Abstract
Vascular ring is a term given to a combination of vascular and often ligamentous structures that encircle the trachea and esophagus. The diagnosis can be difficult because clinical symptoms can be variable and nonspecific, and because vascular arrangements that result in vascular rings in some patients do not form vascular rings in others. The clinical manifestations comprise a spectrum ranging from no symptoms to feeding difficulties, repeated infections, and life-threatening respiratory compromise. The diagnosis of vascular ring can be made by various imaging modalities. Therefore it is imperative that pediatric radiologists be familiar with the anatomic variants that can result in a symptomatic ring needing surgical repair, their imaging appearance and the appropriate imaging algorithm. The goals of this manuscript are to describe common and uncommon types of vascular rings, to simplify the differential diagnosis, and to outline the imaging options for accurate diagnosis.
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Park MK (2002) Vascular rings. In: Park MK (ed) Pediatric cardiology for practitioners, 4th edn. St. Louis, Mosby, p 241
Gross R (1945) Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 233:586–590
Neuhauser E (1946) The roentgen diagnosis of double aortic arch and other anomalies of the great vessels. Am J Roentgenol Radium Ther 56:1–12
Tonkin I, Elliott L, Bargeron L Jr (1980) Concomitant axial cineangiography and barium esophagography in the evaluation of vascular rings. Radiology 135:69–76
Berdon WE, Baker DH (1972) Vascular anomalies and the infant lung: rings, slings, and other things. Semin Roentgenol 7:39–64
Achiron R, Rotstein Z, Heggesh J, et al (2002) Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. Ultrasound Obstet Gynecol 20:553–557
Heck HA Jr, Moore HV, Lutin WA, et al (1993) Esophageal-aortic erosion associated with double aortic arch and tracheomalacia. Tex Heart Inst J 20:126–129
James RC, Murty GE (2000) Previously undiagnosed congenital vascular ring presenting as dysphagia in a six-week post-partum female. J Laryngol Otol 114:881–882
DeLeval M (1994) Vascular rings. In: DeLeval M, Stark J (eds) Surgery for congenital heart defects, 2nd edn. Saunders, Philadelphia, pp 307–316
van Son JA, Julsrud PR, Hagler DJ, et al (1993) Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc 68:1056–1063
Strife JL, Matsumoto J, Bisset HD 3rd, et al (1989) The position of the trachea in infants and children with right aortic arch. Pediatr Radiol 19:226–229
Pickhardt P, Siegel M, Gutierrez F (1997) Vascular rings in symptomatic children: frequency of chest radiographic findings. Radiology 203:423–426
Jaffe R (1991) Radiographic manifestations of congenital anomalies of the aortic arch. Radiol Clin North Am 29:319–334
McFaul R, Millard P, Nowicki E (1981) Vascular rings necessitating right thoracotomy. J Thorac Cardiovasc Surg 82:306–309
Singh GK, Greenberg SB, Balsara RK, et al (1997) Diagnostic dilemma: left aortic arch with right descending aorta—a rare vascular ring. Echocardiography in the preoperative evaluation of vascular rings. Pediatr Cardiol 18:45–48
McLeary MS, Frye LL, Young LW (1998) Magnetic resonance imaging of a left circumflex aortic arch and aberrant right subclavian artery: the other vascular ring. Pediatr Radiol 28:263–265
Frush DP, Herlong JR (2005) Pediatric thoracic CT angiography. Pediatr Radiol 35:11–25
Greil GF, Kramer U, Dammann F et al (2005) Diagnosis of vascular rings and slings using an interleaved 3D double-slab FISP MR angiography technique. Pediatr Radiol 35:396–401
Klinkhamer A (1969) Esophagography in anomalies of the aortic arch system. Excerpta Medica Foundation, Amsterdam
