Symptomatic Rathke's Cleft Cyst Research Articles

P-49 Unexpected spontaneous evolution of a symptomatic Rathke's cleft cyst

Abstract Introduction Rathke's cleft cysts (RCCs) are relatively rare sellar and suprasellar lesions that arise from remnants of Rathke's pouch, an embryonic precursor to the anterior pituitary gland. These cysts are often found incidentally during neuroimaging studies for unrelated issues. Despite their non-neoplastic nature, RCCs can lead to a spectrum of clinical manifestations due to their potential to compress surrounding structures, including the pituitary gland, optic chiasm, and adjacent neurovascular structures. However, the majority of Rathke's cysts will remain asymptomatic and typically do not necessitate any medical intervention. Instances of spontaneous regression of these cysts are infrequent, and cases of regression occurring after a recurrence are exceptionally rare. Clinical Case The patient is a 57-year-old individual with a medical history of well-controlled type 2 diabetes and high blood pressure. Additionally, the patient had a history of right ocular trauma in 1989 resulting in a decrease of visual acuity. The patient presented with persistent chronic headaches, leading to the detection of a Rathke's cleft cyst as revealed by a pituitary MRI showing a cystic structure measuring 15×11 mm in the sellar region. The cyst displayed a predominant left-sided lateralization and was characterized by T1 hypointensity as well as T2 hyperintensity. No enhancement was observed on post-gadolinium contrast injection. The cyst exerted a mass effect on the optic chiasm. Hypophyseal axis assessment revealed intact function. Notably, the patient exhibited significant visual field impairment in the right eye, marked by loss of the superior, nasal, and inferior fields, while the right visual field remained unaffected. The patient was managed conservatively based on the rationale that the visual disturbances were attributed to the ocular trauma and not linked to the RCC. Follow-up hypothalamo-hypophysial MRI conducted in April 2023 demonstrated complete spontaneous regression of the Rathke's cleft cyst, with an improvement of his chronic headache. Conclusion The natural history of RCCs is multifaceted leading to heterogenous outcomes ranging from volume progression with hormonal deficiencies and/or visual disturbances to stability over time to even spontaneous involution. The precise mechanism underlying the latter remains unclear. One possible explanation could involve disruptions in the equilibrium between the secretion and absorption of the cystic fluid, or even the potential occurrence of cyst rupture. The potential for involution or stability of the cyst is influenced by the cyst size. The likelihood of spontaneous involution advocates for a conservative strategy in cases of symptomatic RCCs where headaches are the sole presenting symptom.

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.

Rare Case of Misdiagnosed Rathke Pouch Cleft Leading to Severe Visual Loss

A rare case of symptomatic Rathke cleft cyst resulting in severe visual compromise in a young 45 yr/f who presented with gradual progressive painless diminution of vision in both eyes. Patient noticed diminution of vision in the Left eye an year ago and later on in the Right eye for which she consulted ophthalmologists. She was diagnosed as optic neuritis and underwent treatment for the same, but vision didn’t improve. At presentation,vision in Both eyes was same, counting fingers at 2 meters. Pupillary reactions were normal, however there was bilateral disc pallor and rest fundus was normal. On systemic evaluation patient gave history of irregular menstrual periods since 3-4 months. on further investigation her S. Prolactin levels were markedly raised(59.99ng/ml). Radiology showed well defined hyperintense suprasellar lesion. Patient was operated for right frontotemporal craniotomy with decompression of RCCs by a neurosurgeon. 2 months post surgery visual acuity in RE 4/60 and LE is counting finger close to face.

