Rathke's Cleft Cyst Research Articles

The RAPID Consortium: A Platform for Clinical and Translational Pituitary Tumor Research.

Objectives Pituitary tumor treatment is hampered by the relative rarity of the disease, absence of a multicenter collaborative platform, and limited translational-clinical research partnerships. Prior studies offer limited insight into the formation of a multicenter consortium. Design The authors describe the establishment of a multicenter research initiative, Registry of Adenomas of the Pituitary and Related Disorders (RAPID), to encourage quality improvement and research, promote scholarship, and apply innovative solutions in outcomes research. Methods The challenges encountered during the formation of other research registries were reviewed with those lessons applied to the development of RAPID. Setting/Participants RAPID was formed by 11 academic U.S. pituitary centers. Results A Steering Committee, bylaws, data coordination center, and leadership team have been established. Clinical modules with standardized data fields for nonfunctioning adenoma, prolactinoma, acromegaly, Cushing's disease, craniopharyngioma, and Rathke's cleft cyst were created using a Health Insurance Portability and Accountability Act-compliant cloud-based platform. Currently, RAPID has received institutional review board approval at all centers, compiled retrospective data and agreements from most centers, and begun prospective data collection at one site. Existing institutional databases are being mapped to one central repository. Conclusion The RAPID consortium has laid the foundation for a multicenter collaboration to facilitate pituitary tumor and surgical research. We sought to share our experiences so that other groups also contemplating this approach may benefit. Future studies may include outcomes benchmarking, clinically annotated biobank tissue, multicenter outcomes studies, prospective intervention studies, translational research, and health economics studies focused on value-based care questions.

The surgical outcomes following transsphenoidal surgery for Rathke cleft cysts: Comparison of the surgical approaches at a single institution

The aim of the present study is to retrospectively analyze the surgical outcomes and complications of microscopic and endoscopic transsphenoidal surgery in the management of Rathke cleft cysts (RCCs) at a single institution. A total of 38 patients were enrolled in this study. All patients were diagnosed with RCCs, which were confirmed histopathologically. Endocrine function, radiological, and clinical outcomes were evaluated following surgery. This cohort study consisted of 16 male and 22 female patients. The median age was 48 years (range, 21-72). The median clinical follow-up duration was 57 months (range, 3-187). Twenty-five patients underwent microscopic transsphenoidal surgery and 13 patients underwent endoscopic surgery. The cysts were located within the intrasellar area in 18 (47.4%) patients, and extended to the suprasellar area in 20 (52.6%) patients. The radiological characteristics were similar in the microscope and endoscope groups, except mass volume (1.40 vs 0.65 mm3; P = .003) and peripheral rim enhancement (P = .036). After surgery, 30 (78.9%) of the 38 patients had no residual cysts. There was no significant difference in outcomes between both groups (76.0% vs 84.6%; P = .689). Four (10.5%) patients experienced cyst recurrence in only the microscope group. Twenty-four of the 25 patients who presented with headache showed improvement after surgery. Four patients with visual field defects recovered after surgery. Among the 8 patients with hormonal deficiencies, hormone levels normalized in 5 patients, did not change in 2 patients and worsened in 1 patient. New hormonal deficiencies occurred in 3 patients. Microscopic or endoscopic transsphenoidal surgery for RCCs is a safe and effective treatment option. Complete aspiration of the cyst contents with wide fenestration and wall biopsy, regardless of the surgical approach used, is usually a sufficient treatment for RCCs.

Rathke Cleft Cyst with Occulomotor Palsy: An Unusual Presentation.

Rathke cleft cysts arise from remnants of Rathke's pouch. Often identified as incidental autopsy findings, these cysts can occasionally become large enough to cause headache, vision impairment, hypothalamic pituitary dysfunction and rarely, cranial neuropathy. MRI is the imaging modality of choice with surgical drainage recommended in symptomatic cases.

