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Background: Ependymal cysts (EC) typically present supra-tentorially near the lateral ventricle, juxta ventricular, or temporoparietal regions. Previous cases have also identified infratentorial EC of the brainstem, cerebellum, and subarachnoid spaces. They are mostly asymptomatic. In this paper, we present the first-ever case of a symptomatic medullary ependymal cyst treated with surgery, along with a comprehensive review of the literature on EC of other parts of the brain stem., Case Description: This 51-year-old female presented with hearing loss, dizziness, diplopia, and ataxia. Radiographic imaging indicated the presence of a non-enhancing lesion in the medulla with a mass effect on the brainstem. Pathological examination confirmed its characterization as an ependymal cyst. The patient underwent a suboccipital craniotomy for the fenestration of the medullary ependymal cyst under neuro-navigation, Intra-op ultrasound and intra-operative neuro-monitoring. Histopathological examination confirmed the diagnosis of an ependymal cyst. At one month follow-up, her KPS is 90, ECOG PS 1, and her ataxia has improved with complete resolution of diplopia., Conclusion: Due to their rarity and potential similarity to other cystic structures, EC may be overlooked or incorrectly diagnosed resulting in mismanagement and surgical disaster. Therefore, a comprehensive understanding and awareness of their distinct characteristics are essential for accurate diagnosis and appropriate management., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Rathke's cleft cyst (RCC) apoplexy is an uncommon lesion attributed to abnormal vascular supply to the fragile RCC epithelial wall. It is rare in children and very difficult to diagnose without pathologic confirmation. Here, we report an 8-year-old boy who presented with headache and visual deficit. MRI and CT showed a cystic mass in the sellar region. He underwent endoscopic endonasal surgery, and the cystic mass was resected completely via a trans-sphenoidal approach. The lesion was confirmed as RCC apoplexy by intraoperative observation and histopathological examination. Headache was completely relieved and the visual field deficit improved remarkably after the operation. The authors recommend surgical management for pediatric RCC apoplexy patients who present with severe neuro-ophthalmic signs or deterioration of consciousness, although there are at present no standardized management guidelines for pediatric RCC apoplexy., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Introduction: Rathke's cleft cysts are thought to have a benign clinical outcome apart from associated hypopituitarism and visual defects. Synchronous central nervous system lesions, including pituitary adenoma and intracerebral aneurysms, are rarely reported. Diagnosis of Rathke's cleft cyst after presenting with a subarachnoid hemorrhage due to a ruptured arterial aneurysm is reported only once before., Case Presentation: A 33-year-old Sri Lankan female presented with a subarachnoid hemorrhage due to a ruptured anterior communication artery aneurysm. She underwent pterional craniotomy and aneurysm clipping. She was found to have partial cranial diabetes insipidus and hypogonadotropic hypogonadism. She had a cystic lesion occupying enlarged sella turcica with characteristics of a Rathke's cleft cyst. Subsequently, she underwent trans-sphenoidal excision of the sellar lesion. Histology confirmed the diagnosis of Rathke's cleft cyst., Conclusions: Rare co-occurrence of a Rathke's cleft cyst and an anterior communicating artery aneurysm would have been missed if subtle manifestations atypical for subarachnoid hemorrhage were not further pursued. This could have led to progressive visual deterioration and hypopituitarism., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Introduction: Rathke's cleft cyst (RCC) and primary empty sella syndrome (PESS) are usually incidental findings on magnetic resonance imaging (MRI) scans. In most cases, these lesions do not cause mass effect symptoms and do not require surgical intervention. In patients with RCC or PESS, it is important to exclude secondary adrenal insufficiency (SAI), which may be a life-threatening condition., Material and Methods: The incidence of SAI was assessed in patients with RCC or PESS detected by MRI, using the 1 μg Synacthen stimulation test. A total of 38 patients were analysed. Test results were linked to clinical symptoms and the type of cystic lesion., Results: Assuming that cortisol levels < 14.6 μg/dL in Synacthen test are the criterion of SAI diagnosis, SAI was diagnosed only in 2 patients (5%). Adopting the traditional criterion of cortisol levels < 18 μg/dL, SAI would be diagnosed in 7 patients (18.4 %). Dizziness (Chi2 = 3.89; p = 0.049) and apathy (Chi2 = 3.87; p = 0.049) were significantly more frequent in the PESS group than in the RCC group., Conclusions: The incidence of SAI in the general patient population with empty sella syndrome and Rathke's cleft cysts is low. The 1 μg Synacthen test seems to be a valuable tool in the diagnosis of SAI among patients with RCC and PESS. Further studies are necessary to determine the sensitivity and specificity of the 1 μg Synacthen test with the standardization of test protocol and considering the cortisol level at the 20-minute timepoint. PESS patients report dizziness and apathy more frequently than RCC patients, which does not result from the disturbance of the hypothalamic-pituitary-adrenal axis, but probably from the different pathogenesis of these cystic lesions.

Background: Rathke Cleft Cysts (RCC) are cystic sellar or suprasellar nonneoplastic lesions that are thought to originate from remnants of the rathke pouch. Postoperatively, RCCs have a tendency to reaccumulate, after which preoperative symptoms may recur. However, there exists very little evidence on which treatments are most effective in these patients., Case-Description: In this report, we present a unique case of a symptomatic RCC that recurred four times after initial transsphenoidal surgery. Following each surgery, the patient had significant visual improvement with post-op imaging displaying decompression of the neural elements. However, RCC reaccumulated in strikingly rapid time intervals of 1.5 months, 0.5 months, 1.5 years, and 5 months after each respective prior surgery. Repeat interventions with transsphenoidal, pterional and supraorbital approaches were unsuccessful in providing a durable treatment response. The patient ultimately underwent radiotherapy after a final surgical marsupialization of the cyst and has since displayed stable imaging with improved vision. This patient represented a 'perfect storm' of factors that may contribute to cyst recurrence, including substantial visual field deficits, large cyst size, peripheral wall enhancements on MRI, an intraoperative CSF leak, use of a fat graft, subtotal resection of the portion of cyst wall that adhered to important suprasellar structures, squamous metaplasia noted in cyst wall, and suprasellar extension., Conclusions: We demonstrate that different surgical approaches through repeat surgeries may not assist in prevention of further recurrence; instead, we propose that radiotherapy should be offered early in the treatment course of recurrent cases that have additional risk factors for further reoccurrence., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Cystic lesions arising in the sellar region are not uncommon and encompass cystic pituitary adenomas, Rathke cleft cysts, craniopharyngiomas, and arachnoid cysts. Their clinical presentation may be similar, including headache, visual field defects, and anterior pituitary hormone deficits, which makes differential diagnosis challenging. On the other hand, imaging features may indicate certain pathologies. In this approach to the patient, we describe the case of a patient who presented with right temporal hemianopsia and a sellar/suprasellar cystic lesion, which was determined to be Rathke cleft cyst. We discuss the imaging characteristics that may suggest a particular diagnosis between Rathke cleft cyst, cystic pituitary adenoma, craniopharyngioma, and arachnoid cyst and propose a flowchart for aiding in the imaging differential diagnosis., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