Long-Term Outcomes of Endoscopic Cyst Fenestration for Rathke Cleft Cyst

We sought to elucidate the long-term surgical outcomes and incidence of recurrence and reoperation of endoscopic endonasal cyst fenestration for Rathke cleft cyst (RCC). A retrospective review of the chart and operation record of RCC surgical cases between January 2008 and August 2021 at our institution was conducted. Patient characteristics, intraoperative findings, and postoperative follow-up outcomes were evaluated. A total of 27 patients were analyzed, with a median postoperative follow-up period of 52 months (range, 1-150 months). Preoperative symptoms were visual dysfunction (59%), headache (41%), and pituitary dysfunction (22%). Endoscopic cyst fenestration was performed in all patients. Ten (37%) patients had intraoperative cerebrospinal fluid leakage. Among them, the only patient in whom sellar floor reconstruction was not performed experienced a repair operation due to postoperative cerebrospinal fluid leakage. No patients experienced postoperative hypopituitarism. Preoperative headache, visual dysfunction, and pituitary hormone disorder improved in 73%, 75%, and 67% of patients, respectively. Although postoperative cyst regrowth was observed in 8 patients (30%), no patient experienced worsening or novel symptoms and none required reoperation. Patients with a symptomatic RCC can be effectively treated with endoscopic endonasal cyst fenestration. Reversal of the presenting symptoms resulted, including headache, visual dysfunction, and pituitary hormone dysfunction, in the majority of patients. In our series, appropriate reconstruction of the sellar floor reduced the risk of postoperative cerebrospinal fluid leakage without impacting cyst regrowth. This simple technique appears to effectively disrupt cyst progression in most cases, even after a relatively long-term follow-up period.

Surgical outcomes and quality of life in Rathke's cleft cysts undergoing endoscopic transsphenoidal resection: a multicentre study and systematic review of the literature.

To establish the effect of endoscopic endonasal surgery (EES) on quality-of-life (QoL) in symptomatic Rathke cleft cyst (RCC). Analysis of 38 patients with RCC treated by EES, with regular overall (ASBQ-35) and sinonasal-specific (SNOT-22) QoL assessment during the first postoperative year. A systematic literature review of large case series was performed with pooled analysis. In our series, mean age was 53.6years with a female predominance (73.7%). Larger cysts were seen in males (p < 0.01), those with hypogonadism (p = 0.04), and visual dysfunction (p = 0.04). Complete normalisation of vision was seen in 83.3%. Persistence of visual dysfunction postoperatively was associated with diabetes (p = 0.005), hypertension (p = 0.02), suprasellar only location (p = 0.001), and monocular field cut (p = 0.02). Surgery did not significantly effect hormonal function. Sinonasal QoL transiently worsened after surgery, resolving within 3weeks. A parallel transient worsening of overall QoL normalised by 6weeks, and remained at preoperative baseline thereafter. These results were comparable to the literature, where 76.4% demonstrated improvement of vision and 13.1% had recurrence after treatment. There was no significant difference in outcomes between EES and microscopic approaches. We report longitudinal QoL outcomes in RCC for the first time. Vision commonly improves with surgery, but endocrinopathy is likely to persist. Microvascular risk-factors may compromise visual improvement. Surgery causes a transient worsening of sinonasal symptoms that resolves within 3-6weeks, but patients may not experience significant improvement in QoL within the first postoperative year.

A Rare Case of Rathke’s Cleft Cyst Apoplexy

The occurrence of symptomatic Rathke's cleft cyst (RCC) apoplexy is extremely rare. This is often misdiagnosed due to similar presentations to subarachnoid haemorrhage and pituitary apoplexy. This case highlights an excellent example of similar clinical presentation and serves as a learning case for clinicians. A 40-year-old lady presented to a district hospital with 9 days of worsening severe headache associated with blurring of vision, photophobia, stiff neck, nausea and vomiting. Nuchal rigidity and Brudzinski’s positive. Blood test showed hyponatremia, raised inflammatory markers and normal dynamic pituitary function test. CT Head demonstrated no evidence of space-occupying lesion or intracranial haemorrhage. Lumbar puncture showed xanthochromia positive consistent with subarachnoid haemorrhage. MRI head advised by Neurosurgery team and revealed a focal lesion involving anterior pituitary macroadenoma with mass effect on optic chiasm with possible haemorrhage within. Further assessment in tertiary hospital confirmed loss of visual acuity and field deficit. Patient underwent emergency endoscopic transnasal transsphenoidal resection of apoplectic tumour and repair of CSF leak with graft from thigh. Histopathology report showed a Rathke’s cleft cyst with squamous metaplasia. Post operatively, the patient developed sinusitis which fully recovered, and MRI showed good decompression. The author demonstrated a rare case of symptomatic RCC which was initially presumed to be pituitary apoplexy. Radiology imaging and treatment approach for both conditions are quite similar and can only be differentiated by histopathology. Further research is required to identify the causes and risk factors of RCC apoplexy to aid early detection and diagnosis.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S23

