Bhatt, Alok A.

Genetic tumor syndromes are due to inherited genetic mutations, which have recently come to the attention of clinicians due to the widespread adoption of DNA sequencing, ultimately leading to imaging for surveillance. As a result, radiologists must be familiar with the clinical, genetic, and radiologic features of these syndromes. This article reviews genetic tumor syndromes of the head and neck according to the recently updated WHO’s 5th edition.

Background There is no defined preventive treatment protocol for persistent post-craniotomy headache. In several small case series and individual case reports onabotulinumtoxinA injected into the craniotomy scar has shown possible efficacy. What is lacking is long term follow-up and if focusing on the cranial suture lines along with the craniotomy scar can enhance improvement and provide more sustained benefit. Methods Retrospective chart review with case series. Results Four patients (three women, one man) with ICHD-3 defined persistent post craniotomy headache were treated using a novel onabotulinumtoxinA injection protocol. All the patients presented with continuous head pain of moderate to severe intensity. All had severe allodynia on the side of their craniotomy. All had significant reduction in quality of life. Our application of onabotulinumtoxinA involved injection into both the surgical scar and the transected/irritated cranial suture lines noted on neuroimaging and physical examination. With treatment all patients demonstrated significant benefit including a reduction in daily pain intensity (75%-100%), developing periods of pain freedom (2–7 days per week) and having a dramatic improvement in quality of life (close to 100% in all). The benefit was sustained for at least five years of follow-up. Conclusion From our case series it appears that injection not only along the painful craniotomy scar but into the involved cranial suture lines provides positive efficacy and sustained improvement in patients with persistent post craniotomy headache.

Epstein-Barr virus (EBV) belongs to the group of human herpes virus and can cause clinical and subclinical infections. Although EBV-related disease presentations are similar, they can lead to oncogenic transformation with various clinical manifestations. A thorough workup with morphology, immunohistochemistry, and molecular studies is crucial for the diagnosis of EBV-positive polymorphic B-cell lymphoproliferative disorder, not otherwise specified (NOS), which is a new entity introduced by International Consensus Classification in 2022. We describe an interesting presentation of EBV-positive polymorphic B-cell lymphoproliferative disorder with laryngeal involvement to bring awareness to this entity and we would like to address the need for more accessible treatment options.

There are many lesions that cause compression of nerves and vessels in the head and neck, and they can often be overlooked in the absence of adequate history or if not suspected by the radiologist. Many of these lesions require a high index of suspicion and optimal positioning for imaging. While a multimodality approach is critical in the evaluation of compressive lesions, an MRI utilizing high-resolution (heavily weighted) T2-weighted sequence is extremely useful as a starting point. In this review, we aim to discuss the radiological features of the common and uncommon compressive lesions of the head and neck which are broadly categorized into vascular, osseous, and miscellaneous etiologies.

Objective Schwannomas are common nerve sheath tumors and may occur anywhere in the body. 4% of head and neck schwannomas occur in the sinonasal cavity, and fewer yet have an intracranial component, making these presentations extremely rare. Furthermore, schwannomas present with nonspecific imaging signs and can only be definitively differentiated via histopathologic review, leading to misdiagnosis as various nasal tumors. We aim to conduct a review of published literature on sinonasal schwannomas with and without intracranial extension and provide additional case representations. Methods A literature review was conducted using the PubMed Database with the terms “sinonasal schwannoma,” “intracranial,” “anterior skull base,” and “schwannoma.” Results were reviewed, and additional cases identified were referenced and included in the study. Inclusion criteria were any case with intracranial extension of the schwannoma. There were no exclusion criteria. Review data was compiled into Excel and used for data analysis and comparison. Additionally, a search was done within our institution to identify additional cases of sinonasal schwannoma. Results We identified 17 cases of sinonasal schwannoma with intracranial extension, five from our institution and twelve from literature. Analysis revealed: 8 females (47%), 9 males (53%), 9 patients presented with headaches (53%), 6 patients presented with anosmia (35%), 4 patients presented with nasal obstruction (24%), and 2 patients with no symptoms (12%). Mean age and median were 39.4 ± 10.1 and 40, respectively. For treatment, 4 patients underwent endoscopic resection (24%), 11 underwent craniotomy (65%), and data was unavailable for 2 patients. Post-treatment complications occurred in 6 patients, 5 had CSF leaks (29%) and 1 had a hematoma (6%). Conclusion We identified and discussed 17 cases of sinonasal schwannoma with intracranial extension. We hope our review provides insight for clinicians to maintain schwannoma as a potential differential when evaluating nasal and anterior skull base masses.

Objectives: Superior semicircular canal dehiscence (SSCD) is defined as a defect in the bone overly the superior semicircular canal (SSC). The purpose of this study is to evaluate the pre-operative imaging examinations of patients who have undergone SSCD repair. We hypothesize that these patients will not have a supralabyrinthine air cell on the side of surgery. Material and Methods: Our group retrospectively reviewed 50 consecutive pre-operative computed tomography (CT) temporal bone examinations who had confirmed SSCD on intraoperative examination and underwent repair for the presence of a supralabyrinthine air cell. Results: 100% of patients who had confirmed SSCD on intraoperative examination had no supralabyrinthine air cell on pre-operative CT of the temporal bone. Conclusion: This study shows that a supralabyrinthine air cell is not present in SSCD. When a supralabyrinthine air cell is present, the roof of the SSC is intact. CT and magnetic resonance imaging (MRI) are often performed together to evaluate for SSCD and exclude other etiologies. We propose that if a supralabyrinthine air cell is seen on MRI, no CT is necessary, thus avoiding unnecessary radiation exposure and additional imaging costs to the patient.