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.

Clinical Manifestations, Neuroimaging Findings, and Treatment of Idiopathic Intracranial Hypertension in a Nepalese Tertiary Centre

Introduction: Idiopathic Intracranial Hypertension is characterized by headache, visual impairment, papilledema, and increased cerebrospinal fluid opening pressure. We aim to evaluate clinical manifestations, neuroimaging findings, and treatment of Idiopathic Intracranial Hypertension in a Nepalese tertiary center. Materials and Methods: We retrospectively included patients with the diagnosis of Idiopathic Intracranial Hypertension who were admitted to the Neurology department of Tribhuvan University Teaching Hospital from 2019 June to 2021 May and presented to the Neuro-ophthalmology outpatient clinic of the hospital for follow-up. Results: Out of 16 Idiopathic Intracranial Hypertension patients, 12(75%) patients had either headache or ocular pain. Reduced visual acuity and progressive visual loss were found in 44% of patients. Normal CSF opening pressure was found in 19%, 44% had CSF opening pressure at a range of 20-30 cm H20 and > 30 cm H20 in 37.5%. 15 Idiopathic Intracranial Hypertension patients (93.7%) were subtyped as Typical and 1 patient was diagnosed as Fulminant Idiopathic Intracranial Hypertension. Acetazolamide was used for treatment in 15 patients and 1 patient needed ventriculoperitoneal shunting. 56% Seven patients (44%) had some abnormal findings: partial empty sella (44%), and tortuous optic nerve (31%), flattened posterior eyeball (31%), and hypoplastic transverse sinus (19%). Conclusions: Idiopathic Intracranial Hypertension is an uncommon diagnosis but should be suspected in patients with chronic headaches with visual impairment. In low-resource settings, proper history along with neurological and ophthalmological examinations can even detect the early features and timely referral can save the vision and disability of Idiopathic Intracranial Hypertension patients.

Empty Sella and Unilateral Sixth Nerve Palsy in a Pediatric Patient with Idiopathic Intracranial Hypertension

Purpose: We report the case of a child with idiopathic intracranial hypertension who presented with binocular papillary edema and monocular sixth cranial nerve palsy accompanied by empty sella syndrome evident on brain magnetic resonance imaging.Case summary: A 9-year-old, normal-weight male patient visited the emergency room complaining of headache and diplopia 4 days in duration. The alternative prism cover test revealed esotropia of 16 prism diopters and a -1 right lateral gaze limitation. A fundus examination revealed papilledema and peripapillary hemorrhages in both eyes, and a visual field examination an enlarged, physiological blind spot in the right eye. Brain magnetic resonance imaging revealed elevated cerebrospinal fluid pressure, an empty sella, and posterior scleral flattening. We diagnosed and treated idiopathic intracranial hypertension. After 4 months, the papilledema and peripapillary hemorrhages of both eyes resolved, and the right lateral gaze limitation improved. The empty sella improved on brain magnetic resonance imaging, and we noted no recurrence 8 months after treatment.Conclusions: If a child with suspected idiopathic intracranial hypertension visits a hospital, but it is difficult to perform a lumbar puncture, brain magnetic resonance imaging should be scheduled. If abnormalities are found, these help to determine the course of disease.

