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.

Lesiones sincrónicas selares: adenoma hipofisario y quiste de la bolsa de Rathke / Collision sellar lesions: pituitary adenoma and Rathke cleft cyst

Resumen El quiste de la bolsa de Rathke (QBR) es una entidad benigna de crecimiento lento que proviene del remanente del ectodermo primitivo. Presenta un origen común con los adenomas hipofisarios (AH), sin embargo, la presentación sincrónica de un AH y un QBR es infrecuente. Presentamos el caso de una mujer de 41 años con enfermedad de Cushing. Se realizó resonancia magnética con el hallazgo de dos lesiones en región selar. Se hizo un abordaje transnasal endoscópico, con exéresis completa de ambas. El informe de anatomía patológica reveló un AH corticotropo y un QBR. Tuvo una remisión clínica analítica endocrinológica a los seis meses postquirúrgicos. Ante el hallazgo de una imagen quística a nivel selar concomitante con un adenoma hipofisario, debe ser considerada la posibilidad diagnóstica de un QBR.

Abstract Rathke's cleft cyst (RCC) are a slow-growing, benign, cystic lesions that arises from the remnants of the primitive ectoderm and Rathke's pouch. They present a common origin with pituitary adenomas (PA), however, the concomitant presentation of a PA and a RCC rarely occur. We present a case of a 41-year-old female with Cushing's disease. Magnetic resonance imaging (MRI) showed two synchronic lesions in the sellar region. An endoscopic transnasal approach was performed, with complete excision of both. The histological studies revealed an ACTH secreting PA and a RCC. The patient presented clinical and endocrinological remission six months after surgery. With the presence of cystic lesion at the sellar region, and the concomitant finding of a pituitary adenoma, RCC should be considered.

Quiste de bolsa de Rathke e hiperprolactinemia, una causa infrecuente de amenorrea primaria / Rathke cleft cysts and hyperprolactinemia, an uncommon cause of primary amenorrhea

El quiste de la bolsa de Rathke es una lesión epitelial benigna de la región selar, formada a partir de remanentes embrionarios. La mayoría de los casos son asintomáticos, aunque pudiera presentarse con cefalea, disfunción hipofisaria y trastornos visuales, muy infrecuentemente como apoplejía hipofisaria. Se presenta el caso de una paciente que, habiendo presentado amenorrea primaria, se le realiza el diagnóstico de quiste de la bolsa de Rathke con hiperprolactinemia, logrando menarquia luego del tratamiento con cabergolina.

Rathke's cyst is a benign epithelial lesion of the sellar region, formed from embryonic remnants. Most cases are asymptomatic although it could present with headache, pituitary dysfunction and visual disorders, very infrequently as pituitary stroke. We present the case of a patient who, having presented primary amenorrhea, is diagnosed with Rathke's cyst with hyperprolactinemia, achieving menarche after treatment with cabergoline.

Magnetic resonance imaging characteristics and differential diagnosis of common sellar cystic lesions / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate magnetic resonance imaging (MRI) characteristic and differential diagnostic keypoints of common sellar regional cystic lesions with the purpose of improving differential diagnostic accuracy.@*Methods@#In total, 174 cases of the pathologically diagnosed cystic lesions in sellar region between March 2016 and June 2019 were included in the current retrospective analysis. These cases included Rathke′s cleft cyst (n=68), craniopharyngioma (n=48), pituitary adenoma (n=56) and arachnoid cyst (n=2) in the sellar region. The position, texture and morphology of leisions, signal of cystic and solid part, invasion of surrounding tissues and maximum diameter of cysts on the MRI images were evaluated and compared between different groups. SPSS 25.0 software was used for statistical analysis.@*Results@#There were several specific changes on several MRI sequences in Rathke′s cleft cysts, craniopharyngioma and pituitary adenoma groups. For Rathke′s cleft cysts, round shape, rare polycystic structure, lack of solid part and maximum diameter of cysts which was significantly less than that of craniopharyngioma and pituitary adenoma groups ((17.37±6.12) mm vs (30.29±13.51) mm vs (28.18±11.13) mm, t value was 6.680, 5.838, respectively, all P<0.05), were favorable diagnostic criteria. While, the intracystic nodules were mostly found in craniopharyngioma. There was no high signal intensity in cystic wall on T1WI and T2WI of pituitary adenoma. MRI signal of arachnoid cyst in sellar region was basically the same as CSF signal, with low signal intensity on T1WI and DWI, high signal intensity on T2WI, without enhanced signal.@*Conclusions@#Common sellar regional cystic lesions showed characteristic manifestations on different MRI sequences. Valuable information in the morphology, MRI signal intensity, maximum diameter measurements may improve accuracy of differential diagnosis for sellar regional cystic lesions.

