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Background. Various studies, mainly conducted in adults, have examined the hormonal axis in primary empty sella (PES), and reported various forms of pituitary deficiencies. We report our experience with PES in pediatric patients in terms of pituitary function, associated impairments, and responses to treatment. Methods. We reviewed 10,560 cranial and 325 pituitary magnetic resonance imagings (MRIs) performed at our university hospital between January 2010 and December 2020 and identified patients with PES. Patients with additional abnormal MRI findings, a history of cranial surgery or radiotherapy, autoimmunity, long-term use of chemotherapeutic or immunosuppressive agents or incomplete diagnostic evaluation were excluded. Clinical, radiological and laboratory evaluations were recorded. Results. The study included 17 patients [9 girls, 8 boys; median age 12.4 years (7.25, 4.3 -17)]. The median size of the pituitary was 2 mm (0.7, 1.2 -3). Based on age-dependent pituitary height measurements, fifteen (88%) patients had pituitary gland hypoplasia. Five patients presented with short stature, two had both pubertal delay and short stature, and one had pubertal delay. Nine patients presented with neurological symptoms such as headaches, tinnitus, tics, and dizziness. Five short patients had growth hormone deficiency. None of the patients had hyper-or hypoprolactinemia, adrenal insufficiency, hypothyroidism, or diabetes insipidus. There was statistically no significant association between the size of the pituitary gland and the severity of hypopituitarism (p = 0.42). Conclusions. The high incidence of pituitary dysfunctions ascertain that this entity should not be considered a normal variant but, should instead be carefully evaluated with appropriate basal and dynamic hormonal testing.

This case sieries gives three different cases with three different presentation and different approaches to a patient with short stature. Material: CASE 1: A 13 Year old female brought by her parents with complaints of decreased height appropriate for age. O/E height- 127cm(lt;-3SD), weight 42kg, MPH- 150cm, arm span to height ratio- 1:1, secondary sexual characters- tanners stage 2. X-ray hand with wrist- bone age 0f 12 years. TFT- within normal limits. MRI pituitary- normal study. Patient suspected to have isolated growth harmone deficiency. Growth harmone stimulation test done after sex steroid priming which showed results of growth harmone deficiency. Patient started on 1.5mg growth harmone s/c per day. CASE 2: A 18 year old boy brought by his parents with complaints of decreased height appropriate for age and absence of facial, axillary and pubic hair. O/E height- 145cm, weight 55kg, MPH- 165cm, arm span to height ratio 1.1:1, external genitalia- male, testes in scrotum, testicular volume- 2.5ml, secondary sexual characters- tanners stage 1. Xray hand with wrist- bone age of 17years. TFT within normal limits. FSH, LH, testosterone levels were low. Growth harmone stimulation test after sex steroid priming showed low growth harmone levels. MRI pituitary showed empty sella. Patient diagnosed to have panhypopititarism and started on inj. Growth harmone 0.24mg/kg/week subcutaneous and inj.testosterone 100mg/month IM. CASE 3: A 17 year old female brought by her parents with complaints of decreased height appropriate for age and absent menstruation till date. O/E height- 143cm(lt;- 3SD), weight- 45 kg, MPH- 170cm, arm span to height ratio 1.1:1, external genitalia - female, secondary sexual characters - tanners stage 1. Xray hand with wrist - bone age of 16years. Further evaluation showed TSH-21.8. MRI pelvis showed streak gonads. Serum growth harmone and IGF levels within normal limits. Estradiol was low and FSH, LH were high. Karyotyping showed 45XO. Patient diagnosed to have turner's syndrome. Since x-ray showed non-closure of epiphysis, patient started on growth harmone 0.33 mg/kg/week and advised not to take estrogen till epiphyseal closure. Observation: case 1: functonal pituitary pathology Case 2: structural pituitary pathology Case 3: gonadal pathology Conclusion: Short stature can be a manifestation of isolated GH defeciency, Hypogonadotropic Hypogonadism or Hypergonadotropic hypergonadism.

Objectiveprimary empty sella (PES) represents a frequent finding, but data on hormonal alterations are heterogeneous, and its natural history is still unclear. Our aim was to evaluate the pituitary function of patients with PES over a long follow-up.Designmulticenter retrospective cohort study enrolling patients referred between 1984-2020 to five Pituitary Units, with neuroradiological confirmed PES and a complete hormonal assessment.Methodswe analyzed hormonal (including basal and dynamic evaluations), clinical and neuroradiological data collected at diagnosis and at the last visit (at least 6 months of follow-up).Resultswe recruited 402 patients (females=63%, mean age=51.5 ± 16 years) with PES (partial, total, undefined in 66%, 13% and 21%, respectively). Hypopituitarism was present in 40.5% (hypogonadism=20.4%, hypoadrenalism=14.7%, growth hormone deficiency=14.7%, hypothyroidism=10.2%, diabetes insipidus=1.5%; multiple deficiencies=11.4%) and hypeprolactinemia in 6.5%. Interestingly, hormonal alterations were diagnosed in 29% of incidental PES. Hypopituitarism was associated with male sex (p=0.02), suspected endocrinopathy (pp=0.003) and not with age, BMI, number of pregnancies and neuroradiological grade. A longitudinal assessment was possible in 166/402 (median follow-up=58 months). In 5/166 (3%), new deficiencies occurred, whereas 14/166 (8.4%) showed a hormonal recovery. A progression from partial to total PES, which was found in 6/98 patients assessed with a second imaging, was the only parameter significantly related to the hormonal deterioration (p=0.006).Conclusionsthis is the largest cohort of patients with PES reported. Hypopituitarism is frequent (40%) but hormonal deterioration seems uncommon (3%). Patients need to be carefully evaluated at diagnosis, even if PES is incidentally discovered.

