scholarly journals Spontaneous Rupture, Disappearance, and Reaccumulation of a Rathke's Cleft Cyst

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Katrina Maniec ◽  
Joe C. Watson

Rathke's cleft cysts (RCCs) are benign epithelium-lined intrasellar cysts containing mucoid material and are believed to originate from the remnants of Rathke's pouch. Most are asymptomatic but may cause symptoms secondary to compression of adjacent structures such as visual disturbances and endocrinopathies, especially hypopituitary. Furthermore, inflammation such as an aseptic meningitis syndrome may be associated with these tumors, presumably resulting from leakage of cyst material into the subarachnoid space. We present a unique case of spontaneous rupture and complete disappearance of a known sella-suprasellar cyst associated with a severe headache syndrome, followed by cyst reaccumulation requiring surgery. Although this phenomenon is well accepted, to our knowledge, this is the first report of the complete disappearance of a Rathke's cyst presenting with the classic syndrome. Furthermore, it was remarkable how quickly it recurred and became symptomatic, providing evidence that an “empty sella syndrome” may indeed need clinical follow-up.

2014 ◽  
Vol 121 (4) ◽  
pp. 846-850 ◽  
Author(s):  
Jun Fan ◽  
Songtao Qi ◽  
Yuping Peng ◽  
Xi-an Zhang ◽  
Binghui Qiu ◽  
...  

Rathke's cleft cysts (RCCs) are benign cysts typically located in the sellar or suprasellar region; ectopic isolated lesions are extremely rare. The authors describe the case of a 25-year-old man with a giant symptomatic RCC arising primarily at the cerebellopontine angle (CPA), only the second case reported thus far. The patient presented with a 2-year history of right hearing impairment and tinnitus accompanied by vertigo and headache and a 2-week history of right facial numbness. Subsequently, he underwent total cyst removal via retrosigmoid craniotomy with a good recovery. He experienced no recurrence during a 64-month follow-up period. The possible pathogenesis, differential diagnosis, and surgical treatment of such cysts are discussed in this article. Isolated ectopic RCCs can arise from the ectopic migration of Rathke's pouch cells during the embryonic period. It is still difficult to distinguish ectopic RCCs from other cystic lesions of the CPA given the lack of specific imaging features. Aggressive resection of the cyst wall is not recommended, except when lesions do not closely adhere to adjacent structures.


2011 ◽  
Vol 165 (1) ◽  
pp. 33-37 ◽  
Author(s):  
R Trifanescu ◽  
V Stavrinides ◽  
P Plaha ◽  
S Cudlip ◽  
J V Byrne ◽  
...  

ObjectiveTo clarify the outcome of all cases of Rathke's cleft cysts (RCC) treated surgically and followed up in Oxford during a long-term period.Subjects and methodsThe records of all patients with RCC seen in the Department of Endocrinology between January 1978 and June 2009 were reviewed.ResultsA total of 33 patients (20 females, median age 43 years) were identified. At presentation, major visual field defects were detected in 58% of patients and gonadotrophin, ACTH and TSH deficiency in 60, 36 and 36% of patients respectively. Desmopressin treatment was required in 18% of patients. Treatment consisted of cyst evacuation combined with or without biopsy/removal of the wall. Post-operatively, visual fields improved in 83% of patients with impairment, whereas there was no reversal of ACTH or TSH deficiency or of diabetes insipidus. All but one subject had imaging follow-up during a mean period of 48 months (range 2–267). Cyst relapse was detected in 22% of patients at a mean interval of 29 months (range 3–48 months); in 57% of them, the recurrence was symptomatic. Relapse-free rates were 88% at 24-months and 52% at 48-months follow-up. At last assessment, at least quadrantanopia was reported in 19% of patients, gonadotrophin, ACTH and TSH deficiency in 50, 42 and 47% of patients respectively. Desmopressin treatment was required in 39% of patients.ConclusionsIn this study of patients with RCC and long-term follow-up, we showed a considerable relapse rate necessitating long-term monitoring. Surgical intervention is of major importance for the restoration of visual field defects, but it does not improve endocrine morbidity, which in the long-term affects a substantial number of patients.


1977 ◽  
Vol 44 (3) ◽  
pp. 137-140 ◽  
Author(s):  
E. Xistris ◽  
P. J. Sweeney ◽  
F. A. Gutman

2019 ◽  
Vol 130 (3) ◽  
pp. 831-837 ◽  
Author(s):  
Michelle Lin ◽  
Michelle A. Wedemeyer ◽  
Daniel Bradley ◽  
Daniel A. Donoho ◽  
Vance L. Fredrickson ◽  
...  

