Pennaforte T, Rakza T, Fily A, Mur S, Diouta L, Sfeir R, Aubry E, Bonnevalle M, Storme L
Hôpital Jeanne de Flandre, CHRU de Lille, avenue Eugène-Avinée, 59037Lille, France.
Arch Pediatr. 2013 Sep;20 Suppl 1:S11-8. doi: 10.1016/S0929-693X(13)71404-0.
The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.
先天性膈疝(CDH)患儿存活率的提高伴随着晚期发病率的增加。已描述了许多并发症,包括肺损伤、心血管疾病、胃肠道疾病、生长发育不良、神经认知缺陷和肌肉骨骼异常。CDH的长期肺部发病率包括由于肺结构改变和长期通气支持导致的阻塞性和限制性肺功能损害。肺动脉高压的长期后果尚不清楚。胃食管反流病(GERD)也是总体发病率的一个重要因素。生长发育不良可能由GERD、因口腔厌恶导致的摄入不足以及肺部疾病导致的热量需求增加引起。神经学结局取决于CDH患者出生后最初几天围产期和新生儿低氧血症风险的增加。许多研究探讨了上述健康问题对CDH患者总体健康状况的重大影响,但只有少数研究关注与健康相关的生活质量。考虑到零散的数据表明CDH长期存活者存在大量发病情况,需要进行系统评估长期后遗症的随访研究。基于此类研究,可以建立一种更具针对性的常规多学科随访计划方法。促进知识交流、未来研究和治疗方案的开发是法国协作网络的目标。