A 49-year-old man developed severe hyponatremia associated with transient headache and was diagnosed with syndrome of inappropriate antidiuretic hormone secretion (SIADH). Fluid restriction and sodium supplementation corrected the hyponatremia. However, several days later, the patient exhibited hypernatremia with thirst and polyuria. A detailed examination indicated central diabetes insipidus (CDI) with an intrasellar cystic lesion indicative of Rathke's cleft cyst (RCC). A case of RCC exhibiting headache, hyponatremia, and subsequent hypernatremia has been reported. Our case shows that CDI may appear after SIADH in patients with RCC, especially in those with serum sodium levels that unexpectedly increase rapidly beyond the reference range.

Aim: Coats plus syndrome (CP) is a rare autosomal recessive disorder, characterised by retinal telangiectasia exudates (Coats disease), leukodystrophy, distinctive intracranial calcification and cysts, as well as extra-neurological features including abnormal vasculature of the gastrointestinal tract, portal hypertension and osteopenia with a tendency to fractures. CP most frequently occurs due to loss-of-function mutations in CTC1. The encoded protein CTC1 constitutes part of the CST (CTC1-STN1-TEN1) complex, and three patients have been described with CP due to biallelic mutations in STN1. Together with the identification of homozygosity for a specific loss-of-function mutation in POT1 in a sibling pair, these observations highlight a defect in the maintenance of telomere integrity as the cause of CP, although the precise mechanism leading to the micro-vasculopathy seen at a pathological level remains unclear. Here, we present the investigation of a fourth child who presented to us with retinal exudates, intracranial calcifications and developmental delay, in keeping with a diagnosis of CP, and later went on to develop pancytopenia and gastrointestinal bleeding. Genome sequencing revealed compound heterozygous variants in STN1 as the likely genetic cause of CP in this present case., Methods: We assessed the phenotype to be CP and undertook targeted sequencing., Results: Whilst sequencing of CTC1 and POT1 was normal, we identified novel compound heterozygous variants in STN1 (previous gene symbol OBFC1): one loss-of-function--c.894dup (p.(Asp299Argfs*58)); and one missense--c.707T>C (p.(Leu236Pro))., Conclusion: Given the clinical phenotype and identified variants we suggest that this is only the fourth patient reported to date with CP due to mutations in STN1., (© 2021 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals LLC.)

Purpose: Rathke cleft cysts (RCC) are a target for transsphenoidal surgery when symptomatic; however, the surgical techniques and outcomes continue to be of interest. Our goal is to highlight nuances in diagnosis, surgical philosophy and techniques, and outcomes., Methods: We retrospectively reviewed a single surgeon experience in a contemporary series of RCC from April 2008 through December 2019. The clinical presentation, radiographic studies, intraoperative findings, histopathological diagnosis, postoperative complications, and outcome data were recorded and analyzed., Results: A total of 131 endoscopic endonasal operations were performed on 116 patients (30 male, 86 female), with an average age of 39 years. We identified 102 patients without prior surgery, and 14 patients with prior surgery elsewhere. Postoperative complications included transient diabetes insipidus (DI) (n = 23, 20%), permanent DI (n = 5, 4%), SIADH (n = 6, 5%), epistaxis (n = 6, 5%), postoperative infection (n = 3, 2.5%), postoperative CSF leak (n = 1, < 1%), intrasellar hematoma (n = 1, < 1%), and stroke (n = 1, < 1%). Visual improvement was observed in 74% (n = 31/42) of patients presenting with visual loss, and headache improvement in 86% (n = 80/93). Overall, the recurrence rate in our cohort was 11%, with a lower recurrence rate seen in males (p = 0.02). The median follow-up duration was 15.2 months (interquartile range 1.7-52.3) CONCLUSION: Our results reflect the philosophy of a conservative surgical approach, designed to preserve normal pituitary function, and avoid DI, while achieving the maximal feasible resection. Evacuation of the cyst contents and meticulous sampling of the cyst wall when possible for histopathological diagnosis are crucial. The postoperative relief of symptoms and signs is gratifying, as are the overall rates of recurrence and outcomes., (© 2021. This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply.)

In this article, we present a 31-year-old female who presented with intermittent headache and oligomenorrhea of over 10 years' duration. Imaging revealed a large suprasellar mass with sellar extension. The patient underwent an endoscopic endonasal trans-sphenoidal surgery to resection of the mass. Clinical, radiological, and operative findings from this patient were initially considered to be Rathke's cleft cyst (RCC). However, postoperative histological examinations revealed a mature cystic teratoma. No radiotherapy was performed after surgery. At the most recent follow-up, approximately 1 year later, the patient is doing well with no headache and no recurrence of the teratoma., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Jiang, Wang, You, Wang and Bao.)