Differential diagnosis between Rathke cleft cyst and cystic pituitary adenoma using MRI / 实用放射学杂志

Objective To make correct diagnosis for Rathke cleft cyst(RCC)and cystic pituitary adenomas(CPA)through retrospective analysis of characteristics of MRI.Methods RCC (n=30)and CPA (n=30)confirmed by surgery and pathology were analyzed retrospectively.Following characteristics of lesions were observed:morphology,size,location,range,T1WI signal intensity and patterns of enhancement,presence of intracystic fluid level,septum,nodule,hypointense rim on T2WI and change of the pituitary stalk.The independent sample t-test and χ2 test were used respectively to analyze differences between two groups of continuous variables and categorical variables.Results Most of RCC were oval,less than 2 cm3,under the optic chiasm and within the bilateral cavernous sinus,various for signal intensity,without or with thin-walled contrast enhancement.Intracystic nodule accounted for 40% of cases and there were double cystic nodules in 1 case.Obvious contrast enhancement of intracystic nodule was found in 1 case.Pituitary stalk was in center.Most of CPA were snowman shaped,bigger than 2 cm3,off middle line location,with compression of the optic chiasm and sellar base,had thick-walled contrast enhancement,with intracystic fluid level and septum,had hypointense rim on T2WI.The pituitary stalk was shifted.Conclusion The MRI findings of RCC and CPA are significant differences in the shape,size,intracapsular structure,enhanced performance and changes of surrounding structure.

Giant Sellar Xanthogranuloma after Surgical Treatment of Symptomatic Rathke's Cleft Cyst

Extremely massive sellar xanthogranuloma (XG) are rare, and the surgical outcome and prognosis are not well known. XG remain unknown whether they are derived from Rathke's cleft cysts (RCCs) or craniopharyngiomas (CPs) following extensive inflammation and metaplasia, to the point that no epithelium is readily identifiable. These lesions usually tend to occur in younger patients (mean 28.3 years), have a smaller diameter, and remain primarily intrasellar region with infrequent calcification. This 36-year-old man presented our hospital with visual deterioration. At the time of visit, there were no neurological problems other than visual field defect and hormonal disorder. He visited our hospital in 2007 due to headache and decreased vision, and underwent transphenoid surgery for pituitary RCC. Since then, he has received treatment at our hospital for postoperative hormonal disorders. Through preoperative imaging study, the author suspected CP and underwent surgery. During the operation, the adhesion of the tumor to the surrounding major neurovascular structures was severe in the naked eyes, but the tumor could be removed more easily than expected. The postoperative histological findings were confirmed as XG. The postoperative course was uneventful. Compared to the previous literature, this case is a case where the size of XG is very large in a sellar region and it can be proved that it originated from the RCC. And regular follow-up is necessary to confirm the prognosis after surgery.

Central Diabetes Insipidus Linked to Rathke's Cleft Cyst, Polyuria in a 17-year-old Girl

A 17-year-old girl presented with polyuria (7 L/day) and polydipsia for one year. Initial urine osmolality was 113mOsm/kg H₂O. Following 6 h of fluid restriction, serum plasma osmolality reached 300mOsm/kg H₂O, whereas urine osmolality was 108mOsm/kg H₂O. Urine osmolality was increased by 427% from 108 to 557mOsm/kg after vasopressin challenge. The patient was diagnosed with central diabetes insipidus, possibly derived from the atypical occupation of a Rathke's cleft cyst at the pituitary stalk following magnetic resonance imaging with enhancement. She was discharged with desmopressin nasal spray (10 µg); urine output was maintained at 2-3 L/day, and urine osmolality was >300 mOsm/kg. Additional pituitary image studies and evaluation of hypopituitarism should be included in the differential diagnosis of patients with central diabetes insipidus.

Rathke’s cleft cyst presenting as bilateral optic atrophy.

