探讨新生儿腹膜后淋巴管瘤的临床特点与诊治策略。

选择2017年7月3日，因"生后腹胀31 min"收入成都市妇女儿童中心医院新生儿科，并确诊的1例腹膜后淋巴管瘤新生儿为研究对象。回顾性分析该例新生儿的临床病例资料，总结其临床特点和诊治经过，并进行相关文献复习。本研究对新生儿腹膜后淋巴管瘤相关文献的检索策略为：以"淋巴管瘤""腹膜后淋巴管瘤""淋巴管畸形""新生儿""lymphangioma""retroperitoneal lymphangioma""lymphatic malformation"及"neonate"为关键词，检索中国知网数据库、PubMed等国内外文献数据库建库至2018年12月，收录的关于新生儿腹膜后淋巴管瘤的文献，并总结该病临床特点和诊治方案。本研究符合2013年修订的《世界医学协会赫尔辛基宣言》要求。

本例患儿为女性，入院诊断为腹腔积液待查；入院年龄为生后31 min，其病史采集与诊治过程如下。①病史采集：生后腹胀。母亲于孕龄分别为24、40孕周时，接受产前胎儿超声检查均提示胎儿腹腔见囊性占位、腹腔大量积液。患儿出生后除腹胀、腹围进行性增加外，无其他明显异常表现。生后2 d内对该例患儿行腹腔穿刺2次，均抽出较多淡黄色液体，腹水检查结果显示为渗出液。腹部X射线摄片、超声及CT检查，均显示腹腔囊性占位、腹腔大量积液。②手术、治疗经过及转归：患儿在全身麻醉下，共计进行3次手术，具体如下。第1次，于生后第3天对其进行腹腔镜探查术及淋巴液漏口结扎术，术中清理腹腔淡黄色清亮液体约600 mL，手术后有明显的淋巴液漏，腹腔引流液量大，平均为127.2 mL/d，并且患儿出现营养不良表现。第2次，于生后第28天对其进行腹膜后肿物切除术，术中切除淋巴管样异常增生病灶送组织病理学检查结果显示，"左侧腹膜后肿物符合淋巴管瘤"，术后腹腔引流液量明显减少，但患儿腹胀进行性加重，随访腹部超声检查提示，腹膜后淋巴管瘤复发，弥漫性并逐渐增大。第3次，于生后第49天对其进行剖腹探查术的结果显示，结肠相关广泛淋巴管畸形(LM)伴大范围淋巴液回流障碍，形成弥漫性混合型淋巴管瘤，因此进行结肠次全切除术、腹膜后淋巴管瘤部分切除术，术后腹腔引流液量少，无腹胀发生。第3次手术后14 d，对该例患儿再次开始脱脂奶粉喂养，并且顺利增加至全肠内营养。患儿总住院时间为81 d，治愈出院，随访至18个月龄，患儿生长发育良好，随访腹部影像学检查结果显示，腹膜后淋巴管瘤未复发。③文献检索结果：仅检索到2例新生儿腹腔淋巴管瘤的文献报道。其中，1例为腹腔脐部至右侧腹股沟区的表浅巨囊型淋巴管瘤，因无腹腔脏器受压迫的临床表现，而对其采取生后保守观察，3岁时淋巴管瘤缩小，接受激光治疗；另一例为腹膜后淋巴管瘤，因腹胀明显，于生后第10天对其采取淋巴管瘤手术全切除术。这2例患儿均预后良好。

新生儿淋巴管瘤根据其部位、大小和临床表现，选择合适的干预时间和方式，能取得较好的治疗效果及远期预后。

To explore the clinical characteristics, diagnosis and treatment strategies of neonatal retroperitoneal lymphangioma.

On July 3 of 2017, due to " abdominal distension for 31 minutes after birth", the female neonate was admitted to Department of Neonatology of Chengdu Women′s and Children′s Central Hospital and was definitively diagnosed as retroperitoneal lymphangiomas, was selected as research subject. The clinical data of the neonate was analyzed retrospectively, and the clinical characteristics, diagnosis and treatment process were summarized, and related studies were reviewed. With the following key words of " lymphangioma" " retroperitoneal lymphangioma" " lymphatic malformation" " neonate" both in Chinese and in English, studies related to neonate with retroperitoneal lymphangiomas were searched from China National Knowledge Infrastructure database, PubMed and others domestic and foreign literature databases from construction to December 2018, and clinical features, diagnosis and treatment methods of these studies were summarized. This study was consistent with the World Medical Association Declaration of Helsinki revised in 2013.

The female infant was diagnosed as seroperitoneum to be investigated on admission when she was 31 minutes old after birth. The history taking, diagnosis and treatment processes were as follows. ① Medical history taking: The infant had abdominal distension after birth. Abdominal cystic mass and massive seroperitoneum of fetus were identified by prenatal fetal ultrasound at gestational age of 24 and 40 weeks of her mother. After her birth, there were no other abnormal appearances except abdominal distension and progressive increase of abdominal circumference. Peritoneocentesis were performed twice to her in the first 2 days after her birth, and both aspirated much yellowish fluid, and ascites examination showed exudate. Abdominal X-ray, ultrasonography and CT examinations all showed cystic mass and massive ascites in abdominal cavity. ② Surgery, treatment processes and sequelae: She underwent three operations under general anesthesia, the details were as follows. For the 1st operation, laparoscopic exploration and ligation of lymphatic leakage orifice were performed to her on the 3rd day after her birth. About 600 mL yellowish fluid in peritoneal cavity was sucked out. There was severe lymphatic leakage after operation, and peritoneal drainage fluid was about 127.2 mL/d on average. Meanwhile, she was suffered from malnutrition. For the 2nd operation, the retroperitoneal mass was resected on the 28th day to her after her birth. The lymphangiodysplasia lesions excised during the operation were sent to histopathological examination with the result of " left retroperitoneal mass accorded with lymphangioma". Then peritoneal drainage fluid was significantly reduced, but abdominal distension was gradually aggravated. Follow-up abdominal ultrasound examination indicated the recurrence of retroperitoneal lymphangiomas, which were diffuse and the size was getting bigger. For the 3rd operation, the exploratory laparotomy at the age of 49 d revealed extensive lymphatic malformations (LM) of colon with extensive lymphatic obstruction, which leaded to diffuse mixed lymphangiomas. So, subtotal colectomy and partial resection of retroperitoneal lymphangioma were performed. After 3rd operation, peritoneal drainage fluid was little and there was no obvious abdominal distension occurred. Two weeks after the 3rd surgery, the child was fed again with skimmed milk and reached to total enteral nutrition successfully. The child was cured and discharged from hospital with total length of hospitalization for 81 days. Follow-up result showed that she was growing well at age of 18 months, and result of abdominal imaging examination showed no recurrence of retroperitoneal lymphangiomas. ③ Literature retrieval results were as follows. Only 2 cases of neonatal abdominal lymphangioma were reported. One case was superficial macrocystic lymphangioma from umbilical to right inguinal region in abdominal cavity. Because there was no clinical manifestations of compression of abdominal organs, a " wait and see" strategy was adopted. At the age of 3 years old, the lymphangioma became smaller and the child received laser treatment. Another case was retroperitoneal lymphangioma. Because of abdominal distention, lymphangioma was totally resected 10 days after birth. Both children had good prognosis.

Neonatal lymphangioma can be treated effectively and have a good long-term prognosis with appropriate time and mode of intervention according to different location and size of lymphangioma, and clinical manifestations.