Edwards J (1948) Vascular rings related to anomalies of the aortic arches. Mod Concepts Cardiovasc Dis 17:19–20
Edwards J (1948) Anomalies of the derivatives of the aortic arch system. Med Clin North Am 32:925–949
Gross R, Neuhauser E (1951) Compression of the trachea or esophagus by vascular anomalies. Pediatrics 7:69–83
Knight L, Edwards JE (1974) Right aortic arch. Types and associated cardiac anomalies. Circulation 50:1047–1051
Stewart JR, Kincaid OW, Titus JL (1966) Right aortic arch: plain film diagnosis and significance. Am J Roentgenol Radium Ther Nucl Med 97:377–389
Moes CA, Mawson JB, MacDonald C, et al (1996) Right aortic arch with retroesophageal left aberrant innominate artery. Pediatr Cardiol 17:402–406
Ledwith MV, Duff DF (1994) A review of vascular rings 1980–1992. Ir Med J 87:178–179
Han JJ, Sohn S, Kim HS, et al (2001) A vascular ring: right aortic arch and descending aorta with left ductus arteriosus. Ann Thorac Surg 71:729–731
Zachary CH, Myers JL, Eggli KD (2001) Vascular ring due to right aortic arch with mirror-image branching and left ligamentum arteriosus: complete preoperative diagnosis by magnetic resonance imaging. Pediatr Cardiol 22:71–73
D’Souza VJ, Velasquez G, Glass TA, et al (1985) Mirror-image right aortic arch: a proposed mechanism in symptomatic vascular ring. Cardiovasc Intervent Radiol 8:134–136
Edwards J (1948) Retroesophageal segment of the left aortic arch, right ligamentum arteriosum, and right descending aorta causing a congenital vascular ring about the trachea and esophagus. Proc Mayo Clin 23:108–116
Berman W Jr, Yabek SM, Dillon T, et al (1981) Vascular ring due to left aortic arch and right descending aorta. Circulation 63:458–460
Dominguez R, Oh KS, Dorst JP, et al (1978) Left aortic arch with right descending aorta. AJR 130:917–920
Ergin MA, Jayaram N, LaCorte M (1981) Left aortic arch and right descending aorta: diagnostic and therapeutic implications of a rare type of vascular ring. Ann Thorac Surg 31:82–85
Minami K, Sagoo KS, Matthies W, et al (1986) Left aortic arch, retro-esophageal aortic segment, right descending aorta and right patent ductus arteriosus—a very rare “vascular ring” malformation. Thorac Cardiovasc Surg 34:395–397
Murthy K, Mattioli L, Diehl AM, et al (1970) Vascular ring due to left aortic arch, right descending aorta, and right patent ductus arteriosus. J Pediatr Surg 5:550–554
Park SC, Siewers RD, Neches WH, et al (1976) Left aortic arch with right descending aorta and right ligamentum arteriosum. A rare form of vascular ring. J Thorac Cardiovasc Surg 71:779–784
Watanabe M, Kawasaki S, Sato H, et al (1995) Left aortic arch with right descending aorta and right ligamentum arteriosum associated with d-TGA and large VSD: surgical treatment of a rare form of vascular ring. J Pediatr Surg 30:1363–1365
Whitman G, Stephenson LW, Weinberg P (1982) Vascular ring: left cervical aortic arch, right descending aorta, and right ligamentum arteriosum. J Thorac Cardiovasc Surg 83:311–315
Stewart J, Kincaid OW, Edwards JE (1964) An atlas of vascular rings and related malformations of the aortic arch system. Charles C. Thomas, Springfield, Illinois
Kommerell B (1936) Verlagerung des Osophagus durch eine abnorm verlaufende arteria subclavia dextra (Arteria Lusoria). Fortschr Geb Rontgenstr 54:590–595
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The author thanks Dominic Doyle for the schematic illustrative artwork.
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Hernanz-Schulman, M. Vascular rings: a practical approach to imaging diagnosis. Pediatr Radiol 35, 961–979 (2005). https://doi.org/10.1007/s00247-005-1529-0
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DOI: https://doi.org/10.1007/s00247-005-1529-0