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[Access to the anterior cranial fossa through the frontal sinus].

In 1998 to 2001, the Academician N. N. Burdenko Research Institute of Neurosurgery operated on 15 patients aged 20 to 65 years who had bulky processes in the base of the skull and paranasal sinuses by applying an access through the frontal sinus. Most patients (n = 7) had meningiomas. The others had osteoma of the base of anterior cranial fossa (n = 2), chondroma (n = 2), angiofibroma (n = 1), fibroma (n = 1), esthesioneuroepithelioma (n = 1), and neurinoma (n = 1). The main criteria for choosing this access were the site and extent of a process, the sizes of the frontal sinus, and no signs of acute or chronic rhinosinusitis. The length of frontal sinuses in the most measurement was 3.5 to 5.0 cm, their width was 7 to 10 cm. All patients showed no progression of neurological symptoms. Three patients had a decrease in visual acuity from 1.0 to 0.8; with vascular therapy, visual disorders regressed during 2 months. Olfaction became worse in 4 cases, there was anosmia in 2 cases. Nasal liquorrhea was not observed. When there were tumors in the anterior cranial fossa, an access through the anterior wall of the frontal sinus permits radical removal of both intra- and extracranial parts of a tumor with closure of the defect with a periostal flap.

Adult↗

Kallmann syndrome--a case report.

Kallmann syndrome is a very rare hereditary disease. It is characterized by hypogonadotropic hypogonadism in association with anosmia ot hyposmia, both of which occur as a result of the failure of neuronal migration of the luteinizing hormone releasing hormone (LHRH)--secreting neurons and the neurons of the vemeronasal nerve. It can be autosomal dominant, autosomal recessive, or X-linked mode of inheritance. We report a case of Kallmann syndrome that presented with delay puberty, color blindness, gynecomastia, and absence of smell. Plasma levels of LH, FSH and testosterone were very low. The patient's adrenal and thyroid hormone levels were normal. Chromosome analysis showed 46, XY karyotype without deletion in KAL gene (Xp22.3) from FISH. After 9 months of treatment by HCG and HMG, the amount of pubic hair and the volume of bilateral testes, as well as the level of testosterone had increased. Most importantly, motile sperm count be found in semen.

Adult↗

[Giant anterior communicating artery aneurysm (author's transl)].

A case of giant aneurysm arising from the anterior communicating artery, 24 X 28 X 30 mm in diameter was found in a 30 year old man. About ten years ago he became blind and recently developed right anosmia and diencephalic seizures. No subarachnoid hemorrhage, however, was found. Radiograms and tomograms of the cranium showed a ring-like calcification, but by angiography it couldn't be recognized as a giant aneurysm. The right frontal craniotomy and partial resection, therefore, was performed. A histological study of the resected material revealed that it was a spontaneously thrombosed giant aneurysm. The inner layer of its wall had neither endothelium nor elastic lamina, but had deposits of calcium salt. The outer layer was composed of collagen fibers without cell infiltration. The aneurysm was thrombosed except for its neck but its organization occurred incompletely. We want to emphasize the importance of a correct preoperative diagnosis, as an erroneous operative procedure can result in disaster. Volume, viscosity and tension of flowing blood into the aneurysm as well as the size of its neck and dome regulate dynamic properties. These properties may determine the enlargement rate or growth of the aneurysm. The dynamic characteristics and features of the inner surface of the aneurysmal wall may regulate the formation of thrombosis in the aneurysm. The intraluminal thrombosis and strength of aneurysmal wall, for example, calcium deposits, may prohibit aneurysm from its rupture.

Adult↗

[A case of arteriovenous communication between external and internal carotid arteries and the sinus after removal of the meningioma (author's transl)].

A 60-year-old man was admitted with anosmia, memory disturbance and disorientation in 1971. He was diagnosed the left olfactory groove meningioma and received the removal of the tumor after ligation of the left external carotid artery. Over the following two years he was admitted again with progressive memory disturbance and mental disorder in Aug. '73. Physical examination disclosed no abnormalities except for slight hypertention. His CSF pressure was 120 mm H2O and pneumoencephalograms showed marked dilatation of the lateral ventricles. Right carotid angiography revealed the right olfactory groove meningioma which was receiving a rich circulation from the branches of right ophthalmic artery. And right selective external arteriography showed an arteriovenous communication between the occipital artery and the transverse sinus. Contrast medium flowed to the left transverse sinus. Left internal carotid arteriography showed an anomalous arteriovenous communication between the tentorial branch (Bernasconi-Cassinari artery) of the internal carotid artery and sigmoid sinus. Further left vertebral arteriography revealed another arteriovenous communication between the left occipital artery anastomosed with vertebral artery and the transverse sinus. An operation for the right olfactory groove meningioma was made in Sep. '73. Some considerations on the pathogenesis of developement of these arteriovenous communications were discussed.

