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1.
Front Endocrinol (Lausanne) ; 15: 1323947, 2024.
Article En | MEDLINE | ID: mdl-38405141

Introduction: Pineal cysts have long been considered a benign intracranial variation. However, in our clinical practice, it has been observed that some children with central precocious puberty (CPP) who have pineal cysts experience rapid progression in adolescent development. In recent years, there has been a significant increase in the prevalence of CPP in girls, leading to more diagnoses of CPP among children with pineal cysts. Despite this, there is no consensus regarding whether pineal cysts contribute to CPP as one of its organic factors. This study aimed to analyze the clinical characteristics of pineal cysts in children with CPP and explore the potential effects of pineal cysts on puberty development. Methods: This single-center study retrospectively analyzed clinical data from girls aged 3 to 10 years who underwent head/pituitary magnetic resonance imaging at the Children's Hospital Affiliated to Zhengzhou University between 2019 and 2022. The study categorized the detection rates of pineal cysts based on systematic disease classification and compared the rates of cyst detection between girls diagnosed with CPP and those without CPP. Subsequently, CPP-diagnosed girls with pineal cysts were examined. Among CPP-diagnosed girls meeting the study's criteria, those with pineal cysts formed the 'cyst group,' while those without cysts were matched in a 1:1 ratio based on age and body mass index to form the 'non-cyst group.' Comparative analyses were conducted to assess the clinical characteristics between these two groups. CPP-diagnosed girls with cysts were further subdivided into three groups according to cyst size (≤5 mm, 5.1-9.9 mm, and ≥10 mm) to investigate potential differences in clinical characteristics among these subgroups. The study involved an analysis of clinical data from girls diagnosed with CPP and included imaging follow-ups to explore the progression of pineal cysts over time. Results: Among the 23,245 girls who underwent head/pituitary magnetic resonance imaging scans, the detection rate of pineal cysts was 3.6% (837/23,245), with most cases being associated with endocrine diseases. The detection rate of pineal cysts in CPP patients was 6.4% (262/4099), which was significantly higher than the 3.0% (575/19,146) in patients without CPP. In comparison to the non-cyst group, the cyst group exhibited statistically significant increases in estradiol levels, peak luteinizing hormone (LH) levels, peak LH/follicle-stimulating hormone (FSH) ratios, uterine body length, and cervix length (P < 0.001). As cyst size increased, there were significant rises in LH peak, peak LH/FSH ratio, uterine body length, and cervical length (P < 0.01). Estradiol levels and left ovarian volume also showed an increasing trend (P < 0.05). Among girls who underwent follow-up imaging, 26.3% (5/19) exhibited an increase in cyst size. Conclusion: Pineal cysts are relatively common in children with CPP. They may affect the pubertal development process, with larger cysts correlating to faster pubertal development. Therefore, the authors hypothesize that pineal cysts may trigger CPP in some cases, especially when the cysts are larger than 5 mm in size, as indicated by our data.


Central Nervous System Cysts , Cysts , Puberty, Precocious , Child , Female , Humans , Adolescent , Luteinizing Hormone , Puberty, Precocious/diagnosis , Retrospective Studies , Follicle Stimulating Hormone , Cysts/complications , Cysts/diagnostic imaging , Follicle Stimulating Hormone, Human , Central Nervous System Cysts/complications , Central Nervous System Cysts/diagnostic imaging , Estradiol
2.
Neurochirurgie ; 70(2): 101540, 2024 Mar.
Article En | MEDLINE | ID: mdl-38354572

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.


