Rathke's Cleft
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2021 ◽  
Vol 12 ◽  
Author(s):  
Shenzhong Jiang ◽  
Zhaojian Wang ◽  
Yan You ◽  
Renzhi Wang ◽  
Xinjie Bao

In this article, we present a 31-year-old female who presented with intermittent headache and oligomenorrhea of over 10 years’ duration. Imaging revealed a large suprasellar mass with sellar extension. The patient underwent an endoscopic endonasal trans-sphenoidal surgery to resection of the mass. Clinical, radiological, and operative findings from this patient were initially considered to be Rathke’s cleft cyst (RCC). However, postoperative histological examinations revealed a mature cystic teratoma. No radiotherapy was performed after surgery. At the most recent follow-up, approximately 1 year later, the patient is doing well with no headache and no recurrence of the teratoma.


Author(s):  
Amalina Che Din ◽  
Celine Fong ◽  
Chon Sum Ong

The occurrence of symptomatic Rathke's cleft cyst (RCC) apoplexy is extremely rare. This is often misdiagnosed due to similar presentations to subarachnoid haemorrhage and pituitary apoplexy. This case highlights an excellent example of similar clinical presentation and serves as a learning case for clinicians. A 40-year-old lady presented to a district hospital with 9 days of worsening severe headache associated with blurring of vision, photophobia, stiff neck, nausea and vomiting. Nuchal rigidity and Brudzinski’s positive. Blood test showed hyponatremia, raised inflammatory markers and normal dynamic pituitary function test. CT Head demonstrated no evidence of space-occupying lesion or intracranial haemorrhage. Lumbar puncture showed xanthochromia positive consistent with subarachnoid haemorrhage. MRI head advised by Neurosurgery team and revealed a focal lesion involving anterior pituitary macroadenoma with mass effect on optic chiasm with possible haemorrhage within. Further assessment in tertiary hospital confirmed loss of visual acuity and field deficit. Patient underwent emergency endoscopic transnasal transsphenoidal resection of apoplectic tumour and repair of CSF leak with graft from thigh. Histopathology report showed a Rathke’s cleft cyst with squamous metaplasia. Post operatively, the patient developed sinusitis which fully recovered, and MRI showed good decompression. The author demonstrated a rare case of symptomatic RCC which was initially presumed to be pituitary apoplexy. Radiology imaging and treatment approach for both conditions are quite similar and can only be differentiated by histopathology. Further research is required to identify the causes and risk factors of RCC apoplexy to aid early detection and diagnosis.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S23


2021 ◽  
Vol 12 ◽  
pp. 455
Author(s):  
Jefferson Trivino-Sanchez ◽  
Pedro Henrique Costa Ferreira-Pinto ◽  
Elington Lannes Simões ◽  
Felipe Gonçalves Carvalho ◽  
Diego Rodrigues Menezes ◽  
...  

Background: Spinal dural arteriovenous fistula (SDAVF) is the most frequent vascular malformation of the spine and accounts for approximately 70% of all vascular spinal malformations. In rare cases, SDAVF rupture and subsequent subarachnoid hemorrhage or intramedullary hematoma may occur. The aim of this article is to present a fatal case of SDAVF rupture after a Rathke’s cleft cyst (RCC) endoscopic resection. Case Description: An 80-year-old female was referred to our hospital with a clinical presentation of bilateral reduction in visual acuity, bitemporal hemianopsia, and sellar magnetic resonance imaging (MRI) highly suggestive of RCC. After the first endonasal endoscopic surgery, the cyst was partially removed and vision improved. No signs of cerebrospinal fluid (CSF) leak were observed. After 1 year, the patient returned because of RCC recurrence and decreased visual acuity. In the second procedure, the lesion was totally resected and CSF leak was observed. A nasoseptal flap was rotated to cover the skull base defect. The patient developed subtle paraparesis followed by paraplegia on the 4th postoperative day. The dorsal spine MRI revealed a T3-T4 intramedullary hematoma. A dorsal laminectomy was performed and a SDAVF was observed. During microsurgery, at the right T3 nerve root level, an arteriovenous shunting point was identified, coagulated, and divided. The intramedullary hematoma was evacuated. The patient developed neurogenic and septic shock and died. Conclusion: Venous hypertension, venous wall fragility, and venous thrombosis seem to be the main factors involved in SDAVF rupture. In this particular case, reduction of the extravascular pressure and sudden variation in the pressure gradient caused by sustained CSF leak, also appeared to play an important role in SDAVF rupture. It may represent one more complication related to radical resection of RCC.


Pituitary ◽  
2021 ◽  
Author(s):  
J. Warmbier ◽  
D. K. Lüdecke ◽  
J. Flitsch ◽  
M. Buchfelder ◽  
R. Fahlbusch ◽  
...  

