Cleft Cyst Research Articles

THU104 An Atypical Case Of A Suprasellar Cyst: A Diagnostic Challenge

Abstract Disclosure: A. Chen: None. S. Zachariah: None. B. Wong: None. E. Owusu: None. A. Levitt: None. R. Todor: None. P. Kishore: None. Background: Cystic sellar masses may be challenging to diagnose on a clinical, imaging, and histopathological basis due to overlapping features. We report a case of a cystic sellar mass in which traditional imaging findings did not correspond to its histopathological findings. Clinical Case: A 37-year-old female presented with headaches and bilateral blurry vision for two months with acute worsening of left peripheral vision for one week. She denied irregular periods, hirsutism, or breast discharge. Ophthalmology evaluation found left temporal field vision loss but normal maculae/optic nerves. MRI pituitary exhibited 2.3 x 1.5 x 1.7 cm complex cystic suprasellar mass with partial rim enhancement, thought to represent a cystic macroadenoma. Initial tests were notable for negative HCG, prolactin 96.32 mIU/mL (n < 23.3 mIU/mL), TSH 9.440 uIU/mL (0.270-4.2 uIU/mL), normalized to 0.855 uIU/mL on repeat testing, FT4 0.748 ng/dL (n = 0.75 – 2.0 ng/dL). IGF-1, ACTH, AM cortisol, FSH, and LH were within normal limits. She was started on cabergoline prior to trans-nasal-sphenoid resection with cyst drainage of white and thick content. Histopathology exhibited a squamous epithelium-lined cyst with no columnar epithelium or mucin-containing cells, consistent with an epidermoid cyst (EC), although the differential included a Rathke’s cleft cyst (RCC). No pituitary tumor was noted. A re-evaluation of MRI in multidisciplinary rounds deemed epidermoid cyst unlikely, given that these present with restricted diffusion. Our patient had overall T2 hyperintensity with an associated T2 hypointense nodule and did not restrict diffusion [1]. After interdisciplinary discussion, including imaging and pathology review, it was felt that the most likely diagnosis was RCC with squamous metaplasia. After surgery, the patient's symptoms and visual function completely recovered, and follow-up imaging is scheduled. Conclusion: Differentiating RCC with squamous metaplasia from EC can be challenging since both may present with a squamous epithelial lining [2]. Rates of progression and recurrence vary amongst cysts; therefore, making an accurate diagnosis is essential for selecting management, predicting the likelihood of recurrence, and guiding postoperative surveillance. This case is unique in that radiologic features were more helpful than histopathology in determining the diagnosis and highlights the importance of collectively considering clinical, imaging, and histopathological features in making a final diagnosis.

THU627 Fertility Outcomes And Anti Mullerian Hormone Levels In Women With Hypopituitarism

