Primary intranasal encephalocele: a rare cause of bacterial meningitis ',
Berlit P, Rakicky J, Tornow K. Primary intranasal encephalocele: a rare cause of bacterial meningitis. Acta Neurol Scand 1992: 85: 404-407.
P. Berlit J. Rakicky', K. Tornow'
In four patients with bacterial meningitis a primary intranasal encephalocele was found as portal of entry. In two of the cases the maXormation had been misdiagnosed as a nasal polyp and operated upon. In two patients a cerebrospinal fluid fistula developed spontaneously at the age of 54 years. None of the patients had associated symptoms indicating the presence of a cleft. Encephaloceles can be readily visualized by computed tomography particularly in coronal sections. The treatment of choice is transcranial surgical repair.
1
The embryogenesis closure of the neural tube has usually been completed by the end of the first month. Developmental disorders can lead both to malformations of the surrounding mesenchymal structures (example: spina bifida occulta) and to herniation. If only the meninges filled with Cerebrospinal fluid protrude through the mesenchymal defect we speak of a meningocele, if the sac also contains cerebral or spinal cord tissue it is termed an encephalocele or myelomeningocele. These malformations are most commonly found in the occipital and spinal regions and are usually diagnosed in childhood. Encephaloceles in the temporal or frontal regions are a considerably less common finding. Depending on the structures affected a distinction is made between nasofrontal, nasoethmoidal, nasopharyngeal, frontoethmoidal, sphenomaxillary, sphenopharyhgeal
'
Departments of Neurology, Neuroradiology, Mannheim Clinic, University of Heidelberg, Germanv
Peter Berlit, Neurologische Klinik im Klinikum Mannheim der Universitat Heidelberg, Theodor-Kutzer-Ufer, D-6800 Mannheim 1, Germany Accepted for publication October 16, 1991
and sphenoethmoidal encephaloceles (1, 2). Intranasal encephaloceles pose particular differential diagnostic problems - a nasal cerebrospinal fluid fistula, carrying the risk of bacterial meningitis, can develop either spontaneously or subsequent to ear, nose and throat surgery performed on the basis of a misdiagnosis. We report below the cases of four patients in whom a nasal cnccphalocele was found as the cause of bacterial meningitis (Table 1). Case reports
Patient I . This 60-year-old patient was admitted to hospital with acute onset of headaches and highly febrile temperatures. There was granulocytic ccrcbrospinal fluid(CSF)-pleocytosis of 4400 cells/mm3
Table 1. Clinical and neuroradiologicalfindings in 4 patients with encephalocele
Patient
Age, sex
Bacterial Meningitis
Pathogens isolated
1
60,m
3rd recurrence in 6 yrs
none
2 3
54,m 13,m
first episode 4 yrs previously, presumably several recurrences
peumococci n.p.
4
54, f
first episode
streptococci
n.p. = not performed - = negative
404
CSF leakage
Operation, injury
CT scan
Isotope cistrnogr.
Plain X-ray
Surgery
rhinorrhoea from left nostril for 12 years none rhinorrhoea from right nostril for past year
'polypectomy' 12 years previously
t
t
-
t
-
t
n.p.
-
commotio 6 years previously, 'polypectomy' 4 months previously
n.p.
