Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice

doi:10.1016/j.jpedsurg.2012.01.032

Journal of Pediatric Surgery

Volume 47, Issue 5, May 2012, Pages 836-841

https://doi.org/10.1016/j.jpedsurg.2012.01.032 Get rights and content

Abstract

Background/Purpose

Survivors of congenital diaphragmatic hernia (CDH) have a high incidence of morbidity. Variability in follow-up practices between institutions may affect perception of disability and prevent population-based outcome analysis.

Methods

A survey of follow-up practices at 16 centers within a population-based CDH network was performed. A descriptive analysis of outcomes (minimum 24 months postdischarge) of CDH survivors from the 2 largest centers was performed.

Results

The nature of follow-up of CDH survivors was highly variable in 12 of 16 responding centers, ranging from ad hoc, community-based, and pediatrician-sponsored follow-up to a single perinatal center-based multispecialty CDH clinic. Outcomes at 24 to 36 months were reported from the 2 largest centers (n = 44). Among survivors, neurodevelopmental disability was most common (12/44; 27%) followed by gastrointestinal (9; 20.5%), pulmonary (5; 11.4%), musculoskeletal (5; 11.4%), and cardiac (2; 4.5%). Additional surgery was required in 17 patients (38.6%), including recurrent CDH repair in 7 (15.9%). Five patients (11.4%) had hearing loss. Among 41 children with available 24-month data, 32 (78%), 17 (41.5%), and 14 (34.1%) patients had weights below the 50th, 25th, and 3rd percentiles, respectively.

Conclusion

Congenital diaphragmatic hernia survivorship is associated with significant disability. Standardization of follow-up practices is essential to enable population-based outcomes analysis.

Section snippets

Methods

A questionnaire that specifically sought descriptive details of CDH follow-up at all 16 Canadian perinatal centers that treat CDH was sent to each site. The questionnaire focused specifically on the following attributes of CDH follow-up: (1) whether follow-up was conducted systematically or in an “ad hoc” manner; (2) whether follow-up occurred through a “single-stop,” dedicated multispecialty clinic vs single or multispecialty follow-up through individual clinics; (3) who was identified as the

CDH follow-up practices across CAPSNet centers

Twelve (75%) of 16 CAPSNet centers completed the survey. Of these, only 1 center (B) used a true “one stop” multispecialty and interdisciplinary CDH clinic based at the CAPSNet center. Five of 12 centers reported multidisciplinary follow-up at the specialized center that was decentralized across multiple clinics (eg, surgery, specialty pediatrics, and neonatology). In the remaining centers, follow-up was largely community based under the coordination of a general or specialty pediatrician, with

Discussion

There has been a steady improvement in CDH survival over the past 20 years. This improvement has resulted from a number of practice changes including preoperative stabilization, delayed surgical repair, and the development of new therapies for the treatment of pulmonary hypertension [13], [14]. As CDH survival has improved, so too has the awareness of a need to consider long-term outcomes in the assessment of the quality of care provided. Numerous studies have documented the frequency and

Acknowledgment

The authors acknowledge the financial support from the Canadian Institute for Health Research (CIHR; MOP-69050) and the organizational support of the CIHR team in maternal infant care and the Ontario Ministry of Health and Long-Term Care.

References (31)

D.A. Lazar et al.
Impact of prenatal evaluation and protocol-based perinatal management on congenital diaphragmatic hernia outcomes
J Pediatr Surg
(2011)
D.P. Lund et al.
Congenital diaphragmatic hernia: the hidden morbidity
J Pediatr Surg
(1994)
R. Baird et al.
Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network
J Pediatr Surg
(2011)
E.D. Skarsgard et al.
Canadian Pediatric Surgical Network: a population-based pediatric surgery network and database for analyzing surgical birth defects: The first 100 cases of gastroschisis
J Pediatr Surg
(2008)
F.R. Moya et al.
Evidence-based management of infants with congenital diaphragmatic hernia
Semin Perinatol
(2005)
C. Chen et al.
Long-term functional impact of congenital diaphragmatic hernia repair on children
J Pediatr Surg
(2007)
P.P. Chiu et al.
The price of success in the management of congenital diaphragmatic hernia: is improved survival accompanied by an increase in long-term morbidity?
J Pediatr Surg
(2006)
P.J. Davis et al.
Long-term outcome following extracorporeal membrane oxygenation for congenital diaphragmatic hernia: the UK experience
J Pediatr
(2004)
S.M. Jaillard et al.
Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study
Ann Thorac Surg
(2003)
C.S. Muratore et al.
Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic
J Pediatr Surg
(2001)

C.S. Muratore et al.

