Pituitary apoplexy

Pituitary apoplexy is an infrequent complication of pituitary adenomas, caused by hemorrhagic or ischemic infarction in the tumor, with typical clinical presentation: severe headache of sudden onset, visual disturbances, sleep tendency or comma. Along the last ten years we have treated eight patients with pituitary apoplexy. The diagnostic was clinically established in all of them. Seven cases complained of severe headache and vomiting. Eight patients reported visual disturbances. In one case low level of consciousness and meningeal irritation were the only findings. In six cases the apoplexy was the first pituitary adenoma manifestation. MRI and CT studies demonstrated the pituitary stroke in seven patients. Surgical trans-sphenoidal decompression was performed in seven patients, requiring urgent management in only one case. All patients experienced a marked visual improvement, but there was no amelioration of endocrine preoperative disturbances in any case. We conclude that quick diagnosis, early onset of hormonal therapy and urgent or delayed trans-sphenoidal surgery, depending on clinical manifestations, constitute the principies of the appropriate treatment of pituitary apoplexy.     

Pituitary apoplexy, therapeutic assessment.

The management of pituitary apoplexy with reference to both diagnosis and operative sequelae remains a major challenge. Acute onset of retro-orbital headache in association with visual loss and ophthalmoplegia are the cardinal symptoms; however, obtundation and signs of subarachnoid hemorrhage also may be present. Good quality plain skull radiographs and complete angiography prove sufficient for preoperative radiographic studies. Preoperative endocrine preparation focuses on supplemental glucocorticoids since these patients must be presumed deficient in cortisol reserve. Residual visual deficit appears to be more a function of the extent of damage at the time of ictus rather than rapidity of decompression. Our experience indicates that transsphenoidal decompression in appropriate cases offers an ideal opportunity to minimize mortality and morbidity. The acute onset of severe retro-orbital headache in association with stupor and ocular palsies would alert most physicians to the potential diagnosis of spontaneous subarachnoid hemorrhage. The association of complex ophthalmoplegias and visual defects in this constellation of symptoms should, in addition, alert one to the possibility of an acute intrasellar or parassellar expansile process. During the past two years, we have had the opportunity to care for 8 such patients with confirmed diagnoses of acute hemorrhagic infarction of the pituitary enabling us to formulate diagnostic and therapeutic schemata with reference to management of this problem.     

Incidentally discovered pituitary macroadenoma. Neurosurgical treatment indications illustrated by two cases

Pituitary macroadenomas (more than 10 mm in diameter) are infrequent as casual findings and optimal management strategy for these tumours has not been established. Neurosurgical approach must be always considered in patients with visual field defects or with hormone-secreting adenomas (but prolactinoma), and in those with evidence of lesion's growth or if clinical pituitary apoplexy occurs. We present two cases in which surgical indication was based on patient's young age (case number one), and on hypogonadal status, in a male patient not suitable of androgen substitution (case number two). We also discuss the benefits of including such unusual indications for neurosurgical treatment into the incidentally discovered pituitary macroadenomas evaluation strategy.     

Pituitary apoplexy after cardiac surgery in a patient with subclinical pituitary adenoma: case report with review of literature

We report a case of pituitary apoplexy occurring in a 74-year-old patient 6 hours after cardiac surgery. The patient presented with confusion, unilateral ptosis and ophthalmoplegia. Neurological examination revealed right oculomotor nerve palsy and decreased level of consciousness. Magnetic resonance imaging showed a hemorrhagic and necrotic pituitary macroadenoma. After prompt endocrinological replacement therapy with hydrocortisone and levothyroxine, the confusion of the patient resolved. Removal of a non-functional macroadenoma with large necrotic areas resulted in full recovery. The physician should be aware of pituitary adenoma infarction after open cardiac surgery and should remember that it can be fatal or cause permanent neurological or endocrine damage without proper treatment. Surgical and endocrine treatment can be life-saving procedures.     

