De Bernardo Giuseppe, Giovengo Marta, Sordino Desiree, Giordano Maurizio, Zeccolini Massimo
Department of Woman and Child, Buon Consiglio Fatebenefratelli Hospital, Naples, Italy.
Department of Medicine and Surgery, "Scuola Medica Salernitana", Section of Pediatrics, University of Salerno, Salerno, Italy.
Radiol Case Rep. 2024 Sep 5;19(11):5535-5538. doi: 10.1016/j.radcr.2024.08.069. eCollection 2024 Nov.
The omphalocele is an abdominal wall defect at the base of the umbilical cord, with the worldwide prevalence of 2.6 per 10,000 births. Omphalocele contains herniated abdominal organs and is classified in small and giant based on the size of the defect. Omphalocele is associated with several syndromes or structural anomalies. We reported a case of giant omphalocele of 6 cm with right lung agenesis with tracheal and right bronchial hypoplasia. Laryngeal mask stabilized the newborn allowing endotracheal intubation. Due to serious malformations, the newborn died after few hours of life for cardio-respiratory arrest. Early and accurate prenatal diagnosis allows a better intervention strategies in delivery room, improving the chances of survival and reducing complications for infants with omphalocele.
脐膨出是脐带根部的腹壁缺损，全球每10000例出生中患病率为2.6例。脐膨出包含疝出的腹部器官，并根据缺损大小分为小型和巨型。脐膨出与多种综合征或结构异常有关。我们报告了一例6厘米的巨型脐膨出病例，伴有右肺不发育及气管和右支气管发育不全。喉罩使新生儿情况稳定，得以进行气管插管。由于严重畸形，新生儿出生后数小时因心肺骤停死亡。早期准确的产前诊断有助于在产房采取更好的干预策略，提高脐膨出婴儿的存活几率并减少并发症。
