以反复心包积液为表现的婴儿心脏K-M综合征1例报告
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摘要
目的探讨心脏K-M综合征的临床特点、诊断及治疗。方法回顾分析1例心脏K-M综合征患儿的临床资料,并复习相关文献。结果患儿,男,出生33天。表现为呼吸急促、颈静脉充盈、心音低顿等。彩色多普勒超声心动图及胸部CT提示大量心包积液。血常规显示血小板计数下降。急诊行心包部分切除引流术,升主动脉根部、上腔静脉、右心耳上缘范围可见6 cm×5 cm硬质包块,固定。右室流出道及肺动脉处见长条形凸起包块,质硬,固定。右心室表面部分瘤体及左侧壁层心包病理显示为Kaposi血管内皮瘤。确诊为心脏K-M综合征。给予类固醇激素治疗。结论心脏K-M综合征是一种罕见疾病,主要根据临床表现、影像学及病理学检查诊断,可给予口服类固醇激素等治疗,必要时手术治疗。
Objective To the clinical characteristics, diagnosis and treatment of cardiac K-M syndrome. Method The clinical data of cardiac Kasabach-Merritt syndrome(K-M syndrome) in an infant were retrospectively analyzed and the related literature was reviewed. Results A 33-day-old boy presented with polypnea, jugular venous distention and low cardiac sound. Color doppler echocardiography and chest CT indicated massive pericardial effusion. Blood routine showed a decrease in platelet count. Patient was treated with acute partial resection of pericardium and drainage. Intraoperative findings included a 6 cm × 5 cm fixed firm mass on the surface of ascending aorta root, superior vena cava, the upper edge of right atrial appendage, and long strip protruding hard fixed mass seen in the right ventricular outflow tract and main pulmonary artery. Pathological findings in partial tumor of right ventricular surface and parietal pericardium showed Kaposi's hemangioendothelioma. The infant was diagnosed with cardiac K-M syndrome. The steroid therapy was administered. Conclusion Cardiac K-M syndrome is a rare disease and is primarily diagnosed by clinical manifestations, imaging and pathological examination. It can be treated by oral steroid hormones and surgery if necessary.
Objective To the clinical characteristics, diagnosis and treatment of cardiac K-M syndrome. Method The clinical data of cardiac Kasabach-Merritt syndrome(K-M syndrome) in an infant were retrospectively analyzed and the related literature was reviewed. Results A 33-day-old boy presented with polypnea, jugular venous distention and low cardiac sound. Color doppler echocardiography and chest CT indicated massive pericardial effusion. Blood routine showed a decrease in platelet count. Patient was treated with acute partial resection of pericardium and drainage. Intraoperative findings included a 6 cm × 5 cm fixed firm mass on the surface of ascending aorta root, superior vena cava, the upper edge of right atrial appendage, and long strip protruding hard fixed mass seen in the right ventricular outflow tract and main pulmonary artery. Pathological findings in partial tumor of right ventricular surface and parietal pericardium showed Kaposi's hemangioendothelioma. The infant was diagnosed with cardiac K-M syndrome. The steroid therapy was administered. Conclusion Cardiac K-M syndrome is a rare disease and is primarily diagnosed by clinical manifestations, imaging and pathological examination. It can be treated by oral steroid hormones and surgery if necessary.
引文
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[11]Jahnel J,Lackner H,Reiterer F,et al.Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon:from vincristine to sirolimus[J].Klinische Pediatrie,2012,224(06):395-397.
[12]谭小云,张靖,周少毅,等.激素抵抗型婴幼儿KasabachMerritt综合征治疗中雷帕霉素的临床应用价值探讨[J].中华小儿外科杂志,2017,38(1):20-24.
[13]Su L,Wang D,Fan X.Comprehensive therapy for hemangioma presenting with Kasabach-Merritt syndrome in the maxillofacial region[J].J Oral Maxillofac Surg,2015,73(1):92-98.
[14]Lee JH,Roychowdhury S,Nissenblatt MJ.Neuroembolization of metastatic Merkel cell cancer to the face for treatment of Kasabach-Merritt syndrome[J].BMJ Case Rep,2013.pii:bcr2012010556.
[15]Yamamoto S,Akiyama K,Kaneko R,et al.Successful lowdose radiotherapy treatment for Kasabach-Merritt syndrome[J].Pediatr International,2016,58(10):1084-1086.
[2]任松林,齐鸿燕.卡-梅现象的发病机制和治疗的研究进展[J].中华小儿外科杂志,2017,38(1):75-78.
[3]Lewis D,Vaidya R.Kasabach Merritt syndrome[M/OL].Treasure Island(FL):StatPearls Publishing,2018.
[4]Hall GW.Kasabach-Merritt syndrome:pathogenesis and management[J].Br J Haematol,2001,112(4):851-862.
[5]Wang P,Zhou W,Tao L,et al.Clinical analysis of KasabachMerritt syndrome in 17 neonates[J].BMC Pediatrics,2014,14(1):146.
[6]Freeman I,Ganesan K,Emmerson AJ.Kasabach-Merritt syndrome in a term neonate[J].Arch Dis Child Fetal Neonatal Ed,2012,97(2):139-140.
[7]Alvarez-Mendoza A,Lourdes TS,Ridaura-Sanz C,et al.Histopathology of vascular lesions found in Kasabach-Merritt syndrome:review based on 13 cases[J].Pediatr Dev Pathol,2000,3(6):556-560.
[8]Moreno JP,Rodriguez T,Bolot AL,et al.Kasabach-Merritt syndrome[J].La Revue Du Praticien,2015,65(8):1059.
[9]Wananukul S,Nuchprayoon I,Seksarn P.Treatment of Kasabach-Merritt syndrome:a stepwise regimen of prednisolone,dipyridamole,and interferon[J].Int J Dermatol,2003,42(9):741-748.
[10]Wang Z,Li K.Treatment of Kasabach-Merritt phenomenon[J].J Pediatr,2016,170:345.
[11]Jahnel J,Lackner H,Reiterer F,et al.Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon:from vincristine to sirolimus[J].Klinische Pediatrie,2012,224(06):395-397.
[12]谭小云,张靖,周少毅,等.激素抵抗型婴幼儿KasabachMerritt综合征治疗中雷帕霉素的临床应用价值探讨[J].中华小儿外科杂志,2017,38(1):20-24.
[13]Su L,Wang D,Fan X.Comprehensive therapy for hemangioma presenting with Kasabach-Merritt syndrome in the maxillofacial region[J].J Oral Maxillofac Surg,2015,73(1):92-98.
[14]Lee JH,Roychowdhury S,Nissenblatt MJ.Neuroembolization of metastatic Merkel cell cancer to the face for treatment of Kasabach-Merritt syndrome[J].BMJ Case Rep,2013.pii:bcr2012010556.
[15]Yamamoto S,Akiyama K,Kaneko R,et al.Successful lowdose radiotherapy treatment for Kasabach-Merritt syndrome[J].Pediatr International,2016,58(10):1084-1086.