Summary
We report six cases of segmental agenesis of the internal carotid a. discovered in adult patients: one case of cervical segmental agenesis (no. 1), one case of cervical and petrosal segmental agenesis (no. 2), two cases of vertical cavernous segmental agenesis (nos. 3 and 4) and two cases of distal segmental agenesis, one unilateral (no. 5) and the other bilateral (no. 6). The collateral pathways observed were: the ascending pharyngeal a. which constitutes an “intratympanic course” of the internal carotid a. (no. 1), an intercarotid anastomosis (no. 2), an arterial network at the base of the skull, the so-called “rete mirabile” (nos. 3 and 4) and the posterior communicating a. (nos. 5 and 6). Recognition of these rare dysgeneses relies upon the following radio-anatomic characteristics: reduced caliber of the a., inconsistent sparing of the carotid body, reduced diameter or even absence of the carotid canal and above all, the presence of collateral pathways. The collateral pathways allow an understanding of the segmental nature of carotid a. development and a distinction between congenital and acquired stenoses.
Résumé
Six observations d'agénésies segmentaires de l'artère carotide interne, découvertes chez l'adulte, sont rapportées : un cas d'agénésie du segment cervical (no 1), un cas d'agénésie des segments cervical et pétreux (no 2), deux cas d'agénésie du segment caverneux vertical (no 3 et 4) et deux cas d'agénésie distale de l'artère, unilatérale (no 5) et bilatérale (no 6). Les voies de suppléance observées sont : l'artère pharyngienne ascendante réalisant un “trajet intratympanique” de l'artère carotide interne (no 1), une anastomose intercarotidienne (no 2), un réseau artériel de la base du crâne, dit “réseau admirable” (no 3 et 4) et l'artère communicante postérieure (no 5 et 6). La reconnaissance de ces rares dysgénésies repose sur les caractéristiques radio-anatomiques suivantes : réduction de calibre de l'artère, respect inconstant de sa portion bulbaire, réduction des dimensions voire absence du canal carotidien et surtout voies anastomotiques utilisées. Les anastomoses observées permettent en effet de comprendre le caractère segmentaire du développement de l'artère et de distinguer les sténoses congénitales et les sténoses acquises.
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References
Afifi AK, Godersky JC, Menezes A, Smoker WR, Beli WE, Jacoby CG (1987) Cerebral hemiatrophy, hypoplasia of internal carotid artery and intracranial aneursym. Arch Neurol 44: 232–235
Alexander MWT, Chui MC, Gray T (1984) Transsellar intercavernous carotid anastomosis with agenesis of internal carotid artery and right-sided descending aorta: case report. J Canad Assoc Radiol 35: 92–95
André JM (1973) Les dysplasies vasculaires systématisées. L'expansion, Paris
Armand JP, Dousset V, Viaud B, Huot P, Chehab Z, Dos Santos E, Berge J, Caille JM (1996) Agénésie de l'ACI associé à un anévrisme de l'artère communicante antérieure. J Neuroradiol 23: 164–167
Arnould G, Tridon P, Laxenaire M, Picard L, Thiriet M, Pourel J (1967) Angiomatose carotidenne réalisant un réseau admirable (A propos de deux cas). Rev Neurol 116: 358–366
Austin JH, Stears JC (1971) Familial hypoplasia of both internal carotid arteries. Arch Neurol 24: 1–10
Boudin G, Chomette G, Guillard A, Hauw JJ, Der Agopian P (1972) Aplasie bilatérale de la carotide interne. A propos d'un cas anatomo-clinique. Rev Neurol 127: 329–340
Berisini DC, Hieshima GB, Mehringer CM, Grinnel VS (1981) Bilateral absence of the internal carotid artery with sellar enlargement due to anomalous vascularity. Surg Neurol 15: 9–12
