Hypothalamic-pituitary function and radiographic evaluation of women with hyperprolactinemia and an "empty" sella turcica.

A primary "empty" sella may be associated with significant hypothalamic-pituitary dysfunction. This report characterizes the endocrinologic and radiographic findings in six patients with hyperprolactinemia (range 34.3 to 1,170 ng/ml) ultimately found to have an enlarged empty sella. Lateral skull x-ray films and thin-section tomograms were suggestive of pituitary tumors in all patients. Four patients underwent transphenoidal sellar exploration after pneumoencephalography (PEG) failed to demonstrate air within the sella. The inability of PEG to demonstrate an empty sella in two patients was explainable on the basis of an intact diaphragma sellae with previous transient intrasellar pathology responsible for the sellar enlargement. Dynamic hypothalamic-pituitary testing yielded no consistent pattern of response. These studies suggest that an empty sella: (1) may be associated with hyperprolactinemia, regardless of etiology, (2) is not diagnosable by dynamic hormonal testing, and (3) may be indistinguishable from a pituitary tumor by current radiographic techniques.     

Familial hypopituitarism associated with mosaic form of Turner syndrome.

We present herein an unusual coincidence of familial hypopituitarism associated with a mosaic form of Turner syndrome in two adult sisters (51 and 43 years old). Both patients had hypopituitarism diagnosed in childhood. They have never been administered growth hormone, and remained short in stature. They were not given long-term estrogen-progestin treatment, despite lack of menstruation. Early in childhood both received thyroid hormone substitution. Pituitary imaging revealed pituitary hypoplasia with partial empty sella in one sister, and pituitary hypoplasia in the other. Very recently, during endocrinological evaluation, they were diagnosed with a mosaic form of Turner syndrome, additionally to their hypopituitarism. In this paper, we place special emphasis on the results of hormonal analyses and discuss the differential diagnosis.     

Macroprolactinomas with suprasellar extension: effect of bromocriptine withdrawal during one or more pregnancies.

OBJECTIVE: To investigate the effects of bromocriptine withdrawal during one or more pregnancies in patients who presented with pituitary macroprolactinomas with suprasellar extension. DESIGN: Four infertile patients presenting with a macroprolactinoma with suprasellar extension conceived during treatment with bromocriptine on 10 occasions resulting in eight full-term normal deliveries. Treatment was withheld shortly after conception in each pregnancy. RESULTS: Serum prolactin (PRL) levels fell initially from a mean of 2,776 (range 1,682 to 4,515) to 27 micrograms/L (range 1 to 71) with the development of a partially empty sella in all patients. Recovery of visual field defects occurred in the only affected individual. In case 1, PRL levels remained within the normal range, after bromocriptine withdrawal in the first pregnancy, with the development of an empty sella. Prolactin levels, however, increased substantially in cases 2 to 4. An asymptomatic suprasellar tumor extension returned in cases 2 and 3. After two or more pregnancies (cases 1, 3, and 4), there was a progressive decline in the serum PRL levels. Although still elevated in cases 3 and 4, the PRL levels were considerably below those obtained at presentation or in the first pregnancy. Tumor regression with the development of an empty sella was observed in both these patients as well in their pregnancy or postpartum period. CONCLUSIONS: Bromocriptine may be safely withdrawn during pregnancy in patients presenting with a macroprolactinoma. With multiple bromocriptine induced pregnancies, PRL levels and tumor size may progressively decrease with the eventual development of an empty sella.     

Evaluation of the pituitary. Patients with suspected prolactin-producing tumors

We have reviewed our experience in the radiographic and ophthalmologic evaluation of 1001 patients with symptoms suggesting the presence of a pituitary, prolactin-secreting adenoma. Twenty-seven patients had abnormal or suspicious radiographic examination of the sella turcica. Twenty-two of those had hyperprolactinemia. In only one instance was an abnormality noted on polytomography that was not seen on a conventional four-view study of the skull. Based on these findings, a four-view plain conventional radiographic assessment of the skull suffices as a screening procedure in patients with amenorrhea, galactorrhea, or both. Thin section tomography should be reserved to more thoroughly evaluate those patients with elevated serum prolactin concentrations and/or abnormal conventional radiographs. We found visual field testing to be of little value as an initial screening procedure in these patients.     

Intrasellar subarachnoid herniation or empty sella associated with galactorrhea

Eight of 115 patients with empty sella had concurrent galactorrhea. All 8 patients had abnormal sellae, and the diagnosis of empty sella was made by polytome pneumoencephalography. There were no obvious endocrine dysfunctions, but 2 patients had elevated prolactin levels. One mechanism for production of galactorrhea may involve compression of the hypothalamus and/or pituitary stalk; this was suggested by most of our observations. There may be a coincidental association of empty sella with galactorrhea, and this association is probably more common than previously noted. Evaluation of patients with galactorrhea and abnormal sellae by polytome pneumoencephalography is emphasized.     

