Choudhary Manita, Chen Yufei, Friedman Oren, Cuk Natasha, Ben-Shlomo Anat
Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, California.
Department of Surgery, Cedars-Sinai Medical Center, Los Angeles, California.
AACE Clin Case Rep. 2021 Mar 26;7(5):310-314. doi: 10.1016/j.aace.2021.03.008. eCollection 2021 Sep-Oct.
Pheochromocytoma (PCC) crisis caused by acute catecholamine release from an adrenal PCC or extra-adrenal paraganglioma can be difficult to diagnose and may require an unconventional management strategy to achieve good outcomes. We describe a case of PCC crisis presenting with acute respiratory distress syndrome (ARDS) that resolved with stabilization on veno-venous (VV) extracorporeal membrane oxygenation (ECMO) during adrenalectomy.
A 30-year-old man with a history of severe alcohol use disorder and a prior hospital admission for alcohol withdrawal syndrome presented with sudden-onset hemoptysis, altered mental status, and severe dyspnea that rapidly deteriorated to ARDS requiring ECMO support. He demonstrated hemodynamic collapse after cannulation for VV-ECMO and stabilized after conversion to veno-arterial-ECMO, but ARDS persisted and he developed acute renal failure. Computed tomography without contrast done as part of work-up for a presumed infection revealed a 6.9 × 6.4 cm right adrenal mass suspicious for pheochromocytoma. Plasma and random urine metanephrine levels were markedly elevated. ARDS persisted despite α- and β-adrenoreceptor blockade, and he underwent laparoscopic right adrenalectomy with VV-ECMO support. Pathology confirmed PCC with intermediate risk for malignancy. Postoperatively, he was weaned off respiratory and renal support within 10 days, showed rapid clinical improvement, and was discharged 1 month later.
This case highlights diagnostic and management challenges associated with patients with PCC crisis presenting with ARDS. A multidisciplinary team approach is critical to identifying appropriate treatment strategies.
由肾上腺嗜铬细胞瘤(PCC)或肾上腺外副神经节瘤急性释放儿茶酚胺引起的PCC危象可能难以诊断,可能需要采用非常规的管理策略才能取得良好的治疗效果。我们描述了一例PCC危象患者,其表现为急性呼吸窘迫综合征(ARDS),在肾上腺切除术期间通过静脉-静脉(VV)体外膜肺氧合(ECMO)稳定病情后得以缓解。
一名30岁男性,有严重酒精使用障碍病史,曾因酒精戒断综合征住院,出现突发咯血、精神状态改变和严重呼吸困难,并迅速恶化为需要ECMO支持的ARDS。他在接受VV-ECMO插管后出现血流动力学崩溃,在转换为静脉-动脉-ECMO后病情稳定,但ARDS持续存在,且出现了急性肾衰竭。作为对疑似感染进行检查的一部分而进行的非增强计算机断层扫描显示右肾上腺有一个6.9×6.4 cm的肿块,怀疑为嗜铬细胞瘤。血浆和随机尿甲氧基肾上腺素水平显著升高。尽管使用了α和β肾上腺素能受体阻滞剂,ARDS仍持续存在,他在VV-ECMO支持下接受了腹腔镜右肾上腺切除术。病理证实为PCC,恶性风险为中度。术后,他在10天内脱离了呼吸和肾脏支持,临床迅速改善,并于1个月后出院。
本病例突出了与伴有ARDS的PCC危象患者相关的诊断和管理挑战。多学科团队方法对于确定适当的治疗策略至关重要。