OBJECTIVERathke’s cleft cysts (RCCs) are benign epithelial lesions of the sellar region typically treated via a transsphenoidal approach with cyst fenestration and drainage. At present, there is limited evidence to guide patient selection for operative treatment. Furthermore, there is minimal literature describing factors contributing to cyst recurrence.METHODSThe authors conducted a retrospective analysis of 109 consecutive cases of pathology-confirmed RCCs treated via a transsphenoidal approach at a single center from 1995 to 2016. The majority of cases (86.2%) involved cyst fenestration, drainage, and partial wall resection. Long-term outcomes were analyzed.RESULTSA total of 109 surgeries in 100 patients were included, with a mean follow-up duration of 67 months (range 3–220 months). The mean patient age was 44.6 years (range 12–82 years), and 73% were women. The mean maximal cyst diameter was 14.7 mm. Eighty-eight cases (80.7%) were primary operations, and 21 (19.3%) were reoperations. Intraoperative CSF leak repair was performed in 53% of cases and was more common in reoperation cases (71% vs 48%, p < 0.001). There were no new neurological deficits or perioperative deaths. Two patients (1.8%) developed postoperative CSF leaks. Transient diabetes insipidus (DI) developed in 24 cases (22%) and permanent DI developed in 6 (5.5%). Seven cases (6.4%) developed delayed postoperative hyponatremia. Of the 66 patients with preoperative headache, 27 (44.3%) of 61 reported postoperative improvement and 31 (50.8%) reported no change. Of 31 patients with preoperative vision loss, 13 (48.1%) reported subjective improvement and 12 (44.4%) reported unchanged vision. Initial postoperative MRI showed a residual cyst in 25% of cases and no evidence of RCC in 75% of cases. Imaging revealed evidence of RCC recurrence or progression in 29 cases (26.6%), with an average latency of 28.8 months. Of these, only 10 (9.2% of the total 109 cases) were symptomatic and underwent reoperation.CONCLUSIONSTranssphenoidal fenestration and drainage of RCCs is a safe and effective intervention for symptomatic lesions, with many patients experiencing improvement of headaches and vision. RCCs show an appreciable (although usually asymptomatic) recurrence rate, thereby mandating serial follow-up. Despite this, full RCC excision is typically not recommended due to risk of hypopituitarism, DI, and CSF leaks.


2021 ◽  
Vol 24 (4) ◽  
pp. 315-321
Author(s):  
Khalid Mahmood ◽  
Muhammad Hassan Raza ◽  
Imran Ali ◽  
Omair Afzal

Objective:  To review and report the results in pure endoscopic endonasal trans-sphenoidal surgery done at our unit for pituitary adenomas (PAs) in last 8 years. Material and Methods:  We reviewed 403 consecutive patients who underwent pure endoscopic endonasal trans-sphenoidal surgery for newly diagnosed pituitary adenomas between August 2012 and July 2020 at our neurosurgical unit. Endocrinological, demographic features and outcomes, their complications, and duration of stay in hospital were assessed in these patients who were operated in our neurosurgical Unit. The Mean Follow-up on average was 3.5 months. Results:  403 consecutive cases were reviewed. Majority of cases were in the 4th decade of life at presentation. 227 (56%) were non-functioning pituitary tumors and 176 (44%) were hormone secreting pituitary adenomas. Thirty-one (7.7%) complications were observed in 28 post-operative patients. The most frequently observed complication was diabetes insipidus (temporary in 19 (5%) and permanent in 3 (0.7%) cases), cerebrospinal fluids leaks (5 cases) (5.7%), Syndrome of inappropriate antidiuretic hormone (1 case) (0.2%), internal carotid injury (1 case) (0.2%), Empty Sella syndrome (1) (0.2%) and post-operative cardiac complication (1 case) (0.2%). The Follow-up on average was 3.5 months. Conclusions:  The pure endoscopic endonasal trans-sphenoidal surgery of pituitary adenomas provides acceptable and reasonable results representing a safe alternative procedure to the traditional Trans-sphenoidal microscopic approach.


2018 ◽  
Vol 16 (6) ◽  
pp. 675-684 ◽  
Author(s):  
Michelle A Wedemeyer ◽  
Michelle Lin ◽  
Vance L Fredrickson ◽  
Anush Arakelyan ◽  
Daniel Bradley ◽  
...  

