Yazici Mutlu Uysal, Ekinci Saniye, Turkmen Ozlem Keskin, Yalcin Ebru Gunes, Ciftci Arbay O, Gucer Safak, Orhan Diclehan, Tezcan Ilhan
Department of Pediatrics, Hacettepe University Children's Hospital, Ankara, Turkey.
Department of Pediatric Surgery, Hacettepe University Children's Hospital, Ankara, Turkey.
European J Pediatr Surg Rep. 2014 Jun;2(1):38-42. doi: 10.1055/s-0033-1354745. Epub 2013 Sep 13.
Split notochord syndrome is a rare group of developmental abnormalities caused by abnormal splitting or deviation of the notochord clinically resulting in the duplicated bowel associated with vertebral anomalies. We report on a case of 11-month-old female infant with mediastinal hyperechogenic cyst and intestinal duplication cyst associated with T5-T6 hemivertebrae, scoliosis, and nonfusion of posterior part of T6 vertebrae, presenting with severe hemoptysis and hematemesis. The cysts were surgically removed, and histopathologic analysis revealed that the mediastinal cyst was lined by gastric mucosa and intestinal one was lined with gastric mucosa including ectopic pancreatic tissue. After removal of the lesion the patient made an uneventful recovery and shows no signs of long-term pulmonary sequelae.
脊索分裂综合征是一组罕见的发育异常，由脊索异常分裂或偏移引起，临床上表现为与脊柱异常相关的重复肠管。我们报告一例11个月大的女婴，患有纵隔高回声囊肿和肠重复囊肿，伴有T5 - T6半椎体、脊柱侧弯和T6椎体后部未融合，表现为严重咯血和呕血。囊肿通过手术切除，组织病理学分析显示纵隔囊肿内衬胃黏膜，肠囊肿内衬胃黏膜，包括异位胰腺组织。切除病变后，患者恢复顺利，未出现长期肺部后遗症的迹象。