Nutritional morbidity in survivors of congenital diaphragmatic hernia

J Pediatr Surg

(2001)

K. Vanamo et al.

Chest wall and spinal deformities in adults with congenital diaphragmatic defects

J Pediatr Surg

(1996)

E. Danzer et al.

Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program

J Pediatr Surg

(2010)

F. Morini et al.

Hearing impairment in congenital diaphragmatic hernia: the inaudible and noiseless foot of time

J Pediatr Surg

(2008)

P.Y. Cheung et al.

Prolonged use of pancuronium bromide and sensorineural hearing loss in childhood survivors of congenital diaphragmatic hernia

J Pediatr

(1999)

Cited by (55)

Evaluation of brain development and damage using magnetic resonance imaging of congenital diaphragmatic hernia survivors: An analysis using the global brain abnormality score
2024, Pediatrics and Neonatology
The short-term prognosis of central nervous system in congenital diaphragmatic hernia (CDH) survivors has been determined by magnetic resonance imaging (MRI), but its relationship with acute management is unclear. We aimed to investigate the association between the intubation period and the Global Brain Abnormality Score (GBAS) in CDH survivors using brain MRI.
Fifty-seven patients with CDH who were hospitalized at a single NICU between January 2004 and December 2019 were retrospectively reviewed. After excluding 5 patients who died shortly after birth and two who could not be weaned from the ventilator, the acute management of the 50 remaining patients was investigated. We also investigated the relationship between the GBAS and intubation period in 25 patients who underwent brain MRI at discharge.
The long-intubation group (intubation ≥12 days) had lower Apgar scores and fetal lung-thoracic ratios, and longer time to radical surgery, and parenteral nutrition and tube feeding periods. Nitric oxide inhalation, liver prolapse, patch closure, and extracorporeal membrane oxygenation were independent risk factors for long-intubation. Eighty-four percent of CDH survivors had some imaging abnormalities, including developmental and signaling abnormalities. In the long-intubation group, the body of the corpus callosum was thin and the cerebral hemispheric space was widened, and GBAS deterioration was significantly related to the intubation period.
Brain MRI abnormalities were found in 84% of CDH survivors. Prolonged intubation is associated with worsening of the GBAS. Thus, the duration of intubation may be a surrogate outcome for the neurological prognosis of CDH survivors.
Impact of bowel rotation and fixation on obstructive complications in congenital diaphragmatic hernia
2023, Journal of Pediatric Surgery
Small bowel obstruction (SBO) is a known complication after congenital diaphragmatic hernia (CDH) repair, which can require surgery and even extensive bowel resection causing short bowel syndrome (SBS). We investigate whether specific bowel rotation and fixation can be used as a predictor for SBO including volvulus.
A retrospective review of 256 CDH survivors following repair from 2003 to 2020 was performed. Operative notes and upper gastrointestinal series (UGI) were screened to determine the rotation and fixation of the bowel. Primary outcomes included SBO occurrence, SBO treated surgically, and volvulus. For statistical analysis Fisher's exact test was utilized.
Twenty-two (9%) patients presented with SBO and majority, 19 (86%), required surgery. Adhesion were observed in 10 (45%), recurrence in 5 (23%), and extensive volvulus leading to SBS in 3 (14%). Both rotation and fixation were recorded in 117 (46%). Presence of left CDH with malrotation and nonfixation was a significant predictor for SBO requiring surgery (P<0.05 vs all other groups). All 3 patients with extensive volvulus had left CDH with nonfixed bowel (100%), however only 1 had malrotation (33%).
Malrotation and nonfixation are associated with increased SBO in CDH. Normal rotation is not protective and patients are still at risk for volvulus resulting in SBS. SBO requiring surgical intervention is common in CDH. Bowel rotation and fixation are important determinants that, should be routinely documented and education about the risk of SBO should be included in family counseling.
Level IV – Case Series
Analysis of perceived risk and satisfaction with telematic follow-up in patients and families of congenital diaphragmatic hernia patients during SARS-CoV-2 pandemia
2023, Journal of Healthcare Quality Research
