Pituitary Apoplexy Following Mitral Valvuloplasty

Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome caused by the sudden enlargement of a pituitary adenoma secondary to hemorrhage or infarction. Pituitary apoplexy after cardiac surgery is a very rare perioperative complication. Factors associated with open heart surgery that may lead to pituitary apoplexy include hemodynamic instability during cardiopulmonary bypass and systemic heparinization. We report a case of pituitary apoplexy after mitral valvuloplasty with cardiopulmonary bypass. After early pituitary tumor resection and hormonal replacement therapy, the patient made a full recovery.     

Pituitary macroadenoma causing symptomatic internal carotid artery compression: Surgical treatment through transsphenoidal tumor resection

Pituitary macroadenomas can invade the cavernous sinus and rarely cause occlusion of the internal carotid artery (ICA). Most patients with symptomatic obstruction of the ICA by a pituitary tumor have been reported as a result of apoplexy. The authors review the literature about this condition and report a 48-year-old man who presented with transient ischemic attacks leading to a stroke. Imaging studies demonstrated complete occlusion of the left ICA and critical narrowing of the right ICA at the level of the clinoid processes, most likely due to macroadenoma mass effect. There was no radiologic evidence of apoplexy. Surgical resection of the tumor and ICA decompression via the transsphenoidal route resulted in prevention of further symptoms. Histopathologic analysis confirmed a nonfunctioning pituitary adenoma without evidence of hemorrhage or intratumoral infarction. This patient, to the authors’ knowledge, is the first documented patient with symptomatic carotid compression by a pituitary adenoma without evidence of apoplexy.     

Bilateral ocular paralysis: analysis of 31 inpatients

To my knowledge, no general study of complete ophthalmoplegia is available. This study was performed to determine the seats and causes of bilateral ocular paralysis. The personal records of 13 440 neurology and neurosurgery inpatients were reviewed. Eighteen (58%) of 31 patients had Fisher syndrome (13 cases) or Guillain-Barré syndrome (5 cases). Four cases resulted from midbrain infarction, 3 from myasthenia, and 1 each from pituitary apoplexy, skull base metastasis, botulism, mucormycosis, phenytoin toxicity, and trauma. Many conditions produce complete ophthalmoplegia on rare occasions, but Fisher syndrome, which paralyzes the eyes in nearly one third of cases, was by far the commonest cause.     

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.     

Pituitary Apoplexy due to Pituitary Adenoma Infarction

Cause of pituitary apoplexy has been known as hemorrhage, hemorrhagic infarction or infarction of pituitary adenoma or adjacent tissues of pituitary gland. However, pituitary apoplexy caused by pure infarction of pituitary adenoma has been rarely reported. Here, we present the two cases pituitary apoplexies caused by pituitary adenoma infarction that were confirmed by transsphenoidal approach (TSA) and pathologic reports. Pathologic report of first case revealed total tumor infarction of a nonfunctioning pituitary macroadenoma and second case partial tumor infarction of ACTH secreting pituitary macroadenoma. Patients with pituitary apoplexy which was caused by pituitary adenoma infarction unrelated to hemorrhage or hemorrhagic infarction showed good response to TSA treatment. Further study on the predisposing factors of pituitary apoplexy and the mechanism of infarction in pituitary adenoma is necessary.     

Cushing's disease in a young woman with anorexia nervosa: pathophysiological implications

This report describes a 17-year old student who was found to have Cushing's syndrome two years after she had developed anorexia nervosa (AN). The Cushing's syndrome was treated with bilateral resection of enlarged, hyperplastic, non-tumorous adrenal glands. The diagnosis was further confirmed four years later when, two to three years after new symptoms had appeared, an ACTH secreting pituitary adenoma (that is, Cushing's disease) was found on surgery. The possible mechanism for the development of Cushing's disease in a patient with prior anorexia nervosa, a sequence of events reported once previously, is discussed. It is suggested that increased hypothalamic-pituitary corticotroph stimulation in association with the anorexia nervosa, a now well-established endocrine phenomenon, activated an occult, inactive pituitary basophil adenoma in this patient, eventually resulting in autonomous pituitary overproduction of ACTH by the tumor.     

Cushing’s disease presenting with pituitary apoplexy

Pituitary tumour apoplexy is a rare but life threatening condition. Cushing’s disease usually presents with clinical features of Cushing’s syndrome. We report a 30-year-old male patient with Cushing’s disease who presented with severe headache and right third nerve palsy. MRI of the pituitary gland revealed a pituitary adenoma with infarction suggestive of apoplexy. After a transsphenoidal surgery he developed pan-hypopituitarism with diabetes insipidus. We also review the relevant literature.     

SIADH following pituitary adenoma apoplexy

The rare case of a patient with SIADH following pituitary adenoma apoplexy is reported. Since apoplexy did not exert any mass effect on surrounding structures, the patient was treated conservatively and the anterior pituitary gland insufficiency has been substituted adequately. Seven days after the apoplexy the patient again showed low serum–Na⁺ levels despite cortisol substitution. Diagnosis of SIADH was made. It is essential to be aware of this rare syndrome in patients with pituitary adenoma apoplexy.     