Endoscopic endonasal cyst fenestration into the sphenoid sinus using the mucosa coupling method for symptomatic Rathke's cleft cyst: a novel method for maintaining cyst drainage to prevent recurrence.

Rathke's cleft cyst (RCC) is a benign cystic lesion with a relatively high incidence of local recurrence that occasionally requires repeat surgery. To prevent recurrence, simple cyst fenestration and drainage of the cyst contents to the sphenoid sinus is recommended, but it occasionally recurs. The authors postulated that obstruction of fenestration is a main cause of recurrence, and they developed a method, named the "mucosa coupling method (MC method)," that maintains persistent drainage. In this method, the RCC epithelium and the mucosa of the sphenoid sinus are connected, which promotes re-epithelialization between the two epithelia, maintaining persistent drainage. The outcome of this method was compared with that of conventional cyst fenestration. In a consecutive series of 40 patients with RCC, the surgical strategy was changed during the study period: from December 2009 to September 2014 (the conventional period), 24 patients were scheduled to be treated using the conventional fenestration method, whereas from September 2014 to September 2017 (the MC period), 16 patients were scheduled to be treated using the MC method. However, because of an intraoperative CSF leak, the fenestration was closed during surgery in 3 patients in the conventional period and 2 in the MC period; therefore, these 5 patients were excluded from the analysis. Twenty-one patients treated with the conventional fenestration method (conventional group) and 14 patients treated with the MC method (MC group) were analyzed. All patients regularly underwent MRI after surgery to detect reaccumulation of cyst contents. The rate of reaccumulation with and without reoperation, visual outcomes, endocrinological outcomes, and postoperative complications were compared between these two groups. The median follow-up period in all 35 patients was 48.0 months (range 1-96 months), 54.0 months (range 1-96 months) in the conventional group and 35.5 months (range 12-51 months) in the MC group. No reaccumulation was detected on MRI in the 14 patients in the MC group, whereas it was noted in 9 (42.9%) of 21 patients in the conventional group, and 2 of these 9 patients required repeat surgery. There were no significant differences in visual and endocrinological outcomes and complications between these two groups. The MC method for RCC is effective for preventing obstruction of cyst fenestration, which contributes to preventing cyst reaccumulation. Furthermore, this method is equivalent to the conventional fenestration method in terms of visual and endocrinological outcomes and the complication rate.

Symptomatic Rathke cleft cyst in paediatric patients - clinical presentations, surgical treatment and postoperative outcomes - an analysis of 38 cases.

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

Natural history of Rathke's cleft cysts: A retrospective analysis of a two centres experience.

Rathke's cleft cyst (RCC) is a common sellar lesion which may cause visual impairment, hypopituitarism and headaches from mass effect. The natural history of these lesions is currently unclear. We investigated the natural history of RCCs and compared surgically treated patients with those treated conservatively. We performed a retrospective cohort study of patients diagnosed with a RCC between 1996 and 2016 at Stanford University and Ospedale Maggiore Policlinico di Milano. Patients were divided into 2 cohorts: Group A, 72 subjects who underwent surgical resection of a symptomatic RCC, and Group B, 62 subjects managed conservatively. Compared to Group B, Group A subjects had larger RCCs (79% vs 22% had a largest diameter >10mm, P<.001) and were more likely (41.5% vs 16%, P<.001) to present with hypopituitarism and diabetes insipidus (DI) (18% vs 1.6%, P=.002). In Group A, after a mean follow-up of 53.7months, 12.5% of patients had recurrence and a second surgery. After surgery, 35% of patients recovered pituitary function. Hyperprolactinemia (26.6%) and hypogonadism (66.6%) resolved more commonly that did DI (20.1%). New pituitary deficits appeared in 16.6% of patients after surgery. In Group B, with a mean follow-up of 41months, only 6.4% had cyst enlargement, none underwent surgery, and none developed a pituitary deficit. Our data offer guidance in decision-making regarding the management of RCC patients and confirm the safety of conservative treatment in asymptomatic patients.