Carcinoma, Renal Cell , Central Nervous System Cysts , Kidney Neoplasms , Stroke , Male , Humans , Child , Carcinoma, Renal Cell/complications , Central Nervous System Cysts/complications , Central Nervous System Cysts/diagnosis , Central Nervous System Cysts/surgery , Headache/etiology , Kidney Neoplasms/complications
3.
World Neurosurg ; 185: 113, 2024 May.
Article En | MEDLINE | ID: mdl-38369105

Pineal cysts are typically detected in around 1.3% to 4.3% of patients during routine magnetic resonance imaging (MRI) scans.1,2 The vast majority of pineal cysts are benign, asymptomatic, and typically do not necessitate surgical intervention. Large pineal cysts are known to cause hydrocephalus with its associated symptoms and thus can require in rare cases surgical resection. Even in the absence of hydrocephalus, selected patients with large pineal cysts causing headaches and visual disturbances can find relief after surgical resection.3,4 The supracerebellar infratentorial (SCIT) approach is widely used and represents an extraparenchymatous approach through a natural corridor to the pineal region.5 Performing this approach in a semisitting position allows for an optimal retraction of the cerebellum by gravity. We employ a minimally invasive paramedian SCIT approach for the resection of pineal cysts. In our experience, the paramedian SCIT approach allows for a less steep operating angle and a smaller craniotomy compared with the midline SCIT approach. We present a 24-year-old female complaining of headache. The initial MRI was conducted 2 years before surgery. Following the initial evaluation, the patient experienced progressive headaches without neurologic deficits. A subsequent MRI revealed enlargement of the pineal cyst, leading to the indication for surgical resection. The surgery was performed mainly under the operating microscope with endoscopic visualization in suitable situations as our small approach restricts bimanual dissection with an endoscope. In our experience, this approach provides a versatile and minimally invasive access to the pineal region, making it optimally suitable for pineal cysts requiring surgical resection.


Microsurgery , Neurosurgical Procedures , Pineal Gland , Humans , Female , Pineal Gland/surgery , Pineal Gland/diagnostic imaging , Microsurgery/methods , Neurosurgical Procedures/methods , Young Adult , Magnetic Resonance Imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/complications , Cysts/surgery , Cysts/diagnostic imaging , Cerebellum/surgery , Cerebellum/diagnostic imaging
4.
Hum Vaccin Immunother ; 20(1): 2297455, 2024 Dec 31.
Article En | MEDLINE | ID: mdl-38174857

With the widespread vaccination of COVID-19 vaccine, a few cases have been reported that COVID-19 vaccine may cause endocrine disorders. A 59-y-old man presented with a loss of appetite after the first COVID-19 vaccination, which resolved spontaneously after 3 d. After the second COVID-19 vaccination, the symptoms including the loss of appetite, nausea, and vomiting reappeared and worsened along with loss of vision. He was found to have severe hyponatremia, and further investigations revealed secondary adrenal insufficiency, secondary hypothyroidism and Rathke's cleft cyst. The patient responded well to glucocorticoid and levothyroxine supplementation, and at 1-y follow-up the patient developed hypogonadism. We hypothesize that hypophysitis is probably induced by COVID-19 vaccine and report the rare but serious adverse reactions for early recognition and intervention.


COVID-19 Vaccines , COVID-19 , Central Nervous System Cysts , Hypophysitis , Humans , Male , Central Nervous System Cysts/complications , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Hypophysitis/chemically induced , Middle Aged
5.
Clin Neurol Neurosurg ; 236: 108111, 2024 Jan.
Article En | MEDLINE | ID: mdl-38199117

OBJECTIVES: The purpose of this study was to evaluate the effectiveness of endoscopic endonasal surgery (EES) for Rathke's cleft cysts (RCCs) and the advantages of detailed preoperative imaging evaluation, intraoperative personalized removal and multilevel sellar floor reconstruction. METHODS: The clinical data of 43 patients with RCCs who were treated by EES in the neurosurgery department of affiliated hospital of Jiangnan University and Wuxi No.2 People's Hospital from January 2018 to January 2023 were retrospectively analyzed. The effectiveness of EES for RCCs was analyzed by imaging information, surgical procedures, symptom improvement and complications. RESULTS: All 43 RCCs were completely removed by EES, and all clinical symptoms improved to varying degrees. Postoperative relief of headache was achieved in 23 out of 26 patients (88.5 %); there was improvement in 10 out of 13 patients with visual field disorders (76.9 %) and in 8 out of 10 patients with endocrine abnormalities (80 %). New hormonal deficiency was discovered in 7 of all the patients postoperatively. There were 8 patients with postoperative diabetes insipidus and 1 patient with cerebrospinal fluid leakage. The incidence of new hormonal dysfunction and postoperative DI in expanded EES (33.3 %, 33.3 %) was higher than it in conventional EES (4 %, 8 %) (P < 0.05). The average follow-up time was 29.1 ± 14.8 months, and there were no deaths or infections. Three patients presented with cyst recurrence on MRI. CONCLUSIONS: The clinical manifestations and imaging characteristics of RCCs are variable, and a detailed preoperative review of the imaging is helpful for the development of surgical plans. RCCs can be treated more safely and thoroughly with less trauma and complications by intraoperative personalized removal and multilevel sellar floor reconstruction. The high incidence of new hormonal dysfunction and postoperative DI may be related to the disturbance of the pituitary stalk. EES has unique advantages and high clinical application value for the treatment of RCCs.