Arteriovenous Fistula↗

Rare cause for short stature--Kallmann's syndrome--a case report.

Kallmann's syndrome is a neuroendocrine disorder, characterized by hypogonadotropic hypogonadism with hyposmia. We report a 27 year old male who presented with short stature and pain in the lumbar region. On detailed evaluation he had growth retardation, features of hypothalamic hypogonadism as evidenced by endocrimological tests and anosmia since birth. He had co-existent caries spine T10,T11 causing pain in the lumbar region-MRI brain showed normal olfactory pathway while the response to nasal stimulants was markedly attenuated. We present this case for its very care occurrence in the eastern part of the world and the typical feature being normal MRI brain with functional defect of the olfactory pathway.

Adolescent↗

[Ewing's sarcoma of the head and neck: a case report].

We report a case of Ewing's sarcoma located in the ethmoid sinus. The patient complained of facial pain, anosmia and visual defect. Diagnosis was established at microscopic examination with histoimmunochemistry and molecular biology. Treatment combined chemotherapy and surgical resection with skull base reconstruction and post-operative ratio and chemotherapy.

12E7 Antigen↗

[Chronic and predominantly sensory polyneuropathy in Toroku Valley where a mining company produced arsenic].

We report 9 patients (5 males, 4 females, ages 63-77) with chronic polyneuropathy. They were exposed to arsenic for about 15-40 years in Toroku Valley, Takachiho-Town, Miyazaki Prefecture, Japan, where a mining company produced arsenic from 1920-1962. Predominantly sensory polyneuropathy was the most significant neurological finding. In 5 of them, superficial and deep sensation was equally affected over the entire body, including head, face, and mucous membranes of the mouth. The corneal reflexes were absent or sluggish. Pin-prick and light-touch perception was absent in their hands and legs. Another sensory disorder such as glove and stocking-type was seen in 4 of them. All 9 patients were comfortable with extremely hot water in their beverages, their baths, and their wash basins compared with controls. But these patients felt that their temperature sensation was normal. Motor involvement was minimal. Although motor-nerve conduction velocities were normal or reduced minimally, sensory-nerve conduction velocities were moderately reduced. Sural-nerve biopsy revealed reduction of both small myelinated and unmyelinated fibers, which occurs with axonal degeneration of peripheral nerves. Other main symptoms and signs were tension-type headaches, non-painful tonic spasms of limbs, and losses or significantly decreased sensations of taste and smell. Dantrolene-sodium was effective for the treatment of their non-painful tonic spasms of limbs. As for the general medical condition of these patients, all of them had suffered from chronic bronchitis and skin eruption during childhood or in their early life or in their young adulthood when the mine was producing arsenic. At the time of this study only four of them suffered from chronic bronchitis and none of them had skin eruptions or discoloration of the skin, 37 years after closure of the mine. These 9 patients were diagnosed as having chronic arsenic poisoning and arsenic polyneuropathy. The multiplicity of symptoms found in these patients--anosmia, ageusia, chronic bronchitis, tension-type headaches, and non-painful tonic spasms of limbs--differs from the symptoms previously reported cases with arsenic poisoning. This may be due to combined overexposure to environmentally hazardous arsenic by effluent gas, dust, and drinking water in Toroku Valley. As concerns the sensory disorders described above, it is necessary to investigate sensory conduction pathways, including cerebral sensory cortex, spinal cord, and skin sensory receptors such as Pacini corpuscles, and Meissner corpuscles. Unfortunately, no information has been obtained about this problem because there were no autopsy cases.

Aged↗

[The subcranial approach for tumors involving the anterior skull base].