Giant necrotic pituitary apoplexy

Apoplexy of the pituitary gland is a rare complication of pituitary adenomas, involving hemorrhage with or without necrosis within the tumor. This condition may be either asymptomatic or may present with severe headache, visual impairment, ophthalmoplegia, and pituitary failure. Transsphenoidal surgery is the treatment of choice, and early intervention is usually required to ensure reversal of visual impairment. Reports of pituitary apoplectic lesions exceeding 60.0 mm in diameter are very rare. A 39-year-old man with long-standing history of nasal congestion, decreased libido and infertility presented with a sudden onset of severe headache and diplopia. MRI of the head demonstrated a massive skull base lesion of 70.0 × 60.0 × 25.0 mm, compatible with a giant pituitary macroadenoma. The lesion failed to enhance after administration of a contrast agent, suggesting complete necrotic apoplexy. Urgent surgical decompression was performed, and the lesion was resected via a transnasal transsphenoidal approach. Pathological analysis revealed evidence of necrotic pituitary apoplexy. At the 2 month follow-up, the patient had near-complete to complete resolution of his visual impairment. To the authors’ knowledge, this report is unique as the patient demonstrated complete necrotic apoplexy and it underlines the diagnostic dilemma in such a case.     

经鼻蝶入路显微手术治疗垂体卒中的临床分析

目的总结55例垂体卒中患者的临床表现特征，分析经鼻蝶入路显微手术的疗效。方法回顾性分析经鼻蝶入路显微手术治疗的55例垂体卒中患者的临床和影像学资料。结果本组55例垂体腺瘤中，48例全切除，7例次全切，肿瘤全切率为87．3％。51例视力障碍、视野缺损的患者中46例术后恢复正常（90．2％），5例恢复欠佳；32例伴有内分泌症状的患者中29例明显缓解（90．6％），3例无明显变化。术后一过性尿崩症7例，脑脊液漏5例。无死亡病例5例。结论根据突发头痛及视路症状等相关症状、体征下并结合影像学检查一般可确诊垂体卒中；经鼻蝶入路显微手术和／或内窥镜手术可做为治疗垂体卒中的首选方法。     

Radiological Apoplexy and Its Correlation with Acute Clinical Presentation, Angiogenesis and Tumor Microvascular Density in Pituitary Adenomas

Objective

Pituitary apoplexy is life-threatening clinical syndrome caused by the rapid enlargement of a pituitary tumor due to hemorrhage and/or infarction. The pathogenesis of pituitary apoplexy is not completely understood. We analyzed the magnetic resonance imaging (MRI) of pituitary tumors and subsequently correlated the radiological findings with the clinical presentation. Additionally, immunohistochemistry was also performed to determine whether certain biomarkers are related to radiological apoplexy.

Methods

Thirty-four cases of pituitary adenoma were enrolled for retrospective analysis. In this study, the radiological apoplexy was defined as cases where hemorrhage, infarction or cysts were identified on MRI. Acute clinical presentation was defined as the presence of any of the following symptoms: severe sudden onset headache, decreased visual acuity and/or visual field deficit, and acute mental status changes. Angiogenesis was quantified by immunohistochemical expression of fetal liver kinase 1 (Flk-1), neuropilin (NRP) and vascular endothelial growth factor (VEGF) expression, while microvascular density (MVD) was assessed using Endoglin and CD31.

Results

Clinically, fourteen patients presented with acute symptoms and 20 for mild or none clinical symptoms. Radiologically, fifteen patients met the criteria for radiological apoplexy. Of the fifteen patients with radiologic apoplexy, 9 patients presented acute symptoms whereas of the 19 patient without radiologic apoplexy, 5 patients presented acute symptoms. Of the five biomarkers tracked, only VEGF was found to be positively correlated with both radiological and nonradiological apoplexy.

