Kitano Yoshihiro, Matsuoka Kentaro, Honna Toshiro, Kuroda Tatsuo, Morikawa Nobuyuki, Hayashi Satoshi, Sago Haruhiko
Division of General Surgery, National Center for Child Health and Development, Setagaya-ku, Tokyo 157-8535, Japan.
J Pediatr Surg. 2006 Mar;41(3):490-4. doi: 10.1016/j.jpedsurg.2005.10.080.
BACKGROUND/PURPOSE: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by massive pleural effusion (PE) leading to fetal hydrops. The underlying mechanisms as well as the origin of the fluid are not well understood. This study was performed to find a histological hallmark of an EPS with massive PE. We hypothesized that venous obstruction has a role to play in the mechanisms of fluid production by EPS.
We recently experienced 3 cases of fetal EPS complicated by fetal hydrothorax requiring thoracentesis and eventually thoracoamniotic shunt placement. Total protein content and cell count were measured in the aspirates, which were compared with chylothorax cases (n = 5) requiring fetal shunt placement. After birth, all 3 infants required mass resection for the control of PE. The venous wall thickness was measured on pathology slides stained with Elastica van Gieson staining. Thickness of the media and adventitia was measured in approximately 40 veins per case. They were corrected by external diameter and expressed as percentage of medial thickness and percentage of adventitial thickness. An EPS not associated with PE but with congenital diaphragmatic hernia served as a control.
Total protein and the cell count of the EPS related PE were 0.6 +/- 0.3 mg/dL and 28 +/- 14/microL (mean +/- SD), which were significantly lower than those of PE in chylothorax (2.2 +/- 0.2 mg/dL and 1900 +/- 1100/microL). Percentages of adventitial thickness of EPS with PE were 9.8% +/- 3.8%, 10.4% +/- 3.6%, and 8.3% +/- 3.7%, which were significantly increased compared with the control of 3.1% +/- 1.3% (P < .01). Percentages of medial thickness of EPS with PE were 7.0% +/- 1.9%, 7.3% +/- 1.4%, and 6.6% +/- 2.3%, which were significantly increased compared with the control of 2.3% +/- 0.7% (P < .01).
We conclude that PE associated with EPS is the transudate rather than the lymph. The thickened media and the adventitia found in EPS with PE support the hypothesis that partial obstruction of the venous system leads to an increased transudate production, which ultimately leads to fetal hydrops.
背景/目的：胎儿叶外型肺隔离症（EPS）有时会并发大量胸腔积液（PE），导致胎儿水肿。其潜在机制以及液体的来源尚不清楚。本研究旨在寻找伴有大量PE的EPS的组织学特征。我们假设静脉阻塞在EPS产生液体的机制中起作用。
我们最近遇到3例胎儿EPS并发胎儿胸腔积液，需要进行胸腔穿刺，最终放置胸腔羊膜分流管。测量抽吸液中的总蛋白含量和细胞计数，并与需要放置胎儿分流管的乳糜胸病例（n = 5）进行比较。出生后，所有3例婴儿都需要进行大面积切除以控制PE。在经弹性范吉森染色的病理切片上测量静脉壁厚度。每例测量约40条静脉的中膜和外膜厚度。通过外径进行校正，并表示为中膜厚度百分比和外膜厚度百分比。以不伴有PE但伴有先天性膈疝的EPS作为对照。
与EPS相关的PE的总蛋白和细胞计数分别为0.6±0.3mg/dL和28±14/μL（平均值±标准差），显著低于乳糜胸中PE的水平（2.2±0.2mg/dL和1900±1100/μL）。伴有PE的EPS的外膜厚度百分比分别为9.8%±3.8%、10.4%±3.6%和8.3%±3.7%，与对照组的3.1%±1.3%相比显著增加（P <.01）。伴有PE的EPS的中膜厚度百分比分别为7.0%±1.9%、7.3%±1.4%和6.6%±2.3%，与对照组的2.3%±0.7%相比显著增加（P <.01）。
我们得出结论，与EPS相关的PE是漏出液而非淋巴液。在伴有PE的EPS中发现的中膜和外膜增厚支持了以下假设，即静脉系统的部分阻塞导致漏出液产生增加，最终导致胎儿水肿。
