Pathogenesis of non-traumatic cerebrospinal fluid rhinorrhea

15 consecutive patients with non-traumatic cerebrospinal fluid rhinorrhea were studied. 13 operations were performed on 10 patients. In 8 transcranial operations, an assumed defect in the anterior fossa was plugged with muscle, but only 3 operations were successful. In 4 operations, either transcranial or transsphenoidal, the sella was packed with muscle and rhinorrhea ceased immediately.
Based on radiological and operative findings, 3 groups of patients appeared (1) 9 patients had pathology related to the pituitary gland or the sella turcica: enlarged sella, empty sella, pituitary tumour, intrasellar cyst or erosion of the sellar osseous border. (2) 2 patients had rhinorrhea from extrasellar origin. (3) In 4 patients no abnormality could be found.
Prior to the rhinorrhea, 6 patients (5 from group 1 and 1 from group 3) had experienced episodes of neurological symptoms, compatible with a pituitary apoplexy.
It is suggested that non-traumatic cerebrospinal fluid rhinorrhea in most cases is the result of a spontaneous necrosis in a pituitary adenoma, which has caused sellar bony erosion.     

The empty sella and pituitary adenomas

In 44 consecutive patients with sellar volume larger than 1100 mm³, computer tomography showed that 20 had an empty or partly empty sella. None had radiological evidence of a suprasellar tumor. 10 of the 20 patients had experienced episodes with acute neurological symptoms presumably reflecting a pituitary apoplexy. It is suggested that an unknown proportion of intrasellar adenomas may disappear as a result of an infarction, which may comprise the entire adenoma or part of it - leaving an empty or partly empty sella as diagnosed by computer tomography. Air encephalography will demonstrate the empty sella only if the diaphragmatic aperture is large enough to allow cisternal herniation. The infarction may present clinically with no, slight or severe acute neurological symptoms. Late consequences of a pituitary adenoma infarction may be rhinorrhea or hydrocephalus.     

Does pseudotumor cerebri cause the empty sella syndrome?

Eight patients had both the "primary empty sella syndrome," diagnosed by the finding of an air-filled sella turcica at pneumoencephalography, and pseudotumor cerebri, diagnosed by the finding of an elevated cerebrospinal fluid pressure in the presence of normal ventricular size and position on pneumonencephalography. All eight patients were obese women, and six were hypertensive. Six complained of headaches and menstrual irregularities, and two were asymptomatic. Three had visual symptoms and four had papilledema at the time of examination. These two clinical disorders appear to be frequently related, and when they are related, visual field defects and visual loss are more likely to occur than when either entity appears alone. Chronically increased intracranial pressure from pseudotumor cerebri may produce an empty sella if the diaphragma sella is incompetent and the subarachnoid space herniates into the sella turcica.     

Pituitary apoplexy with sterile meningitis

In 255 patients with evidence of a pituitary tumor, 7 patients presented with typical symptoms of meningitis, which all proved to be sterile. In these patients a pituitary adenoma was surgically verified in 4. In one patient the presence of acromegaly indicated a pituitary adenoma. In 2 patients an enlarged, empty sella was found, taken to reflect spontaneous disappearance of an adenoma. It is thought that the cause of the sterile meningeal reaction in the 7 patients was a spontaneous necrosis of the adenoma with expulsion of necrotic material into the suprasellar subarachnoid space. Although this sequence has been described before it has been considered to occur very rarely. Pituitary apoplexy as the underlying cause of meningitis should be suspected in patients with evidence of pituitary endocrine disturbances or lesion of structures adjacent to the fossa. The diagnosis may be established by computer tomography. Therapeutically, it may be of importance that the nature of the meningeal reaction is promptly recognized. Swift neurosurgical decompression of the sella and adequate hormonal substitution therapy may be essential.     

Adult hydrocephalus and the empty sella

Abstract– A 51-year-old man presented with a pituitary apoplexy with symptoms and signs predominantly of meningitis. Later on, hydrocephalus and an empty sella were disclosed. It is likely that the empty sella and the hydrocephalus were both caused by the adenoma necrosis. Based on this case and cases reported in the literature, it is suggested that concomitant presence of an empty sella and hydrocephalus results from necrosis in a pre-existing pituitary adenoma.     