The impact of COVID-19 in families and patients with congenital diaphragmatic hernia (CDH) is unknown, this situation has generated uncertainty not only in family members but also in the optimal outpatient follow-up. Telehealth has become a fundamental tool for the follow-up during the pandemic. The objective of this survey is to evaluated the impact of SARS-CoV-2 in families and patients with CDH and the satisfaction with telematic follow-up.
Telephone survey of patient's caregivers with CHD, aged 1–16 years, followed in neonatal surgery outpatients, from January 31, 2020 to November 15, 2020. The ethical clearance for this study was taken from the Clinical Research Ethics Committee of our Research Institute vide letter number VHIR/239283/01.01.2021.
81 surveys of 100 patients with active follow-up were carried out. There were no refusals in any contacted parents. There were 30 contacts (37%), 44.8% at school and 27.6% from cohabiting family members. Four infections (4.9%) were diagnosed, half symptomatic. In 40 patients (49.4%) the follow-up was telematic, with a mean score of 3.1 ± 1.3 out of 5. For future controls, 65% prefer presential follow-up, 25% alternate and 10% telematics. 50.6% reported greater anxiety and 34.6% (28/81) extreme measures of isolation, being more accentuated in the group of 3–6 years (p < 0.05).
The impact of COVID19 in patients with CHD is not greater than in the general pediatric population. Although the incorporation of the telehealth was well valued, most of the caregivers prefer the face-to-face outpatient follow-up.
Se desconoce el impacto de la COVID-19 en los familiares y pacientes con hernia diafragmática congénita (HDC). Esta situación ha generado incertidumbre, no solo en los familiares, sino también en el seguimiento óptimo de los pacientes. La telesalud se ha convertido en una herramienta fundamental para el seguimiento durante la pandemia. El objetivo de esta encuesta es evaluar el impacto del SARS-CoV-2 en los familiares y pacientes con HDC, así como la satisfacción con el seguimiento telemático.
Encuesta telefónica realizada a los cuidadores de los pacientes con HDC, de edades comprendidas entre 1 y 16 años, a quienes se realizó un seguimiento en la unidad ambulatoria de cirugía neonatal desde el 31 de enero al 15 de noviembre de 2020. La autorización ética para este estudio se obtuvo del Comité de Ética de Investigación Clínica de nuestro Instituto de Investigación, con número de referencia VHIR/239283/01.01.2021.
Se llevaron a cabo 81 encuestas de 100 pacientes con seguimiento activo. Ninguno de los padres contactados rechazó el seguimiento. Se produjeron 30 contactos (37%): el 44,8% en la escuela, y el 27,6% a los familiares convivientes. Se diagnosticaron cuatro infecciones (4,9%), la mitad de ellas sintomáticas. En 40 pacientes (49,4%), el seguimiento fue telemático, con una puntuación media de 3,1 ± 1,3 sobre 5. Para los controles futuros, el 65% prefirió el seguimiento presencial, el 25% alternativo, y el 10% telemático. El 50,6% reportó una mayor ansiedad, y el 34,6% (28/81) medidas extremas de aislamiento, siendo más acentuadas en el grupo de 3 a 6 años (p < 0,05).
El impacto de la COVID19 en los pacientes con HDC no es mayor en la población pediátrica general. Aunque la incorporación de la telesalud fue bien valorada, la mayoría de los cuidadores prefieren el seguimiento presencial.
Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study
2022, Journal of Pediatric Surgery
Congenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular morbidity.
This was a nationwide population-based prospective case-control study within a cohort of Swedish children with CDH, born 1982–2015. Five controls for each patient were randomly sampled from the population. The outcomes were the corresponding International Statistical Classification of Disease (ICD) codes for cardiovascular diagnoses according to ICD 9 and 10.
There was an overrepresentation of cardiovascular diagnoses in the CDH group after one year of age compared to the control group, 8.0vs 0.5% (n = 53 versus n = 16). The risk of having a cardiovascular diagnosis in this CDH group was 15 times higher compared to the control group (HR 15.8, 95% CI: 9–27.6, p < 0.005). The diagnoses of cardiac arrhythmias and systemic hypertension were less common in the CDH group before the age of one year compared to the CDH group beyond the age of one year. Arrhythmia 3.7 vs 15.1%, systemic hypertension 3.7 vs 7.5%.