Abstract Disclosure: V.W. Fitz: None. C.O. Sailer: None. I. Remba-Shapiro: None. N. Bomani Gonzalez: None. W. Pierre: None. C. Bormann: None. I. Souter: None. L.B. Nachtigall: None. Background: Disruption of the hypothalamic-pituitary-gonadal (HPG) axis due to pituitary gland disorders presents a challenge for patients who desire conception. Women with hypopituitarism often require assisted reproductive technology (ART) to conceive. Anti-Mullerian Hormone (AMH) has been established as a marker of ovarian reserve in patients with normal HPG function, however, levels in women with hypopituitarism have not been reported. In this cohort, we investigate pre-conception AMH levels, reproductive treatments and fertility outcomes in women with hypopituitarism. Methods: We reviewed a large database and identified 19 patients with hypopituitarism who had undergone fertility treatment and 142 age-matched controls with male factor infertility. Clinical characteristics and AMH levels are shown using mean (SD) or median (IQR). AMH ratio using the lower limit of normal as denominator is used to normalize to the reference range as several assays with different reference ranges were used during the timeframe of data collection. Mann-Whitney U was used to compare AMH levels. A p value of <0.05 was considered statistically significant. Results: Patients with hypopituitarism and controls had a mean age of 33.4 years (2.5) and 33.3 years (2.2) p=0.857, at initial fertility evaluation, respectively. 17/19 (89.5%) patients in the hypopituitarism group experienced spontaneous menarche and had a post-pubertal onset of hypopituitarism. Hormonal deficiencies included GH (9), ACTH (8), TSH (10), FSH/LH (13), and vasopressin (3). Causes of hypopituitarism included 8 pituitary adenomas (7 functioning and 1 non-functioning), 3 Rathke’s cleft cysts, 4 intracranial neoplasms, 2 congenital or idiopathic hypopituitarism and 1 intracranial trauma. Median AMH level was 2.60 ng/mL (0.90-3.50) vs. 3.48ng/mL (1.99-4.70) p=0.017, and AMH ratio was 3.11ng/mL (1.13-4.17) vs. 4.11ng/mL (2.60-6.25) p=0.070, in the hypopituitary group and in controls, respectively. Among the 19 patients with hypopituitarism, 13 underwent ovulation induction (OI) with intrauterine insemination (IUI) or timed intercourse (TIC), 12 underwent in vitro fertilization (IVF), and 6 were treated with both OI and IVF. 18/19 (94.7%) conceived and 16/19 (84.2%) had a livebirth. Four patients (21%) conceived spontaneously. Among the patients who underwent OI, 4/13 (30.8%) had ≥1 livebirth with a median of 2 cycles (range 1-4) until first birth. Among the patients who underwent IVF, 10/12 (83.3%) had ≥ 1 livebirth with a median of 1.50 cycles (range 1-4) until first birth. Conclusions/Impact: Patients with hypopituitarism conceive through a variety of assisted reproductive technologies and may conceive spontaneously. Overall, AMH levels are comparable to age matched controls. Reproductive interventions among women with hypopituitarism are successful, with most patients achieving at least one livebirth. Presentation: Thursday, June 15, 2023

THU192 Prevalence Of Brain Abnormalities In Children With Central Precocious Puberty

Abstract Disclosure: A.A. Bastos: None. A.P. Machado Canton: None. L.B. Macena: None. L.F. Pedrosa: None. B.B. Mendonca: None. V.N. Brito: None. A. Latronico: None. Introduction: Brain magnetic resonance imaging (MRI) is the main tool used to investigate the etiology of central precocious puberty (CPP) in children. A systematic review study showed an overall prevalence of 9% of brain lesions in girls with CPP. Aim: To assess the frequency brain lesions in children with CPP. Patients and Methods: In this retrospective study, we recruited data of 163 brain MRI of children with CPP (140 girls,86%; and 23 boys, 14%) followed in a pediatric endocrinology unit from 1990 to 2022. Brain lesions were classified as congenital or acquired, and causative of CPP or incidental. Results: In girls, normal brain MRI was found in 82/140 (59%). Abnormal brain MRI was identified in the remaining 58 girls (41%), with 26 presenting congenital lesions causative of CPP (11 hypothalamic hamartoma, 7 hydrocephalus, 3 optic pathway gliomas, 2 corpus callosum agenesia, 1 pinealoma, 1 tuberous sclerosis, 1 choroid plexus papilloma), and 32 with incidental findings (microadenomas, pituitary stalk deviation, pituitary hyperplasia, rathke cleft cyst, hippocampal alterations). The overall frequency of incidental findings was 20.8%. In addition, the frequency of brain MRI pathological abnormalities in girls older than 6 years without neuro ophthalmological symptoms was 12%(n=7). In boys, normal brain MRI was found in 6/23 (26%). Abnormal brain MRI was identified in the remaining 17 boys, with 14 presenting congenital lesions causative of CPP (9 hypothalamic hamartoma, 4 hydrocephalus, 1 pineal teratoma) and 1 with acquired lesions (1 pilocytic astrocytoma). Incidental findings were detected in only 2 brain MRI (1 pineal cyst and 1 microadenoma). Incidentalomes were more frequent in girls and lesions that can be associated with CPP were more common in boys. Conclusion: The frequency of pathological brain lesions was higher in CPP boys. Hypothalamic hamartoma was the most frequent congenital lesion identified in both sexes. Based on the frequency of pathological brain alterations in girls older than 6 years we recommend to perform brain MRI in all CPP girls. Presentation: Thursday, June 15, 2023

MR Imaging Appearance of Ruptured Rathke Cleft Cyst and Associated Bone Marrow Enhancement.