Background There is only one documented case of intracranial hypertension (IH) and empty sella from cortisol-producing adrenal adenoma so far. And IH and empty sella caused by long-term exogenous hypercortisolism has never been reported before. The purpose of this case report is to alert clinicians to glucocorticoid-induced IH. Case presentation We present retrospectively a 50-year-old woman with cortisol-secreting adrenal adenoma, who progressed to intractable intracranial hypertension and a markedly expanded empty sella due to improper treatment. In 2011, the patient presented with hypertension, lack of cortisol circadian rhythm, low ACTH, a left adrenal adenoma and a partial empty sella, but did not receive low-dose dexamethasone suppression test (LDDST) and 24-h urinary cortisol. In 2014, she exhibited truncal obesity, raised cortisol, LDDST non-suppression, high urinary free cortisol and low ACTH, proving her cortisol-producing adrenal adenoma. She was simultaneously diagnosed with unexplained IH because of papilledema and elevated intracranial pressure, and her partial empty sella changed to a complete empty sella. In 2015, she underwent adrenal adenoma resection. From 2015 to 2018, she kept taking dexamethasone at least 2 mg daily without her doctors’ consent. During this period, she developed transient cerebrospinal fluid rhinorrhea, and her empty sella further worsened. After switching to low dose hydrocortisone, her papilledema disappeared completely, but optic atrophy has become irreversible. Conclusions The patient seems to be just an extreme case, but it may reveal and illustrate a general phenomenon: Both cortisol-producing adrenal adenoma and long-term exogenous hypercortisolism could cause varying degrees of elevated intracranial pressure and empty sella. Clinicians should remain vigilant for this phenomenon in patients with cortisol-producing adrenal adenoma or excessive and prolonged steroid usage and give them corresponding examinations to identify this complication.

Petrous apex cephalocele (PAC) is a rare type of cephalocele. It is usually asymptomatic. It can be unilateral or bilateral. Bilateral cases are less common. They are etiologically and clinically different entities from unilateral ones. A 56-year-old female patient presented to the hospital with the complaint of a severe nonspecific headache. She has been examined in ear nose throat, ophthalmology, and finally neurology clinics. Bilateral PAC was incidentally found in magnetic resonance imaging (MRI) examination. Headache was associated with PAC because no additional pathology could explain the clinical symptomatology. There is a proven relationship between empty sella and PAC. Regarding PAC less than 50 cases are known. Our paper aims to contribute to the literature by exhibiting etiologic and clinic differences between unilateral and bilateral PAC. It highlights the relationship between PAC and broad sella that is a different entity from empty sella and shows this remarkable radiological appearance. The authors presented our case accompanied by clinical and MRI findings.

Purpose: To investigate the clinical importance of the thicknesses of the retinal nerve fibre (RNFL) and ganglion cell and inner plexiform layer (GCL+) by spectral domain optic coherence to...

Background: According to neuroradiological findings, empty sella seems to be deprived of pituitary tissue in sella turcica. Changing size of the pituitary volume is closely related to the occurrence of primary empty sella. The aim of the study is to determine pituitary dysfunction in patients with partial or total primary empty sella and the significance of pituitary volume measurements in these patients. Methods: This study was designed retrospectively. 67 patients (55 females, 12 males) diagnosed with primary empty sella syndrome between the years of 2015-2019 were included in the study. Patients were divided into two groups: partial (PES) and total (TES) empty sella by magnetic resonance imaging (MRI). Basal anterior pituitary and its hormones were assessed. We also included 26 healthy control subjects (19 females, 7 males) to compare the differences in pituitary volumes. Volumes were measured by using Osirix Dicom Viewer (Pixmeo SARL, Geneve, Swiss) in 3.0 Tesla scanner MRI. Results: 82.1% (n=55) of all patients were PES and the others were (n=12) TES. Hypopituitarism, known as one or more pituitary hormones deficiency, was found in 12 patients (17.9%). While 9 of them had total PES, the others had partial PES. Secondary adrenal insufficiency and gonadotropin deficiency were more prevalent in patients with TES. Mean volume measurements of patients with TES, PES and healthy subjects were 0.23±0.17, 0.35±0.15, 0.54±0.17 cm3, respectively. Except for IGF1 values (p=0.026), there was not any significant correlation found between the anterior pituitary hormones and volume measurements. Conclusion: Although volume measurement has helped in the diagnosis of pituitary empty sella (partial or total), it does not seem to have any significant correlation with pituitary secretory function.

Rationale: Some diseases contribute to hypopituitarism without clinical manifestations and the glucocorticoid therapy may unveil central diabetes insipidus. The condition is rare and usually causes problems for clinical physicians. Patient concerns: A 59-year-old woman presented to our hospital due to facial numbness and persistent eyelid heaviness. Diagnosis: Physical examination and cerebrospinal fluid examination supported a diagnosis of Guillain–Barré syndrome. Magnetic resonance imaging showed an empty sella. Hormone test indicated hypopituitarism. Interventions: The patient received intravenous immunoglobulin and glucocorticoid. Central diabetes insipidus appeared after 20 days. Subsequently, the patient was prescribed 1-desamino-8-D-arginine vasopressin and prednisone. Outcomes: During 6 months’ follow-up, the patient's urine output was gradually reduced to normal level. Lessons: This case indicated that hypopituitarism may be caused by an empty sella and be masked by adrenal insufficiency. Central diabetes insipidus may present after glucocorticoid therapy.

Primary empty sella (PES) syndrome is a misnomer as it is not a syndrome but a radiological finding with possible endocrine abnormalities. No specific headache type has been shown to be caused by PES. Endocrine screening may be considered for asymptomatic persons with PES.

Background Primary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans. We intended to review the clinical management and hormonal profile of patients with PES. Methods The study included ten-year retrospective analysis of registry containing PES cases in the period 2007 to 2017, from a single tertiary care center. The keyword ‘primary empty sella’ was used to retrieve patient details from the radiology unit. The clinical and biochemical profile of PES patients was analyzed. Case management of PES patients and their rate of referral to endocrinologists was explored. Results The registry had 765 cases with a male: female ratio of 1:3.8 suggesting female predominance by almost four times. Although not significant, the onset of disease was earlier for males [Mean ± standard deviation (SD) (46.7 years ±17.3 vs 48.8 years±14.1), p = 0.110]. Almost 79% of the cases were found as an incidental finding during Magnetic Resonance Imaging. Of the total PES cases, only 20% were referred to the endocrinologists and the rest were handled by general physicians. Only 1–2.5% of the cases were evaluated for gonadal, growth and adrenal hormones by the general physicians. The hormonal evaluation by the endocrinologists was also found to be sub-optimal. Headache and visual disturbances were the most common presenting complaints followed by menstrual abnormalities. Endocrine abnormalities like thyroid dysfunction, hyperprolactinemia, hypogonadism and hypocortisolism were highly prevalent among those assessed. Conclusion There is a gross under-evaluation of hormonal assessment and minimal case-referral to Endocrinologists. PES is associated with varying degrees of hormonal dysfunction, and hence early assessment and management is needed. Establishing a standard protocol for diagnosis and case management is essential with the involvement of a multidisciplinary team consisting of endocrinologists, neurologists, primary care phys icians and ophthalmologists.

