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A sphenoid mucocele often presents late due to its deep seated anatomical site. And it has varied presentation due to its loose relationship to the cavernous sinus and the base of the skull. We describe a case of large sphenoid sinus mucocele. A middle aged old man suddenly developed third cranial nerve palsy. Brain imaging study revealed an isolated sphenoid sinus mucocele, compressing right cavernous sinus. Endoscopic marsupialization of the mucocele via transnasal approach led to complete resolution of the third cranial nerve palsy. Involvement of the third cranial nerve in isolated mucocele is rare but important neurosurgical implications which must be excluded. In addition, proper and timely treatment must be performed to avoid permanent neurologic deficit.

Background

Solitary involvement of the sphenoid sinus has rarely been reported in non-Hodgkin's lymphoma. Isolated oculomotor nerve palsy is uncommon as an initial presentation of malignant tumors of the sphenoid sinus.

Case presentation

A 53-year-old woman presented with a three-month history of headache and diplopia. Neurological examination revealed complete left oculomotor nerve palsy. Magnetic Resonance Imaging (MRI) demonstrated a homogenous soft-tissue lesion occupying the left sphenoid sinus and invading the left cavernous sinus. The patient underwent transsphenoidal biopsy and the lesion was histologically diagnosed as non-Hodgkin's lymphoma, diffuse large B-cell type. Tumor cells were positive for CD20 and negative for CD3. Following six cycles of chemotherapy, the left oculomotor nerve palsy that had been previously observed was completely resolved. There was no enhancing lesion noted on follow-up MRI.

Conclusion

It is important to recognize that non-Hodgkin's lymphoma of the sphenoid sinus can present with isolated oculomotor nerve palsy, although it is extremely rare. The cranial nerve deficits can resolve dramatically after chemotherapy.

A 58-year-old man presented with a six-month history of intermittent blood-stained posterior nasal discharge. Five years ago, he had a three-week episode of fitful light headaches. Nasal ventilation, olfactory sensation, and facial sensation were normal; there were no ophthalmological complaints. Coronal computed tomography (CT) scans revealed soft masses in the bilateral sphenoid sinuses with bone absorption. The patient underwent bilateral functional endoscopic sinus surgery and resection of right nasal papillary masses. Papillary masses and mucosa in both sphenoid sinuses were also removed. The mass in the left sphenoid sinus was diagnosed as two separate entities, one being a primary monophasic epithelial synovial sarcoma and the other an inverted papilloma, while the mass in the right sphenoid sinus was an inverted papilloma. After surgery, the patient underwent radiotherapy and chemotherapy. At the 50-month follow-up visit, there were no signs of recurrence.

Inflammatory pseudotumor most commonly occurs in the orbit and produces orbital pseudotumor, but extension into brain parenchyma is uncommon. We report a case of inflammatory pseudotumor involving sphenoid sinus, cavernous sinus, superior orbital fissure, orbital muscle, and intracranial extension into left temporal lobe producing right hemiparesis and wernicke's aphasia. The patient improved clinically and radiologically with steroid administration. This paper provides an insight into the spectrum of involvement of inflammatory pseudotumor and the importance of early diagnosis of the benign condition.

We report an uncommon case of a 45-year-old woman who presented with spontaneous rhinorrhea. A computed tomography (CT) scan of the head revealed an abnormally large sphenoid sinus associated with a parasellar bony defect (Sternberg's canal) through which magnetic resonance imaging could detect an encephalocele of the right temporal lobe. An endoscope-assisted trans-sphenoidal approach was performed and, with the aid of image guided surgery, reduction of the encephalocele was obtained and followed by surgical repair of the dural and bony defects. The postoperative course was uneventful and the cerebrospinal fluid fistula was closed as confirmed by the postoperative CT scan and by the absence of rhinorrhea. After three years of monitoring the patient remained asymptomatic.

