Long-Term Follow-Up of Patients With Congenital Diaphragmatic Hernia

Open access Review
Long-term follow-up of patients with
World Journal of Pediatric Surgery: first published as 10.1136/wjps-2023-000758 on 10 April 2024. Downloaded from https://wjps.bmj.com on 17 June 2024 by guest. Protected by
congenital diaphragmatic hernia
Nicole Cimbak ‍ ‍, Terry L Buchmiller
To cite: Cimbak N, Buchmiller TL. ABSTRACT repository to address this need.10 While the
Long-term follow-up of patients Neonates with congenital diaphragmatic hernia encounter CDHSG provides outcome data from index
with congenital diaphragmatic a number of surgical and medical morbidities that persist
hernia. World J Pediatr Surg
hospitalization, institution-based CDH clinics
into adulthood. As mortality improves for this population, provide the majority of research on long-term
2024;7:e000758. doi:10.1136/
these survivors warrant specialized follow-up for their outcomes in CDH neonates.
wjps-2023-000758
unique disease-specific morbidities. Multidisciplinary
The first multidisciplinary long-term clinic
Received 15 December 2023 congenital diaphragmatic hernia clinics are best positioned
to address these complex long-term morbidities,
was formed at Boston Children’s Hospital
Accepted 28 February 2024
provide long-term research outcomes, and help inform in 1990 by Wilson and colleagues. In 2008,
standardization of best practices in this cohort of patients. the American Academy of Pediatrics (AAP)
This review outlines long-term morbidities experienced Section on Surgery published a report
by congenital diaphragmatic hernia survivors that can be outlining recommended multidisciplinary
addressed in a comprehensive follow-up clinic. follow-up (table 1).11 Similarly, the CDH
copyright.
EURO Consortium published guidelines in
2018 emphasizing multidisciplinary CDH
INTRODUCTION postnatal care based on standardized clinical
12
Overall survival in neonates born with assessment and management plans. Center-
congenital diaphragmatic hernia (CDH) has specific protocols are not widely available but
improved over the last 30 years.1 Contrib- a review published in 2014 identifies common-
uting factors include advances in ventilation alities to clinic structure including pres-
strategies, pulmonary hypertension (PH) ence of a pediatric surgeon, pulmonologist,
therapies, standardized postnatal manage- cardiologist, gastroenterologist and devel-
2
ment protocols, extracorporeal membrane opmental pediatrician. Diagnostic studies
oxygenation (ECMO) utilization, and referral completed at these visits vary but include a
to high- volume specialized centers. With chest radiograph (CXR), echocardiogram
improved mortality, the growing number (ECHO), neurodevelopmental assessment,
of CDH survivors often has complex multi- and hearing screening. Other age- specific
system comorbidities that require long-term tests are also performed such as ventilation
management (figure 1).2–9 Dedicated pedi- perfusion (V/Q) scans, pulmonary function
13–16
atric practitioners have responded to this tests (PFTs), and altitude testing.
need with the formation of long-term multi- Organizing a large number of specialty
disciplinary clinics along with research initia- providers, diagnostic testing, and specialized
tives seeking to understand and manage long- resources is not always feasible. In a hospital
term disease- specific morbidity in patients setting without a centralized CDH clinic,
with CDH. This review will outline the impor- families reported that time and distance to
tance of long-term CDH clinics and system- individual appointments were significant
specific advances and recommendations for barriers to seeking follow-up care. Families
long-term follow-up care. also expressed interest in a CDH multidis-
ciplinary clinic.17 Forming family- centered
© Author(s) (or their multidisciplinary clinics offloads the medical
employer(s)) 2024. Re-use burden for families and broadens the catch-
SIGNIFICANCE OF LONG-TERM CDH CLINICS
permitted under CC BY.
Published by BMJ. Given the variation in CDH severity and ment area where CDH survivors can receive
Department of Pediatric Surgery, pathophysiology, research collaboration in multidisciplinary care. Along with providing
Boston Children's Hospital, CDH outcomes forms the foundation for improved care for CDH survivors, multidis-
Boston, MA, USA evidence- based management including the ciplinary clinics can collect and interpret
identification of predictors of survival to data from their centers over longer survival
Correspondence to discharge from index hospitalization. The periods.