A Case Report of Symptomatic Rathke's Cleft Cyst

Background: Rathke's cleft cyst (RCC) is a not-so-common pituitary developmental disorder, it is a benign growth found on the pituitary gland in the brain, specifically a fluid-filled cyst in the posterior portion of the anterior pituitary gland. Symptomatic cysts are very rare. With only few cases have been documented in the literature. Aim of the work: this study aimed to assess the effect of corticosteroids on patientswithsymptomatic Rathke’s cleft cyst. Methodology: we reported a rare case of a 21 years old male patient, who was referred from a primary health center to the endocrine clinic with the complain of decreased visual acuity, blurred vision, and headache. On examination, there were no signs of puberty, no secondary sexual hair, truncal and generalized obesity. The fundus examination showed bilateral temporal pallor, with pale disc and papilledema. On the basis of symptoms and examination of the patient we suspected a lesion in the brain obstructing the optic pathway and therefore suggested MRI. Conclusion: this case led us to conclude that Rathke's cleft cysts should be considered in the differential diagnosis of patients who present with adrenal insufficiency and that the cysts can be reduced by glucocorticoids.

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.

Symptomatic Rathke's cleft cyst with a co-existing pituitary tumor; Brief review of the literature

Pituitary adenomas and Rathke's cleft cysts (RCCs) share a common embryological origin. Occasionally, these two lesions can present within the same patient. We present a case of a 39-year-old male who was found to have a large sellar lesion after complaints of persistent headaches and horizontal nystagmus. Surgical resection revealed components of a RCC co-existing with a pituitary adenoma. A brief review of the literature was performed revealing 38 cases of co-existing Rathke's cleft cysts and pituitary adenomas. Among the cases, the most common symptoms included headache and visual changes. Rathke's cleft cysts and pituitary adenomas are rarely found to co-exist, despite having common embryological origins. We review the existing literature, discuss the common embryology to these two lesions and describe a unique case from our institution of a co-existing Rathke's cleft cyst and pituitary adenoma.

Inflamed symptomatic sellar arachnoid cyst: case report.

Sellar arachnoid cysts are rare; an infected arachnoid cyst is extremely rare as only one case has been reported to date in the literature. Here, we report a patient with an infected or inflamed sellar arachnoid cyst that was successfully treated with transsphenoidal surgery (TSA). A 53-year-old female with a history of chronic sinusitis developed a headache 5 months ago, and one month before admission polyuria, polydipsia, and abnormal vaginal bleeding occurred. The magnetic resonance imaging (MRI) showed a sellar cystic mass with a thickened pituitary stalk. Preoperative hormonal study revealed normal pituitary hormone levels except for a moderate elevation of prolactin. She was diagnosed with diabetes insipidus of the central nervous system origin based on a water-deprivation test. TSA was performed under an impression of symptomatic Rathke's cleft cyst according to the MRI findings. Intraoperative findings showed confirmation of turbid intracystic contents, but micro-organisms were unidentified on microbial culture. Pathology of the cyst wall revealed inflamed meningoepithelial lining cells compatible with an arachnoid cyst.