PMON94 Collision tumor with Rathke cleft cyst and co existing Cushing's disease

Abstract Introduction - Collision tumors, i.e., co-occurring distinct histopathological lesions are rare. We present a case of a 48-year-old female present with co-existing Rathke cleft cyst (RCC) and pituitary adenoma with Cushing disease. Case - 48-year-old female presented for evaluation of an incidental sellar mass with enlarged pituitary noted on MRI brain. The patient complained of symptoms of mental fogginess, confusion, intermittent dizziness for the last 1 year. The patient had complained of easy bruising with no reported weight gain, change in facial appearance, proximal muscle weakness. MRI brain showed mildly enlarged pituitary measuring 13 mm with RCC.Past medical history was significant for recent-onset Type 2 Diabetes and Hypertension. The examination was unremarkable with no signs of Cushing's syndrome. Lab work showed elevated ACTH of 51 pg/ml (10-48) with AM cortisol level of 14.7 mcg/dl (6- 18.4). IGF-1 levels elevated at 307 ng/ml (77- 220). LH, FSH, FT4, prolactin levels were normal. Given elevated ACTH levels, further testing showed elevated midnight salivary cortisol at 0.22 and 0.239 mcg/dl (0.010-0.090), elevated 24-hour urine cortisol 141 ug/ 24 hrs. (6- 44), absence of suppression with 1 mg dexamethasone with cortisol 12.4 mcg/dl (< 1.8), ACTH 123 pg/ml (10-48) with dexamethasone levels 432 ng/dl (140- 295). Lab work was consistent with ACTH-dependent hypercortisolism. MRI pituitary showed 7.5×7.8 mm RCC.8 mg dexamethasone suppression test and CT chest, abdomen, and pelvis were performed to differentiate between ectopic Cushing's and Cushing's disease. Cortisol levels suppressed greater than 50% after 8 mg dexamethasone from baseline cortisol – 17.7 mcg/dl and ACTH 67 pg/ml to cortisol 3.4 mcg/dl and ACTH – 34 pg/ml with dexamethasone levels of 3120 ng/dl (1600–2850). CT chest abdomen, pelvis showed no evidence of bronchial carcinoid or other potential lesions. With neuroimaging findings and biochemical confirmation of Cushing's disease, the patient underwent transsphenoidal resection. Discussion – The association between RCC and pituitary adenoma was found in 0.5–1.9% cases per literature review1, co-occurrence with ACTH producing pituitary adenoma being rarer. The diagnosis of collision tumors can be challenging as the most common presentation on imaging is a cystic mass. Appropriate biochemical testing is indicated based on clinical presentation, initial laboratory testing, and level of suspicion. Elevated ACTH levels along with diabetes and hypertension led to a diagnosis of Cushing's disease in our patient with the initial presentation of an incidental RCC. The knowledge about collision tumors is important, especially in unusual presentations as in our case. References Sagan KP et al. Cushing's Syndrome in a patient with Rathke's cleft cyst and ACTH cell hyperplasia detected by 11C-Methionine PET Imaging-A Case Presentation. Front Endocrinol (Lausanne). 2020; 11: 460. Published 2020 Jul 22. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Endoscopic Treatment of Rathke's Cleft Cysts: The Case for Simple Fenestration.

Rathke's cleft cysts (RCC) arise from the pars intermedia because of incomplete regression of the embryologic Rathke pouch. A subset of RCC becomes symptomatic causing headaches, visual and endocrinological disturbances such that surgical intervention is indicated. Several points in surgical management remain controversial including operative strategy (simple fenestration (SF) vs complete cyst wall resection (CWR)) as well as reconstructive techniques. A retrospective analysis was conducted of pathologically confirmed RCC operated on by endoscopic endonasal approach from 2006 to 2019. Pre-operative symptoms, imaging characteristics, operative strategy, symptom response, complications and recurrences were recorded. Thirty-nine patients were identified. Thirty-three underwent SF and six underwent CWR. Worsening pituitary function was significantly increased with CWR (50%) compared to SF (3%) (p = 0.008). All patients underwent "closed" reconstruction with a post-operative CSF leak rate of 5% (3% SF vs 16% CWR, p = 0.287). Six (15%) recurrences necessitating surgery were reported. Recurrence rates stratified by surgical technique (18% SF vs 0% CWR, p = 0.564) were not found to be significantly different. The current series illustrates variability in the surgical management of RCCs. SF with closed reconstruction is a reasonable operative strategy for most symptomatic RCCs cases while CWR can be reserved for selected cases.

ODP587 A Case of Rathke'S Cleft Cyst Presented With Central Diabetes Insipidus In A Young Female

Abstract Introduction Rathke's cleft cysts (RCC) are cystic sellar and suprasellar lesion derived from remnant of Rathke's pouch. RCC are mainly asymptomatic, but can present with headache, visual disturbance, panhypopituitarism, hypothalamic dysfunction when it enlarges causing compression to the surrounding structure. Diabetes insipidus as a presenting feature of RCC is rare. Even it presents with diabetes insipidus, it occurs more in elderly. Clinical Case A 32-year-old married female, mother of one child, having normal menstrual cycle presented with polyuria, polydipsia of recent onset. Clinical examinations was normal. Serum creatinine, potassium, calcium, plasma glucose were within normal range. Anterior pituitary hormonal evaluation was normal. Serum sodium was in the upper range of normal. Basal urine osmolality and serum osmolality were 87.49 mosm/kg (150 -1150 mOsm/kg) and 287 mOsm/kg (275 -295 mOsm/kg) respectively. Water deprivation test followed by vasopressin challenge test was done, which revealed partial central DI. Magnetic resonance imaging revealed a sellar cystic lesion hyperintense on both T1 and T2 weighted images, suggestive of rathke's cleft cyst and posterior pituitary bright spot was not visible. Tablet desmopressin 50 mcg twice daily was given and within two days patient responded well to the treatment. Urine volume reduced to 2.5 litres/day and water intake reduced to 3. 0 litres/day. Conclusion This is a rare case of Rathke's cleft cyst, presenting as diabetes insipidus as the sole manifestation in a young lady. Presentation: No date and time listed

Minimally invasive endoscopic approaches to pediatric skull base pathologies.

