Pituitary apoplexy of a giant prolactinoma during pregnancy.

AIMS AND METHODS: Prolactinomas are a common cause of sexual dysfunction and infertility. We aimed, through this case report, to illustrate the difficulties of management of women with giant prolactinoma, especially in cases of desire of pregnancy. RESULTS: A 30-year-old woman was referred to our department for secondary amenorrhea. Investigations showed a prolactin level of 5168 ng/mL and giant pituitary adenoma of 4 cm in diameter. Cytoreductive surgery was performed after failure to normalize prolactin levels during three years with medical treatment by cabergoline. After seven months, menstrual cycles have resumed, and after 13 months, the patient became pregnant. At 22nd week of gestation, she was admitted in our hospital for pituitary apoplexy. Medical treatment with bromocriptine was chosen. The vaginal premature delivery at 28 weeks gave birth to twins weighing 1 Kg each who died on the 7th day of life. CONCLUSION: This is a relevant clinical case that illustrates the efficacy of cytoreductive surgery in case of insufficient response to dopamine agonists to restore gonadal function. The possibility of a pregnancy should be considered in these patients since it can be associated with high maternal and fetal risks.

Pituitary Apoplexy: A Comprehensive Review.

Pituitary apoplexy (PA) is a rare, usually vision-threatening and occasionally life-threatening entity. The exact incidence of PA in large series of pituitary adenomas is variable; however, it is more common in macroadenomas and nonfunctioning adenomas though it has been reported rarely in microadenomas. There are several well-known precipitating factors for PA including the use of anticoagulants, surgery, head trauma, pregnancy, etc. The typical clinical presentation is characterized by the near universal presence of headache with or without the following: visual disturbances, extraocular palsies, altered sensorium. MRI is the imaging modality of choice. Most patients have hormonal and/or electrolyte disturbances at the time of presentation which needs to be quickly corrected. Both conservative and surgical treatment modalities have been advised for the management of this condition. However, on the basis of the evidence available in the literature, the treatment should be individualized for each patient with PA. If conservative management is chosen, close clinical monitoring is necessary for early identification of deterioration. Surgery generally is through the trans-sphenoidal route. Most patients have a good recovery in visual function and extraocular palsy. There is some evidence in the literature that surgical intervention, when necessary, should be undertaken early as it is associated with better visual outcome. The majority of the patients will have residual hormonal deficits which will require prolonged hormone replacement therapy. There is a small but significant risk of recurrent PA in patients with residual tumors, especially, in those with large tumor residues. There is also a small risk of tumor recurrence following PA and hence all patients should undergo surveillance imaging periodically to detect the possible recurrence of tumor. In recent years, the mortality from PA has decreased significantly. A high index of suspicion and prompt multidisciplinary management will often lead to an overall good outcome.

Takotsubo cardiomyopathy and pituitary apoplexy: a case report.

BACKGROUND: Takotsubo cardiomyopathy (TTC) has been widely recognized in recent decades and is triggered by either physical or psychological stressors. CASE PRESENTATION: A 70-year-old woman presented to the Emergency Department due to confusion, hypotension, fever, chills, and cough. She had a one-year history of diabetes insipidus. Pituitary function examination at admission revealed decreased thyroid, sex and adrenal hormones. Pituitary MRI displayed findings suggestive of nonhemorrhagic pituitary apoplexy. Electrocardiogram (ECG) revealed T-wave inversion and extended QT interval. Transthoracic echocardiogram (TTE) showed left ventricular apical dysplasia and ballooning, accompanied by reduced left ventricular ejection fraction. Coronary angiography (CAG) revealed no obvious coronary arterial stenosis. The left ventriculogram demonstrated an octopus clathrate appearance. Most ECG and TTE changes recovered 10 days later. CONCLUSIONS: To the best of our knowledge, this is the first report of newly diagnosed TTC associated with pituitary apoplexy.

Rathke Cleft Cysts with Apoplexy-Like Symptoms: Clinicoradiologic Comparisons with Pituitary Adenomas with Apoplexy.

