Intracranial hypertension and empty Sella from adrenal adenoma and excessive and prolonged steroid usage: a case report.

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.

Primary Empty Sella.

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.

Tectal glioma presenting with clinical triad of obesity, amenorrhea and central cord syndrome with radiological pentad of hydrocephalus, empty sella, suprapineal diverticula, Chiari and syrinx.

BACKGROUND: Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. CASE DESCRIPTION: 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. CONCLUSION: 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.

Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: case report with literature review.

Ectopic pituitary adenomas are uncommon entities that may pose substantial diagnostic challenges. In the majority of these cases, patients present with endocrine and/or nasal obstruction symptoms. We report the case of an ectopic pituitary adenoma in a 76-year-old man with an empty sella who initially presented with right-sided hearing loss progressing to bilateral hearing loss over the next 4 years. Neuroimaging studies revealed a large, expansile central skull base mass replacing the clivus and sphenoid sinus, and invading the internal auditory canals and inner ear bilaterally. The tumor also involved the floor of the middle cranial fossae and bilateral medial temporal and occipital bones. Histopathologic examination, including immunohistochemical studies, revealed a sparsely granulated lactotroph adenoma. Hearing loss in a patient with ectopic pituitary adenoma constitutes an extremely unusual presentation. This case was further complicated by the presence of an empty sella and the absence of symptoms related to hyperprolactinemia.

Evaluation of obstetrical factors related to Sheehan syndrome.

AIM: To evaluate factors related to the occurrence of Sheehan syndrome. METHODS: The obstetrical disseminated intravascular coagulation score, total volume of hemorrhage, shock index, level of consciousness at the time of shock occurrence and pituitary magnetic resonance imaging findings were evaluated in nine women who showed massive hemorrhage during delivery. These clinical outcomes were analyzed in all these patients who were prospectively followed-up to identify any possible occurrence of Sheehan syndrome. RESULTS: Compared to six women with non-Sheehan syndrome, three women who were diagnosed with Sheehan syndrome showed significant elevation of the obstetrical disseminated intravascular coagulation score, decrease in the level of consciousness during shock and remarkable pituitary gland atrophic change with an empty sella turcica detected by pituitary magnetic resonance imaging. The volume of hemorrhage during delivery and shock index were not significantly different between these two groups of women. CONCLUSION: Careful attention and follow-up should be paid to women with post-partum massive hemorrhage for early detection and management of women with Sheehan syndrome.

Autoimmune polyglandular syndrome: an unusual presentation with empty sella, premature ovarian failure, and Hashimoto's thyroiditis associated with thyroid cancer.

Autoimmune polyglandular syndromes are rare disorders characterized by failure of several endocrine glands, as well as non-endocrine organs, associated with immune-mediated tissue destruction. We report a rare case of polyglandular syndrome type II in a patient who presented with premature ovarian failure, Hashimoto's thyroiditis and empty sella associated with a diagnosis of differentiated thyroid carcinoma. This case probably represents the first report on this tumor in a patient with polyglandular disorder.

Precocious puberty and empty sella syndrome in a girl cured of acute lymphoblastic leukemia.

We describe a case of precocious puberty in a girl treated with chemoradiotherapy according to the Italian Association of Pediatric Hematology and Oncology ALL 9503 protocol for acute lymphoblastic leukemia (ALL) from the age of 15 months until the age of 3 years and 4 months. The patient was treated with chemotherapy and cranial irradiation (18 Gy in 12 fractions). At 7 years of age, during topical estrogenic treatment for congenital adhesions of the labia minora, she showed bilateral breast development that evolved into precocious puberty. A magnetic resonance imaging of the brain showed an "empty sella" (ES); the etiology of the ES, and the consequent precocious puberty, being presumably iatrogenic. Children treated with cranial radiotherapy should be carefully checked for signs of precocious puberty and the exogenous administration of estrogens should be avoided, as far as possible, because these could act as a trigger factor in a population at higher risk of precocious puberty.

Primary pituitary abscess followed by empty sella syndrome in an adolescent girl.

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.

[Reversible primary empty sella. Case report]. / Silla turca vacía primaria reversible. A propósito de un caso.

We report the case of a 13-year-old girl with an increased hypophysis that mimicked an adenoma, who developed a partial empty sella on MRI after an acute episode of hydrocephalus. After replacement of a CSF shunt, the intracranial pressure returned to normal and the hypophysis filled up again all the sellar fossa. We discuss the possibility of the involvement by an ischemic atrophy of the adenohypophysis in the development of a primary empty sella with idiopathic chronic raised intracranial pressure that prevents the recovery of the gland volume after restoring the intracranial pressure to normal values. Restitution of empty sella may be an indicator of normal intracranial pressure in these cases.

A case of hypothalamic panhypopituitarism with empty sella syndrome: case report and review of the literature.

Empty sella syndrome is frequently accompanied with pituitary dysfunction. Most of the patients with empty sella syndrome demonstrate primary pituitary or stalk dysfunction and few cases show hypothalamic dysfunction. A 71-year-old man manifested appetite loss, nausea and vomiting with hyponatremia and adrenal insufficiency. Hormonal evaluation and cranial MRI revealed a panhypopituitarism with empty sella. Intriguingly, while the response of ACTH to CRH administration was exaggerated, the response to insulin hypoglycemia was blunted. Serum PRL levels were normal. Further, decreased level of fT4, slightly elevated basal levels of TSH, and delayed response of TSH to TRH administration were observed. These findings strongly suggest that the panhypopituitarism is caused by hypothalamic dysfunction. The presence of autoantibodies to pituitary and cerebrum in the patient's serum implies an autoimmune mechanism as a pathogenesis.