BackgroundThe prevalence of childhood-onset growth hormone (GH) deficiency (GHD) is estimated to be approximately 1 in 5000 or more, with the cause unknown in most cases (idiopathic isolated GHD). However, additional disorders of secretion of other pituitary hormones reportedly develop over time, with a frequency of 2–94% (median, 16%). Furthermore, median times to development of other anterior pituitary hormone deficiencies have been reported to be 6.4–9.4 years. On the other hand, adult patients affected by childhood-onset GHD reportedly develop impaired ventilation function due to reduced lung volumes and respiratory pressures, probably due to reductions in respiratory muscle strength. In addition, GH is known to play a role in stimulating the glomerular filtration rate (GFR), and the estimated GFR (eGFR) is decreased in patients with GHD.Case presentationThis case involved a 65-year-old woman. Her short stature had been identified at around 3 years of age, but no effective treatments had been provided. The patient was mostly amenorrheic, and hair loss became apparent in her late 30s. She developed hyperuricemia, dyslipidemia, and hypertension at 45 years of age. In addition, the patient was diagnosed with hypothyroidism at 50 years of age. At 58 years of age, endocrinological examination showed impaired secretion of thyroid-stimulating hormone, luteinizing hormone/follicle-stimulating hormone, and growth hormone, and magnetic resonance imaging showed an empty sella turcica. However, secretion ability of adrenocorticotropic hormone was retained. At 63 years of age, respiratory function tests confirmed a markedly restricted ventilation disorder (vital capacity, 0.54 L; percentage predicted vital capacity, 26.9%). Renal function had also decreased (eGFR, 25.0 mL/min/1.73 m2). Furthermore, she was diagnosed with hypothalamic secondary hypoadrenocorticism. The patient developed CO2narcosis at 65 years of age, and noninvasive positive pressure ventilation was started.ConclusionsThe rare case of a 65-year-old woman with childhood-onset GHD with panhypopituitarism, including late-onset secondary hypoadrenocorticism in her 60s, associated with severely impaired respiratory function and renal dysfunction, was reported. In GHD patients with risk factors for progression from isolated GHD to combined pituitary hormone deficiency, such as empty sella turcica, lifelong endocrinological monitoring may be important.

OBJECTIVECushing’s disease arises from functioning adrenocorticotropic hormone (ACTH)–secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing’s disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing’s disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing’s disease.METHODSA single-institution database of Cushing’s disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing’s disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI.RESULTSOf the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61–6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002).CONCLUSIONSEmpty sella syndrome is associated with an increased rate of MRI-negative Cushing’s disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.

Adult growth hormone deficiency (GHD) is being increasingly recognized to cause premature mortality exacerbated by oxidative stress. A case-control observational study has been performed with the primary objective of evaluating new parameters of oxidative stress and macromolecular damage in adult GHD subjects: serum nitrotryptophan; Total Antioxidant Capacity expressed as LAG time; urinary hexanoil-lysine; urinary dityrosine and urinary 8-OH-deoxyguanosine. GHD was diagnosed using Growth Hormone-Releasing Hormone 50μg iv+arginine 0,5 g/Kg test, with a peak GH response 30 kg/m2. Patients affected by adult GHD were divided into three groups, total GHD (n = 26), partial GHD (n = 25), and controls (n = 29). Total Antioxidant Capacity, metabolic and hormonal parameters have been determined in separate plasma samples; nitrotryptophan in serum samples; hexanoil-lysine, dityrosine, 8-OH-deoxyguanosine in urine samples. Assessment of hexanoil-lysine exhibited a trend to increase in comparing total GHD vs partial and controls, although not significant. Values of 8-OH-deoxyguanosine did not significantly differ among the three groups. Significant lower levels of dityrosine in partial GHD vs total and controls were found. No significant difference in nitrotriptophan serum levels was found, while significantly greater values of Total Antioxidant Capacity were showed in total and partial GHD vs controls. Thus, our result confirm that oxidative stress is increased both in partial and total adult GHD. The lack of compensation by antioxidants in total GHD may be connected to the complications associated to this rare disorder.

Patient: Female, 67-year-old Final Diagnosis: Isolated adrenocorticotropic hormone deficiency Symptoms: Anorexia • fatigue • vomiting • muscle weakness Medication: — Clinical Procedure: Dynamic endocrine testing Specialty: Endocrinology and metabolic Objective: Rare co-existance of disease or pathology Background: Isolated adrenocorticotropic hormone deficiency (IAD) is a rare disorder characterized by central adrenal insufficiency (AI) but normal secretion of pituitary hormones other than adrenocorticotropic hormone. IAD usually presents with unspecific symptoms of AI, such as anorexia and fatigue, but some patients present with a variety of atypical manifestations. Rhabdomyolysis is a potentially life-threatening clinical syndrome caused by skeletal muscle injury with the release of muscle cell contents into the circulation. A wide variety of disorders can cause rhabdomyolysis. Herein, we report an unusual case of IAD presenting with hyponatremia and rhabdomyolysis. Case Report: A 67-year-old Japanese woman with a 2-month history of anorexia and fatigue was diagnosed with severe hyponatremia (serum sodium, 118 mEq/L) and rhabdomyolysis (serum creatine phosphokinase, 6968 IU/L), after 2 days of vomiting and muscle weakness. Physical and laboratory findings did not show dehydration or peripheral edema. Her rhabdomyolysis resolved with normalization of serum sodium levels during administration of sodium chloride. However, her anorexia and fatigue remained unresolved. After reducing the amount of sodium chloride administered, the patient still had hyponatremia. Detailed endocrinological examinations indicated IAD; her hyponatremia was associated with inappropriately high plasma arginine vasopressin levels. The patient received corticosteroid replacement therapy, which resolved her anorexia, fatigue, excessive arginine vasopressin, and hyponatremia. Conclusions: This case highlights the importance of considering the possibility of central AI in patients with hyponatremia and excessive arginine vasopressin levels. In addition, rhabdomyolysis associated with hyponatremia can be an important manifestation of IAD.

We report the case of a 59 year old woman who presented with a six week history of worsening bifrontal headache. On CT brain the only abnormal finding was a partially empty sella potentially indicative of increased intracranial pressure. MRI found a large cerebral venous sinus thrombosis in the superior sagittal sinus. Blood tests and a bone marrow biopsy revealed a diagnosis of JAK2 positive primary polycythaemia rubra vera. The lack of sensitivity and specificity of CT in the diagnosis of CVST should engender a low threshold for MRI in patients with risk factors and/or non-diagnostic abnormalities on initial CT. Management of this dual pathology involves both the immediate treatment of the thrombus with heparin bridging to warfarin and the long treatment for polycythaemia involving repeat venesections and cytoreductive therapy.