The technical approach for anterior skull base tumors has progressed considerably during the last decade. In the classical combined craniofacial resection (CFR) technique, anterior skull base lesions have been approached through bi-frontal craniotomy coupled with Weber-Ferguson and related modifications incisions. The purpose of this work is to present our experience with the subcranial approach for treatment of anterior skull base tumors. During the years 1994-2002, 64 patients underwent 72 procedures of anterior skull base tumor resection via the subcranial approach. The ages of the patients ranged from 2 to 81 years (mean 42 years). Twenty-nine cases involved malignant tumors (40%), and 43 cases involved benign tumors (60%). The most common benign pathology was meningioma (n = 12) and the most common malignant tumor was squamous cell carcinoma (n = 8). The principle skull base reconstruction procedure was performed using a multi-layered fascia: limited defects of the dura were reconstructed with the use of temporalis fascia, whereas reconstruction of large dural defects was performed using a multi-layered fascia lata sheath. Reconstruction of significant bony defects was achieved by utilizing a split calvarial bone graft, posterior sinus wall, or titanium mash covered with pericranial flap. Postoperative follow-up (26 months in average) revealed that 76% of the patients are without evidence of disease, 14% are alive with disease, 3% died of their diseases and 7% died of unrelated causes. Twenty-seven patients (44%) have suffered from anosmia following the operation. The rate of severe complications was 5.6%, and included meningitis (n = 2), cerebrospinal fluid rhinorrhea (n = 1) and tension pneumocephalus (n = 1). We conclude that the extirpation of anterior skull base tumors via the subcranial approach is simple, reproducible and reliable, and is associated with reasonable complication rates.

Adolescent↗

Endoscopic surgical treatment of sinonasal polyposis-medium term outcomes (mean follow-up of 5 years).

BACKGROUND: Between 30 and 50% of patients with sinonasal polyposis (SNP) will require surgical treatment. OBJECTIVE: To determine the medium term risks and benefits of endonasal ethmoidectomy in SNP. METHOD: 132 cases of SNP have been retrospectively studied before and after endoscopic nasal surgery with a mean follow-up of 5 years. Functional symptoms and appearances on endoscopic examination were recorded on graded scales. Non-parametric statistical analyses for matched series were used in the assessment of data. RESULTS: Post-operative normalisation of nasal function was obtained in 62% of cases. Each of the four main nasal symptoms (nasal obstruction, rhinorrhoea, facial pain and anosmia) was improved (p = 0.001). Patients with associated asthma or ASA triad had higher scores for nasal obstruction and rhinorrhoea (Fisher's exact test; p < 0.02). The pre-operative clinical stage of SNP correlated neither with recurrence nor with functional outcomes (Fisher's exact test; p > 0.05). The requirement for steroid treatment of concurrent asthma was also reduced post-operatively (p = 0.001). The association with asthma and its level of treatment was not related to the frequency of recurrence of SNP (Chi2; p > 0.6). CONCLUSIONS: Functional outcomes for patients with SNP and asthma were significantly improved by endoscopic surgical treatment, even if the appearance of the nasal mucosa did not always return to normal.

Asthma↗

Medical findings in nickel-cadmium battery workers.

Thirty-eight workers from a factory producing nickel-cadmium and other types of batteries came to us for medical evaluation. They included 21 women and 17 men (seniority 2-20 years, age range 31-63 years), and represented a self-selected subset of 700-900 ever-employed and 200+ recently or currently employed workers in the factory. Thirty-four worked on the nickel-cadmium assembly line. Symptoms and signs included: headache in 34; weakness, fatigue and lassitude in 26; dizziness in 16; pruritus and skin eruptions in 37; gingivitis, teeth loss and caries in 34; nasal congestion, nosebleeds and anosmia in 30; cough, phlegm production, wheezing and shortness of breath in 26; "asthma" in 14; bone pain in 18; urinary frequency, beta 2 microglobulinuria and kidney stones in 17; and sterility or multiple abortions (33) in 8 of 21 women. One additional patient had died from an "amyotrophic lateral sclerosis-like syndrome", while CT scans in six workers revealed brain atrophy. One other worker had leukemia, and two had died from cancer (lung and pancreas). Those who had worked for more than 10 years had more symptoms and signs than shorter-term employees, especially neurological illness, bone pain and urinary tract problems, including beta 2 microglobulinuria. Past blood and urinary cadmium levels were in the range of 1.6-8.7 micrograms/dl and 8-306 micrograms/l, respectively. Our findings indicated that: a) health risks for workers were not confined to the nickel-cadmium assembly line or to older workers, b) hazardous exposures still existed and illness appeared in new workers after a clean-up and intervention program, and c) exposures involved increased risks for renal disease and cancers. Finally, there is a need to control exposures and determine health risks in the full cohort of those ever employed, in the workers' children, and in the surrounding environment (air, ground, water) due to the dumping of waste from the plant.

Adult↗

[A case of subfrontal schwannoma].

