Operative technique in laryngeal paraganglioma: A case series and literature review.

Background: Laryngeal paragangliomas (LP) comprise a rare subset of head and neck neoplasms and are an important differential in the patient with a submucosal laryngeal mass. Methods: We discuss an operative technique using coblation assisted excision via laryngofissure in the cases of four confirmed LPs and discuss the current literature with respect to diagnosis and management of these patients. Results: Our case series of four patients demonstrate the laryngofissure approach for LP is safe, provides oncological cure and preserves laryngeal function. This technique with coblation has not been previously discussed in the surgical management of LPs. Patient-reported postoperative voice and swallowing outcomes were excellent in all patients. Whole body imaging techniques were utilized in all patients, with octreotide scintigraphy demonstrating synchronous lesions in two of the four patients-one had a retroperitoneal lesion, whilst another patient had multiple lesions in the skull base, paravertebral region, liver, and adrenal gland. Conclusions: Our cases also highlight the changing clinical paradigms in the diagnostic approach of LPs and an emerging role for octreotide scintigraphy in the workup of these patients. Coblation assisted excision via laryngofissure is efficient and effective in the surgical resection of LP. In patients with multicentric forms of LP, the size, side, and anatomic location of synchronous lesions should be considered in surgical planning to determine feasibility and safety of operative management. Level of Evidence: IV.

Combination Immunosuppressive Therapy in Primary Autoimmune Inner Ear Disease in Pregnancy.

Objective: Autoimmune inner ear disease (AIED) is a rare disorder characterized by rapidly progressive, sensorineural hearing loss that demonstrates good responsiveness to corticosteroid and immunosuppressive therapy. The pathophysiology is likely driven by chronic trafficking of immune cells into the inner ear, targeting inner ear proteins to coordinate inflammation. Suppression or modulation of the immune response can minimize cochleitis allowing for potential recovery of hearing. It is an otologic emergency requiring a multidisciplinary approach to management to commence immunosuppressive therapy. This can be achieved using steroids, immunomodulators, plasmapheresis, intravenous immunoglobulin, or biologic agents. Treatment decisions are further complicated in pregnancy and require supervision by an obstetrician and maternal-fetal medicine (MFM) specialist. Concerns include safe dosing of steroids and potential for transplacental migration of immune complexes. We provide the first comprehensive literature review on AIED and its implications in pregnancy. We frame our discussion in the context of the second reported case of primary AIED in pregnancy and the first to show excellent response to immunosuppressive therapy. Methods: We reviewed the presented case and literature on AIED. Results: A 27-year-old, pregnant, HSP-70 positive woman was diagnosed with AIED and had excellent recovery of hearing and balance following a combination of steroid treatment, augmented by oral immunomodulators, plasmapheresis, and IVIG. Conclusion: AIED is a diagnostic challenge, and treatment considerations are complex when encountered in pregnancy. Management requires multidisciplinary involvement between otolaryngologists, immunologists, and obstetricians to balance maternal and fetal health outcomes.

Ramsay Hunt syndrome with multiple cranial neuropathy: a literature review.

BACKGROUND: Ramsay Hunt Syndrome (RHS) is a neurotological disorder involving the reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve (Sweeney and Gilden in J Neurol Neurosurg Psychiatry 71:149-154, 2001). The characteristic presentation involves ipsilateral lower motor neuron type facial paresis, auricular pain with or without hearing impairment, and vesicular lesions of the external auditory canal and outer ear. Involvement of the facial and vestibulocochlear nerve is typical in RHS, whilst multiple cranial neuropathies are rare and associated with poorer prognosis and systemic complications (Arya et al. in Am J Case Rep 19:68-71, 2017; Shinha and Krishna in IDCases 2:47-48, 2015; Shim et al. in Acta Otolaryngol 131:210-215, 2011; Coleman et al. in J Voice 26:e27-e28, 2012; Morelli et al. in Neurol Sci 29:497-498, 2008;). Likely mechanisms involved in the pathogenesis of cranial polyneuropathy include direct peri-neural and trans-axonal spread of viral inflammation between contiguous cranial nerves and haematogenous dissemination between nerves with shared blood supply. Impairments in speech, swallowing, hearing, and oculo-protection can contribute to morbidity and requires a multidisciplinary approach to patient care. METHODS: We present a rare case of RHS with multiple cranial neuropathies followed by a comprehensive review of current literature with regard to the pathophysiology, diagnostic workup, and the management strategies employed in these patients. CONCLUSION: RHSs with multiple cranial neuropathies are important to recognise as they are associated with significant morbidity and poor prognosis. A multidisciplinary approach to patient management is required to address the several complications that can arise from cranial nerve deficits, especially in regard to speech and swallow.

Thoracic aortic aneurysm in the presence of mediastinal and retroperitoneal fibrosis.

Inflammatory aneurysms of thoracic aorta are rare. Of all the aneurysms, only 5-10% are inflammatory in nature and are almost exclusively confined to the infrarenal segment of the aorta. We hereby present a patient with a large inflammatory aneurysm involving the thoracic aorta in association with mediastinal fibrosis and idiopathic retroperitoneal fibrosis.

Helicobacter pylori eradication: a novel therapeutic option in chronic immune thrombocytopenic purpura.

OBJECTIVE: To determine whether Helicobacter pylori eradication is an effective treatment for Australian patients with chronic immune thrombocytopenic purpura (ITP). DESIGN, SETTING AND PATIENTS: Retrospective analysis of clinical records of a consecutive series of ITP patients referred to a gastrointestinal surgeon in a tertiary referral hospital for laparoscopic splenectomy between August 2005 and November 2007. MAIN OUTCOME MEASURES: Platelet response (measured at least 3-monthly) following successful H. pylori eradication therapy (confirmed by urea breath test 4 weeks later). RESULTS: Of 16 patients, seven were H. pylori-negative and underwent laparoscopic splenectomy. Nine were H. pylori-positive and successfully underwent H. pylori eradication therapy; five of the nine had an initial platelet response. Four patients had platelet counts > 100 x 10(9)/L (reference range, 140-450 x 10(9)/L) and were off all immunosuppression at 9 months; three had a sustained response beyond 12 months. One patient had an initial response at 3 months (15 x 10(9)/L to 208 x 10(9)/L), but relapsed 4 months after H. pylori eradication and underwent splenectomy with platelet count recovery. The remaining four patients showed no platelet response and subsequently underwent splenectomy. CONCLUSION: Larger prospective studies are needed to fully ascertain the role of H. pylori in Australian patients with ITP. However, H. pylori eradication is simple and safe. H. pylori screening and eradication should be considered before immunosuppression or splenectomy.