Chiari Malformation Type I with Concomitant Persistent Trigeminal Artery

Abstract

Background: Chiari malformation type I is a stenosis of the subarachnoid space at the level of the foramen magnum due to a small posterior fossa and herniation of the cerebellar tonsils. We present here the unusual case of a Chiari malformation in conjunction with a persistent trigeminal artery and hypoplasia of the basilar artery, also known as a Saltzman anomaly. Case Report: A 34-year-old female physician presented with recurrent headaches, bilateral weakness of deltoid muscles and numbness of the fingertips 3 to 5. A cranial MRI revealed a descent of the cerebellar tonsils to the level of C2 and a supracerebellar cyst. During surgery, a decompressive suboccipital craniectomy was performed. The supracerebellar cyst was fenestrated and the cerebellar tonsils were resected bilaterally. Two days after surgery the patient developed cranial nerve dysfunction and a right-sided hemiparesis. Cerebral angiography revealed a Saltzman type 1 anomaly with persistent primitive trigeminal artery and hypoplasia of the basilar artery. Discussion: This is the first report in the literature about the coincidence of both unusual anomalies. The latest literature of both rare anatomies and the unusual clinical course will be discussed.

Zusammenfassung

Hintergrund: Die Chiari-Malformation Typ I ist eine Stenose des Subarachnoidalraumes auf der Höhe des Foramen magnums, die durch eine kleine hintere Schädelgrube entsteht und zu einer Herniation der Kleinhirntonsillen führt. Wir beschreiben hier einen ungewöhnlichen Fall einer Chiari-Malformation in Verbindung mit einer persistierenden primitiven Trigeminalarterie und einer Hypoplasie der Arteria basilaris. Die begleitende vaskuläre Fehlbildung wird auch Saltzman-Anomalie genannt. Fallbeschreibung: Eine 34-jährige weibliche Ärztin stellte sich mit Kopfschmerzattacken und einer beidseitigen Schwäche des Musculus deltoideus sowie einem Taubheitsgefühl der Fingerspitzen der Finger 3 bis 5 vor. Ein kraniales Kernspintomogramm zeigte eine Verlagerung der Kleinhirntonsillen bis auf die Höhe C2 sowie eine suprazerebelläre Zyste. Während der Operation wurde eine dekompressive subokzipitale Kraniotomie durchgeführt. Die suprazerebelläre Zyste wurde gefenstert und die Kleinhirntonsillen beidseits reseziert. Zwei Tage nach der Operation entwickelte die Patientin Hirnnervenstörungen und eine rechtsseitige Hemiparese. Eine zerebrale Angiographie zeigte eine Saltzman-1-Anomalie mit persistierender primitiver Trigeminalarterie und Hypoplasie der Arteria basilaris. Diskussion: Dies ist der erste Bericht in der Literatur über die Koinzidenz beider seltenen Anomalien. Die neueste Literatur sowie der ereignisreiche klinische Verlauf werden diskutiert.