Endoscopic endonasal approaches to pediatric skull base lesions are rare, challenging, and present distinct considerations from adult pathology. In this study, we describe our endoscopic technical approach to various skull base lesions demonstrating the efficacy and nuances of these approaches in pediatric patients. Pediatric patients underwent endoscopic approach for skull base lesions from January of 2015 to April of 2021 were included in the study. Presenting symptoms, indications for surgery, surgical outcomes, intraoperative and postoperative complications, length of hospital stay, and length of follow-up were documented. A total of 18 patients (median age 12, age-range 1-15, 53% male) underwent 19 endoscopic transsphenoidal procedures. The pathologies included craniopharyngioma (N=5), biopsy for an unknown disease (n=2; orbital meningioma and pituitary lymphocytic hypophysitis), pituitary adenoma (N=2; ACTH-secreting and non-functional), Rathke's cleft cyst (n=2), CSF leak repairs (n=2; post-traumatic and spontaneous meningocele), juvenile nasopharyngeal angiofibroma (n=3, 1 patient had 2 procedures) and rhabdomyosarcoma (n=1). GTR was achieved in 11 out of 15 procedures (73.3%). Out of 10 patients with a sellar lesion, 6 patients had intraoperative CSF leak (60%). No patients experienced post-operative CSF leak. The median follow-up for all patients was 14 months (1-36 months). Anterior and middle skull base pathology in pediatric patients can be effectively operated via an endoscopic approach across a wide variety of patient ages and conditions. Minimally invasive techniques with middle turbinate and nasal septum preservation can be achieved without compromising outcomes. A wide variety of surgical repair strategies can be successfully utilized.

RF26 | PSAT109 The Prevalence of Abnormalities in Cranial MRIs in Children with Short Stature Prior to Growth Hormone Therapy

Abstract Background MRIs of the brain in patients with short stature have shown a number of abnormalities. Some of these radiological findings can have clinical significance. Here we have looked at MRI results in such a population. Objective To review the value and significance of the prevalence of all abnormal MRI findings of children with short stature who are to undergo growth hormone therapy (GHT). Materials and methods This study involved a retrospective review of MRI findings in all children prescribed GHT within a pediatric health network's database from Jan 2020 to Aug 2021. Post-gadolinium contrast enhanced brain and pituitary MRIs utilizing 2 mm slices were used to calculate pituitary volume. Pituitary volume was calculated using the ellipsoid formula (LxWxH/2). Pediatric patients diagnosed with non-acquired GHD or ISS, with MRIs having been performed between Jan 2020 and Aug 2021 and having been prescribed GHT by Aug 2021 were included in this study. Patients who experienced other endocrine abnormalities such as SGA, Turner Syndrome, and Noonan Syndrome were excluded. Patients with obstruction of sellar and parasellar religion due to movement artifacts or magnetic interference on their MRIs were also excluded. Results Of one hundred and twelve patients found, eighty one met criteria for inclusion in this study. Of the eighty one MRIs reviewed, twenty eight children, 34.6%, had normal pituitary anatomy and fifty three, 65.4%, had a pituitary abnormality. Out of the fifty three with a pituitary abnormality, forty three subjects, 81.1%, were determined to have a small pituitary volume, including significant pituitary hypoplasia. Ten subjects (18.9%) had an enlarged pituitary volume (pituitary hyperplasia). Of these ten patients who had an enlarged pituitary volume, eight were pubertal (80%). Nine children with a pituitary abnormality (16.9%) had additional structural anomalies on their MRIs. One had a small left frontal developmental venous anomaly. Two had Rathke's cleft cysts. Two had pars intermedia cysts. One had a small right parietal developmental venous anomaly. One had a small left parietal developmental venous anomaly. One had a left cerebellar tonsillar ectopia bordering on chiari malformation (.5 mm away on the coronal plane). One had a small lobulation (semi-bulbous projection) of the anterior pituitary gland, superior and anterior to the infundibular stalk. Conclusion Prevalence of brain abnormalities in children with short stature who are to undergo GHT is significant and warrants MRI evaluations in these subjects. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m., Monday, June 13, 2022 12:58 p.m. - 1:03 p.m.

