Biliary Atresia: Color Doppler US Findings in Neonates and Infants

Lee, Mu Sook; Kim, Myung Joon; Lee, Mi Jung; Yoon, Choon Sik; Han, Seok Joo; Oh, Jung Tak; Park, Young Nyun

doi:10.1148/radiol.2522080923

Cited by 98 publications

(103 citation statements)

References 21 publications

(25 reference statements)

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“…Mean PELD score of 44 jaundiced infants was 0.39 (0.08). Mean baseline heart rate and average blood pressure were 132 (12) beats per minute and 58 (14) mmHg. They were decreased to 122 (20), 126 (16) beats per minute and 51 (11), 48 (13) mmHg with no statistical significance after 2% and 3% sevoflurane anaesthesia.…”

Section: Resultsmentioning

confidence: 97%

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Effect of sevoflurane anaesthesia on hepatic blood flow in infants with obstructive hepatobiliary disease

Zhou

Wang

Song

et al. 2016

Acta Anaesthesiol. Scand.

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Section: Resultsmentioning

confidence: 97%

“…[13][14][15] Thus, we used transabdominal colour Doppler ultrasound to measure the effect of sevoflurane anaesthesia on HBF in infants with hepatobiliary disease.…”

Section: Editorial Comment: What This Article Tell Usmentioning

confidence: 99%

Effect of sevoflurane anaesthesia on hepatic blood flow in infants with obstructive hepatobiliary disease

Zhou

Wang

Song

et al. 2016

Acta Anaesthesiol. Scand.

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“…Ultrasonographic imaging may reveal an absent or atretic gallbladder, as well as fibrotic regions in the porta hepatis (‘triangular cord') and, intriguingly, increased subcapsular flow [9,14,15,16,17]. The finding of increased subcapsular flow may perhaps provide a readily utilized screening technique to help differentiate biliary atresia from other forms of neonatal cholestasis [14].…”

Section: Biliary Atresia Epidemiology and Salient Clinical Conceptsmentioning

confidence: 99%

Genetic Contributors and Modifiers of Biliary Atresia

Mezina

Karpen²

2015

Dig Dis

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To date, the etiology and pathogenic underpinning of the progression of the most prevalent serious neonatal liver disease, biliary atresia, remains elusive. This disease presents as an aggressive form of neonatal cholestasis characterized by the destruction and obliteration of the extrahepatic bile ducts within the first few weeks of life and a rapid progression of biliary fibrosis, likely due to unremitting cholestasis and retention of biliary constituents including bile acids. In ∼5% of patients, biliary atresia is associated with laterality features, suggesting a genetic underpinning to a disease that begins soon after birth. However, biliary atresia does not occur within families and twins are discordant, indicating an absence of strict mendelian inheritance. Despite this, genes related to bile duct dysmorphogenesis/ciliopathies overlapping with features of biliary atresia in both humans and nonhuman model systems have been proposed. Taken together, strict genetic etiologies leading to a common pathway of a neonatal cholangiopathy resulting in biliary atresia remain elusive. Contributions from fibrogenesis- and inflammation-based studies suggest that early engagement of these pathways contributes to disease progression, but a recent double-blind study did not suggest any benefit from early use of corticosteroids. However, there are genetic contributions to the adaptation and response to cholangiopathies and cholestasis that may be present in certain populations that likely impact upon the response to hepatoportoenterostomy and subsequent biliary tract function. Studies utilizing next generation sequencing technologies (e.g., exome analysis) are ongoing in several laboratories around the world; they are expected to provide insights into genetic contributions to biliary atresia outcomes. Altogether, combinations of exome sequencing and large population studies are expected to reveal causative and modifying genes relevant to patients with biliary atresia as a means to provide therapeutic targets and potential opportunities for genetic screening.

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“…A hint towards biliary atresia is the lack of or a very small gall bladder. Sometimes one can find the 'triangular cord sign' in the porta hepatis; this echogenic triangular-shaped spot or band close to the central portal vein represents a fibrotic remnant from the bile duct and is almost diagnostic for biliary atresia [70]. Rarely one can see signs of cirrhosis already in the neonate, this is more often linked to other (systemic metabolic) disease such as thyrosinosis.…”

Section: Potential Of Modern Us In the Pediatric Livermentioning

confidence: 99%

Role and potential of modern ultrasound in pediatric abdominal imaging

Pilhatsch¹,

Riccabona²

2011

Imaging in Medicine

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REVIEWChildren and especially infants are not small adults -they suffer from specific diseases, present different imaging demands and have increased radiation sensitivity. One important aspect of ongoing continuous improvement of medical imaging is the increasing potential of ultrasound (US); this may help to minimize radiation by replacing or obviating other imaging. This important task, particularly in childhood, can only be achieved by providing dedicated skilled pediatric US to children 24 h, 7 days a week.The purpose of this review is to give some key advice on pediatric US, to list and illustrate typical pediatric conditions, to debate the relevant respective differential diagnoses and how US can reliably provide diagnostically relevant information with implications on therapy. For those who are familiar with pediatric radiology the following should be a useful review with some highlighted details. For everyone else this review should demonstrate what is achievable by modern US in children, completely different from the US potential in (obese) adult patients. We will aim to point out the significance of US in common and important pediatric abdominal queries and emphasize the difference between adults and children not only in terms of pathology, but also in the application and potential of the modality itself.Ultrasound (US) has gained its place as the preferred first-line imaging modality in the child's abdomen. Especially in infants US has advantages -the small amount of tissue and fat creating restrictions to CT and MRI allows for application of high-resolution (especially linear) transducers, offering excellent imaging capabilities; in addition, US is applicable at the bedside and can be performed without sedation. As radiation protection has become a key issue for imaging children, exploiting US to the utmost using all its possibilities and applying modern methods that have significantly widened US potential is an indispensable and increasingly important approach. This review illustrates standard US techniques, basic and new applications throughout the entire abdomen and its different organ systems, trying to point out the enormous potential of US in different clinical scenarios, focusing on gastrointestinal and urogenital issues. Future perspectives provided by new modalities such as contrastenhanced and 3D US will further strengthen the diagnostic value of this powerful imaging tool, hopefully contributing to continuing reduction of diagnostic radiation burden to children, by diminishing the need for CT. KEYWORDS: abdomen n children n gastrointestinal n infants n pediatric ultrasound n urinary tract

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Biliary Atresia: Color Doppler US Findings in Neonates and Infants

Abstract: The presence of hepatic subcapsular flow is useful for differentiating between BA and other causes of neonatal jaundice.

Cited by 98 publications

References 21 publications

Effect of sevoflurane anaesthesia on hepatic blood flow in infants with obstructive hepatobiliary disease

Effect of sevoflurane anaesthesia on hepatic blood flow in infants with obstructive hepatobiliary disease

Genetic Contributors and Modifiers of Biliary Atresia

Role and potential of modern ultrasound in pediatric abdominal imaging

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