Di Paolo Marcello, Di Gaeta Alessandro, Indino Elena Lucia, Mordenti Michela, Palange Paolo
Department of Public Health and Infectious Diseases, "Sapienza" University of Rome, Rome, Italy.
Department of Radiology, Oncology and Pathology, "Sapienza" University of Rome, Rome, Italy.
Respir Med Case Rep. 2020 Feb 5;29:101018. doi: 10.1016/j.rmcr.2020.101018. eCollection 2020.
Pancreatic cystosis (PC) is an uncommon manifestation of pancreas involvement in cystic fibrosis (CF), characterized by the presence of multiple macrocysts partially or completely replacing pancreas. Only few reports are available from literature and management (surgery vs follow up) is commonly based on the presence of symptoms or complications due to local mass effect, although evidence-based recommendations are still not available. We here report the case of a young adult CF patient with PC, in which cardiopulmonary exercise testing (CPET) provided important information to be integrated to the radiological finding of inferior vena cava compression by the multicystic pancreas complex. Through the analysis of oxygen kinetic cardiodynamic phase pattern, CPET may be helpful to safely exclude significant mass effects on blood venous return and to improve the decision-making process on whether to consider surgery or not in patients with PC.
胰腺囊肿病（PC）是胰腺受累于囊性纤维化（CF）的一种罕见表现，其特征是存在多个大囊肿，部分或完全替代胰腺。文献中仅有少数报道，治疗（手术与随访）通常基于局部肿块效应引起的症状或并发症，尽管尚无循证医学推荐意见。我们在此报告一例患有PC的年轻成年CF患者，其中心肺运动试验（CPET）提供了重要信息，可用于综合分析多囊性胰腺复合体对下腔静脉压迫的影像学表现。通过分析氧动力学心脏动力学相位模式，CPET可能有助于安全地排除对静脉血回流的显著肿块效应，并改善PC患者是否考虑手术的决策过程。
