Gasparella Paolo, Singer Georg, Castellani Christoph, Sorantin Erich, Haxhija Emir Q, Till Holger
Department of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria.
Division of Paediatric Radiology, Department of Radiology, Medical University of Graz, Graz, Austria.
J Surg Case Rep. 2020 Aug 26;2020(8):rjaa252. doi: 10.1093/jscr/rjaa252. eCollection 2020 Aug.
Abdominal lymphatic malformations in neonates require sophisticated management. In symptomatic cases, acute complications may necessitate immediate surgery. We present the case of a giant abdominal lymphatic malformation diagnosed in the 18th gestational week. Sonographic concerns about intestinal hypoperfusion in the 33rd week of gestation indicated caesarean section. Postnatal imaging confirmed a macrocystic lymphatic malformation occupying almost the complete abdominal cavity; the intestinal perfusion was normal. Clinical deterioration on Day 13 of life required laparotomy. Intraoperatively, the lymphatic mass was located in the ileocecal mesentery. Two major cysts showed recent hemorrhage explaining the onset of abdominal compartment syndrome. The malformation was completely removed. An ileocecal resection with an ileocolic anastomosis was performed. The postoperative course was uneventful. In neonates with abdominal lymphatic malformations, an onset of abdominal compartment syndrome requires surgical exploration. If feasible, the complete removal of the lesion represents a curative option.
新生儿腹部淋巴管畸形需要复杂的治疗。在有症状的病例中，急性并发症可能需要立即进行手术。我们报告一例在妊娠第18周诊断出的巨大腹部淋巴管畸形病例。妊娠第33周时超声检查发现肠道灌注不足，提示行剖宫产。产后影像学检查证实为一个几乎占据整个腹腔的大囊型淋巴管畸形；肠道灌注正常。出生后第13天临床情况恶化，需要进行剖腹手术。术中，淋巴管肿块位于回盲部系膜。两个主要囊肿近期有出血，这解释了腹腔间隔室综合征的发生。畸形被完全切除。进行了回盲部切除及回结肠吻合术。术后过程顺利。对于患有腹部淋巴管畸形的新生儿，腹腔间隔室综合征的发作需要进行手术探查。如果可行，完全切除病变是一种治愈性选择。
