• Clinical and Experimental Pediatrics
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• v.53 no.7
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• pp.759-765
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• 2010

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 ($R^2$=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.

• Journal of Korean Neurosurgical Society
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• v.30 no.9
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• pp.1130-1133
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• 2001

Athke's cleft cysts are believed to be derived from remnants of Rathke's pouch, a dorsal invagination of the stomodeum. Although these cysts are characteristically small, asymptomatic and intrasellar in location, they occasionally provoke symtoms with enlargement to compress surrounding structures. It is characteristically lined by stratified squamous epithelium with keratinization on a layer of connective tissue. The cells of the anterior pituitary lobe, from which pituitary adenomas develop, are also formed by the proliferation of the anterior wall of Rathke's pouch. Thus, Rathke's cleft cyst and pituitary adenomas are considered to have a common ancestry. We report a rare case in which the preoperative diagnosis was pituitary adenoma, but the pathologic diagnosis was a combination of a Rathke's cleft cyst and a coincidental pituitary adenoma.

• Journal of Korean Neurosurgical Society
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• v.66 no.1
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• pp.82-89
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• 2023

Objective : Rathke's cleft cysts (RCCs) are nonneoplastic cysts. Most of them are asymptomatic and stable; when symptomatic, RCCs are surgically fenestrated and drained. However, the outcomes remain unclear. The authors evaluated the outcomes of RCC decompression. Methods : Between 2004 and 2019, 32 RCCs were decompressed in a single tertiary institution. The clinical characteristics, intraoperative findings, postoperative complications, and endocrinological and surgical outcomes were retrospectively reviewed. Patients who underwent sequential imaging at least twice and at least 12 months after surgery were included in the analysis. Results : Patients' mean age was 40.8±14.9 years, and 62.5% were women. The mean follow-up duration was 62.3±48.6 months. In 21 patients (65.6%), no residual cysts were identified on postoperative magnetic resonance imaging. Of the 18 patients with preoperative visual field defects, 17 (94.4%) experienced postoperative visual improvement. Postoperative complications included endocrinological deterioration in 11 patients (34.4%), permanent diabetes insipidus in 11 (34.4%), infection in four (12.5%), intrasellar hemorrhage in three (9.4%), and cerebrospinal fluid leak in two (6.3%). Follow-up images revealed cyst recurrence in nine patients (28.1%), an average of 20.4 months after surgery; in three patients, the cysts were symptomatic, and resection was repeated. Multivariable analysis revealed that postoperative endocrinological deterioration was the only independent factor associated with cyst recurrence (p=0.028; hazard ratio, 6.800). Conclusion : Our findings showed that although only cyst fenestration for decompression was performed to preserve pituitary function, more pituitary dysfunction occurred than expected. Besides, the postoperative hormonal deterioration itself acted as a risk factor for cyst recurrence. In conclusion, surgery for RCC should be more careful.

• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.82-85
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• 2018

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.

• Journal of Korean Neurosurgical Society
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• v.62 no.6
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• pp.723-726
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• 2019

We report two rare cases of spontaneously regressed Rathke's cleft cyst (RCC). A 52-year-old woman presented with headache. A pituitary hormone study was normal. Brain magnetic resonance imaging (MRI) showed a $0.45-cm^3$ cystic sellar lesion. The cyst was hyperintense on T1-weighed imaging and hypointense on T2-weighted imaging without rim enhancement, comparable to a RCC. Six months later, brain MRI showed no change in the cyst size. Without any medical treatments, brain MRI 1 year later revealed a spontaneous decrease in cyst volume to $0.05cm^3$. A 34-year-old woman presented with headache and galactorrhea lasting 1 week. At the time of the visit, the patient's headache had disappeared. Her initial serum prolactin level was 81.1 ng/mL, and after 1 week without the cold medicine, the serum prolactin level normalized to 11.28 ng/mL. Brain MRI showed a RCC measuring $0.71cm^3$. Without further treatments, brain computed tomography 6 months later showed a spontaneous decrease in cyst volume to $0.07cm^3$. Another 6 months later, brain MRI revealed that the cyst had remained the same size. Neither patient experienced neurological symptoms, such as headache or visual disturbance, during the period of cyst reduction. The RCCs in both patients underwent spontaneous regression without any medical treatment during a period of 6 months to 1 year. Although spontaneous regression of a RCC is rare, it is still possible and a sufficient follow-up period should be considered.

• Journal of Korean Neurosurgical Society
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• v.39 no.5
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• pp.329-334
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• 2006

Objective : In selected cases, the transsphenoidal approach[TSA] can be extended anteriorly to the tuberculum sellae, chiasmatic sulcus, and planum sphenoidale to obtain direct exposure of the suprasellar cisterns and its contents. We applied this modification of the TSA to various lesions of the presellar and suprasellar areas. We evaluate our clinical experience of this technique and review the related literature. Methods : From 1999 to 2004, we used the transsphenoidal supradiaphragmatic intradural approachs[TSIAs] in 9 patients who had various lesions at the pre- and suprasellar regions. Concomitant presellar extension of the bone window was performed with the sublabial or transnasal transseptal transphenoidal techniques. After removal of the lesions, sellar or anterior cranial floor was repaired with silicone plate substitute. Results : The TSIAs have been applied in the following cases : four tuberculum sellae meningiomas, two craniopharyngiomas, two Rathke's cleft cysts, and one non-functioning macroadenoma. The complications were one case of visual acuity decrease and one cerebrospinal fluid rhinorrhea. Conclusion : The TSIA is easily applicable through a minor modification of the standard TSA. It is suitable for removing lesions located in the presellar and suprasellar area adjacent to the pituitary stalk with minimal brain manipulation and decreased morbidity.

• Journal of Korean Neurosurgical Society
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• v.29 no.9
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• pp.1204-1208
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• 2000

Objectives : At the closure of the transsphenoidal approach(TSA), the proper sellar floor reconstruction plays an important role in preventing postoperative complications. The septal cartilage, perpendicular plate of nasal septum, and the sphenoid sinus bone are usually used to repair the sellar floor as a bone splint. The authors evaluate the usefulness of a silicone plate as a substitute for bone splint to close a defect of the sellar floor. Materials and Methods : A silicone plate was used to repair the sellar floor in 7 patients with sellar lesions which included four pituitary adenomas, two Rathke's cleft cysts and one metastatic tumor. Among seven cases, five cases underwent a standard TSAs and two received a extended TSAs. The trajectories of the approach were sublabial in four cases and endonasal routes in three cases. The silicone plate for implantation was cut to a size of slightly larger than that of bone window and inserted with a three-pronged fork, and then adjusted precisely. Results : In six patients, there were no complications which related to sellar floor reconstruction. A postoperative cerebrospinal fluid(CSF) rhinorrhea was observed in one patient with pituitary macroadenoma. Conclusions : From the authors' experience, the advantages of the silicone plate are its simplicity of molding to fit any size of sellar floor defects, and easy detection of previously created bone window at reoperation.