Purpose: Pituitary abscesses (PAs) are a rare clinical entity which may arise from normal pituitary tissue or underlying lesions within the gland. Rathke's cleft cysts (RCCs) are not commonly associated with the development of PA., Methods: Retrospective chart review of three patients with PAs within RCCs at a single university center and review of the literature., Results: Three cases are reported. The first case presented with fever and headache and a history of prior surgery due to RCC and a recent respiratory tract infection. The second case had a history of recent skin infections and presented with sudden onset headache and hypopituitarism. In the third case, chronic visual field impairment prompted an ophthalmologic evaluation resulting in a diagnosis of an adenoma and an infected RCC. In all three cases, an endoscopic endonasal approach was performed to drain infected tissue and allowed microbiological identification of gram-positive cocci, followed by treatment with antibiotics for at least three weeks. Cases in the literature are scarce and the diagnosis is usually made intraoperatively due to non-specific manifestations and imaging. PAs arising from underlying pituitary lesions are less common than primary PAs. Differential diagnosis should include pituitary apoplexy, hypophysitis and other cystic lesions., Conclusion: PAs occurring in RCCs are infrequent. Clinical manifestations are commonly subacute, without septic symptoms. Imaging is usually non-specific. Preoperative diagnosis is infrequent and a broad differential diagnosis should be considered. Empirical antimicrobial therapy should be initiated and adjusted after obtaining cultures to reduce the rate of recurrence and improve clinical outcomes.

Objective: To present a case of Rathke's cleft cyst (RCC) in a middle-aged man complaining of depression and review the relevant literature., Methods: A 51-year-old man firstly visited the outpatient clinic of neurology in our hospital due to 4 years of low mood. The patient was found normal in physical and thyroid function laboratory examinations and achieved a total score of 19 in Hamilton Depression Scale, which indicated depression. After failure to respond to antidepressant medication, the patient again came to the clinic of andrology and was found with sexual dysfunction and aspermia. With the serum FSH, LH, PRL, T and fasting blood glucose levels of 0.88 IU/L, 0.25 IU/L, 30.8 nmol/L, <0.24 nmol/L and 3.9 nmol/L respectively, it was considered to be a case of hypogonadotropic hypogonadism. Pituitary MRI indicated RCC but the adreno-cortico-tropic hormone and growth hormone were normal. All the data obtained led to the final diagnosis of RCC, acquired hypogonadotropic hypogonadism, depression and acquired aspermia and therefore the patient was referred to the department of neurosurgery and underwent navigation- and endoscopy-guided microsurgical resection of the Rathke cleft cyst via the nasopharynx and sella region in 2018., Results: RCC was pathologically confirmed postoperatively. At 3 months after surgery, the patient was found with a serum T level of 0.72 nmol/L and received the testosterone replacement therapy. After 3 months of treatment, the serum T was increased to 10.3 nmol/L, the patient's symptoms of depression, hyposexuality and sexual dysfunction were significantly improved, and a little semen was observed in the ejaculate., Conclusions: RCC is one of causes of hypogonadism in middle-aged men, causing the clinical symptoms mainly by pressing the pituitary gland and inducing gonadotropin deficiency. It is hard for RCC patients with depression and sexual dysfunction to restore gonadotropin to normal even after surgical removal of the cyst and therefore testosterone replacement therapy is necessitated postoperatively.

Sellar region lesions include a broad range of benign and malignant neoplastic as well as non-neoplastic entities, many of which are newly described or have recently revised nomenclature. In contrast to other intracranial sites, imaging features are relatively less specific, and the need for histopathological diagnosis is of paramount importance. This review will describe pituitary adenomas, inflammatory lesions, and tumors unique to the region (craniopharyngioma) as well as tumors which may occur in but are not exclusively localized to the sellar location (schwannoma, metastasis, etc.)., Competing Interests: Disclosure The authors have no relevant financial or commercial disclosures. No specific funding sources were used in the writing of this article., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The most common sellar pathology that merits neurosurgical attention is the pituitary adenoma. However, some developmental, inflammatory, and neoplastic lesions may primarily or secondarily involve the sella, mimicking pituitary tumors. Advances in imaging and endocrinological assessment have helped in the recognition of these less common sellar, supra sellar pathologies, which may occasionally create confusion in management. The most common developmental anomaly is the Rathke's cleft cyst and an increasingly recognized inflammatory pathology is the spectrum of hypophysitis. Neoplasms, viz. Craniopharyngioma, Germinoma, Langerhans's cell histiocytosis or metastasis, have more distinctive features in various age groups and are more likely to be correctly diagnosed on current imaging and managed accordingly. This review looks at mainly intraparenchymal pathologies, namely Rathke's Cleft Cyst and various hypophysitides, and will discuss their management strategies., Competing Interests: None

Introduction: Cystic sellar salivary gland-like lesions (CSSLs) are exceedingly rare, with fewer than a dozen case reports. They contain amorphous colloid identical to Rathke cleft cyst contents, but the cyst wall additionally shows cohesive aggregates of benign salivary glands. We report three new examples., Materials and Methods: Two cases were seen at University of Colorado Denver and one at Memorial Sloan Kettering (MSK). Molecular testing was attempted on two of three., Results: Case 1 is a 20-year-old female who presented with panhypopituitarism and was found to have a suprasellar mass that proved to be a CSSL. She received no postoperative adjuvant therapy, but recurrence of headaches and blurred vision 2 years later prompted return to medical attention. A much smaller local cyst recurrence was now accompanied by a thickened, bulbous infundibular stalk. Second resection yielded a gliotic infundibular stalk and amorphous mucin, but no residual salivary-like glands. She is without further recurrence on 6-year follow-up. Case 2 is a 29-year-old female with headache; while seen initially at a tertiary care center, diagnosis was only made after consultation at MSK. Case 3 is 68-year-old female who had originally presented with apoplexy to an outside hospital 7 years prior to surgery and diagnosis. Molecular testing was uninformative on case 1 and negative for mutations or fusions on case 3., Conclusion: Few pathologists or neuropathologists have encountered CSSLs in their practices; case 1 produced recurrence and significant infundibular stalk damage, and case 3 originally manifested apoplexy, features not previously reported.

