V .J. Novak, MD.
Clinica "Villa im Park" Rothrist, Lucerna, Svizzera
The "neurovascular primary headaches" are syndromes also pertinent to otorhinolaringology when CT demonstrates a reduced volume of the " ethmoidosphenoidal subcribriform chamber" according to the endo-exocranial hemoangiokinetics of this area. It is emphasized that in drug-resistant headaches recovery or consistent definitive improvement can be achieved only after surgical correction of certain anatomical parameters ( P. Bonaccorsi, V.J. Novak, S. Hoover ). Bonaccorsi and Novak independently identified the actual pathophysiologic mechanism and trigger zone ( " dysmorphism " ) for various types of neurovascular primary headaches.
Patients ( n = 446) with various types of headaches ( migraine, cluster headache and so-called idiopathic or primary headaches) were operated upon between 1973 and 1994. Septal correction, resection of the middle and superior concha, ethmoidectomy, and sphenoidectomy on the corresponding headache side or occasionally on both sides were carried out. Most patients (356, 80%) were asymptomatic postoperatively, 45 ( 10%) had a sensation of pressure in the head on rare occasions but no further migraine, and 45 ( 10%) continued to experience headache that occurred only rarely and was mild and of short duration. The overall success rate was 90%.
For cluster headache in 20 patients, we observed the following postoperative results: 19 cured (98%), 1 improved (2%).
Key Words: Pathogenesis, Neurovascular primary headaches, Septum-Ethmoid-Sphenoid Surgery
Many theories have been proposed over the years to explain the etiology of rnigraine, and the various controversies have often led to a theoretical dead end. An early theory based on experiments of Claude Bernard and E.D. Raymond (1860) considered vasospasm to be responsible for rnigraine attacks [9]. Other authors, including Hofmann, believe dilatation of cerebral arteries at the base of the skull to be the cause [9]. Wolff has stated that migraines arise due to vasodilatation and vasoconstriction of extra and intracranial vessels [9]. In Hebner's opinion, migraine pain
Rapid changes of weather have been known for some time to cause migraine [26, 27], as havealcohol, cheese, coffee, tea, seafood, fever, lack of
When such preexisting pathological changes and a central ganglionic predisposition for headache !are present and the nasal mucosa has a marked tendency to edema formation, a predisposition to migraine exists.
Fig. 1. AP sinus X-ray film showing deviation of nasal septum toward right and marked narrowing of right nasal cavity ( " dysmorphism ). Low-lying spina nasalis (arrows) in posterior septal region even with middle concha and middle nasal passage.
Surgery was performed on a total of 446 patients. Operative indications were pharmacologically resistant or frequent (more than 1-2 per week) migraine attacks; patients were initial1y examined by a neurologist and then referred for surgery .The diagnostic work-up was strictly standardized. A rhinological examination was carried out during which a so-cal1ed high septal deviation and narrowing of the area between the middle concha and middle nasal passage was frequently observed. Coronal CT studies of the skul1 and conventional tomography in the pre-computer era were also made in al1 cases. CT or MRI frequently showed dysmorphia (Fig. 1 -lA), for example, extreme narrowing of the middle or superior nasal passage, enlarged or pneumatized middle or superior conchae, a narrow, asymmetrical ethmoid labyrinth, a large ethmoidal bul1a, septum deviation or other morphologic changes of the skul1 base, particularly reduced volume of the ethmidosphenoidal subscribriform chamber (Fig. 2 -2A), and (Fig. 3 -3A). A second examination was then performed during a migraine attack. The swol1en nasal mucosa was sprayed with PrivineR (naphazolin); after decongestion the middle concha and middle nasal passage on the ipsilateral migraine
Fig.1A. CT scan showing samefinding as in Figure 1. A portion of the low-lying spina nasalis is seen in the posterior septal region (arrows) in close proximity to the ethmoid and sphenoid sinuses.
Fig. 2. CT .I(;an .I.liowing reduced volume of ethmoidosphenoidal subcribriform chamber.
Fig. 2A. CT scan and conventional tomogram showing normal ethmoidosphenoidal subcribriform chamber.
Fig. 3. MRI scan showing deviation of nasal septum toward left and marked narrowing of left nasal cavity, enlarged middle and superior concha, reduced volume of ethmoidosphenoidal subcribriform chamber, and thickening of left turbinate mucous membrane.
