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.

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.

Radiographic pituitary stalk disruption: A rare sequela of secondary empty sella syndrome

Background: This two-patient case series describes a rare sequela of postoperative empty sella syndrome (ESS) following transsphenoidal resection of pituitary macroadenomas. This is characterized by progressive hormone dysfunction, diabetes insipidus (DI), and associated MRI evidence of pituitary stalk disruption. Case Description: This phenomenon was retrospectively evaluated in a review of 2000 pituitary tumor resections performed by a single neurosurgeon (KOL). Chart review was retrospectively conducted to gather data on demographics, pituitary hormone status, tumor characteristics, and management. We identified 2 (0.1%) cases of progressive pituitary endocrine dysfunction occurring in the postoperative period associated with MRI evidence of pituitary stalk disruption within 6 weeks of discharge from the hospital. This was felt to be caused by the rapid descent of the residual normal pituitary gland down to the floor of the postoperative empty sella, causing relatively swift stalk stretching. Both patients developed DI, and one patient demonstrated increased pituitary hormone dysfunction. Conclusion: This phenomenon is a rare manifestation of postoperative ESS, secondary to surgical resection of a pituitary macroadenoma. We discuss the associated potential risk factors and strategies for avoidance in these two cases. Routine instillation of intrasellar fat in patients at risk is felt to be protective.

Empty Sella Syndrome as a Window Into the Neuroprotective Effects of Prolactin

Background: The goal of this study was to relate diffusion MR measures of white matter integrity of the retinofugal visual pathway with prolactin levels in a patient with downward herniation of the optic chiasm secondary to medical treatment of a prolactinoma.Methods: A 36-year-old woman with a prolactinoma presented with progressive bilateral visual field defects 9 years after initial diagnosis and medical treatment. She was diagnosed with empty-sella syndrome and instructed to stop cabergoline. Hormone testing was conducted in tandem with routine clinical evaluations over 1 year and the patient was followed with diffusion magnetic resonance imaging (dMRI), optical coherence tomography (OCT), and automated perimetry at three time points. Five healthy controls underwent a complementary battery of clinical and neuroimaging tests at a single time point.Results: Shortly after discontinuing cabergoline, diffusion metrics in the optic tracts were within the range of values observed in healthy controls. However, following a brief period where the patient resumed cabergoline (of her own volition), there was a decrease in serum prolactin with a corresponding decrease in visual ability and increase in radial diffusivity (p < 0.001). Those measures again returned to their baseline ranges after discontinuing cabergoline a second time.Conclusions: These results demonstrate the sensitivity of dMRI to detect rapid and functionally significant microstructural changes in white matter tracts secondary to alterations in serum prolactin levels. The inverse relations between prolactin and measures of white matter integrity and visual function are consistent with the hypothesis that prolactin can play a neuroprotective role in the injured nervous system.

RARE-13. PRIMARY EMPTY SELLA SYNDROME AND PSEUDO-TUMOR CEREBERI: CORRELATION OF ASSOCIATION AND SURGICAL PLAN

Introduction Management of primary empty sella syndrome (ESS) is generally remaining a neurosurgical challenge due to lack of a well standardized approach. Pseudo-tumor cereberi (Benign or idiopathic intracranial hypertension) is commonly associated condition. In this study, we have demonstrated the relationship and surgical plan and outcome of such cases. Patients and Methods: We retrospectively studied 24 patients with primary empty sella syndrome (ESS) for two years who were diagnosed radiologically as ESS. Fundus and other ophthalmological examinations were done. Lumbar puncture and cerebrospinal fluid (CSF) manometer were evaluated in those with papilledema. All patients’ data were collected and analyzed. Results Basically, 24 patients (18 females and 6 males) were radiologically diagnosed as EES. 13 females and only one male were having symptoms of BIH. 17 patients (70.83%) had headache as the first presentation. Second most common presentation in our study was visual in 14 patients (58.3%). Two patients (8.3%) had pituitary hypersecretion namely; growth and prolactin hormones. In those (58.3%) confirmed to have BIH Theco-peritoneal shunts were inserted. Incidental cases (29.17%) without symptoms were followed up. Conclusion Although (ESS) is a well-known radiological hallmark for BIH, in our study not all patients had BIH. Interestingly, pituitary hyperfunction may be the first presentation in some rare cases. Generally, natural history of that entity was benign. Frequent follow-up by neurosurgeons and increased awareness of associations are advised. We believe a more prospective large number cohort is important to outline the natural history.