Isolated Primary Laryngeal Amyloidosis: A Case Series of a Rare Presentation of Change in Voice

Abstract Introduction The incidence of vestibular schwannoma is reported as 12 to 54 new cases per million per year, increasing over time. These patients usually present with unilateral sensorineural hearing loss, tinnitus, or vertigo. Rarely, these patients present with symptoms of hydrocephalus or vision changes. Objective The study aimed to evaluate the surgical management of vestibular schwannoma at a single institution and to identify factors that may contribute to hydrocephalus, papilledema, and the need for pre-resection diversion of cerebrospinal fluid. Patients and Methods A retrospective review examining the data of 203 patients with vestibular schwannoma managed with surgical resection from May 2008 to May 2020. We stratified patients into five different groups to analyze: tumors with a diameter of ≥40 mm, clinical evidence of hydrocephalus, and of papilledema, and patients who underwent pre-resection cerebrospinal fluid (CSF) diversion. Results From May 2008 to May 2020, 203 patients were treated with surgical resection. Patients with tumors ≥40 mm were more likely to present with visual symptoms (p < 0.001). Presentation with hydrocephalus was associated with larger tumor size (p < 0.001) as well as concomitant visual symptoms and papilledema (p < 0.001). Patients with visual symptoms presented at a younger age (p = 0.002) and with larger tumors (p < 0.001). Conclusion This case series highlights the rare presentation of vision changes and hydrocephalus in patients with vestibular schwannoma. We recommend urgent CSF diversion for patients with visual symptoms and hydrocephalus, followed by definitive resection. Further, vision may still deteriorate even after CSF diversion and tumor resection.

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Chronic Skull Base Erosion from Temporomandibular Joint Disease Causes Generalized Seizure and Profound Lactic Acidosis

Case Reports in Critical Care ◽

10.1155/2018/8795036 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Mark A. Dobish ◽

David A. Wyler ◽

Christopher J. Farrell ◽

Hermandeep S. Dhami ◽

Victor M. Romo ◽

...

Keyword(s):

Lactic Acidosis ◽

Skull Base ◽

Temporomandibular Joint ◽

Case Series ◽

Type A ◽

Oxidative Capacity ◽

Joint Disease ◽

Rare Presentation ◽

Cellular Energy ◽

Ph Range

This report displays a rare presentation of lactic acidosis in the setting of status epilepticus (SE). The differential diagnosis of lactic acidosis is broad and typically originates from states of shock; however, this report highlights an alternative and rare etiology, SE, due to chronic skull base erosion from temporomandibular joint (TMJ) disease. Lactic acidosis is defined by a pH below 7.35 in the setting of lactate values greater than 5 mmol/L. Two broad classifications of lactic acidosis exist: a type A lactic acidosis which stems from global or localized tissue hypoxia or a type B lactic acidosis which occurs once mitochondrial oxidative capacity is unable to match glucose metabolism. SE is an example of a type A lactic acidosis in which oxygen delivery is unable to meet increased cellular energy requirements. This report is consistent with a prior case series that consists of five patients experiencing generalized tonic-clonic (GTC) seizures and lactic acidosis. These patients presented with a pH range of 6.8-7.41 and lactate range of 3.8-22.4 mmol/L. Although severe lactic acidosis following GTC has been described, this is the first report in the literature of chronic skull base erosion from TMJ disease causing SE.

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Calcitriol-Mediated Hypercalcemia: Causes and Course in 101 Patients

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.2013-2016 ◽

2013 ◽

Vol 98 (10) ◽

pp. 4023-4029 ◽

Cited By ~ 31

Author(s):

Peter J. Donovan ◽

Lana Sundac ◽

Carel J. Pretorius ◽

Michael C. d'Emden ◽

Donald S. A. McLeod

Keyword(s):

Angiotensin Converting Enzyme ◽

Case Reports ◽

Elevated Serum ◽

Case Series ◽

Lymphocytic Leukemia ◽

Converting Enzyme ◽

Retrospective Case ◽

Rare Presentation ◽

Hospital System ◽

Serum Angiotensin Converting Enzyme

Abstract Context: Hypercalcemia mediated by 1,25-dihydroxy vitamin D (calcitriol) is uncommon, with evidence on etiology limited to small case series or case reports. Objective: The objective of the study was to systematically identify a large series of cases of calcitriol-mediated hypercalcemia and document the presentation, demographics, and clinical course across etiologies. Design, Setting, and Patients: The study was a hospital-based, retrospective case series, identifying subjects from 1999 through 2009 across the public hospital system in Queensland, Australia. All patients aged over 18 years were identified that had persistent hypercalcemia associated with elevated or inappropriately normal calcitriol concentration or elevated serum angiotensin-converting enzyme. Results: A total of 101 cases were identified. Sarcoidosis was the most common etiology (49%), followed by hematological malignancy (17%) and infections (8%). Etiologies not previously described include squamous cell carcinoma of the tongue, ovarian cystadenocarcinoma, and chronic lymphocytic leukemia. Median serum angiotensin-converting enzyme was higher in sarcoid patients compared with all other causes [218 U/L (176–277) vs 155 U/L (110–208), P < .001], but a level above the normal range did not discriminate well between cases of sarcoidosis and other causes (specificity at cutoff of 130 U/L was only 31%). However, a value greater than 250 U/L was highly specific (89%) for sarcoidosis but lacked sensitivity (31%). A calcitriol level greater than 300 pmol/L was not seen in sarcoidosis but was seen with other etiologies. Cases with neoplastic etiologies were older (61.4 ± 11.4 y) than all other subjects (51.7 ± 15.0 y, P = .006). Conclusions: Hypercalcemia mediated by calcitriol remains a rare presentation. In almost half the cases, sarcoidosis was the underlying cause, whereas a third of patients had cancer or systemic infections.

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Laryngeal amyloidosis: diagnosis, pathophysiology and management

The Journal of Laryngology & Otology ◽

10.1017/s0022215117000780 ◽

2017 ◽

Vol 131 (S2) ◽

pp. S41-S47 ◽

Cited By ~ 9

Author(s):

N M Phillips ◽

E Matthews ◽

C Altmann ◽

J Agnew ◽

H Burns

Keyword(s):

Carbon Dioxide Laser ◽

Case Series ◽

Anatomical Location ◽

Amyloid Deposits ◽

Laryngeal Amyloidosis ◽

Laryngeal Lesions ◽

Laser Excision ◽

Endoscopic Microsurgery ◽

The One ◽

Benign Laryngeal Lesions

AbstractBackground:Laryngeal amyloidosis represents approximately 1 per cent of all benign laryngeal lesions, and can cause variable symptoms depending on anatomical location and size. Treatment ranges from observation through to endoscopic microsurgery, laser excision and laryngectomy.Objectives:To highlight the diversity of presentations, increase awareness of paediatric amyloidosis and update the reader on current management.Case series:Five cases are illustrated. Four adult patients were female, and the one child, the second youngest in the literature, was male. Amyloid deposits were identified in all laryngeal areas, including the supraglottis, glottis and subglottis. Treatment consisted of balloon dilatation, endoscopic excision, laser cruciate incision, and resection with carbon dioxide laser, a microdebrider and coblation wands.Conclusion:Laryngeal amyloidosis remains a rare and clinically challenging condition. Diagnosis should be considered for unusual appearing submucosal laryngeal lesions. Treatment of this disease needs to be evaluated on a case-by-case basis and managed within an appropriate multidisciplinary team.

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