Patients (n = 446) with various types of headaches (migraine, cluster headache, and so-calied idi
Fig. 3A. Same MRI in coronal projection.
Fig. 4. Postoperative results in patients with migraine. Total 446 patients.
Most patients {356, 80% ) were asymptomatic postoperative1y, (45, 10%) had a sensation of
Fig. 5. Postoperative results in patients with cluster headache and cluster headache associated with migraine. Total 20 patients.
Fig. 6. VlP in mucosal tissue du ring a migraine attack.
Fig. 7. Serotonin granules in perivascular and mucosal tissue during a migraine attack.
The definitive surgical treatment of migraine and so-called idiopathic headaches has been described by both Bonaccorsi and Novak in numerous publications and at professional meetings [1-8, 14-22]. The two authors developed similar surgical techniques. My technique is more conservative, with septal correction (Cottle modification), resection of the middle concha, and ethmoidectomy on the ipsilateral migraine side. The Bonaccorsi technique is more radical: septal correction, ethmoidectomy, sphenoidectomy and resection of the middle concha are performed. Following the Cephalea Symposium in F1orence in 1985, I also included sphenoidectomy. Other authors have gradually adopted the same diagnostic process and operative technique with equa1ly successful results.
The pathophysiologic explanation is as fo1lows: the preexisting pathological changes in the nose, sinuses, and base of the sku1l and reduced volurne of the etmoidosphenoidal subcribriform chamber predispose to the development of migraine and have been designated by Bonaccorsi as "dysmorphism". Under nCirmal conditions, these dysmorphic changes do not lead to migraine attacks. Alterations in weather with rapid changes in atmospheric pressure, humidity, temperature and electrostatic charges (ionization, electromagnetic fields, etc.), produce edema of the nasal mucosa, which then induces migraine symptoms. Hormonal changes prior to and during menstruation (progesterone, estrogen decrease) cause mucosal edema of the nasal and paranasal sinuses, as do stress, lack of sleep and fatigue. When there is pre-existing narrowing of the nasal and paranasal sinus area and base of the skull, the additional edema caUses direct contact between the middle and superior concha and the septum or middle nasal passage. Edema formation in the sinuses reduces aeration. All these factor lead to tissue hypoxia, which directly causes pain. The pressure and hypoxia cause release of serotonin and other vasoactive substances by the tissues and "perivascular trigeminal and parasympathetic receptors in the base of the skull and later in the meningeal and cerebral vessels, which produces dilatation of the cerebral vessels and the resulting migraine pain [12, 25].
Even weather alterations with changes in electrostatic charge (e.g., positive charges, positive ionization) suffice to induce serotonin secretion, leading to further edema formation and increased pain [26, 27].
The precondition for the headaches is a strong reactivity of the nasal rhinobasal mucosa and perivascular trigeminal and vegetative receptors in the narrow skull base to various exogenic and endogenic noxious agents, modulated by the chronobiological and circadian cycles in combination with a central ganglionic predisposition to cephalea.
The mucosal edema leads to the known mechanisms of hypoxia; secretion of vasoactive tissue hormones such as serotonin, substance P, VIP , neurotension, prostaglandin, bradykinin, somatostatin, and others and activates various enzymes from the mediator cells.
The resulting headache is a consequence of the vasoactive biochemical components in combination with reflex trigeminal pain and, later, cerebral hypoxia.
Mucosal biopsies from the middle nasal meatus (middle concha) were taken from five patients during a migraine attack and during a pain-free interval between attacks. There was edematous tissue with dilated blood vessels, increased membrane permeability, transudation and reduced blood flow in combination with elevated numbers or leucocyte mediator cells, as weIl as an increased number of vasoactive peptides (VIP , Substance P, histamine, and serotonin granules) in perivascular and mucosal tissue taken during the presence of attacks (Figure 6- 7).
Conclusion
The tindings in the present study have enabled the true pathophysiologic background, environment, and trigger zone for the development of migraines and so-calIed vascular idiopathic primary headaches to be documented and have made possible a successful surgical approach to the different types of headache.
Le "cefalee neurovascolari primarie" sono sindromi di pertinenza anche ORL quando con esame TC viene evidenziata una ridotta volumetria della "camera etmoidosfenoidale sottocribrosa", ai fini emoangiocinetici della circolazione endo-esocranica di questo distretto.