Dr Nicole Cimbak; n icole. CDH Study Group (CDHSG) was founded While there are benefits to the granular
cimbak@childrens.harvard.edu in 1995 as a voluntary international data research from single-center long-term clinics,
Cimbak N, Buchmiller TL. World J Pediatr Surg 2024;7:e000758. doi:10.1136/wjps-2023-000758 1

Open access
ultimately provide information to inform standardized
follow-up protocols and guidance to the adult and pedi-
atric community regarding optimal long-term manage-
ment of these complex CDH survivors.
CARDIOPULMONARY SEQUELAE
Pulmonary hypertension
The underdevelopment of the fetal lung parenchyma
in CDH can result in severe pulmonary hypoplasia and
abnormal pulmonary vasculature. The subsequent post-
natal PH secondary to pulmonary arterial muscularization
and pulmonary vasculature remodeling is often a source
of severe morbidity and mortality in CDH neonates.18–21
While the majority of PH associated with CDH in infancy
resolves over time, there is a subset of patients who have
persistent PH warranting continued follow-up.22 Identi-
fying which patients require long-term follow-up for PH
with ECHO, cardiac catheterization, and titration of PH
therapies remains a topic of research efforts.
Figure 1 Multisystem disease morbidity profile of Published reports cite PH rates after hospital discharge
congenital diaphragmatic hernia (CDH) survivor. GERD, between 8% and 38%.23 24 There have been retrospective
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gastroesophageal reflux disease. and larger database studies examining predictive factors
for persistent PH severity to identify higher risk cardiac
collaborative long-term registries provide strength to patients after discharge.22 25–29 These risk factors include
evidence-
based recommendations for follow- up. Chiu duration of mechanical ventilation, ECMO utilization,
and Ijsselstijn outlined the creation of a prospective and nitric oxide use during index hospitalization.23 25
longitudinal database for multidisciplinary clinics to Because of the associated long-term morbidity, the Amer-
aggregate data through the CDHSG which is now in ican Heart Association and American Thoracic Society
progress.5 These robust longitudinal clinic data will report class I evidence for management of CDH neonates,
Table 1 Recommended follow-up schedule and diagnostic testing for CDH survivors
Patient age at clinic visit
Discharge to 1 year Ages 1–2 years Ages Ages 6–21
2–5 years years
Appointment schedule Every 3 months Every 6 months Annually Every 2–3 years
History and physical examination—includes
scoliosis and chest wall deformity examination
Chest X-ray
V/Q scan *
PFTs 5 years As indicated
Echocardiogram As indicated As indicated As indicated
Audiology 3 years 6 years
Upper GI study or pH probe As indicated based on symptoms
Cardiopulmonary exercise testing As indicated
Neurodevelopmental assessment† As indicated based on screening
Brain imaging (MRI/CT) ECMO survivors
Altitude testing evaluation Pending travel screen
The shading indicates whether the intervention is recommended. Green signifies recommended and yellow signifies as
indicated per patient.
*V/Q scan repeated if perfusion or ventilation to affected lung is <30%.
†Consideration of formal testing with Bayley III or IV or WPPSI-IV.
CDH, congenital diaphragmatic hernia; ECMO, extracorporeal membrane oxygenation; GI, gastrointestinal; PFT, pulmonary
function test; V/Q, ventilation perfusion; WPPSI, Wechsler Preschool and Primary Scale of Intelligence.
