The coexistence of newly diagnosed acromegaly with primary empty sella: More frequent than expected?

OBJECTIVE: We investigated the coexistence of newly diagnosed acromegaly with primary empty sella (ES), which is considered to be a rare association, and the impact of ES on the laboratory, radiological and prognostic status of acromegaly. DESIGN: Acromegaly patients diagnosed and followed-up between 2012 and 2021 were included. Empty sella was defined as the pituitary gland and adenoma filling <50% of the sella turcica on preoperative T1 magnetic resonance imaging (MRI). RESULTS: 102 acromegalic patients (45 male, 57 female, 45.5 ± 12.8 (range: 20-70 years) were included and data of a median 3 years (range: 0.5-9 years) were presented. ES was detected in 19 (18.6%) patients and 4 had complete and 15 had partial ES. Although not significant, adenoma size and residual adenoma on MRI on postoperative 3rd month, and disease remission at last control were lower in acromegaly with ES than in acromegaly without ES, while the rate of female gender and remission on postoperative 3rd month were higher. While preoperative serum prolactin and nadir GH responses to OGTT were significantly lower in patients with ES, there was no difference in terms of other pituitary hormones among both groups. CONCLUSION: The present study revealed the coexistence of newly diagnosed acromegaly with primary ES at a rate of nearly 20% which is more frequent than expected and this association is not rare. The presence of ES was not associated with any preoperative/postoperative pituitary hormone levels and remission status, except lower preoperative prolactin and nadir GH responses to OGTT.

A Multicenter Cohort Study in Patients With Primary Empty Sella: Hormonal and Neuroradiological Features Over a Long Follow-Up.

Objective: primary 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. Design: multicenter retrospective cohort study enrolling patients referred between 1984-2020 to five Pituitary Units, with neuroradiological confirmed PES and a complete hormonal assessment. Methods: we 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). Results: we 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 (p<0.001), traumatic brain injury (p=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). Conclusions: this 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.

Bilateral Petrous Apex Cephalocele Associated with a Wide Sella.

ABSTRACT: 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.

Association of an empty sella and grave´s disease in a patient with acromegaly: a case report.

Acromegaly is, in most cases, caused by growth hormone secreting pituitary adenomas. Those patients often develop different pathologies of the thyroid gland, however, the occurrence of Grave´s disease is quite a rare situation. We report a case of a 64-year-old female patient who presented with signs of hyperthyroidism and imbalance of her diabetes mellitus. On physical examination, she had facial features of acromegaly. Biochemical testing confirmed the suspicion of acromegaly and Grave´s disease, with an elevated insulin-like growth factor-1 and a suppressed thyroid stimulation hormone (TSH) with positive TSH-receptor antibodies. A pituitary Magnetic Resonance Imaging (MRI) was performed, revealing a macro-adenoma and an empty sella. The patient successfully underwent a transsphenoidal surgery and obtained a remission of her hyperthyroidism under anti-thyroid drugs.

Pituitary Volume in Patients with Primary Empty Sella and Clinical Relevance to Pituitary Hormone Secretion: A Retrospective Single Center Study.

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.

Characteristics of Patients with Hypopituitarism having Onset Symptoms of Pseudo-Intestinal Obstruction: A Case Report.

BACKGROUND: Gastrointestinal symptoms are often the first symptoms of hypopituitarism. However, pseudo-intestinal obstruction is not a common manifestation of hypopituitarism. Some patients presenting with gastrointestinal symptoms as their chief complaint were admitted to the Department of Gastroenterology and were accurately diagnosed with hypopituitarism at the Department of Endocrinology. CASE SUMMARY: This case pertains to a 57-year-old man with poor appetite, fatigue, weakness, and recent onset recurring abdominal pain. An erect, abdominal X-ray indicated flatulence and gas-fluid levels in the midsection of the abdomen, and pseudo-intestinal obstruction was diagnosed. Subsequently, the patient was referred to the Department of Gastroenterology to identify the cause of the pseudo-intestinal obstruction. An examination of the digestive system did not reveal any abnormalities, but the patient developed hyponatremia and exhibited drowsiness. The patient was transferred to the Department of Endocrinology for further treatment. The patient was eventually diagnosed with hypopituitarism, caused by empty sella syndrome. The patient received prednisone and euthyrox replacement therapy, and pseudo-intestinal obstruction did not occur again. CONCLUSION: In general, endocrine diseases, including hypopituitarism, hypothyroidism, and hyponatremia, should be considered for patients with pseudo-intestinal obstruction combined with hyponatremia and drowsiness, especially if the symptoms of the digestive system are not complicated and the drowsiness is obvious.

Extradural Remodeling in Empty Sella Syndrome: 3-Dimensional Operative Video.

