Rygl M, Snajdauf J, Petrů O, Kodet R, Kodetová D, Mixa V
Department of Pediatric Surgery, Charles University in Prague, 2nd Faculty of Medicine and Teaching Hospital in Motol, Prague, Czech Republic.
Eur J Pediatr Surg. 2006 Dec;16(6):443-8. doi: 10.1055/s-2006-924641.
The aim of the study was to describe clinical and pathological findings in children with rare congenital solitary liver cyst.
Six children with congenital liver cyst were treated at the authors' institution from 1995 to 2002. Clinical records and operative findings of these patients were reviewed retrospectively.
Age at presentation ranged from neonatal to 14 years, and there were four girls and two boys. Hepatomegaly was the most common symptom in neonates while the older children presented with abdominal pain. Ultrasound was conclusive for diagnosis in all children, prenatal ultrasound in three. Intrahepatic cyst was found in five children. Partially extrahepatic cyst in the porta hepatis was found in one. The cyst diameters varied from 8 to 10 cm. Cysts were excised via an open abdominal approach in all patients. Histological findings confirmed the diagnosis of congenital liver cyst. Columnar epithelium was found in newborns while atrophic changes of epithelial lining were seen in older children. In a case of a 13-year-old girl the cyst was lined by metaplastic squamous epithelium with foci of slight dysplasia. Immunohistochemical studies were available in three recent children with positive findings in all of them. The postoperative course was uneventful in all patients. The follow-up period ranged from 36-120 months and no recurrence was observed.
With appropriate selection of symptomatic patients, total excision of a congenital liver cyst is a safe procedure with excellent long-term results. Given the possibility of malignant degeneration of a congenital liver cyst we recommend its surgical excision in most cases.
本研究旨在描述患有罕见先天性孤立性肝囊肿儿童的临床和病理表现。
1995年至2002年,作者所在机构对6例先天性肝囊肿患儿进行了治疗。对这些患者的临床记录和手术结果进行了回顾性分析。
就诊年龄从新生儿到14岁不等,其中有4名女孩和2名男孩。肝肿大是新生儿最常见的症状,而年龄较大的儿童则表现为腹痛。超声对所有儿童的诊断都具有决定性意义,3例为产前超声诊断。5例儿童发现肝内囊肿。1例在肝门处发现部分肝外囊肿。囊肿直径从8厘米到10厘米不等。所有患者均通过开腹手术切除囊肿。组织学检查结果证实为先天性肝囊肿。新生儿可见柱状上皮,而年龄较大的儿童可见上皮衬里萎缩性改变。在1例13岁女孩中,囊肿内衬化生鳞状上皮,有轻度发育异常灶。对最近3例患儿进行了免疫组织化学研究,结果均为阳性。所有患者术后病程平稳。随访时间为36至120个月,未观察到复发。
对于有症状的患者进行适当选择,先天性肝囊肿的完全切除是一种安全的手术,长期效果良好。鉴于先天性肝囊肿有恶变的可能,我们建议在大多数情况下进行手术切除。