Reynisdottir Kristin Fjola, Hjartardottir Hulda, Rosmundsson Thrainn, Thorkelsson Thordur
Faculty of medicine, University of Iceland, Child and Adolescent Psychiatric Department, Landspitali - National University Hospital of Iceland.
Faculty of medicine, University of Iceland, Obstetrics and Fetal Medicine, Landspitali - National University Hospital of Iceland.
Laeknabladid. 2024;110(3):144-150. doi: 10.17992/lbl.2024.03.785.
Gastroschisis and omphalocele are the most common congenital abdominal wall defects. The main purpose of this study was to investigate the incidence, other associated anomalies and the course of these diseases in Iceland.
The study was retrospective. The population was all newborns who were admitted to the NICU of Children's Hospital Iceland due to gastroschisis or omphalocele in 1991-2020. Furthermore, all fetuses diagnosed prenatally or post mortem where the pregnancy ended in spontaneous or induced abortion, were included.
During the study period, 54 infants were born with gastroschisis and five with omphalocele. The incidence of gastroschisis was 4.11 and omphalocele 0,38/10,000 births. There was no significant change in the incidence of the diseases during the study period. In addition, five fetuses were diagnosed with gastroschisis and 31 with omphalocele where the pregnancy was terminated. In addition to gastroschisis in the live born infants and fetuses the most common associated anomalies were in the gastrointestinal or urinary tract but in infants and fetuses with omphalocele anomalies of the cardiac, central nervous or skeletal systems were the most common. Sixteen fetuses diagnosed with omphalocele had trisomy 18. Mothers aged 16-20 were more likely to give birth to an infant with gastroschisis than older mothers (p< 0.001). Primary closure was successful in 86% of the infants. Those reached full feedings significantly earlier and were discharged earlier. Overall survival rate was 95%. Three children were still receiving parenteral nutrition at discharge due to short bowel syndrome.
The incidence of gastroschisis in Iceland is in accordance with studies in other countries but but the incidence of omphalocele is lower, which can be partly explained by spontaneous or induced abortions. Other anomalies associated with omphalocele are more severe than those associated with gastroschisis. Primary closure was associated with more benign course. Children with gastroschisis may need prolonged parenteral nutrition due to shortening of their intestines.
腹裂和脐膨出是最常见的先天性腹壁缺陷。本研究的主要目的是调查冰岛这些疾病的发病率、其他相关异常情况及病程。
本研究为回顾性研究。研究对象为1991年至2020年因腹裂或脐膨出入住冰岛儿童医院新生儿重症监护病房的所有新生儿。此外，还纳入了所有产前诊断或死后诊断的胎儿，这些妊娠以自然流产或人工流产告终。
在研究期间，54例婴儿出生时患有腹裂，5例患有脐膨出。腹裂的发病率为4.11/10000活产儿，脐膨出为0.38/10000活产儿。在研究期间，这些疾病的发病率没有显著变化。此外，5例胎儿被诊断为腹裂，31例胎儿被诊断为脐膨出，这些妊娠均被终止。除了活产婴儿和胎儿中的腹裂外，最常见的相关异常是胃肠道或泌尿系统的异常，但在患有脐膨出的婴儿和胎儿中，心脏、中枢神经或骨骼系统的异常最为常见。16例被诊断为脐膨出的胎儿患有18三体综合征。16至20岁的母亲比年龄较大的母亲更有可能生下患有腹裂的婴儿（p<0.001）。86%的婴儿一期缝合成功。这些婴儿更早达到完全喂养且更早出院。总体生存率为95%。由于短肠综合征，3名儿童出院时仍在接受肠外营养。
冰岛腹裂的发病率与其他国家的研究结果一致，但脐膨出的发病率较低，这部分可以通过自然流产或人工流产来解释。与脐膨出相关的其他异常比与腹裂相关的异常更严重。一期缝合与更良性的病程相关。由于肠道缩短，患有腹裂的儿童可能需要长期的肠外营养。