Central Nervous System Cysts , Cysts , Pituitary Neoplasms , Humans , Retrospective Studies , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Endoscopy , Cysts/complications , Pituitary Gland/diagnostic imaging , Pituitary Gland/surgery , Pituitary Neoplasms/surgery
6.
Endocr J ; 71(3): 285-293, 2024 Mar 28.
Article En | MEDLINE | ID: mdl-38281757

Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered by cystic fluid leakage or hemorrhage. Our aim was to compare clinical characteristics and presenting features of patients with secondary etiology of XG and those with no identifiable founding lesion (primary -"pure" XG) in order to gain new insights into this rare pituitary pathology. In a retrospective review of 714 patients operated for sellar masses, at tertiary center, we identified 16 (2.24%) with histologically confirmed diagnosis of pituitary XG over the period of 7 years (2015-2021). Patients were further analyzed according to XG etiology: "pure"- XG (n = 8) with no identifiable founding lesion were compared to those with histological elements of pituitary tumor or cyst - secondary XG (n = 8). We identified 16 patients (11 male), mean age 44.8 ± 22.3 years, diagnosed with pituitary XG. Secondary forms were associated with Ratke's cleft cyst (RCC, n = 2) and pituitary adenoma (PA, n = 6). The most common presenting features in both groups were hypopituitarism (75%), headache (68.5%) and visual disturbances (37.5%). Predominance of male sex was noted (males 68.75%, females 31.25%), especially in patients with primary forms. Patients with primary pituitary XG were all males (p = 0.0256) and more frequently affected by panhypopituitarism (87.5% vs. 25%, p = 0.0406) compared to patients with secondary causes. Hyperprolactinemia was noted in pituitary tumor group with secondary etiology only (p = 0.0769). Majority of lesions were solid on magnetic resonance imaging - MRI (81.25%). Distinct clinical phenotype was observed dependent on the etiology of XG.


Central Nervous System Cysts , Cysts , Pituitary Diseases , Pituitary Neoplasms , Xanthomatosis , Female , Humans , Male , Young Adult , Adult , Middle Aged , Aged , Pituitary Neoplasms/complications , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/epidemiology , Pituitary Diseases/epidemiology , Pituitary Gland/diagnostic imaging , Pituitary Gland/pathology , Magnetic Resonance Imaging , Central Nervous System Cysts/complications , Cysts/pathology , Granuloma/complications , Granuloma/pathology , Xanthomatosis/epidemiology , Xanthomatosis/pathology
7.
World Neurosurg ; 182: 83-90, 2024 Feb.
Article En | MEDLINE | ID: mdl-37995988