A 53-year-old woman was referred for medical evaluation of therapy-resistant dyslipidaemia accompanied by elevated creatine kinase levels. Because cessation or alteration of her medication did not improve laboratory abnormalities, hypothyroidism was considered, despite the fact that thyroid stimulating hormone levels were within the reference interval. On further evaluation, she was found to have panhypopituitarism and empty sella turcica as shown by MRI. These findings were unexpected since there was no clinical suspicion during detailed evaluation. When supplementary questions were asked, she brought up a history of severe postpartum haemorrhage 30 years ago, for which she underwent a hysterectomy. Based on these findings, the patient was diagnosed with Sheehan’s syndrome. This syndrome is a rare but potentially life-threatening complication of postpartum haemorrhage, characterised by varying degrees of hypopituitarism that are most commonly presented many years after delivery. The patient recovered after adequate hormone replacement therapy.

Objective To investigate outcomes and identified risk factors affecting cure and intraoperative cerebrospinal fluid leak after transsphenoidal surgery using a new classification for growth hormone–secreting pituitary adenoma associated with empty sella. Methods In this retrospective cohort study, 51 patients enrolled from January 2010 to June 2016 were categorized into 3 groups using a new classification scheme: grade A, whole tumor occupation area beneath a horizontal line drawn along the lowest level of empty sella; grade B, tumor occupation area that crossed the horizontal line on 1 side; and grade C, tumor occupation area that crossed the horizontal line on 2 sides. Clinical data were collected and analyzed. Results Cure rate for grade A patients (88.24%; 15/17) was significantly higher than cure rate for grade B (55.00%; 11/20) and grade C (50.00%; 7/14) patients. Occurrence of cerebrospinal fluid leaks in grade C patients (35.71%; 5/14) was higher than in grade A patients (5.88%; 1/17). Logistic regression analysis indicated that risk factors affecting cure included large maximum tumor diameter (P = 0.009, odds ratio [OR] = 1.222), high preoperative fasting growth hormone level (P = 0.031, OR = 1.088), and high classification (P = 0.017, OR = 4.485). Risk factor affecting intraoperative cerebrospinal fluid leak was high classification (P = 0.039, OR = 3.580). Conclusions Transsphenoidal surgery is the current optimal treatment strategy. Empty sella increases the difficulty of surgery with a higher incidence of complications. The new classification scheme was better for predicting the surgical outcome for this disease.

Purpose: To analyze the clinical characteristics of acromegalic patients with empty sella (ES, herniation of the subarachnoid space within the sella turcica) and the impact of ES on transsphenoidal surgery in such patients. Methods: Seventy-eight patients, newly diagnosed with acromegaly who underwent transsphenoidal surgery were included. ES was defined as the pituitary gland and adenoma occupying less than 50% of the sella turcica on midsagittal magnetic resonance (MR) imaging. Results: Twelve patients (15.4%), predominantly female (10 women, p = .047), had ES in preoperative MR imaging. ES patients had smaller mean tumor diameter (6.3 mm) than non-ES patients (11.2 mm, p = .001). In preoperative MR imaging, occult adenoma was found in three (25%) ES and three (4.5%) non-ES patients (p = .044). Intraoperative cerebrospinal fluid (CSF) leakage was more frequent in the ES patients than in the non-ES patients (58.3 vs. 25.8%, p = .024). This led to an increased rate of sellar floor reconstruction using abdominal fat and/or postoperative lumber drainage in the ES patients (ES: 41.7 vs. non-ES: 16.7%, p = .063). Endocrinological remission after surgery was more frequent in the non-ES patients (72.7%) than in the ES patients (58.3%) (p = .248). Conclusions: Co-existence of acromegaly with ES is not rare, and is associated with occult adenoma, intra/postoperative CSF leakage, and a worse endocrinological outcome after transsphenoidal surgery; although, the underlying mechanism remains unclear. © 2017 Springer Science+Business Media New York, Embargo Period 12 months

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.

BACKGROUND AND PURPOSE: Empty sella has been reported in patients with idiopathic intracranial hypertension and is thought to be a sign of elevation of intracranial pressure. However, it can also be found in patients with lateral sinus stenosis presenting with isolated pulsatile tinnitus without signs of intracranial hypertension. We hypothesized that the volume of the sella turcica would be similar in both groups of patients undergoing stent placement for lateral sinus stenosis. MATERIALS AND METHODS: Consecutive patients with idiopathic intracranial hypertension or isolated venous pulsatile tinnitus and undergoing lateral sinus stent placement from January 2012 to December 2017 were included. The primary outcome was the estimated volume of the sella turcica based on preoperative CTA measurements. The ratio of the pituitary gland height/sellar height was calculated on preoperative MR imaging. Sellar volumes were compared among the 3 groups: pulsatile tinnitus, idiopathic intracranial hypertension, and a control group, matched by age and sex. RESULTS: Eighty-eight patients underwent lateral sinus stent placement. The median age was 37 years, and 94% were women. No difference in age, sex, or body mass index was found among the groups. Patients undergoing venous stent placement had significantly higher sellar volumes than the control group (P < 0.001). There was no difference in the sellar volumes (P = .63) or gland/sellar height ratios (P = .25) between the pulsatile tinnitus and idiopathic intracranial hypertension groups. CONCLUSIONS: Empty sella is found in 2 differing groups of patients undergoing lateral sinus stent placement, suggesting that it is a radiologic sign of symptomatic hemodynamic lateral sinus stenosis rather than elevated intracranial pressure.

A 54 year old lady presented to our institute with a history of low grade fever for one week associated with occasional loose stools, vomiting and severe malaise. Initial evaluation revealed low serum sodium. An initial diagnosis of acute gastroenteritis with secondary hyponatremia was made. Work up for infective causes of gastroenteritis was however negative. ENT evaluation and review of drug history did not contribute towards a diagnosis. The patient's symptoms persisted and did not respond to symptomatic treatment. Ultrasound of abdomen revealed cholelithiasis with no evidence of cholecystitis. Further evaluation revealed hypotonic hyponatremia with normal levels of urinary sodium excretion. With other causes of hyponatremia ruled out, an endocrinopathy was suspected as the likely culprit. Follow up hormonal studies revealed hypopituitarism and MRI of brain revealed a partially empty sella. On reviewing the case, a past history of amenorrhea immediately after the birth of her third child (almost 20 years ago!) was elicited. Intractable vomiting is quite an unusual presentation for Sheehan's syndrome, but a thorough case history coupled with a high index of suspicion can contribute towards identifying the cause among a series of confounding clinical and radiological findings, as in our case.