RF13 | PMON83 Culture Negative Pituitary Abscesses: A Case Series

Abstract Introduction Pituitary abscesses are rare, representing less than 1% of pituitary lesions. Patients may present with pituitary hypofunction, headache, and/or vision changes, often without systemic or biochemical signs of infection, with diagnosis only made intraoperatively. Cases A 74-year-old man with history of diabetes and coronary artery disease presented with headache and left-eye ptosis. MRI revealed a 1.8 cm hypoenhancing cystic, hemorrhagic sellar mass extending into the left cavernous sinus. Pre-operative labs showed AM cortisol 1.1 ug/dL, testosterone 43 ng/dL (reference range 250-1100), FSH 15.3 mIU/mL, LH 4.5 mIU/mL consistent with adrenal insufficiency and hypogonadism. He underwent transsphenoidal pituitary resection (TSR) during which frank pus was demonstrated after incision into the dura overlying the pituitary. Surgical pathology showed necrosis and granulation-type reaction with acute inflammatory cell infiltrates. Gram stain and methenamine silver stains did not reveal definite micro-organisms while tissue bacterial cultures showed normal skin flora. He remained hemodynamically stable without fevers throughout the hospitalization and was discharged on empiric antibiotics and hydrocortisone. A 74-year-old female with history of a pituitary macroadenoma was admitted for worsening headaches. Labs were consistent with her known history of secondary adrenal insufficiency and hypogonadotropic hypogonadism. Repeat MRI showed characteristics suggestive of hemorrhage within a 1.8 cm intrasellar mass with inhomogeneous and peripheral enhancement after contrast. She underwent TSR and intraoperatively frank pus was encountered. Surgical pathology showed sinonasal mucosa with chronic inflammation. There was no growth in aerobic, fungal, or AFB cultures; anaerobic cultures grew rare commensal bacteria. She was discharged with a course of antibiotics and remains on hydrocortisone. A 42-year-old male presented with headache and was found to have a 2.5 cm sellar mass with rim enhancement elevating and compressing the optic chiasm on MRI. Labs were significant for AM cortisol 4.9 ug/dL and testosterone 8 ng/dL, FSH 0.4 mIU/mL, LH <0.2 mIU/mL consistent with adrenal insufficiency and hypogonadism. He underwent TSR and was found to have purulent debris in the sella. Pathology showed epithelial tissue fragments insufficient for diagnosis, and cultures grew few skin and oropharyngeal contaminants. He was discharged on hydrocortisone with a course of antibiotics. Conclusion We describe three patient cases presenting with headache and varying degrees of hypopituitarism, found to have purulent material emanating from the sella during transsphenoidal resection. None presented with clinical or biochemical systemic signs of infection. MRI imaging was variable, ranging from cystic, hemorrhagic features to peripheral enhancement. Surgical pathology and cultures were unrevealing for a definitive infectious source or other cystic lesion such as Rathke's cleft cyst. This case series demonstrates the potential nonspecific presentation of pituitary abscesses including negative cultures and pathology. It is important to keep this rare diagnosis in the differential for patients presenting with pituitary disease. Presentation: Sunday, June 12, 2022 12:30 p.m. - 12:35 p.m., Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PMON47 Fluid restriction reduces hyponatremia and hospital readmission rates following pituitary surgery