Introduction: Leukoencephalopathy with calcifications and cysts (LCC) is a rare autosomal recessive cerebral angiomatous-like microangiopathy characterized by diffuse and asymmetric white-matter lesions associated with multiple calcifications and cysts. The disease is caused by SNORD118 mutations. The entire clinical spectrum of LCC is not yet fully determined., Material and Methods: To define the clinical spectrum of LCC, we analyzed data from recently diagnosed cases and from the litterature. Both clinical and imaging features from our five LCC cases harboring compound heterozygous SNORD118 mutations were presented and all cases reported in the litterature reviewed., Results: Ninety-two LCC cases including our five patients were identified. Consanguinity was rare (4%), and 97% of cases were symptomatic. Mean age of first clinical manifestations was 16.1±16.1 years (range 1 month-71 years) and was earlier in men (10.3±14.3 years) than in women (20.2±22.8 years) (P=0.02). The main inaugural symptoms were seizures (36%; mean age at onset: 5.2±9.5 years) and progressive neurological symptoms including ataxia, dystonia and spasticity (26%; 27.8±23.6 years). Intracranial hypertension was less frequently observed (14%), mostly in adults (mean age 31.5±13.2 years). Ischemic or hemorrhagic strokes were inaugural symptoms in two adults (2%). During follow-up, most patients developed progressive extrapyramidal, cerebellar and pyramidal signs (83%), cognitive decline (56%), seizures (37%), intracranial hypertension (30%) or stroke (2%)., Conclusion: In LCC, the clinical spectrum is largely heterogeneous and the course of the disease appears highly variable in contrast to other hereditary cerebral small vessel diseases., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Purpose: To report a Rathke's cleft cyst (RCC) presenting as unilateral incomplete cavernous sinus syndrome and disc edema METHOD: Case report with literature review RESULTS: We present an 18-year-old girl who has been experiencing intermittent binocular diplopia for 1 month. Upon examination, the best corrected visual acuity was 20/20 (OU). Pronounced limitation of ocular motility with ptosis in her right eye was noted. Her pupil constricted normally. However, a relative afferent pupillary defect in the right eye was observed. Fundus examination showed disc edema in the right eye without peripapillary hemorrhage. Moreover, her thyroid function and acetylcholine receptor antibody levels were normal. Brain magnetic resonance imaging revealed a 1.6-cm mass at the suprasellar fossa and optic nerve sheath of right eye enhancement. She underwent transsphenoidal surgery, and pus-like formation was noted. Pathological findings were consistent with RCCs. After the cyst was removed, her ocular motility remarkably improved and ptosis subsided., Conclusion: A case of RCC presenting as multiple cranial nerve palsy was reported. RCCs are benign and usually asymptomatic. Symptomatic RCCs often manifest as headache or visual problems, such as reduced visual acuity and visual field defect. Multiple cranial nerve involvement is uncommon and indicates inflammation or compression of the cavernous sinus and optic nerve. Neuropathy may resolve after surgery; therefore, surgery is indicated for symptomatic RCCs. Further, RCC should be included in the differential diagnosis of patients with disc edema.

Background: Cystic sellar masses (CSMs) pose diagnostic and therapeutic challenges associated with subtotal cyst wall resection, cerebrospinal fluid (CSF) leak repair, and disease recurrence. Current magnetic resonance imaging (MRI) interpretation often cannot reliably differentiate CSMs, mandating adaptable intraoperative strategies. We reviewed our diagnostic and therapeutic experience after endoscopic endonasal approaches (EEAs) for CSMs., Methods: A retrospective record review of patients with CSM managed via EEA at the University of Southern California from 2011 to 2018 was conducted. Patient demographics, preoperative characteristics, surgical details, pathologic findings, and postoperative outcomes were assessed., Results: Analysis included 47 patients (mean age, 43.2 years); of these, 78.7% were women. Preoperative symptoms included headache (76.6%) and vision loss (42.6%). Histologically verified sellar pathology included 27 Rathke cleft cysts (RCCs) (57.4%), 17 cystic pituitary adenomas (CPAs) (36.2%), 2 arachnoid cysts (4.3%), and 1 xanthogranuloma (2.1%). Twelve patients (70.6%) with CPAs underwent complete resection and 5 (29.4%) underwent subtotal resection. All 27 patients with RCC and 2 patients with arachnoid cyst underwent complete fenestration and drainage. One xanthogranuloma was completely resected. There were 14 intraoperative (29.8%) and 4 postoperative CSF leaks (8.5%). Headaches, vision, and endocrinopathy improved in 69.2%, 80.0%, and 33.3% of patients with CPA and 73.9%, 71.4%, and 40.9% of patients with RCC, respectively. There were 2 RCC recurrences and 1 CPA recurrence over the follow-up period., Conclusions: Surgeons must prepare for versatile management strategies of CSMs based on pretest probability associated with MRI and intraoperative findings. Outcomes after EEA for CSMs show low complication profiles and excellent rates of headache and visual improvement, albeit lower rates of endocrine normalization., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: To differentiate pituitary adenoma from Rathke cleft cyst in magnetic resonance (MR) scan by combing MR image features with texture features., Methods: A total number of 133 patients were included in this study, 83 with pituitary adenoma and 50 with Rathke cleft cyst. Qualitative MR image features and quantitative texture features were evaluated by using the chi-square tests or Mann-Whitney U test. Binary logistic regression analysis was conducted to investigate their ability as independent predictors. ROC analysis was conducted subsequently on the independent predictors to assess their practical value in discrimination and was used to investigate the association between two types of features., Results: Signal intensity on the contrast-enhanced image was found to be the only significantly different MR image feature between the two lesions. Two texture features from the contrast-enhanced images (Histo-Skewness and GLCM-Correlation) were found to be the independent predictors in discrimination, of which AUC values were 0.80 and 0.75, respectively. Besides, the above two texture features (Histo-Skewness and GLCM-Contrast) were suggested to be associated with signal intensity on the contrast-enhanced image., Conclusion: Signal intensity on the contrast-enhanced image was the most significant MR image feature in differentiation between pituitary adenoma and Rathke cleft cyst, and texture features also showed promising and practical ability in discrimination. Moreover, two types of features could be coordinated with each other., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2019 Yang Zhang et al.)