t
t
-
t
opacity of paranasal sinuses
rhinorrhoea from left nostril for past year
-
t t
t
Intranasal encephalocele no pathogens were isolated. The symptoms subsided completely under combined antibiotic treatment with penicillin, cefotaxime and gentamicin. The patient’s past history showed that he had undergone ambulatory nasal polypectomy 12 years previously - after this he had noted for the first time that a clear fluid leaked from his left nostril when he bent his head forward. He had already had meningitis 6 and 2 years previously which had been treated with antibiotics in each case. On computed tomography (CT) of the skull a 0.8cm left paramedian bone defect in the planum sphenoidale with a small soft tissue hernia was seen in the rhinobasal region (Fig. 1). In isotope cisternography quantitative evaluation of the cotton pledgets from both nostrils showed definite rhinorrhoea from the left nostril. A primary intranasal encephalocele was confirmed at operation and a duraplasty was performed. At the time of discharge the patient was symptom free. -
Patient 2. This 54-year-old patient was admitted to hospital with increasing headaches since the previous day and temperatures of up to 39°C. Examination of the CSF showed mixed cell pleocytosis of 2000 cells/mm3, culture revealed Diplococcus pneumoniae. Combined antibiotic treatment with peni-
cillin and a cephalosporin led to complete resolution of symptoms. The plain X-rays of skull, paranasal sinuses and skull base were negative. Computed tomography showed a bone defect measuring about 3 mm in the region of the ethmoidal bone to the right of the crista galli. The patient was transferred to an ear nose and throat hospital for further treatment. The encephalocele was confirmed during operation; there were no further symptoms 3 months after discharge from hospital. Patient 3. This 13-year-old boy was admitted to hospital for surgical repair of a right nasal cerebrospinal fluid fistula. He reported commotio cerebri 6 years previously after a fall followed by loss of consciousness for 2 min. 2 years later he had had impaired consciousness lasting several hours and febrile temperatures. Antibiotic treatment had been instituted without hospitalization for further diagnosis. Since then he had had frequent bouts of fever and headache which had subsided under antibiotic treatment in each case. During the last 8 months he had noticed discharge of a clear liquid from his right nostril. Chronic rhinitis had been diagnosed. Finally, 4 months prior to hospitalization he had undergone ambulatory right nasal polypectomy. On histological examination the material excised was diagnosed as
Fig. 1. Bony defect with encephalocele in CT. Sagittal (a) and coronal (b) section
405
Berlit et al. cerebral tissue. The subsequent CT of the skull revealed a bone defect in the region of the anterior ethmoid bone and the posterior wall of the right frontal sinus. An encephalocele protruding in the region of the junction between the posterior wall of the frontal sinus and the anterior ethmoid bone was confrmed at operation. The defect was closed by plastic repair. At discharge, the patient was symptom free. Patient 4 . This 54-year-old patient was admitted to hospital after onset of extremely severe headaches and confusion only a few hours previously. He had marked meningism and febrile temperatures of 38.7 O C. A CSF specimen obtained by lumbar puncture showed mainly granulocytic pleocytosis of 11 000 cells/mm3. The main pathogen isolated was Streptococcus viridans. Under combined antibiotic treatment with penicillin and an aminoglycoside the symptoms receded completely. Isotope cisternography showed increased activity in the left rhinobasal region after only 40 min, increasing markedly in the next 24 h. Asked directly, the patient reported that she had noted discharge of fluid from her left nostril in the course of the past year - especially when she bent her head forward. This had been interpreted as chronic rhinitis. Plain X-rays showed shadowing of the sphenoidal sinus and ethmoidal cells on the left - conventional tomography revealed a bone defect. CT was not performed. At operation an encephalocele was found and repositioned followed by plastic repair of the defect. At follow up after 4 months, the patient complained about weather dependent diffuse headaches, but was symptomfree otherwise. Discussion
In three of the four cases reported bacterial meningitis had been the reason for hospitalization, in the fourth case a history of recurrent meningitis can be assumed. Three of the patients had already had rhinorrhoea - the longest history going back over 12 years. In this latter case and in the young patient ambulatory surgery had been performed following the suspected diagnosis of nasal polyps. In Patient 3 histological examination of the excised material led to the suspected diagnosis of encephalocele. In Patient l no histology was performed but the subsequent radiological and intraoperative findings suggest a diagnosis of primary encephalocele, misdiagnosed as a nasal polyp. The literature contains a number of reports of patients in whom nasal encephaloceles were operated upon being misdiagnosed as nasal polyps or other space occupying processes in the nasal region (3-6). This applies particularly to children as in the