Chiari malformation - persistent primitive trigeminal artery

Chiari-Malformation - persistierende primitive Trigeminalarterie

References

• 1 Abe T, Fujita S, Ozawa H, Kawamura N, Shimazu M, Ikeda H, Izumiyama H, Matsumoto K. Haemorrhagic nonsecreting pituitary adenoma associated with persistent primitive trigeminal artery.  Acta Neurochir. 2000;  142 1423-1424
• 2 Agnoli A L. Vascular anomalies and subarachnoid haemorrhage associated with persisting embryonic vessels.  Acta Neurochir. 1982;  60 183-199
• 3 Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation.  Neurosurgery. 1995;  37 214-218
• 4 Barkovich A J, Wippold F J, Sherman J L, Citrin C M. Significance of cerebellar tonsillar position on MR.  AJNR Am J Neuroradiol. 1986;  7 795-799
• 5 Batzdorf U. Chiari I malformation with syringomyelia. Evaluation of surgical therapy by magnetic resonance imaging.  J Neurosurg. 1988;  68 726-730
• 6 Black S, Cucchiara R F. Tumor surgery. In: Cucchiara RF, Michenfelder JD. Clinical Neuroanesthesia. Churchill Livingstone, Edinburgh 1990; 285-308
• 7 Black S, Ockert D B, Oliver W C, Cucchiara R F. Outcome following posterior fossa craniectomy in patients in the sitting or horizontal positions.  Anesthesiology. 1988;  69 49-56
• 8 Boyko O B, Curnes J T, Blatter D D, Parker D L. MRI of basilar artery hypoplasia associated with persistent primitive trigeminal artery.  Neuroradiology. 1996;  38 11-14
• 9 Cloft H J, Razack N, Kallmes D F. Prevalence of cerebral aneurysms in patients with persistent primitive trigeminal artery.  J Neurosurg. 1999;  90 865-867
• 10 da Silva J A. Basilar impression and Arnold-Chiari malformation. Surgical findings in 209 cases.  Neurochirurgia (Stuttg). 1992;  35 189-195
• 11 Ekinci G, Baltacioglu F, Kilic T, Cimsit C, Akpinar I, Pamir N, Erzen C. A rare cause of hyperprolactinemia: persistent trigeminal artery with stalk-section effect.  Eur Radiol. 2001;  11 648-650
• 12 Elton R J, Howell R S. The sitting position in neurosurgical anaesthesia: a survey of British practice in 1991.  Br J Anaesth. 1994;  73 247-248
• 13 Fields W S. The significance of persistent trigeminal artery. Carotid-basilar anastomosis.  Radiology. 1968;  91 1095-1101
• 14 Floris R, Pastore F S, Silvestrini M, Vagnozzi R, Guazzaroni M, Giuffre R, Simonetti G. Supracerebellar arachnoid cyst and reversible tonsillar herniation: magnetic resonance imaging and pathophysiological considerations.  Neuroradiology. 1992;  34 404-406
• 15 Fortner A A, Smoker W R. Persistent primitive trigeminal artery aneurysm evaluated by MR imaging and angiography.  J Comput Assist Tomogr. 1988;  12 847-850
• 16 Gardner W J. Hydrodynamic factors in Dandy-Walker and Arnold-Chiari malformations.  Childs Brain. 1977;  3 200-212
• 17 Gardner W J, Angel J. The cause of syringomyelia and its surgical treatment.  Cleve Clin Q. 1958;  25 4-8
• 18 Gardner W J, Goodall R J. The surgical treatment of Arnold-Chiari malformations in adults. An explanation of its mechanism and importance of encephalography in diagnosis.  J Neurosurg. 1950;  7 199-206
• 19 Goel A, Bhatjiwale M, Desai K. Basilar invagination: a study based on 190 surgically treated patients.  J Neurosurg. 1998;  88 962-968
• 20 Halamandaris G G, Batzdorf U. Adult Chiari malformation.  Contemp Neurosurg. 1989;  11 1-6
• 21 Heiss J D, Patronas N, DeVroom H L, Shawker T, Ennis R, Kammerer W, Eidsath A, Talbot T, Morris J, Eskioglu E, Oldfield E H. Elucidating the pathophysiology of syringomyelia.  J Neurosurg. 1999;  91 553-562
• 22 Ide C, Cahill M, Pierre P, De Coene B, Baudrez V, Osseman M, Trigaux J P. Persistent trigeminal artery associated with basilar artery hypoplasia: MR and MRA findings.  Eur Radiol. 1999;  9 (5) 1006
• 23 Kida H, Nishikawa N, Matsunami K, Kawahito M, Ota M, Miyao S. [Sitting position in the neurosurgery: the results of a questionnaire sent to neurosurgeons of medical colleges].  Masui. 2000;  49 566-569
• 24 Klekamp J, Batzdorf U, Samii M, Bothe H W. The surgical treatment of Chiari I malformation.  Acta Neurochir. 1996;  138 788-801
• 25 Klekamp J, Batzdorf U, Samii M, Bothe H W. Treatment of syringomyelia associated with arachnoid scarring caused by arachnoiditis or trauma.  J Neurosurg. 1997;  86 233-240
