Ribcage deformity and the altered breathing pattern in children with osteogenesis imperfecta

LoMauro, Antonella; Fraschini, Paolo; Pochintesta, Simona; Romei, M.; Aliverti, Andréa

doi:10.1002/ppul.24039

Cited by 29 publications

(20 citation statements)

References 34 publications

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“…We believe that this interaction is mediated more by the combination of spinal and ribcage deformities rather than by the single one. Indeed, we have previously shown that the altered breathing pattern in severe OI worsens with age as a consequence of the combination between the congenital pectus carinatum and spinal deformity that starts in later childhood to develop thereafter [10]. The results of the present study seem to go towards this direction, with the combining index of deformity signi cantly being higher in OSA group.…”

Section: Discussionsupporting

confidence: 73%

“…For a respiratory point of view, OI is classi ed as a restrictive disease [6][7][8][9]. The two main factors predisposing to restricted respiratory problems in severe OI are severe kyphoscoliosis and structural modi cations of the ribcage, with the most important effect being thoraco-abdominal asynchrony with thoracic paradoxical inward movement [8,10,11]. Because in the most severe OI form these alterations systematically occur at rest in supine position, we wonder if they may negatively affect sleep.…”

Section: Introductionmentioning

confidence: 99%

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Eat, Breathe, Sleep With Osteogenesis Imperfecta

LoMauro

Landoni

Fraschini

et al. 2021

Preprint

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Background: Although Osteogenesis Imperfecta (OI) affects the connective tissue causing extremely brittle bones with consequent skeletal deformities, it is important to go beyond bones. Indeed, the quality of life in OI does not only depends on bones status, as OI might affect also other important functions. We have therefore implemented a multidisciplinary study to assess lung function, breathing pattern, sleep quality and nutritional status in 27 adult OI type III and IV patients (median age: 34.6 years; 19 women; 14 type III).Results: According to nocturnal oxygen desaturation, two groups were identified: 13 patients with (OI_OSA, incidence: 48.2%) and 14 without (no_OSA) obstructive sleep apnea. The former was characterized by higher spinal and ribcage deformity, by more restrictive lung function, by paradoxical thoracic breathing in supine position, by rapid and shallow breathing, by higher body mass index, by longer neck and waist circumferences; by higher abdominal volume and by greater percentage of body fat mass, particularly localized in the trunk. The best predictor of OI_OSA was the negative value of the supine ribcage contribution to tidal volume, followed by the ratio between the neck and the waist circumferences with body height and the supine thoraco-abdominal volumes phase shift angle. Conclusions: The pathophysiology of OI ensued a dangerous vicious circle, in which breathing, sleep and nutritional status are tightly linked, and they might all end up in negatively affecting the quality of life. The vicious circle is fed by some intrinsic characteristics of the disease (thoracic, cranial and mandibular deformities) and some bad daily habits of the patients (i.e. physical inactivity and low dietary quality). The former impacts on restricting the respiratory function, the latter makes Olers more prone to experience overweight or obesity. The main consequence is a high incidence of obstructive sleep apnea, which remains an underdiagnosed disorder in individuals with severe OI who are obese, with a neck to height ratio over than 31.6%, and characterized by paradoxical breathing in supine position. A multidisciplinary approach, including evaluations of breathing, sleep and nutrition, is required to better manage the disease and fulfil the maximizing well-being of OI patients.

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Section: Discussionsupporting

confidence: 73%

Section: Introductionmentioning

confidence: 99%

Eat, Breathe, Sleep With Osteogenesis Imperfecta

LoMauro

Landoni

Fraschini

et al. 2021

Preprint

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“…Tidal breathing analysis is a noninvasive method to assess respiratory function that does not require sedation . LoMauro et al evaluated the chest geometry and breathing pattern in children with severe and mild osteogenesis imperfecta (OI) using opto‐electronic plethysmography (OEP). They demonstrated that the abdominal muscle contribution to tidal volume was increased, the pulmonary rib cage contribution to tidal volume was decreased and the sternal angle was lower in OI children, indicating a mechanical disadvantage for respiratory function.…”

Section: Introductionmentioning

confidence: 99%

Pediatric Pulmonology year in review 2018: Asthma, physiology/pulmonary function testing, and respiratory infections

Loughlin

Muston

et al. 2019

Pediatric Pulmonology

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Pediatric Pulmonology publishes original research, reviews, and case reports related to a wide range of children's respiratory disorders. In our “Year in Review” series, we summarize publications in our major topic areas from 2018, in the context of selected literature in these areas from other journals relevant to our discipline. This review covers selected articles on asthma, physiology/lung function testing, and respiratory infections.

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“…The association of OI and chest wall deformities has been reported but is infrequent. 5 6 7 LoMauro et al evaluated pulmonary function in 22 patients with OI and found 7 cases of PC; all had Type III OI. 6…”

Section: Introductionmentioning

confidence: 99%

Successful Brace Treatment of Pectus Carinatum in Osteogenesis Imperfecta Using the Dynamic Compression System

Orrick

Pierce

Snyder

et al. 2019

European J Pediatr Surg Rep

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Osteogenesis imperfecta (OI) is a genetic disorder of collagen resulting in a “fragile” skeleton with increased fracture risk and other complications, dependent on the specific variant. Pectus deformities of the chest wall, while not common, can be associated with OI. The use of a pectus carinatum brace in a patient with OI poses unknown risks for fractures and adverse treatment outcomes. We successfully applied external compression bracing using the dynamic compression system to one such patient. This case illustrates the ability to treat an OI patient with pectus carinatum using a nonsurgical brace, without complications, resulting in an excellent cosmetic result.

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Ribcage deformity and the altered breathing pattern in children with osteogenesis imperfecta

Cited by 29 publications

References 34 publications

Eat, Breathe, Sleep With Osteogenesis Imperfecta

Eat, Breathe, Sleep With Osteogenesis Imperfecta

Pediatric Pulmonology year in review 2018: Asthma, physiology/pulmonary function testing, and respiratory infections

Successful Brace Treatment of Pectus Carinatum in Osteogenesis Imperfecta Using the Dynamic Compression System

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