Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects

doi:10.1016/S0022-3468(96)90095-0

Journal of Pediatric Surgery

Volume 31, Issue 8, August 1996, Pages 1096-1100

https://doi.org/10.1016/S0022-3468(96)90095-0 Get rights and content

Abstract

Between 1948 and 1980, 107 of 164 patients survived after repair of a congenital diaphragmatic defect. Sixty of the survivors (mean age, 29.6 years; SD, 9.0 years) underwent clinical examination, chest radiography, spirometry, and diffusing capacity measurement 11 to 41 years after the diaphragmatic repair. Twenty-seven of the 60 had body plethysmography, xenon 133 radiospirometry, and a test of bronchial hyperreactivity. Subjective physical performance was below average for eight patients (13%), seven patients (12%) had asthma, and four patients (7%) reported increased susceptibility to respiratory infections. Thirty-one patients (52%) had ventilatory impairment, which was obstructive in nine (15%), restrictive in seven (12%), and obstructive and restrictive in 15 patients (25%). Nine (35%) of the 26 patients tested had bronchial hyperreactivity. The presence of ventilatory impairment and bronchial hyperreactivity correlated with the initial clinical severity of the affliction. Chest asymmetry (29 patients; 48%) and scoliosis (16 patients; 27%) were more common among patients with ventilatory impairment than among those with normal spirometric findings. Ventilatory impairment and thoracic deformities are common in adults with repaired diaphragmatic defects. Surveillance should begin in infancy and continue into adulthood.

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Cited by (96)