Abstract Rationale: Hyponatremia occurs in approximately 20% of patients after transsphenoidal pituitary surgery (TSS). Symptomatic delayed hyponatremia from SIADH is a major cause of morbidity and hospital readmission. Prophylactic fluid restriction (FR) protocols starting 2-5 days after surgery may potentially reduce rates of symptomatic hyponatremia and readmissions. Methods Patients who underwent TSS for sellar and parasellar masses at a tertiary referral center were discharged with a one-liter fluid restriction protocol. Serum sodium levels were measured on post-operative day (POD) 7 with telephone follow-up care. Rates of hyponatremia and readmissions were compared to a cohort of 316 post-operative patients treated prior to the protocol. Numerical variables were summarized as mean ± SD and significance testing calculated by Chi-square and t-test. Results 105 patients were enrolled on the FR protocol upon discharge. Twelve patients were excluded as they had diabetes insipidus or prolonged hospitalization beyond POD 7. Ninety-three patients were included in the analysis, of whom 74.3% had pituitary adenomas, 5.4% Rathke's Cleft cysts, 8.6% meningiomas, 5.4% apoplexy, 2.2% craniopharyngiomas, and 4.3% other masses. Hyponatremia occurred in 13/93 (14%) patients. Mean POD 7 sodium was 137.9 mmol/L across the entire cohort. Two patients (2.1%) were readmitted for hyponatremia, while 3 were re-admitted for other causes. 88/93 (94.6%) of patients complied with the FR protocol. Of the 5 non-compliant patients, 2 developed hyponatremias (40%) compared to 3/88 (3.4%) in those who complied (p=0.008). Mean POD7 sodium was 138 (+/-4.5) mmol/L in those that complied and 135 (+/- 4.7) mmol/L in those who did not. Overall readmission rate for those with postoperative hyponatremia was 23% compared to 2.5% for those with normal sodium levels (p=0.0023). 15.4% of patients with hyponatremia were re-admitted for hyponatremia treatment, compared to 0/80 (0%) of those who did not develop post-operative hyponatremia (p=0.0004). Age and BMI did not impact hyponatremia rates. In 316 patients treated in 2012-2018 prior to the FR protocol, 78 (24.7%) developed delayed hyponatremia with 6% readmitted for hyponatremia. Thirty nine percent of patients with delayed hyponatremia were re-admitted, compared to 7.6% for those without hyponatremia. Compared to patients not on FR, patients on the FR protocol had 50% reduced risk of hyponatremia (OR=0.49 (95% CI 0.26-0.94, p=0.03), and a 3-fold reduced risk of overall readmissions (OR=0.31; 95% CI 0.12-0.81, p=0.0157) and readmission for hyponatremia (OR= 0.34;95% CI 0.08-1.5, p=0.16). A post-operative CSF leak or use of a lumbar drain were associated with an increased readmission rate in the historical cohort. Conclusion Instituting a one-liter daily FR protocol in patients after TSS results in significantly reduced rates of hyponatremia, overall readmissions, and readmission for symptomatic hyponatremia. A FR protocol should become routine practice in the post-operative care of patients undergoing pituitary surgery. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PSAT043 Liraglutide Ameliorates Symptoms of Cushing Syndrome in a Pediatric Patient

Abstract Background Cushing Syndrome (CS) is a rare disease characterized by hypercortisolism. Pituitary adenomas resulting in ACTH-dependent disease may be difficult to visualize radiologically, resulting in a prolonged diagnostic course and delay in definitive treatment. Adjuvant medical treatment with pharmacologic agents, including steroidogenesis inhibitors and adrenolytics, can be utilized for symptom management. However, hypercortisolemia may be refractory to medical therapy making it necessary to consider symptomatic treatments. We present a case of a pediatric patient with Cushing syndrome in whom treatment with liraglutide, a GLP-1 agonist approved for the treatment of hyperglycemia and obesity, resulted in improvement of symptoms of hypercortisolemia. Case A 13 year old Caucasian female presented with a 2 year history of linear growth arrest, accelerated weight gain and delayed puberty. Her physical examination was remarkable for overt signs of hypercortisolemia. Initial laboratory evaluation showed normal thyroid function, hyperglycemia with HbA1c 7.2% (normal < 5.7%), dyslipidemia and elevated midnight salivary cortisol 0.898 (normal < 0.112 mcg/mL). Hypercortisolemia was confirmed when morning serum cortisol was 25.6 mcg/dL after 1 mg overnight dexamethasone suppression test (DST). This along with an elevated morning ACTH level, 72.8 (7.2- 63.3 pg/mL), confirmed a diagnosis of ACTH-dependent Cushing syndrome. Magnetic Resonance Imaging of the pituitary gland with contrast showed a 4 mm non- enhancing lesion consistent with a Rathke's cleft cyst which was unlikely to be the source of excess ACTH. Results from high dose (8 mg overnight) DST, Corticotropin Releasing Hormone (CRH) stimulation test, and Inferior Petrosal Sinus Sampling for ACTH post CRH stimulation were partially inconsistent with pituitary disease. Further workup for ectopic source(s) of ACTH showed normal imaging of the neck, chest, abdomen and pelvis. A DOTATATE PET scan failed to identify an ACTH source. Adrenolytic adjuvant treatment with mitotane was started but was discontinued due to intolerable gastrointestinal effects. Treatment was changed to ketoconazole, however, hypercortisolemia remained refractory to treatment as evidenced by continued elevation of 24- hour urine free cortisol levels. Metformin was initiated for hyperglycemia treatment but was not tolerated due to persistent nausea and vomiting. The patient was switched to liraglutide and within 3 months she had 10% weight loss, normalization of fasting blood glucose levels and reduction of HbA1c to 5.7%. Additionally, the patient's energy level and baseline hidradenitis suppurativa improved. Conclusion In some pediatric patients with CS, definitive treatment of hypercortisolemia may be delayed or not feasible due to a lack of biochemical or radiological evidence showing the specific etiology for CS. Medical treatment with pharmacologic agents can be utilized for symptom management, however, hypercortisolemia may persist and even be recalcitrant to therapy. In this setting, clinicians should consider using liraglutide to ameliorate some of the cardinal symptoms of CS in pediatric patients. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Surgical Management and Long-Term Results of Rathke's Cleft Cyst.

