Dataset Information

Case report on surgical repair of unusual dissection of arterial duct involving main pulmonary artery in a child with vein of Galen malformation.

ABSTRACT:

Background

Dissection involving patent arterial duct (PDA) extending to main pulmonary artery (MPA) is an extremely rare condition.

Case summary

We report a case of complex dissection involving PDA extending to MPA, in a child with vein of Galen malformation. This was surgically corrected under deep hypothermic circulatory arrest, and we describe the pathophysiology and surgical rationale in this report.

Discussion

Management of intracardiac lesion in the presence of intracranial arterio-venous malformations such as vein of Galen is very difficult, and employing deep hypothermic circulatory arrest allows one to manage cerebral protection while offering the visualization of intracardiac anatomy more effectively.

SUBMITTER: Muthialu N

PROVIDER: S-EPMC6426031 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature

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Publications

Case report on surgical repair of unusual dissection of arterial duct involving main pulmonary artery in a child with vein of Galen malformation.

Muthialu Nagarajan N Robertson Alex A Mortensen Kristian K Khambadkone Sachin S

European heart journal. Case reports 20181124 4

<h4>Background</h4>Dissection involving patent arterial duct (PDA) extending to main pulmonary artery (MPA) is an extremely rare condition.<h4>Case summary</h4>We report a case of complex dissection involving PDA extending to MPA, in a child with vein of Galen malformation. This was surgically corrected under deep hypothermic circulatory arrest, and we describe the pathophysiology and surgical rationale in this report.<h4>Discussion</h4>Management of intracardiac lesion in the presence of intrac ...[more]

PMID: 31020216

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Project description:ImportanceMajor neurological complications from the embolization of vein of Galen malformations (VOGMs) are poorly understood. We provide a detailed analysis of contributors to periprocedural neurological complications and lessons learned.ObjectiveTo assess the rate of major periprocedural neurological complications following VOGM embolization with major procedural and strategic contributors.Design, setting, and participantsThis retrospective cohort study was conducted at a quarternary referral pediatric hospital (Hospital for Sick Children; Toronto, Ontario, Canada) from January 1999 to December 2018 with a mean clinical follow-up of 44.7 months; all children with VOGM diagnosed and/or treated were eligible (n = 48). Thirty-three patients who underwent endovascular treatment were included.InterventionsEndovascular staged transarterial embolization performed in 33 patients over 91 sessions.Main outcomes and measuresThe primary outcome was the rate of periprocedural neurological complications (occurring within 1 week of embolization). The secondary outcomes were mortality, long-term neurological outcomes, and contributing anatomical and management factors to neurological complications.ResultsOf 33 patients who underwent embolization (31 boys [64.6%]; 17 girls [35.4%]; median age at first embolization, 4 months [range, 0-29 months]), 10 patients (30.3%) developed major periprocedural neurological complications. Five of these patients died. Univariate logistic regression analyses identified internal cerebral vein drainage to the main venous sac of the VOGM and use of a microcatheter with a distal outer diameter of more than 2.0F as significant predictors of poor neurological outcomes. Lessons learned from our experience include the need to assess the internal cerebral vein drainage pattern on preprocedural magnetic resonance venography, avoidance of excessive embolization into the venous sac, treatment of more distal fistulae before proximal fistulae to avoid a sump effect, and preferably use of smaller (<2.0F outer diameter) microcatheters in neonatal embolization procedures.Conclusions and relevanceIn this cohort, 10 patients with VOGM treated with embolization (30.3%) experienced major periprocedural neurological complications, half of whom died. While these outcomes are superior to historic conservative and surgical treatment results, ongoing improvements in treatment and pretreatment diagnostic approaches are needed. Awareness of the lessons learned from our experience can help to avoid similar complications in the future for this vulnerable population.

Project description:IntroductionUnderstanding outcomes after Vein of Galen malformation (VOGM) embolization has been limited by small sample size in reported series and predominantly single center studies. To address these limitations, we perform an individual-participant meta-analysis (IPMA) to identify risk factors associated with all-cause mortality and clinical outcome after VOGM endovascular embolization.MethodsWe performed a systematic review and IPMA of VOGM endovascular outcomes according to PRISMA guidelines. Individual patient characteristics including demographic, intra/post-operative adverse events, treatment efficacy (partial or complete occlusion), and clinical outcome were collected. Mixed-effects logistic regression with random effects modeling and Bonferroni correction was used (p ≤ 0.003 threshold for statistical significance). The primary and secondary outcomes were all-cause mortality and poor clinical outcome (moderate/severe developmental delay or permanent disabling injury), respectively. Data are expressed as (mean ± standard deviation (SD)) or (odds ratio (OR), 95% confidence interval (CI), I 2, p-value).ResultsThirty-five studies totaling 307 participants quantifying outcomes after endovascular embolization for VOGM were included. Follow up time was 42 (±57) months. Our analysis contained 42% neonates (<1 month) at first embolization, 45% infants (1 month ≤2 years), and 13% children (>2 years). Complete occlusion was reported in 48% of participants. Overall all-cause mortality was 16%. Overall, good clinical outcome was achieved in 68% of participants. First embolization as a neonate [OR = 6.93; 95% CI (1.99-24.08); I 2 < 0.01; p < 0.001] and incomplete embolization [OR = 10.87; 95% CI (1.86-63.55); I 2 < 0.01; p < 0.001] were associated with mortality. First embolization as a neonate [OR = 3.24; 95% CI (1.47-7.15); I 2 < 0.01; p < 0.001], incomplete embolization [OR = 5.26; 95% CI (2.06-13.43); I 2 < 0.01; p < 0.001], and heart failure at presentation [OR = 3.10; 95% CI (1.03-9.33); I 2 < 0.01; p = 0.002] were associated with poor clinical outcomes. Sex, angioarchitecture of lesion, embolization approach (transvenous vs. transarterial), and single or multistage embolization were not associated with mortality or clinical outcome.ConclusionsWe identify incomplete VOGM embolization independently associated with mortality and poor clinical outcome. While this study provides the highest level of evidence for VOGM embolization to date, prospective multicenter studies are needed to understand the optimal treatment strategies, outcomes, and natural history after VOGM embolization.

Dataset Information

Case report on surgical repair of unusual dissection of arterial duct involving main pulmonary artery in a child with vein of Galen malformation.

Background

Case summary

Discussion

Publications

Case report on surgical repair of unusual dissection of arterial duct involving main pulmonary artery in a child with vein of Galen malformation.

Similar Datasets

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