Conclusion

While pituitary apoplexy is currently defined in cases where clinical symptoms can be histologically confirmed, we contend that cases of radiologically identified pituitary hemorrhages that present with mild or no symptoms should be designated subacute or subclinical apoplexy. VEGF is believed to have a positive correlation with pituitary hemorrhage. Considering the high rate of symptomatic or asymptomatic pituitary tumor hemorrhage, additional studies are needed to detect predictors of the pituitary hemorrhage.     

Spontaneous infarction in pituitary tumors: neurologic and therapeutic aspects.

In addition to progressive endocrine dysfunction and progressive visual loss, pituitary neoplasms may annouce their presence by the more catastrophic alternative of spontaneous tumor infarction. In two patients reported, illness due to the spontaneous infraction of pituitary tumors was heralded by sudden onset of focal headache associated with diplopia. Stupor, confusion, and evidence of increased intracranial pressure occurred without subarachnoid hemorrhage or massive extrasellar extension of tumor. One patient developed inappropriate antidiuretic hormone secretion with spontaneous infarction in a large but clinically silent chromophobe adenoma. In both patients, skull x-rays suggested a long-standing intrasella mass. Both underwent prompt treatment with endocrinologic replacement therapy and subsequent successful transsphenoidal removal of voluminous, infarcted, pituitary masses.     

Precipitating factors in pituitary apoplexy

Pituitary apoplexy is a rare but life threatening condition caused by sudden haemorrhage or infarction of the pituitary gland. Potential precipitating factors in the occurrence of acute pituitary apoplexy in 30 consecutive patients were identified and compared with the clinical characteristics and outcome of patients with and without associated factors. Six patients had a previously known pituitary adenoma. All patients complained of severe headaches, associated with neuro-ophthalmological symptoms and signs in 83% and altered mental status in 30%. Potential risk factors were identified in nine patients (30%). When there was an associated factor, the clinical presentation was no different than in patients without such factors although altered mental status may be more frequent in patients with associated diseases. In these patients, the visual prognosis was worse and the diagnosis was more difficult to establish. Acute pituitary apoplexy is unpredictable and should be considered in any patient with abrupt neuro-ophthalmological deterioration associated with headache. Patients with pituitary apoplexy often have an associated disease that confounds recognition and treatment despite a typical presentation.     

Pituitary Apoplexy in a Teenager—Case Report

BackgroundPituitary apoplexy is a rare clinical emergency which results from hemorrhage or infarction in the pituitary gland.PatientWe present a 14-year-old girl with pituitary apoplexy and review the literature.ResultsOur patient experienced blurred vision, nausea, and headache. Her best-corrected visual acuity was 20/200 and 20/20. Confrontation test visual field testing revealed bitemporal hemianopsia. Brain imaging demonstrated a suprasellar mass. The microscopic endonasal transsphenoidal approach only found 5-10 mL brownish fluid-like material. Pathology confirmed no malignancy. Pituitary apoplexy was diagnosed. Her nausea and headache gradually improved. Six months after operation, her best-corrected visual acuity had improved to 20/30 and 20/20.ConclusionsAlthough pituitary apoplexy is rare in pediatric patients, prompt evaluation including detailed ophthalmic examination, biochemical evaluation, endocrine workup, and image study are very important.     

亚急性垂体卒中49例临床分析

目的探讨亚急性垂体卒中的诊断及评价手术治疗效果。方法亚急性垂体卒中49例,其中男27例,女22例,分别采用额下入路、翼点入路、经鼻—蝶窦入路行垂体腺瘤切除术。结果 术后对视力损害、视野缺损及眼肌麻痹疗效满意,有效率分别为84.04％、79.22％、100.00％。结论 亚急性垂体卒中临床表现以视力、视野损害及头痛为主,症状多不典型,头颅CT/MRI扫描有助于明确诊断,应在术前行完整的内分泌功能评估,于常规应用肾上腺糖皮质激素的基础上择期手术治疗,其手术术式不能一概而论。     