406
case of our third patient. However, nasal polyps are rare before the age of 5 , so that CT of the skull should always be performed before planned polypectomy (6). The reported cases show impressively that a primary nasal encephalocele can also become symptomatic for the first time in advanced age. Three of the patients were 50 years old or more at the time of diagnosis. In 2 cases initial symptoms in the form of bacterial meningitis occurred at the age of 54 years. If - as in our Patient 2 - typical pathogens of primary bacterial meningitis such as meningococci or pneumococci are found in patients of this age the danger of overlooking a cerebrospinal fluid fistula is particularly great. This case demonstrates that even in the event of apparently typical pathogens neuroradiological examination should be performed to clarify possible portals of entry. Whle the bone defect can sometimes be shown in plain X-rays (as in one of our four cases) the diagnostic method of choice for detection ofbony changes and the encephalocele itself is CT which, particularly with additional coronal sections, permits visualization of both the defect and the hernia itself (6-8). A midline defect in the region of the anterior cranial fossa should be looked for, primary encephaloceles usually being found at the level of the foramen caecum. The defect occurs at the junction between the orbital portions ofthe frontal bone and the ethmoidal bone. It usually measures between 0.5 and 3 cm (9). The cerebrospinal fluid fistula itself can be diagnosed by testing the nasal discharge for glucose and can be elegantly documented by isotope cisternography. Encephaloceles in the frontal and temporal regions occur considerably more often as a consequence of injuries, surgical procedures, inflammation or tumors - the so-called secondary forms. Primary encephaloceles occur in 1 of 4000 births in the USA and Europe, in Asia they appear to be considerably more common (6, 9). Spontaneous encephaloceles prolapsing through the tegmen tympani or tegmen mastoideum into the middle ear or mastoid cavity are rather less common. Such temporal encephaloceles lead to otorrhoea (10, 11). Very rarely both frontal and temporal clefts with CSF leakage are found in one and the same patient (12). Clinical signs of the malformation can be present in the case of both temporal and frontal encephaloceles. These include hypertelorism, exotropia, oral clefts and alterations of the optic nerve (8, 13). Pale optic disc, dysplasia and coloboma have been described (14, 15). It is not clear why the first manifestation of rhinorrhoea in nasal encephalocele frequently occurs in advanced age without evidence of prior trauma or surgery in the nasal region (16, 17). Conceivable reasons might be a - possibly only transient - elevation of intracranial pressure such as suspected by Iizuka (4) and Vaquero et al. (18) in patients with concom-
Intranasal encephalocele itant meningioma, or other fornis of mechanical irritation (e.g. minor head injuries). In the literature manifestation of a cerebrospinal fluid fistula in patients with nasal encephalocele has been described after pneumoencephalography (3) and after haemodialysis (8). In the interests of the patient it is crucial that bacterial meningitis should lead not only to a search for inflammatory changes in the ear, nose and throat regions but that the possibility of a congenital malformation is also entertained. This also applies in older patients with no evidence of external clinical changes - none of our patients had signs indicating the presence of a congenital malformation. Particular attention is called for if there is a history of rhinoor otorrhoea or if “nasal polyps” have been removed in the past. The treatment of choice for primary nasal encephalocele not complicated by other malformations is transcranial surgery. A subfrontal approach is recommended (9, 19, 20). References 1. PALENCIA LUANCES R, BLANCOQUIROSA, GOMEZS, IZQUIERDOJM. Meningitis recurrente por meningoencefalocele esfenoetmoidal. An Esp Pediatr 1982: 16: 92-94. M, VONWILDK, GRAUH, HACKERH. Zur 2. SCHAEFER Diagnostik und Therapie der nasoethmoidalen Enzephalozelen Laryngol Rhinol Otol (Stuttg) 1976: 55: 581-585. 3. CHOUDHURY AR, TAYLOR JC. Primary intranasal encephalocele. Report of four cases. J Neurosurg 1982: 57: 552-555. 4. IIZUKA J. Spontane frontobasale Liquorfistel. Zentralbl Neurochir 1980: 41: 201-208. 5. SHUGARJM, SOM PM, EISMANW, BILLERHF. Nontraumatic cerebrospinal fluid rhinorrhea. Laryngoscope 1981: 91: 114-120. 6. VITALJP, PENHAR, VEIGAG, OLIASJ, CRUZM. Intranasal encephalomeningocele-case report. Rev Laryngol Otol Rhinol (Bord) 1987: 108: 497-499.
