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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Preliminary study of endocrine dysfunction in patients with Rathke's cleft cyst / 中华内分泌代谢杂志

A retrospective study of endocrine dysfunction in 65 patients with Rathke's cleft cyst (RCC) was conducted. Before surgical operation, most patients with RCC had various degrees of pituitary dysfunction.Impaired gonadotropin secretion and prolactinemia were the prevalent abnormalities. Erectile dysfunction or diminished libido in male and menstrual disorder or galactorrhea in female were frequently seen. The panhypopituitarism appeared in a few patients with RCC. The patients would benefit by surgical removal, however,attention should be paid to avoid new damage of the pituitary gland during operation.

Rathke's cleft cyst and partial feet adactyly: an unusual association

A 53 year-old woman presented a recurrent bifrontal headache of 2 years duration and bilateral progressive visual disturbance. The clinical and neurological examination showed a bilateral feet adactyly and bitemporal hemianopsia. The brain MRI demonstrated a Rathke's cleft cyst. The patient was operated by a transnasal endoscopic approach. It seems that this unusual association has never been described before.

Mulher de 53 anos com história recorrente de cefaléia com duração de 2 anos bilateral e progressiva, acompanhada de distúrbios visuais. O exame clinico e neurológico mostrou uma adactilia dos pés e hemianopsia bitemporal. A ressonância nuclear magnética cerebral mostrou um cisto de Rathke. A paciente foi operada por via transnasal endoscópica. Aparentemente esta é a primeira vez que esta associação é descrita na literatura.

A case of lymphocytic hypophysitis associated with Rathke's cleft cyst / 대한내과학회지

Lymphocytic hypophysitis is a rare disorder characterized by focal or diffuse inflammatory infiltration and destruction of the pituitary gland. It has classically been described as affecting female patients during peripartum period and its pathogenesis has been attributed with autoimmunity. Pressure symptoms and visual impairment may arise from the sellar mass lesion, and mass effect, involvement of the pituitary stalk, and pituitary destruction may result in endocrine dysfunction. 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. In symptomatic Rathke's cleft cysts, the common presentations include headache, pituitary dysfunction, and visual loss caused by compression of the optic chiasm in the event of suprasellar extension. Recently we experienced a case of lymphocytic hypophysitis with Rathke's cleft cyst resected by transsphenoidal approach under the preoperative impression of pituitary adenoma. This coexistence is exceedingly rare event, and to our best knowledge, this may be the first case of lymphocytic hypophysitis with Rathke's cleft cyst in Korea.

Transient Isolated Trochlear Nerve Palsy Associated with Rathke's Cleft Cyst

We report the case of a 52-year-old woman who developed vertical diplopia of 1-days duration. Neuro-ophthalmological testing revealed left trochlear nerve palsy, and sellar MRI revealed a 1.5 cm-sized pituitary mass lesion, a Rathke's cleft cyst. The diplopia disappeared spontaneously after 6 days.

A Case of Klinefelter's Syndrome with Rathke's Cleft Cyst / 대한내분비학회지

Klinefelter's syndrome is one of the most common forms of primary hypogonadism presenting with gynecomastia, azospermia and increased follicle-stimulating hormone. It is well known that this syndrome has an increased incidence of neoplasia, especially breast cancer and extragonadal germ cell tumors. However, it is rarely associated with an intracranial tumor of maldevelopmental origin, especially in the suprasellar area. We report, for the first time, a case of Klinefelter's syndrome, with a Rathke's cleft cyst is the patient was a 32-year-old male who was known to have an incidentaloma form brain computed tomography, which was clinically diagnosed as a suprasellar tumor. After operating, the suprasellar mass was confirmed as a Rathke's cleft cyst, and his hormonal abnormality, an elevated level of follicle-stimulating hormone, was not normalized. Therefore, we performed chromosomal analysis, and diagnosed Klinefelter's syndrome with the XXY karyotype.