2 Cimbak N, Buchmiller TL. World J Pediatr Surg 2024;7:e000758. doi:10.1136/wjps-2023-000758

Open access
including long-term monitoring with a PH specialist and lung function included liver position within the chest,
ECHO evaluation.30 patch repair, ECMO utilization, and duration of mechan-
CDH-associated PH is particularly refractory to medical ical ventilation.8 16 44 Alterations in PFTs persist into adult-
therapy, often invoking off- label use of PH medica- hood and can worsen over time.15 45 46 There is variability
tions.31 32 There has been an expansion of pharmacolog- in the responsiveness of pulmonary function to bron-
ical PH therapies in patients with CDH targeting multiple chodilator therapy.38 45 46 Given the complexity of airway
PH pathways.25 31 33–35 Results of pharmacological studies disease in patients with CDH, standardized evaluation by
have been promising with minimal associated clinically a pediatric pulmonologist is warranted to ensure appro-
significant adverse events in CDH survivors.31 36 As the priate diagnostic testing and medical management to
use of these medications increases, it follows that more optimize outcome.
neonates may be discharged on oral PH medications. Postnatal lung growth in patients with CDH is altered
Given the off-label use of PH medications in the pediatric with decreased airway generation and radial alveolar
population, management should be led by pediatric PH counts in the ipsilateral lung accompanied by pulmo-
specialists in the outpatient setting, preferably in a multi- nary muscularization in the contralateral lung.19 This
disciplinary CDH clinic. remodeling is demonstrated by changes in V/Q imaging
with lower rates (30–40% V/Q rates) correlating with
Cardiac function increased pulmonary morbidity in survivors.47 Ipsilat-
PH can reduce pulmonary blood flow with subsequent eral V/Q mismatch worsened over time in a long-term
adaptations to the exercise response that differ from CDH neonatal population.14 As CDH-associated pulmo-
healthy controls.37 Cardiac capacity can be evaluated nary morbidity worsens with age in a subset of patients,
using cardiopulmonary exercise testing (CPET) in the it is imperative to have longitudinal follow-up to identify
outpatient setting. Multiple studies have examined these at-risk individuals who may benefit from tailored
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CPET in CDH survivors, with initial testing at ages 7–10 pulmonary therapies and rehabilitation. There is a small
years, showing quantitatively impaired exercise tolerance subset of patients whose severe pulmonary morbidity
marked by lower peak oxygen consumption and minute requires prolonged ventilatory support with tracheostomy
ventilation. Patients report higher rates of dyspnea, feel- placement. Factors predictive of tracheostomy include
ings of throat closing, and effort perception compared major cardiac anomalies, larger defect size, ECMO use,
to controls.29 37–40 Patients with higher rates of base- and intrathoracic liver.48 Patients with tracheostomies
line activity had improved maximum exercise capacity certainly mandate specialized pulmonary follow-up for
compared with sedentary patients.40 Targeted initiatives, ventilator weaning and if possible, future decannulation.
such as exercise programs for CDH survivors, based on
collected data and research could improve cardiorespira- Pulmonary protection
tory capabilities. Given the pulmonary morbidity in patients with CDH,
ensuring appropriate prophylaxis against respira-
Concomitant neonatal congenital heart disease tory infections, particularly influenza, COVID- 19, and
There is a special population of survivors with both CDH respiratory syncytial virus (RSV), is crucial.49 In a study
and congenital heart disease (CHD) with a broad range of 201 patients with CDH, CDH neonates had a four-
of disease complexity. CHD may range from simpler fold increased risk of hospitalization secondary to RSV
conditions such as atrial septal defects, ventricular septal compared with normal-risk infants. The role of palivi-
defects, and tetralogy of Fallot to complex cardiac disease zumab in preventing RSV in CDH survivors has recently
including double outlet right ventricle, hypoplastic left been evaluated showing that CDH neonates benefit from
heart syndrome and single ventricle lesions.41 Histori- vaccination.50 In July 2023, the monoclonal antibody,
cally, complex CHD and CDH neonates were not typically nirsevimab, was approved by the US Food and Drug
offered ECMO, which affected survival in these complex Administration to protect newborns against RSV.51 The
neonates. Although CDH/CHD infants continue to have Advisory Committee on Immunization Practices and the
