Augustin Goran, Romic Ivan, Miličić Iva, Mikuš Mislav, Herman Mislav
Department of Surgery, School of Medicine, University of Zagreb, Zagreb 10000, Croatia.
School of Medicine University of Zagreb, Zagreb 10000, Croatia.
World J Gastrointest Surg. 2023 Aug 27;15(8):1784-1798. doi: 10.4240/wjgs.v15.i8.1784.
Choledochal cysts (CC) are cystic dilatations of the biliary tract, usually diagnosed during childhood, with an estimated incidence in the general population of 1:100000. Complications related to CC include rupture, biliary obstruction, and cholangitis. Maternal CC in pregnancy are rarely reported, and there are no guidelines on optimal management.
To systematically review maternal CC diagnosed during pregnancy or postpartum with regard to the clinical presentation of CC, the mode of treatment and delivery, and maternal outcomes.
A literature search of cases and case series of maternal CC in pregnancy and postpartum was conducted using MEDLINE/PubMed, Web of Science, Google Scholar, and Embase. There were no restrictions on language or publication year. Databases were lastly accessed on September 1, 2022.
Overall, 71 publications met the inclusion criteria, reporting 97 cases. Eighty-eight cases were diagnosed during pregnancy and nine in the puerperium. The most common symptoms were abdominal pain (81.2%) and jaundice (60.4%). Interventions for CC complications were required in 52.5% of the cases, and 34% of pregnancies were induced. Urgent cesarean section (CS) was done in 24.7%. The maternal mortality was 7.2%, while fetal mortality was inconsistently reported. Cholangitis, CC > 15 cm, and bilirubin levels > 80 mmol/L were associated with a higher likelihood of urgent CS and surgical intervention for CC. Bilirubin levels positively correlated with CC size. There was no correlation between age and cyst dimension, gestational age at cyst discovery, and CC size.
Although rare, maternal CC in pregnancy should be included in the evaluation of jaundice with upper abdominal pain. Symptomatology and clinical course are variable, and treatment may range from an expectative approach to emergent surgical CC treatment and urgent CS. While most cases were managed by conservative measures or drainage procedures, CC > 15 cm and progressive cholangitis carry the risk of CC rupture and septic complications, which may increase the rates of unfavorable maternal and fetal outcomes. Therefore, such cases require specific surgical and obstetric interventions.
胆总管囊肿（CC）是胆道的囊性扩张，通常在儿童期被诊断出来，在普通人群中的估计发病率为1:100000。与CC相关的并发症包括破裂、胆道梗阻和胆管炎。孕期母亲患CC的情况鲜有报道，并且对于最佳管理也没有相关指南。
系统回顾孕期或产后诊断出的母亲CC，涉及CC的临床表现、治疗和分娩方式以及母亲结局。
使用MEDLINE/PubMed、科学网、谷歌学术和Embase对孕期和产后母亲CC的病例及病例系列进行文献检索。对语言或出版年份没有限制。数据库最后一次访问时间为2022年9月1日。
总体而言，71篇出版物符合纳入标准，报告了97例病例。88例在孕期被诊断出来，9例在产褥期被诊断出来。最常见的症状是腹痛（81.2%）和黄疸（60.4%）。52.5%的病例需要对CC并发症进行干预，34%的妊娠被引产。24.7%进行了紧急剖宫产（CS）。母亲死亡率为7.2%，而胎儿死亡率的报告不一致。胆管炎、CC>15 cm以及胆红素水平>80 mmol/L与紧急CS和CC手术干预的可能性较高相关。胆红素水平与CC大小呈正相关。年龄与囊肿大小、发现囊肿时的孕周以及CC大小之间没有相关性。
尽管罕见，但孕期母亲CC应纳入对上腹疼痛伴黄疸的评估中。症状和临床过程各不相同，治疗方法可能从期待疗法到紧急手术治疗CC和紧急CS不等。虽然大多数病例通过保守措施或引流程序进行处理，但CC>15 cm和进行性胆管炎存在CC破裂和感染并发症的风险，这可能会增加不良母婴结局的发生率。因此，此类病例需要特定的手术和产科干预。