OBJECTIVE: Intracranial dermoid cyst (DC) is a rare benign, slow-growing lesion, most commonly arising along the midline. They can occur in the supratentorial compartment, very rarely involve the sellar region and only exceptionally are intrasellar. The aim of our study is to address the challenges in the diagnosis and management of sellar DCs. METHODS: We performed a systematic review of sellar DCs, in keeping with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and described an intrasellar DC in a 32-year-old female who presented with bilateral blurring vision. RESULTS: The review identified 4 intrasellar, 29 suprasellar, and 28 parasellar cases. Intrasellar DCs more likely present with progressive visual impairment and pituitary hormone dysfunctions during the fifth decade of life. Suprasellar and parasellar DCs are typically diagnosed during the third decade of life because of diplopia, ptosis, trigeminal hypoaesthesia/para-esthesia or cyst's rupture. Sellar DCs are typically hypodense on computed tomography scans and contain calcifications. Magnetic resonance imaging features include T1 hyperintensity, T2 heterogeneous intensity, no restriction on diffusion-weighted images, and no contrast enhancement. Surgery is the treatment of choice. Gross total resection is achieved in 60% of intrasellar and 61.9% of suprasellar and parasellar DCs. Early postoperative complications are reported in 40.0%, 16.7%, and 23.8% of intrasellar, suprasellar, and parasellar DCs, respectively. CONCLUSIONS: Intrasellar DCs are rare lesions typically diagnosed later than suprasellar and parasellar DCs due to their different clinical presentations. However, they should be considered in the differential diagnosis of cystic lesions of the sella, including epidermoid cysts, craniopharyngiomas, Rathke's cleft cysts, and teratomas.


Central Nervous System Cysts , Dermoid Cyst , Epidermal Cyst , Pituitary Neoplasms , Female , Humans , Adult , Dermoid Cyst/diagnostic imaging , Dermoid Cyst/surgery , Dermoid Cyst/complications , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/surgery , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Epidermal Cyst/complications , Magnetic Resonance Imaging , Sella Turcica/diagnostic imaging , Sella Turcica/surgery , Sella Turcica/pathology
9.
Endokrynol Pol ; 74(6)2023.
Article En | MEDLINE | ID: mdl-38159204

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.


Adrenal Insufficiency , Carcinoma, Renal Cell , Central Nervous System Cysts , Empty Sella Syndrome , Kidney Neoplasms , Pituitary Neoplasms , Humans , Empty Sella Syndrome/complications , Empty Sella Syndrome/diagnosis , Hydrocortisone , Hypothalamo-Hypophyseal System , Dizziness , Pituitary-Adrenal System , Adrenal Insufficiency/etiology , Adrenal Insufficiency/complications , Central Nervous System Cysts/complications , Central Nervous System Cysts/diagnosis , Magnetic Resonance Imaging , Kidney Neoplasms/complications , Pituitary Neoplasms/complications
11.
Childs Nerv Syst ; 39(12): 3467-3474, 2023 Dec.
Article En | MEDLINE | ID: mdl-37898987

BACKGROUND: Pineal cysts are a rare lesion of the pineal gland. Pineal cysts are benign lesions, generally asymptomatic, and are usually an incidental discovery on MRI performed for other problems. The management of pineal cysts in children remains a matter for debate. Here, we report our own retrospective paediatric cases that have been surgically treated and review the paediatric literature on this topic. METHODS: This is a retrospective monocentric study. All patients operated by the senior author (CM) for a benign pineal cyst from 2000 to 2021 were included. All other pineal region cystic lesions were excluded. Medical and surgical data were extracted from the hospital medical database. RESULTS: Twelve patients were included. The clinical symptomatology was characterized by headaches in seven patients, visual troubles in two patients, precocious puberty in one patient, signs of intracranial hypertension in two patients, seizures associated with headache in one patient, and headaches associated with behavioural troubles in another patient. No major post-operative complications were observed in this series. It is to noted that surgery was performed because a suspicion of a true pineal parenchymal tumour has been made. Histopathological study came back with the diagnosis of pineal cyst. CONCLUSIONS: Pineal cyst is rare. If the radiological diagnosis is clear, no surgery is advocated except in cases associated with hydrocephalus and rapid growth. In case of a suspicion of a true pineal parenchymal tumour, a surgery may be needed to confirm the diagnosis. Lastly, we stress that only cystic lesions of the pineal gland itself should be considered as pineal cyst.


Brain Neoplasms , Central Nervous System Cysts , Cysts , Pineal Gland , Pinealoma , Humans , Child , Pinealoma/diagnostic imaging , Pinealoma/surgery , Cysts/diagnostic imaging , Cysts/surgery , Cysts/complications , Retrospective Studies , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/surgery , Brain Neoplasms/complications , Magnetic Resonance Imaging/adverse effects , Pineal Gland/diagnostic imaging , Pineal Gland/surgery , Headache/etiology
13.
J Med Case Rep ; 17(1): 388, 2023 Sep 12.
Article En | MEDLINE | ID: mdl-37697403

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.


