Suprasellar Rathke's Cleft Cyst Research Articles

Vertical triband flag sign for differential diagnosis of Rathke's cleft cyst

BackgroundThe Rathke cleft cyst (RCC) is a type of cystic growth that is benign, circular, and well-defined with an incidence rate of 4 %. This study aims to identify a useful diagnostic imaging sign that can aid in the differentiation of RCC from other cystic lesions. MethodsWe retrospectively analyzed the records of 42 symptomatic RCC patients who were referred to our facility between 2016 and 2023. The data for the study were obtained from our electronic database. All magnetic resonance imaging (MRI) studies were performed using a 1.5-T superconducting magnetic scanner. All patients underwent endonasal transsphenoidal surgical resection. All MRIs were reviewed and evaluated by a neurosurgeon and a neuroradiologist. ResultsThere were 8 (19 %) males and 34 (81 %) females with a mean age of 37.2-years. Our study identified a distinct imaging characteristic in 38 of the cases, which we have named the “vertical triband flag sign”, due to the growth of the cyst developing a specific appearance. The flag sign was mostly observed only in the T1-images (71.5 %), while in four cases the sign was spotted only in T2-images, and in four cases it appeared in both T1 and T2. In 4 cases, the flag sign was not observed in which further investigations revealed that these cases were suprasellar or small sellar RCCs. The dot sign, which is a characteristic finding in RCCs was only observed in one of our cases. ConclusionEarly diagnosis of RCCs may be facilitated by utilizing the vertical triband flag sign.

Clinical features and surgical outcomes of Rathke cleft cysts with suprasellar components: a single-center experience of 157 cases.

Both intrasuprasellar and suprasellar Rathke cleft cysts (RCCs) have suprasellar components, and we aimed to explore their clinical features and surgical outcomes. 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. 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). 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.

Craniopharyngioma masquerading as a suprasellar Rathke cleft cyst in a young patient with a history of Ewing sarcoma: A case report

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Expanded Endonasal Endoscopic Approach for Suprasellar Rathke Cleft Cyst: Treatment Outcome and Surgical Nuances

Rathke cleft cysts (RCCs) are benign lesions originating from remnants of the Rathke pouch. RCCs have been classified into the intrasellar-based or purely suprasellar types. This research aims to study the optimal treatment nuances and evaluate the surgical outcomes of this distinct type of RCCs. This study retrospectively reviewed 36 patients with purely suprasellar RCCs who were treated by expanded endonasal endoscopic approach (EEA) from September 2018 to January 2021. The demographic data, presenting symptoms, endocrine status, imaging findings, operative details, and surgical outcomes were analyzed. According to the relationship with pituitary stalk, the suprasellar RCCs can be divided into 2 different growth patterns: intrastalk cyst (ISC, 52.8%) and extrastalk cyst (ESC, 47.2%). Preoperative endocrine dysfunctions occurred more in patients with ISC than ESC (P= 0.007), which are characterized by hypoadrenalism (P= 0.004) and hypogonadism (P= 0.008). Operation exerted different impacts on endocrine functions between ISC and ESC (P= 0.012). When identifying by single hypothalamic-pituitary axis, 4 patients with normal endocrine function developed a new hormonal deficit, 14 patients with preoperative endocrine dysfunctions benefited from our expanded EEA surgical treatment, and 3 of them completely recovered from the endocrinopathy after surgery. The postoperative endocrinopathy was positively associated with the numbers of preoperative abnormal endocrinal axes. Thirty-four (94.4%) patients had achieved gross total resection. Pituitary stalk was intactly preserved in 23 of 36 patients (63.9%), partly preserved in 10 patients, and not preserved in 3 patients. Purely suprasellar RCCs are a distinct type of RCCs, which originate from the pituitary stalk above the diaphragmatic sellae, and the treatment strategy via expanded EEA is a rational choice. Classification based on growth patterns would be beneficial to reduce endocrine disorders after surgery.

Trans-eyebrow supraorbital keyhole approach for suprasellar and intra-suprasellar Rathke cleft cysts: the experience of 16 cases and a literature review

