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产前发现的肝囊肿:病因和特征,干预指征。

Antenatally-detected liver cysts: Causes and characteristics, indications for intervention.

机构信息

Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London, UK SE5 9RS.

出版信息

J Pediatr Surg. 2020 Mar;55(3):441-445. doi: 10.1016/j.jpedsurg.2019.03.023. Epub 2019 May 1.

Abstract

AIM

The prevalence of antenatally-detected hepatic cysts is increasing owing to increased use of maternal ultrasonography (USS). Diagnostic precision, however, is challenging and subsequently there is no clear consensus on postnatal management. The aim of the study was to evaluate the natural history and long-term follow up of congenital simple hepatic cysts.

METHODS

Single-center review of prospectively-maintained dataset collected over a 25-year period. Data are quoted as median (range).

RESULTS

In the period 1991-2016, 31 infants presented with an antenatally-detected cyst which, on postnatal imaging, was likely confirmed as a simple parenchymal hepatic cyst. These together with a further infant who presented at 4 days without any antenatal imaging comprised the study group (n = 32). Gestational age at detection was 23 (13-38) weeks. Maximum antenatal cyst diameter was measured at 19 (4-120) mm. Only the largest required percutaneous aspiration at 35 weeks' gestation being associated with polyhydramnios. Postnatally, serial USS was performed alongside MRI/CT or liver scintigraphy if there was diagnostic doubt. Initial maximum cyst diameter was 30 (12-120) mm. Five infants came to surgical intervention and this included excision ± marsupialization (n = 4), and a cyst-jejunostomy en Roux in one child thought to have a connection with the biliary tract on intraoperative cholangiography. The mucosal lining in two cysts showed areas of squamous metaplasia, with one more showing an intact squamous lining probably more consistent with an epidermoid cyst. In the remaining cases (n = 27), all asymptomatic, serial USS showed volume maintenance (n = 16) actual volume regression (n = 4) and resolution (n = 7). Median follow-up in these cases was 42 (4-252) months.

CONCLUSION

This is the largest case series to date of antenatally-detected simple hepatic cysts. Most can be managed conservatively, and relative regression or resolution is likely. Surgical intervention should be reserved for those: that are large at the outset; show rapid cyst growth - these being typically exophytic in nature; wall irregularity or where there is diagnostic doubt.

TYPE OF STUDY

Case series.

LEVEL OF EVIDENCE

Level IV.

摘要

目的

由于母体超声检查(USS)的广泛应用,产前发现的肝囊肿的患病率正在增加。然而,其诊断精度具有挑战性,因此对于其出生后的处理并没有明确的共识。本研究旨在评估先天性单纯性肝囊肿的自然病史和长期随访结果。

方法

回顾性分析 25 年间在单中心前瞻性维护的数据集。数据以中位数(范围)表示。

结果

在 1991 年至 2016 年间,31 名婴儿在产前检查中发现了一个囊肿,在产后影像学检查中,该囊肿很可能被证实为单纯的实质肝囊肿。其中包括另一名在 4 天无任何产前影像学检查时就诊的婴儿,总共 32 名婴儿纳入了研究组。发现囊肿时的胎龄为 23(13-38)周。最大的产前囊肿直径为 19(4-120)mm。仅最大的囊肿需要在 35 周妊娠时进行经皮抽吸,这与羊水过多有关。产后,若存在诊断疑虑,则进行连续 USS 检查,并联合 MRI/CT 或肝闪烁扫描。初始最大囊肿直径为 30(12-120)mm。有 5 名婴儿接受了手术干预,包括切除+造袋术(n=4),以及在术中胆管造影显示与胆道有连接的情况下,进行囊肿空肠吻合术(n=1)。两个囊肿的黏膜衬里显示出鳞状上皮化生的区域,其中一个更显示出完整的鳞状上皮,这更符合表皮样囊肿。在其余病例(n=27)中,所有婴儿均无症状,连续 USS 检查显示囊肿体积保持不变(n=16)、实际体积缩小(n=4)和囊肿消失(n=7)。这些病例的中位随访时间为 42(4-252)个月。

结论

这是迄今为止最大的一组产前发现的单纯性肝囊肿病例系列。大多数病例可以保守治疗,并且可能会出现相对的消退或消失。手术干预应保留给那些:囊肿在初始时较大;囊肿生长迅速——这些囊肿通常为外生性;囊壁不规则或存在诊断疑虑。

研究类型

病例系列。

证据等级

IV 级。

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