Background: Acromegaly caused by Rathke cleft cyst (RCC) mimicking a plurihormonal pituitary adenoma (PA) is rare., Case Description: We report a 71-year-old woman who presented with hyperhidrosis in 2013. Magnetic resonance imaging performed in April 2018 revealed that the patient had a pituitary tumor, and she was referred to our hospital. She presented with an acromegaly-like appearance with mild hypertrophy at her limb extremities. Preoperative blood tests, magnetic resonance imaging, and an endocrine tolerance test indicated that the patient's symptoms satisfied the diagnostic criteria for acromegaly, with a suspected diagnosis of an RCC and growth hormone (GH)-producing PA. Endoscopic transsphenoidal surgery (eTSS) was performed. Permanent pathologic diagnosis showed an RCC mimicking a plurihormonal PA, which was confirmed via immunohistochemistry. Blood sampling 2 months post surgery showed reduced GH (0.41 ng/mL) and increased insulin-like growth factor-1 (IGF-1) (356 ng/mL) levels. In addition, a postoperative endocrine tolerance test revealed a parasitic reaction of GH and secondary adrenocortical hypofunction. No RCC recurrence was found, and the GH (0.32 ng/mL) and previously increased IGF-1 (169 ng/mL) levels were normalized 12 months after eTSS., Conclusions: We reported a rare case of acromegaly caused by RCC mimicking a plurihormonal PA. This case suggests that inflammation associated with RCC might be involved in the development of adenomatous cells. Postoperative clinical symptoms and elevated fibrinogen and IGF-1 levels later improved. This outcome suggested that the transient increase in IGF-1 2 months after surgery might reflect RCC-induced inflammation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Rationale: Endodermal cysts are rare benign lesions in the central nervous system. Their common symptoms include headache and neck pain caused by mass effect or inflammatory reaction. We report the case of an elderly woman with intracranial endodermal cyst who presented with nonobstructive hydrocephalus., Patient Concerns: A 78-year-old woman presented with acute deterioration of consciousness caused by acute hydrocephalus. She subsequently underwent ventriculoperitoneal shunt placement. Eighteen months after this operation, she developed numbness of extremities and gait and progressive cognitive disturbances., Diagnosis: Initially, the endodermal cyst could not be recognized, but it became clinically evident in the craniocervical junction after ventriculoperitoneal shunt placement. MRI revealed multiple cystic lesions in the pontomedullary cistern. Postoperative pathology confirmed the diagnosis of endodermal cyst., Interventions: Subtotal resection of the cystic lesion was performed. Intra-operatively, multiple cysts containing a milky white fluid were noted and the medullary surface including the median and lateral apertures of the fourth ventricle were covered by thick, cloudy arachnoid membrane., Outcomes: Although the numbness of extremities improved after the surgery, she remained bedridden due to deterioration in cognitive function and generalized muscle weakness. The patient developed recurrence 2 months after the surgery; however, no additional surgery was performed owing to her poor general condition., Lessons: Endodermal cysts rarely present with nonobstructive hydrocephalus caused by recurrent meningitis. In such cases, the lesions are often invisible on initial diagnostic imaging, and complete resection of the lesions is typically difficult because of strong adhesion between the cyst walls and contiguous vital structures.

Purpose of Review: To review the recent literature regarding the growing role of rhinologists and otolaryngologists with neurosurgeons in the joint multidisciplinary team approach for managing patients with Rathke's cleft cysts (RCC)., Recent Findings: The transnasal endoscopic approach to the skull base has become relatively mainstream for surgical treatment of RCCs. Suprasellar lesions, especially those that are purely suprasellar, are associated with higher recurrence rates, though an extended approach may improve dissection and access and therefore aid in lesion removal. Endoscopic cyst drainage is a well tolerated and effective way to treat RCC, and often avoids the postoperative endocrinopathies associated with complete cyst wall removal. Novel techniques have been described for maintaining tract patency, including the use of stents and flaps, in order to prevent cyst stenosis and reaccumulation. A frontier in skull base surgery is in applications for pediatric patients, and managing RCCs in this population surgically appears to be associated with positive outcomes overall., Summary: Team-based endoscopic skull base surgery has spurred advances in our understanding of skull base disease, including RCCs. Optimal outcomes are most apparent when the experience and technique of both the endoscopist and neurosurgeon have developed jointly over time.

Neuroglial cysts (also known as glioependymal cysts) are rare, benign, epithelial-lined cystic lesions that can potentially occur anywhere in the neuraxis. They can be intra, or within, a cranial nerve with the former being more common. The frontal lobe is thought to be the most common location. On magnetic resonance imaging, they follow cerebrospinal fluid signal and have smooth nonenhancing borders. This video demonstrates in a step-by-step fashion the technical nuances of the supraorbital keyhole approach through an eyebrow incision, for fenestration and biopsy of a neuroglial cyst within the left optic tract. The patient was a 27-year-old female who had peripheral vision disturbance postpartum. The cyst on magnetic resonance imaging was initially observed, but follow-up Humphrey visual field testing demonstrated worsening of the partial left superior quadrantanopsia. The keyhole supraorbital approach was selected to provide a direct route to the pathology with minimal soft tissue disruption. The patient was discharged on postoperative day 2 without any complications. Follow-up at 6 weeks demonstrated near-complete resolution of her quadrantanopsia and good cosmesis with no frontalis palsy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Rathke cleft cysts (RCC) are benign, epithelium-lined intrasellar and/or suprasellar cysts believed to originate from the remnants of the Rathke pouch. The aim of this study was to analyse the symptoms and surgical outcome of patients with the diagnosis of RCC verified in a histopathological examination of the postoperative material., Methods: The study is a retrospective analysis of 38 cases of children who underwent a neurosurgical treatment due to RCC at the Children's Memorial Health Institute in Warsaw, Poland, between 1994 and 2015., Results: At diagnosis, the mean age was 13 years and 8 months (6 years and 11 months-17 years and 10 months, sex ratio was 1:0.9 with a female prevalence). The most common symptoms were the following: headache (50%), hypothyroidism (50%), short stature and/or decreased growth velocity (47%), delayed puberty and menstrual abnormalities (37%), diabetes insipidus or polydipsia and polyuria (26%), adrenal dysfunction (26%), sleepiness and general weakness (13%) and visual disturbances (11%). Due to the gravity of symptoms and size of the lesion, all the patients underwent a surgical treatment. All but one were successful (one patient died due to postoperative neurosurgical complications). The most common postoperative complications were the following: adenohypopituitarism (67%) and diabetes insipidus (45%)., Conclusions: RCC can present with serious symptoms that significantly deteriorate patients' quality of life. Despite a successful neurosurgical treatment in most of the analysed cases, patients required long-term pharmacological treatment.

