Empty Sella Research Articles

Clinical features and enzyme replacement therapy in 10 children with Fabry disease.

To summarize the clinical features, diagnosis and enzyme replacement therapy(ERT) of Fabry disease (FD) in children. The clinical data, laboratory tests, genetic variations and treatment of 10 FD children diagnosed in Shandong Provincial Hospital from September 2020 to June 2022 were retrospectively analyzed. Among the 10 cases from 6 families, 7 patients were boys of 4 to 13 years of age, and 3 were girls of 12 to 15 years of age. There were 7 symptomatic patients, including 6 boys and 1 girl. All 7 patients presented with acral neuralgia. Five patients had little or no sweating. Five patients presented with cutaneous angiokeratoma. Two patients had abdominal pain. One patient developed joint symptoms. Four patients had corneal opacity. One patient had hearing loss; one patient had short stature. One patient had mild proteinuria and 1 patient had dysplasia of the right kidney with decreased eGFR (55.28 ml/min.1.73 m2). The left ventricular mass index was slightly elevated in 1 patient. Three patients had mild obstructive ventilatory dysfunction; a small amount of effusion in the intestinal space of the lower abdomen or mild fatty liver was found in 2 patients. Partial empty sella turcica in 1 patient. A total of 6 GLA gene variants were detected in 10 children, among which C.1059_1061delGAT (p.met353del) was a newly discovered mutation. Five children received ERT, of which 4 were treated with agalsidase beta and 1 was treated with agalsidase alpha. Only 1 patient had anaphylaxis. Lyso-GL-3 levels decreased significantly in the first 3 months of ERT initiation and remained relatively stable thereafter in 3 patients. The Lyso-GL-3 level was decreased, but renal impairment continued to progress in 1 patient treated with agalsidase alpha. The clinical manifestations of FD in childhood are diverse, and it is necessary to make a definite diagnosis by combining family history, enzyme activity, biomarkers, gene testing and other indicators. Pedigree screening and high-risk population screening are helpful for early identification, early diagnosis and early treatment. No serious adverse reactions were found during the short-term treatment with agalsidase alpha and beta.

Brain MRI findings in patients with bullous pemphigoid: A case-control study.

Evidence suggests that bullous pemphigoid (BP) is associated with multiple neurological disorders. We aimed to compare brain magnetic resonance (MRI) findings between BP patients and a control group. This case-control study included patients with BP referred to two dermatology clinics during a two-year period. A group of individuals attending the same clinics for cosmetic procedures were selected as controls. First, participants' general information including age, gender, education, weight and underlying disease was recorded. For BP patients, the drugs and the BP Disease Area Index (BPDAI) were recorded as well. Then, all participants underwent brain MRI without contrast. The Fazekas scale, the general cerebral atrophy (GCA) score, and the Medial Temporal lobe Atrophy (MTA) score were used to assess MRI images. Overall, 24 BP patients and 24 controls were evaluated in this study. Both groups were comparable regarding age, gender and education. However, diabetes and hypertension were more frequent in the control group. The mean BPDAI total score was 51.39 ± 68.92 in BP patients and most of them used rituximab (41.7%). None of the participants had MS or Alzheimer MRI patterns. There was no difference between groups in terms of GCA and MTA scores. Furthermore, the frequency of partially empty sella did not differ significantly between BP patients and controls (p= 0.461); nevertheless, grade-3 Fazekas was significantly higher in BP patients compared to controls (25% vs. 0%, p= 0.019). Of note, one BP patient had an epidermoid cyst and another had moderate enlargement of three ventricles. Also, new infarcts were observed in two and old infarcts in four BP patients. Although the majority of abnormal brain MRI findings were more frequent in BP patients compared to controls, only grade-3 Fazekas was significantly higher and acute infarcts were exclusively observed in BP patients.