ABSTRACT

Cerebrospinal fluid (CSF) leaks result from a communication between the subarachnoid space and the upper aerodigestive tract. Because of the risk of complications such as meningitis, brain abscess, and pneumocephalus, all persistent CSF leaks should be repaired. Surgical repair may be achieved transcranially or extracranially using a wide variety of autogenous, allogenic, and synthetic patching materials. We report our results with a transnasal transsphenoidal endoscopic approach for the repair of CSF leaks coupled with a multilayer closure using acellular dermis (Alloderm™). We conducted a retrospective review of all patients presenting to our institution over the past 5 years with isolated sphenoid sinus CSF fistulas. Results: Twenty-one patients were included in the study. Nineteen patients (90.5%) had their sphenoid sinus CSF fistula repaired during the first attempt; 2 patients (9.5%) needed a second attempt. The multilayer repair of the CSF leak using acellular dermis via a transsphenoidal endoscopic approach is an effective and successful method of surgical repair of the fistula site. Neither the number, size, nor cause of the CSF fistula affected surgical outcomes. However, the presence of hydrocephalus was a significant negative variable, altering the surgical outcomes of our patients. The acellular dermis offers the advantage of not requiring autogenous tissue for the effective repair of CSF leaks in the sphenoid sinus.

Painful ophthalmoplegia due to extramedullary plasmacytoma is a rare initial manifestation of multiple myeloma. The present report describes a 48-year-old man who suffered an acute onset of retro-orbital pain, left abducens palsy and left facial hypoesthesia. In addition, he exhibited an elevated erythrocyte sedimentation rate and partial responsiveness to corticosteroid treatment, all of which resemble the features of Tolosa–Hunt syndrome. Imaging studies revealed a multilobulated tumour invading the left sphenoid bone and sphenoid sinus, later confirmed as a plasmacytoma at pathology. Multiple myeloma was also diagnosed by bone marrow examination. After completion of chemotherapy and radiotherapy, the patient has been free of symptoms for 10 months. Although cranial neuropathies with any combination of oculomotor, abducens, trochlear, ophthalmic and maxillary nerves may indicate a cavernous sinus lesion, neuropathies exclusive to the abducens and maxillary nerves may raise the possibility of extracavernous sinus origin. Cranial imaging is crucial in diagnosing painful ophthalmoplegia with additional minimal cranial nerve signs.

The purpose of this case report is to familiarize the sinus surgeon with the possibility of the rapid development of internal carotid artery aneuryams from fungal infections of the sphenoid sinuses. A renal dialysis patient with progressive loss of vision was treated with high doses of steroids for the presumed diagnosis of temporal arteritis. Subsequent work-up included a magnetic resonance arteriogram (MRA) and computed tomography (CT) with contrast that failed to demonstrate aneurysmal changes of the carotid arteries but suggested the presence of a mycotic infection of the sphenoid sinuses. During a sphenoidotomy two days later, in addition to the anticipated aspergillus infection of the sinuses, an aneurysm extending from the left internal carotid artery into the sphenoid sinus was encountered. An emergency arteriogram immediately following the surgery revealed a second newly developed large mycotic aneurysm of the right internal carotid artery filling the right sphenoid sinus as well. This case report documents the rapidity with which mycotic aneurysms can develop from a sphenoid sinus infection secondary to aspergillosis in an immunocompromised host.

Images

We present the case of a rare sinonasal tumour originating in a rare location in close proximity to vital structures. We describe how the patient was managed, including detailed description of how the mass was removed.

A 54-year-old man presented with a long history of nasal blockage, recently complicated by severe headaches and a left VIth nerve palsy. CT scan showed complete opacification of the left sphenoid sinus.

Symptoms improved following administration of steroids and nasal decongestants. Histology from endoscopic debulking of the mass confirmed the presence of inverted papilloma. Fine-cut CT showed the course of the carotid artery and optic nerve, closely related to the tumour, allowing for complete removal during a second endoscopic procedure. Five years following the procedure, the patient remains symptom free.

With appropriate imaging, isolated sphenoid sinus tumours can be removed endoscopically even when in close relation to vital structures.

Cholesterol granuloma (CG) is usually associated with chronic middle ear disease, and is not common in the paranasal sinuses. Additionally, it is very rare for cases of CG to be associated with a fungal infection. However, in this paper, we report a case of sphenoid sinus CG that is associated with aspergilloma in a 78-year-old male patient who presented with right hemifacial pain, headache and toothache. CT revealed the presence of an expansile cystic mass lesion in the sphenoid sinus that showed a high signal intensity on both the T1 and T2 weighted images. This mass was later determined to be CG. The suspected etiologic mechanisms of both CG and aspergilloma of the paranasal sinuses are similar, and impaired drainage and obstruction of the ventilation of the paranasal sinuses are considered to be the causative mechanism of both diseases. Overall, the results of this study indicate that the use of MRI findings could be helpful for differentiating CG from other paranasal sinus mass lesions.

