313.4 📋 章末速記 Summary

313.4.1 🔑 一句話總結

Mediastinum 分 anterior (4 Ts: thymoma + teratoma + thyroid + terrible lymphoma)、middle (LAD + bronchogenic cyst + aortic aneurysm)、posterior (neurogenic tumors)；mediastinitis acute (Boerhaave, post-cardiac surgery — emergency) vs chronic fibrosing (histoplasmosis + IgG4 + idiopathic)；pneumomediastinum 多由 Macklin effect (alveolar rupture from cough, vomiting, MV)；diaphragm disorders: unilateral paralysis (often asymptomatic) vs bilateral (severe orthopnea); diaphragmatic hernias (Bochdalek, Morgagni); eventration; hiccups；chest wall：pectus excavatum (Nuss procedure)、kyphoscoliosis (restrictive)、ankylosing spondylitis；neuromuscular weakness：ALS、GBS、MG、muscular dystrophy → NIV；OHS (BMI ≥ 30 + PaCO2 > 45 awake) treated with NIV + weight loss；CCHS / Ondine’s curse = PHOX2B mutation + lifelong ventilation。

313.4.2 💊 治療精要

  • thymoma：surgical resection + chemotherapy/RT for invasive；pembrolizumab refractory (caution paraneoplastic)
  • lymphoma (mediastinal)：chemotherapy ± RT；PMBCL (DA-R-EPOCH 90% cure)；Hodgkin (ABVD)
  • germ cell：seminoma (cisplatin + RT)；NSGCT (BEP chemo)
  • acute mediastinitis：emergency surgical exploration + IV broad-spectrum antibiotics
  • chronic fibrosing：treat underlying (histoplasmosis = antifungal in active; IgG4 = steroids + rituximab)
  • diaphragm paralysis unilateral symptomatic：plication (VATS or open)
  • OHS：CPAP / BiPAP + weight loss (bariatric surgery very effective)
  • pectus excavatum：Nuss procedure for symptomatic Haller index > 3.25
  • neuromuscular respiratory failure：NIV (BiPAP); ALS = NIV + riluzole + edaravone + AMX0035

313.4.3 🎯 盧醫師的考前提醒

  1. anterior mediastinum 4 Ts：Thymoma (MG 30-40%) + Teratoma/germ cell (β-hCG, AFP) + Thyroid (substernal goiter) + Terrible lymphoma (Hodgkin, PMBCL, DLBCL)
  2. thymoma + MG：anti-AChR / anti-MuSK；thymectomy can help even non-thymomatous MG (MGTX trial 2016)
  3. acute mediastinitis 兩大原因：post-cardiac surgery (S. aureus 50%) + Boerhaave (esophageal rupture post-vomiting)；都是 surgical emergency；mortality 10-30%
  4. Boerhaave syndrome：spontaneous esophageal rupture from violent vomiting → left posterior wall；high amylase in pleural fluid + acidic pH；CT with oral contrast diagnostic
  5. pneumomediastinum 多 self-limited：Macklin effect (alveolar rupture)；但要 rule out Boerhaave + tracheobronchial injury
  6. chronic fibrosing mediastinitis：US 大多 histoplasmosis (Ohio/Mississippi endemic)；其他 sarcoid + TB + IgG4-related disease + idiopathic
  7. bilateral diaphragm paralysis：orthopnea worse supine (abdominal contents push diaphragm up)；severe respiratory failure；NIV + diaphragm pacing for select；ALS 是常見原因
  8. OHS diagnosis：BMI ≥ 30 + PaCO2 > 45 awake；often OSA + OHS overlap；treatment = CPAP/BiPAP + bariatric surgery 是 single most effective
  9. CCHS (Ondine’s curse)：PHOX2B mutation；automatic breathing 喪失 during sleep；lifelong nocturnal ventilation + diaphragmatic pacing；diagnosis 多在 pediatric
  10. Nuss procedure for pectus excavatum：minimally invasive substernal bar；symptomatic + Haller index > 3.25；adolescents to young adults；bar removed after 2-3 years