Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.
Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.
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儿童的先天性门体系统分流：协会，并发症和结果。
DOI：
10.1007/s10620-019-05834-w
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发表时间：
2020-04
影响因子：
3.1
通讯作者：
Nathan JD
中科院分区：
文献类型：
--
作者：
DiPaola F;Trout AT;Walther AE;Gupta A;Sheridan R;Campbell KM;Tiao G;Bezerra JA;Bove KE;Patel M;Nathan JD
先天性门体分流（CPSS）是一种罕见的畸形，其中内脏静脉血流绕过肝脏。CPSS与其他先天性异常和综合征有关，并可能伴有危及生命的并发症。CPSS及其治疗在文献中报道不足。在此，我们回顾了来自两个儿科中心的一组儿童和青年CPSS患者的临床特征、治疗和结果。
对辛辛那提儿童医院医疗中心和C.S.莫特儿童医院的CPSS病例进行了回顾，以明确CPSS的解剖结构、相关异常、并发症、干预措施和结果。详细描述了肝脏病变的影像学特征和组织病理学。
共确定了11例病例。中位年龄为10岁（范围0 - 26岁）；8例（73%）为女性。相关异常包括6例内脏异位（55%）、5例先天性心脏病（45%）、3例特纳综合征（27%）以及2例脐膨出、膀胱外翻、肛门闭锁、脊柱缺陷（OEIS）综合征（18%）。8例（73%）有高氨血症±脑病。1例4个月大的患儿出现肝肺综合征，1例12岁患儿出现肺动脉高压。8例患者（73%）有肝脏病变，包括5例癌前腺瘤和3例高分化肝细胞癌（HCC）。4名儿童成功进行了CPSS封堵/结扎。3名儿童因HCC接受了肝移植（2例）或肝切除（1例），在长期随访中无复发。
CPSS与多种异常（内脏异位、先天性心脏病）和综合征（特纳综合征）有关。由于存在癌前腺瘤和HCC的风险，应非常仔细地评估CPSS肝脏病变。CPSS的严重并发症可在年轻时发生，但可通过血管内治疗或开放手术进行处理。
Congenital portosystemic shunt (CPSS) is a rare malformation in which splanchnic venous flow bypasses the liver. CPSS is associated with other congenital anomalies and syndromes and can be associated with life-threatening complications. CPSS and their management remain underreported in the literature. Here, we review the clinical characteristics, management, and outcomes of a cohort of children and young adults with CPSS from two pediatric centers. Cases of CPSS from Cincinnati Children’s Hospital Medical Center and C.S. Mott Children’s Hospital were reviewed to define CPSS anatomy, associated anomalies, complications, interventions, and outcomes. The imaging features and histopathology of liver lesions were characterized in detail. A total of 11 cases were identified. Median age was 10 years (range 0–26); 8 (73%) cases were female. Associated anomalies included six patients with heterotaxy (55%), five patients with congenital heart disease (45%), three patients with Turner syndrome (27%), and two patients with omphalocele, exstrophy, imperforate anus, spinal defects (OEIS) complex (18%). Eight (73%) cases had hyperammonemia ± encephalopathy. A 4-month-old presented with hepatopulmonary syndrome, and 12-year-old presented with pulmonary hypertension. Eight patients (73%) had liver lesions including five with premalignant adenomas and three with well-differentiated hepatocellular carcinoma (HCC). Four children underwent successful CPSS occlusion/ligation. Three children underwent liver transplant (2) or resection (1) for HCC without recurrence at extended follow-up. CPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.
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影响因子：
3.1
作者：
Iida, Taku;Ogura, Yasuhiro;Uemoto, Shinji
通讯作者：
Uemoto, Shinji
DOI：
10.1016/j.jvir.2010.01.038
发表时间：
2010-06-01
影响因子：
2.9
作者：
Kuo, Michael D.;Miller, Franklin J.;Finch, Michael
通讯作者：
Finch, Michael
影响因子：
1.4
作者：
Nagata, Hazumu;Yamamura, Kenichiro;Hara, Toshiro
通讯作者：
Hara, Toshiro
影响因子：
9.6
作者：
JOYCE, AD;HOWARD, ER
通讯作者：
HOWARD, ER
影响因子：
13.5
作者：
Kojiro, Masamichi;Wanless, Ian R.;Toniakos, Dina
通讯作者：
Toniakos, Dina