7. TAKAHASHI M, SUZUKIS, NAGAOH, MATSUDA H. A case of transethmoidal encephalocele with recurrent bacterial meningitis. No To Hattatsu 1988: 20: 64-68. 8. YAMASHITA H, KURIHARA M, KAWANOT, MORI K, KUNIMURA M. Basal encephalomeningocele in an adult - a case report and clinico-anatomical classification. No Shinkei Geka 1985: 13: 425-431. 9. RAHMANNU. Nasal encephalocele. Treatment by transcranial operation. J Neurol Sci 1979: 42: 73-85. 10. KAMERERDB, CAPAROSA RJ. Temporal bone encephalocele - diagnosis and treatment. Laryngoscope 1982: 92: 878882. 11. KEMINKJL, GRAHAM MD, KARTUSHJM. Spontaneous encephalocele of the temporal bone. Arch Otolaryngol Head Neck Surg 1986: 112: 558-561. 12. BRODSKY L. Spontaneous cerebrospinal fluid otorrhea and rhinorrhea coexisting in a patient with meningitis. Laryngoscope 1984: 94: 1351-1354. 13. PORADOWSKA W, LODZINSIUK, RESZKES, JAWORSKA M, LENKIEWICZ T, TRACZYNSKA-KUBIN H. Przepuklini oponowo-mozgowe przednie i heterotopie thanki nerwowej u dzieci. Pediatr Pol 1972: 48: 1359-1366. 14. BULLARD DE, CROCKARD HA, MCDONALD WI. Spontaneous cerebrospinal fluid rhinorrhea associated with dysplastic optic discs and a basal encephalocele: case report. J Neurosurg 1981: 54: 807-810. 15. GOLDHAMMER Y, SMITHJL. Optic nerve anomalies in basal encephalocele. Arch Ophthalmol 1975: 93: 115-1 18. 16. DEMPSEY PK, HARBAUGH RE. Encephalomeningocele presenting with spontaneous cerebrospinal fluid rhinorrhea in al elderly man: case report. Neurosurgery 1988: 23: 637-640. 17. FOCHEM K, KLUMAIR J, ZECHNER G. Meningoenzephalozele als Ursachen einer rhinogenen Liquorrhoe. Rontgenblatter 1983: 36: 96-98. 18. VAQUEROJ, CABEZUDO JM, SALAZARAR, BRASAJ. Symptomatic intrasphenoidal meningoencephalocele after removal of a parasagittal meningioma. Acta Neurochir (Wien) 1981: 57: 61-65. 19. LUYENDIJK W. Encephaloceles intranasales. A propos de 8 cas opiires. Neurochirurgie 1969: 15: 240-241. 20, RUSSEGGER L, FISCHER J, TWERDY K. Zur operativen Versorgung frontobasaler Defekte auf transkraniell subfrontalem Zugangswege. Zentralbl Neurochir 1988: 49: 290-297.
407
Berlit P, Rakicky J, Tornow K. Primary intranasal encephalocele: a rare cause of bacterial meningitis. Acta Neurol Scand 1992: 85: 404-407.
P. Berlit J. Rakicky', K. Tornow'
In four patients with bacterial meningitis a primary intranasal encephalocele was found as portal of entry. In two of the cases the maXormation had been misdiagnosed as a nasal polyp and operated upon. In two patients a cerebrospinal fluid fistula developed spontaneously at the age of 54 years. None of the patients had associated symptoms indicating the presence of a cleft. Encephaloceles can be readily visualized by computed tomography particularly in coronal sections. The treatment of choice is transcranial surgical repair.
1
The embryogenesis closure of the neural tube has usually been completed by the end of the first month. Developmental disorders can lead both to malformations of the surrounding mesenchymal structures (example: spina bifida occulta) and to herniation. If only the meninges filled with Cerebrospinal fluid protrude through the mesenchymal defect we speak of a meningocele, if the sac also contains cerebral or spinal cord tissue it is termed an encephalocele or myelomeningocele. These malformations are most commonly found in the occipital and spinal regions and are usually diagnosed in childhood. Encephaloceles in the temporal or frontal regions are a considerably less common finding. Depending on the structures affected a distinction is made between nasofrontal, nasoethmoidal, nasopharyngeal, frontoethmoidal, sphenomaxillary, sphenopharyhgeal
'
Departments of Neurology, Neuroradiology, Mannheim Clinic, University of Heidelberg, Germanv
Peter Berlit, Neurologische Klinik im Klinikum Mannheim der Universitat Heidelberg, Theodor-Kutzer-Ufer, D-6800 Mannheim 1, Germany Accepted for publication October 16, 1991
and sphenoethmoidal encephaloceles (1, 2). Intranasal encephaloceles pose particular differential diagnostic problems - a nasal cerebrospinal fluid fistula, carrying the risk of bacterial meningitis, can develop either spontaneously or subsequent to ear, nose and throat surgery performed on the basis of a misdiagnosis. We report below the cases of four patients in whom a nasal cnccphalocele was found as the cause of bacterial meningitis (Table 1). Case reports
Patient I . This 60-year-old patient was admitted to hospital with acute onset of headaches and highly febrile temperatures. There was granulocytic ccrcbrospinal fluid(CSF)-pleocytosis of 4400 cells/mm3
Table 1. Clinical and neuroradiologicalfindings in 4 patients with encephalocele
Patient
Age, sex
Bacterial Meningitis
Pathogens isolated
1
60,m
3rd recurrence in 6 yrs
none
2 3
54,m 13,m
first episode 4 yrs previously, presumably several recurrences
peumococci n.p.