The Need for Emphasis on Intracranial Hypertension Management in Spontaneous Skull Base Meningoencephaloceles

To provide an analysis of patients with spontaneous skull base meningoencephaloceles (MECs) to determine whether definitive surgical treatment requires management of elevated intracranial pressure (ICP). Data of 10 subjects with spontaneous MECs were collected and retrospectively evaluated. Measurement of ICP, prior interventions, treatment with acetazolamide, and characteristics of long-term elevated ICP, among others, were analyzed. Our own indications for cerebrospinal fluid (CSF) diversion and use of postoperative external lumbar drain were analyzed as well. The sphenoid region was the most common location of MECs. CSF leak was diagnosed in all subjects. The most common graphical signs of elevated ICP were empty sella and arachnoid pits, both of which were present in 90% of cases. Lumbar puncture with opening pressure measurement was performed in 7 patients. Ventriculoperitoneal shunt insertion was indicated in 4 cases before skull base repair and in 2 cases after skull base repair. Two postoperative CSF leaks were managed with external lumbar drain and subsequent shunt installation. Spontaneous MECs are often associated with CSF leak. ICP assessment should be a standard of care to ensure successful operative repair of MECs. Insertion of a CSF diversion device must be considered where direct or indirect signs of intracranial hypertension are present.

Sustained hypercortisolemia after bilateral adrenalectomy in Cushing's disease

Abstract Introduction Cushing's disease caused by pituitary corticotropin (ACTH)-secreting tumors. These tumors are almost always benign and are usually microadenomas. In 30 to 40 percent, the microadenoma is so small that it is not detectable by magnetic resonance imaging (MRI). Primary therapy consists of transsphenoidal surgery or pituitary irradiation. Patients who fail this first approach can be treated either by repeat transsphenoidal surgery, medical therapy, radiotherapy, or, as a final resort, surgical or medical adrenalectomy. Clinical Case A 38-year-old male patient presented with headache and fatigue. The laboratory examinations reviewed ACTH depending Cushing's syndrome. A suspicious lesion in the pituitary with no contrast enhancement was observed in Sella MRI. Cavernous sinus sampling consistent with central sources. He was operated in 2018 for pituitary Cushing's disease. Reticulin framework disrupted compatible with adenoma but, ACTH could not be stained in immunohistochemical staining for technical reasons. Due to the high cortisol levels of the patient in the postoperative follow-up, the patient underwent bilateral adrenalectomy in 2019. Because of active Cushing's disease, patient was re-evaluated in 2022. In laboratory examinations, HGB 16.6 g/dL, WBC 6400 /uL, NEU 3200 /uL, PLT 228000 uL, cre 0.76 mg/dL, TSH 1.5 mIU/L, FT4 1.2 ng/dl, FSH 4.6 U/L, LH 7.98 U/L, PTH 23.7 ng/L, PRL 17 µg/L, Total Testosterone 571 ng/dl, Progesterone 0.06 µg/L, GH 0.04 ug/L, IGF1 83 µg/L were detected. Basal cortisol was 35 ug/dl and ACTH 67 ng/L. Cortisol with 1 mg of DST resulted in 28 ug/dl. 2 mg DST resulted in cortisol 38 ng/L. Free cortisol in the urine was found to be 281 mg/day (n<130 mg/day). Empty sella was observed in sella MR. No prominent contoured lesion was observed in the pituitary gland. Adrenal gland was not observed in the Abdominal MRI. No tumor was detected in Thorax CT. The patient's pasireotide 0.3 mg 1×1 treatment was continued. Metyropane 250 mg 2×1 was added to the treatment. Conclusion In Cushing's disease, the ACTH-secreting adenoma may be too small to be detected.

Compare of Brain Anomalies and Psychopathology of Patients in a Psychiatric Clinic: A Retrospective Study