worse survival compared with CDH- only counterparts, Centers for Disease Control and Prevention in the USA
improvements in management, including the availability unanimously approved the administration of nirsevimab
of ECMO for appropriate patients, have led to survi- for protection against RSV in neonates.52
vors into adulthood.42 This special cohort requires close CDH- associated pulmonary hypoplasia and chronic
follow-up with congenital cardiac outpatient specialists in lung disease predispose patients to hypoxic events. Fami-
addition to CDH specialists. lies should be counseled about air travel or prolonged
time at higher altitudes. While there is not currently a
Lung function and development standardization of altitude testing for patients with CDH,
Pulmonary function in CDH survivors can be quantified the British Thoracic Society recommends altitude testing
with PFTs, V/Q scans and chest tomography (CT). PFTs for neonates less than 1 year old with a history of lung
show significantly lower forced expiratory volume in 1 disease or any child who required supplemental oxygen
s (FEV1), forced vital capacity (FVC), and FEV1/FVC in in the prior 6 months.53 In one multidisciplinary CDH
CDH survivors.15 16 38 43 Factors predictive of decreased clinic, high altitude is simulated by providing a fraction

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junction, impaired diaphragm crura function, intratho-
racic stomach, liver position, and patch repair.67–69 76–79
GERD prevalence varies considerably in long-term clinic
literature, ranging from 30% to 80%.4 6 69 80 81 High rates
of silent esophagitis are reported, along with rare cases of
Barrett’s esophagus and one case of esophageal adeno-
carcinoma.70 82 Unfortunately, despite this high risk of
silent esophagitis, no reliable GERD predictive factors
after age 6 years are identified.67
Most patients are discharged on acid suppression
therapy for the treatment or prophylaxis of reflux, but
the duration of treatment is undefined. Length of treat-
ment can be based on symptom questionnaires or invasive
testing.83 Understanding which patients would benefit
from acid suppression warrants continuing investigation,
as acid suppression therapy is not without its own side
effects which can include alterations to bone healing and
Figure 2 Severe air trapping and bullae in congenital growth, depressed immunological function of the gastro-
diaphragmatic hernia (CDH) survivor.
intestinal and respiratory systems, and alterations in the
gastrointestinal microbiome.84
Even more challenging is determining the role of
of inspired oxygen of 0.15 via non-rebreather mask while
fundoplication to improve growth, prevent respiratory
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oxygen saturation levels are monitored. Only 30% of
infections and manage esophagitis.78 79 85 86 Prospec-
patients who underwent high altitude testing were able to
tive evaluation of concomitant fundoplication at time
maintain oxygen saturations greater than 90% on initial
of CDH repair did not show a significant reduction in
attempt.13 Patients who do not pass altitude testing may
GERD symptoms in patients who underwent fundoplica-
benefit from supplemental oxygen use while at increased
tion compared with controls. Similarly, no difference in
altitude levels. Additionally, CDH survivors should be
growth parameters was achieved at 2 years of age when
counseled on the importance of avoiding smoking, vaping
comparing neonates who underwent fundoplication to
and secondary environmental exposures as incurred
controls.85 One of the largest studies from the French
damage to already structurally altered lung parenchyma
CDH Registry of over 700 neonates identified intratho-
could lead to worsening pulmonary function.
racic liver position, larger CDH defect size, prenatal
diagnosis, and patch repair as predictive variables for
Interventions to address severe V/Q mismatch
fundoplication.78 As an alternative to fundoplication to
Changes in perfusion and ventilation that worsen over
manage GERD, postpyloric tube feeding has been used
time are associated with poor functional status.14 A
in some centers.
small subset may also have air trapping with the devel-
Close follow-up in a multidisciplinary CDH clinic with a
opment of emphysematous bullae in both the ipsilat-
gastroenterologist and a nutritionist is necessary as GERD
eral and contralateral lungs on CXR and CT imaging
symptoms and growth failure often persist throughout
(figure 2).54–57 In adults with chronic obstructive pulmo-
childhood. Ongoing failure to thrive has multiple
nary disease or emphysema, research is ongoing on how
adverse consequences including impact on cognition.