Bracard S, Roland J, Picard L (1986) Variations des artères de l'encéphale. Guerbet
Brihaye J, Dhaene R (1962) Rétrécissement congénital important de l'artère carotide interne. Neurochirurgie 8: 349–350
Cali RL, Berg R, Rama K (1993) Bilateral internal carotid artery agenesis: a case study and review of the literature. Surgery 113: 227–233
Chen MC, Liu HM, Huang KM (1994) Agenesis of the internal carotid artery associated with neurofibromatosis type II. Am J Neuroradiol 15: 1184–1186
Desmond Hawkins T, Scott WC (1967) Bilateral rete carotidis in man. Clin Radiol 18: 163–165
Dilenge D (1975) Bilateral agenesis of internal carotid artery. J Canad Assoc Radiol 26: 91–94
Elefante R, Fucci G, Granata F, Graziussi G, Smaltino F (1983) Agenesis of the right internal carotid artery with an unusual trans-sellar intracavernous intercarotid connection. Am J Neuroradiol 4: 88–89
El Khamlichi A, Amrani F, El Azzusi M, El Oufir M, Khamlichi AM (1989) Hypoplasie bilatérale des carotides internes associée à un anévrysme de la communicante postérieure droite. A propos d'une observation. Neurochirurgie 35: 23–30
Ernest C, Hirsch JF, Metzger J, Chahinian P, Leblanc J, Godeau P (1973) Hypoplasie bilatérale de la carotide interne avec persistance d'un réseau admirable. A propos d'un cas chez l'adulte. Nouv Presse Med 2: 1655–1658
Faivre J, Vallée B, Carsin M, Scarabin JM, Simon J (1978) Agénésie des portions cervicale et pétreuse de la carotide interne gauche. Revascularisation de sa portion caverneuse par une anastomose intercarotidienne transsphénoidale. J Neuroradiol 5: 133–138
Goldman C, Singeton GT, Holly EH (1971) Aberrant internal carotid artery presenting as a mass in the middle ear. Arch Otolaryng 94: 77–88
Goldstein SJ, Lee C, Young AB, Guidry GJ (1984) Aplasia of the cervical carotid artery and malformation of the circle of Willis associated with Klippel-Trenaunay syndrome. J Neurosurg 61: 786–789
Grand CM, Louryan S, Bank WO, Baleriaux D, Brotchi J, Raybaud C (1993) Agenesis of the internal carotid artery and cavernous sinus hypoplasia with contralateral cavernous sinus meningioma. Neuroradiology 35: 588–590
Groslambert R, Fau R, Crouzet C, Château R, Sarrazin R (1969) Les dysgénésies de la carotide interne. Problèmes nosographiques posés à propos d'une observation personnelle. Rev Neurol 120: 345–349
Guérin J, Gouazé A, Lazorthes G (1976) Le polygone de Willis de l'enfant et les facteurs de son modelage. Neurochirurgie 22: 217–225
Guy G, Elouatassi K, Lancien G, Le Marec B, Pecker J. (1974) Hypoplasie bilatérale des carotides internes. A propos d'un cas anatomo-clinique. Sem Hôp Paris 50: 809–818
Handa J, Matsuda I, Nakasu S, Nakano Y (1980) Agenesis of an internal carotid artery: angiographic, tomographic and computed tomographic correlation. Neuroradiology 19: 207–211
Harispe L, Creissard P, Martinel G, Metzger J (1970) Une artère communicante anormale unissant les deux artères carotides internes intra-crâniennes. J Radiol Electrol 51: 759–762
Hills J, Sament S (1968) Bilateral agenesis of the internal carotid artery associated with cardiac and other anomalies. Case report. Neurology 18: 142–146
Huber G (1980) Intracranial carotid anastomosis and partial aplasia of an internal carotid artery. Neuroradiology 20: 207–212
Janicki PC, Limbacher JP, Guinto FC. (1979) Agenesis of the internal carotid artery with a primitive transsellar communicating artery. Am J Roentgenol 132: 130–132
Jones RR, Wetzel N (1970) Bilateral carotid vertebrobasilar rete mirabile. Case report. J Neurosurg 33: 581–586