Aneurysm, Ruptured , Central Nervous System Cysts , Hypopituitarism , Intracranial Aneurysm , Subarachnoid Hemorrhage , Adult , Female , Humans , Asian People , Craniotomy , Hypopituitarism/diagnosis , Hypopituitarism/etiology , Intracranial Aneurysm/complications , Intracranial Aneurysm/diagnostic imaging , Intracranial Aneurysm/surgery , Subarachnoid Hemorrhage/diagnostic imaging , Subarachnoid Hemorrhage/etiology , Subarachnoid Hemorrhage/surgery , Central Nervous System Cysts/complications , Central Nervous System Cysts/diagnosis
14.
Acta Neurochir (Wien) ; 165(9): 2435-2444, 2023 09.
Article En | MEDLINE | ID: mdl-37530890

PURPOSE: Although Rathke cleft cysts (RCC) are benign lesions of the sellar region, recurrence is frequent after surgical treatment. Nuclear translocation of ß-catenin (NTßC), a key effector of the wnt-signaling pathway that is responsible for cell renewal, has been shown to act as a proto-oncogene and is considered to be a potential risk factor for increased recurrence in RCC. In this study, we analyzed a surgically treated cohort into patients with and without NTßC expression in order to identify clinical and imaging differences and further evaluate the risk of recurrence. METHODS: Patients with resection of RCC between 04/2001 and 11/2020 were included. Histological specimens were immunohistochemically stained for ß-catenin. Study endpoints were time to cyst recurrence (TTR) and functional outcome. Functional outcome included ophthalmological and endocrinological data. Furthermore, MRI data were assessed. RESULTS: Seventy-three patients (median age 42.3 years) with RCC underwent mainly transsphenoidal cyst resection (95.9%), 4.1% via transcranial approach. Immunohistochemical staining for ß-catenin was feasible in 61/73 (83.6%) patients, with nuclear translocation detected in 13/61 cases (21.3%). Patients with and without NTßC were equally likely to present with endocrine dysfunction before surgery (p = 0.49). Postoperative new hypopituitarism occurred in 14/73 (19.2%) patients. Preoperative visual impairment was equal in both groups (p = 0.52). Vision improved in 8/21 (33.3%) patients and visual field deficits in 22/34 (64.7%) after surgery. There was no difference in visual and perimetric outcome between patients with and without NTßC (p = 0.45 and p = 0.23, respectively). On preoperative MRI, cyst volume (9.9 vs. 8.2 cm3; p = 0.4) and evidence of hemorrhage (30.8% vs. 35.4%; p = 0.99) were equal and postoperative cyst volume decreased significantly in both groups (0.7 vs. 0.5 cm3; p < 0.0001 each). Cyst progression occurred in 13/73 (17.8%) patients after 39.3 ± 60.3 months. Cyst drainage with partial removal of the cyst wall resulted in improved recurrence-free survival without increasing the risk of complications compared with cyst fenestration alone. Patients with postoperative diabetes insipidus had an increased risk for recurrence according to multivariate analysis (p = 0.005). NTßC was evident in 4/15 patients (26.7%) and was not associated with a higher risk for recurrence (p = 0.67). CONCLUSION: Transnasal transsphenoidal cyst drainage with partial removal of the cyst wall reduces the risk of recurrence without increasing the risk of complications compared with fenestration of the cyst alone. Patients with postoperative diabetes insipidus seem to have an increased risk for recurrence. In contrast, NTßC was not associated with a higher risk of recurrence and did not provide stratification for clinically distinct patients.