PURPOSE: Reporting a case of Rathke’s cleft cyst presenting as bilateral Optic atrophy. Optic atrophy could have been prevented with appropriate surgery if the patient would have consulted ophthalmologist earlier. Case report: A 23 year-old male presented with bifrontal headache and diminution of vision over 6 months period. On fundus examination right eye showed partial optic atrophy and left eye total optic atrophy. MRI Brain showed well defined lobulated cystic lesion in the sella with suprasellar extension, peripheral rim calcification causing widening of sella and displacing anterior cerebral artery and optic chiasma superiorly. Histopathology of the biopsied material was suggestive of Rathke’s cleft cyst. Endoscopic endonasal transsphenoidal sinus surgery was done and biopsy was sent which suggested Rathke’s cyst. Following surgery there was improvement in the field of vision in right eye. Conclusion: Early diagnosis and removal of a Rathke’s cleft cyst has a good prognosis in visual acuity and visual field. Patient ended with optic atrophy because of delayed consultation for headache.

The clinical analysis of 62 cases of Rathke's cleft cysts treated by microsurgical transsphenoidal surgery / 中华显微外科杂志

Objective To discuss the preoperative diagnosis and the effect of microsurgical transsphenoidal surgery of pituitary Rathke's cleft cysts.Methods Retrospective analysis was performed from January,2011 to May,2015 on 62 cases of Rathke's cleft cyst which confirmed by surgery and pathology at the First Affiliated Hospital of Sun Yat-sen University.Sixty-two cases were performed by the surgery of transsphenoidal approach.Results There were 50 cases with a correct preoperative diagnosis of and consider Rathke's cleft cyst,12 cases of misdiagnosis.Postoperative follow-up was performed within 6-12 months,and the patient's clinical symptons were improved in different degrees,1 case of recurrence,no deaths and serious complications.Conclusion The diagnosis of Rathke's cleft cyst need for comprehensive considerations from many aspects,and the improvement of diagnosis rate base on the clinical features,endocrine examination,and imaging data.The microsurgical transsphenoidal surgery is safe and effective treatment for Rathke's cleft cyst.

Spontaneous Involution of Rathke’s Cleft Cysts without Visual Symptoms

BACKGROUND: There have been various reports in the literature regarding the conservative management of pituitary apoplexy, pituitary incidentalomas and Rathke cleft cysts (RCCs). However, to the best of our knowledge, spontaneous involution of cystic sellar mass has rarely been reported. We report 14 cases of cystic sellar masses with spontaneous involution. METHODS: A total of 14 patients with spontaneous regression of cystic sellar masses in our hospital were included. The median age was 35 years (range, 5–67), and 8 patients were male. Clinical symptoms, hormone study and MRI were evaluated for all patients. The initial MRI showed all 14 patients with RCCs. Eight patients were presented with sudden onset of headache, and 1 patient with dizziness. Another patient, a 5-year-old child, was presented with delayed growth. Three patients had no symptoms via regular medical work up. All 14 patients had no visual symptoms. The follow-up period ranged from 5.7 to 42.8 months, with the mean of 17.3 months. RESULTS: The mean initial tumor size was 1.29 cm³ (range, 0.05 to 3.23). After involution, the tumor size decreased to 0.23 cm³ (range, 0 to 0.68) without any treatments. Repeated MRI showed a spontaneous decrease in tumor volume by 78% (range, 34 to 99). The initial MRI showed that the tumor was in contact with the optic chiasm in 7 patients, while compressing on the optic chiasm in 3 patients. Five patients were initially treated with hormone replacement therapy due to hormone abnormality. After the follow-up period, only 2 patients needed a long-term hormone replacement therapy. CONCLUSION: The spontaneous involution of RCCs is not well quantified before. Their incidence has not been well demonstrated, but this phenomenon might be underreported. Conservative management can be a treatment option in some RCCs without visual symptoms, even in those that are large in size and in contact with the optic nerve via imaging study.

Endoscopic endonasal transsphenoidal surgery of symptomatic Rathke′s cleft cyst in sellar region / 重庆医学

Objective To explore the clinical characteristic of Rathke′s cleft cyst and assess the effect of endoscopic en-donasal transsphenoidal surgery .Methods A retrospective research was performed on 9 patients who underwent endoscopic en-donasal transsphenoidal surgery .Neural endoscopic surgery by single nostril transsphenoidal approach ,partial resection of the cyst wall with drainage of the intracystic contents was performed in all patients .Results Symptoms in all patients were resolved or alle-viated .No CSF rhinorrhea ,permanent diabetes insipidus and hypopituitarism happened .All patients were followed up for 4 to 12 months and no recurrence was found .Conclusion Endoscopic endonasal transsphenoidal surgery is a effective method for symptom-atic Rathke′s cleft cysts .