Background Purely suprasellar and some complex intra-suprasellar Rathke cleft cysts (RCCs) are commonly treated via extended endonasal endoscopic approach or traditional transcranial approach. The feasibility of the trans-eyebrow supraorbital keyhole approach (TSKA) for RCCs was evaluated in this retrospective study. Methods A cohort of 16 patients (11 females and 5 males) with RCC was surgically treated via TSKA between January 2013 and November 2021. The medical data and follow-up results were analyzed. Results Eight patients had a purely suprasellar RCC, and 8 patients had an intra-suprasellar RCC with most of pituitary gland occupying the sellar floor. The mean maximal size of the cysts was 15.4 (range 7–29) mm. Postoperatively, complete cyst drainage was achieved in 15 (93.7%) patients. Preoperative headache, visual dysfunction, and hypopituitarism improved in 12 (100.0%), 3 (75.0%) and 3 (75.0%) patients, respectively, and hyperprolactinemia normalized in all patients. Except 2 (12.5%) cases of transient diabetes insipidus (TDI), no other complications were observed. During the mean follow-up period of 41.0 (range 4–102) months, 1 (6.3%) radiological recurrence was found. Conclusions For the treatment of purely suprasellar and some intra-suprasellar RCCs with most of the pituitary occupying the sellar floor, the endoscopic TSKA has the advantage of the minimal invasiveness, excellent visualization of the intrasellar compartment, no additional damage to the underlying pituitary gland, and no risk of CSF leakage. TDI or DI is relative commonly found in treatment of these RCCs. During surgery, care should be taken to avoid damage to the surrounding structures, including the pituitary stalk.

The Supraorbital Keyhole Approach to the Suprasellar and Supra-Intrasellar Rathke Cleft Cysts Under Pure Endoscopic Visualization

To evaluate the feasibility of the supraorbital keyhole approach for suprasellar or intrasuprasellar Rathke cleft cysts (RCCs) under pure endoscopic visualization. Thirteen surgically treated patients with symptomatic RCCs were analyzed retrospectively. Preoperative manifestations included headache (77%), visual impairment (38%), polyuria (31%), and pituitary dysfunction (15%). The maximal cyst diameter averaged 29.3 mm (23-46 mm). Eight patients (62%) had a purely suprasellar lesion, and 5 (38%) had a sellar lesion with suprasellar extension. All patients underwent surgery via supraorbital keyhole approach under pure endoscopic view. The mean duration of follow-up was 21.4 months (9-45 months). The cyst content was removed completely in all cases, and the cyst wall was resected partially without transgressing surrounding structures. Preoperative headaches, visual dysfunction, diabetes insipidus, and hypopituitarism resolved in 80%, 60%, 50%, and 50% of patients, respectively. Postoperative complications included transient polyuria in 2 cases and temporary subcutaneous fluid collection in one. No radiological recurrence was observed during the follow-up. The supraorbital approach under pure endoscopic visualization provides a valuable optional minimally invasive access for suprasellar and intrasuprasellar RCCs. This approach combines the advantage of minimal invasiveness and improved visualization while avoiding the risk of cerebrospinal fluid leaks, rhinonasal complications, and gland injury. It is particularly suitable for those cysts with the normal pituitary gland occupying the whole or most part of sellar floor.

Surgical experiences of Rathke's cleft cyst resection via the fully endoscopic supraorbital trans-eyebrow keyhole approach

Objective To summarize the surgical techniques of Rathke's cleft cyst resection via the fully endoscopic supraorbital trans-eyebrow keyhole approach and to evaluate its efficacy and the advantages and disadvantages. Methods Twelve patients with Rathke's cleft cyst confirmed by pathology at the Department of Neurosurgery, Nanfang Hospital, Southern Medical University from May 2012 to October 2014 were analyzed retrospectively. The clinical manifestations included headache, visual impairment, polydipsia, polyuria, and pituitary dysfunction. CT and MRI revealed the cystic masses in saddle areas. The maximum mean diameter was 29.3 mm, in which 4 lesions were located entirely on the suprasellar areas, and 8 were located on intrasellar suprasellar areas. All patients were treated with cyst resection via the fully endoscopic supraorbital trans-eyebrow keyhole approach. Results The cyst contents were all removed and part of cyst walls were resected in 12 patients. All were pathologically confirmed as Rathke's cleft cyst. The preoperative symptoms of all patients were cured or improved. Two patients had transient polyuria, and they discharged at 3 to 4 days after procedure. They were followed up for 3 to 24 months after procedure. No recurrence was found on imaging examinations. Conclusions The fully endoscopic supraorbital trans-eyebrow keyhole approach is suitable to intrasellar suprasellar Rathke's cleft cyst which is entirely located on the suprasellar area or the main body is located on the suprasellar area. This approach has the advantages of minimal invasiveness, rapid recovery and the absence of cerebrospinal fluid leakage and other complications. The neuroendoscopic technology may further reduce trauma and expand the field of vision, especially beneficial for the observation and removal of intrasellar cyst. Key words: Neuroendoscopy; Central nervous system cysts; Rathke cleft cyst; Trans-eyebrow approach; Keyhole surgery

GH-Producing Pituitary Adenoma and Concomitant Rathke's Cleft Cyst: A Case Report and Short Review.