Primary empty sella syndrome and amenorrhea.

The empty sella syndrome is defined anatomically and radiologically. A case report of an amenorrheic patient who was diagnosed as having the primary empty sella syndrome is presented. Its pathogenesis, clinical profile, and endocrine relationships are discussed. Specific reference is directed toward the capability of the empty sella syndrome to produce pituitary impairment and possibly amenorrhea.     

Prolactin-secreting pituitary microadenoma: detection and evaluation

Eleven women with secondary amenorrhea associated with hyperprolactinemia were studied. Base line evaluations, visual field determinations, and routine sella turcica x-rays were normal. Prolactin-secreting pituitary microadenomas were found in all of the patients only after further diagnostic studies were carried out. These studies included polytomography of the sella turcica; dynamic pituitary testing of growth hormone reserve, adrenocorticotropic hormone reserve, and gonadotropin reserve; and prolactin suppression with L-dopa. The early diagnosis of a small prolactin-secreting adenoma may be possible if several diagnostic criteria are utilized. The most sensitive diagnostic indices available are (1) polytomography, (2) the magnitude of the plasma prolactin elevation, and (3) the failure to suppress prolactin secretion with L-dopa. Our findings emphasize the importance of an extensive evaluation of all women with amenorrhea associated with hyperprolactinemia.     

Four decades without diagnosis: Sheehan‘s syndrome,a retrospective analysis

Aim: Sheehan‘s syndrome (SS) remains a frequent cause of hypopituitarism in undeveloped and developing countries, but due to improvements in obstetric care, it is rare in developed countries. We aimed to share the results of a retrospective study analyzing the demographic, clinical, imaging, and hormonal characteristics of a large group of patients with SS, and also increase awareness of this syndrome especially in developed countries.

Methods: The medical records of 124 patients with SS patients who were followed up in the Endocrinology Department of Dicle University between 1995 and 2015 were assessed retrospectively.

Results: The mean period of diagnostic delay was 20.37?±?8.34 years on average. 5.7% of patients with SS were literate; 62% of patients delivered at home. Anemia was identified in 64.5% of SS patients. Mean blood sodium levels were 129.8?±?11.3 mEq/L. The mean urine densities were 1013?±?6.5. Osteoporosis and osteopenia were found in 44 (35.4%) and 71 (57.2%) patients, respectively, According to pituitary magnetic resonance imaging (MRI) analyses, 92 (74.2%) patients with SS had completely empty sella, 29 (23.3%) had partially empty sella, and 1 patient had microadenoma, and 2 had normal pituitary MRI results.

Conclusions: Improved obstetric care and effective interventions for postpartum hemorrhage have limited the prevalence of SS in developed countries. However, in developing countries like Turkey, SS due to postpartum bleeding remains common. Thus, physician’s awareness of the symptoms of SS is urgently required to avoid the associated morbidity and mortality.     

Sheehan’s syndrome

Abstract

Sheehan’s syndrome (SS) is characterized by various degrees of hypopituitarism, and develops as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. Increased pituitary volume, small sella size, disseminated intravascular coagulation and autoimmunity are the proposed factors in the pathogenesis of SS. Hormonal insufficiencies, ranging from single pituitary hormone insufficiency to total hypopituitarism, are observed in patients. The ?rst most important issue in the diagnosis is being aware of the syndrome. Lack of lactation and failure of menstrual resumption after delivery that complicated with severe hemorrhage are the most important clues in diagnosing SS. The most frequent endocrine disorders are the deficiencies of growth hormone and prolactin. In patients with typical obstetric history, prolactin response to TRH seems to be the most sensitive screening test in diagnosing SS. Other than typical pituitary deficiency, symptoms such as anemia, pancytopenia, osteoporosis, impairment in cognitive functions and impairment in the quality of life are also present in these patients. Treatment of SS is based on the appropriate replacement of deficient hormones. Growth hormone replacement has been found to have positive effects; however, risk to benefit ratio, side effects and cost of the treatment should be taken into account.     

Primary empty sella syndrome with elevated serum prolactin

A young, adult, white female with long-standing amenorrhea-galactorrhea syndrome and known pituitary enlargement since 1969 is presented. Further evaluation revealed PRL levels elevated in the microadenoma range and an empty sella. The presence of a pituitary adenoma, however, could not be confirmed by our studies. The question now arises--in a young woman desirous of pregnancy, should an induction of ovulation be attempted in view of the elevated serum PRL and an empty sella?     

