Discussion
The advance of neonatal intensive care, including the gentle ventilation strategy with high frequently oscillation ventilator (HFOV) and the advent of ECMO, has improved the survival rate of patients with CDH, especially those who are in severe conditions [
4‐
6]. At the same time, increasing number of children who have survived arduous neonatal period is giving rise to new issues related to long-time morbidity and transition of follow-up care [
7‐
9]. There are evidences of high morbidity rate (60%) of CDH survivors and it was reported that they frequently experience growth delay (80%), gastric acid reflux (60%), recurrent pneumonia and chronic cough (10%), scoliosis (30%), and pectus deformity (20%) [
3,
10‐
12]. Additionally, there are also neurological issues including developmental delay, which is potentially result from exposure to hypoxia and muscle relaxants in the early neonatal period [
11,
13]. Hearing loss is another relevant neurological morbidity and reported to be observed in 20% of CDH survivors [
13,
14]. A correlation between long-time sequelae and severity of CDH is generally acknowledged, but even patients who are successfully treated without ECMO nor patch repair are at risk of abovementioned symptoms [
15‐
17]. Therefore, it is not straightforward to predict the long-time sequalae at the time of surgery and it is desirable to diligently follow up each patient in a comprehensive way. This is particularly relevant because of their impact on QOL of CDH patients and their families. For the first time, our group looked into those effects on the daily life CDH survivors. Thanks to the CDH UK, we could collect unique information which helped understanding patients’ perspective. The most important message is that, although it is usually kept within pre-clinical level, most of the patients and families are feeling stress in their daily activity. Feeding problems such as dysphagia and food intolerance seemingly have great impact on their QOL, by forcing the families to spend much time for feeding their children, which can put stress on their relationship.
The most common feeding issue is GER, being reported by over 90% of responders. Previous reports have described the incidence of GER in CDH survivors as 30–60% with variability depending on diagnostic criteria [
18‐
20]. We assume the higher rate in our result is representing the recognition of the patients, reporting reflux as any vomit or regurgitation. Recently, multichannel intraluminal impedance (MII), which objectively detects the non-acidic reflux, has been introduced and revealed the high prevalence of non-acidic reflux at 80% [
21]. Moreover, impaired motility of esophagus after CDH repair has been identified to induce prolonged clearance time, which subjects lower esophagus to risk of chronic esophagitis due to excess acid exposure. Even though patients have no typical symptom, GER possibly exist and silent reflux is a risk factor of later complications, such as Barrett’s esophagitis and consequent adenocarcinoma [
20,
22,
23]. These facts indicate that GER is common, but non-negligible condition, recommending clinicians to carefully detect a sign of reflux and apply antipeptic therapy at proper timing. Fortunately, it is reported that the incidence of GER decreases after 1 year of age [
18,
21].
Growth retardation is also problematic and experienced by 60% of the CDH survivors. Although increased catabolic stress and restricted water intake based on pulmonary impairment are likely to have some influence, the major factor of delayed growth is probably the lack in nutrition related to dysphagia, GER, and oral aversion [
12]. The important thing is to routinely assess patient’s nutritional condition and find any sign of nutritional deficiency. The etiology of oral aversion is unspecified, but there is the speculation that long history of intubation and delayed oral feeding affect the mouth sensitivity [
16]. In addition, delay in initiation of oral feeding related to dependence on tube feeding can influence developmental delay [
11]. Further negative point of dysphagia is its impact on the QOL, indicated by the fact that more than half of the responders complained the meal time is the hardest thing to cope with. It is not hard to imagine how oral aversion confuses and frustrates parents. However, there is hopeful data that oral aversion tends to improve gradually coincided with the start of school or social activity [
16]. In addition, early intervention of nutritional support and rehabilitation may have potential to moderate this issue.
As CDH survivors are vulnerable to various problems and remain complex beyond the neonatal period, the importance of multidisciplinary team (MDT) approach is increasing. MDT usually consists of different type of professionals including pediatric surgeon, pediatrician, pulmonologist, cardiologist, neurodevelopmentalist, dietitian, and so on, smoothing comprehensive assessment of patients in complicated condition. MDT clinics specifically designed for particular congenital disease, such as CDH, anorectal malformation, and abdominal wall defects have been gradually increasing. Moreover, the trial to consolidate post-discharge care of CDH in MDT clinic, so called CDH clinic, has been under way in some areas [
10,
17]. Unfortunately, according to our survey, only one third of the patients have ever attended a CDH clinic, indicating constructing effective networks which facilitates easy access to MDT care will be a future subject. One of the concerning findings of this study is that very few patients received sufficient information on feeding problems and related supporting resource. This creates anxiety to CDH families who find it very difficult to cope with this long-term problem. Informing not only about survival, but also long-time outcome in advance helps the parents imagine the daily life after discharge of the baby and prepare themselves emotionally [
24]. In our opinion, this should be discussed early with the family, even before birth.
This research has some limitations due to the patient-led nature of the survey. The subject of the study included only the registered members, thus the data doesn’t represent all cohort of CDH survivors, and incompleteness of questionnaire in a third of the responders potentially skews the data. Although the data are lacking detailed clinical information, such as age, weight, and operative procedure, direct responses assembled by volunteers are valuable, helping surgeons consider the transitional care for CDH survivor form the different aspect.
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