CDH survivors have increased cardiovascular morbidity during childhood and young adulthood. This implies that structured follow up programs, covering cardiovascular morbidity, needs to be developed and should be offered in pediatric and adult care. Being born with CDH seems to be a risk factor for future cardiovascular diagnoses.
Level 3: Case-Control Study
Long term outcomes in CDH: Cardiopulmonary outcomes and health related quality of life
2022, Journal of Pediatric Surgery
Citation Excerpt :
Primary vs patch diaphragm repair [30] Thirteen studies [16,26,27,31–40] investigated pulmonary hypertension in CDH survivors. Five of which were case control studies [16,27,31–33], six retrospective cohort studies [34–37,39,40], and two prospective cohort studies [26,38].
With improvements in clinical management and an increase in CDH survivorship there is a crucial need for better understanding of long-term health outcomes in CDH.
To investigate the prevalence of cardiopulmonary health morbidity and health related quality of life (HRQoL) in CDH survivors.
We included all studies (n = 65) investigating long-term cardiopulmonary outcomes in CDH patients more than 2 years published in the last 30 years. The Newcastle-Ottawa Scale and the CASP checklist for cohort studies were utilized to assess study quality. Results were reported descriptively and collated by age group where possible.
The incidence of pulmonary hypertension was highly variable (4.5–38%), though rates (%) appeared to diminish after 5 years of age. Lung function indices and radiological outcomes were frequently abnormal, and Health Related Quality of Life (HRQoL) reduced also. Long term diseases notably emphysema and COPD are not yet fully described in the contemporary literature.
This study underscores cardiopulmonary health morbidity and a reduced HRQoL among CDH survivors. Where not already available dedicated multidisciplinary follow-up clinics should be established to support these vulnerable patients transition safely into adulthood. Future research is therefore needed to investigate the risk factors for cardiopulmonary ill health and morbidity in CDH survivors.
Systematic review of case control and cohort studies.
Proton pump inhibitors are still overprescribed for hospitalized children
2022, Archives de Pediatrie
The use of proton pump inhibitors has increased exponentially over the past 20 years. Several side effects have been reported and concerns exist about the consequences of long-term proton pump inhibitors on health, leading to limitation of their use. The present study analyzed prescriptions of proton pump inhibitors at inpatient units and assessed their compliance with current recommendations.
This single-center, observational, retrospective study reviewed medical file of patients hospitalized at the pediatric medical departments of the Bordeaux University Hospital between April 1 and September 30, 2019. Patients younger than 18 years, hospitalized in the pediatric hospital units and treated with proton pump inhibitors were included. Prescriptions of proton pump inhibitors were compared with French and international guidelines.
Proton pump inhibitors were prescribed for 251 of 2237 children (11%), mainly for gastroesophageal reflux disease (47%) and prevention of peptic ulcer disease (32.7%). Proton pump inhibitor prescription complied to recommendations in 34.5% of cases, less often in children aged younger than 1 year (13.5%) than in older children. Compliance to recommendations was lower when proton pump inhibitors were indicated for the prevention of peptic disease (5%) than for gastroesophageal reflux disease (48%).
Proton pump inhibitors are frequently prescribed for hospitalized children, and indications comply with recommendations in only 35% of the cases. Efforts in spreading awareness of the recommendations on the use of proton pump inhibitors in children are mandatory among hospital pediatricians.

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CAPS paperMulti-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice☆

Abstract

Background/Purpose

Methods

Results

Conclusion

Section snippets

Methods

CDH follow-up practices across CAPSNet centers

Discussion

Acknowledgment

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Semin Perinatol

J Pediatr Surg

J Pediatr Surg

J Pediatr

Ann Thorac Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

CAPS paper
Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice☆