripheral recurrent nerve paralysis.

Vascular compression of the airway: establishing a functional diagnostic algorithm.

IMPORTANCE: Pediatric imaging carries the risk of radiation exposure. Children frequently undergo computed tomography with angiography (CTA) for findings on bronchoscopy with limited knowledge regarding the necessity of such imaging. OBJECTIVE: To report our experience with all pediatric patients at our institution over an 8-year period with airway symptoms warranting bronchoscopy followed by CTA for potential vascular anomaly. Goals were to report the percentage of positive findings seen on CTA leading to surgery; discuss relative radiation exposure risk and sedation risk for additional radiologic studies; and propose a functional diagnostic algorithm. DESIGN, SETTING, AND PARTICIPANTS: Retrospective chart review of 42 children aged 2 months to 11 years with tracheomalacia who underwent CTA between 2004 and 2012 in our tertiary aerodigestive center. INTERVENTIONS: Bronchoscopy and CTA. MAIN OUTCOMES AND MEASURES: Presence of vascular anomaly and need for thoracic surgery. RESULTS: Of these 42 children, 21 (50%) had a vascular anomaly identified on CTA. Of these 21, 17 (81%) had innominate artery compression; 1 (5%) had double aortic arch; 1 (5%) had right aortic arch; 3 (14%) had bronchial compression by pulmonary artery; and 1 (5%) had dextrocardia with duplicated vena cava. Six (29%) of these 21 had clinical symptoms and CTA findings requiring thoracic surgery. The most common symptoms in children requiring thoracic surgery were cough, cyanosis, and stridor. CONCLUSIONS AND RELEVANCE: Deciding when to obtain imaging for bronchoscopic findings suggestive of vascular compression remains challenging. A diagnostic algorithm is proposed as a means to provide the best clinical care while weighing risks of additional radiation exposure vs sedation and exposure to general anesthesia.

Patterns of perineural tumor spread in head and neck cancer.

Perineural tumor spread (PNS) is a mode of neoplastic spread whereby tumor cells use neural conduits to escape the borders of a primary tumor. MRI is generally favored over CT for evaluating PNS, and findings include obliteration of fat within skull base foramina, enlargement and enhancement of the involved nerves, and enlargement and destruction of the bony foramina. Careful examination of the entire course of the nerve allows detection of skip lesions. Recognition of the complete extent of PNS is crucial for correct treatment because it facilitates both surgical and radiotherapy targeting of entire extent of disease.

Benign conditions of the thyroid gland.

Infectious and autoimmune diseases account for the majority of benign conditions of the thyroid gland. They are usually diagnosed and followed by clinical examination and laboratory analyses, but when imaged, ultrasonography and computed tomography are the modalities of choice. In particular, fine needle aspiration under ultrasound guidance may be invaluable for diagnostic and therapeutic purposes.

Color Doppler ultrasound: effective monitoring of the buried free flap in facial reanimation.

OBJECTIVE: The gracilis muscle free flap has become a reliable means for smile reanimation for patients with facial paralysis. Because it is a buried flap, it presents a postoperative monitoring challenge. We sought to evaluate our experience with color Doppler ultrasound in the monitoring of gracilis free flap viability in the immediate postoperative setting. STUDY DESIGN: Case series with planned data collection. SETTING: Tertiary medical center. METHODS: Patients with facial paralysis treated with gracilis muscle free flap for smile reanimation performed between March 2009 and November 2010 were evaluated by color Doppler ultrasound and included in the study. Our experience with the use of the color Doppler ultrasound to monitor the gracilis muscle flap is presented. RESULTS: Forty-six patients were identified. In all cases, color Doppler ultrasound was used postoperatively to assess flow through the vascular pedicle. Outcomes included an early flap survival rate of 100%, with no instances of equivocal or absent flow on either the arterial or venous side. Color Doppler ultrasound provided important objective information regarding muscle perfusion postoperatively in several instances of equivocal postoperative perfusion of the flap. CONCLUSION: Color Doppler ultrasound is a safe, noninvasive method that can be performed serially to evaluate a buried free flap. We have had success in verifying normal arterial and venous flow through the pedicle using this method of monitoring of the gracilis muscle free flap during facial reanimation, and in 3 instances, it eliminated the need for wound exploration to verify appropriate muscle perfusion.

The use of diffusion MRI in ischemic optic neuropathy and optic neuritis.

Ischemic optic neuropathy and optic neuritis are the most common causes of unilateral non-glaucomatous visual loss. While they have distinctive clinical features, they also share some overlapping profiles that make it difficult to clinically distinguish these two entities. MRI with gadolinium has been proven to be helpful to confirm the diagnosis of optic neuritis, but ischemic optic neuropathy remains a clinical diagnosis. Diffusion MRI, a newly advanced technique, has been used in studying the pathophysiology of optic neuritis, but its use in ischemic optic neuropathy is limited. Diffuse MRI may potentially be of help to diagnose ischemic optic neuropathy.