慢性耳:10岁男童双侧胆脂瘤1例

Mate Naszai, J. Ramsden
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摘要

胆脂瘤是一种罕见的疾病,每年每10万人中有9-12.6名成人和3-15名儿童患胆脂瘤[1-4],在儿科人群中表现更为积极[5]。间歇性耳漏(耳液)是一半以上胆脂瘤患者的主诉[6,7]。胆脂瘤的发病高峰期为5-15岁[8],与中耳炎[9]和外耳炎[10]的高发期有明显重叠,这两种疾病的发病方式常与胆脂瘤相同。这导致诊断可能需要数年时间。如果不及时治疗,胆脂瘤会造成严重的持久损害,表现为耳聋、眩晕、面瘫、脑膜炎和脑脓肿,这可能是致命的[11]。目前的治疗选择仅限于手术切除,目的是建立一个安全和可管理的耳朵,而维持听力是次要的。手术器械的改进提高了手术成功率,然而,翻修手术仍然是实践的主要内容。在实践中,这意味着双侧病变患者通常需要进行4次或更多次手术[12]。这对患者来说是一个很大的负担。决定确切的手术入路(管壁向上还是管壁向下)需要仔细权衡安全性与功能性,利弊必须根据现有证据和外科医生的技能进行权衡[13]。
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The chronic ear: A case report of bilateral cholesteatoma in a 10-year-old boy
Cholesteatoma is a rare condition affecting 9-12.6 adults and 3-15 children per 100,000 per annum [1–4], with a more aggressive presentation in the paediatric population [5]. Intermittent otorrhea (ear discharge) is the presenting complaint in over half of cholesteatoma patients [6, 7]. The peak incidence of cholesteatoma is 5-15 years of age [8] which overlaps significantly with a period of high incidence in otitis media [9] and externa [10], diseases that often present the same way as cholesteatoma. This results in diagnosis that may take several years. Left untreated, cholesteatoma can cause significant lasting damage in the form of deafness, vertigo, facial paralysis, meningitis, and brain abscesses which may prove fatal [11]. Current treatment options are limited to surgical excision with the aim to establish a safe and manageable ear, while maintaining hearing is secondary. Improving surgical instrumentation has allowed a better success rate, however, revision surgeries remain a mainstay of practice. In practical terms, this means that those affected by bilateral disease often undergo surgery 4 or more times [12]. This represents a significant burden for patients. The decision about the exact surgical approach (canal wall up vs canal wall down) is a careful balancing act of safety versus functionality, and the pros and cons must be weighed in light of available evidence and the skill of the surgeon [13].
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