Symptomatic Rathke cleft cyst

Rathke cleft cysts (RCC) are uncommon intrasellar lesions. Although their clinical manifestations, radiological features and treatment are frequently reported, controversy remains as a result of their rarity. We reviewed the preoperative clinical manifestations, neurological examination findings, visual acuity and fields, endocrinological function, radiographic study findings, surgical and pathological records, and prognosis of 45 patients with RCC (21 males, 24 females, average age: 47years) admitted to our department between January 2002 and January 2011. The most common clinical manifestations included headaches, and visual and hormonal disturbances. Most RCC were intrasellar with a suprasellar extension. The most common MRI patterns were hypointense on T1-weighted and hyperintense on T2-weighted images, isointense on T1-weighted and hyperintense on T2-weighted images, and hyperintense on T1-weighted and hyperintense on T2-weighted images. Aspiration and biopsy of the cyst wall were performed in most patients. Most patients experienced improved headaches and visual disturbance, but the hormonal disturbance rarely returned to normal, especially in those patients with a serious preoperative hormonal disturbance. The recurrence rate was 14%, which was associated with the extent of cyst removal, inflammation and rim enhancement, as well as the surgical approach. Aspiration and biopsy of the cyst wall still seems to be an effective treatment for most RCC for its low morbidity and good prognosis. Conservative treatment and close follow-up may be suitable for small cysts with subtle clinical manifestations.

A Case of Ectopic Rathke's Cleft Cyst in the Prepontine Cistern

A Rathke's cleft cyst (RCC) is a benign pituitary cyst derived from the remnant of Rathke's pouch, and usually presents as an intrasellar lesion with varying degrees of suprasellar extension. However, to date, a description of a primary prepontine RCC with no intrasellar component has not been reported. The author describes an exceptional case of a symptomatic RCC located behind the sella turcica in a 41-year-old woman who presented with severe headache. The author also provides an embryological hypothesis of the development of an ectopic RCC, with a special emphasis on radiologic characteristics.

Clinicopathological characteristics in patients presenting with acute onset of symptoms caused by Rathke’s cleft cysts

Symptomatic Rathke's cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke's cleft cyst with acute onset and discuss the clinical significance. In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke's cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively. Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases. Rathke's cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke's cleft cyst is difficult to diagnose pre-operatively, Rathke's cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.

Large Ossified Rathke's Cleft Cyst: A Case Report and Review of the Literature

We report a rare case of symptomatic Rathke's cleft cyst with thick calcified wall. Brain CT scans revealed a large cystic mass with round thick calcified wall. In this case, we selected the pterional approach instead of transsphenoidal approach due to the possibility of cystic craniopharyngioma. Histopathologically, it was calcified Rathke's cleft cyst with focal epithelial metaplasia. This case illustrates that calcification of the suprasellar cyst does not always suggest craniopharyngioma and the calcification pattern of Rathke's cleft cyst is different from that of the craniopharyngioma.

The Endocrinological Characteristics of Rathke's Cleft Cyst -Pathologically Confirmed in Seven Cases-

Rathke's cleft cyst is a cystic sellar or suprasellar lesion thought to originate from the remnants of Rathke's pouch. It is characteristically lined by a single layer of ciliated cuboidal or columnar epithelium. Although small asymptomatic Rathke's cleft cysts have been reported at autopsy in 2~26% of normal pituitary glands, symptomatic cysts are rare. For patients with symptomatic Rathke's cleft cysts presentation with headache, pituitary dysfunction, and visual loss caused by compression of the optic chiasm in the event of suprasellar extension, is common. We report the endocrine characteristics of seven cases of patients with symptomatic Rathke's cleft cyst diagnosed with pathologic confirmation. (J Kor Endocrinol Soc 22:74~79, 2007)