Kane AG, Dillon WP, Barkovich J, Norman D, Dowd CF, Kane TT (1996) Reduced caliber of the internal carotid artery: A normal finding with ipsilateral absence or hypoplasia of the A1 segment. Am J Neuroradiol 17: 1295–1301
Kikuchi K, Kowada M, Kojima H (1995) Hypoplasia of the internal carotid artery associated with spasmodic torticolis: The possible role of altered vertebrobasilar haemodynamics. Neuroradiology 37: 362–364
Kishore PRS, Kaufman AB, Melichar FA (1979) Intrasellar carotid anastomosis simulating pituitary microadenoma. Radiology 132: 381–383
Kubis N, Zuber M, Meder JF, Mas JL (1996) CT scan of the skull base in internal carotid artery hypoplasia. Cerebrovasc Dis 6: 40–44
Kunishio K, Yamamoto Y, Sunami N, Asari S (1987) Agenesis of the left internal carotid artery, common carotid artery, and main trunk of the external carotid artery associated with multiple cerebral aneurysms. Surg Neurol 27: 177–181
Lapayowker MS, Liebman EP, Ronis ML, Safer JN (1971) Presentation of the internal carotid artery as a tumor of the middle ear. Radiology 98: 293–297
Lasjaunias P, Berenstein A (1987) Surgical Neuro-angiography. 1. Functional anatomy of craniofacial arteries. Springer-Verlag, Berlin Heidelberg
Lasjaunias P, Santoyo-Vazquez A (1984) Segmental agenesis of internal carotid artery: angiographic aspects with embryological discussion. Anat Clin 6: 133–141
Lazorthes G, Gouazé A, Salamon G (1976) Vascularisation et circulation de l'encéphale. Masson, Paris
Leonetti JP, Smith PG, Linthicum FH (1990) The petrous carotid artery: anatomic relationships in skull base surgery. Otolaryngol Head Neck Surg 102: 3–12
Lepoire J, Tridon P, Montaut J, Hepner H, Renard M, Picard L (1969) Malformations angiomateuses artério-artérielles du système carotidien. Neurochirurgie 15: 5–18
Lhermitte F, Gautier JC, Poirier J, Tyrer JH (1968) Hypoplasia of the internal carotid artery. Neurology 18: 439–446
Lie TA (1968) Congenital anomalies of the carotid arteries. Excerpta Medica Found, Amsterdam
Lo WWM, Solti-Bohman LG, Mc Elveen JT (1985) Aberrant carotid artery: radiological diagnosis with emphasis and high resolution computed tomography. Radiographics 5: 985–993
Manelfe C, Tremoulet M, Roulleau J (1972) Etude artériographique des branches intracaverneuses de la carotide interne. Neurochirurgie 7: 581–598
Michel D, Aimard G, Goutelle A, Bochu M, Gratadou G (1971) Une série de onze observations de thrombose bilatérale de la carotide interne. Discussion de l'aspect d'hypoplasie bilatérale des carotides internes à propos de 3 cas. J Med Lyon 52: 5–61
Midkiff RB, Boykin MW, Mc Farland DR, Bauman JA (1994) Agenesis of the internal carotid artery with intercavernous anastomosis. Am J Neuroradiol 16: 1356–1359
Milder DG, Lance JW (1984) Intermittent claudication of one hemisphere. Neurology 34: 692–694
Minagi H, Newton TH (1966) Carotid rete mirabile in man. A case report. Radiology 86: 100
Moret J (1982) La vascularisation de l'appareil auditif normal. Variantes. Tumeurs glomiques. J Neuroradiol 9: 209–260
Nakai H, Kawata Y, Aizawa S, Tanaka T, Yonemasu Y (1992) Unilateral agenesis of the internal carotid artery in a patient with ruptured aneurysm of the anterior communicating artery; a case report. Neurol Surg 20: 893–898
Nakamura H, Yamada H, Nagao T, Fujita K, Tamaki N (1993) A case report of hypoplasia of the left internal carotid artery manifested as convulsion attack. Neurol Surg 21: 843–848
Occleshaw JV, Garland P (1969) Bilateral rete carotidis. Br J Radiol 42: 851–853