Background: In parallel with the development of neuroimaging methods, become increasingly possible to explore the relationship between mental disorders and structural brain lesions. It is aimed to examine the relationship between the clinicalfeatures and psychopathologies of psychiatric patients and their anomalies. Methods: Two hundred-six female patients who were treated in Clinic of Psychiatry, Rize Training and Research Hospital between01/Jan/2016 and 31/Dec/2021 and had brain magnetic resonance imaging (MRI) were included in this study. Sociodemographic and clinical characteristics data form was recorded for each patient. Simultaneously, the size and location of the anomalies were recorded by scanning the brain MRI. Results: When the brain MRI scans of the patients were evaluated, 56% of 206 patients had at least one of the related anomalies. Enlarged Virchow-Robin space (VRS) in 2.4%, mega cisterna magna (MCM) in 14.6%, total empty sella (ES) in 8.3%, partial ES in 27.7%, cavum vergae (CV) in 1.9%, hippocampal remnant cyst (HRC) in 17%and 6.8% had arachnoid cyst (AC) findings. When the sociodemographic data and clinical characteristics of the patients were compared with the brain MRI findings, a significant correlation was found between the presence of MCM and the use of antidepressant medication (p=0.026). A significant correlation was found between the presenceof AC, disease duration and regular treatment history (p=0.0002). No significant correlation was found between otherdata and MRI (p>0.05). Conclusions: In our study, it is noteworthy that a higher rate of anomaly was detected in brain imaging of caseswith a history of psychiatric diagnosisand treatment comparedto the general population.

Analysis of clinical features of primary empty sella

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

Empty sella syndrome managed with homoeopathy: A case report

The empty sella Syndrome, is a condition resulting from herniation of the subarachnoid space through the diaphragma sella, displacing the normal pituitary gland. Empty sella can be of two types: partial or complete. Partial means, cerebrospinal fluid fills less than 50% of the sella. Total means cerebrospinal fluid fills more than 50% of sella, with the thickness of gland being < 2 mm. This is a case of a 40-year-old female, with recurrent attacks of sudden and excruciating pain in head with redness in right eye. She was admitted on 5/2/2020 having IP NO: 747, at Govt. Homoeopathic Medical College Kozhikode, for excruciating pain in right side of head radiating to right eye, ear, face and back of head, with redness in right eye. Clinical diagnosis was made after reviewing MRI brain. As in conventional medication, without the analgesic, managed successfully with Homoeopathic Medications. After individualising the case, homoeopathic medicine Apis mellifica 6 C and Onosmodium Q was prescribed, and within 1 week, her ailments got better.

The Link Between Empty Sella Syndrome, Fibromyalgia, and Chronic Fatigue Syndrome: The Role of Increased Cerebrospinal Fluid Pressure.

The etiopathogenesis of fibromyalgia (FM) and chronic fatigue syndrome (CFS) is not yet elucidated. Hypothalamo-pituitary-adrenal (HPA) axis dysfunction is reflected in the hormonal disturbances found in FM and CFS. Some study groups have introduced a novel hypothesis that moderate or intermittent intracranial hypertension may be involved in the etiopathogenesis of FM and CFS. In these conditions, hormonal disturbances may be caused by the mechanical effect of increased cerebrospinal fluid pressure, which hampers blood flow in the pituitary gland. Severe intracranial pressure may compress the pituitary gland, resulting in primary empty sella (ES), potentially leading to pituitary hormone deficiencies. The aim of this narrative review was to explore whether similar hormonal changes and symptoms exist between primary ES and FM or CFS and to link them to cerebrospinal fluid pressure dysregulation. A thorough search of the PubMed and Web of Science databases and the reference lists of the included studies revealed that several clinical characteristics were more prevalent in primary ES, FM or CFS patients than in controls, including increased cerebrospinal fluid pressure, obesity, female sex, headaches and migraine, fatigue, visual disturbances (visual acuity and eye motility abnormalities), vestibulocochlear disturbances (vertigo and neurosensorial hearing loss), and bodily pain (radicular pain and small-fiber neuropathy). Furthermore, challenge tests of the pituitary gland showed similar abnormalities in all three conditions: blunted adrenocorticotropic hormone, cortisol, growth hormone, luteinizing hormone, and thyroid stimulating hormone responses and an increased prolactin response. The findings of this narrative review provide further support for the hypothesis that moderately or intermittently increased cerebrospinal fluid pressure is involved in the pathogenesis of FM and CFS and should stimulate further research into the etiopathogenesis of these conditions.

Pituitary imaging findings in pediatric patients with idiopathic hypogonadotropic hypogonadism.