Concomitant pituitary adenoma (PA) and Rathke's cleft cyst (RCC) are rare. In some cases, such PA is known to produce pituitary hormones. A 53-year-old man was admitted to our hospital with a diagnosis of lacunar infarction in the left basal ganglia. Magnetic resonance imaging (MRI) incidentally showed a suprasellar mass with radiographic features of RCC. When he consulted with a neurosurgical outpatient clinic, acromegaly was suspected based on his appearance. A diagnosis of growth hormone- (GH-) producing PA was confirmed from hormonal examinations and additional MRI. Retrospectively, initial MR images also showed intrasellar mass that is compatible with the diagnosis of PA other than suprasellar RCC. The patient underwent endonasal-endoscopic removal of the PA. Since we judged that the RCC of the patient was asymptomatic, only the PA was completely removed. The postoperative course of the patient was uneventful and GH levels gradually normalized. Only 40 cases of PA with concomitant RCC have been reported to date, including 13 cases of GH-producing PA. In those 13 cases, RCC tended to be located in the sella turcica, and suprasellar RCC like this case appears rare. In a few cases, concomitant RCCs were fenestrated, but GH levels normalized postoperatively as in the cases without RCC fenestration. If radiographic imaging shows typical RCC, and PA is not obvious at first glance, the possibility of concomitant PA still needs to be considered. In terms of treatment, removal of the RCC is not needed to achieve hormone normalization.

Extended endoscopic transsphenoidal approach infrachiasmatic corridor.

An extended endoscopic transsphenoidal approach is required for skull base lesions extending to the suprasellar area. Inferior approach using the infrachiasmatic corridor allows access to the lesions through the tumor growth that is favorable for the extended transsphenoidal approaches. Infrachiasmatic corridor is a safer route for the inferior approaches that is made up by basal arachnoid membrane and Liliequist's membrane with its leaves (diencephalic and mesencephalic leaf). This area extends from the optic canal and tuberculum sella to the corpus mamillare. We performed extended endoscopic approach using the infrachiasmatic corridor in 52 cases, including tuberculum sella meningiomas (n:23), craniopharyngiomas (n:16), suprasellar Rathke's cleft cyst (n:6), pituitary adenoma (n:2), fibrous dysplasia (n:1), infundibular granulosa cell tumor (n:2), and epidermoid tumor (n:2). Total resection was achieved in 17 of 23 (74%) with tuberculum sellae meningioma using infrachiasmatic approach. Twenty patients presented with visual disorders and 14 of them improved. There were two postoperative cerebrospinal fluid (CSF) leakages and one transient diabetes insipidus and one permanent diabetes insipidus. Sixteen patients were operated on by the infrachiasmatic approach for craniopharyngiomas. Improvement was reached in seven of eight patients presented with visual disorders. Complete tumor resection was performed in 10 of 16 cases and cyst aspiration in 4 cases, and there were remnants in two cases. Postoperative CSF leakage was seen in two patients. Infrachiasmatic corridor provides an easier and safer inferior route for the removal of middle midline skull base lesions in selected cases.

A giant Suprasellar Rathke cleft cyst with psychiatric manifestations: Case report

Rathke cleft cyst (RCC) is a benign, cystic remnant of the cranopharyngeal duct, typically located in the sellar region [1]. Purely uprasellar RCC situated above a normal sella is rare [2]. The comon clinical presentations of symptomatic RCCs include headache, isual impairment, and endocrine disturbance. Descriptions of psyhiatric manifestations associated with RCCs were exceedingly are [2–8], and their potential neuropsychiatric significance may e underestimated. In this report, the authors presented the case f a giant suprasellar RCC with psychiatric manifestations which esolved spontaneously after surgical intervention.

An interesting case of Rathke′s cleft cyst presenting as bilateral cryptorchidism

Rathke's cleft cyst (RCC) are benign, epithelium-lined sellar and suprasellar cysts believed to originate from remnants of the Rathke pouch. Here, we report a rare case of entirely suprasellar RCC in a 4.5-year-old child causing hypopituitarism, who presented to us with bilateral cryptorchidism. Subsequently we discuss the distinct rarity of entirely suprasellar RCC presenting in this fashion and the relevant literature is also discussed.

A Rathke's Cleft Cyst Presenting with Apoplexy

The occurrence of symptomatic pituitary hemorrhage into a Rathke's cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.