Empty sella associated with inappropriate TSH secretion

A child diagnosed with long-standing primary hypothyroidism at age 10 and subsequent pseudotumor cerebri after initiation of thyroid therapy developed an empty sella and inappropriate thyrotropin secretion. No other evidence of thyroid hormone resistance was evident nor was there evidence of other endocrine dysfunction. This case confirms previous reports of a relationship between autoimmune thyroid disease, pseudotumor cerebri, empty sella and inappropriate anterior pituitary function.     

Rapidly progressive bilateral ophthalmoplegia and enlarging sellar mass caused by amelanotic melanoma.

A 63-year-old woman with diplopia and bilateral ptosis underwent brain MRI that showed a pituitary mass with signal characteristics suggestive of adenoma. Within one week she had developed nearly complete bilateral ophthalmoplegia. A repeat MRI showed extension of the mass into both cavernous sinuses. Hypophysectomy disclosed an amelanotic melanoma. Extensive search for a primary source was unsuccessful. Despite local radiation treatment, the tumor continued to grow and the patient became blind and died within several months of diagnosis. There are seven reported cases of melanoma arising primarily in the sella turcica. Two cases of metastatic melanoma to the cavernous sinuses have been reported. Amelanotic melanoma has not been reported as a cause of cavernous sinus syndrome.     

MRI in the diagnosis of so-called pituitary apoplexy: seven cases

The authors describe seven cases of pituitary adenoma with acute neurological symptoms (the so-called pituitary apoplexy), which were examined by magnetic resonance imaging (MRI). Four patients underwent surgery and the diagnosis of pituitary adenoma was histologically confirmed. In 3 patients, conservative treatment was decided and serial MRI examinations showed progressive decrease in size of the tumour, leading in one case to an empty sella. Computerized tomography (CT) was positive in all 7 cases, but less contributive than T1-weighted MRI images both for tumoral volume determination and detection of intratumoral haemorrhage. Although theoretical problems may arise concerning the diagnosis of acute intratumoral bleeding at an early stage, the authors emphasize the value of MRI in showing the extension of the tumor into the retroclival cistern and the cavernous sinus, especially when surgery is not performed in the acute period.     

垂体腺瘤经蝶切除术后发生迟发性视功能减退原因及处理探讨

目的 探讨经蝶切除垂体腺瘤术后继发迟发性视功能减退的原因，尤其是其与空蝶鞍的关系，提出积极防治的意义。方法 分析了北京协和医院诊治过的4例经蝶手术切除垂体腺瘤术后继发不同程度空蝶鞍的迟发性视功能减退患者，并结合文献加以讨论。结果 这4例患者中2例再次经颅手术，1例再次经蝶手术，1例接受保守治疗，治疗后其视功能均有不同程度地改善或恢复正常。结论 结合文献复习，认为经蝶术后合并空蝶鞍的患者发生迟发性视功能减退与视神经和视交叉局部的手术后瘢痕组织机械性牵拉以及血运改变有关。综合治疗后4例患者的视功能均有不同程度的改善或恢复正常，这既说明了上述原因的存在，又显示了积极防治的意义。     

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy

We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis.     

Cavernous aneurysm and pituitary adenoma: management of dual intrasellar lesions.

A 53-year-old female with a functioning pituitary adenoma was found to also have an unruptured asymptomatic aneurysm in the cavernous sinus portion of the internal carotid artery on MRI. The adenoma had a suprasellar extension with optic chiasm compression and extended into the right cavernous sinus. An aneurysm-like flow-void adjacent to the left internal carotid artery in the sella and embedded in the adenoma was also found. The aneurysm was confirmed by conventional angiography. We used a right fronto-pterional approach to clip the aneurysm and to remove the pituitary tumor in a one-stage procedure. The outcome was good on long-term follow-up. It is important to thoroughly evaluate the anatomic relations around the sella turcica prior to procedures with limited exposure, including transsphenoidal surgery, to avoid unrecognized complications and morbidity.     

