Mito Kumiko, Amano Yusuke, Oshiro Hisashi, Matsubara Daisuke, Fukushima Noriyoshi, Ono Shigeru
Department of Diagnostic Pathology, Jichi Medical University Hospital.
Division of Pediatric Surgery, Department of Surgery, Jichi Medical University School of Medicine, Shimotsuke, Tochigi, Japan.
Medicine (Baltimore). 2019 Jan;98(4):e14211. doi: 10.1097/MD.0000000000014211.
Liver heterotopia associated with congenital diaphragmatic hernia (CDH) is a rare condition; to the best of our knowledge, only 17 cases have been reported to date. The histogenesis and clinicopathological features are largely unknown. We herein report 2 cases of liver heterotopia associated with CDH along with 17 cases described in the literature to shed light on their clinicopathological characteristics.
Case 1 was a vaginally delivered male newborn who presented with respiratory distress immediately after birth. Case 2 was a female fetus who was found to have left-sided CDH during gestation.
In case 1, a chest X-ray revealed left-sided CDH. In case 2, magnetic resonance imaging performed at 33 weeks of gestation revealed left-sided CDH.
Case 1 underwent diaphragmatic patch repair surgery 3 days after birth. Histopathological examination following surgery in case 1 revealed the presence of ectopic liver tissue in the hernia sac. Case 2 was delivered by Cesarean section, and diaphragmatic patch surgery was performed 3 days after birth. During surgery, an isolated nodule was identified on the peritoneal side of the border of the defective foramen of the diaphragm. Histopathological examination following surgery in case 2 confirmed the presence of an epidermal cyst in the hernia sac. In addition, the isolated nodule was histopathologically found to be ectopic liver tissue.
In Case 1, CDH recurred at 6 months after surgery, and a second patch repair surgery was performed. The surgically removed hernia sac was found to contain microscopic ectopic liver tissue on histopathology. Case 1 recovered well after surgery, and there was no critical change during the 10-month postoperative period. Case 2 recovered well after surgery, and there was no critical change during the 20-month postoperative period.
There were no secondary pathological conditions associated with the presence of ectopic liver in CDH, such as torsion, infarction, rupture, intra-abdominal bleeding, or tumorization. Our observations suggest that liver heterotopia is a rare but asymptomatic condition in patients with CDH.
与先天性膈疝（CDH）相关的肝脏异位是一种罕见病症；据我们所知，迄今为止仅报道了17例。其组织发生及临床病理特征大多未知。我们在此报告2例与CDH相关的肝脏异位病例，并结合文献中描述的17例病例，以阐明其临床病理特征。
病例1是一名经阴道分娩的男性新生儿，出生后立即出现呼吸窘迫。病例2是一名女性胎儿，在孕期被发现患有左侧CDH。
病例1中，胸部X线显示左侧CDH。病例2中，孕33周时进行的磁共振成像显示左侧CDH。
病例1出生后3天接受了膈肌补片修复手术。病例1术后的组织病理学检查显示疝囊内存在异位肝组织。病例2通过剖宫产分娩，出生后3天进行了膈肌补片手术。手术期间，在膈肌缺损孔边缘的腹膜侧发现一个孤立结节。病例2术后的组织病理学检查证实疝囊内存在表皮样囊肿。此外，经组织病理学检查发现该孤立结节为异位肝组织。
病例1术后6个月CDH复发，进行了第二次补片修复手术。手术切除的疝囊在组织病理学上发现含有微小的异位肝组织。病例1术后恢复良好，术后10个月期间无严重变化。病例2术后恢复良好，术后20个月期间无严重变化。
CDH中异位肝的存在未伴有诸如扭转、梗死、破裂、腹腔内出血或肿瘤形成等继发性病理状况。我们的观察结果表明，肝脏异位在CDH患者中是一种罕见但无症状的病症。