4
54, f
first episode
streptococci
n.p. = not performed - = negative
404
CSF leakage
Operation, injury
CT scan
Isotope cistrnogr.
Plain X-ray
Surgery
rhinorrhoea from left nostril for 12 years none rhinorrhoea from right nostril for past year
'polypectomy' 12 years previously
t
t
-
t
-
t
n.p.
-
commotio 6 years previously, 'polypectomy' 4 months previously
n.p.
t
t
-
t
opacity of paranasal sinuses
rhinorrhoea from left nostril for past year
-
t t
t
Intranasal encephalocele no pathogens were isolated. The symptoms subsided completely under combined antibiotic treatment with penicillin, cefotaxime and gentamicin. The patient’s past history showed that he had undergone ambulatory nasal polypectomy 12 years previously - after this he had noted for the first time that a clear fluid leaked from his left nostril when he bent his head forward. He had already had meningitis 6 and 2 years previously which had been treated with antibiotics in each case. On computed tomography (CT) of the skull a 0.8cm left paramedian bone defect in the planum sphenoidale with a small soft tissue hernia was seen in the rhinobasal region (Fig. 1). In isotope cisternography quantitative evaluation of the cotton pledgets from both nostrils showed definite rhinorrhoea from the left nostril. A primary intranasal encephalocele was confirmed at operation and a duraplasty was performed. At the time of discharge the patient was symptom free. -
Patient 2. This 54-year-old patient was admitted to hospital with increasing headaches since the previous day and temperatures of up to 39°C. Examination of the CSF showed mixed cell pleocytosis of 2000 cells/mm3, culture revealed Diplococcus pneumoniae. Combined antibiotic treatment with peni-
cillin and a cephalosporin led to complete resolution of symptoms. The plain X-rays of skull, paranasal sinuses and skull base were negative. Computed tomography showed a bone defect measuring about 3 mm in the region of the ethmoidal bone to the right of the crista galli. The patient was transferred to an ear nose and throat hospital for further treatment. The encephalocele was confirmed during operation; there were no further symptoms 3 months after discharge from hospital. Patient 3. This 13-year-old boy was admitted to hospital for surgical repair of a right nasal cerebrospinal fluid fistula. He reported commotio cerebri 6 years previously after a fall followed by loss of consciousness for 2 min. 2 years later he had had impaired consciousness lasting several hours and febrile temperatures. Antibiotic treatment had been instituted without hospitalization for further diagnosis. Since then he had had frequent bouts of fever and headache which had subsided under antibiotic treatment in each case. During the last 8 months he had noticed discharge of a clear liquid from his right nostril. Chronic rhinitis had been diagnosed. Finally, 4 months prior to hospitalization he had undergone ambulatory right nasal polypectomy. On histological examination the material excised was diagnosed as
Fig. 1. Bony defect with encephalocele in CT. Sagittal (a) and coronal (b) section
405
Berlit et al. cerebral tissue. The subsequent CT of the skull revealed a bone defect in the region of the anterior ethmoid bone and the posterior wall of the right frontal sinus. An encephalocele protruding in the region of the junction between the posterior wall of the frontal sinus and the anterior ethmoid bone was confrmed at operation. The defect was closed by plastic repair. At discharge, the patient was symptom free. Patient 4 . This 54-year-old patient was admitted to hospital after onset of extremely severe headaches and confusion only a few hours previously. He had marked meningism and febrile temperatures of 38.7 O C. A CSF specimen obtained by lumbar puncture showed mainly granulocytic pleocytosis of 11 000 cells/mm3. The main pathogen isolated was Streptococcus viridans. Under combined antibiotic treatment with penicillin and an aminoglycoside the symptoms receded completely. Isotope cisternography showed increased activity in the left rhinobasal region after only 40 min, increasing markedly in the next 24 h. Asked directly, the patient reported that she had noted discharge of fluid from her left nostril in the course of the past year - especially when she bent her head forward. This had been interpreted as chronic rhinitis. Plain X-rays showed shadowing of the sphenoidal sinus and ethmoidal cells on the left - conventional tomography revealed a bone defect. CT was not performed. At operation an encephalocele was found and repositioned followed by plastic repair of the defect. At follow up after 4 months, the patient complained about weather dependent diffuse headaches, but was symptomfree otherwise. Discussion