• 26 Lazareff J A, Galarza M, Gravori T, Spinks T J. Tonsillectomy without craniectomy for the management of infantile Chiari I malformation.  J Neurosurg. 2002;  97 1018-1022
• 27 Levy W J, Mason L, Hahn J F. Chiari malformation presenting in adults: a surgical experience in 127 cases.  Neurosurgery. 1983;  12 377-390
• 28 Muth C M, Shank E S. Gas embolism.  N Engl J Med. 2000;  342 476-482
• 29 Nightingale S, Williams B. Hindbrain hernia headache.  Lancet. 1987;  1 731-734
• 30 Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa.  J Neurosurg. 1997;  86 40-47
• 31 Oakes W S, Tubbs R S. A comprehensive guide to the diagnosis and management of neurological problems.  In: Winn H (ed). Youman's Neurological Surgery.. WB Saunders, Philadelphia. 2004;  3 3347-3361
• 32 Ohshiro S, Inoue T, Hamada Y, Matsuno H. Branches of the persistent primitive trigeminal artery - an autopsy case.  Neurosurgery. 1993;  32 144-148
• 33 Oldfield E H. Syringomyelia.  J Neurosurg. 2001;  95 153-155
• 34 Oldfield E H. Cerebellar tonsils and syringomyelia.  J Neurosurg. 2002;  97 1009-1010
• 35 Oldfield E H, Muraszko K, Shawker T H, Patronas N J. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment.  J Neurosurg. 1994;  80 3-15
• 36 Oran I, Parildar M, Memis A, Yunten N. Catheter and MR angiography of persistent trigeminal artery associated with occipital arteriovenous malformation.  Comput Med Imaging Graph. 2000;  24 33-35
• 37 Padget D H. Development of cranial arteries in human embryo.  Contrib Embryol. 1948;  32 205-262
• 38 Paul K S, Lye R H, Strang F A, Dutton J. Arnold-Chiari malformation. Review of 71 cases.  J Neurosurg. 1983;  58 183-187
• 39 Piotin M, Miralbes S, Cattin F, Marchal H, Amor-Sahli M, Moulin T, Bonneville J F. MRI and MR angiography of persistent trigeminal artery.  Neuroradiology. 1996;  38 730-733
• 40 Pollack I F, Pang D, Kocoshis S, Putnam P. Neurogenic dysphagia resulting from Chiari malformations.  Neurosurgery. 1992;  30 709-719
• 41 Porter J M, Pidgeon C, Cunningham A J. The sitting position in neurosurgery: a critical appraisal.  Br J Anaesth. 1999;  82 117-128
• 42 Rhoton A L. Microsurgery of Arnold-Chiari malformation in adults with and without hydromyelia.  J Neurosurg. 1976;  45 473-483
• 43 Richardson D N, Elster A D, Ball M R. Intrasellar trigeminal artery.  AJNR Am J Neuroradiol. 1989;  10 205
• 44 Salas E, Ziyal I M, Sekhar L N, Wright D C. Persistent trigeminal artery: an anatomic study.  Neurosurgery. 1998;  43 557-561
• 45 Salcman M. Persistent trigeminal artery and pituitary adenoma.  J Neurosurg. 1989;  71 301-302
• 46 Saltzman G F. Patent primitive trigeminal arteries studied by cerebral angiography.  Acta Radiol. 1959;  51 329-336
• 47 Schaffranietz L, Grothe A, Olthoff D. [Use of the sitting position in neurosurgery. Results of a 1998 survey in Germany].  Anaesthesist. 2000;  49 269-274
• 48 Schuierer G, Laub G, Huk W J. MR angiography of the primitive trigeminal artery: report on two cases.  AJNR Am J Neuroradiol. 1990;  11 1131-1132
• 49 Shapiro H M, Drummond J C. Neurosurgical anesthesia and intracranial hypertension. In: Miller RD (ed). Anesthesia. Churchill Livingstone, New York 1990; 1737-1789
• 50 Suttner N, Mura J, Tedeschi H, Ferreira M A, Wen H T, de Oliveira E, Rhoton A L. Persistent trigeminal artery: a unique anatomic specimen - analysis and therapeutic implications.  Neurosurgery. 2000;  47 428-433; discussion 433-434
• 51 Tommasino C, Rizzardi R, Beretta L, Venturino M, Piccoli S. Cerebral ischemia after venous air embolism in the absence of intracardiac defects.  J Neurosurg Anesthesiol. 1996;  8 30-34
• 52 Tovar E A, Del Campo C, Borsari A, Webb R P, Dell J R, Weinstein P B. Postoperative management of cerebral air embolism: gas physiology for surgeons.  Ann Thorac Surg. 1995;  60 1138-1142
• 53 Williams B. Chronic herniation of the hindbrain.  Ann R Coll Surg Engl. 1981;  63 9-17
• 54 Williams B. Progress in syringomyelia.  Neurol Res. 1986;  8 130-145
• 55 Williams D L, Umedaly H, Martin I L, Boulton A. Chiari type I malformation and postoperative respiratory failure.  Can J Anaesth. 2000;  47 1220-1223
• 56 Wollschlaeger G, Wollschlaeger P B. The primitive trigeminal artery as seen angiographically and at postmortem examination.  Am J Roentgenol. 1964;  92 761-768