Pituitary corticotroph hyperplasia preceding adenoma in a patient with Nelson's syndrome

We report the case of a 42-year-old woman with Cushing's disease and Nelson's syndrome. When she was 17 years old, transsphenoidal surgery was performed. A detailed morphologic study demonstrated nodular hyperplasia of corticotroph cells but no adenoma. Following a long-lasting remission (14 years), Cushing's disease recurred. After an unsuccessful second transsphenoidal surgery, Cushing's disease persisted and both adrenals were removed (at the age of 34). Subsequently the patient developed Nelson's syndrome. The pituitary tumor proved to be a corticotroph adenoma; it was removed by the transsphenoidal approach (at the age of 42). Although in most patients Cushing's disease is due to an ACTH-secreting pituitary corticotroph adenoma which precedes the manifestation of Nelson's syndrome, our case indicates not only that corticotroph hyperplasia may cause Cushing's disease but that it may exist before the development of Nelson's syndrome after the removal of both adrenals. Our study supports the view that protracted stimulation of corticotrophs resulting from the elimination of the negative inhibitory feedback effect by corticosteroids plays a role in adenoma initiation.     

Severe myopathy and psychosis in a patient with Cushing's disease macroadenoma

Cushing's disease is most commonly caused by a corticotrope adenoma of the pituitary. Between 50 and 70% of patients with spontaneous hypercortisolism have ACTH-producing pituitary adenomas. The tumors are usually microadenomas with approximately 20% of patients with the disease showing no evidence of tumor on CT-scans or MR imaging of their pituitary glands. In contrast to patients with ectopic ACTH production, plasma ACTH concentrations in patients with spontaneous disease are generally within the normal range. We describe here a patient with a pituitary macroadenoma that showed evidence of necrosis on MRI. The patient had an atypical clinical presentation with plasma ACTH levels considerably higher than that seen in patients with non-ectopic ACTH-secretory syndrome, markedly elevated urine free cortisol, lack of phenotypical signs of hypercortisolism such as wide purplish striae, and whose most prominent and distressing symptom was severe myopathy that resulted in the patient becoming bed-ridden. Psychosis was another striking feature in this patient who during his hospital course developed multiple opportunistic infections that contributed to his demise.     

A case of seasonal bipolar disorder exacerbated by Cushing's disease

While depression is common in Cushing's syndrome from whatever cause (pituitary, adrenal, or ectopic adrenocorticotropic hormone-secreting tumor or hyperplasia, or exogenous administration of glucocorticoids) and hypercortisolemia is prevalent in major depression, any association between seasonal affective disorder and Cushing's syndrome is unknown. We present a case of seasonal bipolar disorder, gradually worsening for more than 9 years (1985-1994), accompanied by increasing osteoporosis, mild weight gain, and slight truncal obesity in a middle-aged woman. In January 1991, her seasonal affective disorder was successfully treated with light therapy, but in the following year, bipolar mood swings with a seasonal pattern emerged, which were refractory to light therapy and antidepressants but responsive to lithium. In August 1992, she became depressed despite a 1500-mg lithium daily dosage along with light therapy, and, in 1993, a diagnosis of Cushing's disease (Cushing's syndrome as a result of a pituitary adrenocorticotropic hormone-secreting tumor) was made. The pituitary tumor was removed in February 1994, and pituitary function was fully restored by 1996. While the symptoms of Cushing's syndrome subsided, her bipolar illness continued to require maintenance treatment with low doses of lithium but did not require light therapy.     

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.     

Ectopic pituitary adenoma of the clivus presenting with apoplexy: case report and review of the literature

Ectopic pituitary adenomas usually occur within sphenoid sinus or nasopharynx, and seldom within the clivus. There is only a single reported example of ectopic adenoma with clinical apoplexy, albeit not from clivus. We report a 78-year-old male with known prostate carcinoma admitted with acute onset of blurred vision, suggestive of apoplexy. Work-up revealed unilateral cranial nerve VI palsy and neuroimaging showed a mass confined to the clivus; sellar region was normal. Preoperative considerations included chordoma, chondrosarcoma, or metastatic prostate carcinoma to bone. Resection was via endoscopic transsphenoidal approach to the clivus. An ectopic null cell pituitary adenoma with bland infarction was identified as the cause of the patient's clinical apoplexy. No antecedent precipitating factors for apoplexy were present; specifically the patient had not received leuprolide preoperatively, a known precipitant of pituitary apoplexy in prostate cancer patients who receive drug. We review the literature on ectopic clival pituitary adenomas, apoplexy in ectopic adenomas, and the link between apoplexy and leuprolide usage.     

Cavernous angioma of the second cranial nerve and chiasmatic apoplexy

We present the case of a female patient who developed chiasmatic apoplexy and menstrual alterations. CT scanning showed a suprasellar hemorrhage. She underwent surgery with the presumptive diagnosis of pituitary tumor. At surgery, we find a brown-grayish lesion involving left optic nerve and chiasm. Cavernous angioma was diagnosed by histopathology. Cavernous angiomas constitute nearly 15% of all central nervous system vascular malformations. Location at the optic pathway is very rare, but must to be ruled out in the diagnosis of a patient with chiasmatic and/or optic apoplexy. Surgery is useful in preventing worsening of the previous deficit or a new visual defect.     