The effect of multiparity on intrasellar prolactinomas

A complete reassessment of ovulation, pituitary reserve and function, and sella turcica anatomy was carried out in nine multiparous patients with intrasellar prolactinomas to determine whether long-term bromocriptine therapy was required and to document the natural history of the disease after two or more pregnancies. After the last pregnancy, bromocriptine was discontinued and pituitary function and anatomy and prolactinoma activity were reassessed with documentation of ovulation (basal body temperature graphs and menstrual history), search for fat droplet-positive galactorrhea, pituitary fossa tomography, computerized tomographic scan, triple-bolus testing, and visual fields. These data were compared with a similar workup carried out prior to the first pregnancy. Three groups of eventual outcomes were identified radiologically. Anterior pituitary gland function and reserve remained normal in all, and no neurological sequelae were noted. Four patients did not require long-term treatment. A hypothesis of autoinfarction of the adenoma is raised, since three patients were shown to have empty sellae.     

A practical approach for the evaluation of women with abnormal polytomography or elevated prolactin levels

Based upon the experience gained in the evaluation of 60 patients with abnormal polytomography and/or elevated prolactin levels, the following observations can be made: Patients with amenorrhea, amenorrhea and galactorrhea, galactorrhea alone, or anovulatory cycles and infertility may or may not have pituitary tumors. Clinical symptoms do not always correlate with the prolactin level, and patients with normal prolactins may have pituitary tumors. The incidence of empty sella is significant (15.8% in this series). Visual field examination is not a useful screening procedure, but evaluation of thyroid function is important to detect the occasional patient with hypothyroidism (3.5% in this series). The insulin tolerance test is not helpful in detecting the presence of pituitary tumors or in guiding management decisions, and the CT scan contributes little and should be omitted from the evaluation process. A straightforward, economical, and efficient approach to this clinical problem is presented.     

Coexistent empty sella and prolactin-secreting microadenoma

The empty sella turcica may be found in people with no antecedent history of intracranial disease, as well as in those with known pituitary pathology or following therapy to the pituitary gland. We have evaluated 3 women with galactorrhea and hyperprolactinemia, 2 of whom had amenorrhea. Each had an empty sella. In all cases polytomograms demonstrated asymmetry of the sella floor with focal bony erosion, conventional pneumoencephalography showed intrasellar air, and polytomographic pneumoencephalography confirmed air limited to one side of the pituitary fossa with tumor and/or residual normal tissue on the opposite side. In 2 patients who had extensive endocrine evaluation, pituitary function was normal with the exception of hyperprolactinemia. Transsphenoidal excision of microadenomas resulted in postoperative normalization of the serum prolactin concentration and resumption of regular menses in the previously amenorrheic women.     

Adrenal adenocarcinoma and empty sella syndrome in a 37-year-old woman.

The case of a 37-year-old woman with secondary amenorrhea and clear signs of hyperandrogenism is reported. The patient underwent hormonal evaluation including circadian rhythm of cortisol, gonadotropin-releasing hormone/thyroid-stimulating hormone (GnRH/TRH) test, corticotropic-releasing hormone (CRH) test and dexamethasone suppression test. She also underwent pelvic and adrenal ultrasound examination, adrenal computed axial tomography (CAT) scan and cranial nuclear magnetic resonance (NMR). A mass about 10 cm in size was detected in the left adrenal region. The sella was empty and the pituitary displaced downward. Suspected adrenal adenocarcinoma was confirmed by histological examination after surgical removal of the mass. This case is of interest for physicians because of the mixed androgen and cortisol secretion of the adenocarcinoma in a hyperprolactinemic patient with empty sella. Moreover, it suggests the need to investigate the adrenal gland in patients with hyperprolactinemia and hirsutism.     

Anorexia nervosa in woman with Turner's syndrome

The authors report a case of anorexia nervosa in woman with Turner's syndrome (45, X). In this case there was a low blood concentration of LH and FSH, and there was a lack of reaction to intravenous administration of 100 micrograms of GnRH. Partial syndrome of "empty sella" was found in a presented case by use of magnetic resonance imaging. There was a connection between the onset of anorexia nervosa and the tragic death of the patient's father. The inconsiderate start with HRT aggravated a course of a anorexia nervosa. The authors considered the plausible existence of relations between "empty sella" and the development of anorexia nervosa.     