Objective: To investigate the clinical features and diagnostic bases of childhood leukoencephalopathy with cerebral calcifications and cysts (LCC). Methods: The clinical data involving manifestations and laboratory examinations of 4 children with LCC admitted to Beijing Children's Hospital Affiliated to Capital Medical University from 2012 to 2017 were retrospectively summarized. Each patient had a follow-up visit ranging from 4 months to 5 years and 9 months after initial examination. Results: Patients consisted of 2 males and 2 females, whose age of onset was respectively 2 years and 9 months, 6 years and 2 months, 7 years and 10 months, and 5 years and 1 month. The main clinical symptoms of these cases included headache, dizziness, partial seizure and claudication, and two of these cases had insidious onset. Cerebral calcifications and cysts with leukoencephalopathy were detected by neuroimaging in all patients. In addition, multifocal microhemorrhages and calcifications were observed by magnetic susceptibility-weighted imaging (SWI) series in 3 patients. Brain biopsy performed on 1 case disclosed a neuronal reduction in the cerebral cortex, loosening of focal white matter, multifocal lymphocyte infiltration, fresh hemorrhages, and gliosis, as well as angiomatous changes of blood vessels with hyalinized thicken-wall, stenotic or occlusive lumina and calcification deposits. The compound heterozygous mutations of n.*10G>A and n.82A>G in SNORD118 were identified in 1 case by target-capture next-generation sequencing. Sanger sequencing verified that the variant n.*10G>A was a novel mutation and it was of paternal-origin, while the variant n.82A>G was of maternal-origin, which had already been reported to be pathogenic to LCC. Follow-up study had shown continued partial seizure in 1 case and remissive claudication in another, while the remaining 2 cases had a relatively favorable outcome without obvious neurological symptoms at present time. Conclusions: The clinical manifestations of LCC are nonspecific, and the onset of the disease tends to be insidious. The triad neuroimaging findings of cerebral calcifications, cysts and leukoencephalopathy are essential to the diagnosis of the disease, and the signals of microhemorrhages revealed by SWI series provide another eloquent reference for the diagnosis. As biopsy is invasive and usually unavailable in the early stage, gene assessment, instead of pathological data, should be the gold standard in the diagnosis of LCC.

Context: The persisting embryonal infundibular recess (PEIR) is a rare anomaly of the floor of the third ventricle with a debated pathogenesis. It can be a cause of misdiagnosis in the case of cystic lesions of the sellar and suprasellar area., Objective: To describe two recently evaluated cases and provide a systematic literature review., Evidence Acquisition and Case Descriptions: PEIR has been previously reported in six adult patients. Because in some cases it was associated with hydrocephalus and/or empty sella, a possible role of altered intracranial pressure in PEIR formation has been postulated. We evaluated two female patients, aged 34 and 50 years, referred to the Pituitary Surgery Clinic of the University of Brescia with the diagnosis of a sellar cyst and craniopharyngioma, respectively. Endocrine screening and visual field testing were normal. No signs of hydrocephalus or empty sella, as well as other indirect signs of intracranial hypertension, were visible on MRI scans. After a multidisciplinary reevaluation, diagnosis of PEIR was made in both cases. Both patients are followed but have not developed any disturbance related to the PEIR in the following 18 months., Conclusions: PEIR is a rare condition, probably unrecognized and the result of dysembriogenesis, which should be included in the differential diagnosis of cystic sellar lesions. Imaging features (funnel pituitary stalk and cyst in the sella) appear pathognomonic. A normal endocrine evaluation might help in the diagnosis and warrants conservative treatment.

We report a case of a 25-year-old woman with unilateral Coats'-like disease. Her brother was previously diagnosed with an autosomal dominant form of dyskeratosis congenita. Genetic testing was performed by screening the TERC gene for mutations and identified heterozygosity for the n.68&#95;124del mutation. Our case demonstrates that the exudative retinopathy seen in Coats'-like disease can be caused by mutations in a telomere-capping gene TERC as a part of the dyskeratosis congenita spectrum without other systemic involvement. This is an interesting case that illustrates that retinal Coats'-like involvement can be the first manifestation of dyskeratosis congenita.

Competing Interests: Competing interests: None declared.

The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions. The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe-sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke's cleft cyst, which became possible with the use of ¹¹C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke's cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess. The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig-ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.

Endodermal cysts, also known as neurogenic, neuroenteric, foregut, bronchogenic, respiratory, epithelial, teratomatous, or gastrocytoma cysts, can be found in the central nervous system, predominantly in the subarachnoid space of the cervical and thoracic spinal cord. We describe a child with an endodermal cyst of the third nerve and highlight neuroimaging findings, pathological correlation, clinical course, and patient management.

Background: The management of high-grade glioma (HGG) has been affected by recent landmark trials and is now more proactive. More aggressive treatment leads to hospitalization due to side effects, however. Space-occupying tumor bed cysts have been described, but not systematically assessed. We sought to analyze this complication in a contemporary HGG cohort., Methods: We performed a retrospective review of patients with HGG treated between 2007 and 2013, identified patients with space-occupying tumor bed cysts, and reviewed their hospital notes for relevant variables. Statistical analyses were performed, and odds ratios (ORs) with 95% confidence intervals (CIs) were calculated., Results: Tumor bed cysts were found in 12 of 282 patients (4%). The main symptoms were increased intracranial pressure (n = 11), new focal deficits (n = 6), and pseudomeningocele (n = 3), presenting at a median of 19 days since the last resection. Cysts were treated with cystoperitoneal (n = 7) and ventriculoperitoneal (n = 5) shunts, resulting in clinical benefit in 75% of those treated. Intraoperative opening of ventricles is a risk factor, with an OR of 39.339. We propose a classification system comprising 3 cyst types: isolated cyst, cyst with local cerebrospinal fluid (CSF) disturbance, and cyst with global CSF disturbance., Conclusions: In modern neuro-oncology, the rate of tumor bed cysts complicating HGG management appears stable compared with historical data. Shunt implantation is feasible and effective. We propose a classification system as a common data element for comparison across future studies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Shunt malfunction represents one of the most common problem to deal with in the daily neurosurgical practice. Among the variety of possible presentations, the cyst-like dilatation of the brain around the proximal catheter is one of the most rare and misleading type. The case of a boy with a 'tumour-like' proximal shunt malfunction is described and the pertinent literature is analysed.