Clinical features and surgical outcome of clinical and subclinical pituitary apoplexy

The clinical features of pituitary hemorrhage vary from asymptomatic to catastrophic. Clinical symptomatic pituitary apoplexy is a well-described syndrome characterized by sudden headaches, visual impairment, hypopituitarism and, at times, impaired consciousness. Subclinical pituitary apoplexy is characterized by minimal atypical clinical manifestations and, as a result, may be misdiagnosed or inappropriately treated. The purpose of the study was to evaluate the clinical manifestations, surgical outcome and post-operative pituitary function in patients who had clinical or subclinical pituitary apoplexy. We retrospectively identified 65 patients (30 men, 35 women; age range 21–87 years, mean age 48.4 years) who underwent transsphenoidal surgery. Of these, 25 patients were diagnosed as having clinical apoplexy, while the remaining 40 patients were classified as having subclinical pituitary apoplexy. The follow-up period ranged from 21 to 73 months (mean 44 months). Vision improved in 14 of 22 patients with clinical apoplexy (64%) and in 26 of 28 patients with subclinical apoplexy (93%) (p = 0.017); the total resection rate was 57% and 42%, respectively (p = 0.285). After 2 years of follow-up, hormone replacement therapy was needed in only 26% and 36% of patients, respectively (p = 0.149). Our findings suggest that the incidence of subclinical pituitary apoplexy is higher than that of clinical pituitary apoplexy. Both the tumor resection rate and mean age were higher in those with clinical pituitary apoplexy, while those who had subclinical pituitary apoplexy demonstrated better visual improvement. During long-term follow-up, the need for hormonal replacement decreased in both groups.     

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. A review

Pituitary apoplexy constitutes a syndrome that is characterized by the ischemic infarction or hemorrhage into a pituitary tumour. Clinically the patient develops sudden headache, meningismus, visual disturbances, even blindness, and occasionally decrease in level of consciousness. To diagnose it is basic to perform a cerebral MRI. Treatment consists in urgent sellar decompression by transsphenoidal surgery and substitute therapy with steroids.     

A case of pituitary apoplexy with severe consciousness disturbance following mild head trauma]

A rare case of pituitary apoplexy following mild head trauma is reported. A 56-year-old woman was referred to our hospital for loss of consciousness following a traffic accident. Magnetic resonance (MR) imaging showed a large suprasellar tumor containing a low intensity area on the T 1-, T 2-weighted images and fluid-attenuated inversion recovery (FLAIR) image. These findings indicated fresh intratumoral hemorrhage. The tumor extended to the suprasellar region and put pressure on the hypothalamus and brain stem. The tumor, with marked intratumoral hemorrhage, was resected with an emergency right front-temporal craniotomy. And the patient was left severely disabled. The tumor histology was diffuse-type pituitary adenoma. In our case, we suggest that the cause of pituitary apoplexy following head trauma was shearing stress between the intra- and suprasellar part of the easy bleeding tumor, or direct injury by bone structure such as teberculum sellae, dorsum sellae, or anterior clinoid process.     

Pituitary apoplexy as a cause of internal carotid artery occlusion.

Occlusion of intracranial arteries by pituitary apoplexy with resulting infarction is a rare occurrence. A 50-year-old man who presented with a history of sudden onset of frontal headache and visual impairment was admitted to another medical centre and MRI revealed a non-enhancing sellar lesion with suprasellar and infrasellar extension. Thereafter, the patient's consciousness deteriorated progressively and he showed signs of herniation; he was then referred to our centre for further evaluation. CT scanning revealed infarction of the left internal carotid artery territory. Transcranial resection of the tumour followed by a large decompressive craniotomy restored the blood flow in the internal carotid artery. Histological examination revealed the tumour to be a pituitary adenoma that contained formed blood elements. The patient's neurological status did not improve and he died on the ninth postoperative day despite vigorous treatment for controlling intracranial pressure. This case study documents a rare presentation of pituitary apoplexy that caused signs of raised intracranial pressure due to mechanical obstruction of an internal carotid artery with resulting infarction.     

岩下窦静脉取血在疑难ACTH依赖性库欣综合征诊断中的应用

目的 探讨岩下窦静脉取血(IPSS)在疑难库欣综合征诊断中的价值.方法 对20例疑难库欣综合征行岩下窦和外周静脉取血,测定血ACTH浓度比,评价其对库欣病诊断的敏感性和特异性.结果 18例患者的岩下窦与外周静脉血ACTH的比值＞2,其中13例行经蝶窦垂体术后病理为垂体ACTH腺瘤;1例行鞍区γ-刀治疗、4例行生长抑素治疗后病情缓解.2例岩下窦与外周静脉血ACTH的比值＜2,其中1例为右肺类癌,另1例为垂体ACTH腺瘤.本组IPSS诊断库欣病的敏感性和特异性分别为93%和100%.结论 IPSS操作安全,并发症少,可作为疑难库欣综合征的重要鉴别方法.