CAJ | 학술논문

目的总结儿童胰腺胸膜瘘(PPF)的临床特点,提高对该病的认识,以期早期诊断及治疗.方法以首都医科大学附属北京儿童医院2007年12月至2014年3月确诊的PPF病例为研究对象,收集病例的临床资料、实验室检查、影像学检查及治疗、预后等资料,并进行描述性分析.结果诊断PPF患儿5例,年龄2岁～10岁5个月,病史1～6个月余.5例患儿中最主要的临床表现为胸闷(3例)、胸痛(3例)、发热(3例)、喘息(1例)、呼吸困难(1例),入院主诉中有腹痛、腹胀症状者1例.5例患儿均存在大量胸腔积液,累及右侧3例,左侧1例,双侧1例;1例合并大量腹水.5例患儿胸水淀粉酶均明显升高(＞1 000 U/L),最高者＞50 000U/L.5例患儿行腹部B超发现胰腺病变4例;行磁共振胰胆管造影(MRCP)均显示胰腺形态学改变,合并胰腺假性囊肿4例,发现PPF 4例.5例患儿均先行内科保守治疗,但病情反复,后3例行经内镜逆行性胰胆管造影术检查并予胰管支架治疗,2例行外科手术治疗.结论儿童PPF较罕见,临床以大量胸腔积液伴随的肺部症状为主要表现,胸水淀粉酶水平明显升高,PPF的诊断通常依赖于影像学检查,MRCP能够清晰地显示胰管及与瘘管的关系,且相对于经内镜逆行性胰胆管造影术的无创性特点,可作为诊断PPF的首选检查.对于内科治疗效果不佳的PPF患儿可行内镜下治疗或外科手术.
목적 총결인동이선흉막루(PPF)적림상특점,제고대해병적인식,이기조기진단급치료.방법 이수도의과대학부속북경인동의원2007년12월지2014년3월학진적PPF병례위연구대상,수집병례적림상자료、실험실검사、영상학검사급치료、예후등자료,병진행묘술성분석.결과 진단PPF환인5례,년령2세～10세5개월,병사1～6개월여.5례환인중최주요적림상표현위흉민(3례)、흉통(3례)、발열(3례)、천식(1례)、호흡곤난(1례),입원주소중유복통、복창증상자1례.5례환인균존재대량흉강적액,루급우측3례,좌측1례,쌍측1례;1례합병대량복수.5례환인흉수정분매균명현승고(＞1 000 U/L),최고자＞50 000U/L.5례환인행복부B초발현이선병변4례;행자공진이담관조영(MRCP)균현시이선형태학개변,합병이선가성낭종4례,발현PPF 4례.5례환인균선행내과보수치료,단병정반복,후3례행경내경역행성이담관조영술검사병여이관지가치료,2례행외과수술치료.결론 인동PPF교한견,림상이대량흉강적액반수적폐부증상위주요표현,흉수정분매수평명현승고,PPF적진단통상의뢰우영상학검사,MRCP능구청석지현시이관급여루관적관계,차상대우경내경역행성이담관조영술적무창성특점,가작위진단PPF적수선검사.대우내과치료효과불가적PPF환인가행내경하치료혹외과수술.
Objective To summarize the clinical features of the pancreaticopleural fistula (PPF) in children in order to improve the understanding of PPF and to make early diagnosis and treatment of the disease.Methods Five cases of pediatric PPF in Beijing Children's Hospital Affiliated to Capital Medical University from December 2007 to March 2014 were studied by retrospective analysis.The clinical features, laboratory results, image characteristics, treatment and prognosis were reviewed.Results Five cases of PPF were diagnosed aged between 2 years to 10 years and 5 months old, history from more than 1 month to 6 months.The main symptoms were chest tightness (3 cases), chest pain (3 cases) , fever(3 cases) , wheeze (1 case) , dyspnea (1 case).Only 1 case was with abdominal pain and abdominal distension when he was admitted to hospital.All patients had massive pleural effusions, included right side (3 cases),left side(1 case) ,bilateral sides(1 case), 1 case complicated with massive ascites.Pleural fluid amylase of all the cases was significantly elevated (＞ 1 000 U/L) ,the highest was more than 50 000 U/L.Four cases had positive findings of pancreas by transabdominal ultrasound.Five cases had morphological changes in pancreas by magnetic resonance cholangiopancreatography (MRCP).Four cases had PPF and pancreatic pseudocysts respectively.Conservative treatment was given to 5 cases, but further 3 cases reveived stent insertion by endoscopic retrograde cholangiopanereatography, and 2 cases reveived surgical therapy.Conclusions PPF is rare in children, the main clinical feature is massive pleural effusion with respiratory symptoms.Pleural fluid amylase would be significantly elevated.The diagnosis of PPF generally relies on imaging, MRCP is considered the imaging study of choice for PPF due to its superiority in identifying a fistula in the pancreatic region and its noninvasiveness as compared to endoscopic retrograde cholangio pancreatography.Endoscopic and surgical therapy can be used if internal therapy is not satisfactory.