部分空蝶鞍合并垂体腺瘤诊断和手术治疗

报道１９７９年４月至１９９５年２月经手术和（或）影像学证实的１５例，部分空泡蝶鞍（ＰＥＳ）合并垂体腺瘤，占同期垂体腺瘤的１．６％，其中合并肢端肥大症６例，柯兴氏病５例，泌乳素腺瘤３例，无功能腺瘤合并自发性脑脊液鼻漏１例。本组１４例经蝶窦显微外科手术切除垂体腺瘤，术后均达到治愈和缓解，无１例发生术后脑脊鼻漏。本文就其病因、发病机制、诊断、处理原则和手术技巧等进行了较详细的讨论。     

Ectopic Pituitary Adenoma Associated with an Empty Sella: A Case Report and Review of the Literature

A case of ectopic pituitary adenoma in the sphenoid sinus associated with an empty sella is reported. The coexistence of an ectopic pituitary adenoma and an empty sella is quite rare. The diagnosis was made with an intraoperative finding of the intact dura mater of the sellar floor. In the present case, the hypointense line that coated the pituitary gland was clearly demonstrated on 3‐tesla T2‐weighted magnetic resonance imaging. The hypointense line is considered to be the pituitary capsule and was critical in diagnosing this rare entity.     

Endocrinology of pseudotumor cerebri

This article will review the endocrine function of obese young women with pseudotumor cerebri and primary empty sella syndrome. The literature can be difficult to interpret. Much of it predates the era of CT scanning. Often, cases of primary and secondary empty sella syndrome are comingled. The author reviews specific endocrinologic disorders that have sometimes been associated with increased intracranial pressure excluding pituitary adenomas.     

侵袭性垂体腺瘤侵袭性的综合判断及其与临床预后的关系

目的分析经侵袭性垂体腺瘤侵袭性的判断标准及其与临床预后的关系。方法回顾性分析我科自2000年1月至2003年5月行经蝶手术切除的具有完整影像学、病理学和随访资料的侵袭性垂体腺瘤82例,随访时间5～30个月。结果侵袭性垂体腺瘤MR影像学特点为鞍底受侵下陷,部分肿瘤突入蝶窦;颈内动脉包绕;海绵窦受累,鞍隔突破等。病理检查以鞍底硬膜浸润的发生率最高。PRL腺瘤的全切率明显低于无功能腺瘤(P<0.01),无功能腺瘤、GH腺瘤与ACTH腺瘤之间相差不明显。PRL腺瘤的复发率较无功能腺瘤和GH腺瘤高(P<0.05)。结论垂体腺瘤的侵袭性生物学行为导致手术全切几率下降,是术后复发的主要原因。     

侵袭性催乳素腺瘤治疗后海绵窦残留或伴空蝶鞍综合征

目的 探讨侵袭性催乳素(PRL)腺瘤经治疗后海绵窦残留伴空蝶鞍综合征的临床特征和治疗策略.方法 18例侵袭性PRL腺瘤(Knosp分级Ⅲ或Ⅳ级)经治疗后出现海绵窦肿瘤持续残留,首选药物治疗者9例,首选手术治疗结合药物和(或)放疗9例.结果 平均随访55个月,8例PRL水平正常,7例视力改善.14例因鞍内肿瘤吸收遗留空蝶鞍,7例PRL仍升高,其中5例有不同程度的视交叉下疝(P<0.05).PRL水平正常、无临床症状的7例患者采用低剂量溴隐亭治疗达到PRL水平和肿瘤体积的长期控制.结论 PRL腺瘤治疗后海绵窦肿瘤残留伴发空蝶鞍,这一现象应该引起高度重视,尤其对伴有视交叉下疝的病例,长期的密切随访是必需的.无内分泌和压迫症状的海绵窦残留病例可以采用低剂量的药物治疗达到长期的肿瘤控制和内分泌控制.     

Panuveitis in association with pseudotumor cerebri

We report a case of an 11-year-old girl with bilateral panuveitis in association with pseudotumor cerebri. The patient underwent complete ophthalmologic, neurologic, and laboratory evaluations and was treated with therapy for pseudotumor cerebri. The patient met the diagnostic criteria for pseudotumor cerebri and also had panuveitis. Symptoms and findings of pseudotumor cerebri and panuveitis improved significantly after combination therapy of oral acetazolamide and weight reduction. The index case illustrates that pseudotumor cerebri can be associated with panuveitis. Therapy for pseudotumor cerebri might also help with the resolution of uveitis.     