to manage severely damaged lung and large pulmonary
It is important to identify these high-risk children who
bullae including use of endobronchial stents or valves
would benefit from nutrition-focused interventions such
and lung volume reduction surgery.58–66
as home visits and family education.72 87–89
GASTROINTESTINAL AND NUTRITIONAL SEQUELAE

CDH survivors often have gastrointestinal and nutritional MUSCULOSKELETAL CHANGES
morbidities including growth impairment, gastroesoph- Musculoskeletal anomalies, including scoliosis and
ageal reflux disease (GERD), esophagitis, functional pectus deformities, occur in 20–48% of patients with
esophageal abnormalities, hypermetabolic states, and CDH, much higher than the background incidence.90–92
oral aversion.67–70 The occasional need for fluid restric- Surgical approach is not associated with scoliosis rates,
tion in the setting of pulmonary disease can contribute supporting the theory that scoliosis in CDH is congen-
to the failure to reach growth goals.4 71–73 While gastros- ital and not acquired.93–96 Patients with CDH require
tomy placement theoretically allows for consistent caloric screening for scoliosis into adulthood and those with
intake, it is not a reliable marker of achieving growth scoliosis benefit from early referral for specialized ortho-
goals.72 74 75 pedic interventions including bracing and potential
GERD in CDH survivors is secondary to multiple factors surgery. Reported chest wall deformities include pectus
including abnormal positioning of the gastroesophageal excavatum (12–57%), pectus carinatum (2–13%) and

Open access
chest wall asymmetry (49%).43 92 95 97 As with scoliosis trial evaluating the impact of standardized and repeti-
deformities, CDH repair with primary closure, patch or tive cognitive training in a group of CDH survivors aged
muscle flap does not seem to be related to rates of chest 8–12 years showed improved verbal working memory and
wall deformities.90 93 95 98 99 visuospatial memory after intervention. Improvements
Defect size does appear to be related to the incidence in working memory did not persist past the 1-year time
of chest wall deformities and scoliosis.90 93 Altered in point, possibly due to the cessation of the intervention.119
utero development of musculoskeletal structures and the These types of cognitive training programs could be of
postnatal operative repair of CDH are both likely contrib- benefit to this long-term population and be organized
utors to the presence and degree of these reported through multidisciplinary clinics.
musculoskeletal anomalies. Discerning these prenatal Sensorineural hearing loss (SNHL) is an addi-
and postnatal predictive factors will require review of tional contributor to neurodevelopmental sequelae in
patients with long-term CDH in multidisciplinary clinics 7–56%.107 123 124 Predictors of SNHL include duration of
to inform clinical decisions. aminoglycoside treatment, loop diuretic therapy, inhaled
nitric oxide, longer duration of mechanical ventilation,
and use of high-frequency oscillation.107 123 125 Auditory
NEURODEVELOPMENT evaluation prior to discharge with methodical screening
Neurodevelopmental morbidity is a significant sequela thereafter is important to identify those at risk who could
affecting multiple neurological domains: fine and gross benefit from intervention.4 The AAP recommends at least
motor skills, auditory ability, visuospatial perception, one diagnostic audiology assessment by 24–30 months of
cognition, and language.5 8 100–107 Postnatally, neonates age followed by developmentally appropriate audiolog-
with critical illness have increased rates of neurodevel- ical screening.126 ECMO utilization is also a significant
opment deficits that persist into adulthood.108–111 CDH risk factor for hearing loss, and this population should
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neonates who use ECMO have impaired rates of verbal undergo enhanced surveillance screening.127
memory, working memory, visuospatial capabilities,
cognitive processing and motor skills.106 112 MRI imaging
in CDH survivors and ECMO survivors of other neonatal SURGICAL NEEDS
diseases shows alterations in the limbic system and white Patients often undergo additional surgical interven-
matter microstructure which correlated with neurolog- tions after CDH repair such as feeding tube insertion,
ical clinical sequelae in school-age children.108 Predictors fundoplication, pectus excavatum repair, recurrent
of worse neurodevelopmental outcomes are related to CDH repair, or operations for bowel obstruction. CDH
disease severity: size of defect, ECMO utilization, patch recurrence rates vary considerably ranging from 3% to
repair, intrathoracic liver position and prolonged oxygen 20% with potential under-reporting due to lack of stand-