Carcinoma, Renal Cell , Central Nervous System Cysts , Cysts , Diabetes Insipidus , Kidney Neoplasms , Humans , Adult , beta Catenin , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Diabetes Insipidus/etiology , Magnetic Resonance Imaging/adverse effects , Catenins , Retrospective Studies , Cysts/complications , Treatment Outcome
15.
Acta Neurochir (Wien) ; 165(8): 2267-2276, 2023 08.
Article En | MEDLINE | ID: mdl-37160776

BACKGROUND: Both intrasuprasellar and suprasellar Rathke cleft cysts (RCCs) have suprasellar components, and we aimed to explore their clinical features and surgical outcomes. METHOD: Patients with surgically treated intrasuprasellar or suprasellar RCCs were retrospectively analyzed. All patients with intrasuprasellar RCCs were treated with the standard endoscopic endonasal approach (EEA, group I); the patients with suprasellar RCCs received the extended EEA (group II) or supraorbital keyhole approach (SKA, group III) according to the relevant indications. A surgical strategy of maximal safe resection aiming to protect neuroendocrine function was adopted. In addition, patients (distinguished from the above 3 groups) who had aggressive resection of suprasellar RCC were also enrolled for comparison of different surgical strategies. RESULTS: A total of 157 patients were eligible, including 121 patients with intrasuprasellar RCCs in group I, 19 patients with suprasellar RCCs in group II, and 17 patients with suprasellar RCCs in group III. Preoperatively, the patients with suprasellar RCC (groups II and III) more commonly presented with visual dysfunction, diabetes insipidus (DI), and hyperprolactinemia than the patients with intrasuprasellar RCCs (all p<0.05). A higher incidence of hypopituitarism and a larger diameter were observed for intrasuprasellar RCCs (both p<0.05). Postoperatively, group II had a higher rate of new-onset DI, hyponatremia, and recurrence than group I (all p<0.025) and similar outcomes to group III. For suprasellar RCCs, comparison of the maximal safe resection vs. aggressive resection (supplementary patients: 14 with extended EEA, 12 with SKA) showed similar improvement and recurrence, with higher rates of DI and hyponatremia with the latter strategy (all p<0.05). CONCLUSIONS: Suprasellar RCC is associated with more complicated preoperative presentations, intricate postoperative complications, and frequent recurrence compared with intrasuprasellar RCC. Under rational indications, both extended EEA and SKA achieve satisfactory outcomes. The strategy of maximal safe resection is recommended for greatest functional preservation.


Carcinoma, Renal Cell , Central Nervous System Cysts , Diabetes Insipidus , Hyponatremia , Kidney Neoplasms , Pituitary Neoplasms , Humans , Retrospective Studies , Diabetes Insipidus/complications , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Treatment Outcome , Pituitary Neoplasms/surgery , Pituitary Neoplasms/complications
16.
Acta Neurochir (Wien) ; 165(8): 2277-2282, 2023 08.
Article En | MEDLINE | ID: mdl-37046123

BACKGROUND: The most common presenting symptom in patients with both small and large Rathke cleft cysts (RCC) is headache (H/A). It is well established that patients with large RCC can have significant symptomatic improvement after cyst drainage. However, patients with small RCC (≤ 1 cm) are rarely operated on, even if they present with debilitating H/A. It is not well understood whether resection of these smaller RCCs can lead to durable H/A resolution. METHODS: A retrospective search of our institutional database for sub-centimeter RCCs presenting with intractable H/A and treated with an endoscopic endonasal approach was carried out. A detailed H/A questionnaire as well as patient chart review was conducted to assess the long-term outcome of these patients after surgical intervention. RESULTS: Ten consecutive patients with 11 endonasal surgeries met inclusion criteria. Eight responded to the questionnaire. The median cyst diameter was 6 mm (IQR 3-9). Median preoperative H/A duration was 12 months (range 2 months-15 years). H/As occurred on average for 20 days per month and all required analgesics for symptomatic control for more than 15 of these 20 days. Half of the patients also had to miss work because of H/A. Average preoperative H/A intensity was 8.7 (scale 0-10) compared with postoperative scores of 2.9 at one month, 1.6 at 3 months, and 0.9 at 1 year. There were no permanent endocrinological or other surgical complications. After a median follow-up of 2 years, one patient had radiographic and symptomatic recurrence which resolved after re-operation. CONCLUSIONS: Endoscopic fenestration of sub-centimeter RCCs provides a safe and durable treatment for patients with intractable H/A.


