Analysis of clinical features of primary empty sella.

OBJECTIVE: A retrospective analysis of clinical data of 60 patients with primary empty sella (PES) was conducted to further improve the understanding of the disease. METHODS: The clinical data of 60 patients diagnosed with PES admitted to the Department of Endocrinology of Wuhan Union Hospital for different reasons from January 2000 to September 2018 were analyzed. RESULTS: The 60 cases comprised 22 (36.67%) male and 38 (63.33%) female patients, for a male-to-female ratio of 1:1.73. There was predominance of 50-59 year-olds and women with multiple pregnancies. Of the 60 patients, 41.67% showed fatigue, 26.67% dizziness and headache, 21.67% nausea and vomiting, 18.33% anorexia, and 6.67% visual impairment and other symptoms. Twenty-seven patients (45.0%) had normal pituitary function, and 33 (55.0%) had hypopituitarism. Complete PES was more prone to result in hypopituitarism than partial PES. Men with PES were more likely to have hypopituitarism than women. CONCLUSION: The incidence of PES was significantly higher in women than in men; PES was common in middle-aged and older women with multiple pregnancies. Symptoms were diverse and clinical manifestations may lack specificity compared to other diseases and need to be differentiated. About half of PES patients may develop hypopituitarism. Therefore, for patients with empty sella detected on magnetic resonance imaging (MRI) with or without clinical symptoms, the pituitary function should be thoroughly and promptly evaluated.

Primary empty sella: The risk factors and associations with the cerebral small vessel diseases-An observational study.

OBJECTIVE: To investigate the risk factors of primary empty sella (PES) and its associations with cerebral small vessel diseases (CSVD). METHODS: A total of 132 consecutive patients were recruited from Department of Neurology, The Second Affiliated Hospital of Chongqing Medical University from December 2018 to January 2020, including 69 cases of PES, and age, gender-matched 63 subjects without PES. Demographics and clinical characteristics were recorded. Enlarged perivascular spaces (PVS) and white matter hyperintensities (WMH), which are image markers for CSVD, were assessed. Univariate logistic regression models and multivariate logistic regression models were performed to predict the independent risk factors of PES. RESULTS: There was a significant difference in baseline characteristics in terms of hypertension (p < 0.001) and pregnancy (p = 0.019) between PES and the control group; among markers of CSVD, whole WMH (p = 0.030) and periventricular hyperintensities (PVH) (p = 0.027) were significantly different; however, no significant differences concerning deep WMH, total PVS, basilar ganglia-PVS and centrum semiovale-PVS (p > 0.05). After adjusting relevant potential confounders, multivariate logistic regression revealed hypertension (OR=3.158, 95 %CI: 1.452∼6.865, p = 0.004) and pregnancy (OR=2.236, 95 %CI: 1.036-4.826, p = 0.040) were independent risk factors for PES. CONCLUSION: Hypertension and pregnancy are independent risk factors of PES. There is a possible correlation between PES and WMH, especially PVH, however, further studies are required to confirm these findings.

Variation in the MRI appearance of the canine pituitary gland.

A recent publication described pathological findings in the pituitary gland incidentally discovered during routine necropsies of the brain of dogs and cats; however, imaging characteristics of these lesions were not reported. Aims of this retrospective, observational study were to characterize MRI variants and incidental lesions in pituitary glands of dogs with no clinical signs of pituitary disease. Cranial MRIs from dogs with no suspicion of pituitary disease, based on history and presenting clinical signs, were retrieved from a veterinary teleradiology database during the period of January 2014 to January 2016. Images were reinterpreted by two observers and pituitary lesions were described based on consensus. A total of 580 scans were evaluated and pituitary lesions were detected in 78 dogs (13.44%). Pituitary cystic lesions were the most common finding and occurred in 31 dogs (5.34%). Of these 31 dogs, the majority (74%) were of toy or brachycephalic breed. Partial or total empty sella lesions were detected in 14 dogs (2.41%), and all of these were small or toy breeds. A significantly increased incidence of the partial empty sella lesion was found in male dogs (P = .034). Pituitary lesions greater than 1 cm occurred rarely (0.69%). There was a significant association between low-field (LF) MRI strength and detection of a partial or total empty sella lesion (P = .0112), and detection of a pituitary lesion greater than 1 cm (P = .0125). A significant difference was present between the MRI field strength (FS) that identified pituitary cysts and the FS that detected an empty sella (P = .0068), with the former being a high FS and the latter a LF strength. The findings from this study indicated that up to 13% of dogs with no presenting clinical signs of pituitary disease may have MRI pituitary lesions.

