Lipsett P A, Pitt H A, Colombani P M, Boitnott J K, Cameron J L
Department of Surgery, Johns Hopkins University, Baltimore, Maryland.
Ann Surg. 1994 Nov;220(5):644-52. doi: 10.1097/00000658-199411000-00007.
The authors compared the presentation, treatment, and long-term outcome of children and adults with choledochal cysts.
The typical patient with choledochal cyst disease has been the female infant with the triad of jaundice, an abdominal mass, and pain. However, the recent experience of the authors suggested that the disease currently is recognized more commonly in adults.
Forty-two patients (11 children, 32 adults) with choledochal cyst disease were treated primarily at this institution between 1976 and 1993. Patients presentation, clinical evaluation, and operative treatment were obtained from existing records. Long-term follow-up was obtained by records, physician, or direct patient contact.
One child--but no adults--had the classic triad of jaundice, abdominal mass, and pain. Children were more likely to have two of the three signs or symptoms (82% vs. 25%; p = < 0.05). Adult patients most commonly had abdominal pain and were thought to have pancreatitis (23%) or acute biliary tract symptoms, prompting cholecystectomy (50%). The type of choledochal cyst seen in children and adults was similar; the fusiform extrahepatic (Type I) was most common (50%), and the combined intrahepatic and extrahepatic (Type IVA) was the next most prominent (33%). For both children and adults, treatment consisted of excision of the cyst and biliary reconstruction with a hepaticojejunostomy. There was no surgical mortality. Gallbladder or cholangiocarcinoma was identified in three adults (9.7%), two of which were manifest on presentation. Long-term follow-up revealed one patient with a biliary stricture and three patients with Type IVA cysts who had intrahepatic stones.
Children and adults differ in presentation of choledochal cysts, with adults commonly having acute biliary tract or pancreatic symptoms. Surgical treatment with cysts excision and biliary bypass is safe and effective in children and adults with excellent long-term results that minimize the development of malignancy.
作者比较了儿童和成人胆总管囊肿的临床表现、治疗方法及长期预后。
典型的胆总管囊肿病患者为患有黄疸、腹部肿块和疼痛三联征的女婴。然而，作者近期的经验表明，目前该病在成人中更为常见。
1976年至1993年间，该机构共收治了42例胆总管囊肿病患者（11例儿童，32例成人）。患者的临床表现、临床评估及手术治疗情况均来自现有记录。通过查阅记录、医生反馈或直接与患者联系进行长期随访。
仅有1例儿童出现黄疸、腹部肿块和疼痛的经典三联征，而成人无一例出现。儿童更易出现三联征中的两项体征或症状（82% 对25%；p = < 0.05）。成年患者最常见的症状是腹痛，其中23%被认为患有胰腺炎，50%因急性胆道症状而行胆囊切除术。儿童和成人中所见的胆总管囊肿类型相似；梭形肝外囊肿（I型）最为常见（50%），肝内外联合囊肿（IVA型）次之（33%）。儿童和成人的治疗均包括囊肿切除及肝管空肠吻合术进行胆道重建。无手术死亡病例。3例成人（9.7%）被诊断为胆囊癌或胆管癌，其中2例在初诊时即有表现。长期随访发现1例患者出现胆道狭窄，3例IVA型囊肿患者有肝内结石。
儿童和成人胆总管囊肿的临床表现有所不同，成人常见急性胆道或胰腺症状。对儿童和成人而言，囊肿切除及胆道搭桥的手术治疗安全有效，长期效果良好，可将恶性肿瘤的发生风险降至最低。