Prolactin response to the dopamine antagonists sulpiride and domperidone. Further evidence for pituitary dopamine deficiency in hyperprolactinemic disorders of different etiology

The PRL response to the dopamine antagonists sulpiride (100 mg i.m.) or domperidone (2 or 8 mg i.v.) was evaluated in healthy controls and in 148 patients with different hyperprolactinemic disorders (50 with idiopathic hyperprolactinemia, 58 with microprolactinoma, 19 with macroprolactinoma, 2 with empty sella, 8 with acromegaly, 7 with organic lesions of the hypothalamus, and 4 with idiopathic hypopituitarism of presumed hypothalamic origin). Mean PRL response to both drugs was significantly lower in all groups of patients than in controls, and significantly higher in subjects with idiopathic hyperprolactinemia than in those with pituitary adenomas or hypothalamic disease. Absent or impaired PRL responses were found in 38% of idiopathic patients, in 91.5% of microprolactinomas and in all of the patients with either macroprolactinoma, acromegaly, or hypothalamic disorders. Since the PRL response to dopamine antagonists depends on the presence of an endogenous dopaminergic tone, it is suggested that these figures reflect the incidence of major dopamine deficiency at pituitary lactotrophs in different hyperprolactinemic states. These data suggest that the pathophysiology of hyperprolactinemia in many patients with idiopathic disease is different from that of microprolactinoma. However, the finding of a normal PRL response to sulpiride in some subjects with radiologically or surgically proven microprolactinoma indicates that this test has no diagnostic value in the individual case.     

Continuous bromocriptine treatment of empty sella syndrome aggravating pregnancy. A case report.

Pregnancy, aggravated by hyperprolactinaemic empty sella syndrome, is a risky and rare event. A case with such complication and continuous bromocriptine treatment during the whole gestation is presented. Neither fetal abnormalities nor deterioration of maternal pituitary pathology or the course of gestation are observed.     

Normalization of visual fields following bromocriptine treatment in hyperprolactinemic patients with visual field constriction

Two patients with galactorrhea-amenorrhea and bilateral visual field defects were studied. Routine radiologic examination of each patient revealed a normal sella turcica and no demineralization of the posterior clinoid process. Serum prolactin levels were elevated (patient V. G., 80 ng/ml; patient S. R., 204 ng/ml). Within 2 months of bromocriptine therapy, the serum prolactin levels were normal (patient V. G., 12.21 ng/ml; patient S. R., 8.25 ng/ml) and the bilateral visual field defects were corrected. Bromocriptine has been shown to control prolactin secretion in patients with prolactin-secreting pituitary tumors. Normalization of restricted visual fields following bromocriptine therapy indicates the possibility of an anatomical regression of pituitary hyperplasia or an underlying prolactin-producing microadenoma. It is speculated that the modality of functional galactorrhea reflects hyperplasia of the lactotrophs preceding a nodular and ultimately an adenomatous change. The continuous and prolonged administration of bromocriptine may prevent such a progressive sequence. Further experience is required to validate this possibility.     

Two-stage cervical cancer screening: an alternative for resource-poor settings

OBJECTIVE: We sought to introduce 2-stage cervical cancer screening in which 2 screening tests are performed sequentially (the second test is performed only if the first result is positive), followed by treatment if both test results are abnormal. STUDY DESIGN: A total of 1423 women from Cape Town, South Africa, were screened by direct visual inspection, human papillomavirus deoxyribonucleic acid testing, cytologic testing, and cervicography. If an abnormality was identified with any test, women were referred for colposcopy. RESULTS: Direct visual inspection, cytologic testing, human papillomavirus deoxyribonucleic acid testing, and cervicography, when used alone, identified 24, 26, 23, and 23 cases of disease (high-grade squamous intraepithelial lesion or cancer) per 1000 women, respectively, and would classify 182, 71, 137, and 112 women without disease as having abnormal results. Two-stage screening with direct visual inspection first, followed by cytologic testing, human papillomavirus deoxyribonucleic acid testing, or cervicography, would detect 18, 16, and 18 cases per 1000 women, respectively, and would substantially reduce the number of women without disease who were classified as having abnormal results. CONCLUSION: Two-stage screening for cervical cancer provides an attractive alternative to conventional screening for low-resource settings.     

鞍区Rathke囊肿诊断与治疗

目的:探讨鞍区Rathke囊肿的诊断与治疗方法。方法:回顾性分析12例经手术和病理证实的鞍区Rathke囊肿,采用经蝶入路显微外科手术方式治疗。结果:术后头痛8例均好转,4例视力较前有所恢复。结论:鞍区Rathke囊肿临床症状多样,术前MRI和内分泌检查可以提供帮助,经蝶显微操作手术是首选方法。