Background Sheehan syndrome refers to a series of clinical symptoms resulting from avascular necrosis of anterior pituitary due to various reasons. Case We report a case of multiple myeloma patient after one cycle of chemotherapy consisting of bortezomib and dexamethasone diagnosis of Sheehan syndrome with obstinate diarrhea, low blood glucose, and coma symptom, especially, the fluorodeoxyglucose positron emission tomography (FDG-PET) and brain magnetic resonance imaging (MRI) revealed that the manifestations in the saddle area were in accordance with empty sella syndrome. A single administration with lenalidomide was given for both consolidative and maintenance treatment, and satisfactory efficacy was achieved. With the patient now remaining in satisfactory medical condition, the success of this therapy has been shown. Conclusions This is the first report showing a patient with combined multiple myeloma and rarely seen Sheehan syndrome, in which lenalidomide was given for treatment, and satisfactory efficacy was achieved.

To investigate the risk factors of primary empty sella (PES) and its associations with cerebral small vessel diseases (CSVD).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.There was a significant difference in baseline characteristics in terms of hypertension (p0.001) 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 (p0.05). 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.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.

Purpose of the study Familial occurrence of either Turner syndrome or hypopituitarism is very rare. Particularly, their association is an uncommon finding. In this context, we describe for the first time 4 sisters with Turner syndrome, hypopituitarism was reported in three among them. Patients and methods Our cohort consists of four Tunisian adult sisters belonging to a consanguineous family. Biochemical analysis, resonance magnetic imaging and cytogenetic analyses were performed. Results Turner syndrome was diagnosed at the ages of 14, 17, 31 and 43 years in cases 1, 2, 3 and 4 respectively. They suffered from short stature, dysmorphic syndrome and/or delayed puberty. Interestingly, 3 among them showed also hypopituitarism, hypogonadotrophic hypogonadism and central hypothyroidism. Somatotropic insufficiency was proven in one case. Pituitary MRI has shown an empty sella turcica with hypoplastic pituitary gland in three cases. Their karyotypes were compatible with 45X in one case, 45X/46XX in the second and 45X/46XX/47XXY with x label in two cases. Conclusion Hence, the presence of these familial cases of TS must evoke new etiopathogenetic arguments. Coincidence of hypopituitarism in this family, might suggest common genetic background for the two diseases. This particular family would be a precious tool for an extensive molecular analysis. More attention should be given to other family's members mainly in the presence of delayed puberty and sterility in other members.

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.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).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]).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.

Objective To review the results of sphenoid sinus lateral recess (SSLR) cerebrospinal fluid (CSF) leaks treated with the endoscopic endonasal minimal transpterygoid approach (EEMTPA) and to discuss the surgical technique and outcomes. Methods We performed a retrospective analysis of 13 cases who underwent SSLR CSF leak repair through the EEMTPA in our clinic between September 2008 and December 2017. Demographic and etiological features with reconstruction and surgical outcomes were examined. Mean follow-up time was 6.1 years. Results In regard to etiology, the SSLR CSF leaks included 9 patients with spontaneous, 2 patients with traumatic, and 2 with iatrogenic causes. CSF leak was at the left lateral recess in 8 cases and at right lateral recess in 5 cases. Nine patients had empty sella syndrome, and 11 patients had meningoencephaloceles in addition to SSLR CSF leaks. All patients underwent surgery through the EEMTPA, and a multilayer closure with tissue overlay grafts were used for reconstruction. A pedicled nasoseptal flap and/or pedicled middle turbinate flap were applied to the area of the leak in all cases. One patient had a persistent CSF leak and another had recurrence, both of which required revision surgery. Our overall success rate was 100%. Conclusions EEMTPA is a safe and effective method that can be used to treat challenging pathologies at the SSLR, including CSF leaks accompanying meningoencephaloceles. Furthermore, the success rate of EEMTPA for SSLR CSF leaks can be increased by applying endoscopic skull base reconstruction techniques such as the pedicled nasoseptal flap and pedicled middle turbinate flap.

Ectopic pituitary adenoma is a rare entity that is most commonly located in the sphenoid sinus. We report a case of a patient with ectopic pituitary adenoma with no functional expression associated with empty sella turcica, which gives rise to a broad differential diagnosis. Although it is a benign neoplasm, necrosis is encountered in a proportion of cases. Magnetic resonance imaging is the diagnostic method of choice for hypothalamic-pituitary-related endocrine diseases with endoscopic biopsy for histological confirmation. It is important to include pituitary markers in the immunohistochemical diagnostic panel.

Describe clinical and radiographic features of sigmoid sinus wall anomalies (SSWA) associated with pulsatile tinnitus (PT) and determine factors predictive of response to surgery.Preoperative diagnostic imaging and treatment response were reviewed after surgical repair of 40 ears among 38 consecutive patients presenting with PT associated with SSWA who underwent transtemporal sinus wall reconstruction.Twenty-three ears had isolated sigmoid sinus dehiscence, and 17 had diverticulum. The rates of transverse sinus stenosis (TSS) and empty sella, 66% and 32% respectively, were significantly higher than in historical controls (P = 0.02 and 0.001). Thirty-six out of 40 subjects (90%) had complete resolution of their PT following surgery, including all those with a diverticulum. For subjects with dehiscence alone without diverticulum, a favorable response to surgery was strongly associated with the presence of TSS (P = 0.01) and empty sella (P = 0.02).Sigmoid sinus diverticulum and dehiscence are a clinically important cause of PT. Women of childbearing age with an elevated body mass index (BMI) are commonly affected, and there is a high rate of associated TSS and empty sella. Transtemporal sinus wall reconstruction has a high rate of success in appropriately selected patients. Patients with isolated sinus wall dehiscence without diverticulum, TSS, or empty sella are less likely to respond to transtemporal sinus wall reconstruction. These data imply a multifactorial cause of PT in at least some patients with SSWA.4 Laryngoscope, 128:S1-S13, 2018.

To assess and quantify the prevalence and co-occurence of a sellar floor lowering and empty sella phenomenon in patients with GH (growth hormone)-secreting pituitary adenoma.A total number of 159 acromegalic patients were included in this study, as well as two control groups (150 patients with non-GH-secreting adenomas and 50 patients without pituitary adenomas). Magnetic resonance images of all patients were evaluated for presence of an empty sella, downward and lateral tumor extension, and maximum superoinferior diameter of the mass. Additionally, these values were correlated with growth hormone and IGF-1 levels.The empty sella phenomenon was detected significantly more often in patients with a GH-secreting adenoma with a prevalence of 22% vs. 5.3% in non-GH-secreting adenomas (p 0.001) or 8% in the healthy control group (p = .036). Moreover, GH-secreting adenomas presented with a significant rate of downward tumor extension (74.8% vs. 35.5%; p 0.001), whose extent correlated inversely but weakly with the GH hormone level (r = - 0.17; p = .036). It was also found that a decreased superoinferior diameter and higher ratio of intrasellar to suprasellar extension are predictive quantitative values for the presence of a GH-secreting adenoma (area under curve, 0.712).GH-secreting pituitary adenomas are frequently associated with an empty sella phenomenon. Moreover, GH-secreting adenomas are frequently accompanied by an enhanced, quantitatively measurable impression of the sellar floor. Hypothetically, this is caused by tumor-induced local bone remodeling processes.

