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.