In three of the four cases reported bacterial meningitis had been the reason for hospitalization, in the fourth case a history of recurrent meningitis can be assumed. Three of the patients had already had rhinorrhoea - the longest history going back over 12 years. In this latter case and in the young patient ambulatory surgery had been performed following the suspected diagnosis of nasal polyps. In Patient 3 histological examination of the excised material led to the suspected diagnosis of encephalocele. In Patient l no histology was performed but the subsequent radiological and intraoperative findings suggest a diagnosis of primary encephalocele, misdiagnosed as a nasal polyp. The literature contains a number of reports of patients in whom nasal encephaloceles were operated upon being misdiagnosed as nasal polyps or other space occupying processes in the nasal region (3-6). This applies particularly to children as in the
406
case of our third patient. However, nasal polyps are rare before the age of 5 , so that CT of the skull should always be performed before planned polypectomy (6). The reported cases show impressively that a primary nasal encephalocele can also become symptomatic for the first time in advanced age. Three of the patients were 50 years old or more at the time of diagnosis. In 2 cases initial symptoms in the form of bacterial meningitis occurred at the age of 54 years. If - as in our Patient 2 - typical pathogens of primary bacterial meningitis such as meningococci or pneumococci are found in patients of this age the danger of overlooking a cerebrospinal fluid fistula is particularly great. This case demonstrates that even in the event of apparently typical pathogens neuroradiological examination should be performed to clarify possible portals of entry. Whle the bone defect can sometimes be shown in plain X-rays (as in one of our four cases) the diagnostic method of choice for detection ofbony changes and the encephalocele itself is CT which, particularly with additional coronal sections, permits visualization of both the defect and the hernia itself (6-8). A midline defect in the region of the anterior cranial fossa should be looked for, primary encephaloceles usually being found at the level of the foramen caecum. The defect occurs at the junction between the orbital portions ofthe frontal bone and the ethmoidal bone. It usually measures between 0.5 and 3 cm (9). The cerebrospinal fluid fistula itself can be diagnosed by testing the nasal discharge for glucose and can be elegantly documented by isotope cisternography. Encephaloceles in the frontal and temporal regions occur considerably more often as a consequence of injuries, surgical procedures, inflammation or tumors - the so-called secondary forms. Primary encephaloceles occur in 1 of 4000 births in the USA and Europe, in Asia they appear to be considerably more common (6, 9). Spontaneous encephaloceles prolapsing through the tegmen tympani or tegmen mastoideum into the middle ear or mastoid cavity are rather less common. Such temporal encephaloceles lead to otorrhoea (10, 11). Very rarely both frontal and temporal clefts with CSF leakage are found in one and the same patient (12). Clinical signs of the malformation can be present in the case of both temporal and frontal encephaloceles. These include hypertelorism, exotropia, oral clefts and alterations of the optic nerve (8, 13). Pale optic disc, dysplasia and coloboma have been described (14, 15). It is not clear why the first manifestation of rhinorrhoea in nasal encephalocele frequently occurs in advanced age without evidence of prior trauma or surgery in the nasal region (16, 17). Conceivable reasons might be a - possibly only transient - elevation of intracranial pressure such as suspected by Iizuka (4) and Vaquero et al. (18) in patients with concom-
