Fukuda Ryo, Ohara Hirotoshi, Shimizu Yusuke, Nagatsuka Taiki, Ntege Edward H
Plastic and Reconstructive Surgery, University of the Ryukyus Hospital, Ginowan, JPN.
Plastic and Reconstructive Surgery, International University of Health and Welfare, Mita Hospital, Tokyo, JPN.
Cureus. 2025 Jun 30;17(6):e87074. doi: 10.7759/cureus.87074. eCollection 2025 Jun.
Benign symmetric lipomatosis (BSL), or Madelung disease, is a rare condition characterized by non-encapsulated, symmetrical adipose tissue accumulation. Although uncommon, circumferential cervical involvement can pose a life-threatening risk of airway obstruction. We report a 58-year-old Japanese man with chronic alcohol dependence presenting with progressive bilateral neck swelling, dysphagia, and nocturnal respiratory distress. Initial magnetic resonance imaging revealed 21 mm of adipose tissue at the C4/5 level. Following posterior cervical lipectomy and subsequent disease recurrence, imaging five years later demonstrated progression to 70 mm of circumferential adipose proliferation with impending airway compromise. A quadrant-based, four-stage open resection protocol was performed between March 2023 and February 2024, utilizing sequential lateral, anterior, and posterior approaches. This staged approach successfully avoided tracheostomy while removing approximately 2,100 grams of adipose tissue. At 16-month follow-up, cervical thickness reduced to 48 mm with complete resolution of dysphagia and respiratory symptoms. Residual submental laxity from platysmal diastasis and submandibular gland hypertrophy remained, though secondary procedures were deferred due to questionable alcohol abstinence evidenced by persistently elevated γ-GTP levels. This case demonstrates that systematic staged resection can safely manage circumferential cervical BSL while preserving airway integrity. However, sustained alcohol abstinence remains critical for preventing recurrence, and comprehensive functional assessments should guide future management protocols for this rare condition.
良性对称性脂肪瘤病（BSL），又称马德隆病，是一种罕见疾病，其特征为非包膜性、对称性脂肪组织堆积。尽管并不常见，但颈部周围受累可带来危及生命的气道梗阻风险。我们报告一例58岁慢性酒精依赖的日本男性，表现为进行性双侧颈部肿胀、吞咽困难和夜间呼吸窘迫。初始磁共振成像显示C4/5水平有21毫米的脂肪组织。在进行颈椎后路脂肪切除术及随后疾病复发后，五年后的影像学检查显示圆周性脂肪增生进展至70毫米，即将出现气道受压。在2023年3月至2024年2月期间，采用基于象限的四阶段开放切除方案，依次采用外侧、前方和后方入路。这种分期方法成功避免了气管切开术，同时切除了约2100克脂肪组织。在16个月的随访中，颈部厚度减至48毫米，吞咽困难和呼吸症状完全缓解。尽管由于γ-谷氨酰转肽酶水平持续升高表明戒酒情况存疑，导致二期手术推迟，但仍存在因颈阔肌分离和下颌下腺肥大引起的颏下松弛残留。该病例表明，系统性分期切除可在保留气道完整性的同时安全处理颈部周围的BSL。然而，持续戒酒对于预防复发仍然至关重要，全面的功能评估应指导针对这种罕见疾病的未来管理方案。