BACKGROUND: Rathke cleft cyst (RCC) can cause acute symptoms mimicking pituitary adenoma (PA) apoplexy. We evaluated the clinicoradiologic features for distinguishing RCC from PA apoplexy. METHODS: We retrospectively evaluated 22 patients with RCC and 24 patients with PA with apoplexy-like symptoms who underwent surgery via a transsphenoidal approach between November 1999 and December 2016. We compared the clinical data and magnetic resonance (MR) images between the 2 groups. RESULTS: The RCC group was younger and had smaller tumors compared with the PA group (P = 0.02 and 0.001, respectively). The incidences of visual deficits and cranial nerve palsy were lower in the RCCs than in the PAs (P ≤ 0.02 for all). MR images showed more frequent intracystic nodules in the RCCs (P < 0.001), whereas nodular enhancement and lateral deviation of the pituitary stalk were more commonly seen in the PAs (P ≤ 0.003 for both). However, the presence of endocrine dysfunction or decreased consciousness, and the recurrence ratio, were not significantly different between the groups (P ≥ 0.48 for all). In the multivariable logistic regression analysis, patients without nodular enhancement had a 15.84-fold greater risk of RCC than did those with nodular enhancement (P = 0.031). The probability of RCC decreased 0.59-fold with each 1-cm3 increase in tumor volume. CONCLUSIONS: RCC with apoplexy-like symptoms has different clinicoradiologic features compared with PA apoplexy. Patients with RCC present with milder ocular symptoms and smaller tumor volumes compared with those with PA apoplexy. The absence of nodular enhancement on MR images could suggest RCC.

Pituitary apoplexy mimicking meningoencephalitis: case report and scoping study.

Background: Pituitary apoplexy (PA) is a rare but potentially life-threatening condition that may require urgent surgical intervention.Case Presentation: We report a case of a patient who was initially diagnosed with meningoencephalitis (ME) based on clinical presentation and cerebrospinal fluid (CSF) analysis, but was eventually diagnosed with PA. We present a summary of other cases reported in the literature of PA mimicking ME and analyze their clinical features and CSF findings.Results: Among all 22 PA cases reviewed, headache was the most commonly reported symptom. Hypopituitarism was seen in 94.4% of the cases; of these, panhypopituitarism was noted in 38.9%. The sensitivity of magnetic resonance image (MRI) for detecting PA was 94.7%, much higher than that of computed tomography (CT), which was only 31.6%. Neutrophil predominant pleocytosis was present in all cases with a neutrophil percentage ranging from 73% to 98%. CSF leukocyte count was less than 1000/ul in 86% of the cases. CSF erythrocytosis was seen in 92.9% of the cases with a count ranging from 15 to 2030/ul. Elevated CSF protein was present in all cases with a range of 69.8 to 239 mg/dl. CSF glucose level varied with a range between 12 and 136 mg/dl; the level was greater than 40 mg/dl in 73% of the cases.Conclusion: PA tends to be misdiagnosed as ME due to the similarities of semiology and CSF findings. PA should be considered in refractory acute headache cases, especially those with visual and endocrine abnormalities. Early recognition and treatment may lead to significant reduction in morbidity and mortality.Abbreviations: ACTH: adrenocorticotropic hormone; CSF: cerebrospinal fluid; CT: computed tomography; GRE: gradient echo; HRT: hormone replacement therapy;HSV: Herpes Simplex Virus; IV: intravenous; ME: meningoencephalitis; MRI: magnetic resonance image; PA: pituitary apoplexy; RBC: red blood cell; WBC: white blood cell.

Visual and Endocrine Recovery Following Conservative and Surgical Treatment of Pituitary Apoplexy: A Meta-Analysis.