Sellar arachnoidocele is a term used to define the herniation of the subarachnoid space to the sella.1 This is a rare radiologic finding that, in most cases, does not require treatment.2-5 When symptoms appear, the term empty sella syndrome is used. Two varieties exist: primary and secondary empty sella syndrome.2 The aim of this 3-dimensional operative video (Video 1) is to demonstrate the extradural microsurgical remodeling of the sellar fossa with autologous bone in 2 cases of primary empty sella syndrome. Both patients signed an informed consent for the procedures and agree with the use of their images for research purposes. In both cases, magnetic resonance imaging scans showed herniation of the subarachnoid space into the pituitary fossa and an anchor-like silhouette on coronal view. Patients evolved favorably, improving their visual deficit after the surgery, as can be observed in the postoperative visual field study. If surgery is indicated due to visual loss, the procedure is known as chiasmapexy. Recently, Guinto et al3 described a technique for chiasmapexy. Our team considers this procedure to be useful, technically simple, and low cost. Being autologous, rejection possibilities are almost null. This 3D video serves as a complement to illustrate the technique.

Central diabetes insipidus unveiled by glucocorticoid therapy in a patient with an empty sella: A case report and literature review.

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-Barre[Combining Acute Accent] 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.

Does pituitary compression/empty sella syndrome contribute to MRI-negative Cushing's disease? A single-institution experience.

OBJECTIVE: Cushing'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. METHODS: A 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. RESULTS: Of 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). CONCLUSIONS: Empty 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.

Hipertensión craneal, silla turca vacía primaria y papiledema / Intracraneal hypertension, primary empty sella and papilloedema

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Growth hormone deficiency with late-onset hypothalamic hypoadrenocorticism associated with respiratory and renal dysfunction: a case report.

BACKGROUND: The 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 PRESENTATION: This 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 CO2 narcosis at 65 years of age, and noninvasive positive pressure ventilation was started. CONCLUSIONS: The 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.

[Ectopic pituitary adenoma associated with empty sella turcica]. / Adenoma hipofisario ectópico asociado a silla turca vacía.

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.

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 (AU).

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 (AU).

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.

Petrified pinna and pericarditis in autoimmune polyendocrine syndrome.

Petrified pinna refers to the calcification or ossification of the external auricular cartilage. It is an uncommon clinical entity and is most often associated with local trauma, frostbite or inflammation. Auricular calcification may be the exclusive cutaneous marker of underlying endocrinopathy. It has been most commonly associated with adrenal insufficiency and other endocrine conditions like diabetes mellitus, hypothyroidism and acromegaly. We present a 47-year-old Caucasian manwho presented with acute pericarditis with tamponade physiology, who was found to have petrified pinnae as a telltale sign of the underlying autoimmune polyendocrine syndrome type 2.

Partial empty sella in a woman with cerebral venous sinus thrombosis: A rare presentation of polycythaemia rubra vera.

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.

Nivolumab-induced hypophysitis leading to hypopituitarism and secondary empty sella syndrome in a patient with non-small cell lung cancer.

We describe the clinical course of a 64-year-old woman with stage IVa lung adenocarcinoma who presented with over 1 month of fatigue, unintentional weight loss and emesis. She initiated treatment with nivolumab immunotherapy 1 year prior and had been tolerating the treatment well. A comprehensive workup revealed multiple endocrinological abnormalities consistent with hypophysitis leading to hypopituitarism in the form of central adrenal insufficiency and hypogonadism as well as a partially empty sella on imaging. This case demonstrates that while receiving novel forms of treatment such as immunotherapy, patients should be monitored closely for a wide range of adverse effects.

Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome.

BACKGROUND: Empty sella is an anatomic finding that is usually asymptomatic. However, when patients with empty sella finding present with visual deficits, surgical treatment may be necessary. The main goal of surgery is to elevate sellar content through a transsphenoidal approach. The aim of this study was to demonstrate a new technique for precise reconstruction of the sellar floor using a heterologous bone block to restore the anatomic elements of the sella turcica. METHODS: Three patients with primary empty sella who presented with visual field defects were prospectively included. Surgery was performed through a microsurgical transsphenoidal approach and involved elevating the sellar content by using a tricortical heterologous bone graft, the dimensions of which were obtained considering the exact dimensions of each patient's sella turcica. The graft was premodeled outside the surgical bed and carefully introduced into the sella turcica to achieve the required elevation (chiasmapexy). RESULTS: Vision of all 3 patients improved, and the improvement persisted throughout the follow-up period. 2 patients, this improvement was noted immediately after surgery, and in the third patient, this change in vision occurred 1 week later. Long-term imaging studies showed the persistence of the bone graft in the sella turcica and the exact elevation of the sellar content at the end of the follow-up period. There were no complications in the present series. CONCLUSIONS: The technique presented here is simple and reproducible and allows an almost exact and persistent elevation of the sellar content.