Objective. Idiopathic hypogonadotropic hypogonadism in children is a disease leading to a puberty absence. Some hypothalamic and pituitary defects cause hypogonadotropic hypogonadism. Pituitary magnetic resonance imaging is routinely performed in these patients. In our study, we provide an information about pituitary pathologies associated with an idiopathic hypogonado-tropic hypogonadism in childhood. Methods. Twenty-two patients, who were admitted to the pediatric endocrine outpatient clinic of our hospital because of their undeveloped secondary sex characteristics during adolescence, were included in our study. Age, gender, history, physical examination findings, and laboratory tests were recorded in patients. Pituitary magnetic resonance imaging results were examined. The criteria for the diagnosis of hypogonadism were: absence of puberty or delayed puberty, clinical signs or symptoms of hypogonadism, and presence of low or normal gonadotropin levels. Results. In the present study, 22 patients were diagnosed with hypogonadotropic hypogonadism. The mean age of the patients was 15.90±1.09 years. Basal and stimulated luteinizing hormone and follicular stimulating hormone levels of the patients were found to be low. Prolactin, cortisol, adrenocorticotropic hormone, free thyroxine, and thyroid stimulating hormone levels were within normal limits in all patients. The pituitary magnetic resonance imaging revealed six patients with pituitary adenoma, one with empty sella turcica, and five with pituitary hypoplasia. Conclusions. The present data showed that in the presence of hypogonadotropic hypogonadism, the hypothalamic-pituitary abnormalities are more likely to be present in the children compared to the adult population. Thus, it can be strongly emphasized the importance of the pituitary imaging examination, especially in the idiopathic hypogonadotropic hypogonadism cases.

Aneurysmal subarachnoid haemorrhage in the pituitary fossa of a patient with acromegaly - a rare phenomenon.

The anatomy of the pituitary fossa is complex. The wall of the fossa can vary, resulting in inconsistencies in the nature and integrity of the sella barrier. Cerebrospinal fluid is generally confined to the subarachnoid space and does not circulate freely in the pituitary fossa. Spontaneous haemorrhage in the fossa typically occurs in the context of pre-existing intrasellar pathology such as a pituitary adenoma. Extravasation of blood into the subarachnoid space can rarely be observed following pituitary apoplexy. We describe the unique occurrence of subarachnoid haemorrhage in a largely empty pituitary fossa after the rupture of a cerebral aneurysm. Pituitary apoplexy and subarachnoid haemorrhage (SAH) are both high in the differential diagnosis of sudden onset severe headaches. Haemorrhagic pituitary apoplexy may result in extravasation into the subarachnoid space, resulting in typical SAH symptoms and signs. This is the first reported case of primary SAH resulting in blood pooling in an empty sella arising from previous surgical resection of a large macroadenoma.

Role of Magnetic Resonance Imaging in the Evaluation of Sellar, Parasellar and Suprasellar Lesions

Introduction: Magnetic resonance imaging (MRI) is now widely available, and considerable body of experiencehas been accumulated in using it to evaluate this region. MRI is now accepted as the imaging procedure of choicein the evaluation of sellar and parasellar pathology.The aims and objectives are to know the characteristic MRIfeatures of the sellar, parasellar and suprasellar lesions and to describe the incidence of the various lesions andtheir age and sex distribution in the study population.Methodology: Prospective study of 30 patients referred for imaging having clinical features raising suspicion ofsellar, parasellar and suprasellar lesions. They underwent magnetic resonance imaging of the brain-sella using1.5 TESLA PHILIPS ACHIEVA MRI MACHINE in the Department of Radiodiagnosis, KIMS &amp; RF, Amalapuram.Results: Out of 30 cases 4 cases were pituitary macroadenoma, 3 cases each of pituitary microadenoma,craniopharyngioma, meningioma and aneurysm. 2 cases of each hypothalamic hamartoma, Pituitary colloidcyst, Rathke’s cleft cyst, Arachnoid cyst and primary empty sella were found. And 1 case of each Suprasellartuberculoma, Trigeminal schwannoma, Suprasellar retinoblastoma and PNS rhabdomyosarcoma were found.Conclusion: MR imaging characteristics of the most common lesions like pituitary macroadenoma, microadenoma,craniopharyngioma, meningioma and aneurysm were sufficiently distinct to allow them to be differentiatedfrom each other and from most other entities. Other characteristics such as extrasellar versus intrasellar location,nature of contrast material enhancement, the presence of cystic components, and clinical findings permit furtherdifferentiation among the various other abnormalities. The superior resolution and multiplanar capacity of MRimaging best depicts the extent of sellar, parasellar and suprasellar lesions