requirement.102 113 114 Delays in motor performance can ardized radiological follow-up in many centers.68 128–130
be compounded by the inability to participate in physical The majority of recurrences, including those that may
exercise due to severe cardiopulmonary compromise.100 not undergo surgical intervention, occur within the
The AAP Section on Surgery recommends neurode- first year of life. In one study, 35% of recurrences were
velopmental screening starting at 9–12 months and then identified on routine follow-up imaging in a multidisci-
annually until 5 years of age.11 Neurodevelopmental plinary clinic.128 Monitoring for CDH recurrence and
assessment tools are challenging to implement in a young obstruction is particularly important as they can have life-
population as the responses often rely on parent-reported threatening consequences.4 75 79 128 131 132
outcomes and the training of the provider completing Risk factors for CDH recurrence include larger CDHSG
the assessment.115–118 defect size and patch repair.133 134 Meta-analysis showed a
Neurodevelopment beyond 5 years of age warrants threefold higher risk of recurrence in CDH neonates who
continued evaluation as deficits can persist into underwent thoracoscopic repair versus open repair.135
school-age children and even adulthood.103 105 Schiller The ability to follow CDHSG patients longitudinally with
and colleagues have completed multiple studies on prospective data collection will be of key importance to
neurodevelopmental outcomes in CDH survivors. Their understanding the long-term recurrence rates in patients
work supports the theory that neonates with a neuro- who undergo minimally invasive repair.
logical insult may have deficits that become apparent Bowel obstruction has been reported in up to 20% of
with older age when higher neurocognitive processing survivors and can be attributed to adhesions, volvulus,
is needed.119 120 CDH survivors have increased rates of or CDH recurrence.68 128 132 Patients with CDH have
learning disability, attention deficit hyperactivity disorder, abnormal fixation of the bowel secondary to hernia-
and developmental disability compared with matched tion into the chest which may contribute to the higher
controls.103 121 122 Standardization of neurological assess- rates of bowel obstruction.68 132 One study showed that
ment until adolescent survivors is supported as patients obstructive complications were higher in neonates with
may benefit from additional academic support. malrotation and non-fixed bowel documented on initial
Interventions, such as cognitive-based repetition, show CDH repair. Documenting the rotational status during
promise for neurodevelopment. A randomized controlled the initial CDH operations may help in predicting future

Open access
risk. Additionally, concurrent Ladd procedure with the grown to include social media outlets and discussion boards.
index CDH repair dependent on the clinical stability Interviewed parents commented on the anxiety of caring for
of the patient was shown to be protective against future a CDH child, particularly immediately after discharge, and
small bowel obstructions in one study by Heiwegen and reported the importance of having reliable follow-up, emer-
colleagues.132 136 Given the increased risk of obstructive gency plans, and a point of contact for issues as they arise.151
pathology in neonates who undergo CDH repair regard- Multidisciplinary clinics can provide this reliable follow-up
less of repair type, a posterior-anterior and lateral chest with regular telemedicine and in-person clinic visits.
radiograph is supported at every visit to evaluate for
recurrence as well as patient/ family education regarding
TRANSITIONING TO ADULT CARE
signs and symptoms of bowel obstruction.
Long-term follow-up for patients with CDH should extend
Finally, there is a higher prevalence (18%) of unde-
into adulthood as many alterations in cardiopulmonary
scended testes in males with CDH compared with the
physiology, nutrition, neurodevelopment and musculoskel-
generalized population, and most commonly occur on
etal development persist beyond adolescence.152 Attention
the ipsilateral side as CDH defect. This is theorized to be
to appropriate transition of care models has increased for a
secondary to decreased antenatal intra-abdominal pres-
variety of congenital anomalies. There are multiple barriers
sure and possibly the absence of diaphragmatic tissue
to transitioning to adult care including lack of patient
near the urogenital ridge leading to impaired testicular
interest and paucity of adult provider expertise.153 Significant
descent.137 138 Testicular position needs to be documented
planning is required to transition these patients to multiple
on physical examination, with surgical intervention
adult providers.
undertaken according to established guidelines.