A Case of Rathke Cleft Cyst Apoplexy / 계명의대학술지

Rathke cleft cysts (RCCs) are benign epithelial lesions of the sellar and suprasellar region. Most RCCs remain clinically silent throughout an individual's life. Symptomatic patients with RCCs manifest headaches, endocrinopathies, and visual disturbances secondary to parasellar extension. Rarely, RCCs can present in a manner similar to pituitary tumor apoplexy. One such case is reported herein. This 53-year-old man presented with acute headache, vomiting, and partial hypopituitarism. Surgical drainage of the cyst contents via a transnasal route confirmed the diagnosis of RCC and pituitary infarction.

Rathke cleft cysts in 22 children diagnosed by pituitary magnetic resonance / 中华实用儿科临床杂志

Objective To evaluate the clinical features of Rathke cleft cysts(RCCs) in children diagnosed by pituitary magnetic resonance(MR) and their features on MR.Methods Twenty-two children with RCCs aged 2-18 years old who visited the Affiliated Hospital of Qingdao University Medical College between Jan.2002 and Feb.2012 were enrolled.RCCs was conformed by pituitary MR.The clinical symptoms and imaging features were reviewed retrospectively.Results The clinical presentation of symptomatic children were as follows:endocrinopathy in 13 cases (59.1％),headache in 5 cases(22.7％) and visual disturbance in 1 case(4.5％) and variety of symptoms in 3 cases (13.6％),which including 1 case of short stature and dysgenitalism,1 case of type 1 diabetes with electrolyte disorder and the other of headache associated with visual impairment.Endocrinopathy included short stature 5 cases(22.7％),precocious puberty 4 cases(18.2％)and diabetes insipidus 4 cases(18.2％).Generally,RCCs appeared various on Tl-weighted MR,whereas on T2-weighted sequences the signal intensity was mostly high.High signals in the T1-weighted image on brain MR were related to pituitary hormone deficiency.Hypointensity of the cysts in T1-weighted was appeared when enhanced images.Conclusions The most common clinical manifestation of children with RCCs is endocrinopathy.Pituitary MR shows a certain characteristics and it is favorable in agreement with pathological diagnosis.MR may be of predictive value for the preoperative diagnosis.

Lesiones quísticas de línea media de base de cráneo anterior / Cystic lesions in midline of anterior skull base

Las lesiones quísticas en línea media del segmento posterior de la base de cráneo anterior corresponden mayoritariamente a malformaciones congénitas, cuya ubicación puede ser selar, supraselar o intraesfenoidal. Generalmente asintomáticas, pueden presentar síntomas determinados por su crecimiento y/o el compromiso mecánico sobre las estructuras adyacentes. Su diagnóstico suele realizarse producto de un hallazgo imagenológico o endoscópico, constituyendo la resonancia magnética la mejor alternativa ante una aproximación diagnóstica inicial. Estas lesiones quísticas presentan un comportamiento benigno y no todas requieren un manejo quirúrgico, siendo suficiente en la gran mayoría de ellas, un seguimiento clínico. La necesidad de cirugía deberá decidirse en cada caso y depende principalmente de la sintomatología producida por la compresión de estructuras vecinas e hipertensión endocraneana. El abordaje quirúrgico endoscópico extendido es el método resectivo de elección. Presentamos una serie de casos clínicos, correspondiente a cinco pacientes con lesiones quísticas en línea media de base de cráneo anterior. Tanto la sintomatología como el diagnóstico etiológico tuvieron una presentación diversa en la serie expuesta; independiente de lo cual, todos fueron manejados exitosamente por cirugía endoscópica. Presentamos finalmente, una propuesta de diagnóstico inicial, basado en el estudio imagenológico con resonancia magnética de estas lesiones.

Cystic lesions in the midline of the anterior skull base are rare and mostly congenital malformations. Usually asymptomatic, symptoms can be determined by their growth and/or mechanical compromise on adjacent structures. The diagnosis is usually the result of imaging or endoscopic findings, MRI is the best alternative for the initial diagnostic approach. These cystic lesions are benign xx and not all require a surgical management. Some of them require a watchfull follow up. If they need surgery the expanded endoscopic approach is the method of choice in our hands. We present a case series, correspondent to five patients with cystic lesions in the midline of the skull base. All these were successfully managed by an extended endoscopic approach. Finally, we presented a proposal for initial diagnosis approach, based on the MRI findings of these lesions.