Hemorrhagic necrosis of pituitary adenomas

A clinicopathological study of 41 cases of pituitary apoplexy in a series of 324 surgically treated pituitary adenomas is presented. In 23 patients, the predominant operative finding was hemorrhage with or without necrosis. However, there were 15 (37.7%) cases where pale, necrotic tissue with no evidence of hemorrhage was found at surgery. Pale, necrotic material was particularly found when there was a long interval between the acute clinical event and surgery. It is concluded that the pale, necrotic debris represents one stage in the resorption process of blood after hemorrhagic necrosis of pituitary adenomas. This entity needs to be kept in mind especially since the material closely resemble the pultaceous material seen in craniopharyngiomas and epidermoid cysts.     

Pituitary apoplexy in a child presenting with massive subarachnoid and intraventricular hemorrhage.

A case of pituitary apoplexy in a child with massive subarachnoid and intraventricular hemorrhage is reported. The patient presented with a sudden onset of headache, diminution of visual acuity and a bitemporal visual field defect. Computerized tomography revealed a massive subarachnoid hemorrhage with extension of blood into the third ventricle. Angiography did not show an aneurysm. The patient was operated on and altered blood within a necrotic pituitary adenoma was decompressed. His vision and field defect improved along with resolution of headache in the postoperative period.     

Pituitary apoplexy causing optic neuropathy and horner syndrome without ophthalmoplegia.

A 47-year-old woman presented with headache, acute monocular vision loss, and ipsilateral Horner syndrome. Apart from the optic neuropathy, all cranial nerve function was intact. Magnetic resonance imaging revealed an enlarged pituitary gland with compression of the orbital apex. The surgical specimen was consistent with pituitary apoplexy. The combination of headache, acute visual loss, and ipsilateral Horner syndrome without ophthalmoplegia, which may suggest carotid artery dissection, is evidently an unusual manifestation of pituitary apoplexy.     

Silent corticotroph adenomas: presentation of two cases that presented with pituitary apoplexy

Among the group of pituitary adenomas surgically treated, about 25-30% are not associated with clinical or analytical findings of hormonal hypersecretion. The development of immunohistochemical techniques has allowed the demonstration of a subgroup of adenomas that show immunoreactivity against several hormones among the group of, apparently, non-functioning adenomas. This subgroup has been called silent adenomas. Silent adenomas positive for ACTH show a singular clinical picture and different from those adenomas producing Cushing's disease, as they present more frequently as macroadenomas, with more frequent pituitary apoplexy, invasion of cavernous or sphenoidal sinus and recurrences. We present two new cases of silent corticotroph adenomas in two female patients that presented with pituitary apoplexy, one of them after giving birth after a normal full-term pregnancy. Both of them presented with macroadenomas that invaded the sphenoidal and cavernous sinus. Although both tumors were immunoreactive for ACTH, none of the patients presented clinical or analytical findings compatible with Cushing's disease.     

Acute monocular disturbances mimicking optic neuritis in pituitary apoplexy

Three patients with acute monocular central scotoma and headache had the initial diagnosis of optic neuritis. Computed tomography showed large pituitary adenomas with necrosis and cyst formation consistent with the clinical symptoms of a previous pituitary apoplexy. After transsphenoidal adenomectomy the visual disturbances disappeared.     