Risk Factors for Preoperative Pneumothorax in Neonates With Isolated Left-Sided Congenital Diaphragmatic Hernia: An International Cohort Study
2024, Journal of Pediatric Surgery
We aimed to investigate the clinical characteristics and outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH) who developed preoperative pneumothorax and determine its risk factors.
We performed an international cohort study of patients with CDH enrolled in the Congenital Diaphragmatic Hernia Study Group registry between January 2015 and December 2020. The main outcomes assessed included survival to hospital discharge and preoperative pneumothorax development. The cumulative incidence of pneumothorax was estimated by the Gray test. The Fine and Gray competing risk regression model was used to identify the risk factors for pneumothorax.
Data for 2858 neonates with isolated left-sided CDH were extracted; 224 (7.8%) developed preoperative pneumothorax. Among patients with a large diaphragmatic defect, those with pneumothorax had a significantly lower rate of survival to discharge than did those without. The competing risks model demonstrated that a patent ductus arteriosus with a right-to-left shunt flow after birth (hazard ratio [HR]: 1.78; 95% confidence interval [CI]: 1.21–2.63; p = 0.003) and large defects (HR: 1.65; 95% CI: 1.13–2.42; p = 0.01) were associated with an increased risk of preoperative pneumothorax. Significant differences were observed in the cumulative incidence of pneumothorax depending on defect size and shunt direction (p < 0.001).
Pneumothorax is a significant preoperative complication associated with increased mortality in neonates with CDH, particularly in cases with large defects. Large diaphragmatic defects and persistent pulmonary hypertension were found to be risk factors for preoperative pneumothorax development.
LEVEL Ⅲ Retrospective Comparative Study.
Impact of a standardized management guideline for infants with CDH: A single-center experience
2023, Journal of Pediatric Surgery
Infants with congenital diaphragmatic hernia (CDH) are at high risk of death, even despite extracorporeal membrane oxygenation (ECMO) support. In January 2012 we implemented a standardized clinical practice guideline (CPG) to manage infants with CDH. We hypothesized that infants with CDH managed with CPG had better clinical outcomes, less ECMO utilization, and increased survival to discharge.
We conducted a retrospective pre-post study of infants with CDH admitted between January 2007 and July 2021 (n = 133). Patients were divided into Cohort 1, pre-CPG (January 2007 to December 2011, n = 54), and Cohort 2, post-CPG (January 2012 to July 2021, n = 79).
More patients in Cohort 1 were small for gestational age than in Cohort 2. No other patient demographics were different between cohorts. Cohort 2 had significantly lower ECMO utilization as compared to Cohort 1 (18% vs 50%, p<0.001). Cohort 2 had significantly higher survival to discharge compared to Cohort 1 (85% vs 57%, p<0.001). Survival for ECMO-treated patients in Cohort 2 was significantly higher than in Cohort 1 (71% vs 26%, p = 0.005). In Cohort 1, 70% of the non-survivors were repaired, of which 81% were repaired on ECMO. In Cohort 2, 8% of the non-survivors were repaired, none on ECMO. Only 3% in Cohort 2 were discharged with pulmonary hypertension medication.
A standardized CPG to manage patients with CDH decreased ECMO utilization and improved clinical outcomes including survival to discharge. Refinement of management strategies, implementation of new interventions, and meticulous care can improve outcomes in patients with CDH.
Lung volume reduction surgery for ipsilateral emphysematous bullae after congenital diaphragmatic hernia repair
2023, Journal of Pediatric Surgery Case Reports
Citation Excerpt :
Respiratory complications typically include the need for prolonged mechanical ventilation, chronic lung disease of infancy, frequent respiratory infections, recurrent asthma, and obstructive lung disease on pulmonary function tests (PFTs) [5,6]. While obstructive findings on PFTs are common in children with CDH [6–10], reports of true lobar emphysema in this population are rare. We report a case in which a patient with severe left-sided CDH underwent successful on-ECMO repair but required prolonged mechanical ventilation with the development of emphysematous bullous disease of the left lung.
Congenital diaphragmatic hernia (CDH) is characterized by a diaphragmatic defect with herniation of abdominal organs into the thoracic cavity. Outcomes are largely dependent on the degree of pulmonary hypoplasia and pulmonary hypertension, with the most severe cases associated with approximately 50% mortality. Long term pulmonary complications are well known, however, development of lobar emphysema leading to hemodynamic instability is exceedingly rare. We report the case of a patient with severe left-sided CDH who underwent successful diaphragmatic hernia repair but subsequently developed emphysematous bullous disease of the left lung with hemodynamic compromise, requiring emergent lung volume reduction surgery.
Survivors of congenital diaphragmatic hernia repair face barriers to long-term follow-up care
2021, Journal of Surgical Research
Congenital diaphragmatic hernia (CDH) carries high morbidity and mortality, and survivors commonly have neurodevelopmental, gastrointestinal, and pulmonary sequela requiring multidisciplinary care well beyond repair. We predict that following hospitalization for repair, CDH survivors face many barriers to receiving future medical care.
A retrospective review was conducted of all living CDH patients between ages 0 to 12 years who underwent repair at Riley Hospital for Children (RHC) from 2010 through 2019. Follow-up status with specialty providers was reviewed, and all eligible families were contacted to complete a survey regarding various aspects of their child's care, including functional status, quality of life, and barriers to care. Bivariate analysis was applied to patient data (P < 0.05 was significant) and survey responses were analyzed qualitatively.
