Chest wall and spinal deformities in adults with congenital diaphragmatic defects

Vanamo, Kari; Peltonen, Jari; Rintala, Risto J.; Lindahl, Harry; Jääskeläinen, Juha E.; Louhimo, I

doi:10.1016/s0022-3468(96)90152-9

Cited by 90 publications

(73 citation statements)

References 19 publications

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“…The majority of patients have a pectus excavatum [32,59,64,68,142]. The close relationship between development of the lung, diaphragm, and thoracic cage is the reason that deformities of the chest wall are more common in patients with CDH [32].…”

Section: Chest Wall Deformitiesmentioning

confidence: 99%

“…First of all the thoracic cavity on the affected side may be reduced in size due to the smaller lung volume. Secondly, the increased work of breathing in these children may contribute to the development of a pectus abnormality [32,110] because more negative intrapleural pressure is required to inflate the lungs [142]. Thirdly more negative intrathoracic pressure promotes retraction of the chest wall in its most compliant section, the cartilaginous anterior wall [142].…”

Section: Chest Wall Deformitiesmentioning

confidence: 99%

“…Secondly, the increased work of breathing in these children may contribute to the development of a pectus abnormality [32,110] because more negative intrapleural pressure is required to inflate the lungs [142]. Thirdly more negative intrathoracic pressure promotes retraction of the chest wall in its most compliant section, the cartilaginous anterior wall [142]. Postoperative empyema has been shown to increase the likelihood of postoperative thoracic deformity [143,144].…”

Section: Chest Wall Deformitiesmentioning

confidence: 99%

“…Postoperative empyema has been shown to increase the likelihood of postoperative thoracic deformity [143,144]. Since chest wall deformities are more common among patients with anatomically large diaphragmatic defects, it is suggested that repair of a large defect causes tension, interfering with normal development of the thoracic cage and promoting asymmetry and/or flat chest [142].…”

Section: Chest Wall Deformitiesmentioning

confidence: 99%

“…Lund et al [32] described that 4 of 33 patients had thoracic scoliosis concave towards the hernia side, only one of them required bracing. The only study focusing primarily on chest wall deformities and scoliosis in CDH patients came from Vanamo et al [142] describing sixteen (27%) patients with significant radiological scoliosis (Cobb's angle C10°). The mean curve was significantly greater in patients with a large defect.…”

Section: Scoliosismentioning

confidence: 99%

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The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

et al. 2008

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Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a mortality rate of approximately 40-50%, depending on case selection. It has been suggested that new therapeutic modalities such as nitric oxide (NO), high frequency oxygenation (HFO) and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation. When these new therapies indeed prove to be beneficial, a larger number of children with severe forms of CDH might survive, resulting in an increase of CDH-associated complications and/or consequences. In follow-up studies of infants born with CDH, 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. CDH has also been associated with persistent pulmonary vascular abnormalities, resulting in pulmonary hypertension in the neonatal period.

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Section: Chest Wall Deformitiesmentioning

confidence: 99%

Section: Chest Wall Deformitiesmentioning

confidence: 99%

Section: Chest Wall Deformitiesmentioning

confidence: 99%

Section: Chest Wall Deformitiesmentioning

confidence: 99%

Section: Scoliosismentioning

confidence: 99%

See 3 more Smart Citations

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

et al. 2008

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Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia

et al. 2005

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Our objective was to study long-term respiratory outcomes of congenital diaphragmatic hernia (CDH) treated in the perinatal period. This was a cohort study with 26 adolescent survivors and age- and gender-matched controls. Medical histories were retrieved from hospital charts and questionnaires. Pulmonary function testing included measurement of maximum inspiratory and expiratory pressures (MIPS and MEPS) and maximum voluntary ventilation (MVV). Unpaired two-tailed t-test and nonlinear regression were used for statistical analysis. Significant differences were found in forced expiratory volume in one second (FEV(1)) (79% +/- 16% vs. 94% +/- 10%, P < 0.001), FEF(25-75) (62% +/- 24% vs. 84% +/- 15%, P < 0.001), FRC (114% +/- 20% vs. 95% +/- 13%, P < 0.001), RV/TLC (31% +/- 10% vs. 22% +/- 6%, P < 0.001), MVV (74% +/- 16% vs. 90% +/- 13%, P < 0.001), and MIPS (69% +/- 19% vs. 84% +/- 16%, P < 0.01), with numbers indicating percent predicted of reference values +/- SD. Reduction of MVV was not independent from FEV1 (r = 0.83). Forty-eight percent of patients vs. 4% of controls showed significant improvement of FEV1 after bronchodilators (86% +/- 15 vs. 98% +/- 10, P < 0.01). Forty-six percent of patients had abnormalities of the chest wall or spinal column such as pectus excavatum, pectus carinatum, and scoliosis, mostly mild or moderate. In conclusion, long-term respiratory outcome in adolescent CDH is associated with mild to moderate airway obstruction, a high prevalence of response to bronchodilators, and decreased inspiratory muscle strength. This should guide follow-up scheduling and should be taken into account for perioperative and critical care management.

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Pulmonary function and exercise testing in adult survivors of congenital diaphragmatic hernia

Peetsold

Vonk‐Noordegraaf

Heij

et al. 2007

Pediatric Pulmonology

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Congenital diaphragmatic hernia (CDH) is accompanied by pulmonary hypoplasia and structural abnormalities of the pulmonary vascular bed. It is unknown whether pulmonary function, exercise capacity, and gas exchange during exercise are impaired in adult CDH survivors. The objective of this study was to assess the long-term pulmonary function, exercise capacity, and gas exchange during exercise and relate these findings with quality of life. Of the 23 patients eligible for this study, 12 adult CDH survivors (mean age, 24.3 +/- 4.1 years) with high-risk CDH agreed to participate. Pulmonary function tests, diffusion capacity, and a cardiopulmonary exercise test (CPET) were performed. The FEV1 (mean z-score +/- SD; -1.30 +/- 1.37), FEF25-75% (-1.49 +/- 1.14), and the KCO (-1.03 +/- 1.24) were found to be lower in CDH survivors. The RV/TLC ratio (28.2% +/- 5.0%) was found to be higher. Despite these abnormalities, percent predicted work load (102% +/- 17.2%) and percent predicted maximal oxygen uptake (90.8% +/- 18.9%) were normal in most of the patients. The quality of life of CDH survivors, assessed with the SF-36 questionnaire, is comparable to the general population. Comparison of participants to non-participants did not reveal significant differences in clinical characteristics. In this first study assessing pulmonary function in adult survivors of CDH, mild airway obstruction was observed in most of the patients together with a slightly reduced diffusion capacity for CO. Exercise capacity and gas exchange parameters were normal in this group, indicating that patients do not have a physical impairment, as reflected by a normal quality of life of CDH patients.

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Chest wall and spinal deformities in adults with congenital diaphragmatic defects

Cited by 90 publications

References 19 publications

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia

Pulmonary function and exercise testing in adult survivors of congenital diaphragmatic hernia

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