The psycho-neuroendocrine-immune approach relies on the concept of considering diseases from a holistic point of view: the various components (psyche, nervous system, endocrine system, and immune system) control the diseased organ/apparatus and in turn are influenced by a feedback mechanism. In this article, we will consider the psycho-neuroendocrine-immune approach to coloproctological disorders, by providing clinical cases and discussing them in light of this approach.

Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma. She was subjected to endoscopic third ventriculostomy (ETV). All of her symptoms improved after ETV and the tumor is being followed up. The possible pathogenesis of such a highly atypical clinico-radiological presentation is described. This unusual syndromic presentation of tectal glioma without clinical features of increased intracranial pressure is probably due to increased compliance of third ventricular walls, and is relieved with ETV.

Background Chiasmapexy is used to treat empty sella syndrome, and various materials are used for the elevation of the optic chiasm. However, the use of artificial substances may have the risk of graft infection, and fat and muscle may be absorbed over the long term after surgery. In addition, bone and cartilage may be unavailable in adequate amounts. Here, we describe a new technique for chiasmapexy using an iliac crest bone graft. Case Description The first patient was a 71-year-old woman who had undergone transsphenoidal surgery twice for the treatment of pituitary adenoma and Rathke cleft cyst. The optic chiasm collapsed after the second surgery and her visual field worsened gradually. We performed chiasmapexy using fat, fascia, and a septal mucosal flap, but the optic chiasm did not remain in the normal position because of graft shrinkage. Finally, we used an iliac crest bone graft, which resulted in good visual function. The second patient was a 58-year-old man who was incidentally diagnosed with empty sella syndrome. The patient's bitemporal hemianopia gradually progressed. As in the first case, we used an iliac crest bone graft, which halted the deterioration of visual function after chiasmapexy. Conclusions The advantages of iliac bone are that it is less likely to absorb and become infected than synthetic materials. This method may be suitable for reoperative cases, especially those wherein the septal cartilage has been removed in a previous surgery. This method will halt visual deterioration and may be one of the considerable options for chiasmapexy operations.

Macroprolactinemia is a polymeric form of prolactin-release, causing mildly symptomatic clinical pictures. The former can be isolated or associated with other causes of hyperprolactinemia. The association with an empty sella syndrome is rare. We report a case of a female patient discovered with this association. It's about a female patient 47 years old, followed up since the age of 31 years for bilateral galactorrhea and a spaniomenorrhea. There has been no associated drug intake. Her exploration has showed a serum prolactin level of 635 mIU/L. Thyroid test results were normal T4 = 10,2ng/L and TSH = 1.76 mIU/L. A brain scan has showed an empty sella turcica. Despite the unchanged levels of prolactinemia, the evolution under dopaminergic 5 mg /D has been marked by the occurrence of a pregnancy with persistent moderate hyperprolactinemia in the postpartum. Chromatography has showed a predominance of the macroprolactin form with: Prolactin monomer at 4.8%, Big Prolactin at 5% and Big Big Prolactin at 83%, thus stopping bromocriptine. Our observation suggests that macroprolactinemia can be associated with conventional etiologies of moderate hyperprolactinemia as the empty sella syndrome. Its detection would prevent the use of dopaminergic therapy which seems not useful.

Background Skull base cerebrospinal fluid (CSF) leak after gamma knife radiosurgery (GKRS) is a very rare complication. In patients who were treated with both GKRS and transsphenoidal resection (TSR) for pituitary lesions, early CSF leak occurs at a comparable rate with the general TSR population (4%). Delayed CSF leak occurring more than a year after TSR, GKRS, or dual therapy is exceedingly rare. Methods Retrospective chart review and review of the literature. Results We present 2 cases of delayed CSF leak after GKRS to treat pituitary adenoma. One patient developed CSF rhinorrhea 16 years after GKRS for growth hormone–producing pituitary adenoma. The patient had previously undergone TSR surgery 7 years prior to GKRS without complication. Additionally, a second patient developed high-flow CSF rhinorrhea 2 years after GKRS for a prolactinoma that failed dopamine agonist therapy. Both patients underwent a complicated clinical course after presentation, requiring multiple revisions for definitive CSF leak repair. Conclusions Delayed CSF leak is a rare but serious complication after GKRS independent of TSR status; urgent repair is the treatment of choice. Based on our experience, these leaks have the potential to be refractory, and we recommend aggressive reconstruction, preferably with a vascularized flap, and potentially supplemented by placement of a lumbar drain and acetazolamide. Current evidence is scant and provides little insight regarding an underlying mechanism, which may include bony destruction by the tumor, delayed radiation necrosis, or a secondary empty sella syndrome.