Variaciones anatómicas de la silla turca en radiografías laterales de cráneo. Hospital "Faustino Pérez", 2017-2018 / Anatomic variants of sella turcica in lateral crania radiographies. Hospital "Faustino Pérez¨, 2017-2018

RESUMEN Introducción: diversos autores reflejan que la morfología de la silla turca constituye un factor predisponente para algunas enfermedades. Por ejemplo, se considera que existe correlación entre la morfología de esta estructura anatómica y varias patologías; como el síndrome de la silla turca vacía, síndrome de Williams, paladar hendido, entre otras. Objetivo: describir las variaciones anatómicas de la silla turca que se observan en las radiografías laterales de cráneo del Hospital Universitario "Faustino Pérez" de la ciudad de Matanzas, en el período de enero del 2017 a enero del 2018. Materiales y métodos: el universo fue 140 radiografías laterales de cráneo, de estas 85 pertenecieron al sexo femenino y 55 al masculino. Las variables estudiadas fueron edad, sexo y variaciones anatómicas de la silla turca. Se emplearon métodos teóricos y empíricos. Resultados: se observó un predominio de la variación anatómica de la silla turca en forma de U, en ambos sexos. Seguido de la forma de J, predominando la variación en forma de U en el grupo etario de 41 a 50 años y la forma de J en los pacientes mayores de 60 años. Conclusiones: es imprescindible el conocimiento de la anatomía normal de la silla turca y de sus variaciones anatómicas, tanto para las especialidades quirúrgicas como para las no quirúrgicas. Un análisis exhaustivo de la morfología de esta estructura es necesario para establecer parámetros que excluyan determinadas patologías.

ABSTRACT Introduction: several authors declare that sella turcica morphology is a predisposing factor to several diseases. For example, it is considered that there is a correlation between the morphology of this anatomical structure and several pathologies like empty sella turcica syndrome, Williams syndrome, cleft palate and others. Objective: to describe the anatomical variants of sella turcica observed in side cranial radiographies of the University Hospital ¨Faustino Perez¨ of Matanzas, in the period January 2017-January 2018. Materials and methods: the universe was 140 side cranial radiography: 85 belonged to female patients and 55 to male patients. The studied variables were age, sex and sella turcica anatomical variables. Theoretic and empirical methods were used. Results: it was observed a predominance of the U-shaped sella turcica anatomical variant in both sexes, followed by the J-shaped one. The U-shaped form predominated in the 41-50-years-old age-group and the J-shaped form in patients elder than 60 years. Conclusions: it is essential to know sella turcica normal anatomy and its anatomical variables, both for the surgical specialties and for the non-surgical ones. It is necessary the exhaustive analysis of this structure to establish parameters excluding several pathologies.

Primary Empty Sella Syndrome and the Prevalence of Hormonal Dysregulation.

BACKGROUND: Empty sella is the neuroradiological or pathological finding of an apparently empty sella turcica containing no pituitary tissue. The prevalence of primary empty sella, i.e., empty sella without any discernible cause, is not precisely known; estimates range from 2% to 20%. Technical advances in neuroradiology have made empty sella an increasingly common incidental finding. It remains unclear whether, and to what extent, asymptomatic adult patients with an incidentally discovered empty sella should undergo diagnostic testing for hormonal disturbances. METHODS: To answer this question, the authors carried out a systematic search in the PubMed and Web of Science databases for publications that appeared in the period 1995-2016 and that contained the search term "empty sella" (registration: PROSPERO 2015: CRD42015024550). RESULTS: The search yielded 1282 hits. After the exclusion of duplicates, pediatric reports, case reports, and veterinary studies, 120 publications on primary empty sella syndrome (PES) were identified. 4 of these dealt with the prevalence of pituitary insufficiency in patients with PES as an incidental finding. Among patients with PES, the relative frequency of pituitary insufficiency in the pooled analysis was 52% (95% confidence interval [38; 65]). CONCLUSION: The data on PES as an incidental finding are too sparse to enable any evidence-based recommendation on the potential indications for hormone testing or its nature and extent. We advise basic neuroendocrinological testing (fasting cortisol, free thyroxine [fT4], estradiol or testosterone, insulin-like growth factor 1 [IGF-1], and prolactin). There is an unexplained discrepancy between the reported high prevalence of pituitary insufficiency among persons with PES and its low prevalence in epidemiologic studies. We suspect that the former may be high because of selection bias in the publications that we reviewed, or else the latter may be erroneously low.