Long-term clinics have begun publishing on important
patient benchmarks for transitioning care, including patient
SPECIAL POPULATIONS
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and adult provider disease-specific education about relevant
Fetal endoscopic tracheal occlusion (FETO) is a maternal/ surgical anatomy, yearly readiness assessments, and collabo-
fetal intervention aimed towards improving the degree of rative initiatives between pediatric and adult providers.154–156
pulmonary hypoplasia with minimally invasive techniques.139 The AAP, American College of Physicians and American
Long- term outcomes in neonates who underwent FETO Academy of Family Physicians published a detailed guide for
therapy are evolving. One study of 32 FETO neonates showed the transition of care citing six core elements vital for the
similar morbidity profiles though FETO survivors had higher transition process.157 These six tenets outline specific guide-
rates of pulmonary morbidity at 2 years (oxygen, bronchodi- lines and considerations when transitioning care, including
lator use) even after adjusting for disease severity.140 Sferra autonomy of decision- making in adult care, information
et al reported similar findings with 58% of FETO survivors privacy, and patient readiness questionnaires.157 Patients
requiring bronchodilator therapy or supplemental oxygen must become their own advocates, which is often a new role.
compared with non-FETO CDH survivors.141 FETO survivors Adequate education and preparation should begin early in
are likely to have tracheomegaly which has not been found long-term clinics to facilitate smooth transitions.
to have significant clinical impact.142 143 These long- term
studies confirm that neonates who underwent FETO inter-
RESEARCH OPPORTUNITIES
vention have favorable long-term survival rates and similar
This review has presented follow-up data from long-term
morbidity that should be managed in specialized long-term
clinics and larger multicenter analyses. One significant
CDH clinics.
assumption of long- term clinic data analysis is that the
patients who are followed in long-term CDH clinics repre-
PSYCHOSOCIAL IMPACT ON PATIENT AND FAMILY sent the patient population that was originally discharged.
Even with significant morbidity associated with CDH, most In the long-term multidisciplinary clinic at Boston Children’s
survivors report similar quality of life compared with controls Hospital, unpublished data have shown a 30% clinic attrition
in multiple domains including physical well-being, psycho- rate. However, there is a similar disease severity profile of
social well-being, autonomy, and feeling of belonging in a active patients compared with those who are lost to follow-up.
school or community.144–147 Michel and colleagues reported We encourage long- term clinics to include information
the largest contributors to decreased quality of life were poor about attrition rates in their publications so that readers may
peer/social support and lack of autonomy.148 better understand possible selection biases impacting the
Primary caregivers bear much of the emotional and finan- reported outcomes.
cial burden of caring for CDH children in the long term. CDH neonates who survive into adulthood remain a
Parents of CDH neonates report use of medical equipment target for research, particularly the manifestation of cardio-
utilization (62%), home health services (18%), and special pulmonary alterations with aging. Kraemer et al published
education services (28%).146 149 45% of families had to outcomes in a CDH survivor population with a median age
change their previous employment status to care for a child of 23 years. Peak oxygen consumption and O2 pulse were
with CDH. Families with less financial stability have worse significantly lower in ECMO-treated CDH survivors during
emotional well- being scores on quality of life surveys.150 exercise function. Right ventricular systolic pressure was
Support systems for parents of children with CDH have significantly elevated in ECMO-treated group compared with

Open access
non-ECMO survivors.29 These authors elected to additionally 7 Safavi A, Synnes AR, O’Brien K, et al. Multi-institutional follow-up
of patients with congenital diaphragmatic hernia reveals severe
complete a sex-based analysis which prompts the question disability and variations in practice. J Pediatr Surg 2012;47:836–41.