AbstractInflammatory pituitary lesions account for 1.8% of all specimens from the German Pituitary Tumor Registry. They occure in 0.5% of the autoptical specimens and in 2.2% of the surgical cases. Women are significantly more often affected than men and are often younger when first diagnosed. In general, primary and secondary inflammation can be distinguished, with secondary types occurring more frequently (75.1%) than idiopathic inflammatory lesions (15.4%). In primary inflammation, the lymphocytic type is more common (88.5%) than the granulomatous type of hypophysitis (11.5%). The most common causes of secondary inflammation are Rathke’s cleft cysts (48.6%), followed by tumors (17.4%) such as the craniopharyngioma (9.1%), adenoma (5.5%) or germinoma (2.0%). More causes are tumor-like lesions (7.1%) such as xanthogranuloma (3.5%) or Langerhans histiocytosis (3.5%), abscesses (5.5%), generalized infections (5.1%), spreaded inflammations (4.7%) and previous surgeries (4.0%). In 1.6% of all specimens the reason for the inflammation remains unclear. The described classification of hypophysitis is important for specific treatment planning after surgery.


2021 ◽  
Author(s):  
Emre Gezer ◽  
Burak Çabuk ◽  
Büşra Yaprak Bayrak ◽  
Zeynep Cantürk ◽  
Berrin Çetinarslan ◽  
...  

Abstract PurposeHypophysitis (HP) is a rare disease which develops secondary to chronic or acute inflammation of the pituitary gland and may cause symptoms related to pituitary dysfunction and mass compression. Lymphocytic HP is the most common subtype of primary HP, while xanthomatous HP (XHP) is considered the rarest form, with 35 reported cases, to date. Case ReportA 35-year-old woman was initially admitted to a Gynecology clinic with a 2-year history of amenorrhea and headache. She was started on cabergoline 0.5 mg twice a week for macroprolactinoma. Due to persistent amenorrhea with low gonadotropins, she was referred to our Endocrinology clinic. Her pituitary function profile revealed panhypopituitarism and a 13 x 11 x 12 mm sized sellar mass with diffuse enhancement which sustained toward the infundibulum and dura was observed on the gadolinium-enhanced pituitary MRI. The patient underwent an endoscopic endonasal transsphenoidal approach for tumor resection and thick yellowish fluid draining from the lesion was observed. The histopathological diagnosis was reported as a rupture of an Rathke’s cleft cyst and a xanthomatous hypophysitis. The surgery did not improve the symptoms/pituitary functions, however, headache recovered immediately after the first dose of high dose methylprednisolone treatment. ConclusionThe inflammatory process in a xanthomatous lesion may actually be a secondary response to mucous fluid content release from a ruptured cyst, thus recommended to classify XHP as secondary hypophysitis. Since the differentiation of XHP from other pituitary tumors may be challenging preoperatively, surgery is the major diagnostic tool and also, the most recommended therapeutic option.


Author(s):  
Michael Zhang ◽  
Anil K. Mahavadi ◽  
Michael L. Deftos ◽  
Arshad Ali ◽  
Harminder Singh

Abstract Objective Sellar xanthogranulomas (XGAs) are a rare pathological subtype of hypophysitis reflecting a degenerative process of Rathke's cleft cyst with predilection in young adults. While the histological features have been described, there is limited discussion on the technical expectations in surgical management. We present the clinical, radiographic, and surgical features of the third literature-reported XGA in the pediatric population. Setting The patient was a 17-year-old boy who first identified by ophthalmologically confirmed peripheral vision loss. Subsequent endocrine workup identified delayed-onset puberty and hypopituitarism. Magnetic resonance imaging (MRI) showed a nonenhancing 2.6-cm T1 and T2 sellar-based hyperintense mass with suprasellar extension and mass effect on the optic chiasm. A small T1 hypointense encased nodule was also seen (Fig. 1). Consent for resection was obtained. Results Intraoperatively the tumor was firm and adherent, requiring piecemeal removal. Radiofrequency ablation enabled ergonomic debulking and minimize thermal injury (Fig. 2).1 We used initial settings of 25 W, equivalent to 55 W. A cystic component with motor oil–like fluid was encountered and decompressed. The tumor was notably very adherent to the optic nerve and infiltrated the stalk, requiring its truncation. Closure was achieved by fat graft dead space plugging, fascia lata underlay, Medpor gasket seal, and nasoseptal flap. Conclusion Pathology confirmed dense fibrous tissue with features of chronic inflammation, cholesterol clefts, hemosiderin pigment, multinucleate giant cells, and foamy macrophages. Additional cyst wall sampling identified squamous and ciliated epithelial lining, collectively consistent with Rathke's cleft cyst and xanthogranulomatous reaction. These lesions can undergo surgical cure with resection, most commonly by transsphenoidal approach.The link to the video can be found at: https://youtu.be/S2n5iQ3aFgc.


2021 ◽  
Vol 146 ◽  
pp. 157-161
Author(s):  
Kohei Tsujino ◽  
Naokado Ikeda ◽  
Seigo Kimura ◽  
Akira Higashiyama ◽  
Motomasa Furuse ◽  
...  

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