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.

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.

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.

Radiological Characteristics in Rathke's Cleft Cyst

OBJECTIVE: The study is conducted to define the preoperative radiological findings of Rathke's cleft cysts (RCCs) differentiating these lesions from other sellar/parasellar cystic tumors. METHODS: A retrospective study of 65 patients with RCC patients from two institutes (53 cases / 12 cases) was performed. All patients had preoperative magentic resonance imaging(MRI) studies, and computed tomography(CT) studies were available in 25 patients. RESULTS: Calcification detectable on CT scanning was present in only 8% of RCC patients. Of the patients who were available for precontrast CT images, 48% had cysts of low attenuation, 28% had cysts of the same attenuation, and 24% had cysts of increased attenuation relative to the brain. Of the patients who had postcontrast CT images, 84% showed no significant change following contrast agent administration, and 16% demonstrated rim enhancement. MR imaging revealed multiple patterns. In T1-weighted sequences, 55.4% showed hyperintense signals, 27.7% showed hypointense signals, 12.3% showed isointense signals, and we saw a mixed-intense signal in 4.6%. In T2-weighed images, we observed a hyperintense signal in 60%, a hypointense signal in 20%, a mixed-intense signal in 13.8%, and an isointense signal in the remaining 6.2%. In Gd-DTPA enhanced images, 72.3% of the 65 patients showed no enhancement either of the cyst contents or of the cyst wall, and 27.7% demonstrated rim enhancement. Forty-five of the 65 patients were preoperatively diagnosed as RCCs. CONCLUSION: RCCs show variable CT and MRI characteristics. There is no unique finding for this pathology. Thus, even with CT and MRI studies, differential diagnosis with other cystic lesions of the sellar/parasellar region remains difficult.

Rathke Cleft Cysts:Comparative Study of Pathology,Clinic and Imaging / 实用放射学杂志

Objective To study the correlation among pathological,clinical and the imaging features(CT and MRI) of the Rathke cleft cysts.Methods CT,MRI and clinical findings of Rathke cleft cysts in 43 patients confirmed by operation and pathology were retrospectively studied.Results 27 cysts located at intrasella and suprasella and 16 cysts entirely located at intrasella.Cysts were round or oval in shape with definite borders.The size of 30 cysts exceed 10mm in diameter.On CT scans reviewed,the cysts were low density in 9 cases,hyper-density in 9 cases and isodensity or mixed density in 8 cases.The cyst's wall with calcification was seen in one.On postcontrast CT scans,6 cases showed circular and peripheral cyst's wall enhancement and others were no enhancement.On MR imaging,the lesions were low or isodense on T1WI and hyper-intensity on T2WI in 18 cases,both were high signal intensity on T1WI and T2WI in 6 cases,high signal intensity on T1WI and mixed signal intensity on T2WI in 4 cases.An intracystic nodule having high signal intensity on T1WI,and low or mixed signal intensity on T2WI was observed in 4 cases.On contrast-enhanced MR imaging,enhancement of the cyst's wall was shown in 9 cases.During surgery,the lesions were noted to have a cyst of semisolid consistency,and cystic contents were described from CSF-like clear fluid in 10 cases,jellied-like brown mucoid fluid in 15 cases,caseous-like mucoid fluid in 12 cases,machine oil-like mucoid fluid in 6 cases.At histopathology,a part of cystic fluid included cholesterol crystal and necrotic debris.Cholesterol clefts and hemosiderin pigment,and granuloma were shown by staining with HE in 11 cases.The PAS staining was positive in 16 cases,cystic fluid contained partial mucopolysaccharides and protein.Conclusion Typical Rathke cysts can be dignosised in the preoperative,the findings of CT and MRI are not specific in atypical cysts.The imaging features were different with the fluid component of Rathke cleft cysts..

Clinical Experience of Rathke's Cleft Cysts

Rathke's cleft cysts(RCCs) are classically described as benign epithelium-lined intrasellar cysts containing mucoid material. They are thought to orignate from remamants of the Rathke's pouch. These cysts are most frequently small and asymptomatic, and generally regarded as rare lesions. With the introduction of modern neuroimaging technology, they are being diagnosed much more frequently. We reviewed clinical, radiographic, and pathological findings in nine patients with symptomatic RCCs who were treated at out hospital from June 1989 to October 1994. The lesions were more often encountered in female than male patients, and the mean age at presentation was 25 years. The most common symptom and sign were headache, visual disturbance, and pituitary dysfunction. Two cysts were entirely intrasellar and seven had intra- and suprasellar components. Pre-enhanced CT scans revealed low dense or isodense lesions in five cases, and contrast-enhanced CT scans showed ring enhancement in two of five cases. MRI findings were varied with no characteristic pattern. Six patients underwent surgery by the transsphenoidal approach, and another three patients were treated by the transcranial approach. Partial excision and drainage of the cysts was most commonly performed, and there was no cyst recurrence. The cyst lining was usually composed of couboidal or columnar epithelium. Most symptoms and signs improved or resolved following surgery with the exception of hypopituitarism. Because of the relative safety and low recurrence rate, we think that partial excision and drainage of the cyst by the transsphenoidal approache is the treatment of choice.