Rathke cleft cysts are common cystic pituitary lesions seen on MR imaging. A subset of Rathke cleft cysts can rupture within the sella and are uncommon. The imaging appearance of a ruptured Rathke cleft cyst has been previously described with nonspecific imaging findings. We present 7 cases of ruptured Rathke cleft cysts and basisphenoid bone marrow enhancement below the sella that could be used to potentially distinguish a ruptured Rathke cleft cyst from other cystic lesions.

THU088 Perioperative Management Of Patients With Pituitary Tumors Undergoing Surgery: A Retrospective Review

Abstract Disclosure: J.T. Batch: None. H. Belal: None. G.Y. Gandhi: None. Background: Pituitary tumors account for 10% of brain neoplasms with transsphenoidal resection of these tumors responsible for 20% of all intracranial operations performed for primary brain tumors. Hypothesis: We suspected significant heterogeneity and lack of standardized protocols in the perioperative endocrine evaluation and management of patients undergoing resection of sellar masses. Methods: From June 2016 through November 2022, we identified 46 adult patients (23 females, mean age 52 years, age range 25 to 84 years) who underwent pituitary mass resection (44 transsphenoidal and two transcranial approaches) at our institution. We abstracted information about perioperative endocrine evaluation from their medical records. Results: The vast majority (32 patients) had nonfunctioning pituitary macroadenomas. Other indications for surgery included Cushing’s disease (3), prolactinomas (2), acromegaly (3), pituitary metastasis (1), gonadotroph adenomas (2), Rathke cleft cyst (2), and epidermoid cyst (1). Preoperative cortisol level was not measured in 8 patients. The decision to administer intraoperative corticosteroids was not consistently based on preoperative cortisol levels. 24 patients had cortisol levels <11 mcg/dL; 22 received intraoperative corticosteroids at anesthesia induction. 13 patients had cortisol levels ≥11 mcg/dL, and 11 received intraoperative steroids. Two patients had very large pituitary masses (over 4 cm), and one received intraoperative corticosteroids. Of the two patients with Cushing’s disease, one received intraoperative corticosteroids. Cortisol level was checked in 27 patients in the first four weeks after surgery; in the rest, it was measured between 2 months and three years after surgery. All five patients with confirmed adrenal insufficiency with cortisol levels <3 mcg/dL were treated with corticosteroids. 22 patients had cortisol levels in the equivocal zone (3 to 15 mcg/dL), and 16 were treated with corticosteroids. Nine patients ultimately did not have a follow-up in endocrinology. The long-term permanence of the diagnosis of diabetes insipidus could not be established due to a lack of uniform follow-up testing. Conclusions: Patients with pituitary gland tumors represent a heterogeneous yet commonly encountered neurosurgical population. Successful surgical management is critically dependent on the quality of perioperative care. Understanding preoperative evaluation, intraoperative management, potential complications, their management, and strategies for avoidance are fundamental to reduce morbidity and mortality. High-quality evidence guiding perioperative practices in this population is needed. We have used the information gathered in this study to create a multidisciplinary perioperative protocol with expertise from endocrinologists, neurosurgeons, neuro-ophthalmologists, and neuroradiologists. Presentation: Thursday, June 15, 2023

Brain magnetic resonance imaging (MRI) findings in central precocious puberty patients: is routine MRI necessary for newly diagnosed patients?