Rathke's cleft cysts (RCCs) are nonneoplastic cysts. Most of them are asymptomatic and stable; when symptomatic, RCCs are surgically fenestrated and drained. However, the outcomes remain unclear. The authors evaluated the outcomes of RCC decompression. Between 2004 and 2019, 32 RCCs were decompressed in a single tertiary institution. The clinical characteristics, intraoperative findings, postoperative complications, and endocrinological and surgical outcomes were retrospectively reviewed. Patients who underwent sequential imaging at least twice and at least 12 months after surgery were included in the analysis. Patients' mean age was 40.8±14.9 years, and 62.5% were women. The mean follow-up duration was 62.3±48.6 months. In 21 patients (65.6%), no residual cysts were identified on postoperative magnetic resonance imaging. Of the 18 patients with preoperative visual field defects, 17 (94.4%) experienced postoperative visual improvement. Postoperative complications included endocrinological deterioration in 11 patients (34.4%), permanent diabetes insipidus in 11 (34.4%), infection in four (12.5%), intrasellar hemorrhage in three (9.4%), and cerebrospinal fluid leak in two (6.3%). Follow-up images revealed cyst recurrence in nine patients (28.1%), an average of 20.4 months after surgery; in three patients, the cysts were symptomatic, and resection was repeated. Multivariable analysis revealed that postoperative endocrinological deterioration was the only independent factor associated with cyst recurrence (p=0.028; hazard ratio, 6.800). Our findings showed that although only cyst fenestration for decompression was performed to preserve pituitary function, more pituitary dysfunction occurred than expected. Besides, the postoperative hormonal deterioration itself acted as a risk factor for cyst recurrence. In conclusion, surgery for RCC should be more careful.

Pediatric Endoscopic Endonasal Skull Base Surgery: A Retrospective Review Over 11 Years

To show the safety and efficacy of the endoscopic endonasal approach (EEA) for skull base surgery in pediatric patients through descriptive analysis of cases over an 11-year period. The study comprised 94 patients undergoing EEA for skull base surgery, between January 2007 and June 2018, at 2 tertiary pediatric hospitals. Descriptive statistics are presented regarding the presentation, intraoperative details, and complications. Over the study period, 130 surgeries were performed in 94 patients: 94 primary surgeries and 36 reoperations. The mean patient age was 13.8 years and 48.9% of patients were female. Presenting signs/symptoms included endocrinopathies (56.4%), vision abnormalities (37.2%), and cranial nerve deficits (20.2%). EEA alone was used in 95.7% of primary surgeries and 91.7% of reoperations. Diseases treated included craniopharyngioma (18.1%), pituitary adenoma (17.0%), Rathke cleft cyst (13.8%), chordoma (9.6%), osteosarcoma (5.3%), juvenile nasopharyngeal angiofibroma (4.3%), skull base fracture (4.3%), and encephalocele (3.2%). A lumbar drain was used in 20.2% of primary surgeries and 25% of reoperations. A nasoseptal flap was used in 36.2% of primary surgeries and 25% of reoperations. Postoperative complications included cerebrospinal fluid leak (12.8%), sinusitis (7.4%), bacterial meningitis (3.2%), and carotid artery injury in 1 reoperation. EEA for anterior cranial base disease is safe and efficacious in pediatric patients and can be used to treat many of the rare and heterogenous diseases that arise in this anatomic location. Management strategies and rates of sinonasal and intracranial complications including cerebrospinal fluid leak rate are similar to those reported in adult cohorts.

Nonneoplastic and noninfective cysts of the central nervous system: A histopathological study.

Nonneoplastic epithelial cysts involving the central nervous system are diverse and are predominantly developmental in origin. This study represents a surgical series describing the histopathological features of 507 such epithelial cysts with clinical and imaging correlation. Age at surgery ranged from 7 months to 72 years (mean: 33 years) affecting 246 male and 261 female patients. Colloid cyst was the most frequently resected cyst, followed by epidermoid cyst, arachnoid cyst, Rathke cleft cyst, dermoid cyst, neurenteric cyst, Tarlov cyst, and choroid plexus cyst. Diagnosis was based on the location of the cysts and the nature of the lining epithelium. Rathke cleft cyst showed the highest propensity for squamous metaplasia, significant inflammation, and xanthogranulomatous reaction. Ulceration of lining epithelium and calcification were most frequent in dermoid cyst. Radiopathological concordance was maximal for colloid cyst, followed by epidermoid and arachnoid cysts. Epidermoid and dermoid cysts exhibited the highest propensity for local tumor progression, followed by Rathke cleft cyst.