Abstract Background Limited data exist pertaining to outcomes following surgery for recurrent Rathke's cleft cysts (RCC). Objective To determine treatment outcomes in patients undergoing reoperation for recurrent or residual RCCs. Methods A retrospective analysis of 112 consecutive RCC operations in 109 patients between 1995 and 2017 was conducted. Results Eighteen patients underwent 21 RCC reoperations with a mean follow-up of 58 mo. Patient symptoms prior to reoperation included headaches (14, 66.7%) and vision loss (12, 57.1%). Thirteen of 18 patients (72.2%) required hormone supplementation prior to reoperation including 5 with diabetes insipidus (DI). Mean RCC diameter was 16 mm and 76% had suprasellar extension. Compared to index RCC cases, intraoperative cerebrospinal fluid leak repair was more common in reoperation cases (15/21, 71% vs 43/91, 47%, P = .05). There was 1 carotid artery injury without neurological sequelae, and 2 postoperative cerebrospinal fluid (CSF) leaks (9.5%). Rates of transient hyponatremia (3/10, 30% vs 4/91, 4.4%, P = .04) and transient DI (5/10, 50% vs 17/91, 18.7%, P = .04) were higher in the reoperation vs index group. Improved headaches and vision were reported in 4/12 (33%) and 8/12 (61.5%) of RCC reoperation patients, respectively. Two patients developed new permanent DI. A higher proportion of reoperation patients had RCC squamous metaplasia (24% vs 5.4%, P = .02) or wall inflammation (42.9% vs 2.2%, P &lt; .001) on pathological examination. CONCLUSION Reoperation for RCCs is generally safe at tertiary pituitary centers and often results in improved vision. Hypopituitarism is less likely to improve following reoperation for recurrent RCCs. Several histopathological features may help characterize “atypical RCCs” with a higher likelihood of recurrence/progression.


1980 ◽  
Vol 53 (1-2) ◽  
pp. 69-78 ◽  
Author(s):  
M. Baldini ◽  
L. Mosca ◽  
L. Princi

Neurosurgery ◽  
1979 ◽  
Vol 4 (1) ◽  
pp. 63-65 ◽  
Author(s):  
Lucas J. Martinez ◽  
Jewell L. Osterholm ◽  
Richard G. Berry ◽  
Francis K. Lee ◽  
Norman J. Schatz

Abstract Symptomatic Rathke's cleft cysts are uncommon. We present a case with suprasellar extension manifested by hypopituitarism and visual disturbances. The treatment was trans-sphenoidal evacuation and partial removal of the capsule. We suggest that the trans-sphenoidal approach to these lesions is usually adequate and that radical removal of the capsule is not necessary.


Author(s):  
Megha Mukundan ◽  
Prativa Sethi ◽  
Prasan Kumar Panda

Empty Sella syndrome with typical facies is a rare presentation of panhypopituitarism. Here, authors presented a case of 45-year- old male with a history of altered behaviour such as low mood, passivity, talkativeness with low-hoarse voice and spells of cries for three months. He also had headache, vomiting, abdominal pain, ataxic gait, and loss of libido. He had meningitis 10 years back since then he had multiple similar episodes. He had sparse thinned out hair, male pattern temporal balding, madarosis, brownish pigmentation of face and chest and dry coarse skin. Patient had hypotension but without hypoperfusion. Hormone levels including thyroid hormones, Follicle Stimulating Hormone (FSH), Luteinizing hormone (LH), total testosterone cortisol, and plasma Adrenocorticotropic Hormone (ACTH) were less than normal. Magnetic Resonance Imaging (MRI) brain suggested streak pituitary gland (empty sella). He was supplemented with required hormones. On follow-up, he improved significantly. Trio composite hypothyroidism, hypocortisolism, and hypogonadism have characteristic facies. This unique presentation of patient with blank look facies gives a hint of empty sella, thus leading the clinician to diagnose the disease through timely evaluation and work-up.


2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i43-i43
Author(s):  
Mohamed Arnaout

Abstract Introduction Management of primary empty sella syndrome (ESS) is generally remaining a neurosurgical challenge due to lack of a well standardized approach. Pseudo-tumor cereberi (Benign or idiopathic intracranial hypertension) is commonly associated condition. In this study, we have demonstrated the relationship and surgical plan and outcome of such cases. Patients and Methods: We retrospectively studied 24 patients with primary empty sella syndrome (ESS) for two years who were diagnosed radiologically as ESS. Fundus and other ophthalmological examinations were done. Lumbar puncture and cerebrospinal fluid (CSF) manometer were evaluated in those with papilledema. All patients’ data were collected and analyzed. Results Basically, 24 patients (18 females and 6 males) were radiologically diagnosed as EES. 13 females and only one male were having symptoms of BIH. 17 patients (70.83%) had headache as the first presentation. Second most common presentation in our study was visual in 14 patients (58.3%). Two patients (8.3%) had pituitary hypersecretion namely; growth and prolactin hormones. In those (58.3%) confirmed to have BIH Theco-peritoneal shunts were inserted. Incidental cases (29.17%) without symptoms were followed up. Conclusion Although (ESS) is a well-known radiological hallmark for BIH, in our study not all patients had BIH. Interestingly, pituitary hyperfunction may be the first presentation in some rare cases. Generally, natural history of that entity was benign. Frequent follow-up by neurosurgeons and increased awareness of associations are advised. We believe a more prospective large number cohort is important to outline the natural history.


Sign in / Sign up

Export Citation Format

Share Document