Pituitary apoplexy with hypercreatinekinasemia

A 59 year-old female with hypercereatinekinasemia associated with pituitary apoplexy was presented. The patient showed headache, nausea, vomiting and pyrexia. On admission, slight nuchal rigidity and photophobia were observed. However all the cranial nerves were intact; neither ophthalmoplegia nor visual defect were observed. Cerebrospinal fluid analysis revealed an elevated protein concentration of 164 mg/dl. There were 157 cells/mm2 (30% neutrophils). Skull X-P disclosed the ballooning of the sella turcica. CT scan, endocrinological examination and angiography lead us to the diagnosis of pituitary apoplexy. By the sphenoidal approach necrotic tissue with a little chromophobe adenoma were removed. No haematoma was detected. The isozyme pattern of serum CK showed 100 percent MM type. Serum CK concentration reached as high as 2502 IU/l on the fifth day from the onset of the symptom and then normalized in 12 days. Though the cause of the hypercreatinekinasemia uncertain, the similar pattern of hypercreatinekinasemia is known in the acute stage of cerebrovascular accident, and it is more often observed in thalamic hemorrhage. We assumed that the hypercreatinekinasemia in our case was caused by hypothalamic irritation, which lead hyperpermeability of sarcolemma and leakage of the enzymes of muscle origin.     

A Rathke's Cleft Cyst Presenting with Apoplexy

The occurrence of symptomatic pituitary hemorrhage into a Rathke''s cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.     

Pituitary adenomas: presentations and outcomes in a South Asian country

OBJECTIVE: The purpose of this retrospective review of all operated cases of pituitary adenomas in the last decade, is to define the demographic patterns and characteristics of such tumors and to assess surgical outcomes with regards to safety and efficacy of trans-sphenoidal tumor removal in our institution. METHODS: Surgically treated pituitary adenomas presenting from 1995 till 2005 were reviewed for different variables. Results were expressed as mean, standard deviation and median for continuous and number with percentage for categorical data. Chi square test was applied to measure differences and significance was taken atp value < 0.05. RESULTS: One hundred and twenty-five patients were operated for pituitary adenoma. Sixty-three percent were male and mean age was 37 years. Sixty percent of the patients presented with headache and/or visual symptoms. Twelve percent presented with pituitary apoplexy and 28% presented with symptoms due to pituitary hyperfunction. Fifty-five percent of patients had functioning and 44% had nonfunctioning adenomas. Mean pre operative tumor diameter from 86 pre op MRI scans was 26.76 mm (3-78 mm). Eighty-four percent of patients underwent trans-sphenoidal tumor resection and three percent had craniotomy. Mean size of post op residual tumor as calculated from 76 available post operative scans was 5.3 mm (range 0-31 mm). 17.6% of the patients required hormone replacement beyond three months and 10% were re-operated. Overall mortality was 1.6%. CONCLUSION: In Pakistan, patients are more likely to present either with apoplexy or with a giant pituitary adenoma than patients reported from developed countries. Overall, our results have been satisfactory and comparable with the literature.     

急性垂体腺瘤卒中的临床研究

目的　对急性垂体卒中的临床表现、诱发因素、辅助检查、治疗和结果进行分析讨论。方法　回顾性分析1996年1月至2001年12月经手术治疗的35例急性垂体卒中病人。男17例,女18例。年龄19～69岁,平均46.5岁。所有患者术前、术后均行内分泌激素及头颅CT和/(或)MRI检查。结果　头痛是急性垂体卒中最常见的临床症状,其次是视觉障碍,再次为眼肌麻痹。急性垂体卒中的可能诱发因素为高血压、糖尿病。CT和MRI检查对急性垂体卒中确诊率分别为26%和90%。经蝶手术22例,开颅手术13例,无手术死亡。无功能性垂体腺瘤最多见,占65.7%。在急性垂体卒中后7天内手术,术后视力、视野改善率较高(P<0.05)。术后随访时间平均为3.2年,均恢复良好,肿瘤复发2例。术后4例放疗。术后长期需要甲状腺激素、皮质类固醇激素、睾酮和抗利尿激素替代治疗者分别为51%、56%、34%(男)、和3%。结论　突发头痛是急性垂体卒中最常见的症状。高血压和糖尿病是急性垂体卒中的重要诱发因素,无功能腺瘤在急性垂体卒中最多见。MRI是急性垂体卒中首选的检查方法。经蝶入路手术减压是治疗垂体卒中安全有效的方法。在垂体卒中后7d内手术视力、视野改善更明显。术后放疗仅适用于肿瘤残留或复发者。