Rathke Cleft Cysts

Object. Microscopic Rathke cleft cysts are a common incidental autopsy finding, but some Rathke cleft cysts can become sufficiently large to cause visual impairment, hypothalamic-pituitary dysfunction, and headaches. In this study patients were evaluated preand postoperatively to ascertain the clinical significance of surgical intervention on endocrine and visual improvement. Factors correlated with cyst recurrence were also evaluated. Methods. A retrospective analysis was conducted in 160 patients with Rathke cleft cysts who were treated between 1984 and 1995 and completed at least a 5-year follow-up period. Of these 160 patients, 118 initially exhibited symptoms of visual impairment or endocrine dysfunction, became symptomatic during the follow-up period, or were found to have cyst enlargement. These 118 patients underwent transsphenoidal surgery. Forty-two patients with incidental lesions that demonstrated no growth on magnetic resonance (MR) images were followed up without an operation. Complete resection, as observed on MR images 3 months postoperatively, was obtained in 114 (97%) of 118 patients. Vision improved postoperatively in 57 (98%) of 58 patients. Hypogonadism improved in 11 (18%) of 62 patients, growth hormone deficiency resolved in 14 (18%) of 78 patients, and hypocortisolemia resolved in one (14%) of seven patients. Twenty-two patients (19%) began to exhibit symptoms of diabetes insipidus, which had not been present preoperatively. The total 5-year recurrence rate was 18% (21 of 118 patients), with 12 patients requiring a repeated operation. Surgical and pathological factors that were found to be statistically associated with recurrence were the use of a fat and/or fascial graft for closure (p < 0.01) and the presence of squamous metaplasia in the cyst wall (p < 0.01). The extent of resection of the cyst wall was not associated with an increased rate of recurrence. In 42 (69%) of 61 patients the incidental cysts did not progress on imaging studies or clinically. Conclusions. This is the largest series of patients with symptomatic Rathke cleft cysts who received operative intervention and participated in the longest postoperative follow up reported in the literature. The high recurrence rate (18%) supports the theory that a relationship exists between a symptomatic Rathke cleft cyst and craniopharyngioma. Improvements in visual and endocrine dysfunction can be expected after surgical decompression of the optic apparatus and the hypothalamic-pituitary axis.

Surgical outcomes in 118 patients with Rathke cleft cysts

Microscopic Rathke cleft cysts are a common incidental autopsy finding, but some Rathke cleft cysts can become sufficiently large to cause visual impairment, hypothalamic-pituitary dysfunction, and headaches. In this study patients were evaluated pre- and postoperatively to ascertain the clinical significance of surgical intervention on endocrine and visual improvement. Factors correlated with cyst recurrence were also evaluated. A retrospective analysis was conducted in 160 patients with Rathke cleft cysts who were treated between 1984 and 1995 and completed at least a 5-year follow-up period. Of these 160 patients, 118 initially exhibited symptoms of visual impairment or endocrine dysfunction, became symptomatic during the follow-up period, or were found to have cyst enlargement. These 118 patients underwent transsphenoidal surgery. Forty-two patients with incidental lesions that demonstrated no growth on magnetic resonance (MR) images were followed up without an operation. Complete resection, as observed on MR images 3 months postoperatively, was obtained in 114 (97%) of 118 patients. Vision improved postoperatively in 57 (98%) of 58 patients. Hypogonadism improved in 11 (18%) of 62 patients, growth hormone deficiency resolved in 14 (18%) of 78 patients, and hypocortisolemia resolved in one (14%) of seven patients. Twenty-two patients (19%) began to exhibit symptoms of diabetes insipidus, which had not been present preoperatively. The total 5-year recurrence rate was 18% (21 of 118 patients), with 12 patients requiring a repeated operation. Surgical and pathological factors that were found to be statistically associated with recurrence were the use of a fat and/or fascial graft for closure (p < 0.01) and the presence of squamous metaplasia in the cyst wall (p < 0.01). The extent of resection of the cyst wall was not associated with an increased rate of recurrence. In 42 (69%) of 61 patients the incidental cysts did not progress on imaging studies or clinically. This is the largest series of patients with symptomatic Rathke cleft cysts who received operative intervention and participated in the longest postoperative follow up reported in the literature. The high recurrence rate (18%) supports the theory that a relationship exists between a symptomatic Rathke cleft cyst and craniopharyngioma. Improvements in visual and endocrine dysfunction can be expected after surgical decompression of the optic apparatus and the hypothalamic-pituitary axis.