Viene posto in risalto che i casi farmacoresistenti possono ottenere guarigioni o sostanziali, costanti miglioramenti solo dopo la correzione chirurgica di alcuni parametri anatomici. (P. Bonaccorsi, V.J. Novak, S. Hoover). Bonaccorsi e Novak indipendentemente hanno identificato l'attuale meccanismo fisiopatologico e la zona trigger (dismorfismi) per vari tipi di cefalee neurovascolari primarie.
Dal1973 al1994 sono stati operati n. 446 pazienti sofferenti di varieforme di cefalee (emicranie, cefalee a grappolo ed altre cefalee codificate come idiopatiche o primarie). Sono state praticate correzioni deI setto, resezioni deI turbinato medio e superiore, etmoidectomie e sfenoidectomia sul lato corrispondente alla cefalea o occasionalmente in entrambi i lati.
La maggioranza dei pazienti risulta asintomatica dopo l'intervento (356 = 80%), 45 pazienti ( 10%) hanno una sensazione di pressione al capo rare volte ma non hanno piu accusato emicrania e 45 (10%) continuano ad avere cefalea solo raramente e di minore e breve durata. La positivita globale e deI 90%.
Nei 20 pazienti sofferenti di cefalea a grappolo, osserviamo i seguenti risultati post operatori: 19 guariti (98%), 1 migliorato (2%).
[1] BONACCORSI P.: Terapia chirurgica (etmoido-sfenectomia decompressiva neurovascolare) dell.emicrania comune. classica, comitata e delle cefalee a grappolo a patogenesi ringogena. Rel. all'lncontro di Aggiornamento sulle cefalee della Lega italiana per la lotta medica e chirurgica contro le cefalee. Firenze. 23 novembre 1985. Abstracts, 22-47. 1985. Ed. Tipografia Molinari, Piacenza, 1985.
[2] BONACCORSI P.: Headache relieved by ethmoidosphenoidectomy. Com. e film presentati al Cong. Annuale della Soc. Danese di Rinologia, Odense, Danimarca, 10-11 Maggio 1985.
[3] BONACCORSI P.: L 'utilita della TAC per la diagnostica e la chirurgia rinocranica dell.emicrania e delle cefalee neurovascolari. Com. al 72 Cong. Naz. della Soc. It. O.R.L. e Chirurgia CervicoFacciale, Viareggio, 15-18 maggio 1985. Abstracts 90-91, 1985.
[4] BONACCORSI P.: Indagine epidemiologica clinicoanamnestica sulle cefalee della eta evolutiva eseguita su di un campione di 2158 soggetti (in col. con LA Lieto, M Pens, L Olivati). Poster presentato al1'8 Cong. Naz. della Soc. it. per 10 Studio delle Cefalee, Belgirate, 12-23 febbraio 1985. Abstracts, 1963-64, 1985.
[5] BONACCORSI P.: The surgical therapy of pharmacological resistant cluster headache. In: Rizzi R. Nicoscia S, eds. Proceedings of the 1 st ItalianPo1ish meeting on pain. Vercelli, april 2-5 1987. Auditorium S. Chiara. Abstracts 128-130.
[6] BONACCORSI P.: Primary headaches compared with idiopathic rhinogenic headaches: the actual diagnostic misunderstandings. Rhino1ogy. Suppl 1 :63, 1988.
[7] BONACCORSI P.: Surgical therapy ofprimary neurovascular headaches with nasal trigger: statistical and operatory technics updating. XXVI World Cong. Intern. College Surgeons, Milano. In: Montorsi M., Granelli P. (Eds.), Specialist Surgery, Bologna: XII Monduzzi Ed. 127-131, 1988.
[8] BONACCORSI P., NOVAK V.J.: The neurovascular decompressive surgery of the rhino-skull base in primary headaches with rhinogenous trigger. Proceedings of XV World Congress of Otorhin01aryng010gy Head and Neck Surgery , Instanbul, V 01. II, 547-553, 1993.
[9] GLA v AN L.: Zivcane Blesti: Med. Knijga Zagreb 6: 866-868, 1983.