The overall incidence of central precocious puberty (CPP) has increased in recent decades, and brain magnetic resonance imaging (MRI) evaluations are recommended in cases of suspected brain lesions. This study aimed to investigate the prevalence of MRI abnormalities and to evaluate the need for routine brain MRI in patients with newly diagnosed CPP. This retrospective study reviewed the data of patients newly diagnosed with CPP who underwent routine pituitary MRI at Korea University Anam Hospital from March 2020 to September 2021. A total of 199 girls and 24 boys was enrolled in this study. Positive MRI findings were categorized as abnormal pituitary, nonpituitary incidental, and pathological. In addition, we investigated the incidence of MRI abnormalities and evaluated their associations with clinical and biochemical factors. Positive brain MRI findings were observed in 84 patients (37.7%). Pituitary abnormalities were found in 54 patients (24.2%), with Rathke cleft cysts being the most common (16.1%). Incidental nonpituitary findings were observed in 29 patients (13.0%), while a pathological brain lesion (diagnosed as hypothalamic hamartoma) was observed in only 1 female patient (0.4%). No significant differences in sex or age were found in incidence of pituitary abnormalities or nonpituitary incidental findings. Compared with headache controls, significant associations were observed between abnormal pituitary findings on MRI and CPP (unadjusted odds ratio, 3.979; 95% confidence interval, 1.726-9.173). True pathological findings were rare, even though the prevalence of abnormalities on pituitary MRI in patients with CPP was relatively high. Considering its cost-effectiveness, MRI screenings should be carefully considered in patients with CPP.

Rathke’s cleft cyst discovered with ruptured anterior communicating artery aneurysm: a case report

IntroductionRathke’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 presentationA 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.ConclusionsRare 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.

Ultrasound diagnosis and treatment of branchial cleft cyst and preoperative management.

The ultrasonic diagnosis of cervical and facial cystic masses, as well as cases of missed diagnosis and misdiagnosis, was examined, to improve the diagnosis of branchial cleft anomalies. A retrospective analysis was conducted on 17 patients with branchial cleft cyst anomalies, including 11 males and 6 females, aged 12-53years, with an average age of 33 ± 2years, were unilateral single. All patients who underwent an ultrasound examination and image storage for retrospective analysis, and both longitudinal and transverse sections were scanned to observe the shape, size, boundary, peripheral relationship, and blood flow signal of the masses. All cases were examined with an enhanced CT scan, and pathological reports were generated. Among the 17 cases of branchial cleft anomalies, 15 cases were branchial cleft cysts, while one case involved fistula formation and one case involved sinus tract formation. Based on the type of branchial cleft, the first, second, and third cysts were classified in 4, 12, and 1 case, respectively. The sensitivity rate and specificity of ultrasonic diagnosis were 14/17 (82.4%) and 4/6 (66.7%), respectively. Ultrasonic characteristic analysis for the masses can be found in simple cystic masses or hypoechoic masses, most of them are of a regular shape and have a distinct boundary, and almost no blood flow signal. All patients who were misdiagnosed exhibited blood flow signals, including 1 patient with an abundant blood flow signal, 1 patient suspected of having ectopic thyroid with an abnormal function due to the rat-tail sign, 2 patients misdiagnosed as local inflammatory focus, and 1 patient misdiagnosed with tuberculous lymphadenitis. Ultrasound has a detection rate of up to 100% for cervical and facial masses, providing a fundamental determination of lesion characteristics and specific guidance for preoperative diagnosis. If the blood flow signals can be identified and carefully considered their peripheral relationship, the diagnostic rate can be improved.

Surgical indication of pediatric Rathke's cleft cyst based on a 20-year retrospective cohort.

Rathke's cleft cyst (RCC) is the most commonly encountered pituitary incidentaloma in children. Because RCC is not frequently diagnosed in children, there are few reports on pediatric RCCs. The natural course of the disease and appropriate treatments are still obscure. The present study aimed to elucidate the natural history and surgical indications of RCCs in children. The authors retrospectively reviewed the clinical presentations, imaging features, ophthalmological evaluations, endocrine evaluations, and surgical outcomes of pediatric RCCs at a single institution from January 2000 to October 2022. Clinical outcomes between the surgery and observation groups were compared. Among 93 patients, there were 41 patients in the surgery group and 52 patients in the observation group. The mean age at diagnosis was 10.9 years, and the mean follow-up period was 5.6 years. Headache fully or partially improved after surgery (86.2%), but the rate of improvement was not different from that of the observation group (70.0%). Ophthalmological abnormalities were effectively improved by surgical treatment (93.3%). Both the improvement and deterioration rates of endocrine abnormalities were significantly higher in the surgery group (p = 0.026 and p < 0.001, respectively), but the deterioration rate (43.9%) was higher than the improvement rate (14.6%). In the surgery group, the recurrence rate was 17.1% and the reoperation rate was 4.9%. Compared with total cyst wall resection, cyst fenestration with partial wall resection was associated with a higher recurrence rate (26.9%, p = 0.035) but a lower rate of endocrine abnormalities (30.8%, p = 0.049). Pediatric RCCs of ≥ 10 mm in size were analyzed. Ophthalmological abnormalities are the major surgical indications for pediatric RCCs. Headache and partial endocrine abnormalities may be improved with surgery, but they are not absolute indications for surgery. Cyst fenestration with partial wall resection via an endoscopic endonasal approach is the most recommended surgical method. Follow-up is essential to monitor for the occurrence of visual field defects and the recurrence of cysts.