Diagnosis and microsurgical treatment of symptomatic rathke cleft cysts in 23 cases / 第二军医大学学报

Objective To discuss the diagnosis and surgical treatment of symptomatic Rathke cleft cysts (RCCs). Methods Twenty-three symptomatic RCCs, who were verified by microsurgeries and pathological examination from Jan. 2001 to Oct. 2010, were retrospectively analyzed. Nine of them were males and fourteen of them were females. The main clinical manifestations included headache (65. 2%), visual disturbance (34. 8%) and pituitary dysfunctions (47. 8%). The magnetic resonance imaging (MRI) showed 10 pure intrasellar lesions, 10 lesions of intrasellar with a suprasellar extension, and 3 pure suprasellar lesions. Sixteen cases were treated by transsphenoidal microsurgery and 7 were treated by craniotomy. The contents of the cysts were drained totally during the operation, and part of or the total cyst wall were safely moved and kept open. Results Postoperative follow-up period ranged from 14 months to 10 years. All the 8 patients were released from visual disturbance. Headache was improved in 13 of the 15 patients and pituitary dysfunction was improved in 7 of the 11 patients. Recurrence was noted in 1 patient. Conclusion Preoperative definite diagnosis for the symptomatic RCCs is difficult due to a variety of clinical manifestations and MRI signals. Most of the lesions can be treated via transsphenoidal approach. Transcranial approach is necessary for the pure suprasellar RCCs. Microsurgery is an effective strategy for the treatment of symptomatic RCCs, because it can safely relieve the symptoms and make definite diagnosis.

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.

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.

Hyponatremia in a Patient with a Sellar Mass / 전남의대학술지

A 59-year-old man with confused mental status was admitted to our hospital. Laboratory reports showed him to have severe hyponatremia, and additional studies revealed panhypopituitarism. Brain magnetic resonance imaging showed a sellar cystic lesion, which consisted of a Rathke cleft cyst. Thus, the mass effect of the Rathke cleft cyst resulted in panhypopituitarism and finally induced euvolemic hyponatremia. On the basis of these results, supplementation with thyroid hormone and glucocorticoid was started, and the patient's serum sodium level was gradually corrected and maintained within the normal range. Here, we report this case of euvolemic hyponatremia caused by a Rathke cleft cyst.

Hyponatremia in a Patient with a Sellar Mass / 전남의대학술지

A 59-year-old man with confused mental status was admitted to our hospital. Laboratory reports showed him to have severe hyponatremia, and additional studies revealed panhypopituitarism. Brain magnetic resonance imaging showed a sellar cystic lesion, which consisted of a Rathke cleft cyst. Thus, the mass effect of the Rathke cleft cyst resulted in panhypopituitarism and finally induced euvolemic hyponatremia. On the basis of these results, supplementation with thyroid hormone and glucocorticoid was started, and the patient's serum sodium level was gradually corrected and maintained within the normal range. Here, we report this case of euvolemic hyponatremia caused by a Rathke cleft cyst.

Risk factor for pituitary dysfunction in children and adolescents with Rathke's cleft cysts / 소아과

PURPOSE: This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts. METHODS: Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients. The clinical, hormonal, and imaging features were reviewed retrospectively. RESULTS: The clinical presentation of symptomatic patients was as follows: headache (65%), endocrinopathy (61%), and visual disturbance (19%). Endocrinopathy included central precocious puberty (18%), diabetes insipidus (14%), general weakness (11%), and decreased growth velocity (7%). After surgery, hyperprolactinemia resolved in all patients, but growth hormone insufficiency, hypothyroidism, and diabetes insipidus did not improve. Pituitary insufficiency except gonadotropin abnormality correlated significantly with severe headache, visual disturbance, general weakness, and cystic size. Suprasellar extension of cysts and high signals in the T2-weighted image on brain MRI were related to hypothyroidism, hypocortisolism, and diabetes insipidus. Multivariable linear regression analysis showed that only general weakness was a risk factor for pituitary insufficiency (R2=0.549). CONCLUSION: General weakness is a risk factor for pituitary insufficiency in patients with Rathke's cleft cysts. When a patient with a Rathke's cleft cyst complains of general weakness, the clinician should evaluate pituitary function and consider surgical treatment.