Padget DH (1948) The development of the cranial arteries in the human embryo. Contrib Embryol Carneg Inst 32: 207–261
Pascaud JL, Vigneu P, Hummel P, Bouchet JB, Rihollet J, Pascaud-Ged E (1982) Absence partielle de carotide interne avec anastomose intercarotidienne trans-sellaire. Nouvelle observation et revue de la littérature. J Radiol 63: 37–40
Patay Z, Berky M (1988) Clinico-radiological aspects of the hypoplastic internal carotid artery syndrome. In: Proc XVth Congress European Soc Neuroradiology, Würzburg, pp. 593–596
Picard L, André JM, Renard M, Montaut J, Tridon P (1972) Réseau admirable carotidien humain. Problèmes étiologiques et nosologiques. Acta Radiol 13: 205–219
Quint DJ, Boulos RS, Spera TD (1989) Congenital absence of cervical and petrous internal carotid artery with cavernous anastomosis. Am J Neuroradiol 10: 435–439
Quint DJ, Silbergleit R, Young WC (1992) Absence of the carotid canal at skull base CT. Radiology 182: 477–481
Schlenska GK (1986) Absence of both internal carotid arteries. J Neurol 233: 263–266
Servo A (1977) Agenesis of the left internal carotid artery associated with an aneurysm of the right carotid siphon: case report. J Neurosurg 46: 677–680
Smith RR, Jay Kees C., Hogg ID. (1972) Agenesis of the internal carotid artery with an unusual primitive collateral. J Neurosurg 37: 460–462
Smith KR, Nelson J, Dooley JM (1968) Bilateral “hypoplasia” of the internal carotid arteries. Neurology 18: 1149–1156
Staples GS (1979) Transsellar intracavernous intercarotid collateral artery associated with agenesis of the internal carotid artery: A case report. J Neurosurg 50: 393–394
Tangchai P, Khaoborisut V (1970) Agenesis of internal carotid artery associated with aneurysm of contralateral middle cerebral artery. Neurology 20: 809–812
Teal JS, Maheedy MH, Hasso AN (1980) Total agenesis of the internal carotid artery. Am J Neuroradiol 1: 435–442
Teal JS, Rumbaugh CL, Bergeron T, Segall HD (1973) Congenital absence of the internal carotid artery associated with cerebral hemiatrophy, absence of the external carotid artery and persistence of stapedial artery. Am J Roentgenol 118: 534–545
Tharp B, Heyman A, Pfeiffer JB, Young WG (1965) Cerebral ischemia. Result of hypoplasia of internal carotid artery. Arch Neurol 12: 160–164
Udzura M, Kobayashi H, Taguchi Y, Sekino H (1988) Intrasellar intercarotid communicating artery associated with agenesis of the right internal carotid artery: Case report. Neurosurgery 33: 770–773
Veillon F (1991) Imagerie de l'oreille. Flammarion, Paris, pp 457–478
Waga S, Okada M, Kojima T (1978) Saccular aneurysm associated with absence of the left cervical carotid arteries. Neurosurgery 3: 208–212
Willinsky RA, Lasjaunias P, Berenstein A (1987) Intracavernous branches of the internal carotid artery (ICA). Comprehensive review of their variations. Surg Radiol Anat 9: 201–215
Willinsky RA, Ter Brugge K, Lawson V, Mirich D, Lasjaunias P (1990) Aberrant carotid artery: recognition on high resolution computed tomography. J Oto-Laryngology 19: 249–251
Worthington C, Olivier A, Melanson D (1984) Internal carotid artery agenesis: correlation by conventional and digital substraction angiography, and by computed tomography. Surg Neurol 22: 295–300
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Meder, J.F., Blustajn, J., Trystram, D. et al. Radiologic anatomy of segmental agenesis of the internal carotid artery. Surg Radiol Anat 19, 385–394 (1997). https://doi.org/10.1007/BF01628506
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DOI: https://doi.org/10.1007/BF01628506