Kobayashi A, Ohbe Y
Arch Dis Child. 1977 Feb;52(2):121-8. doi: 10.1136/adc.52.2.121.
Choledochal cyst was diagnosed before operation in 14 out of 16 infants and children by means of a combination of diagnostic tests. Ultrasonic echogram showed a cystic mass in all 11 patients examined. The characteristic displacement of the duodenum was noted in 9 of 14 patients by contrast studies of the upper gastrointestinal tract, and hepatobiliary scintiscanning confirmed choledochal cyst in 7 of 8 patients (88%). A cyst was shown by oral or intravenous cholangiography in 3 of 4 patients. Onset of symptoms occurred from 7 days of age to 9-5 years; in 10 patients onset occurred under 12 months of age, in 3 from 13 to 24 months, and in the remaining 3 from 2 to 9-5 years. The first symptoms were jaundice, vomiting, acholic stools, and poor sucking. Obstructive jaundice and acholic stools were found in more than 80% of the patients, and abdominal mass in 60%. Vomiting occurred in 50% of the patients, one-third of whom showed failure to thrive, and 70% of patients over the age of 1-5 years had abdominal pain. About half of the patients had a mild to moderate degree of fever. In all patients primary excision of the cyst was followed by hepaticojejunostomy in Roux-en-Y fashion. The postoperative course was uneventful in 14 patients; 2 patients died, one from ascending cholangitis and one from hepatic failure.
16例婴幼儿及儿童中,14例在术前通过综合诊断检查确诊为胆总管囊肿。超声心动图显示,所有11例接受检查的患者均有囊性肿块。14例患者中有9例通过上消化道造影检查发现十二指肠有特征性移位,8例患者中有7例(88%)经肝胆闪烁扫描确诊为胆总管囊肿。4例患者中有3例通过口服或静脉胆管造影显示有囊肿。症状出现的年龄从7天至9.5岁不等;10例患者在12个月龄以下出现症状,3例在13至24个月龄出现症状,其余3例在2至9.5岁出现症状。首发症状为黄疸、呕吐、无胆汁粪便及吸吮无力。超过80%的患者出现梗阻性黄疸和无胆汁粪便,60%的患者有腹部肿块。50%的患者出现呕吐,其中三分之一的患者生长发育迟缓,1.5岁以上患者中有70%出现腹痛。约一半的患者有轻度至中度发热。所有患者均先行囊肿一期切除,然后行Roux-en-Y式肝空肠吻合术。14例患者术后恢复顺利;2例患者死亡,1例死于上行性胆管炎,1例死于肝衰竭。