Hsu Wei-Fan, Yu Cheng-Ju, Yeh Chun-Chieh, Huang Wen-Hsin, Peng Cheng-Yuan
Division of Hepatogastroenterology, Department of Internal Medicine, China Medical University Hospital.
Graduate Institute of Biomedical Sciences, China Medical University.
Medicine (Baltimore). 2018 Oct;97(40):e12552. doi: 10.1097/MD.0000000000012552.
Lymphangiomatosis is rare and benign, and slowly proliferating lymphatic vessels of unknown etiology and visceral lymphangiomatosis involving the spleen is rare. Since lymphangiomatosis may be asymptomatic or present as a sense of fullness, splenic cystic lymphangiomatosis is a disease of little concern.
A 34-year-old woman suffering from progressive epigastric fullness after oral intake for two weeks.
Physical examination showed a palpable mass which was more than 10 cm in size over the left hypochondrium. An abdominal computed tomography disclosed marked splenomegaly with multiple cystic lesions in the spleen, causing external compression with right-sided deviation of the adjacent organs and varices in the upper abdomen. Esophagogastroduodenoscopy revealed portal hypertensive gastropathy.
Conventional total splenectomy was performed in this patient because of an enlarged spleen and unknown etiology, preoperatively. Upon surgery, splenomegaly with polycystic content and varicose vessels over the omentum were noted. Autologous spleen transplantation was not performed because of limited orthotopic and vascularized spleen.
The patient is doing well 18 months after splenectomy.
This was a rare case presenting with splenic cystic lymphangiomatosis in association with omental varices and portal hypertension. Splenic cystic lymphangiomatosis should be considered in the differential diagnosis of patients with a palpable painless mass over the left hypochondrium.
淋巴管瘤病罕见且为良性，病因不明的淋巴管缓慢增生，累及脾脏的内脏淋巴管瘤病更是罕见。由于淋巴管瘤病可能无症状或仅表现为饱胀感，脾囊性淋巴管瘤病是一种不太受关注的疾病。
一名34岁女性，进食后上腹部进行性饱胀两周。
体格检查发现左季肋部可触及一个直径超过10厘米的肿块。腹部计算机断层扫描显示脾脏明显肿大，脾内有多个囊性病变，导致相邻器官受压并向右移位，上腹部有静脉曲张。食管胃十二指肠镜检查显示门静脉高压性胃病。
由于脾脏肿大且病因不明，术前对该患者实施了传统全脾切除术。手术中，发现脾脏肿大，有多个囊肿，大网膜上有曲张血管。由于原位和带血管化脾脏有限，未进行自体脾移植。
脾切除术后18个月，患者情况良好。
这是一例罕见的脾囊性淋巴管瘤病合并大网膜静脉曲张和门静脉高压病例。对于左季肋部可触及无痛性肿块的患者，鉴别诊断时应考虑脾囊性淋巴管瘤病。
