Congenital left brachiocephalic vein and superior vena cava aneurysms in an infant: final update with autopsy findings

Congenital left brachiocephalic vein and superior vena cava aneurysms in an infant: final update with autopsy findings To the Editor: We previously have described the case of an infant with congenital left brachiocephalic vein and superior vena cava (SVC) aneurysms in The Journal.1,2 Unfortunately, the patient died of chronic respiratory failure at 22 months of age because a tracheostomy could not be performed, and an autopsy was performed. Results of a gross inspection revealed numerous collateral veins of various sizes, which branched from the aneurysms and connected to the esophageal varices. The dilated blood vessel that we initially described as an SVC aneurysm was actually found to be a right brachiocephalic vein undergoing fusiform dilatation, and the SVC was not dilated. The left brachiocephalic vein aneurysm was saccular and composed of numerous interconnected cystic lesions containing thrombi (Figure 1). Examination of histologic sections revealed systemic proliferation of multilocular hemangiomatous tissue that penetrated into the submucosa and muscular layer of the esophagus and rectum (Figures 2). Immunohistochemical investigation results demonstrated that the endothelial cells of the hemangioma were CD34-negative (Figure 3). The overall clinicopathologic findings led to a diagnosis of angiomatosis of the venous type. Angiomatosis is a diffuse form of hemangioma that affects a large segment of the body in a contiguous fashion,

either by vertical extension to involve multiple tissue planes or by crossing muscle compartments to involve tissues of similar types.3 Vascular malformation, arteriovenous malformation, and venous malformation have been used as synonyms for angiomatosis.3 Our experience suggests that venous-type angiomatosis should be included in the differential diagnosis when huge mediastinal masses are present at birth. Akihisa Nitta, MD Kiyoshi Nishikura, MD Hironobu Fukuda, MD Shigemi Yoshihara, MD Junichi Hirao, MD Osamu Arisaka, MD Department of Pediatrics Hadzki Matsuda, MD Department of Anatomic and Diagnostic Pathology Dokkyo Medical University School of Medicine Mibu, Shimotsuga-gun, Tochigi, Japan 10.1016/j.jpeds.2007.10.037

REFERENCES 1. Nitta A, Suzumura H, Kano K, Arisaka O. Congenital left brachiocephalic vein and superior vena cava aneurysms in an infant [letter]. J Pediatr 2005;147:405. 2. Nitta A, Nishikura K, Hirao J, Suzumura H, Yoshihara S, Arisaka O. Congenital left brachiocephalic vein and superior vena cava aneurysms in an infant: an update. J Pediatr 2006;148:708-9. 3. Weiss SW. Angiomatosis. In: World Health Organization classification of tumors. Pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press; 2002. p. 161.

445

Figure 1. A partially shrunk left brachiocephalic aneurysm with numerous interconnected cystic lesions.

Figure 2. A, Elastica-Masson staining revealed hemangioma involving the submucosa and muscular layer of the esophagus. B, Hematoxylin-Eosin staining revealed hemangioma involving the submucosa and muscular layer of the rectum. C, Elastica-Masson staining revealed hemangioma of the left brachiocephalic aneurysm.

Figure 3. CD34 immunostaining of the left brachiocephalic aneurysm indicated that the endothelial cells were CD34-negative.

446

The Journal of Pediatrics • March 2008