Stereotactic radiosurgery for Rathke's cleft cysts: an international multicenter study.

Rathke's cleft cysts (RCCs) are sellar collections from an incompletely regressed Rathke's pouch. Common symptoms of RCCs can include headaches, visual loss, and endocrinopathy. Surgery is required in some cases of symptomatic or growing RCCs. Recurrence after surgery is common (range 10%-40%). Stereotactic radiosurgery (SRS) has been used in an attempt to control growth and symptoms, but outcomes are not well known. The authors sought to study the outcomes of RCCs following Gamma Knife surgery for both salvage and initial treatment. The outcomes of 25 patients with RCCs who underwent SRS between 2001 and 2020 were reviewed. Four patients received initial SRS and 21 were treated with salvage SRS. Diagnosis was based on imaging or histopathology. Cyst control was defined as stability or regression of the cyst. Kaplan-Meier analysis was used to determine time to recurrence and determine potential factors for recurrence. The respective median clinical follow-up and margin dose were 6.5 years and 12 Gy. Overall control was achieved in 19 (76%) of 25 patients, and 4 recurrences required further intervention. The average time to recurrence was 35.6 months in those RCCs that recurred. Visual recovery occurred in 14 (93.3%) of 15 patients and no new post-SRS visual deficits occurred. The presence of a pretreatment visual deficit was often an indicator of RCC regrowth. All 3 patients with pretreatment hyperprolactinemia experienced resolution after SRS. New endocrinopathy related to SRS was noted in 5 (20%) of 25 patients, all of which were thyroid and/or cortisol axis related. Upfront SRS was used in 4 patients. No new endocrinopathies or visual deficits developed after upfront SRS, and the 1 patient with a pretreatment visual deficit recovered. One of the 4 patients with upfront SRS experienced a recurrence after 7.5 years. SRS produced effective recovery of visual deficits and carries a low risk for new visual deficits. Cyst control was achieved in approximately three-fourths of the patients. Following SRS, patients without pretreatment visual deficits are less likely to have RCC regrowth. Endocrinopathy can occur after SRS, similar to other sellar mass lesions. Initial SRS shows the potential for long-term cyst control, with improvement of symptoms and a low risk for complications.

Predictive factors for delayed hyponatremia after transsphenoidal surgery in patients with Rathke's cleft cysts.

PurposeWe aimed to assess factors influencing the occurrence of delayed hyponatremia after transsphenoidal surgery in patients with Rathke’s cleft cysts (RCCs).MethodsWe retrospectively collected the clinical data of patients who underwent transsphenoidal surgery for RCCs from January 2014 to January 2022. Univariate and multivariate analyses were used to determine the factors influencing the occurrence of postoperative delayed hyponatremia.ResultsOf the 78 microscopic transsphenoidal surgery recipients with RCCs, 15 experienced postoperative delayed hyponatremia. There were 35 men and 43 women, and mean age was 43.75 ± 14.95 years. The clinical manifestations of RCCs were headache (62 cases, 79.5%), visual dysfunction (35 cases, 44.9%), endocrine dysfunction symptoms (12 cases, 15.4%). After transsphenoidal surgery, 93.5% (58/62) had improvements in headache, and 97.1% (34/35) had improved or resolved compressive visual symptoms. Delayed hyponatremia occurred on average on day 6.46 and lasted on average for 4.40 days. Logistic regression analysis showed that the independent influencing factor of delayed hyponatremia after transsphenoidal surgery in patients with RCCs was postoperative diaphragma sellae height.ConclusionPostoperative diaphragma sellae height was identified as an independent influencing factor for delayed hyponatremia after transsphenoidal surgery in patients with RCCs.

Exploring the Impact of Using Patient-Specific 3D Prints during Consent for Skull Base Neurosurgery.

Objectives Informed consent is fundamental to good practice. We hypothesized that a personalized three-dimensional (3D)-printed model of skull base pathology would enhance informed consent and reduce patient anxiety. Design Digital images and communication in medicine (DICOM) files were 3D printed. After a standard pre-surgery consent clinic, patients completed part one of a two-part structured questionnaire. They then interacted with their personalized 3D printed model and completed part two. This explored their perceived involvement in decision-making, anxiety, concerns and also their understanding of lesion location and surgical risks. Descriptive statistics were used to report responses and text classification tools were used to analyze free text responses. Setting and Participants In total,14 patients undergoing elective skull base surgery (with pathologies including skull base meningioma, craniopharyngioma, pituitary adenoma, Rathke cleft cyst, and olfactory neuroblastoma) were prospectively identified at a single unit. Results After 3D model exposure, there was a net trend toward reduced patient-reported anxiety and enhanced patient-perceived involvement in treatment. Thirteen of 14 patients (93%) felt better about their operation and 13/14 patients (93%) thought all patients should have access to personalized 3D models. After exposure, there was a net trend toward improved patient-reported understanding of surgical risks, lesion location, and extent of feeling informed. Thirteen of 14 patients (93%) felt the model helped them understand the surgical anatomy better. Analysis of free text responses to the model found mixed sentiment: 47% positive, 35% neutral, and 18% negative. Conclusion In the context of skull base neurosurgery, personalized 3D-printed models of skull base pathology can inform the surgical consent process, impacting the levels of patient understanding and anxiety.

