ObjectivePreoperative embolization has proven beneficial in the surgical treatment of juvenile nasopharyngeal angiofibromas (JNA). However, the consensus for the best embolization practices remains unclear. This systematic review seeks to characterize the reporting of embolization protocols throughout the literature and to compare differences in surgical outcomes.Data SourcesPubMed, Embase, and Scopus.Review MethodsStudies investigating embolization in the treatment of JNA from 2002 to 2021 were selected from defined inclusion criteria. All studies underwent a 2‐stage blinded screening, extraction, and appraisal process. Embolization material, time to surgery, and embolization route were compared. Embolization complications, surgical complications, and rate of recurrence were pooled.ResultsOf 854 studies, 14 retrospective studies with 415 patients met the criteria for inclusion. A total of 354 patients underwent preoperative embolization. A total of 330 patients (93.2%) underwent transarterial embolization (TAE) and 24 patients had a combination of direct puncture embolization and TAE. Polyvinyl alcohol particles were the most used embolization material (n = 264, 80.0%). The most common reported time to surgery was 24 to 48 hours (n = 8, 57.1%). Pooled results showed an embolization complication proportion of 3.16% (95% confidence interval [CI]: 0.96‐6.60) (n = 354), a surgical complication proportion of 4.96% (95% CI: 1.90‐9.37) (n = 415), and a recurrence proportion of 6.30% (95% CI: 3.01‐10.69) (n = 415).ConclusionThe current data on JNA embolization parameters and their effect on surgical outcomes remains too heterogenous to provide expert recommendations. Future studies should use uniform reporting to allow for more robust comparisons of embolization parameters, which, in turn, may lead to optimized patient outcomes.
Objective: Behcet’s syndrome (BS) is a chronic, relapsing multisystemic inflammatory perivasculitis and can affect any tissue, including the nervous system. Neuro-Bechet’s syndrome (NBS) most commonly affects the CNS parenchyma and presents with a subacute brainstem syndrome that includes cranial neuropathies. Here we describe a rare case of palato-pharyngo-laryngeal myoclonus as a manifestation of NBS and discuss it from a laryngology perspective. Methods: Case report at tertiary care center. Informed consent was obtained from patient. IRB approved as non-human subjects research. Results: A 52-year-old male presented with a progressive history of ataxia, fatigue, apathy, dysphagia, depressed mood, dizziness, poor appetite, subjective fever and recurrent orogenital lesions. He was diagnosed with NBS and treated with methylprednisolone, followed by infliximab and methotrexate. Despite treatment, his severe spastic dysarthria, dysphagia, and aspiration worsened over the next few months, necessitating a gastrotomy tube. With concern for laryngospasm, he was referred to otolaryngology and found to have synchronous and symmetric palatal, pharyngeal, and laryngeal rhythmic myoclonus bilaterally at a frequency of 2 Hz with inappropriate vocal cord closure. Treatment with baclofen and a scopolamine patch improved his breathing and reduced choking events. Conclusions: Palato-pharyngo-laryngeal rhythmic myoclonus can be a presentation of brainstem NBS in the otolaryngology clinic. We theorize perivascular disease in NBS results in a brainstem lesion in the denato-rubro-olivary tract, which results in hypertrophic olivary degeneration and subsequent activation of the inferior olives oscillatory activity, causing palato-pharyngo-laryngeal rhythmic myoclonus. Common symptoms include significant dysarthria, dysphonia, and dysphagia with concern for obstructive sleep apnea and airway compromise. Treatments include pharmacologic therapy, laryngeal botox, and tracheostomy in cases of significant airway compromise.
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