Bagolan P
Minerva Pediatr. 2010 Jun;62(3 Suppl 1):95-7.
Congenital diaphragmatic hernia (CDH) is one of the most serious and controversial congenital anomalies. Pulmonary hypoplasia with/without pulmonary hypertension (and major associated anomalies) are reported the main causes of death in these patients. New treatment strategies for CDH have allowed an increase of survival also of more severe patients. As a consequence many attentions are now focused on long term follow up of these delicate babies. Many reports, in the last years, have highlighted associated morbidities in CDH survivors, such as pulmonary sequelae, neurodevelopmental delay, auxological deficits, gastrointestinal and orthopedic disorders. Therefore it is nowadays quite evident that this group of patient is a complex population to care during the first year of life but also throughout infancy and childhood, thus requiring a well defined follow up. Single institution data on overall survival have been frequently reported, only few of these however reported long term follow up of their patients. The aim of this short review is to describe the mid and long term outcomes of CDH survivors, suggesting also a personal view of follow up protocol for these babies.
先天性膈疝(CDH)是最严重且最具争议的先天性畸形之一。据报道,伴有或不伴有肺动脉高压(以及主要相关畸形)的肺发育不全是这些患者的主要死因。针对CDH的新治疗策略使病情更严重的患者的存活率也有所提高。因此,现在许多注意力都集中在对这些脆弱婴儿的长期随访上。在过去几年中,许多报告都强调了CDH幸存者存在的相关疾病,如肺部后遗症、神经发育迟缓、生长发育缺陷、胃肠道和骨科疾病。因此,如今很明显,这组患者在生命的第一年以及整个婴儿期和儿童期都是一个需要精心护理的复杂群体,因此需要明确的随访。关于总体存活率的单机构数据经常被报道,然而其中只有少数报道了对其患者的长期随访。这篇简短综述的目的是描述CDH幸存者的中长期结局,并提出对这些婴儿随访方案的个人观点。