A 15-year-old Girl with a Lateral Neck Mass Turning Out to Be Papillary Thyroid Carcinoma-Lateral Ectopic Papillary Thyroid Carcinoma or Lymph Node Metastasis?

Lateral neck lesions in children are common and involve various infectious or inflammatory etiologies as well as embryological remnants such as branchial cleft cysts. Although unusual, ectopic thyroid tissue can also present as a lateral neck mass. Here, we present an unusual case of a 15-year-old girl treated for an asymptomatic lateral neck mass that after surgical removal was found to be papillary thyroid carcinoma (PTC). However, after removal of the thyroid gland, no primary thyroid tumor was found. The question arose whether the lateral neck lesion was a lymph node metastasis without identifiable primary tumor (at histological evaluation) or rather malignant degeneration of ectopic thyroid tissue. Total thyroidectomy was performed with postoperative adjuvant radioactive iodine ablation. Even though PTC in a lateral neck mass without a primary thyroid tumor has been described previously, pediatric cases have not been reported. In this report we share our experience on diagnosis, treatment and follow-up, and review the existing literature.

Nuclear translocation of beta catenin in patients with Rathke cleft cysts—clinical and imaging characteristics and risk of recurrence

PurposeAlthough 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.MethodsPatients 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.ResultsSeventy-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).ConclusionTransnasal 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.

Clinical Pearls of Branchial Cleft Cyst Management in an Adult

INTRODUCTION: Second branchial cleft anomalies most commonly present as cysts followed by sinuses and fistulae. They have been classified into four different sub-types by Bailey. Type II is most common type where the branchial cleft cyst (BCC) lies anterior to the sternocleidomastoid muscle, posterior to the submandibular gland, adjacent and lateral to the carotid sheath. In this article, a case of type II second branchial cleft anomaly is presented. CASE REPORT: This article aims to portray how to evaluate a patient with second branchial cleft cyst focussing, focusing on how its diagnosed and its appropriate management. A young woman who had chief complaint of swelling of left side of the neck visited our outpatient department. She underwent complete excision of the lesion. There was no recurrence at 1year follow-up visit. DISCUSSION: Most branchial anomalies arise from the second branchial apparatus. Most second BCCs are located in the submandibular space. Patients with BCCs are usually older children or young adults. MR imaging provide the surgeon adequate preoperative information. Treatment for these lesions is complete surgical excision.

Incidence, risk factors, management and prevention of severe postoperative epistaxis after endoscopic endonasal transsphenoidal surgery: a single center experience.