Solitary extramedullary plasmacytomas are isolated plasma cell tumors of soft tissue that typically do not metastasize. They are rare and account for 4% of all plasma cell tumors. To our knowledge, only 14 cases of solitary extramedullary plasmacytomas in the sphenoid sinus have been reported. A 32-year-old man presented to our department with complaint of ocular pain in the right eyeball and diplopia. Physical and neurological examinations revealed intact and prompt direct and indirect light reflexes in both pupils and limitation of extraocular muscle movement seen with the lateral gaze of the right eyeball. Magnetic resonance imaging suggested the presence of mucocele or mycetoma, therefore surgical resection was performed with endoscopic endonasal transsphenoidal approach. Histopathology was consistent with plasmacytoma. Systemic work-up did not show any evidence of metastasis and the sphenoid sinus was the sole tumor site, and therefore the diagnosis of solitary extramedullary plasmacytoma was confirmed. We report a rare case of solitary extramedullary plasmacytoma in the sphenoid sinus with successful treatment using the endoscopic endonasal transsphenoidal resection and adjuvant radiotherapy.

Lateral sphenoid encephalocele, especially within the lateral aspect of the sphenoid sinus, when the sphenoid sinus has pneumatized extensively into the pterygoid recess, are considered exceedingly rare. We report a rare case of lateral intrasphenoidal encephalocele with spontaneous cerebral spinal fluid (CSF) rhinorrhea. Computed tomography demonstrated bilateral arachnoid pit, extensive sphenoid sinus pneumatization, and a defect in the superior wall of the left lateral recess of the sphenoid sinus. Magnetic resonance imaging demonstrated anteromedial temporal lobe herniating through the bony defect.

Background:

Giant cell reparative granulomas (GCRGs) are rare lesions in the cranial bones. We present a case of this rare lesion emanating from the clivus and replacing the sphenoid sinus, a highly unusual location for this entity.

Case Description:

The case and clinical course of a 29-year-old female who presented with a large sphenoid mass are described here. The patient presented with symptoms of severe headache and diplopia; imaging demonstrated a large sphenoid mass which was completely resected via an endoscopic endonasal approach. It was based on the clivus and was shown to be a GCRG.

Conclusion:

GCRGs are benign granulomatous lesions which should be considered in the differential in the setting of a sphenoid mass.

Ophthalmoplegia due to 3rd nerve palsy is a common occurrence, and is usually a sign of diabetes mellitus or a serious intracranial disease. We report a rare case of pupil sparing 3rd nerve palsy caused by mucocele of the sphenoid sinus. The patient regained 3rd nerve functions after successful decompression of the mucocele. Early and correct diagnosis of this relatively benign condition is important to prevent permanent neurological deficits, including visual loss by optic nerve atrophy. Etiology, clinical manifestations and treatment of the sphenoid mucoceles is discussed and available literature is reviewed.

Notes were reviewed for 68 patients with brain abscess diagnosed at Auckland Hospital, Auckland, New Zealand between 1978 and 1988. Mean age was 30 years (range one week to 74 years). There were 48 men and 40% were Maori or Pacific Island Polynesians. Seventy-two per cent of patients had headache, 54% had fever and 72% had lateralizing neurological signs. Thirty-one per cent of abscesses were associated with contiguous infection (otic, sinus, dental). Forty-four per cent were in the frontal lobe. Two abscesses were sterile; 197 bacterial isolates were cultured from the remainder. Fifty-four per cent contained obligate anaerobes, which were the only isolates in 22%. Streptococcus anginosus was the single most common isolate present in 22% of the abscesses. Amoxycillin plus metronidazole provided cover for approximately 95% of the total isolates on the basis of sensitivity testing. Treatment was with surgery and antibiotics in all but three patients, who were cured with antibiotics alone. Sixty per cent had a definitive regimen of penicillin (or ampicillin/amoxycillin) and/or metronidazole, always intravenous initially but subsequently often orally. Median duration of antibiotic treatment was 57 days (range 28 to 206). Seventy-five per cent had initial aspiration, 9% open drainage and 7% were excised initially. Seventy-one per cent had a good functional outcome. Mortality was 8.8%. Factors associated with a poor outcome were trauma as a cause, and delays after admission of more than seven days to diagnosis and/or operation.

