Acquisition bias may have led to acceptance of the false null hypothesis that prevalence of scoliosis is the same in cystic fibrosis as the general population

Hathorn, C; Fall, A.; McGurk, Simon; Urquhart, Don S.

doi:10.1002/ppul.22947

Cited by 5 publications

(11 citation statements)

References 4 publications

(6 reference statements)

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“…Recently, Oliazadeh et al found abnormalities in osteoblast primary cilia in idiopathic scoliosis patients and Grimes et al have demonstrated a developmental zebra-fish model that suggests a link between ependymal cell cilia development and the onset of scoliosis [18,19]. On the other hand, a pilot study on 48 PCD patients in The Netherlands did not result in retrieval of scoliosis cases, and in other pediatric pulmonary diseases, such as cystic fibrosis, a scoliosis prevalence of 2-11% has been described [20,21]. Based on the curve pattern and clinical presentation in our study of PCD patients, all curves were considered to be of the idiopathic-type, but selection bias cannot be excluded.…”

Section: Discussionmentioning

confidence: 99%

Scoliosis convexity and organ anatomy are related

et al. 2017

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Purpose Primary ciliary dyskinesia (PCD) is a respiratory syndrome in which 'random' organ orientation can occur; with approximately 46% of patients developing situs inversus totalis at organogenesis. The aim of this study was to explore the relationship between organ anatomy and curve convexity by studying the prevalence and convexity of idiopathic scoliosis in PCD patients with and without situs inversus.Methods Chest radiographs of PCD patients were systematically screened for existence of significant lateral spinal deviation using the Cobb angle. Positive values represented right-sided convexity. Curve convexity and Cobb angles were compared between PCD patients with situs inversus and normal anatomy. Results A total of 198 PCD patients were screened. The prevalence of scoliosis (Cobb [10°) and significant spinal asymmetry (Cobb 5-10°) was 8 and 23%, respectively. Curve convexity and Cobb angle were significantly different within both groups between situs inversus patients and patients with normal anatomy (P B 0.009). Moreover, curve convexity correlated significantly with organ orientation (P \ 0.001; / = 0.882): In 16 PCD patients with scoliosis (8 situs inversus and 8 normal anatomy), except for one case, matching of curve convexity and orientation of organ anatomy was observed: convexity of the curve was opposite to organ orientation. Conclusions This study supports our hypothesis on the correlation between organ anatomy and curve convexity in scoliosis: the convexity of the thoracic curve is predominantly to the right in PCD patients that were 'randomized' to normal organ anatomy and to the left in patients with situs inversus totalis.

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Section: Discussionmentioning

confidence: 99%

Scoliosis convexity and organ anatomy are related

et al. 2017

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“…The prevalence of scoliosis in CF ranges from 9.9 to 15.5%, has features of adolescent idiopathic scoliosis, and is greater in children older than 10 years ( Table 1 ) [ 3 , 7 , 10 ]. This is higher than the 0.5–3.2% frequency of scoliosis recorded in the general population [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…This is higher than the 0.5–3.2% frequency of scoliosis recorded in the general population [ 4 ]. Common deformity pattern is that of nonprogressive thoracic curves with T6–T8 apex; scoliosis progression has been observed in 28.6% of adolescent patients with mean curves 12° and in 11.1% of adult patients despite the small curves of mean 15°, which is contrary to adolescent idiopathic scoliosis [ 3 , 7 ]. This may be associated with the delayed bone age observed in patients with CF [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…Case series of children and adolescents with CF and scoliosis have been reported by a number of centres including our own centre [ 2 – 4 , 7 , 9 , 10 ]. However, the paradigm of a radiologically significant (Cobb angle > 10°) versus a clinically important scoliosis remains a key message from these reports.…”

Section: Introductionmentioning

confidence: 99%

“…However, the paradigm of a radiologically significant (Cobb angle > 10°) versus a clinically important scoliosis remains a key message from these reports. Reduced respiratory reserve when present due to underlying lung disease may make CF patients unsuitable surgical candidates; however the spinal deformity is usually mild and does not require surgical correction [ 3 , 7 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

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Scoliosis Surgery in Cystic Fibrosis: Surgical Considerations and the Multidisciplinary Approach of a Rare Case

Mataliotakis

Pearson

Urquhart

et al. 2016

Case Reports in Orthopedics

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Spinal deformity in patients with cystic fibrosis (CF) is usually mild requiring no treatment. These patients are rarely considered as surgical candidates for scoliosis correction, as the pulmonary condition and other comorbidities increase the risk of general anaesthesia and recovery. This paper reviews all the literature up to date with regard to scoliosis in patients with CF and reports this unique case of a 14-year-old Caucasian girl with progressive scoliosis, who was treated surgically at the age of 17. She underwent a posterior spinal fusion T2-L3 with the use of unilateral segmental instrumentation. Preoperative workup included respiratory, cardiac, anaesthetic, endocrine, and dietician reviews, as well as bone density optimisation with zoledronic acid and prophylactic antibiotics. Surgical time was 150 minutes and intraoperative blood loss was 47% of total blood volume. Postoperative intensive care included noninvasive ventilation, antibiotic cover, pain management, chest physiotherapy, pancreatic enzyme supplementation, and nutritional support. She was discharged on day 9. At follow-up she had a good cosmetic outcome, no complaints of her back, and stable respiratory function. Multidisciplinary perioperative care and meticulous surgical technique may reduce the associated risks of major surgery in CF patients, while achieving adequate deformity correction and a good functional outcome.

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