Postoperative epistaxis is a very rare but severe complication after endoscopic endonasal transsphenoidal surgery (EETS) that can lead to catastrophic consequences. However, the incidence, risk factors, management and prevention of postoperative epistaxis remain unclear. Consecutive patients with pituitary adenoma (PA), Rathke's cleft cyst, craniopharyngioma, or clival chordoma who received EETS in our department between September 2020 and November 2022 were retrospectively analyzed. The incidence, risk factors, management and prevention of postoperative epistaxis were investigated and analyzed. A total of 557 consecutive patients who received EETS were included in this study. Eight patients (1.4%) (7 PAs and 1 Rathke's cleft cyst) experienced severe postoperative epistaxis. The size of the PAs was 9.6 mm-46.2 mm, with a median size of 22.1 mm. Epistaxis occurred 4 h to 30 days (median 14.5 days) postoperatively. Bleeding was stopped in 3 patients after nasal packing with iodoform gauze. The remaining 5 patients for whom nasal packing was insufficient were all sent to the operating room, and posterior nasal septal artery (PNSA) bleeding was identified and successfully treated with endoscopic bleeding artery electrocauterization under general anesthesia. In the EETS, all 8 patients had downward extension of the septal mucosal incision, in which 6 had intraoperative bleeding of PNSA that were cauterized by bipolar diathermy. Four patients had causative factors, including administration of antiplatelet agents, Valsalva-like manoeuvre, nose blowing and removal of nasal packing, respectively. No patients had recurrent epistaxis during the follow-up period. Post-EETS epistaxis is a rare but severe complication that could lead to catastrophic consequences, and one of the most common bleeding sources is the PNSA. Endoscopic bleeding artery electrocauterization under general anesthesia may be a safe, economic and effective measure for epistaxis refractory to nasal packing. Avoiding excessive downward extension of the septal mucosal incision could contribute to the prevention of postoperative epistaxis.

Rathke’s cleft cyst classification and outcomes after endoscopic endonasal approach

BackgroundRathke’s cleft cyst is rare, with variable characteristics and no unified categorization system. This study aimed to evaluate long-term outcomes, based on different categorizations, of symptomatic Rathke’s cleft cysts treated with endoscopic endonasal approach. MethodsThis retrospective study of 38 patients with symptomatic Rathke’s cleft cyst treated with endoscopic endonasal approach from 2006/06–2021/08 recorded pre- and post-operative clinical presentation, endocrine and visual tests, radiological findings, and resection status. Rathke’s cleft cysts were categorized by both cyst consistency and radiological features and clinical characteristics were analyzed. ResultsThe most common pre-operative symptoms were visual field deficit (65.8%) and hypopituitarism (39.5 %). Visual field deficit improved in 84% of affected patients, and hyperprolactinemia improved in 80% of affected patients. Pre-operative hypothyroidism and hypogonadism were associated with radiological type 3 cysts, while headache was more common in type B and C. Type 3 cysts were also associated post-operative hypogonadism and hypothyroidism. Permanent Diabetes insipidus was found in 3 patients (7.9%). Cyst height was a significant factor related to pre-operative visual field deficit and post-operative Diabetes insipidus. Residual cysts were found in 11 cases (30.6%) and 9 patients experienced regrowth/recurrence. Residual cysts were a significant factor in regrowth/recurrence. Recurrence rate and post-operative complications were not correlated with different subtypes. ConclusionsEndoscopic endonasal approach for removal of Rathke’s cleft cyst is a safe and effective intervention. It leads to significant improvement in visual field deficit and recovery of hyperprolactinemia. Although the incidence of post-operative Diabetes insipidus is high, it is usually temporary. Although different categorizations are not correlated to recurrence rate, they could help predict the status of hormone deficit.

Unusual Branchial Cleft Cyst at Parapharyngeal Space: Case Report and a Review of Literature.

Branchial cleft cyst in parapharyngeal space is a very rare occurrence. Only 0.5% of all head and neck tumours constitute parapharyngeal space tumours. It is mainly congenital but can be seen at a later age following infection. MRI is the gold standard for diagnosis. This is a case report of a 19-year-old with right sided neck swelling without any significant history. It has been excised by transcervical approach.

Modified Nasoseptal Flap Technique to Prevent the Recurrence of Rathke's Cleft Cyst.