We encountered a rare case of subfrontal schwannoma. A 55-year-old woman had received resection of a left frontal tumor because of hyposmia, at the age of 28 years. On June 10, 1989, she was admitted with the chief complaint of progressive contraction of visual field. Neurologic examination revealed anosmia, impaired vision and concentric contraction of visual field. Fundoscopy showed optic atrophy. CT examination demonstrated a calcified mass of mixed density which was occupying her nasal cavity, ethmoid sinus and anterior skull base. The lesion was enhanced with contrast medium. MRI clearly depicted the extension of the lesion and a low signal intensity area in the left frontal lobe as a postoperative scar. Angiography showed hypovascularity. The tumor was totally removed by bifrontal craniotomy on August 22, 1989. Infiltration into the brain or compression of the optic nerve was not detected. The dura on the cranial base side was damaged and lost by infiltration of the tumor, normal olfactory bulb was not able to be identified, and the cribriform plate was broken. The anterior skull base was reconstituted by covering the dural defect with cadaveric dura and the bony defect with a pericranium. HE staining showed Antoni A&B types of schwannoma. Postoperative course was uneventful. In this case, it is most likely that a remnant of the tumor resected when she was 28 years old had developed subfrontal schwannoma a long time after the operation, although the histological type at that time was unknown. It is also possible that a primary tumor in the nasal cavity or paranasal sinus may have extended into the cranium.(ABSTRACT TRUNCATED AT 250 WORDS)

Craniotomy↗

[Nasal polyposis: long term functional results in 203 patients treated by ethmoidectomy combined with intranasal corticotherapy].

OBJECTIVE: Evaluate the long term quality of life in patients after ethmoidectomy associated with intranasal corticotherapy for nasal polyposis on the appreciation of the intensity of nasal symptoms. MATERIAL ET METHOD: The authors report their experience about 203 patients treated by endonasal endoscopic ethmoïdectomy intranasal followed by a long term intransal corticotherapy, with a mean follow up of six years. In this prospective study, each symptom was evaluated using an analogic visual scale and a questionnaire. The evolution of asthma after surgery and the patients global satisfactory rate were noted. RESULTS: There is a global improvement of the nasal symptoms with a mean postoperative individual scores calculated at 32% for nasal obstruction, at 34% for rhinorrhea and at 51% for anosmia. No impact on asthma or improvement of asthma was encountered in 92% of the patients. The patients global satisfactory rate of this medico-surgical approach of the disease is 93.6%. A positive correlation was found between the severity of the olfactory disorders and the oral corticosteroid therapy. CONCLUSION: Endoscopic endonasal ethmoïdectomy followed by intranasal corticotherapy represents a valuable protocol in treating patients with severe nasal polyposis.

Adolescent↗

Disturbance of social cognition after traumatic orbitofrontal brain injury.

A patient with traumatic orbitomedial frontal lobe damage demonstrated good neurocognitive recovery but a lasting, profound disturbance of emotional regulation and social cognition. Initial neuropsychological findings included a complete anosmia, mildly reduced fluency and disturbed motor regulation. The impairments of fluency and motor regulation resolved, and formal measures of "frontal lobe" functioning were generally intact. However, she remained impaired on tasks requiring the interpretation of social situations, which mirrored her impairment in real life functioning. This disturbance in social cognition appeared related to difficulty appreciating and integrating the relatively subtle social and emotional cues required for the appropriate interpretation of events. The patient's presentation represents an intermediate position between patients with profound neurobehavioral deficits and patients with impaired real-life social cognition despite intact neuropsychological performance following orbitofrontal damage. Variations in the orbitofrontal behavioral syndrome may be related to extent of lesion, time post injury and the course of recovery in different patients.

Journal Article↗

The orbitomedial frontal syndrome.

An orbitomedial frontal syndrome is proposed, characterized by anosmia, amnesia with confabulation, Go-NoGo deficits, personality change, and hypersensitivity to pain. The orbitomedial frontal syndrome is distinct from the clinical picture that results from dorsolateral frontal damage. Aspects of orbitomedial damage have been discussed previously in isolation, but we argue that recognition of this syndrome in toto is clinically important. It appears to be associated with poor social and vocational adjustment after brain injury, and the co-occurrence of features of the syndrome provides clues to underlying mechanisms for disinhibition and confabulation in frontal lobe patients.

Journal Article↗

[Long-term outcomes and analysis of its relative factors of nasal endoscopic sinus surgery in children].

