Non‐invasive CT screening for pulmonary arteriovenous malformations in children with confirmed hereditary hemorrhagic telangiectasia: Results from two pediatric centers

Soysal, N.; Eyries, Mélanie; Verlhac, Suzanne; Escabasse, Virginie; Remus, Natascha; Tamalet, Aline; Rioux, Jean-Yves; Franchi‐Abella, Stéphanie; Vasile, Manuela; Robert, Sarah; Delestrain, Céline; Hau, Isabelle; Pointe, Hubert Ducou-Le; Soubrier, Florent; Carette, Marie-France; Epaud, Ralph

doi:10.1002/ppul.23649

Cited by 10 publications

(5 citation statements)

References 38 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…While most PAVMs were diagnosed at the same time as their initial HHT screening, this study demonstrates that $30% of children who were initially negative for PAVMs subsequently developed them within five years. The PAVM prevalence of 60% is slightly higher than that reported in other pediatric studies to date (22-52%), 14,[26][27][28][29][30][31][32] which may be secondary to the longitudinal nature of our study and that fact that new PAVMs do, in fact, develop over time. Also, a referral bias may be at play in patients seen at an HHT Center.…”

Section: Discussioncontrasting

confidence: 80%

“…Compared to other pediatric studies of HHT, not only did we find a higher prevalence of PAVMs in our pediatric cohort of 129 children, but also a higher rate of embolization: 29% of all patients with HHT underwent embolization. Comparatively, in the French series by Soysal 32 where all pediatric patients with HHT were screened for PAVM with chest CT (59 patients), only 7% of all children with HHT underwent embolization. Furthermore, the Dutch study of Hosman et al., 31 using a more conservative screening approach designed to detect large PAVMs, reported only a 22% prevalence of PAVMs in 175 children with HHT compared to 60% in our study.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study

et al. 2018

View full text Add to dashboard Cite

Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing. Transthoracic contrast echocardiography (TTCE) was the primary screening modality in all patients and PAVMs were diagnosed based on Barzilai criteria. Moderately positive TTCE (Barzilai criteria ≥ 2) was confirmed with subsequent contrast chest CT. New PAVMs were diagnosed with a positive TTCE after an initial negative TTCE. Embolization of PAVMs were performed according to HHT consensus guidelines. Of 129 children with HHT, 76 (59%) were found to have PAVMs. Sixty-seven (88%) were positive for PAVMs on initial screening. Of 63 children without PAVMs on initial screening, 31 were followed for >1 year. Nine of the 31 (29%) developed new PAVMs after initial negative study. Thirty-eight (50%) of the total 76 children with PAVMs had or developed lesions large enough to be treated with embolization. Nine patients with PAVMs initially too small to be treated with embolization, developed progression of disease and ultimately were treated with embolization over time. The majority, 60% (23/38), of the children with large PAVMs had no related clinical symptoms. After embolization, 21% (8/38), of patients underwent repeat interventions. Genetic diagnosis, age, and gender were not associated with risk of having PAVM nor with need for repeat interventions. Nearly 60% of children with HHT develop PAVMs. The risk for new PAVMs to develop, small PAVMs to become large, and previously embolized PAVMs to require further intervention remains throughout childhood. Thus, children with HHT require continued follow-up until adulthood.

show abstract

Section: Discussioncontrasting

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study

et al. 2018

View full text Add to dashboard Cite

show abstract

“…CT scan is needed for children, especially for infants with recurrent pneumonia, refractory wheezing or hemoptysis existed to detect airway lesions. Even enhanced CT is performed to further clarify vascular malformations 15,16 . Since the introduction of IR algorithms, they have been widely applied by different companies 17–20 .…”

Section: Discussionmentioning

confidence: 99%

Feasibility study of using one-tenth mSv radiation dose in young children chest CT with 80 kVp and model-based iterative reconstruction

Sun

Zhang

et al. 2019

Sci Rep

View full text Add to dashboard Cite

CT has become a routine imaging modality based on its excellent ability of displaying lung structures and diseases. But, how to reduce radiation dose of routine CT examination is a concern for radiologists. Our study aimed to evaluate the feasibility of using 80kVp and a model-based iterative reconstruction (MBIR) algorithm to achieve one-tenth mSv dose chest CT in infants and young children. Thirty-two cases (study group, average age 1.71 ± 1.01 years) underwent non-contrast chest CT examination at low dose with 80 kV, 4mAs and was reconstructed with MBIR (LD-MBIR) and the standard adaptive statistical iterative reconstruction (ASIR) algorithm (LD-ASIR); another group (control group) of 32 children underwent routine-dose chest CT with 100 kV and was reconstructed with ASIR only (RD-ASIR). The subjective and objective image quality of the three groups were measured and statistically compared. The radiation dose for the low dose scan was 0.09 ± 0.02 mSv, 6% of the routine dose. All LD-MBIR images were diagnostically acceptable. Compared with the RD-ASIR images, the LD-MBIR images were similar in noise in the left ventricle, muscles, lung field, on-par in displaying large airways, lung lucency and mediastinum, but were inferior in displaying lung marking, small airways and mediastinum. Thus, MBIR images with low dose in pediatric chest CT can be used in the diagnosis for lung field and air way disorders in infants and young children.

show abstract

“…Following embolization or surgical procedure for management of these vascular lesions, CT, as a noninvasive imaging modality, can also aid in confirming the presence or absence of residual PAVMs and pulmonary varices. In addition, the recent study highlighted the usefulness of CT as an efficient approach to detect nonsymptomatic PAVMs in children carrying the diagnosis of hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome [117].…”

Section: Vascular Lesionsmentioning

confidence: 99%

Focal Lung Disorders

Lee

2019

Imaging in Pediatric Pulmonology

View full text Add to dashboard Cite

Pulmonary cysts seen on plain chest radiographs as completely or partially air-filled, round masses may be congenital, infectious/inflammatory, traumatic, or neoplastic in origin. The differential diagnosis can be refined based on the age of the patient (neonate and young infant vs. child) and distribution within the lungs (unilateral vs. bilateral), as well as the clinical presentation and the presence of other findings such as nodules or wedge-shaped opacities.In neonates or young infants, unilateral findings most often represent bronchopulmonary foregut malformations, while bilateral involvement suggests chronic lung disease of prematurity (Table 7.1). Young children most commonly present with symptoms of infection with or without an underlying congenital anomaly. A solitary lesion suggests a congenital anomaly or bacterial infection in a previously healthy child. If findings are bilateral, a systemic disease should be considered. Langerhans histiocytosis and cystic fibrosis (CF) are bilateral upper lung zone predominant diseases early on, while septic emboli involve the lower lobes preferentially. The presence of nodules and cysts suggests conditions such as Langerhans cell histiocytosis (LCH), granulomatosis with polyangiitis (previously known as Wegener granulomatosis), cystic fibrosis, and papillomatosis. The presence of wedge-shaped opacities suggests a vascular etiology as in granulomatosis with polyangiitis or septic emboli (Table 7.2).

show abstract

Non‐invasive CT screening for pulmonary arteriovenous malformations in children with confirmed hereditary hemorrhagic telangiectasia: Results from two pediatric centers

Cited by 10 publications

References 38 publications

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study

Feasibility study of using one-tenth mSv radiation dose in young children chest CT with 80 kVp and model-based iterative reconstruction

Focal Lung Disorders

Contact Info

Product

Resources

About