[Analysis of the joint and a posteriori probability between primary empty sella, its comorbidities and audiovestibular pathology]. / Análisis de la probabilidad conjunta y a posteriori entre aracnoidocele selar primario, sus comorbilidades y enfermedad audiovestibular.

BACKGROUND: Primary empty sella is a herniation of the sellar diaphragm into the pituitary space. It is an incidental finding and patients may manifest neurological, ophthalmological and/or endocrine disorders. Episodes of vertigo, dizziness, and hearing loss, have been reported. OBJECTIVE: To determine the conditional probability, as well as the statistical dependency, through the Bayesian analysis in patients with primary empty sella and audiovestibular disorders. PATIENTS: Individuals who attended the National Rehabilitation Institute from January 2010 to December 2011, diagnosed with primary empty sella and audiovestibular disorders. MATERIAL AND METHODS: An analysis was performed on a sample of 18 patients with a diagnosis of primary empty sella confirmed with magnetic resonance studies and who had signs of vertigo, hearing loss and dizziness. RESULTS: Of the 18 patients studied, 3 (16.66%) had primary empty sella as the only clinical evidence. In 9 patients (50%) empty sella was associated with vertigo, and 16 patients (88.88%) were diagnosed with hearing loss, with sensorineural hearing loss being the most frequent (77.77%). The intersection between the proportions of primary empty sella with the presence and type of hearing loss was calculated. Thus for sensorineural hearing loss, the calculated ratio was P(AB)=0.6912, and for conductive and mixed hearing loss the value of P(AB)=0.0493 in both cases. CONCLUSIONS: Bayesian analysis and conditional probability enables the dependence between two or more variables to be calculated. In this study both mathematical models were used to analyse comorbidities and audiovestibular disorders in patients diagnosed with primary empty sella.

Severe growth hormone deficiency and empty sella in obesity: a cross-sectional study.

Obesity is associated with blunted growth hormone (GH) secretion. In some individuals, hypothalamic-pituitary (HP) structural lesions may contribute to GH deficiency (GHD). We explored pituitary morphology in obese patients with suspected GHD and its association with cardiovascular risk factors, body composition, and cardiac morphology. One hundred and eighty-four adults obese patients with symptoms and signs of GHD (147 females and 37 males; mean age 46.31 ± 12.11 years), out of 906 consecutive white obese outpatients, were evaluated. The main measures were anthropometric data, blood pressure, lipid profile, glycemic parameters, pituitary hormones, and insulin-like growth factor-1 values, echocardiography, magnetic resonance imaging (MRI) of the HP region, body composition, and growth hormone-releasing hormone plus arginine test. Seventy patients had GHD (GH peak values <4.2 µg/mL). GHD patients showed significantly higher body mass index and fat mass, lower lumbar bone mineral density, increased left ventricular mass index, and epicardial fat thickness. The MRI of the HP region showed empty sella (ES) in 69 and normal pituitary in one of the 70 GHD patients; the 114 patients with normal GH response had ES (n = 62, 54 %), normal pituitary (n = 37, 32 %), microadenomas (n = 10, 8 %), and other pituitary abnormalities (n = 5, 4 %). ES was a significant independent predictor of GH secretory capacity as determined by multiple regression analysis. The close relationship between ES and GH secretory capacity points out to the possibility of the organic nature of GHD in a portion of obese individuals and opens a new scenario with regard to the potential of GH treatment on metabolic consequences of obesity.

Growth hormone secreting pituitary microadenomas and empty sella - An under-recognized association?