A basal encephalocele often shows an insidious clinical course. Only two cases of temporal lobe encephalocele accompanied with tension pneumocephalus have previously been reported. In this paper, we describe a case of lateral sphenoid sinus encephalocele presenting with intraventricular tension pneumocephalus. A 54-year-old man was referred to our institution presenting with intraventricular tension pneumocephalus. He had undergone ventriculoperitoneal shunt placement for postmeningitis hydrocephalus 3 months before this admission. Precise imaging examinations detected evidence suggestive of a lateral sphenoidal sinus recess encephalocele. Endoscopic transnasal approach was performed for surgical repair of the encephalocele. The encephalocele was removed with subsequent repair of the bony defect. Histological examination showed that the encephalocele includes a part of the ventricular system. This indicates that air might enter directly into the ventricular system after rupture of the temporal lobe encephalocele. A lateral sphenoid sinus encephalocele would potentially cause intraventricular tension pneumocephalus, although pneumocephalus is an extremely unusual complication of this type of basal encephaloceles.

ABSTRACT

Trans-sphenoid anterobasal temporal lobe meningoceles are rare and can be associated with temporal lobe epilepsy or recurrent meningitis. Surgical treatment is described via complicated infratemporal or intracranial approaches with high morbidity. A 32-year-old man presented with an 18-year history of two types of seizures and confirmed epileptic activity in electroencephalogram. A trans-sphenoid meningocele in the left pterygopalatine fossa was found on computed tomography and magnetic resonance imaging. A midfacial degloving was chosen instead of a large neurosurgical approach to remove it and to repair the dura defect with lyophilized dura, collagen, and abdominal fat. The postoperative course was uneventful without visible scars and the patient remains free of seizures without antiepileptic medication 3 years after surgery. Although midfacial degloving is not described yet as a treatment of trans-sphenoid meningoceles, this approach proved to be fast, safe, effective, and reliable and should be considered as the only alternative to large external approaches.

A case of painful ophthalmoplegia associated with an extensive lesion involving the sella turcica, superior orbital fissure, and sphenoid sinus in a 57-year-old man is reported. Even though nasal and ocular symptoms and signs represent the usual features of sphenoidal mucoceles, extension to the intracranial cavity as seen in this lesion is rare. Surgical exploration via a sublabial, transseptal approach revealed a mucocele of the sphenoid sinus. This case exhibited extensive and aggressive behavior simulating a malignant neoplasm.

Images

We present a case (55-year-old) with a metastasis to the sphenoid sinus from an occult prostatic adenocarcinoma as the first evidence of disease. The patient’s symptoms were progressive left-sided headache of a few months duration and a 2-week history of blurred vision with diplopia. Clinical examination revealed a left cranial nerve III palsy and ptosis. A computed tomography scan showed a 2 cm large, well delineated soft tissue lesion involving the left sphenoid sinus and extending to the left cavernous sinus with sclerosis of the adjacent sphenoid bone. Histological and immunohistochemical examinations revealed a poorly differentiated adenocarcinoma that was positive for prostate specific antigen (PSA) and broad-spectrum cytokeratins (Cam 5.2). Subsequent urological examination confirmed a locally advanced prostatic carcinoma with significantly increased serum-PSA (4,561 μg/l) and bone scintigraphy showed widespread metastasis.

Temple trauma that appears initially localized to the skin might possess intracranial complications. Early diagnosis and management of such complications are important, to avoid neurologic sequelae. Non-penetrating head injuries with intracranial hemorrhage caused by a driven bone fragment are extremely rare. A 53-year-old male was referred to our hospital because of intracerebral hemorrhage. He was a mechanic and one day before admission to a local clinic, tip of metallic rod hit his right temple while cutting the rod. Initial brain computed tomography (CT) and magnetic resonance imaging demonstrated scanty subdural hematoma at right temporal lobe and left falx and intracerebral hematoma at both frontal lobes. Facial CT with 3-D reconstruction images showed a small bony defect at the right sphenoid bone's greater wing and a small bone fragment at the left frontal lobe, crossing the falx. We present the unusual case of a temple trauma patient in whom a sphenoid bone fragment migrated from its origin upward, to the contralateral frontal lobe, producing hematoma along its trajectory.