Spontaneous globe subluxation: a case report and review of the literature

Background Spontaneous globe subluxation (SGS) is an atraumatic anterior dislocation of the eyeball. It is exceedingly rare. Understanding SGS predisposing factors may help uncover its etiology and undertake vision-saving management. Case presentation A 48-year-old female presented to the ED with her right eye out of its socket. She reported blurry vision, photophobia, and pain in the affected eye. She was unable to close her right eyelid and was in obvious distress. On arrival, her blood pressure was elevated. Her medical history was notable for hypertension and obesity. On physical examination, extraocular eye movements were not intact, and the globe appeared whole and round. She was also unable to count fingers with the affected eye. There was no visible trauma to the face. Multiple wet gauzes with sterile saline were placed over the displaced eyeball. Direct and even pressure was applied on the globe. Within 30 s, the globe was reduced back in. The patient was able to close her eyelids and reports substantial pain relief with reduction. A CT scan of the orbits was then obtained, demonstrating mild bilateral proptosis. The globes were normal and symmetric. No intraconal or extraconal abscess or infection was seen. There were no intraconal or extraconal masses. There was no acute orbital traumatic injury, no avulsion of the optic nerve, ocular rupture, or retrobulbar hematoma. After reviewing the case with an ophthalmologist, a follow-up appointment with the ophthalmologist was arranged. The patient was discharged on erythromycin ointment. Post-discharge investigation of the CT imaging revealed dilated optic nerve sheaths, tortuosity of the optic nerve, and empty sella. Conclusions In addition to causing distress and severe anxiety, SGS poses numerous immediate as well as long-term complications. Traction of the optic nerve and retinal vasculature may potentially cause retinal venous congestion and loss of visual acuity with potential vision loss. In the absence of known risk factors or disease processes, orbital imaging and serological studies for thyroid ophthalmopathy should be considered.

Rathke’s Cleft Cyst Abscess—An Unusual Guest in The Sella

Abscess formation within a Rathkes’s cleft cyst (RCC) is extremely rare, particularly at a young age. We report the case of a young girl with abscess formation in RCC. A 21-year-old female presented with headache, vomiting, visual deterioration and features suggestive of hypopituitarism. She had bitemporal hemianopia with impairment of visual acuity. MRI revealed a cystic lesion in the sella with suprasellar extension and peripheral rim enhancement. On the basis of history and imaging, this was indistinguishable from more commonly encountered pituitary pathology. She underwent transsphenoidal decompression, which revealed yellowish purulent material that when cultured grew Staphylococcus epidermidis. Histological examination revealed numerous neutrophils and cyst wall lining with features characteristic of RCC. Postoperatively, she received antibiotics and replacement therapy for hypopituitarism. Three months later, she experienced deterioration in visual fields. Considering persistent disease, she underwent redo surgery which revealed similar findings. Postsurgery, pituitary MRI revealed an empty sella syndrome. Thereafter, follow-up for 1 year was stable with permanent diabetes insipidus and multiple pituitary hormone deficiency on supplementation. Although uncommon, we recommend considering RCC abscess as a differential diagnosis of a pituitary mass lesion, as predicting its presence can be difficult preoperatively. Persistent or recurrent disease is common in these cases, so timely diagnosis and adequate surgical drainage leads to lower morbidity and mortality.

Retrobulbar neuritis associated with empty sella syndrome: A diagnostic challenge

A 35-year-old female presented with sudden diminution of vision to finger counting at half meters in right eye (RE) since 15 days. RE showed grade III RAPD. Rest of the ocular examination was normal. She had no significant past medical history. Neuroimaging showed empty sella. We suspected retrobulbar neuritis (RBN) as a cause of loss of vision since there was no evidence of acute change in morphology of sella turcica like hemorrhage, trauma or ischemia, furthermore there were no pathological findings in RE and visual cortex to explain acute visual loss. Therefore, we prescribed intravenous steroids followed by oral steroids. Vision in RE improved to 6/12 after 15 days. We found that RBN can be a cause of sudden vision loss in cases with empty sella syndrome (ESS) and can pose diagnostic challenge that whether the vision loss is due to ESS or RBN. RBN can be a cause of acute vision loss in patients with ESS and can create diagnostic confusion.