After exclusions, 70 survivors were contacted. Thirty-three (47%) were deemed lost to follow up to specialist providers, and were similar to those who maintained follow-up with respect to defect severity type (A-D, P = 0.57), ECMO use (P = 0.35), number of affected organ systems (P = 0.36), and number of providers following after discharge (P = 0.33). Seventeen (24%) families completed the survey, of whom eight (47%) were deemed lost to follow up to specialist providers. Families reported distance and time constraints, access to CDH-specific information and care, access to CDH-specific resources, and access to healthcare as significant barriers to care. All respondents were interested in a multidisciplinary CDH clinic.
CDH survivors require multidisciplinary care beyond initial repair, but attrition to follow-up after discharge is high. A multidisciplinary CDH clinic may address caregivers’ perceived barriers.
Congenital Diaphragmatic Hernia
2019, Fetal Medicine: Basic Science and Clinical Practice
Medium and long-term respiratory outcome in patients operated from congenital diaphragmatic hernia: From a series of 56 patients
2018, Revue de Pneumologie Clinique
Les progrès récents concernant le diagnostic prénatal, la ventilation oscillatoire à haute fréquence et les thérapeutiques de l’hypertension artérielle pulmonaire (HTAP) ont progressivement amélioré la survie des patients porteurs de hernie diaphragmatique congénitale (HDC). Cependant, la HDC s’accompagne d’une morbidité élevée qui touche environ la moitié des enfants survivants. Les principales séquelles observées sont respiratoires (asthme, HTAP persistante [HTAPP], dysplasie broncho-pulmonaire, infections respiratoires) et digestives (reflux gastro-oesophagien [RGO]).
Cette étude rétrospective porte sur l’évolution respiratoire à moyen et long terme d’une cohorte de 56 enfants opérés d’une HDC au CHU de Strasbourg entre 1999 et 2017.
L’âge moyen était de 6,6 ans (minimum : 5 mois ; maximum : 19 ans). Dix-sept patients (30 %) présentaient des manifestations asthmatiques. Les EFR montraient un syndrome obstructif chez 5/11 patients, dont 2 avec une réversibilité post-bronchodilatateur et un syndrome restrictif chez 5/11 patients dont 3 présentaient une déformation thoracique et/ou une scoliose. Treize patients (23 %) ont présenté une dysplasie broncho-pulmonaire. Peu de patients présentaient des infections respiratoires répétées (3,6 %) et une insuffisance respiratoire chronique (5,3 %). Trente-neuf patients (70 %) ont présenté une HTAP initiale dont 2 ont évolué vers une HTAPP. Seize patients (29 %) présentaient une déformation thoracique et 48 patients (86 %) présentaient un RGO, dont 10 ont bénéficié d’une fundoplicature.
L’évolution respiratoire à long terme des patients opérés de HDC est, caractérisée par une prévalence modérée de symptômes respiratoires et d’altérations de la fonction respiratoire, dont il est important de tenir compte dans le suivi médical à l’adolescence puis à l’âge adulte. L’HTAP est fréquente en période néonatale, mais semble rarement persister.
Recent advances in prenatal diagnosis, high frequency oscillatory ventilation and pulmonary arterial hypertension (PAH) therapies have progressively improved the survival of patients with congenital diaphragmatic hernia (CDH). However, CDH is associated with high morbidity that affects about half of surviving children. The main sequelae observed are respiratory (asthma, persistent PAH [PPAH], bronchopulmonary dysplasia, respiratory infections) and digestive (gastroesophageal reflux disease [GERD]).
This retrospective study focuses on the medium and long-term respiratory evolution of a cohort of 56 children with CDH and operated at Strasbourg University Hospital between 1999 and 2017.
The mean age at assessment was 6,6 years (minimum: 5 months; maximum: 19 years). Seventeen patients (30 %) had asthmatic manifestations. Pulmonary Function Tests (PFT) showed obstructive patterns in 5/11 patients (2 with post-bronchodilator reversibility), and restrictive impairment in 5/11 patients, 3 of whom had thoracic deformity and/or scoliosis. Thirteen patients (23 %) had bronchopulmonary dysplasia. Few patients had recurrent respiratory infections (3.6 %) and chronic respiratory insufficiency (5.3 %). Thirty-nine patients (70 %) presented with an initial PAH, two of whom progressed to PPAH. Sixteen patients (29 %) had thoracic deformity and/or scoliosis and 48 patients (86 %) had GERD, 10 of whom had fundoplication.
The long-term respiratory outcome of CDH operated patients is characterized by a moderate prevalence of respiratory symptoms and alterations in PFT that are important to consider in their medical follow-up during adolescence and adulthood. PAH is common in the neonatal period but rarely seems to persist.

View all citing articles on Scopus

: Presented at the 1995 Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, San Francisco, California, October 13–15, 1995.

: Supported by the Foundation for Pediatric Research, Helsinki, Finland.

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Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects

Abstract

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

The mortality of congenital diaphragmatic hernia. Is total pulmonary mass inadequate, no matter what?

Ann Surg

Congenital diaphragmatic hernia: A 20 year experience

Br J Surg

Fate of hypoplastic lungs after repair of congenital diaphragmatic hernia

Arch Dis Child

Devenir a long terme des hernies diaphtagmatiques congenitales. Etude de 17 patients

Arch Fr Pediatr

Eventrations diaphragmatiques de l'adulte. A propos de vingt cas

Ann Chir Thorac Cardiovasc

Pulmonary sequelae in survivors of congenital diaphragmatic hernia

Thorax

Long-term effects on lung function after repair of congenital diaphragmatic hernia

Posterolateral diaphragmatic hernia—Long-term results