Intracranial Pathologies and Endocrine Results: Single Pediatric Endocrinology Center Experience

ABSTRACT Intracranial pathologies can affect pituitary hormone levels whether they are primarily related to the pituitary gland or not. In pituitary hormone deficiencies, the size and location of the pituitary gland may be normal, as well as one or more hormone effects may be observed after hypoplasia, partial empty sella, ectopic neurohypophysis, Rathke's cleft cyst, adenomas and other pituitary masses or surgeries. In early puberty, the pituitary dimensions are usually increased, rarely hypoplasia and rathke cleft cyst are seen as MRI findings. Non-pituitary pathologies cause especially early puberty, single or multiple pituitary hormone deficiency. In this study, we aimed to categorize the patients with pituitary/cranial lesions and hormonal pathology and to determine their frequency and distribution in the single pediatric endocrinology clinic for ten years. The data of 485 patients, 186 (38.3%) male, were retrospectively analyzed. Their mean age was 9.3 ± 3.2 years. The incidental pituitary lesion was found in 28 (5.77%) of all cases. There was no hormonal influence in 20 (71.4%) of them. In most of the 454 cases with hormonal disorders, MRI (Magnetic Resonance Imaging) findings were normal in most of the 454 cases with hormonal disorders (71.8%). The most common hormonal pathologies were precocious puberty and isolated Growth Hormone deficiency, 57.9% and 26.4% respectively. Non-pituitary lesions (n=23, 5%) were most frequently accompanied by early puberty (39.1%). The hypothalamus-pituitary axis may be affected by primary lesions in its own region, or it may be seen as a result of other cranial pathologies. Cranial evaluation is also important in addition to pituitary imaging in pituitary hormone disorders.

Hipopitüitarizm ile Birlikte Olan Papiller Tiroid Kanserinin Takibinde Zorluk: Olgu Sunumu ve Literatürün Gözden Geçirilmesi

Aim: We present an interesting case of papillary thyroid cancer co-existent with hypopituitarism.&#x0D; Case: Fortysix-year-old female was applied with a complaint of painful palpabl lump in the right side of the neck, difficulty swallowing, cough and dyspnea. The patient was diagnosed with type 2 diabetes mellitus 8 years ago, underwent craniotomy for nonfunctioning pituitary adenoma 15 years ago, and received gamma knife 10 years ago. She had been taking insulin glargine and lispro, prednisolone, and levothyroxine (LT4). Physical examination was unremarkable. Pituitary MRI revealed partial empty sella. A solid nodule of 33x27x30 mm with irregular borders and containing microcalcifications in the right thyroid lobe was detected on sonography. Fine needle aspiration cytology revealed “strongly suspicious features for malignancy”. Papillary thyroid carcinoma (PTC) was detected after right lobectomy and then complementary thyroidectomy. Follow-up sonography performed 14 months later than radioactive iodine (RAI) showed an avascular solid area of 30x14x15 mm in the right. We decided to monitor free thyroxine (fT4), thyroglobulin and anti-thyroglobulin levels. LT4 dose was adjusted to keep fT4 level closer to the upper limit of normal. No complications or recurrences were detected. &#x0D; Conclusion: Studies on the follow-up of PTC cases with hypopituitarism are limited. We performed RAI after total thyroidectomy, and treated the patient with LT4 by adjusting fT4 level.