An Overview of Pituitary Incidentalomas: Diagnosis, Clinical Features, and Management.

Simple SummaryA pituitary incidentaloma is a pituitary tumor or mass that is incidentally discovered in imaging studies which have been performed for reasons other than the symptoms of pituitary lesions. The majority of pituitary incidentalomas are pituitary neuroendocrine tumors (PitNETs) and Rathke cleft cysts. PitNETs have received attention because of their distinction from pituitary adenoma in the new World Health Organization (WHO) classification. The natural history of PitNETs is partially known, and the management of pituitary incidentalomas has been determined based on this history; however, the pathology of PitNETs has significantly changed with the new WHO classification, and studies with a high level of evidence are required to consider treatment guidelines for pituitary incidentalomas.Pituitary incidentalomas are tumors or mass lesions of the pituitary gland. These are incidentally discovered during imaging studies for symptoms that are not causally related to pituitary diseases. The most common symptom that triggers an examination is headache, and the most common type of pituitary incidentalomas are pituitary neuroendocrine tumors (PitNETs) and Rathke cleft cysts. The existing treatment strategy is controversial; however, surgical resection is recommended in cases of clinically non-functioning PitNETs with optic chiasm compression. In contrast, cystic lesions, such as Rathke cleft cysts, should be followed if the patients are asymptomatic. In this case, MRI and pituitary function tests are recommended every six months to one year; if there is no change, the follow-up period should be extended. The natural history of PitNET is partially known, and the management of pituitary incidentalomas is determined by this history. However, the pathogenesis of PitNET has significantly changed with the new World Health Organization classification, and follow-up is important based on this new classification. Therefore, a high level of evidence-based research is needed to consider treatment guidelines for pituitary incidentalomas in the future.

Reconstruction with Mucosal Graft Reduces Recurrence After Endoscopic Surgery of Rathke Cleft Cyst

Rathke cleft cysts (RCCs) arise from the development of the Rathke pouch. Recurrence is common after either drainage or cyst removal. The endoscopic endonasal approach (EEA) is increasingly utilized for the management of RCC. Various techniques have been described to try to reduce the rates of recurrence. We studied the effect of fenestration with a nasoseptal flap (NSF) on recurrence rates by comparing a cohort of patients undergoing this technique to a cohort of patients undergoing conventional drainage. Patients who underwent EEA for RCC between 2011 and 2020 were identified and divided into 2 cohorts: conventional fenestration versus fenestration with NSF. Surgical approach, reconstructive method, and recurrences were recorded. Primary end point was symptomatic or radiographic recurrence. 21 patients were identified undergoing EEA. An NSF was used to line the cyst cavity in 11 cases. Conventional fenestration without mucosal reconstruction was performed in the remaining 10 cases. In the cases without NSF, 5 (50%) developed recurrence requiring revision surgery, while there was only one recurrence in the NSF group (P < 0.05). In patients requiring revision, all had an NSF placed and none had a second recurrence of their RCC. NSF placement into a fenestrated RCC is useful to prevent cyst reaccumulation and reoperation. Typical fenestration carries an unacceptably high rate of recurrence.

Rathke's cleft cysts: A single-center case series.

Background: Rathke’s cleft cysts (RCCs) are common benign sellar or suprasellar lesions. The aim of this study is to report our experience on the management of 27 RCC cases.Methods: We retrospectively analyzed a series of 27 patients with symptomatic RCC who were referred to our department between January 2016 and January 2020. Data regarding patients’ demographics, clinical evaluations, laboratory and neuroimaging findings, pathologic records, surgical treatment, and complications were extracted from our electronic database. All patients underwent RCC removal through a direct endoscopic endonasal transsphenoidal (EETS) approach, except for two cases.Results: Data of 27 patients (3 men and 24 women; mean age: 38 years) with symptomatic RCC were reviewed. The most common presenting symptom was headache, occurring in 20 (74.0%) patients. In 16 (59.2%) cases, the tumor was primarily located in the sella turcica. Nine (33.3%) cases exhibited a secondary suprasellar extension.Conclusion: Our experience with RCC patients showed that EETS is a safe method of treatment, with minimal recurrence.