Carcinoma, Renal Cell , Central Nervous System Cysts , Cysts , Kidney Neoplasms , Humans , Retrospective Studies , Headache/etiology , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Cysts/diagnostic imaging , Cysts/surgery , Cysts/complications , Treatment Outcome
17.
Neurol Sci ; 44(8): 2715-2729, 2023 Aug.
Article En | MEDLINE | ID: mdl-37004603

Leukoencephalopathy with calcifications and cysts (LCC; OMIM #614561) is a rare disease and at present there are less than 100 cases reported worldwide. Mutations in the SNORD118 gene is now known to be the cause of LCC. We present a case who was heterozygous for the n.70G>A and n.6C>T sequence variants of the SNORD118 gene, variants which to date have not been described. Compared with the cases that we reviewed, our patient had the second longest time to diagnosis (age 56) from onset of symptoms 40 years prior. Moreover, his cousin's family has a high prevalence of epilepsy. This paper reviewed all published reports to date that had descriptive cases involving LCC as well as testing for the SNORD118 gene. Since 1996 only 85 patients have been described in 59 case reports. In this review, we summarize their clinical features, especially central nervous system symptoms, treatment, pathology, and gene testing results.


Calcinosis , Central Nervous System Cysts , Cysts , Leukoencephalopathies , Humans , Adult , Central Nervous System Cysts/complications , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/genetics , Cysts/genetics , Cysts/pathology , Leukoencephalopathies/complications , Leukoencephalopathies/diagnostic imaging , Leukoencephalopathies/genetics , Calcinosis/complications , Calcinosis/diagnostic imaging , Calcinosis/genetics , Mutation/genetics
18.
Oper Neurosurg (Hagerstown) ; 24(4): 368-376, 2023 04 01.
Article En | MEDLINE | ID: mdl-36701658

BACKGROUND: Rathke cleft cysts (RCCs) are common benign skull-base lesions arising from embryologic remnants of Rathke pouch. Though frequently asymptomatic, RCCs can become symptomatic because of compression of adjacent neural structures. Transcranial and neuroendoscopic surgical treatments have been described for symptomatic RCCs, but recurrence rates remain as high as 30%. Bioabsorbable steroid-eluting (BASE) stents significantly decrease adhesions and recurrent ostia obstruction after endoscopic sinus surgery. We sought to use BASE stents to aid marsupialization of symptomatic RCCs. OBJECTIVE: To present long-term results of our initial experience with endoscopic-endonasal fenestration and placement of BASE stents for RCCs. METHODS: Patients undergoing neuroendoscopic transsphenoidal fenestration of RCCs with BASE stent placement were identified and their medical records retrospectively reviewed. RESULTS: Four patients underwent neuroendoscopic transsphenoidal fenestration and BASE stent placement from March 2016 to April 2018 for symptomatic RCCs. After the cyst contents were evacuated, a BASE stent was deployed in the cyst fenestration to prevent cyst wall regrowth or closure and facilitate marsupialization to the sphenoid sinus. No perioperative complications were encountered, and all patients reported symptom resolution by 2 weeks postoperatively. Postoperative endoscopic evaluation demonstrated epithelization of the cyst wall opening and patent marsupialization into the sphenoid sinus in all cases. After a mean follow-up of 56 ± 12 months, all patients remained asymptomatic with baseline visual function and no radiographic evidence of recurrence. CONCLUSION: Bioabsorbable steroid-eluting stent placement is a safe, facile, viable augmentation of neuroendoscopic technique for symptomatic RCCs with the potential to reduce long-term recurrence rates.