ABSTRACT

Cerebral abscesses are rare but often lethal complications of invasive Aspergillus sinusitis. Treatment is difficult and usually depends on a combination of neurosurgical drainage and intravenous antifungal therapy. We report a case of intracerebral frontal abscess in continuity with the anterior skull base in a 53-year-old immunocompromised female with invasive rhino-orbital aspergillosis. An aperture was created by drilling the anterior skull base during endonasal sinus surgery, and the abscess was drained through its lower pole. The aperture was left open after surgery to ensure complete abscess drainage. Follow-up at 8 months revealed no cerebrospinal fluid leak or meningitis, and no abscess recurrence. This case validates the feasibility of endonasal trans-ethmoidal drainage of intracranial abscesses.

Streptococcus pneumonia and Haemophilus influenzae account for more than 50% of bacterial acute sinusitis. Isolated sphenoid sinusitis is a rare disease with potentially devastating complications such as cranial nerve involvement, brain abscess, and meningitis. It occurs at an incidence of about 2.7% of all sinus infections. There have been no previous reported cases of unilateral sphenoid sinusitis presenting as septicaemia in an otherwise healthy young immunocompetent adult.

Background:

Intrasphenoidal encephaloceles are extremely rare findings. Sternberg's canal is a lateral craniopharyngeal canal resulting from incomplete fusion of the greater wings of the sphenoid bone with the basisphenoid. It acts as a weak spot of the skull base, which may lead to develop a temporal lobe encephalocele protruding into the lateral recess of the sphenoid sinus (SS).

Case Description:

We present two cases of intrasphenoidal encephalocele due to persistence of the lateral craniopharyngeal canal. The first case presented with cerebrospinal fluid (CSF) rhinorrhea and the second one was referred to the neurosurgical department with CSF rhinorrhea and meningitis. Radiological investigations consisted of computed tomography (CT) scan, CT cisternography and magnetic resonance images in both cases. These imaging studies identified a herniated temporal lobe through a bony defect which communicates the middle cranial fossa with the lateral recess of the SS. Both patients underwent a transcranial repair of the encephalocele because of the previous failure of the endoscopic surgery. There was no complication related to the surgical procedure and no recurrence of CSF leakage occurred 2 and 3 years after surgery, respectively.

Conclusion:

Encephalocele within the lateral recess of the SS is a rare entity which must be suspected in patients who present with spontaneous CSF rhinorrhea. Congenital intrasphenoidal encephaloceles, which are located medial to the foramen rotundum, seem to be due to persistence of the Sternberg's canal. Transcranial approach is a good option when a transnasal approach had failed previously.

For osteoblastoma, with its predilection for the spinal column and appendicular skeleton, the skull is an unusual site, and paranasal sinus involvement is very rare. Herein, we report on a case in which the disease was located within the sphenoid bone. To the best of our knowledge, this is the 4th reported case of osteoblastoma with a sphenoid origin (1). We report an osteoblastoma of the sphenoid sinus in a 12-year-old girl who presented with exophthalmos. Computed tomography (CT) demonstrated an expansile lesion of the sphenoid which caused the orbital contents to be compressed and deviated to the right. In the magnetic resonance imaging scan, the lesion was found to invade the cranial base in the frontal and temporal region, approximating to the cavernous sinus and internal carotid artery on the right. Bilateral fronto-orbital craniotomy was performed. Histologically, the lesion was composed of proliferating osteoblasts along with vascular stroma. The tumor was described as an aggressive osteoblastoma. In the follow-up CT four months later, a pathological mass was observed in the area of the nasal septum, and a signal void was present on all sequences in the densely sclerotic areas. A second resection was performed. The patient has been disease-free for 61 months. Herein, we present the diagnosis and management of this unusual lesion. The histopathology and the imaging characteristics are shown.

Isolated sphenoid sinusitis (ISS) is a rare entity. ISS accounts for about 1–2% of all sinus infections. Isolated sphenoid sinus involvement may include mucoceles, pyoceles and isolated mycotic infections. We report a case of isolated sphenoid pyocele in a 35 year-old female who presented in August 2007 with frontal and occipital headache, post nasal discharge and bilateral nasal obstruction for 6 years. CT scan showed isolated right sphenoid sinusitis. Diagnostic nasal endoscopy showed a streak of post nasal discharge on right side above the torus tubaris confirming right sphenoiditis. A cyst in the nasopharynx was incidentally detected which was confirmed by fluid aspiration to be Thornwaldt’s cyst (TC). TCs have reported prevalence of 0.2–5% and though most are asymptomatic their location renders them vulnerable to infection. Sphenoidotomy was done and the cyst in the nasopharynx was removed and marsupialised. Postoperative course was uneventful.