Spontaneous Globe Subluxation: A Case Report And Review Of The Literature

BackgroundSpontaneous globe subluxation (SGS) is an atraumatic anterior dislocation of the eyeball. It is exceedingly rare. Understanding SGS predisposing factors may help uncover its etiology and undertake vision-saving management.Case presentationA 48-year-old female presented to the ED with her right eye out of its socket. She reports blurry vision, photophobia, and pain in the affected eye. She was unable to close her right eyelid and was in obvious distress. On arrival, her blood pressure was elevated. Her medical history was notable for hypertension and obesity. On physical examination, extraocular eye movements were not intact, and the globe appeared whole and round. She was also unable to count fingers with the affected eye. There was no visible trauma to the face. Multiple wet gauzes with sterile saline were placed over the displaced eyeball. Direct and even pressure was applied on the globe. Within thirty seconds, the globe was reduced back in. The patient was able to close her eyelids and reports substantial pain relief with reduction. A CT scan of the orbits was then obtained, demonstrating mild bilateral proptosis. The globes were normal and symmetric. No intraconal or extraconal abscess or infection was seen. There were no intraconal or extraconal masses. There was no acute orbital traumatic injury, no avulsion of the optic nerve, ocular rupture, or retrobulbar hematoma. After reviewing the case with an ophthalmologist, a follow up appointment with the ophthalmologist was arranged. The patient was discharged on erythromycin ointment. Post-discharge investigation of the CT imaging revealed dilated optic nerve sheaths, tortuosity of the optic nerve, and empty sella. ConclusionsIn addition to causing distress and severe anxiety, SGS poses numerous immediate as well as long-term complications. Traction of the optic nerve and retinal vasculature may potentially cause retinal venous congestion and loss of visual acuity with potential vision loss. In the absence of known risk factors or disease processes, orbital imaging and serological studies for thyroid ophthalmopathy should be considered.

The primary empty Sella: An endocrine study on 36 cases

Introduction: The term primary empty sella (PES) makes reference to the herniation of the subarachnoid space within the sella turcica in patients with no history of pituitary tumor, surgery or radiotherapy. Materials and Methods: We studied 36 patients; 27 females and 9 males. The mean age at diagnosis was 50, 4 years [21-80 years]. Most diagnoses were made by magnetic resonance imaging (n = 32). The anterior pituitary function was evaluated by basal hormonal measurements. Then, we had compared two groups of patients: G1(n=17), who had a pituitary disorder, and G2 (n=10), patients without hormonal disease, in order to determine risk factors for endocrine dysregulation in PES. Results: The reasons for ordering pituitary scans were: headaches (65%), visual disturbances (32%) and neurological symptoms in 8% of cases. Sixty-six point five per cent of women were multiparous. Diabetes, obesity and hypertension were found in 19.45 %, 11 % and 19.45 % of the studied population respectively. Hyperprolactinemia was present in 17.24 % of patients. Fifty-five percent of our patients had some degree of hypopituitarism. We didn’t find any correlation in our study, between pituitary insufficiency and age, gender, weight, parity nor post-partum hemorrhage. Failure of lactation was significantly associated with a hormonal disorder. On the other hand, headache was negatively correlated with hormonal effects in PES. Conclusion: PES was most commonly found in middle-aged multiparous women. In most patients, PES is a heterogeneous condition that ranges from hypopituitarism to various degrees of isolated GH deficiency, and which needs careful endocrine assessment, treatment and follow-up.

AYURVEDIC MANAGEMENT OF PARTIAL EMPTY SELLA SYNDROME: A CASE REPORT

Empty Sella Syndrome is a disorder that involves the presence of Cerebro-spinal fluid in Sella turcica in an excess amount. In empty Sella syndrome the Sella turcica is either partially filled with cerebrospinal fluid and an atrophied pituitary gland lying in the floor of the Sella (Partial Empty Sella Syndrome) or filled with cerebrospinal fluid which pushes the pituitary gland into a side of Sella turcica resulting in non-visualization of the gland. (Completely empty Sella). A 37-year-old female diagnosed with partial empty Sella syndrome was managed with Pathyakshadhatryadi kashayam 15 ml twice daily in empty stomach with 45 ml lukewarm water, Rasnadi choornam tailam application, mahatraiphala ghritam 10 ml at night after food, Kshirabala 7 Avarti tailam pratimarsha nasyam. After completion of the treatment, there was considerable relief in the primary complaint of the patient which was a recurrent headache and the follow up MRI showed a marked change in the condition. This case report highlights the role of Ayurveda in the management of partial empty Sella syndrome and sheds light for further study on the same.