OBJECTIVE: To describe an association of growth hormone (GH) secreting pituitary microadenomas and empty sella (ES), which has been described in case reports - the underlying mechanisms are unclear. METHODS: We retrospectively analyzed patients operated for GH-producing pituitary adenomas between February 2004 and February 2009. Magnetic resonance imaging (MRI), computed tomography (CT) imaging, and pituitary function testing were performed. All cases underwent transsphenoidal surgery (TSS). Mean follow up was 38 months (range 12-80 months). RESULTS: Out of 152 patients with acromegaly due to GH-producing pituitary adenomas (female:male=73:79; age range 17-63 years), 69 patients had microadenomas (45.4%; 38 females, 31 males). We found 14 cases (14/69, 20.3%), all microadenomas, with presurgical evidence of ES - 10 females (71%) and 4 males (29%) (female:male=2.5:1). When compared with 103 patients with GH-negative microadenomas treated in the same time period (ES in 4 of 103; 3.9%), ES was highly significantly associated with GH production by the microadenoma (p=0.001). In acromegalics with empty sella, no cases of ectopic adenoma were found. Postoperatively, GH and IGF-1 levels fell in all patients, and 7 cases had random GH and IGF-1 levels consistent with cure. CONCLUSION: The combination of GH-producing microadenomas and empty, enlarged sella is not rare. In this setting, preoperative CT scans are very useful and the transsphenoidal approach is efficient and safe. The mechanism underlying the association of GH-producing microadenomas and empty sella remains unclear and requires further studies.

Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease.

OBJECT: Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD). METHODS: Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height > 2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point. RESULTS: Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p < 0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27). CONCLUSIONS: Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.

Primary empty sella and GH deficiency: prevalence and clinical implications.

Primary empty sella (PES) is a particular anatomical condition characterized by the herniation of liquor within the sella turcica. The pathogenesis of this alteration, frequently observed in general population, is not yet completely understood. Recently reports demonstrated, in these patients, that hormonal pituitary dysfunctions, specially growth hormone (GH)/insulin- like growth factor (IGF-I) axis ones, could be relevant. The aim of this paper is to evaluate GH/IGF-I axis in a group of adult patients affected by PES and to verify its clinical relevance. We studied a population of 28 patients with a diagnosis of PES. In each patient we performed a basal study of thyroid, adrenal and gonadal - pituitary axis and a dynamic evaluation of GH/IGF-I after GH-releasing hormone (GHRH) plus arginine stimulation test. To evaluate the clinical significance of GH/IGF-I axis dysfunction we performed a metabolic and bone status evaluation in every patients. We found the presence of GH deficit in 11 patients (39.2%). The group that displayed a GH/IGF-I axis dysfunction showed an impairment in metabolic profile and bone densitometry. This study confirms the necessity to screen the pituitary function in patients affected by PES and above all GH/IGF-I axis. Moreover the presence of GH deficiency could be clinically significant.

Empty sella and primary autoimmune hypothyroidism.

In order to assess the association between empty sella (ES) and primary autoimmune hypothyroidism, and the possibility of a common pathogenesis. We retrospectively studied all patients with presumed ES diagnosed in the last 20 years, most of whom were treated by our Endocrinology Department. Subjects with a known etiology were excluded. Incomplete records or those with a doubtful diagnosis were also excluded. A total of 56 subjects were included in the study. ES was diagnosed by pituitary MRI. The measurement of free T4, TSH, and antithyroid antibodies (TPOAb and TgAb) was assayed using commercial kits. The cases of hypothyroidism obtained were compared with those in another group of similar patients, diagnosed with diabetes mellitus type 2, through chi2 test. A total of 15 (26.78%) patients of 56 with ES had autoimmune thyroid disease (subclinical or clinical hypothyroidism). Primary hypothyroidism with negative antithyroid autoantibodies was found in a further 13 patients (23.21%). The 46.42% of ES had primary hypothyroidism; this result had obtained a statistically significant difference when compared to the ratio obtained in the group of diabetes mellitus type 2 (P < 0.0029). There is an important association between ES and autoimmune thyroid disease, which reached 26.78% in our series. We suggest the possibility of a common pathogenesis for certain cases of ES and autoimmune thyroid disease, with the end point of ES in the pituitary, and atrophy in the thyroid gland.

Etiology, functional status and short term outcome of patients with pituitary lesions. An experience from a developing country.