OBJECTIVE: To evaluate the results of endoscopic sinus surgery (ESS) in children with chronic sinusitis. The long-term outcomes and the relative factors were analyzed retrospectively. MATERIALS AND METHODS: A total of 268 children (432 sides), 186 males and 82 females, aged 3 to 17 years (average, 14.3) were included in this study (underwent ESS). The postoperative follow-up time of 188 cases (305 sides, 70.1%) was over one year. The management during follow-up was adopted under nasal endoscope and local anesthesia. The postoperative treatment included cleaning operative cavity, nasal irrigation, antibiotics, steroid, immune regulator, etc. RESULTS: Overall cases included 89 cases (147 sides) of chronic sinusitis, 86 cases (142 sides) of nasal polyps and 13 cases (16 sides) of mucocele respectively. The cure rate was 70.2% (132 cases). 43 cases (22.9%) were improved, while other 13 cases were of no effect. Overall symptoms, 93.1% children with headache were improved, 85.1% children were free of nasal obstruction and 60.1% children gained a relief of purulent discharge. 11 cases had no change and 2 cases were worse compared with before surgery. 34 cases with anosmia were improved or recovered after ESS. The total effective rate of ESS was 93.1%. 8 cases received a revision ESS. CONCLUSIONS: ESS in children with chronic sinusitis and polyps has a good long-term outcomes. The reasonable reservation of mucosa during ESS must be emphasized. The quality of follow-up is an important factor affecting the results of ESS. Whether the disciplinary preoperative management was made or not is the hypothesis of ESS indications.

Adolescent↗

Olfaction in patients with nasal polyposis: effects of systemic steroids and radical ethmoidectomy with middle turbinate resection (nasalization).

AIM: In this prospective study the effect of medical and surgical treatment on subjective olfaction was studied in patients with nasal polyposis (NPS). The effects on nasal obstruction, anterior and posterior rhinorrhea, sneezing and itching are reported in another article in this issue. PATIENTS AND METHODS: Protocol 1. Twenty-four patients with NPS who complained about anosmia were treated with a 7-days course of systemic steroids. Their subjective overall sense of smell was determined with a visual analog scale (VAS) before treatment, immediately after treatment, and two months later. Subsequently all patients underwent surgery bilaterally according to the nasalization principles. The sense of smell was re-evaluated at 1, 3, 6, 9, and 12 months postoperatively. Protocol 2. Thirty-two patients with NPS not responding to medical therapy who, for different reasons, did not receive oral steroid treatment, received surgery only and were followed up during one year after nasalization. Of these patients, 25 were anosmic and 7 normosmic. RESULTS: Protocol 1. Following the 7-day treatment with systemic steroids the olfactory score increased significantly. During the waiting time for surgery (64 +/- 39 days) this score deteriorated again in a significant way. One month after nasalization which included a depot injection of triamcinolone 80 mg the day after surgery, the olfactory score ameliorated again and remained stable at 3, 6, 9, and 12 months. None of the patients reported any intake of systemic steroids during the one-year of follow-up. Statistically, there was a trend suggesting that the 12 month post-nasalization score was better than the immediate post-oral steroid score. A good correlation between the improvement of the sense of smell after 7 days of systemic steroids and one year after nasalization was found. Protocol 2 One month after the nasalization protocol, olfaction in patients of the hypo-anosmic group had improved considerably. Scores at 3, 6, 9, and 12 remained very stable. The sense of smell in the normosmic group did not change after surgery and remained stable during the year of follow-up. In total, 49 patients with a severe loss of smell showed a significant improvement at 12 months after surgery. CONCLUSION: The present study shows that 1) long-lasting correction of olfactory dysfunction produced by nasal polyposis can be achieved through the combination of nasalization and low dose of nasal steroids, 2) middle turbinate resection does not alter the possibilities to restore the sense of smell.

Adolescent↗

[Kallmann's syndrome in a child--a case report].

The patient was a male child 6 years and 6 months old. He visited our hospital because of bilateral cryptorchism and true phimosis. Since he was found to have fairly small testis, micropenis, and anosmia, endocrinological examination was performed. The plasma levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) were at the lower limit of normal values. LH gave a low reaction to the LH-releasing hormone test, while the reaction of FSH was near the lower limit of normal values. In the human chorionic gonadotropin test, there was no rise in the plasma levels of testosterone. Nor was there any reaction to the venous olfactory test with Alinamine (thiamine propyldisulfide). Accordingly, the patient was diagnosed as having Kallmann's syndrome. A search of the literature reveals few reports that deal with Kallmann's syndrome in infancy. Early diagnosis seems to be of significance, however, to institute early appropriate treatment to prompt the development of proper secondary sexual characteristics and ensure normal future fertility.

Child↗