Rathke's cleft cysts (RCCs) are benign epithelial lesions arising from the Rathke's pouch remnants that fail to regress during embryogenesis. Some RCCs become symptomatic and require treatment. Cyst fenestration and drainage of its contents is the preferred procedure to treat symptomatic cases but carries a risk of recurrence. We propose the use of a novel modified nasoseptal flap technique to partially line the cyst wall to avoid recurrence. This was a prospective, observational study that included all RCC patients admitted to the Department of Neurosurgery, Aster Medcity, from April 2015 to May 2018. The modified nasoseptal flap technique was performed in all patients. They underwent preoperative and postoperative ophthalmological, endocrine, endoscopic, and MRI evaluations to look for recurrence. Ten patients underwent the modified nasoseptal flap technique. The median follow-up was 36 months. Postoperatively, all patients were relieved from headaches. Moreover, their visual fields and pituitary functions normalized. None of the patients developed recurrence of RCC on follow-up brain MRI. On endoscopic examination, all patients had retained patency of the fenestra. The longest follow-up was 72 months. The modified nasoseptal flap technique maintains patency and avoids recurrence of RCCs on long-term follow-up.

Epidemiology, Genetics and Pathophysiology of Pituitary Tumors

Pituitary tumors or tumors of the sella turcica include pituitary neuroendocrine tumors, Rathke's cleft cysts, craniopharyngiomas, tuberculum sellae, planum sphenoidale meningiomas, germ cell tumors, and hypophysitis. In addition, some rare tumors, such as pituicytomas, granular cell tumors, spindle cell oncocytomas, and chordomas or chondrosarcomas, arise from the parasellar regions. The treatment strategy is completely different for each lesion; therefore, accurate diagnosis is essential.

Rathke's cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case.

Rathke's cleft cyst (RCC) is a benign sellar/suprasellar lesion often discovered incidentally. Rarely, symptomatic cases can present with headache and may exhibit concomitant aseptic meningitis or apoplexy. The authors describe a patient with an RCC presenting with recurring episodes of aseptic meningitis and ultimately inflammatory-type apoplexy. A 30-year-old female presented with three episodes of intractable headaches over 2 months. Each episode's clinical picture was consistent with meningitis though cerebrospinal fluid cultures, and viral tests remained negative. Imaging demonstrated a sellar lesion, initially thought to be coincidental. On the third presentation, there was rapid interval growth of the lesion, adjacent cerebritis, and new endocrinopathy. Resection was then performed via an endoscopic endonasal approach. Pathology showed an RCC with acute and chronic inflammation and no evidence of hemorrhage. Cultures were negative for organisms. The patient received several weeks of antibiotic treatment with the resolution of all symptoms and no recurrence. Recurrent aseptic meningitis with apoplexy-like symptoms is a rare presentation of RCC. The authors propose the term inflammatory apoplexy to describe such a presentation without evidence of abscess, necrosis, or hemorrhage. The mechanism is unclear although may be due to intermittent microleakage of cyst contents into the subarachnoid space.

Cervical Thymic Cyst - An Overlooked Entity

Thymic cysts are one of the rare causes of neck masses in the paediatric age group. Patients may complain of neck swelling either central or lateral neck swelling. There is difficulty in diagnosing a cervical thymic cyst as it may mimic a branchial cleft cyst or a cervical lymphangioma. Definitive diagnosis of a thymic cyst requires histopathological examination of thymic tissue. We report a case of a 6-year-old girl who presented with painless lateral neck swelling for 2 months duration. Computed tomography (CT) scan showed a left branchial cyst with retropharyngeal extension. The patient underwent complete excision of the mass and histological examination was reported as a cervical thymic cyst. Postoperatively, she was able to be discharged after serial follow-ups with no signs and symptoms of recurrence.

Endoscopic transsphenoidal surgery and Haddad nasoseptal flap of Rathke’s cleft cysts

Introduction: Rathke’s cleft cysts (RCC) grow formed from remnants of the embryologic Rathke’s pouch and are often found incidentally in the sellar or suprasellar region. These lesions are usually asymptomatic in most people. However, as the cyst progressively enlarges, they may develop symptoms typical of RCC, including headache, vision loss, and endocrine dysfunction. The diagnosis procedure involves an MRI scan of the pituitary gland. The endoscopic transsphenoidal approach has become the most common surgical approach to treat RCC. In the last 15 years, endonasal endoscopy has been widely used in the surgical management of these lesions. Case report: A 58-year-old Asian woman was reported mainly with a complaint of headache that had increased over the last two months, occasional nausea and vomiting, and blurred vision experienced for 1 month.