OBJECTIVE: To describe the etiology, functional status and short term outcome of patients with pituitary lesions (PL). METHODS: Brain or pituitary MRI reports of 3753 patients were analyzed for PL over the period of 2000 to 2007, done at the Aga Khan University Hospital (AKUH), Karachi, Pakistan. MRIs with reported PL and all those ordered by Endocrinologists with or without pituitary abnormalities, were included in the analysis. This made a total of 338 (9%) MRI reports. PL were defined as pituitary tumors, cysts, haemorrhage, hypoplasia; and empty sella. Patients with these PL, were analyzed for symptoms, hormonal profile and short term outcome in the hospital retrospectively. RESULTS: In the analysis of 338 MRI reports, 23% had normal pituitary gland on MRI examination. Hypogonadotrophic hypogonadism was the commonest (38.5%) endocrine abnormality seen with normal pituitary MRI, followed by hypopituitarism (5.1%). Most common PL identified were macroadenoma (38.7%), empty sella (16.5%) and microadenoma (12.7%). Patients with macroadenoma had 58% of non-functional tumours while 19.1% had hypopituitrism, 17.6% had acromegaly and 17.5% had prolactinoma. Majority of these lesions were causing headache and visual symptoms (42.7%) and were treated with surgery (75.5%). Patients with microadenoma included 39.5% prolactinomas, 18.6% cushing disease, 14% acromegaly, 4.7% hypogonadotrophic hypogonadism and 2.3% hypopituitrism. Majority of these lesions were treated with drugs and 37.3% were treated with surgery. In empty sella patients, 32% patients had endocrine abnormalities among which 21.4% had hypopituitrism, 2% had hypogonadotrophic hypogonadism, and only 6% patients had normal endocrine workup. A significant number of patients (62.5%) never had endocrine hormonal checkup. CONCLUSION: Although PL are less common entities (9% in 3,753 reports) but they are associated with significant endocrinal abnormalities and need prompt treatment. Empty sella, which is not considered as a distinct abnormality, is also associated with significant endocrine deficiencies.

Aspectos de interés sobre el síndrome de silla turca vacía en los niños: a propósito de 5 pacientes / Aspects of interest on the empty sella syndrome in children: apropos of 5 patients

Se presentan 5 pacientes menores de 12 años, atendidos en el Servicio de Endocrinología del Hospital Pediátrico Docente ®William Soler¼ a quienes se les realizó un estudio imagenológico con tomografía axial computada y resonancia magnética nuclear, para confirmar diagnóstico de endocrinopatía. Tras el estudio se diagnosticó síndrome de la silla turca vacía. Existen diferencias clínicas entre niños y adultos con silla turca vacía; las más relevantes radican en que en los niños no hay predominio de sexo ni asociación con la obesidad, y en ellos se observa la presencia de afecciones endocrinas como expresión de alteraciones hipotálamo-hipofisarias.

The cases of 5 patients under 12 that received attention at the Endocrinology Service of William Soler Pediatric Teaching Hospital and that underwent an imaging study with computerized axial tomography and nuclear magnetic imaging to confirm endocrinopathy were presented. The empty sella syndrome was diagnosed after concluding the study. Clinical differences were observed between children and adults with empty sella syndrome. The most significant were found in children, among whom there was neither predominance of sex nor association with obesity. The presence of endocrine affections as expression of hypothalamus-hypophyseal alterations was observed in them.

Primary empty sella.

CONTEXT: The term primary empty sella (PES) refers to a number of endocrine and/or neurological disturbances that may be caused by the herniation of subarachnoid space within the sella. SETTING: The records of all patients with a diagnosis of empty sella between 1985 and 2002 seen at the Catholic University of Rome and University of Brescia were examined retrospectively. PATIENTS: We have observed 171 female and 42 male patients affected by PES (over 4:1 sex ratio). The mean age at diagnosis in our subjects was 51.8 +/- 2.1 yr. Mean body mass index was 27.3 +/- 3.5 kg/m2. MAIN OUTCOME MEASURE: All the patients have been analyzed first either with sellar computed tomography scan or magnetic resonance imaging. All patients underwent neurological, ophthalmological, and baseline endocrine evaluation (appropriate stimulation tests were performed when hypopituitarism was suspected). RESULTS: In the overall population, 40 of 213 patients had documented endocrine abnormalities, specifically 31 females and nine males. Twenty-two patients (10.3% of total patients; 18 women, 10.5% of all women, with a mean age of 38.6 +/- 1.1 yr and four males, with a mean age 46.5 +/- 3.52 yr) presented with hyperprolactinemia. Global anterior hypopituitarism was confirmed in nine patients. Eight patients presented an isolated GH deficiency. One hundred thirty-eight of our patients presented a so-called partial empty sella at computed tomography scan/magnetic resonance imaging, and 75 had total PES. CONCLUSIONS: PES may be associated with variable clinical conditions ranging from mild endocrine disturbances to severe intracranial hypertension and rhinorrhea. The need for treatment of hyperprolactinemia as well as for replacement hormone therapy must be assessed in PES. Symptomatic intracranial hypertension makes cerebrospinal fluid shunting procedures necessary.