Objectives: Sellar reconstruction with intrasellar packing following endoscopic resection of pituitary macroadenomas remains a subject of clinical and radiological discussion particularly, when an intraoperative cerebrospinal fluid (CSF) leakage is absent. This study was conducted to contribute our experience with sellar reconstruction after a standard endoscopic surgery of pituitary macroadenomas without intraoperative CSF leakage to the ongoing discussion between techniques with and without intrasellar packing. Methods: A consecutive series of 47 pituitary macroadenomas undergoing excision via a standard endoscopic endonasal transsphenoidal surgery (EETS) without evident intraoperative CSF leakage were retrospectively evaluated over a 10-months mean follow-up period. According to the sellar reconstruction technique, three groups could be identified: Group A – with no intrasellar packing, Group B – with haemostatic materials packing, and Group C – with abdominal fat packing. Postoperative clinical and radiological assessments of the three groups were documented and analyzed for differences in outcome. Results: Postoperative clinical assessment did not differ significantly between the three groups. In group A, postoperative CSF leakage, sphenoid sinusitis and empty sella syndrome were not observed. However, a significant difference in radiological assessment could be identified; the interpretation of sellar contents in postoperative MRI of group A succeeded earlier and more reliably than in other groups with intrasellar packing. Conclusions: There is no difference in the incidence of postoperative CSF leakage and empty sella syndrome among the various reconstructive techniques with and without intrasellar packing, irrespective of size and extension of the pituitary adenoma. Sellar reconstruction without intrasellar packing following a standard EETS is not inferior to other techniques with packing and even shows more radiological advantages, which made it our preferred technique, at least if no intraoperative CSF leakage is evident., Ziele: Die Rekonstruktion der Sella mit intrasellärer Tamponade nach endoskopischer Resektion von Hypophysenmakroadenomen bleibt in der klinischen und radiologischen Diskussion umstritten, besonders wenn keine Liquorfistel vorhanden ist. Diese Studie wurde durchgeführt, um unsere Erfahrungen mit der Sellarekonstruktion nach einer endoskopischen Standardresektion von Hypophysenmakroadenomen ohne intraoperative Liquorfistel zur laufenden Diskussion über Techniken mit und ohne intrasellärer Tamponade beizutragen. Methoden: Eine konsekutive Serie von 47 Hypophysenmakroadenomen, die in einem chirurgischen Standardverfahren endoskopisch über einen endonasalen transsphenoidalen Zugang ohne ersichtliche intraoperative Liquorfistel entfernt wurden, wurde nachträglich während einer durchschnittlich zehnmonatigen Nachbeobachtungszeit ausgewertet. Bezüglich der sellären Rekonstuktionstechnik konnten drei Gruppen identifiziert werden: Gruppe A – ohne intraselläre Tamponade, Gruppe B – mit Tamponade aus hämostatischen Materialien und Gruppe C – mit Bauchfetttamponade. Die postoperative klinische und radiologische Bewertung der drei Gruppen wurde dokumentiert und auf Unterschiede in den Ergebnissen hin analysiert. Ergebnisse: Die postoperative klinische Beurteilung ergab keine signifikanten Unterschiede zwischen den drei Gruppen. In der Gruppe A wurden postoperativ weder Liquorfistel noch Keilbeinhöhlenentzündung oder Empty-Sella-Syndrom beobachtet. Allerdings konnte ein signifikanter Unterschied in der radiologischen Bewertung identifiziert werden: die Interpretation von sellären Inhalten bei der postoperativen MRT der Gruppe A gelang früher und zuverlässiger als in anderen Gruppen mit intrasellärer Tamponade. Schlussfolgerung: Es gibt keinen Unterschied in der Inzidenz von postoperativen Liquorfisteln und Empty-Sella-Syndromen bei den verschiedenen rekonstruktiven Techniken mit und ohne intrasellärer Tamponade, unabhängig von Größe und Ausdehnung des Hypophysenadenom. Die Sellarekonstruktion ohne intraselläre Tamponade, die während eines chirurgischen Standardverfahrens endoskopisch über einen endonasalen transsphenoidalen Zugang durchgeführt wird, ist anderen Techniken mit Tamponade nicht unterlegen und bietet zudem Vorteile bei der radiologischen Diagnostik, was sie zu der von uns bevorzugten Technik macht, solange offensichtlich keine intraoperative Liquorfistel vorliegt., GMS German Medical Science; 14:Doc07

Clinical manifestations of empty sella syndrome include hypopituitarism cerebrospinal fluid rhinorrhea, headache, and visual abnormalities. A 21-year-old woman reported a 6-month history of worsening vision 3 years after decompression of a sellar-suprasellar Rathke cleft cyst. Her magnetic resonance imaging (MRI) showed a well-defined recurrent cyst in the sellar-suprasellar region causing chiasmatic compression. She underwent an endonasal, endoscopic decompression of the cyst, with subsequent improvement in her vision. A postoperative computed tomography confirmed good decompression of the cyst. Ten days after surgery, she reported sudden loss of vision in both eyes. MRI revealed an empty sella with herniation of both anterior cerebral arteries and optic chiasm into the sella. She underwent transnasal packing of the sellar floor with fat graft and bone plaques, and experienced gradual improvement in vision in her right eye.

Alström syndrome (AS) is a rare monogenic ciliopathy characterized by cone-code dystrophy, leading to early blindness, and obesity. Early endocrinological dysfunctions, especially growth hormone deficiency and hypogonadism, are detected in about half of AS patients. This MRI study investigates the presence of pituitary gland abnormalities in a large cohort of AS patients.Pituitary morphological changes (gland flattening with partial or total empty sella) were evaluated on midsagittal high-resolution T1-weighted images of 32 AS patients (mean-age 23.2±9.4 years; range: 6-45, 15 females) and 21 unrelated healthy subjects (mean age 23.2±11.2 years; range: 6-43; 10 females).Among AS patients, 11/32 (34%) had total empty sella and 6/32 (19%) partial empty sella, while 3/21 (14%) of controls had partial empty sella and none presented with total empty sella (P0.005). AS patients harboring a total or partial empty sella did not differ from those with normal pituitary gland for gender (P=0.98), BMI (P=0.10) or visual impairment (P=0.21), while the presence of empty sella was associated with an older age (P=0.007) being especially frequent above the age of 30.Total or partial empty sella appears commonly during the course of AS. Pituitary gland flattening might represent the morphological underpinning of subtle endocrinologic dysfunctions and raises the need to further investigate the pituitary function in this rare ciliopathy.

Thyrotropin-secreting pituitary adenomas are very rare tumors, known to present overexpression of somatostatin receptor subtype 2 and which may consequently demonstrate abnormal uptake on Ga-DOTA-TOC PET/CT. A 67-year-old woman with a history of operated pituitary macroadenoma presented with symptoms of hyperthyroidism including a large goiter. Her serum thyroid hormone levels were in favor of central hyperthyroidism. Pituitary MRI depicted an empty sella but visualized an ambiguous lesion centered on the left sphenoidal sinus. Complementary Ga-DOTA-TOC PET/CT finally demonstrated intense uptake by the sphenoidal lesion, confirming recurrence of the pituitary adenoma.

