Neonatal Intensive Care Unit, Department of Pediatrics, University of Genoa, IRCCS Gaslini, Genoa, Italy.
Lymphology. 2012 Sep;45(3):91-102.
This retrospective study was carried out at eight Neonatal Intensive Care Units (NICU) Centers worldwide on 33 newborns presenting at birth with pleural, pericardial, or abdominal chylous effusions. Diagnosis of chylous effusion is based on findings of fluid with a milk-like appearance, a concentration of triglycerides in pleural effusion >1.1 mmol/l, and a total cell count >1,000 cells/ml with a predominance of >80% lymphocytes. Thirty-three newborns met the inclusion criteria and were studied. Six subjects who presented at birth with fetal effusion were treated by in-utero pleuro-amniotic shunt. Five of these patients are alive at follow-up. At birth, pleural drainage was performed in 29/33 patients and abdominal drainage was carried out in 3/33. Total parenteral nutrition (TPN) was given to 32/33 patients; 19/23 patients were fed a medium-chain triglycerides (MCT). No adverse effects were observed. Eight patients were treated with Octreotide at dosages ranging from 1 to 7 mcg/kg/hour for 8 to 35 days. All patients showed decreased chylous production. Two patients were treated by pleurodesis. Twenty-two babies are alive after at least 6 months follow-up, 9/33 are deceased, and 2 were lost to follow-up. Clinical conditions of survivors are basically good except for lung involvement [chronic lung disease (CLD) or lung lymphangiectasia] and lymphedema. All patients were using a MCT diet at follow-up with good control of chylous effusion. Visceral chylous effusions of the fetus and neonate are rare disorders, and there currently is only partial agreement on decision-making strategies. We suggest the need for an international prospective trial in an effort to establish the efficacy and effectiveness of diagnostic and therapeutic options described in this article.
这项回顾性研究在全球 8 个新生儿重症监护病房（NICU）中心进行，共纳入 33 例出生时即存在胸腔、心包或腹腔乳糜性积液的新生儿。乳糜性积液的诊断基于以下发现：外观似牛奶的液体、胸腔积液中三酰甘油浓度>1.1mmol/L 以及总细胞计数>1000 个/ml，且以淋巴细胞为主>80%。33 例新生儿符合纳入标准并进行了研究。6 例出生时存在胎儿积液的患者接受了宫内胸腔-羊膜分流术治疗。这 5 例患者在随访时仍存活。出生时，29/33 例患者进行了胸腔引流，3/33 例患者进行了腹腔引流。32/33 例患者给予全肠外营养（TPN），19/23 例患者给予中链甘油三酯（MCT）喂养。未观察到不良反应。8 例患者接受奥曲肽治疗，剂量为 1 至 7μg/kg/h，持续 8 至 35 天。所有患者的乳糜产量均减少。2 例患者接受了胸膜固定术。22 例婴儿在至少 6 个月的随访后存活，9/33 例死亡，2 例失访。幸存者的临床状况基本良好，但存在肺部受累[慢性肺病（CLD）或肺淋巴管扩张]和淋巴水肿。所有患者在随访时均使用 MCT 饮食，乳糜性积液得到良好控制。胎儿和新生儿的内脏乳糜性积液较为罕见，目前对于决策策略仅有部分共识。我们建议需要进行国际前瞻性试验，以确定本文中描述的诊断和治疗选择的疗效和有效性。