No evidence for autoimmunity as a major cause of the empty sella syndrome.

OBJECTIVE: The cause of empty sella syndrome (ESS) remains largely unknown. We measured eleven organ-specific autoantibodies in serum in order to evaluate possible autoimmune components in ESS. PATIENTS: Thirty patients with ESS and 50 healthy blood donors participated in the study. MEASUREMENTS: Detection of pituitary autoantibodies was performed by immunoblotting with human pituitary cytosol as antigen. Thyroid peroxidase (TPO) and TSH receptor (TRAK) autoantibodies were analysed by radioimmunoassay. The remaining eight autoantibodies were detected by in vitro transcription and translation of the autoantigens and immunoprecipitation. RESULTS: The majority of the ESS patients (18/30) exhibited no immunoreactivity at all. None of the remaining 12 ESS patients reacted against more than one autoantigen. No immunoreactivity was found more frequently among ESS patients than healthy blood donors. Pituitary autoantibodies were not correlated to the ESS patients' pituitary function or sellar size, although the results indicated a tendency of increased autoimmunity in patients with hypopituitarism and normal sella size respectively. CONCLUSION: Detection of autoantibodies is a valuable tool in the diagnostic work-up of autoimmune diseases. By analysing a large number of organ-specific autoantibodies we found no evidence of ESS being associated with any specific autoimmune disease. The pathogenesis of ESS is believed to be heterogeneous and our findings suggest autoimmune components to be of minor importance. In some selective cases, ESS in combination with hypopituitarism may be the result of an autoimmune disease in the pituitary gland but this needs further investigation.

Significance of empty sella in cerebrospinal fluid leaks.

OBJECTIVE: The role of elevated cerebrospinal fluid (CSF) pressures in the pathophysiology of various CSF leaks is not clear. Empty sella syndrome (ESS) is a radiographic finding that can be associated with elevated CSF pressures and may represent a radiographic indicator of intracranial hypertension. We present our experience with CSF leaks of various causes, the prevalence of ESS in the spontaneous and nonspontaneous categories, and the potential pathophysiology and unique management issues of the spontaneous CSF leak group. METHODS: We conducted a retrospective review of medical records, imaging studies, and surgical treatment of CSF leaks in patients treated by the senior author. RESULTS: Sixteen patients with spontaneous CSF leaks and 12 patients with nonspontaneous CSF leaks were surgically treated from 1996 through 2002. In the spontaneous group, 15 patients had complete imaging of the sella turcica. Ten had completely empty sellae and 5 had partially empty sellae, for a total of 100% (15 of 15). In the nonspontaneous group, 9 patients had complete imaging of the sella. Only 11% (1 of 9) had a partially empty sella and that was a congenital leak. Comparison of proportions between these 2 groups was significant (P = 0.01). The spontaneous group consisted primarily of obese, middle-aged females (13 of 16 patients). CONCLUSION: Empty sella probably represents a sign of elevated intracranial pressure that leads to idiopathic, spontaneous CSF leaks. Spontaneous CSF leaks are strongly associated with the radiographic finding of an empty sella and are more common in obese females, similar to benign intracranial hypertension. This unique population may require more aggressive surgical and medical treatment to prevent recurrent or multiple leaks.

Empty sella in children as a key for diagnosis.