Partial Empty Sella Syndrome Presenting with Panhypopituitarism and Masked Central Diabetes Insipidus

Empty sella syndrome (ESS) is an uncommon cause of hypopituitarism. It is usually detected incidentally or with neurological symptoms whereas it is rare to present with symptomatic endocrine dysfunction. A 48-year old previously healthy man presented with generalized body weakness, lethargy, fatigue and pos­tural dizziness of one month with no headache or visual disturbance. He also had features suggestive of hypo­gonadism and hypothyroidism. Panhypopitutarism secondary to partial ESS was diagnosed based on pituitary hormone testing and MRI brain. After starting glucocorticoid (GC) replacement patient developed polyuria and polydipsia and was diagnosed to have partial central diabetes insipidus (DI). He was successfully treated with hydrocortisone, thyroxin, intranasal vasopressin and testosterone. ESS causes significant pituitary dysfunction in considerable proportion of patients.

Clinical Profile of Patients with Bilateral Optic Disc Swelling Attending in an Apex Eye Hospital in Bangladesh

Background: Patient with optic disc swelling demands thorough ophthalmic as well as systemic evaluation as it may reflect intraocular as well as systemic diseases. Early diagnosis and proper management of bilateral disc swelling is important, as it can save vision as well as life. This study is aimed to determine the socio-demographic and clinical profile of patients with bilateral optic disc swelling in a tertiary eye hospital of Bangladesh.&#x0D; Methods: This cross sectional study had been conducted over 102 patients with bilateral disc swelling at the Neuroophthalmology clinic of National institute of ophthalmology and Hospital, Dhaka between April 2019 and March 2020. Patients were selected purposively based on specific selection criteria. Detail history was taken, ocular examinations like visual acuity, color vision, field of vision, pupillary light reaction, fundus examination were done and relevant investigations included color fundus photograph, visual field analysis, Computed tomography scans and Magnetic Resonance Imaging were performed. All the information’s were recorded in a pre-designed data collection sheet.&#x0D; Results: The mean age of the patients was 30.2±10.8 years where 37.3% patients were from 18-27 year age group. Females (67.7%) were commonly presented with bilateral disc swelling than male (33.3%). Most of the patients commonly presented with the symptoms like headache (71.6%), dimness of vision (62.8%), nausea &amp; vomiting (42.2%), ocular pain (21.6%) etc. Best corrected visual acuity of the study population was within 0-0.3 log MAR unit in the right eye in 55.9% cases and in the left eye in 59.8% cases. Majority of the patients (Right eye: 60.8%, Left eye: 63.7%) could read all plates while color vision assessment. About 20% patient had RAPD. About 17% patients had field defect in their right eyes whereas about 15% had visual filed defect in left eyes. Other than disc swelling, 14 patients had diplopia (13.7%), 12 patients had uveitis (11.8%). Out of 102 patients, 36 patients were clinically diagnosed as IIH (35.3%). Others were diagnosed as ICSOL (24.5%), bilateral optic neuritis (12.7%), VKH (12.7%), grade-IV hypertensive retinopathy (8.8%). The analysis of neuro imaging findings among the patients showed 72 patients (70.6%) had no space occupying lesions, 9 patients (8.8%) had meningioma, 6 patients had CP angle tumor (5.9%), 5 patients had empty sella (4.9%).&#x0D; Conclusions: Most of the Bangladeshi patients with bilateral disc swelling report to neuro-ophthalmology clinic in NIO&amp;H are female and belong to 18-37 years age group. Among them headache is the most common presenting complaint and IIH is the most common clinical diagnosis.&#x0D; J. Natl. Inst. Ophthalmol. 2022;5(1):39-45

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

ObjectiveWe 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. DesignAcromegaly 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). Results102 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. ConclusionThe 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.

Neuroimaging in the diagnosis and treatment of intracranial pressure disorders.