Intranasal encephalocele itant meningioma, or other fornis of mechanical irritation (e.g. minor head injuries). In the literature manifestation of a cerebrospinal fluid fistula in patients with nasal encephalocele has been described after pneumoencephalography (3) and after haemodialysis (8). In the interests of the patient it is crucial that bacterial meningitis should lead not only to a search for inflammatory changes in the ear, nose and throat regions but that the possibility of a congenital malformation is also entertained. This also applies in older patients with no evidence of external clinical changes - none of our patients had signs indicating the presence of a congenital malformation. Particular attention is called for if there is a history of rhinoor otorrhoea or if “nasal polyps” have been removed in the past. The treatment of choice for primary nasal encephalocele not complicated by other malformations is transcranial surgery. A subfrontal approach is recommended (9, 19, 20). References 1. PALENCIA LUANCES R, BLANCOQUIROSA, GOMEZS, IZQUIERDOJM. Meningitis recurrente por meningoencefalocele esfenoetmoidal. An Esp Pediatr 1982: 16: 92-94. M, VONWILDK, GRAUH, HACKERH. Zur 2. SCHAEFER Diagnostik und Therapie der nasoethmoidalen Enzephalozelen Laryngol Rhinol Otol (Stuttg) 1976: 55: 581-585. 3. CHOUDHURY AR, TAYLOR JC. Primary intranasal encephalocele. Report of four cases. J Neurosurg 1982: 57: 552-555. 4. IIZUKA J. Spontane frontobasale Liquorfistel. Zentralbl Neurochir 1980: 41: 201-208. 5. SHUGARJM, SOM PM, EISMANW, BILLERHF. Nontraumatic cerebrospinal fluid rhinorrhea. Laryngoscope 1981: 91: 114-120. 6. VITALJP, PENHAR, VEIGAG, OLIASJ, CRUZM. Intranasal encephalomeningocele-case report. Rev Laryngol Otol Rhinol (Bord) 1987: 108: 497-499.
7. TAKAHASHI M, SUZUKIS, NAGAOH, MATSUDA H. A case of transethmoidal encephalocele with recurrent bacterial meningitis. No To Hattatsu 1988: 20: 64-68. 8. YAMASHITA H, KURIHARA M, KAWANOT, MORI K, KUNIMURA M. Basal encephalomeningocele in an adult - a case report and clinico-anatomical classification. No Shinkei Geka 1985: 13: 425-431. 9. RAHMANNU. Nasal encephalocele. Treatment by transcranial operation. J Neurol Sci 1979: 42: 73-85. 10. KAMERERDB, CAPAROSA RJ. Temporal bone encephalocele - diagnosis and treatment. Laryngoscope 1982: 92: 878882. 11. KEMINKJL, GRAHAM MD, KARTUSHJM. Spontaneous encephalocele of the temporal bone. Arch Otolaryngol Head Neck Surg 1986: 112: 558-561. 12. BRODSKY L. Spontaneous cerebrospinal fluid otorrhea and rhinorrhea coexisting in a patient with meningitis. Laryngoscope 1984: 94: 1351-1354. 13. PORADOWSKA W, LODZINSIUK, RESZKES, JAWORSKA M, LENKIEWICZ T, TRACZYNSKA-KUBIN H. Przepuklini oponowo-mozgowe przednie i heterotopie thanki nerwowej u dzieci. Pediatr Pol 1972: 48: 1359-1366. 14. BULLARD DE, CROCKARD HA, MCDONALD WI. Spontaneous cerebrospinal fluid rhinorrhea associated with dysplastic optic discs and a basal encephalocele: case report. J Neurosurg 1981: 54: 807-810. 15. GOLDHAMMER Y, SMITHJL. Optic nerve anomalies in basal encephalocele. Arch Ophthalmol 1975: 93: 115-1 18. 16. DEMPSEY PK, HARBAUGH RE. Encephalomeningocele presenting with spontaneous cerebrospinal fluid rhinorrhea in al elderly man: case report. Neurosurgery 1988: 23: 637-640. 17. FOCHEM K, KLUMAIR J, ZECHNER G. Meningoenzephalozele als Ursachen einer rhinogenen Liquorrhoe. Rontgenblatter 1983: 36: 96-98. 18. VAQUEROJ, CABEZUDO JM, SALAZARAR, BRASAJ. Symptomatic intrasphenoidal meningoencephalocele after removal of a parasagittal meningioma. Acta Neurochir (Wien) 1981: 57: 61-65. 19. LUYENDIJK W. Encephaloceles intranasales. A propos de 8 cas opiires. Neurochirurgie 1969: 15: 240-241. 20, RUSSEGGER L, FISCHER J, TWERDY K. Zur operativen Versorgung frontobasaler Defekte auf transkraniell subfrontalem Zugangswege. Zentralbl Neurochir 1988: 49: 290-297.
407