Diagnostic value of super-selective bilateral cavernous sinus sampling with hypothalamic stimulating hormone loading in patients with ACTH-producing pituitary adenoma

AIMS: Early diagnosis and early treatment by transsphenoidal surgery is desirable for ACTH-producing pituitary microadenoma, but accurate localization of the functional lesion is not always possible before surgery because magnetic resonance (MR) imaging may provide false negative and/or positive findings. The diagnostic value of super-selective bilateral cavernous sinus sampling with the administration of corticotropin-releasing hormone (CRH) was assessed in patients with functioning ACTH-producing pituitary adenoma. METHODS: Fifteen patients with pituitary adenoma (14 with microadenoma) aged from 23 to 74 years (mean 46.7 years) underwent cavernous sinus sampling with or without the CRH loading test and subsequent transsphenoidal surgery in our institute from October 1997 through to November 2002. MR imaging including dynamic scan failed to detect the adenomatous lesion in all patients. To eliminate the bias due to uneven blood flow in the cavernous sinuses and the multi-hormonal response to CRH administration, the ACTH/FSH ratios were evaluated. The inter-cavernous gradient (ICG) was calculated as the higher/lower ACTH venous blood levels in the right and left cavernous sinuses with or without CRH loading. The adjusted ICG was calculated using the ACTH/FSH ratios. The results were compared with the surgical findings. An ICG of 1.4 or greater was considered to indicate the localization of the responsible lesion. RESULTS: Transsphenoidal surgery revealed the functioning lesion on the right in five cases, the left in six, the midline in three and the bilateral lateral wings (double adenoma) in one. Adjusted ICG with CRH loading had a localization accuracy of 93.3% (14/15), which was significantly higher than that of 73.3% (11/15) using ICG without hypothalamic stimulating hormone loading (p = 0.0402). CONCLUSIONS: Super-selective cavernous sinus sampling with hypothalamic stimulating hormone administration can provide accurate localization of the responsible lesion in patients with ACTH-producing pituitary adenoma.     

Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri

Cerebrospinal fluid (CSF) pressure was measured in four groups of patients: 116 with acute pseudotumor cerebri, 18 with chronic pseudotumor cerebri, 41 obese and 15 nonobese normal patients undergoing spinal anesthesia. Spinal fluid pressure between 200 and 250 mm H2O was found in members of each group. This suggests that when elevated CSF pressure is suspected, confirmation requires values greater than 250 mm H2O. The CSF pressure and degree of obesity could not be correlated in any meaningful way. There was no significant statistical difference between the mean CSF pressures obtained in the obese and nonobese normal populations.     

A case of TSH-secreting pituitary adenoma associated with an unruptured aneurysm: successful treatment by two-stage operation and gamma-knife]

We report a successfully treated case of invasive TSH-secreting pituitary adenoma associated with an unruptured internal carotid artery aneurysm by two-stage operation and gamma-knife radiosurgery. A 64-year-old woman was admitted to our department with a 3-year history of general fatigue and 1-year history of anxiety, palpitation and hyperhydrosis. Endocrinological examination revealed hyperthyroidism with elevated TSH, GH and somatomedin C. Magnetic resonance images demonstrated a tumor in the sella turcica which extended into the left cavernous sinus, furthermore, indicated aneurysm-like flow void at the ventral part of the left internal carotid artery. The aneurysm was confirmed by conventional angiography. Neck clipping of the aneurysm was performed through pterional approach as the first operation. One month later, at the second operation, the pituitary adenoma except for cavernous sinus portion was resected via the transsphenoidal approach. Immunohistological examination revealed positive for TSH and GH. Gamma-knife radiosurgery with a central dose of 33.3 Gy and peripheral dose of 17 Gy was carried out for residual tumor at the cavernous sinus under both MRI and CT guidance. Posttreatment course was uneventful with normalization of thyroid function at 16 months after gamma-knife. Two-stage operation and gamma-knife radiosurgery is effective for TSH-secreting adenoma extending into the cavernous sinus associated with an unruptured aneurysm.