To review the clinical features, proposed pathophysiology, and the role of medical imaging in the diagnosis and treatment of idiopathic intracranial hypertension and spontaneous intracranial hypotension. The authors conducted a narrative review of the current literature on intracranial hypertension and hypotension syndromes, with a focus on imaging findings and role of neurointerventional radiology as a therapeutic option for these pathologies. Idiopathic intracranial hypertension commonly presents in obese women of childbearing age, being headache and papilledema the main clinical manifestations. Characteristic radiological findings consist of increased cerebrospinal fluid around the optic nerve, partially empty sella turcica and stenosis of the transverse sinuses. Transverse sinus stenting is a treatment alternative that has proven valuable utility in the recent years. Spontaneous intracranial hypotension in most of cases presents with orthostatic headache and has predilection for female population. The typical radiological features in the brain consist of subdural fluid collections, enhancement of the dura, engorgement of the venous structures, pituitary enlargement, and sagging of the brain. In this pathology, a cerebrospinal fluid leak in the spine associated with a defect in the dura, meningeal diverticulum, or a cerebrospinal fluid-venous leak must be actively ruled out. Neurologic complaints secondary to changes in intracranial pressure exhibit certain clinical features that in combination with fairly specific radiological patterns allow a highly accurate diagnosis. The diverse specialists in neurosciences should be aware of the multiple image modalities in the study of these syndromes as well as the treatment alternatives by neurointerventional radiology.

PMON125 A Case of Hypothalamic Lipoma Causing Hyperprolactinemia in a Patient With Family History of Birt-Hogg-Dubé Syndrome

Abstract A 41year old female with a medical history significant for depression presented for evaluation of bilateral nipple discharge for 5 months. The patient reported that for the last 5 months she started noticing expressible nipple discharge. She reported no associated headaches, vision changes. She denied the use of vitamins, supplements, herbal remedies. No associated fatigue, changes in the shoe/ring size, heat/cold intolerance, tremors, recent weight changes. She denied neck enlargement, neck pain, dysphagia. The patient reported her menstrual cycles had always been heavy and irregular (every 33-50 days). She has never been pregnant. She has been on Sertraline for the last 4 years with no recent dose changes. Family history was significant for Birt-Hogg-Dubé (BHD) syndrome in father, brother, and sister. The patient has not been tested yet. Initial blood test revealed TSH 1.180 uiu/ml (0.45-4.5 uiu/ml), free T4 1.15 ng/dl (0.82- 1.77 ng/dl), T3 91ng/dl (71-180 ng/dl), FSH 3.8 mIU/ml, LH 3.7 mIU/ml, IGF-1 83 ng/ml (74-239 ng/ml), prolactin 107 ng/ml (4.79-23.3 ng/ml), monomeric prolactin 75.7 ng/ml, macroprolactin 23%, estradiol 16 pg/ml, AM Cortisol 6.5 ug/dl (6.2-19.4ug/dl). The urine pregnancy test was negative. Given low normal AM cortisol, a Cosyntropin stimulation test was done, which was normal. MRI Pituitary with contrast showed flattening of pituitary contour compatible with a partially empty sella turcica and an 8 mm fat intensity in hypothalamic region compatible with a lipoma, stable since last 4 years. The patient was referred to Neurosurgery for further evaluation. The patient remained asymptomatic and medical therapy was not indicated. Genetic testing for BHD was discussed. The patient will be followed in the clinic with a follow-up of prolactin levels and imaging as needed. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PMON81 A Case of Hypophysitis Associated With SARS-CoV2 Vaccination