Endoscopic Endonasal Resection of Sellar and Suprasellar Rathke's Cleft Cysts: Review of 34 Cases

Introduction: Rathke's cleft cysts are benign lesions that may be diagnosed as an incidental finding, associated with headaches or pituitary dysfunction. They are typically in a sellar or suprasellar location.

Pathological laughter in a patient with Rathke cleft cyst

Rathke cleft cysts (RCCs) arise from embryonic remnants of Rathke’s pouch, and usually manifest as headaches, visual impairments and endocrine disturbances. We report a rare case of suprasellar RCC with the main symptom of pathological laughter. A 14-year-old girl presented with involuntary laughter, ataxia in the right arm and slightly impaired speech articulation. MRI revealed a huge suprasellar cyst causing severe compression of the midbrain and pons. The cyst was removed via a sphenoid ridge keyhole approach. Histologic examination resulted in a diagnosis of RCC. The spontaneous laughing spells disappeared immediately after surgery. In this case, compression of the upper brainstem might have been the cause of the pathological laughter.

EXTENDED ENDOSCOPIC ENDONASAL TRANSSPHENOIDAL APPROACH FOR THE REMOVALOF SUPRASELLAR TUMORS

The widespread use of the endoscope in transsphenoidal pituitary surgery has recently contributed to the extension of the approach beyond the tuberculum sellae and planum sphenoidale for the management of lesions located in the suprasellar area, either with an endoscope-assisted or purely endoscopic technique. Based on our previous experience with more than 450 standard endoscopic transsphenoidal operations, we have retrospectively evaluated the effectiveness of the extended endoscopic endonasal transsphenoidal approach in the management of lesions mainly located in the suprasellar area. Between January 2004 and December 2005, 20 consecutive patients underwent extended endoscopic endonasal transsphenoidal surgery for a total of 21 procedures. The series consisted of seven pituitary adenomas, seven craniopharyngiomas, three suprasellar Rathke's cleft cysts, two tuberculum sellae meningiomas, and one pilocytic astrocytoma of the chiasm. Tumor removal, as assessed by postoperative magnetic resonance imaging, revealed complete removal of the lesion in four out of seven pituitary adenomas, five out of seven craniopharyngiomas, three out of three Rathke's cleft cysts, and two out of two tuberculum sellae meningiomas. One patient (5%) with craniopharyngioma had a postoperative cerebrospinal fluid leak that required reoperation. The same patient experienced a sphenoid mycosis, which was treated with medical therapy. Some specific conditions associated with the anatomy of the surgical route, as well as to the morphology of the lesion, have resulted to condition the feasibility of the approach. Small and medium sized suprasellar lesions located in the midline, with or without a limited parasellar extension and without involvement of vascular structures, seem amenable to be resected through such extended endoscopic transsphenoidal approach. Improvements in closure techniques and the use of new materials and surgical glues seem to significantly reduce the postoperative cerebrospinal fluid leak rate and meningitis.

Rathke cleft cyst of the sphenoid bone and nasopharynx

1095 Manuscript received May 31, 2004; accepted in final form August 30, 2005. Address reprint requests to: Karim Ben Hamouda, M.D., Service de Neurochirurgie, Institut National de Neurologie, La Rabta, 1007, Tunis, Tunisia. email: kbenhamouda@lycos.com. We report an exceptional case of a sphenoidal and nasopharyngeal Rathke cleft cyst (RCC). In a literature review, we found that this cyst had never been described in this region. This 41-year-old man presented in August 2002 with progressive headaches, ptosis, and diplopia. In November 2002, he experienced a spontaneous episode of rhinorrhea and a complete subsequent resolution of the symptoms. In February 2003, the frontal headaches relapsed, associated this time with a progressive decrease in left-sided visual acuity. On clinical examination in April 2003, the patient had an isolated loss of visual acuity (1/10) in the left eye. Endocrinological assessment demonstrated normal results. Computerized tomography scanning revealed that the sphenoid region had been destroyed by a large noncalcified low-density cystic lesion. Magnetic resonance (MR) imaging demonstrated a cystic sphenoidal extradural lesion that was isointense on T1-weighted sequences and peripheral enhancement after Gd enhancement (Fig. 1 left); hyperintensity was observed on T2-weighted images (Fig. 1 right). The maximal diameter of the lesion was 4 cm. There was also a smaller contiguous cyst on the posterior wall of the nasopharynx. A transrhinoseptal approach was used. The wall of the sphenoid sinus was paper thin; a motor oil–like fluid escaped. The cyst widely communicated with the nasal fossa, and its wall was partially extirpated. The dura covering the sellar floor was unperforated. Pathological examination showed a columnar ciliated epithelium (Fig. 2) consistent with an RCC. The postoperative course was uneventful; all symptoms resolved and no neurological symptoms developed. Imaging performed 1 week after surgery revealed partial evacuation of the cyst, with reappearance of the pituitary gland and a morphologically normal pituitary stalk. We also observed the persistence of a nasopharyngeal cyst under the sphenoidal lesion. One year later imaging revealed the disappearance of the sphenoidal cyst and the persistence of the lesion located on the posterior wall of the nasopharynx (Fig. 3). The sphenoidal, infrasellar origin of this RCC is supported by the absence of a suprasellar extension and by its entirely extradural location. Remnants of the craniopharyngeal canal can originate in a spectrum of cystic lesions, from simple RCCs to complex craniopharyngiomas.1 According to Erdheim’s theory,2 a craniopharyngioma can arise from every part of the craniopharyngeal canal. This may be also true for an RCC. In our case, it seems that the cyst derived from the canal enclosed in the cranial base bones, in contact with the walls of the pharynx. Computerized tomography and MR imaging features of RCC are not specific and are seen in more common lesions in this area such as craniopharyngiomas and mucoceles. The preoperative diagnosis of RCC is theoretically important because it may limit the operative management to biopsy sampling of the cyst wall and drainage of the contents via the transsphenoidal route.1 Voelker and colleagues3 concluded in a review of 155 cases that the transsphenoidal approach is optimal choice for sellar and suprasellar RCC and that partial excision and drainage of these cysts are the recommended treatments. In this case the sphenoid cyst disappeared after a partial excision of its walls.