Background: Hypophysitis is a rare disease entity mimicking a pituitary adenoma. Despite crucial disease involving a pituitary gland which is a main component of the hormonal axis, there have been minimal researches regarding the hypophysitis. In this study, we described the constellation of the preoperative findings including clinical, radiologic, and endocrinologic features, as well as postoperative outcomes of the hypophysitis., Methods: The authors reviewed the data retrospectively of a total of 2814 patients who underwent microsurgery at our institution to treat a pituitary mass lesion. Among them, the patients proven as hypophysitis in histologic examination were enrolled, followed by stratification into tumor-associated hypophysitis and isolated hypophysitis depending on the existence of associated tumor lesion. As a result, a total 21 cases were enrolled consisting of the 13 cases of tumor-associated and the 8 cases of isolated hypophysitis. In this study, the isolated hypophysitis was focused on the analysis. All patients with isolated hypophysitis were evaluated the clinical, radiologic, and endocrinologic tests preoperatively. After microsurgery, all patients had a minimum follow up of 6 months (6∼72 months). The clinical symptoms, endocrinologic tests, and brain MRI were checked at every 6 months during first 2 year, since then, at every 12 months., Results: Among 2814 cases of pituitary mass lesion underwent microsurgery, the hypophysitis were found 21 cases (0.74 %). The 13 cases were the tumor-associated hypophysitis, consisting of rathke's cleft cyst (7 cases), craniopharyngioma (3 cases), pituitary adenoma (2 cases), and germinoma (1 case). The 8 cases were the isolated hypophysitis including lymphocytic (4 cases), granulomatous (3 cases), and IgG4-related (1 case) hypophysitis. The patients presented a wide range of clinical symptoms from general weakness to panhypopituitarism. In addition, the most remarkable radiologic features of the isolated hypophysitis were infundibular thickening and the low signal intensity in T1WI and T2WI. All 8 patients with isolated hypophysitis presented endocrinologic deficiency with various extent in a preoperative biochemistry. After surgery, the hormonal status were not changed in 4 patients (50%), however, aggravated in 4 patients (50%). The 3 patients (37.5%) complained visual field defects in preoperative ophthalmologic test, but improved all after surgery., Conclusion: In this study, the preoperative clinical manifestations, radiologic and endocrinologic features of hypophysitis were described comprehensively. In addition, we also revealed the long-term outcomes of hypophysitis in the aspect of visual function and endocrinologic outcomes. Preexisting visual field defects were improved in all patients (3 cases). However, all patients presented endocrinologic deficiency in preoperative biochemistry, among them, the 4 patients (50 %) were unchanged and the 4 patients (50 %) were exacerbated after surgery. In conclusion, the visual outcomes were satisfactory, however, the endocrinologic outcomes were vice versa, because the preoperative endocrinologic deterioration was unchanged or even aggravated after surgery in some cases., (Copyright © 2016. Published by Elsevier Inc.)

Background: Rathke cleft cyst (RCC) is a rare cystic sellar entity, which is usually small in size and asymptomatic in most patients. RCC presenting panhypopituitarism and a cystic lesion with rim enhancement on magnetic resonance imaging is extremely rare. Therefore, it is easy to be misdiagnosed as pituitary abscess because of the similar clinical manifestations and neuroimaging changes., Case Summary: We report a rare case of RCC masquerading as pituitary abscess clinically and radiologically with no evidence of central nervous system infection. The patient was initially suspected to be diagnosed with pituitary abscess, which was denied by the histopathological findings of RCC with no intraoperative drainage of abscess.We present an uncommon case of RCC masquerading as pituitary abscess in a 62-year-old Chinese male patient. The patient was admitted to Peking Union Medical College Hospital complaining of severe frontal pulsatile headache, visual acuity deficit, polyuria, polydipsia, and slight disturbance of consciousness. The biochemical and endocrinological examinations revealed severe hyponatremia and panhypopituitarism. Magnetic resonance imaging showed a sellar lesion with the apparent cystic change and rim enhancement. Accordingly, pituitary abscess was misdiagnosed at the beginning.The patient received hormone replacement therapy and underwent a trans-sphenoidal surgery. The surgical findings were uneventful. The histopathological examinations showed no infiltration of inflammatory cells or pus, and proved the lesion to be RCC., Conclusion: Through this rare case, we aim to emphasize that the differential diagnosis of sellar lesions requires constant vigilance and that RCC may lead to clinical and radiological changes similar with pituitary abscess.