Abstract Objective Although SARS-CoV2 vaccines have been developed with multiple novel technologies and rapidly disseminated worldwide, the full profile of adverse effects has not been known. Recently, there are sporadic but increasing reports of endocrinopathies in relation to SARS-CoV2 vaccination. Here, we report a rare case of hypophysitis with acute onset of diabetes insipidus after SARS-CoV2 vaccination. Case Report A 48-year-old female who had been in her usual state of health until she received the first SARS-CoV2 vaccine. Two days after the vaccination, she started to have flu-like symptoms including severe headache and myalgia as well as persistent headache, polydipsia and polyuria. On presentation, her vital signs were stable, with her pulse being 81 bpm, BP: 122/84 mm Hg, temperature: 98.6F. Her physical examination was unremarkable. Laboratory evaluation revealed normal values of the basic metabolic panel including sodium level of 142 mmol/L and normal complete blood count. Due to her prolonged and worsening headache, she underwent a brain MRI which revealed a 4 mm round shape of thickening pituitary stalk and partial empty sella. The polydipsia, polyuria, and the thickening of the pituitary stalk led to further pituitary work-up. She underwent the overnight water deprivation test followed by the desmopressin challenge test. Her overnight water deprivation test revealed hypernatremia (Na 147 mmol/L), elevated serum osmolarity (309 mmol/kg), and low urinary osmolarity (83 mmol/kg) which were compatible with diabetes insipidus. Her IGF1 level revealed low normal range (66 ng/mL). Her 250 mcg cosyntropin test showed appropriate response without adrenal insufficiency. DDAVP was started. 3 months after the vaccination, her symptoms have partially improved, and on repeating the MRI brain she has persistent pituitary stalk thickening. Discussion We report a rare case of diabetes insipidus from hypophysitis associated with SARS-CoV2 vaccine. Mechanisms of SARS-CoV2 vaccination-associated endocrinopathy is unknown. From our literature search, we found increasing numbers of the cases of endocrinopathy reported after the SARS- CoV2 vaccination. The thyroid seems the most frequently reported endocrine organ (83%), followed by the pituitary (11%) and adrenal (6%). Average onset is 1-5 days after the vaccinations and reported with all types of SARS-CoV2 vaccines. More mid-age (average age 46) female (78%) cases have been reported. Although associations are not confirmed, endocrinopathies may be underestimated in the post vaccinated population. Further studies are warranted to better understand SARS-CoV2 vaccinations and potential associations of endocrinopathy. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PSAT253 Differentiation of Pituitary Hyperplasia From Adenoma in the Setting of Severe Hypothyroidism

Abstract Introduction Pituitary hyperplasia due to long-standing or profound primary hypothyroidism is a rare finding in children. It occurs because of constant stimulation of thyrotrope cells by a thyrotropin-releasing hormone secreted from the hypothalamus due to a lack of negative feedback from thyroid hormones. The clinical presentation and imaging can mimic a pituitary mass. Patients can present with features of hypothyroidism, hyperprolactinemia, and even visual field defects if the hyperplastic pituitary gland compresses the optic chiasm. This case highlights this uncommon finding and discusses subtle clinical signs of hypothyroidism in children. Case Presentation A 10-year-old boy was incidentally found to have a pituitary lesion (15 mm×14 mm) when an MRI of the brain was performed as a healthy volunteer screening procedure. History and physical exam revealed cold intolerance, lack of height growth for 2 years (crossing down from 75th to 25th percentile), and dry skin with the barely palpable thyroid gland. There was no family history of thyroid or auto-immune disease. Evaluation of pituitary hormones showed remarkably high thyroid-stimulating hormone (&amp;gt;500 mcIU/ml) in the setting of undetectable free thyroxine, positive thyroid peroxidase antibody, low insulin-like growth factor-1, mildly elevated prolactin (67.4 mcg/L), and mild macrocytic anemia. Conclusions Differentiation of pituitary hyperplasia from pituitary hormone-secreting or non-secreting adenomas is crucial, as the primary modality of treatment of pituitary hyperplasia secondary to primary hypothyroidism is thyroid hormone replacement and not surgery unless the patient has visual field defects necessitating of decompression of the optic chasm to avoid permanent vision loss. In general, pituitary hyperplasia is described as homogeneously enhancing with the "nipple sign," which is when the pituitary gland has smooth contours, but a central protrusion of a pituitary mass is present. Elevated TRH can also lead to lactotroph hyperplasia, causing increased prolactin. Growth hormone secretion may be reduced by hypothyroidism and/or by damaged somatotroph cells due to space-occupying pituitary hyperplasia. Macrocytic anemia has been reported in patients with hypothyroidism and is thought to be an adaptation to a decreased basal metabolism. Early recognition and treatment of hypothyroidism during childhood is essential for normal growth and development. In children with the prolonged disease, final height may be compromised, and academic performance and psychosocial well-being may decline. Gradual replacement of thyroid hormone to therapeutic doses by slowly escalating doses of levothyroxine may be beneficial to prevent complications of therapy such as pseudotumor cerebri or empty sella due to rapid shrinkage of lactotroph and thyrotrope cells. Previous studies have shown approximately 85% of those with pituitary hyperplasia due to primary hypothyroidism demonstrate decreased pituitary gland size after starting thyroxine replacement and recommend repeating pituitary MRI after 2-4 months. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.