BACKGROUND: Pituitary apoplexy (PA) can manifest with visual and endocrine defects. The literature lacks strong support for either surgical or conservative management with respect to symptomatic improvement of these deficits. This meta-analysis compared visual and endocrine outcomes in conservative and surgical treatment of PA. METHODS: A systematic literature search was performed in PubMed, Cochrane, and Ovid MEDLINE for articles published between 1988 and 2018. Recovery outcomes were binarized, such that complete and partial improvements were combined as "improvement." Primary outcome variables evaluated via a binary random-effects model were improvements in endocrine dysfunction, visual field and acuity deficits, and ophthalmoplegia or ocular nerve palsy. RESULTS: Of 483 published articles, 14 studies comprising 457 cases (259 surgical treatments and 198 conservative treatments) were included. On initial examination, 58% of patients had endocrine dysfunction, 37% had visual acuity or field deficit, and 47% had ophthalmoplegia or ocular nerve palsy. Evaluation of outcomes for surgically and conservatively treated patients yielded odds ratios of 0.609 (95% confidence interval [CI], 0.199-1.859; P = 0.383), 0.763 (95% CI, 0.307-2.374; P = 0.763), 1.167 (95% CI, 0.433-3.146; P = 0.760), and 0.801 (95% CI, 0.305-2.105; P = 0.653) for improvements in endocrine dysfunction, visual acuity dysfunction, visual field dysfunction, and ophthalmoplegia or ocular nerve palsy. CONCLUSIONS: Both surgical intervention and conservative management of PA can lead to visual and endocrine recovery, although the management decision may heavily rely on severity of initial deficits. Treatment of PA can be multifaceted and tailored to the individual case and clinical judgment. Further investigation into appropriate intervention based on longitudinal outcome data is warranted.

Pituitary Apoplexy: Results of Surgical and Conservative Management Clinical Series and Review of the Literature.

OBJECTIVE: Pituitary apoplexy is associated with visual, cranial nerve, and endocrine dysfunction. In this article, the results of surgical and conservative management of pituitary apoplexy in a single center are evaluated and a review of the literature is presented. METHODS: A retrospective analysis was made of patients with pituitary apoplexy who underwent surgery or conservative management at our center between January 2007 and June 2017. Surgery was typically selected for patients who presented with acute deterioration of visual status and/or level of consciousness. Patients with no visual field deficit and those who had medical contraindications to undergo a surgical procedure because of previous comorbidities typically had conservative treatment. Baseline characteristics and clinical and radiologic outcomes were reviewed. A review of the literature (1990-2018) was performed according to PRISMA guidelines. Studies comparing the results of conservative and surgical management were identified. Visual, cranial nerve, and endocrine outcomes and tumor recurrence were analyzed. RESULTS: Forty-nine patients (73.1%) were managed surgically and 18 (26.9%) conservatively. After careful case selection, patients underwent surgical or conservative treatment. Patients who underwent conservative treatment had fewer visual deficits. At diagnosis, visual deficit (38.8% vs. 75.5%; P = 0.008) and cranial nerve palsy (27.7% vs. 51%; P = 0.058) were less common in the conservative group. Conservative and surgical treatments had similar visual and cranial nerve improvement rates (75% vs. 58.3%, P = 0.63 and 75% vs. 69.2%, P = 1.0, respectively). In the conservative group, tumor shrinkage was observed in 76.4% of cases. The systematic review retrieved 11 studies. No significant difference between conservative and surgical treatment for clinical outcomes (visual field recovery, odds ratio [OR], 1.45; 95% confidence interval [CI], 0.72-2.92; cranial nerve recovery, OR, 2.30; 95% CI, 0.93-5.65; and hypopituitarism, OR, 1.05; 95% CI, 0.64-1.74) or tumor recurrence (OR, 0.68; 95% CI, 0.20-2.34) was observed. CONCLUSIONS: A tailored approach to pituitary apoplexy, one that does not include an absolute need for surgery, is appropriate. Conservative management is appropriate in selected patients presenting without visual deficits.

Endoscopic Endonasal Surgery for Pituitary Apoplexy: Evidence On a 75-Case Series From a Tertiary Care Center.