We performed magnetic resonance imaging of the pituitary gland in 354 children who were under the care of our pediatric neurological outpatient department. Among them, an empty sella was recognized in 11 children, who all showed normal hormonal results. The frequency of an empty sella was significantly high in idiopathic intracranial hypertension (4/4; P<0.0001) and nevoid basal cell carcinoma syndrome (3/5; P<0.0001). The ratio of an empty sella in children, excluding patients with idiopathic intracranial hypertension and nevoid basal cell carcinoma syndrome, is estimated to be 4/345 (1.2%), which is markedly lower than that in adults. Magnetic resonance imaging of the pituitary gland in children could provide important clues for the diagnosis of idiopathic intracranial hypertension or nevoid basal cell carcinoma syndrome.

The association of Cushing's disease and primary empty sella turcica.

The empty sella turcica is defined as the extension of the subarachnoid space toward the intrasellar region with displacement of the pituitary towards the posteroinferior wall. By autopsy studies, the incidence in the general population is around 20%. The association of Cushing's disease (CD) and empty sella has been infrequently reported. In our group, from a total of 68 patients with CD studied by magnetic resonance imaging (MRI), we found the presence of a primary empty sella syndrome (ESS) in 11 (16%). Of these, 9 had partial and 2 total ESS, and in four of them a microadenoma could be identified. Remission, ascertained by subnormal postoperative cortisol levels in blood and/or urine was obtained in 5 of 6 patients operated on by the transphenoidal route. Following surgery, 2 patients presented cerebrospinal fluid (CSF) leakage, 2 diabetes insipidus, and 2 some form of hypopituitarism, figures apparently higher than in non-ESS patients with CD. Ketoconazole was indicated as second line treatment in 2 patients and as primary therapy in 4, resulting in normal urinary free cortisol (UFC) values, with no complications. The relationship of ESS and CD is probably fortuitous given that the frequency of ESS in the general population is similar. Although in empty sella both surgery and radiotherapy seem to have greater risk of complications, surgery remains the first line treatment. Nevertheless, chronic treatment with ketoconazole could be a useful first choice, particularly when no adenoma is seen, or in those who have contraindications for surgery.

Association of empty sella and neuroendocrine disorders in childhood.

Empty sella syndrome (ESS) is a multicausal entity. The incidence of primary empty sella syndrome (PESS) in children with neuroendocrine dysfunction is not known. In the pediatric age group, frequency seems to have been underestimated. A total of 117 cases of neuroendocrine disorders, including complete growth hormone deficiency, primary hypothyroidism with pituitary resistance to thyroid hormone, obesity, central precocious puberty, hypothalamic hypogonadism and central diabetes insipidus, have been studied with computed tomography and/or magnetic resonance imaging of sellar region for etiologic evaluation. Twenty-one patients were found to have PESS. We noted a high incidence of PESS in children with neuroendocrine dysfunction (17.9%). Children with neuroendocrine dysfunction should be investigated with respect to PESS, and children with PESS recognized coincidentally should be studied with the particular consideration of subclinical neuroendocrine dysfunction.

[The empty sella syndrome. Clinical, radiological and endocrinologic analysis in 20 cases]. / La sindrome della sella vuota. Analisi clinica, radiologica ed endocrinologica in 20 casi.

Empty sella syndrome is an anatomical entity in which the pituitary fossa is enlarged and partially filled with cerebrospinal fluid owing to the arachnoid herniation, while the pituitary gland is compressed against the posterior rim of the fossa. This condition can be due to an inherent weakness of the diaphragm sella and/or to an increase in intracranial pressure which promote the herniation of the arachnoid membrane into the pituitary fossa (primary empty sella) or it can results following surgery, radiation or vascular and tumorous pituitary diseases (secondary empty sella). Empty sella can be associated with neuroradiological and endocrine symptoms. This study reports the clinical, endocrine, and roentgenographic features in 20 patients with primary empty sella syndrome. Disturbances of hypothalamic-pituitary function were detected in 6 patients (hyperprolactinemia, hypopituitarism, central diabetes insipidus, hypothalamic hypothyroidism). Three patients exhibited hypergonadotropic hypogonadism. This report supports the following conclusions: a) there is no correlation between size of pituitary fossa, type an extension of arachnoid herniation and the degree of hypothalamic-pituitary dysfunction; b) endocrine alterations are frequent in the empty sella syndrome; c) the association of empty sella and primary diabetes insipidus is not a very rare event.