Central Nervous System Cysts , Cysts , Drug-Eluting Stents , Neuroendoscopy , Humans , Retrospective Studies , Absorbable Implants , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Central Nervous System Cysts/complications , Steroids
19.
Oper Neurosurg (Hagerstown) ; 24(6): 572-581, 2023 06 01.
Article En | MEDLINE | ID: mdl-36716050

BACKGROUND: Absence of hydrocephalus on neuroimaging may impart a false sense of security for patients with pineal cysts. In this case series, we characterize a subset of patients with pineal cysts having an occult presentation. Unifying features of worsening paroxysmal headaches suggesting intermittent obstructive hydrocephalus and radiographic evidence of third ventricular invagination characterize these patients as high risk. OBJECTIVE: To define features of occult, high-risk pineal cysts and outcomes of endoscopic cyst fenestration. METHODS: Charts were retrospectively reviewed for patients with pineal cysts evaluated at our institution between 2018 and 2021 who underwent endoscopic cyst fenestration. To capture cysts presenting as occult, patients were excluded if hydrocephalus was noted at presentation. Relevant clinical history, imaging, operative data, and clinical outcomes were reviewed. RESULTS: Of 50 pineal cyst patients, 4 satisfied inclusion criteria. All patients presented with worsening paroxysmal headaches. In addition, 75% (3/4) also experienced intermittent syncope. Patients exhibited no hydrocephalus (n = 3) or fluctuating ventricular size on longitudinal imaging (n = 1). In all cases, high-resolution sagittal 3-dimensional T2 magnetic resonance imaging demonstrated invagination of the cyst anteriorly into the posterior third ventricle. All patients underwent endoscopic cyst fenestration with complete symptom resolution (mean follow-up of 20.6 months; range 3.5-37.4 months). CONCLUSION: The clinical history for occult, high-risk pineal cysts is notable for worsening paroxysmal headaches and episodic alterations of consciousness suggesting intermittent obstructive hydrocephalus. Because ventricular size can appear normal on standard imaging protocols, clinical suspicion should trigger workup with high-resolution magnetic resonance imaging designed to detect these cysts. Endoscopic cyst fenestration is a safe and efficacious management strategy.


Brain Neoplasms , Central Nervous System Cysts , Cysts , Hydrocephalus , Humans , Retrospective Studies , Cysts/complications , Cysts/diagnostic imaging , Cysts/surgery , Hydrocephalus/diagnostic imaging , Hydrocephalus/etiology , Hydrocephalus/surgery , Brain Neoplasms/surgery , Central Nervous System Cysts/complications , Central Nervous System Cysts/diagnostic imaging , Central Nervous System Cysts/surgery , Headache/etiology
20.
Childs Nerv Syst ; 39(3): 775-780, 2023 03.
Article En | MEDLINE | ID: mdl-36323954

OBJECTIVES: Intracranial cysts are fluid-filled sacs within the brain. There is a diversity of intracranial cysts with different incidences in addition to the growing awareness about comorbidities and the consequences. The present study aimed to evaluate cystic findings in children who were admitted to the department of pediatric neurology. METHODS: Children who were admitted to the Clinic of Pediatric Neurology and who had an MRI between 2016 and 2021 were evaluated. The MRI examination was performed with the pediatric epilepsy protocol. Children with primary intracranial cysts were enrolled in the study. Demographic and clinical findings were evaluated from the hospital's database and patients' files. RESULTS: Among the 78 patients, 36 (46.2%) were male and 42 (53.8%) were female. The mean age was 7 ± 5.4 years. The most frequent presenting complaint was a seizure (47.4%). Approximately one-quarter (28.2%) had mental and/or motor retardation. Nine (11.5%) of the children had a neuropsychiatric diagnosis. Most of the cysts were located at the midline (41%) with the majority located extra-axial (71.8%) and supratentorial (78.2%). Arachnoid cysts were observed most frequently with a percentage of 64.1%, followed by pineal cysts (15.4%). The history of seizure, epilepsy, presence of mental retardation, and neuropsychiatric problems were evaluated according to the population ratios based on z approximation in which significantly higher rates were observed among cases with intracranial cysts. CONCLUSION: Intracranial cysts should be taken into consideration for comorbid pathologies, especially in the childhood period. Early evaluation in patients with intracranial cysts for developmental delay and neuropsychiatric problems is important.


Arachnoid Cysts , Central Nervous System Cysts , Epilepsy , Child , Humans , Male , Female , Infant , Child, Preschool , Arachnoid Cysts/diagnostic imaging , Arachnoid Cysts/pathology , Central Nervous System Cysts/complications , Magnetic Resonance Imaging/adverse effects , Epilepsy/etiology , Epilepsy/complications , Seizures/complications
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