Objective: To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA).
Methods: All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively.
Results: Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%.
Conclusion: ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.
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http://dx.doi.org/10.1016/j.jpeds.2015.01.038 | DOI Listing |
Int J Hematol
January 2025
Associated Department With Mie Graduate School of Medicine, Mie Prefectural General Medical Center, Yokkaichi, Japan.
This study discusses disseminated intravascular coagulation (DIC) associated with solid cancers and various vascular abnormalities, both of which generally exhibit chronic DIC patterns. Solid cancers are among the most significant underlying diseases that induce DIC. However, the severity, bleeding tendency, and progression of DIC vary considerably depending on the type and stage of the cancer, making generalization difficult.
View Article and Find Full Text PDFCureus
December 2024
Neurosurgery, Southmead Hospital, North Bristol NHS, Bristol, GBR.
Cerebral arteriovenous malformations (AVMs) are tangles of abnormal vessels with early arteriovenous (AV) shunting that can lead to intracerebral hemorrhage, seizures, neurologic deficit, or headache. To date, only a few cases of carcinomas metastasizing to pre-existing cerebral AVMs have been reported in the literature. However, renal clear cell carcinoma (RCC) brain metastases that exhibit early AV shunting, where AVM pathology is not present, are extremely rare.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Pediatric Cardiovascular Surgery, Kanazawa Medical University, Ishikawaken, Japan.
Background: The study focuses on vascular compression of the main bronchus in the aortopulmonary space, examining potential contributors within the same axial plane. Its goal is to uncover mechanisms of bronchial compression in patients with intracardiac anomalies and review surgical outcomes, aiming to enhance future results.
Methods: The morphology and topology of structures within the axial plane of the aortopulmonary space were objectively analyzed, including the sternum, ascending aorta, heart, pulmonary artery, descending aorta, and other relevant elements.
Ann Thorac Surg Short Rep
September 2024
Division of Cardio-thoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine at Christus Children's Hospital of San Antonio, Texas.
Aneurysms of the superior vena cava are rare vascular malformations of systemic veins. This report presents the case of a 27-year-old woman with an incidental finding of mediastinal shadow widening on the chest roentgenogram that was confirmed by computed tomographic angiography to be a superior vena cava saccular aneurysm >4 cm in diameter. Surgical resection was recommended on the basis of aneurysmal size and shape and was performed through median sternotomy by using cardiopulmonary bypass.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Introduction: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease and screening to detect pulmonary arteriovenous malformations (PAVMs) is important to prevent complications. In adults, transthoracic contrast echocardiogram (TTCE) is used to screen PAVMs. In children, a conservative screening method seems to be sufficient to rule out major PAVMs and prevent them from PAVM-related complications.
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