Empty sella may be the final outcome in lymphocytic hypophysitis.

INTRODUCTION: Lymphocytic hypophysitis (LH) is an autoimmune disorder associated with the infiltration of the pituitary gland by lymphocytes leading to different degrees of hypopituitarism. Females are affected more frequently than males and the disease is usually associated with pregnancy or postpartum period. CASE: We present a case of LH who was first diagnosed with diabetes insipidus and hyperprolactinemia. In the follow-up, the patient developed growth hormone, gonadotropin, and thyroid stimulating hormone deficiency. The typical appearance of increased stalk thickness and diffuse homogenous contrast enhancement of pituitary on magnetic resonance imaging resulted in empty sella by time. CONCLUSION: The present case demonstrates the natural course of LH over a 13-year period in which the empty sella was the final outcome.

Anterior skull base cerebrospinal fluid fistulas in Puerto Rico: treatment and outcome.

BACKGROUND: The surgical management of anterior skull base cerebrospinal fluid (CSF) fistulas has evolved throughout the last decade. Endonasal endoscopic surgery of the anterior skull base has become the standard procedure for the repair of most these fistulas. OBJECTIVES: To describe the presenting symptoms, etiology, treatment, and long-term outcomes of patients with anterior skull base CSF fistulas treated endoscopically at our institution. MATERIAL/ METHODS: Retrospective analysis of 25 patients with CSF fistulas treated endoscopically at the University of Puerto Rico Hospital from November 2004 to August 2008. RESULT: The etiology was spontaneous leak in 10 patients, menigoencephalocele in 7 patients, previous sinus surgery in one patient, and trauma in one patient. The most common location of leak was the cribiform plate, followed by the ethmoid roof. An overlay technique was used for repair in 61% of the procedures versus 39% for the underlay technique. The mean follow up was 23 months. Our overall initial rate of closure was 94% with 100% after a second procedure. CONCLUSIONS: Endoscopic repair of anterior skull base CSF fistulas has a high success rate and lower morbidity and mortality when compared with open approaches.

Empty Sella Is a Sign of Symptomatic Lateral Sinus Stenosis and Not Intracranial Hypertension.

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.

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.

Endonasal Chiasmapexy Using Autologous Cartilage/Bone for Empty Sella Syndrome After Cabergoline Therapy for Prolactinoma.

BACKGROUND: Visual field deterioration caused by secondary empty sella after cabergoline therapy for prolactinoma is a rare event. Chiasmapexy is performed to treat empty sella syndrome. Although various materials have been used for the elevation of the optic chasm, the most appropriate material remains to be established. Here, we describe the efficiency of chiasmapexy for empty sella syndrome following dopamine agonist treatment and the utility of septal cartilage and sphenoidal sinus bone as materials for chiasmapexy. CASE DESCRIPTION: A 35-year-old male with a history of cabergoline therapy for prolactinoma presented with visual deterioration. His magnetic resonance imaging revealed optic chiasm herniation into the empty sella. Endoscopic endonasal transsphenoidal chiasmapexy was performed using septal cartilage and sphenoidal sinus bone as materials for elevating the chiasm. Visual function improved immediately after operation. CONCLUSIONS: Chiasmapexy is an effective surgical method for treating visual deterioration caused by empty sella after cabergoline treatment. Endoscopic endonasal chiasmapexy with septal cartilage and sphenoidal sinus bone is a considerable option because it is minimally invasive and involves decreased risk of infection.

[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.

Empty sella associated with growth hormone deficiency and polydactyly.

Empty sella means the absence of the pituitary gland on cranial computed tomography or magnetic resonance imaging. Empty sella syndrome is the pathological variant of the imaging-described empty sella. We present the case of a male Caucasian child, aged four years and two months, for short stature and diagnosed by imaging procedures as empty sella. The cause of short stature was isolated growth hormone (GH) deficiency. Associated he presented left hand postaxial polydactyly. In connection with this particular case, we propose a review of current knowledge in empty sella syndrome. The particularity of reported case consists of association empty sella with GH deficiency and polydactyly. The association of empty sella with polydactyly is not reported yet in the medical literature and is probably coincidental.

The empty sella.

Empty sella (ES) is an extension or herniation of the subarachnoid space into the pituitary fossa through an incompetent sellar diaphragm that is commonly associated with clinical manifestations and endocrine abnormalities. The ES may occur secondary to a variety of pituitary disorders or as a primary entity. The pathogenesis of the primary ES appears to be multifactorial though the precise mechanisms remain unclear. The etiology of ES in children may differ from that in adults and is not uncommon in the presence of pituitary disorders. Patients with the ES should always undergo endocrine, neurologic, and ophthalmologic evaluation at the time of initial presentation and should be monitored as determined by the initial results. Treatment should be individualized as the clinical features and biochemical abnormalities vary widely among patients from the asymptomatic state with a normal hormone profile to sight-threatening visual disturbances, CSF rhinorrhea, and panhypopituitarism. The ES should not be considered as merely an incidental finding and clinicians should be aware of its varying presentations and ramifications.