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General Surgery
Published in Tjun Tang, Elizabeth O'Riordan, Stewart Walsh, Cracking the Intercollegiate General Surgery FRCS Viva, 2020
Rebecca Fish, Aisling Hogan, Aoife Lowery, Frank McDermott, Chelliah R Selvasekar, Choon Sheong Seow, Vishal G Shelat, Paul Sutton, Yew-Wei Tan, Thomas Tsang
A 22-year-old girl presents with multiple cutaneous soft nodules and brown patches on her skin. What is the most likely diagnosis, and how would you classify the disease?This sounds like neurofibromatosis (von Recklinghausen's disease). Patients present with multiple neurofibromas, more than six café-au-lait patches, axillary freckling and Lisch nodules on the iris. It is familial (autosomal dominant) and is present at birth.Patients can develop acoustic neuromas, and there is a 5%–10% risk of malignant change. A neurofibroma is a hamartoma − an overgrowth of neural tissue.There are four types of neurofibromatosis. The first is type I, with the features already mentioned. Type II is associated with neurofibromas in the central nervous system, which can damage the cranial nerves. Type III is schwannomatosis, which is very painful and very rare. Type IV has plexiform neurofibromas, which are infiltrative lesions on the head and neck, have a higher risk of malignant transformation and a rapid metastatic spread.
Central nervous system
Published in A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha, Clark’s Procedures in Diagnostic Imaging: A System-Based Approach, 2020
A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha
MRI has become the gold standard for detection of acoustic neuroma as it visualises the cranial nerve nucleus and the nerve itself as it crosses the posterior fossa CSF space in the cerebellopontine angle, and courses through the internal auditory canal. Neuromas may enhance avidly making contrast MRI more sensitive than unenhanced MRI. Surgical removal of acoustic neuroma may cause complete deafness and MRI can be used to track progression (or otherwise) of neuromas allowing surgery to be deferred in suitable cases.
The ear, nose and sinuses
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
It has been said that not only can clinicians not agree on the cause of Menières disease, they cannot even agree on the spelling. There is certainly evidence of endolymphatic hydrops (long-standing high pressure changes within the inner ear) in pathological specimens of patients who have had the condition. The condition is characterised by a triad of symptoms: intermittent attacks of vertigo, a unilateral fluctuating sensorineural hearing loss and tinnitus. The patient often has a sensation of pressure in the affected ear before an attack. The hearing loss typically affects the lower frequencies. The vertigo characteristically lasts between 30 minutes and 6 hours and is often accompanied by nausea and vomiting. The investigations include pure tone audiometry and an MRI scan to exclude an acoustic neuroma. The only evidence-based treatment is intratympanic therapy whereby repeated injections of dexamethasone or gentamicin are given into the middle ear. Traditional medical treatment starts with a low salt diet, betahistadine and diuretics, but this is not evidence based
Secondary cerebellopontine angle oligodendroglioma after cranial irradiation: a case report and literature review
Published in International Journal of Neuroscience, 2023
Zhifei Guo, Dekun Li, Yongsheng Xie, Jin Qian, Bing Zhao
Epilepsy was the most common first symptom in oligodendroglioma patients, followed by focal neurological deficit and cognitive impairment [11]. The symptoms of focal neurological deficits vary according to the location of the tumor, such as hemiplegia, aphasia, limb numbness, blurred vision, and abnormal smell, among others [2]. Our patient’s symptoms are similar to those of acoustic neuroma, which is characterized by hearing loss. Thus, it is easily misdiagnosed as acoustic neuroma. During the operation, it was found that the tumor invaded the internal auditory canal, compressed the cochlear nerve, and caused hearing loss. At the same time, ataxia caused by cerebellar damage, such as walking instability, was found. The majority of oligodendrogliomas in the CPA described by Ellenbogen were mostly due to tumors located in the cerebellar hemisphere, and the patient’s symptoms were mainly intracranial hypertension and ataxia [8].
Identification of factors associated with tinnitus outcomes following the microsurgical treatment of vestibular schwannoma patients
Published in Acta Oto-Laryngologica, 2021
Chi Zhang, Fangyuan Wang, Wei Cao, Xiaoyan Ma, Jiyue Chen, Weidong Shen, Shiming Yang
Vestibular schwannomas (VSs) are benign, slow-growing tumors that develop from the Schwann cells of the superior vestibular nerve (the vestibular branch of the eighth cranial nerve). These tumors account for 6% of all intracranial tumors, but 85% of cerebellopontine angle tumors [2]. The Acoustic Neuroma Association (ANA) found that tinnitus was present in up to 70% of VS patients, and was the second most common presenting system after asymmetric hearing loss. Tinnitus was also reported to be the most common issue of concern among surveyed VS patients, followed by dizziness and fatigue [3]. Historically, surgical treatment of VS patients focused on tumor removal and the preservation of hearing and facial nerve function. Recent advances in microsurgical techniques, however, have emphasized the importance of focusing on patient postoperative quality of life [4]. Tinnitus can significantly lower VS patient quality of life, even when it does not seriously disrupt the activities of daily living. Further research on postoperative tinnitus in these patients is therefore warranted.
The characteristics of vHIT gain and PR score in peripheral vestibular disorders
Published in Acta Oto-Laryngologica, 2021
Yi Du, Lili Ren, Xingjian Liu, Weiwei Guo, Ziming Wu, Shiming Yang
We retrospectively analyzed 386 patients admitted to the vertigo clinic at the Chinese PLA General Hospital from December 2019 to May 2020. Two authors (ZMW and LLR) carefully reviewed patients’ information, including the history, medical records, imaging results, neuro-otological examination, audiology, and vestibular function tests. Acoustic neuroma (AN) were radiologically diagnosed and went through vHIT before surgery. MD was diagnosed according to the AAO-NHS criteria [10]. Vestibular migraine (VM) patients were identified according to the Barany Society diagnostic criteria [11]. The diagnosis of benign paroxysmal positional vertigo (BPPV) is based on the Barany Society 2015 diagnostic criteria [12]. Bilateral vestibular hypofunction (BVH) is diagnosed according to the Barany Society 2017 diagnostic criteria [13]. Ramsay-Hunt syndrome (RHS) with vertigo is diagnosed with an ipsilateral herpetic eruption on the auricle and external ear canal, facial palsy, and vertigo. The diagnosis of unilateral vestibular dysfunction(UVD) was based on the history of acute sustained vertigo or imbalance, abnormal results in the caloric test (unilateral weakness >25%) and no additional central lesion signs.