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could be altered in pregnancy. No studies to date have been up of patients with a congenital diaphragmatic hernia: a broad
spectrum of morbidity. Pediatr Surg Int 2009;25:1–17.
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We urge researchers to contribute to larger international Pediatr Surg 2006;41:888–92.
CDH registry groups and to publish information on these 10 Tsao K, Lally KP. The congenital diaphragmatic hernia study
relevant topics in CDH survivors. These research initiatives group: a voluntary International Registry. Semin Pediatr Surg
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12 IJsselstijn H, Breatnach C, Hoskote A, et al. Defining outcomes
following congenital diaphragmatic hernia using standardised
clinical assessment and management plan (SCAMP) methodology
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The emerging population of patients with CDH continues 13 Whitlock AE, Sheils CA, Zalieckas JM, et al. High altitude simulation
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Surg 2022;57:195–8.
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16 Wigen RB, Duan W, Moraes TJ, et al. Predictors of long-term
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17 Tragesser CJ, Hafezi N, Kitsis M, et al. Survivors of congenital
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18 Sanchez Mejia AA, Rodgers NJ. Evaluation and monitoring of
pulmonary hypertension in neonates with congenital diaphragmatic
Contributors Both authors made substantial contribution to this manuscript
hernia. Curr Treat Options Cardiovasc Med 2019;21:11.
including conception of design, drafting and editing, and are in agreement with its 19 Beals DA, Schloo BL, Vacanti JP, et al. Pulmonary growth and
publication. remodeling in infants with high-risk congenital diaphragmatic
Funding The authors have not declared a specific grant for this research from any hernia. J Pediatr Surg 1992;27:997–1001;
20 Shehata SMK, Tibboel D, Sharma HS, et al. Impaired structural
funding agency in the public, commercial or not-for-profit sectors.
remodelling of pulmonary arteries in newborns with congenital
Competing interests None declared. diaphragmatic hernia: a histological study of 29 cases. J Pathol
1999;189:112–8.
Patient consent for publication Not applicable. 21 Roubliova X, Verbeken E, Wu J, et al. Pulmonary vascular
Ethics approval Not applicable. morphology in a fetal Rabbit model for congenital diaphragmatic
hernia. J Pediatr Surg 2004;39:1066–72.
Provenance and peer review Not commissioned; externally peer reviewed. 22 Wong M, Reyes J, Lapidus-Krol E, et al. Pulmonary hypertension in
congenital diaphragmatic hernia patients: Prognostic markers and
Data availability statement No data are available.
long-term outcomes. J Pediatr Surg 2018;53:918–24.
Open access This is an open access article distributed in accordance with the 23 Kraemer US, Leeuwen L, Krasemann TB, et al. Characteristics of
Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits infants with congenital diaphragmatic hernia who need follow-up of
others to copy, redistribute, remix, transform and build upon this work for any pulmonary hypertension. Pediatr Crit Care Med 2018;19:e219–26.
purpose, provided the original work is properly cited, a link to the licence is given, 24 Lewis L, Sinha I, Kang S-L, et al. Long term outcomes in CDH:
cardiopulmonary outcomes and health related quality of life. J
and indication of whether changes were made. See: https://creativecommons.org/ Pediatr Surg 2022;57:501–9.
licenses/by/4.0/. 25 Mahmood B, Murthy K, Rintoul N, et al. Predicting treatment of
pulmonary hypertension at discharge in infants with congenital
ORCID iD diaphragmatic hernia. J Perinatol 2022;42:45–52.
Nicole Cimbak http://orcid.org/0009-0001-0317-2815 26 Dao DT, Patel N, Harting MT, et al. “Early left ventricular dysfunction
and severe pulmonary hypertension predict adverse outcomes in
“low-risk” congenital diaphragmatic hernia”. Pediatr Crit Care Med
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Long-Term Follow-Up of Patients With Congenital Diaphragmatic Hernia

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Long-Term Follow-Up of Patients With Congenital Diaphragmatic Hernia

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