BACKGROUND: The optimal management of pituitary apoplexy (PA) remains debated. The aim of this study was to assess the outcome of the transsphenoidal approach for PA in a large surgical experience. MATERIALS: Each consecutive case of PA consecutively operated by endoscopic endonasal approach from our tertiary care center, from 1998 to 2015, was included in this series. RESULTS: Seventy-five patients (47 male; mean age 52.4 ± 16.2 years) were included. Mean follow-up was 69.3 ± 46.7 months. On admission, all patients presented with abrupt severe headache (100%), associated with anterior hypopituitarism in 51 patients (68%), visual disturbances in 55 (73.4%), ophthalmoplegia in 38 (50.7%), and a remarkable reduction of consciousness in 2 (2.6%). Apoplexy proved to be ischemic in 35 patients (46.7%) and hemorrhagic in 40 (53.3%). Patients with hemorrhagic necrosis presented more often with major suprasellar expansion (P = 0.012) Radical removal was achieved in 60 cases (80%). Surgical morbidity consisted in one case of postoperative cerebrospinal fluid leak (1.3%). Anterior hypopituitarism worsened in 15 cases (20%), and diabetes insipidus occurred in 4 cases (5.3%). Ophthalmoplegia improved/normalized in 71% and visual symptoms in 85.5% of the patients, with better results achieved in ischemic forms (P = 0.043). The 2 comatose patients regained normal consciousness. CONCLUSIONS: The endoscopic endonasal approach represents a valid, effective, and safe technique in the management of PA. Favorable outcomes can be achieved by referring patients to dedicated pituitary centers with a multidisciplinary team. Further studies are still needed to define criteria for surgical indication and to identify outcome predictors.

[PITUITARY ADENOMA APOPLEXY].

We present 3 cases of pituitary adenoma apoplexy in young women (one microadenoma and two macroadenomas). The possible risk factors, clinical presentation and outcome of pituitary apoplexy are discussed. Diagnostic errors as regards pituitary adenoma apoplexy are analyzed.

A primer on pituitary injury for the obstetrician gynecologist: Simmond's disease, Sheehan's Syndrome, traumatic injury, Dahan's Syndrome, pituitary apoplexy and lymphocytic hypophysitis.

The pituitary gland plays a critical role in reproduction. In response to the hypothalamus the anterior pituitary secretes prolactin, thyroid-stimulating hormone, adreno-corticotropic hormone, follicle-stimulating hormone, luteinizing hormone and growth hormone. Dysregulation in these hormones often lead to reproductive failure. Multiple mechanisms of pituitary injury exist. Simmond's disease is atrophy or destruction of the anterior lobe of the pituitary gland resulting in hypopituitarism. Sheehan's syndrome is post-partum pituitary injury due to massive hemorrhage. Traumatic injury resulting in hemorrhage in a non-pregnancy state can also cause partial or complete pituitary failure. Dahan's syndrome is pituitary injury due to severe vasospasm, without significant hemorrhage. Pituitary apoplexy is infarction of a pituitary adenoma and intra-mass hemorrhage with result injury to hormone production by the gland. Lymphocytic infiltration is the most common cause of hypophysitis and the mechanism is often unknown, although it may be autoimmune-related. The mechanism and treatments of each of these pathologies will be discussed in a context of reproduction.

Pituitary apoplexy masquerading as functional decline in an older person.

We report the case of an older lady who was admitted to a tertiary teaching hospital with sub-acute functional decline of two months' duration, which was initially attributed to a concomitant urinary tract infection. Further investigations, however, revealed the diagnosis of pituitary apoplexy with central hypocortisolism. Subsequent treatment with physiological doses of steroids improved patient's function and overall well-being. This report adds to the sparse literature on pituitary apoplexy in the older adults and emphasizes the non-specific presentation of this clinical syndrome.

Complex effects of apoplexy secondary to pituitary adenoma.

Pituitary adenoma apoplexy is a well-known clinical syndrome induced by insulin infusion, cardiac surgery, trauma, and hypothalamic releasing factors. Pituitary apoplexy can cause secondary cerebral infarct and internal carotid artery occlusion. With blockade of tumor perfusion, apoplexy triggers a sudden onset of headache, visual impairment, cranial nerve palsy, disturbances of consciousness, eyelid ptosis, and hemiparesis. However, pituitary adenoma cells with high metabolic demand cannot survive with deficient blood supply and glucose concentrations. Moreover, a number of case reports have shown that spontaneous remission of syndromes, such as acromegaly, may be caused by pituitary adenoma after apoplexy. Therefore, understanding mechanism that underlies the balance between pituitary adenoma apoplexy and subsequent spontaneous remission of syndromes may suggest new approaches for treatment of pituitary adenoma apoplexy.

Surgical treatment of pituitary apoplexy in association with hemispheric infarction.