Objective: Spontaneous cerebrospinal fluid (CSF) leaks represent a significant challenge due to frequent association with elevated intracranial pressure (ICP) and higher risk of surgical failure. The study objective was to review management strategy and identify factors associated with need for acetazolamide and/or ventriculoperitoneal shunt (VPS) placement. Study Design: Retrospective data analysis. Methods: Chart review performed from 1999 to 2009 at a tertiary-care medical center. Results: A total of 105 patients underwent CSF leak repair; 39 patients (37.1%) were treated for spontaneous CSF leaks. Mean age was 57.7 years and 33 were female (85%). Average body mass index (BMI) was 38.5 kg/m2. The most common sites were cribriform plate (51%), sphenoid lateral pterygoid recess (31%), and ethmoid roof (8%). All patients underwent endoscopic repair utilizing image guidance with multilayered closure in most cases. Five patients (12.8%) developed recurrent CSF leak with mean ICP of 27.0 cm H2O, compared to 25.0 cm H2O for those without recurrence (P = .33). All had successful rerepair at mean follow-up of 2.8 years. Acetazolamide was used in nine patients, whereas six patients underwent VPS placement for elevated ICP management. Diagnosis of benign intracranial hypertension (BIH) was statistically associated with need for acetazolamide or VPS (P < .001), whereas elevated ICP reached borderline significance (P = .049). Conclusions: Management of spontaneous CSF leaks requires a comprehensive strategy after endoscopic repair. Diagnosis of BIH may be associated with requirement of further ICP treatment. Close ICP monitoring, coupled with selective use of acetazolamide and VPS, may decrease risk of failure. Laryngoscope, 2010

Objective To propose an approach for the diagnosis of secondary adrenal insufficiency (AI) by presentation of 2 clinical cases and review of the literature. Methods We describe 2 patients who were considered to have a normal hypothalamic-pituitary-adrenal axis on the basis of an appropriate response to the high-dose (250 μg) cosyntropin stimulation test (HST), with use of a cutoff value of 20 μg/dL. Our first patient had undergone resection of a 4-cm pituitary tumor a few months previously, and the second patient had hyponatremia with empty sella syndrome. Both patients, however, had strong clinical evidence suggestive of secondary AI. On testing by the insulin tolerance test (ITT) in the first patient and the overnight metyrapone test (OMT) in the second patient, secondary AI was diagnosed. We reviewed the literature to compare the utility of the different tests for the diagnosis of secondary AI. Results An 8 am serum cortisol value less than 5 μg/ dL or above 13 μg/dL and a stimulated cortisol level less than 16 μg/dL on both the low-dose cosyntropin stimulation test (LST) and the HST as well as above 22 μg/dL on the LST and above 30 μg/dL on the HST can reliably predict the functional status of the hypothalamic-pituitaryadrenal axis in chronic secondary AI. Values between these utoff points may necessitate further assessment with the OMT or ITT based on clinical suspicion. Conclusion We recommend a 3-step diagnostic approach, with the first 2 steps performed together—starting with the 8 am basal cortisol determination in conjunction with either the LST (preferably) or the HST. In cases of an indeterminate response coupled with a strong clinical suspicion, the final step should be definitive testing with the OMT or ITT. (Endocr Pract. 2010;16:686-691)

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 χ2 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.

53 cases of partial empty sella turcica have been studied, both clinically and radiologically. Excluding 12 with an incomplete endocrine study, 41 remain of which 31 cases (75.6%) were found to have an adenohypophyseal hyperfunction of the hyperplasia (primary hypothyroidism, etc.) or adenoma type (acromegaly, etc.); 4 (9.7%) had a clear neurological cause (head trauma, thrombosis in the venous sinus, hydrocephalus) and only 6 (14.6%) were of unknown, primary or idiopathic cause. The published cases of "primary" partial empty sella have been revised, proposing: the substitution of the term "primary" for "spontaneous", since as we have already pointed out, the majority of the "spontaneous" cases are attributable to pituitary hyperfunction and a few of them to neurological or neurosurgical processes. Finally, we should like to emphasize the importance of empty sella in the prognosis and treatment of those patients whose most exact diagnostic terminology would be, for example, hypothyroidism "with" partial empty sella turcica, or, as another example, prolactinoma "with" empty sella.

The pituitary function in 20 patients with primary empty sella syndrome has been evaluated. The only endocrine symptom was secondary amenorrhoea in four patients. In half of the patients, pituitary function tests showed minor abnormalities, the most common being impaired growth hormone response to glucagon. In addition to the empty sella, other intracranial abnormalities such as pituitary tumour and hydrocephalus were found in four patients. Although commonly a benign condition, the empty sella may occasionally be associated with clinically important endocrine dysfunction, pituitary tumours and other intracranial abnormalities.

In 255 patients with evidence of a pituitary tumor, 7 patients presented with typical symptoms of meningitis, which all proved to be sterile. In these patients a pituitary adenoma was surgically verified in 4. In one patient the presence of acromegaly indicated a pituitary adenoma. In 2 patients an enlarged, empty sella was found, taken to reflect spontaneous disappearance of an adenoma. It is thought that the cause of the sterile meningeal reaction in the 7 patients was a spontaneous necrosis of the adenoma with expulsion of necrotic material into the suprasellar subarachnoid space. Although this sequence has been described before it has been considered to occur very rarely. Pituitary apoplexy as the underlying cause of meningitis should be suspected in patients with evidence of pituitary endocrine disturbances or lesion of structures adjacent to the fossa. The diagnosis may be established by computer tomography. Therapeutically, it may be of importance that the nature of the meningeal reaction is promptly recognized. Swift neurosurgical decompression of the sella and adequate hormonal substitution therapy may be essential.

A woman with isolated ACTH deficiency and empty sella in a background of primary antiphospholipid syndrome (APS) is described. A 24-year-old woman was admitted for premature delivery at 30 weeks of gestation and was found to have severe pulmonary hypertension and right sided heart failure. A few hours after delivery, without excessive blood loss, she suddenly lost consciousness, and was found to be hypotensive and hypoglycemic. The findings on hormonal evaluation were consistent with isolated secondary adrenal insufficiency as both ACTH and cortisol levels were very low. Magnetic resonance imaging of the pituitary fossa showed an empty sella. Her severe cor pulmonale was found to be due to primary APS. The patient was given replacement of glucocorticoid and treated with frusemide, spironolactone, warfarin, sildenafil and inhaled iloprost for her pulmonary hypertension, after which her clinical status improved substantially. Follow-up tests of the hypothalamic-pituitary axis found normal serum LH, FSH, TSH, FT4 and prolactin secretion; serum ACTH and plasma cortisol levels remained low.

Primary pituitary abscess is a rare pituitary pathology, particularly at a young age and is characterized by atypical clinical features making the diagnosis difficult. Correct diagnosis and therapy are mandatory due to the potentially lethal outcome of pituitary infection. We report the case of an adolescent girl presenting with headache, diabetes insipidus and central thyro-gonadic insufficiency with no history of infection, in whom the intra-operative diagnosis of primary pituitary abscess was made. Bacterial cultures indicated infection with Streptococcus spp. One year after neurosurgery and antibiotic therapy, recovery of diabetes insipidus and pituitary insufficiency was documented except for persistence of subnormal growth hormone secretion. Post-surgery, pituitary magnetic resonance imaging revealed an empty sella syndrome.