Symptomatic Rathke’s cleft cysts: A report of 24 cases

We review the clinical, hormonal and imaging features of 24 consecutive patients with symptomatic Rathke's cleft cysts (RCCs), and assess the long-term effectiveness and complications of transsphenoidal cyst removal. Out of 250 consecutive patients, 24 (10%) underwent endonasal transsphenoidal surgery for RCC; 19 (79%) were women. Symptoms at presentation included headaches (83%), hyperprolactinemia (38%), central hypothyroidism (21%), galactorrhea (13%), diabetes insipidus (13%), IGF-1 deficiency (13%), central adrenal insufficiency (8%) and visual loss (8%). In total, 37% of women had irregular menses and 60% of men sexual dysfunction and hypogonadism. Two girls presented with precocious puberty. Cyst size varied from 7 to 25 mm. Fifteen (60%) had a suprasellar component. Initial and 3-month post-operative imaging revealed complete cyst resection in 23 of 24 patients. Headaches resolved in 65% of subjects and visual loss resolved in both patients who presented with this symptom. Of those presenting with endocrinopathy, 56% had improvement of at least one anterior pituitary axis; two subjects (8%), both with suprasellar RCC, developed a new hormone deficiency post-operatively and two sujects young girls, (8%) had RCC recurrence, one at 36 months after surgery, requiring a second operation, and the other had a small asymptomatic recurrence 6 months after surgery. In conclusion, RCC accounts for 10 % of surgically treated sellar and suprasellar masses. Headache, hyperprolactinemia, menstrual irregularities and sexual dysfunction are common presenting symptoms. Simple cyst removal via a transsphenoidal approach offers a safe and effective treatment. Cyst recurrence may be more common in children.

An entirely suprasellar Rathke's cleft cyst: case report and review of the literature

Rathke's cleft cysts usually present with an intrasellar or a combined intrasellar and suprasellar localization. In this report we present a 58-year-old man with a Rathke's cleft cysts in an entirely suprasellar location with intact diaphragm and intact sella. Although this presentation of a Rathke's cleft cyst is extremely rare, it belongs in the differential diagnosis of entirely suprasellar lesions. The relevant literature is discussed.

Intracranial Approach to Suprasellar Rathke's Cleft Cyst

The occurrence of a symptomatic Rathke's cleft cyst without extension into the sella turcica is rare. The purpose of this report is to present such a case, with its clinical manifestation, diagnostic imaging characteristics, operative approach, pathology and review of the literature.

Entirely suprasellar Rathke's cleft cyst の1手術例

今回われわれは,非常に稀な entirely suprasellar Rathk's cleft cystの1例を経験したので報告した.症例は,58歳,女性.主訴は,視力・視野障害,意欲低下.CTでトルコ鞍上部でややlow densityを呈し,石灰化や増強効果は認められなかった.MRIでは,T1,T2強調画像ともに均一なhigh intensityを呈した.術前cystic craniopharyngiomaとの鑑別が困難であったため,全摘が可能であるinterhemispheric trans-lamina termina1is approachで手術を行った.自験例を含めて診断,組織像,ならびに手術方法などについて,文献的考察を加えた.