We report a patient with pituitary apoplexy in whom cerebral infarction developed, possibly secondary to vasospasm. Pituitary apoplexy is a clinical syndrome caused by acute hemorrhage or infarction of the pituitary gland. Our patient's clinical symptoms and radiographic findings greatly improved after surgical resection of the apoplectic pituitary gland. An extensive literature review was performed, including all previously reported cases of pituitary apoplexy leading to cerebral infarction. The clinical features, pathophysiological mechanisms, management and outcome of cerebral infarction following pituitary apoplexy are discussed. We show that cerebral infarction following pituitary apoplexy is associated with much poor prognosis. Early surgical decompression of the tumor and hemisphere should be performed in patients with severe or progressive neurological deficits, however, those with less severe presentations may be treated conservatively or with delayed elective surgery.

Pituitary apoplexy: pathophysiology, diagnosis and management.

Pituitary apoplexy is characterized by sudden increase in pituitary gland volume secondary to ischemia and/or necrosis, usually in a pituitary adenoma. Most cases occur during the 5th decade of life, predominantly in males and in previously unknown clinically non-functioning pituitary adenomas. There are some predisposing factors as arterial hypertension, anticoagulant therapy and major surgery. Clinical picture comprises headache, visual impairment, cranial nerve palsies and hypopituitarism. Most cases improve with both surgical and expectant management and the best approach in the acute phase is still controversial. Surgery, usually by transsphenoidal route, is indicated if consciousness and/or vision are impaired, despite glucocorticoid replacement and electrolyte support. Pituitary function is impaired in most patients before apoplexy and ACTH deficiency is common, which makes glucocorticoid replacement needed in most cases. Pituitary deficiencies, once established, usually do not recover, regardless the treatment. Sellar imaging and endocrinological function must be periodic reevaluated.

Management of clinically non-functioning pituitary adenoma.

Clinically NFPA is currently the preferred term for designing all the pituitary adenomas which are not hormonally active (in other words, not associated with clinical syndromes such as amenorrhea-galactorrhea in the context of prolactinomas, acromegaly, Cushing's disease or hyperthyroidism secondary to TSH-secreting adenomas). They account for 15-30% of pituitary adenomas. Diagnosis is usually made either in the context of mass effect due to a macroadenoma or, increasingly, fortuitously during imaging performed for some unrelated purpose; the latter case is known as pituitary incidentaloma. Surgery is indisputably indicated in case of tumoral syndrome, but other aspects of NFPA (hormonal work-up, follow-up, and especially postoperative follow-up, management of remnant or recurrence, the special case of incidentaloma, or apoplexy) remain controversial. The French Endocrinology Society (SFE) therefore set up an expert working group of endocrinologists, neurosurgeons, ophthalmologists, neuroradiologists, pathologists and biologists to draw up guidelines, at the 2012 SFE Congress in Toulouse, France. The present article presents the guidelines suggested by this group of French-speaking experts.

Management of endocrine disease: pituitary tumour apoplexy.

Pituitary tumour apoplexy (PA) is a rare clinical syndrome that occurs as a result of acute haemorrhage and/or infarction within a frequently undiagnosed pituitary tumour. The sudden enlargement of the pituitary mass undergoing PA is responsible for a wide range of acute symptoms/signs (severe headache, visual loss, diplopia, hypopituitarism, impaired consciousness) which, together with the radiological evidence of a pituitary lesion, establish the diagnosis. The optimal care of PA requires involvement of a multidisciplinary team including endocrinologist, neurosurgeon, neuroophthalmologist and the management strategy that depends on the clinical manifestations, as well as the presence of co-morbidities. Prompt surgical decompression is initially indicated in cases with severe or progressive impairment of the visual acuity or the visual fields or with altered mental state and leads to visual and neurological recovery in most of the patients. The patients with mild, stable clinical picture (including those with isolated ocular palsies) can be managed conservatively (support of fluid and electrolyte balance and stress doses of steroids in most cases) with favourable visual and neurological outcome. Frequent reassessment is mandatory because the clinical course can be unpredictable; if progression of symptoms occurs, later elective surgery is indicated and is beneficial, especially in terms of visual outcome. The endocrinological outcome is less favourable, irrespective of the treatment option, with many patients remaining on long-term replacement therapy. Despite the above guidelines, clear proof of optimal outcomes in the form of randomised controlled trials is lacking. Regrowth of the pituitary tumour years after a PA episode is possible and patients require long-term surveillance.