Background: Leukoencephalopathy, cerebral calcifications, and cysts (LCC) is a rare disease that was first reported by Labrune in 1996. A case of adult-onset LCC was successfully followed up for a long period., Case Presentation: A 30-year-old female presented with visual field disturbance and seizure on several occasions. Radiographic images revealed multiple supratentorial cysts and calcifications in the bilateral nucleus basalis and cerebella. Aspiration, Ommaya reservoir placement, and nodule removal were performed for the responsible cysts, and the patient had a good postoperative course., Discussion: A tiny, strongly enhanced nodule was identified before cyst formation on her radiographic images. Thus, cyst growth may be related to nodule microbleeding. According to our review, if the responsible cyst is located on the noneloquent area, surgical removal of the cyst should be considered. However, if the responsible cyst is located on the eloquent area, the nodule should be first removed because nodules can bleed and enlarge cysts., Conclusion: Careful follow-up is needed, especially for cysts with a strongly enhanced nodule., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Rational: Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) is believed to be an autosomal recessive genetic disease, with disorders in multisystem organs. Its characteristic neurological disorders manifested on neuroimaging are a triad of leukoencephalopathy, intracranial calcifications, and parenchymal cysts. In this paper, we report a CRMCC patient with multisystem involvement, focusing on the neuroimaging features, to get a better understanding of the rare disease and improve our diagnostic ability., Patient Concerns: The 23-year-old female patient firstly presented with an adolescence onset of ophthalmological manifestations. Four years later, hematological and neurological disorders occurred, the latter of which demonstrated a relatively slow progression in the following 7 years preceding her presentation to our hospital., Interventions: During hospitalization, disorders involving digestive, cardiovascular and respiratory systems were also detected. In addition, a more comprehensive depiction of neurological disorders on neuroimaging was also obtained., Diagnoses: On the basis of multiple system disorders and the detection of mutations in conserved telomere maintenance component 1(CTC1) gene, a diagnosis of CRMCC was made., Outcomes: After supportive therapy during her 4-week hospitalization, the patient's general condition improved and was released from the hospital., Lessons: CRMCC could be primarily diagnosed with the aid of its multiple system disorders and remarkable neuroimaging features. Cerebral micro hemorrhages determined by the combination of CT and T2-weighted magnetic resonance images in our case could provide some additional information for diagnosis. Furthermore, several other associated disorders were depicted for the first time in our case, expanding the clinical spectrum of CRMCC., Competing Interests: The authors have no conflicts of interest to disclose.

Background: Myofibroblastoma is a rare benign soft tissue tumor comprised of contractile myiod cells mostly in mammary gland. Only 4 prior cases arising in the central nervous system have been reported in the literature. We present a case of myofibroblastoma with a cystic component., Case Description: The patient is a 76-year-old man with a history of Parkinson disease. The tumor was found incidentally after a minor fall. Magnetic resonance imaging revealed a 6.7-cm well-circumscribed, partly cystic mass with a 2.4-cm calcified central nodule located at the left sylvian fissure. The frontal dural base showed avid enhancing after gadolinium injection. Gross total resection was achieved. The tumor was marked by dense collagenous tissue and bland spindled cells in pathology review. The spindled cells demonstrated positive staining with antibodies to CD34, estrogen receptor, and smooth muscle actin. A blush of immunoreactivity is observed in scattered cells with antibody to progesterone receptor. Patient recovered well postoperatively., Conclusions: This is a rare condition of a benign soft tissue tumor of mammary gland presented primarily in the brain. The literature on myofibroblastomas arising in the central nervous system is reviewed., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Suprasellar arachnoid cysts (SAC) in children are considered rare, but the incidence is increasing due to the improvement of prenatal diagnosis. We present 15 cases of SAC diagnosed during the antenatal period between 2005 and 2015. The records were reviewed retrospectively by specifying the radiological characteristics, treatment modalities, outcomes, and long-term monitoring. Mean follow-up was 71 months. The forms (SAC-1) accounted for 2 cases (13%) with hydrocephalus. We observed 8 (53%) lower forms (SAC-2) with interpeduncular cistern expansion without hydrocephalus. The 5 (33.5%) remaining patients showed asymmetrical forms (SAC-3). Six patients (40%) were treated by ventriculo-cysto-cisternostomy, 1 by fetoscopy, 1 (6.5%) by ventriculo-peritoneal shunt, 2 (13.5%) by pterional craniotomy, and 6 (40%) were simply followed. The surgical outcomes were initially favorable in 9 cases (100%), 1 patient (13%) had to be re-treated later. Non-operated patients were all type 2 and showed no radiological changes. In the long-term, 1 patient (6.5%) had endocrine disruption, 1 had delayed development, 2 (13.5%) had minor neuropsychological impairments, and 1 had epilepsy. Combined monitoring with prenatal MRI and ultrasound can be used to distinguish three subtypes of SAC. SAC-1 and SAC-3 have an excellent prognosis after treatment in the perinatal period. SAC-2 can benefit from simple monitoring and remain asymptomatic in their majority. This classification allows a better prognosis estimation and better treatment decision., (Copyright © 2016. Published by Elsevier Masson SAS.)

Background: Dermoid cysts are benign lesions which contain epidermal and dermal elements. Dermoid cysts usually contain a dermal sinus tract but this is not mandatory. Dermoid cysts can manifest by spinal cord infection without a dermal sinus tract. An infected spinal dermoid cyst associated with a holocord spinal abscess poses diagnostic and surgical challenges. Although radical surgical drainage is considered as the main treatment modality for spinal abscess, less extensive surgery for microbiological sampling and appropriate antibiotic treatment can be another alternative modality., Case Description: A 1-year-old boy patient was admitted to our hospital with progressive paraplegia, bladder dysfunction, and neck rigidity. Medical history of the patient included recurrent urinary tract infection and cephalosporin treatments several times. Initial neurological examination revealed confusion, fever, neck rigidity, paraplegia (also, the motor power of the right upper extremity was three fifths that of the upper extremities). He had urinary and gastrointestinal retention. Conservative surgery was performed to take pathological and microbiological samples. With appropriate antibiotic regimen, the nuchal rigidity and fever improved dramatically. Infectious parameters in blood biochemistry significantly decreased after the antibiotic regimen., Discussion and Conclusion: Holocord spinal abscesses are a rare entity. The source of the disease can be hemopoietic spread or contagious spread. The dermal sinus tract is major risk factor for contagious spread. The major hemopoietic sources are urogenital infection, endocarditis, and infective lung diseases. The hemopoietic spread is a more common source for pediatric patients. The thoracolumbar region is the most common site of involvement. Spinal infection has a tendency to extend longitudinally throughout spinal fibers. The pathogenesis of the holocord edema or syrinx is uncertain. The underlying etiology may be inflammation, infection, and associated venous congestion within the spinal canal. Also, CSF circulation alteration due to arachnoiditis may be another etiological factor. The most important entities on outcome are appropriate surgical treatment and antibiotic administration.

Rathke cleft cysts (RCCs) are nonneoplastic lesions that are thought to be the remnants of Rathke cleft pouch. The authors report a patient presented with a headache and was diagnosed with RCC on imaging. The lesion underwent spontaneous involution. The authors suggest that patients presenting solely with a headache to be treated conservatively, because it is uncertain whether a headache is definitively associated with RCCs and because there is the possibility of spontaneous regression.