[10] HANINGTON E.: Migraine: a platelet hypothesis. Biomedicine 30: 65-66, 1970.
[11] HOOVER S.: The nasal patho-physiology of headachesand migraines. Diagnosis and treatment of the allergy, infection and nasal septal spurs that cause them. Rhinology (Suppl 2): 3-20. 1987.
[12] MCKENZIE E.T., EDVINSSON L.: Effects ofserotonin on cerebra[ circulation and metabolism as related to cerebrovascular disease. In: Bes A, Geraud G, eds, Cerebral circulation and neurotransmitters. Amsterdam: Excerpta Medica, 1963- 171, 1980.
[13] MONRO J., BROSTOFF J., CRINI C., ZILKA K.: Food a[[ergy in migraine. Study of dietary exclusion and RAST. Lancet 5: 1-4, 1980.
[15] NOVAK V.J.: Pathogenesis and surgical therapy of migraine attacks caused by weather and menstruation. Rhinology 22: 165-170, 1984.
[16] NOVAK V.J.: Pathogenesi e Terapia chirurgica di varie sindromi emicraniche con "Tigger" rinogeno. Rel. a11'Incontro di aggiornamento sulle cefalee della Lega italiana per la lotta medica e chirurgica contro le cefalee, Firenze, 23 novembre 1985. Abstracts, 16-1985. Ed. Tipograafia Molinari, Piacenza, 1985.
[17] NOVAK V.J.: Pathogenesi e terapia chirurgica di varie sindromi emicraniche riferibili a variazioni atmosferiche (favonio), menstruazione, stress ad alimentazione. 74 Cong. Naz. della Soc. it. O.R.L. e Chirurgia Cervico-Facciale, Milano, 27-30 Maggio 1987. Abstracts, 73, 1987.
[18] NOVAK V.J., NOVOSELAC M., MÜLLER W.: Pathogenesis and surgical treatment of migraine attacks caused ba weather (föhn), menstruation. stress and nutrition. Rhinology. Suppl 1, 1988. Abstract Book. 12th Congress European Rhinologic Society including the VIIth I.S.I.A.N. Amsterdam, June 19-22, 1988. Abstracts 62.
[19] NOVAK V.J., MAKEK M.: Pathogenesis and surgical tratment of migraine and neurovascular headaches with rhinogenic trigger. Head and Neck 14: 467-472, 1992.
[20] NOVAK V.J.: Pathogenesis of migraine and Neurovascular headaches with rhlnogenic trigger. 14th Congress of the European Rhinologic society and 11 International Symposium of Infection and AIlergy of the nose. Roma October 6-10 1992; (Suppell): 59-74.
[21] NOVAK V.J.: Patogenesi e trattamento chirurgico della Emicrania e delle Cefalee neurovascolari con trigger Rinogeno. Algologia. Six-rnonthly J. of studies on Pain editd ba Pier1uigi Zucchi, 2-5 April 1992. Abstracts, 115,1992.
[22] NOVAK V.J.: Pathogenesis and surgical treatment of neurovascular primay headaches. The J of the Croatian Medica1 Association-Rijeka, 19-22, 1994, Rijeka. Medicina, Vo1. 30, supplement 2 Rijeka 1994.
[23] POLLERI A., MURIALDO G., MARTIGNONI E., NAPPI G., SAVOLDI F.: Benserazide induces migraine attacks. Irrelevance of concomitat hyperprolactineamia. Farmaco (Sci) 34: 465-468, 1979.
[24] SICURETI F., FANCIULACCI M., FRANCH G., DEL BIANCO P.L.: Serotin-bradykinin potentiation on the pain receptors in man. Life Sci 4: 309-316, 1965.
[25] SICURETI F., ANSELMI B., FANCIULAcCI M.: The serotonin (5HT) theory of migraine. Adv. Neurol. 4: 383-394, 1974.
[26] SULLMANN F.G.: Migraine and haedache due to weather and allied causes and its specific treatment. Ups J Med (Suppel 31): 41-44, 1980.
[27] WILKINSON M., WOODROW, J.: Migraine and weather. Headache 19: 375-378, 1979.
[28] WILSON C.W., KRIKER J.G., WARNES H., O'MALLEY M.: The clinical features of migraine as a manifestation of allergic disease. Postgrad Med. J. 56: 617-621, 1980.