Journal of Clinical Neuroscience 18 (2011) 760–762
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Clinical Study
Endoscopic endonasal resection of symptomatic Rathke cleft cysts Tao Xie , Fan Hu , Yong Yu, Ye Gu, Xuejian Wang, Xiaobiao Zhang ⇑ Department of Neurosurgery, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China
a r t i c l e
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Article history: Received 26 August 2010 Accepted 15 October 2010
Keywords: Endonasal endoscopic approach Rathke cleft cyst Neuroendoscopy
a b s t r a c t We aimed to retrospectively evaluate the transsphenoidal endoscopic endonasal approach (EEA) for the resection of symptomatic Rathke’s cleft cysts (RCC) in 23 patients (11 male, 12 female, average age 43 years). The patients were followed-up for between 3 months and 36 months. Headache was the primary pre-operative symptom (15/23, 65%) and all patients with headache improved after surgery. Seven patients with initial visual symptoms (9/23, 39%) also improved after treatment. Three of the six patients with pre-operative pituitary dysfunction (6/23, 26%) showed post-operative improvement. There was no permanent pituitary dysfunction. One patient developed temporary diabetes insipidus and two patients had a post-operative infection. Three patients had post-operative cerebrospinal fluid leaks, two of which were repaired using the EEA and the other using a lumbar drain. Two patients had recurrent cysts and both patients refused reoperation. We concluded that the EEA is safe and effective in the treatment of symptomatic RCC. Fenestration and aspiration of the cysts with partial excision of the cyst wall is usually sufficient. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Rathke’s cleft cysts (RCC) are benign cystic lesions in the sellar or suprasellar region believed to be derived from remnants of Rathke’s pouch. Nonetheless, several studies have suggested that RCC might originate from endodermal cells, neuroepithelial cells or metaplastic anterior pituitary cells.1 In recent years, the introduction of MRI has undoubtedly made it simpler and more accurate to diagnose such cysts before surgery or incidentally. Most RCC seem to remain asymptomatic, however some cysts become symptomatic. When RCC are associated with headaches, endocrinological symptoms or visual disturbances, surgical treatment becomes necessary. The treatment for symptomatic RCC is primarily transsphenoidal surgery.2–6 In this study, we used an endoscopic endonasal approach (EEA) for fenestration and aspiration of RCC with partial excision of the cyst wall, and reviewed the surgical outcomes.
2. Patients and methods Twenty-three patients were included in the present study. These patients had undergone an EEA with the histological diagnosis of RCC between January 2007 and June 2010. They were aged between 15 and 77 years (median age, 43.3 years). There were 11 male and 12 female patients. Fifteen patients (15/23, 65%) had ⇑ Corresponding author. Tel.: +86 21 64041990x5047; fax: +86 21 64041990.
E-mail address:
[email protected] (X. Zhang). T. Xie and F. Hu contributed equally to this study.
0967-5868/$ - see front matter Ó 2010 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2010.10.014
experienced mild and chronic headache, nine (9/23, 39%) complained of visual impairment, while six (6/23, 26%) suffered from pituitary dysfunction (Table 1), including hyperprolactinemia (four patients), diabetes insipidus (two patients) and sexual disturbance (one patient). All patients underwent MRI of the pituitary region. Seven patients had intrasellar lesions, and one patient had a completely suprasellar lesion (Fig. 1), whereas the other 15 patients had an intrasellar mass with suprasellar expansion. Two patients also had nodules in the cysts. Post-contrast MRI showed that 11 patients had peripheral rim enhancement (Table 1). Surgical treatment of the cysts was performed via an EEA. A standard binaral approach was used to access the sphenoid sinus. The bilateral middle turbinates were lateralized and the natural sphenoid ostium on the left was opened to create wide bilateral sphenoidotomies. A small portion of the posterior nasal septum was then resected. Key anatomical landmarks (Fig. 2A) were identified by neuronavigation. The floor of the sella was carefully removed using a high-speed drill and a Kerrison rongeur. The dural opening was based on the position of the pituitary gland, as revealed by pre-operative imaging. Most cysts were accessed directly behind the adenohypophysis with minimal gland manipulation. Fenestration and aspiration of the RCC with partial excision of the cyst wall were then performed. Where a cerebrospinal fluid (CSF) leak had occurred, repair of the sella was performed by plugging it with abdominal fat. A small amount of biomedical fibrin sealant or a Foley balloon was used to support the floor of the sella. If the CSF leak occurred during surgery, a lumbar drain was inserted for 1 week.
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T. Xie et al. / Journal of Clinical Neuroscience 18 (2011) 760–762 Table 1 Results of 23 patients with symptomatic Rathke cleft cysts who underwent endoscopic endonasal resection Cyst location
No. of patients
Pituitary dysfunction
Headache
Visual impairment
Nodules on MRI (no. patients)
Peripheral rim enhancement on MRI (no. patients)
Post-operative complications (no. patients)
Recurrence
Sellar Sellar–suprasellar Suprasellar
7 15 1
4 1 1
4 11 0
1 8 0
0 2 0
3 8 0
Sinusitis (2) CSF leak (3) DI transient (1)
0 2 0
CSF = cerebrospinal fluid, DI = diabetes insipidus.
Three patients had post-operative CSF leaks, two of which were repaired using an EEA and the other using a lumbar drain. Two patients had recurrent cysts and both patients refused reoperation. One patient with a recurrence reported decreased visual acuity in his left eye. 4. Discussion
Fig. 1. (A) Pre-operative pre-contrast coronal T1-weighted MRI showing a completely suprasellar lesion. (B) Post-operative pre-contrast coronal MRI showing complete evacuation of the Rathke’s cleft cyst.
3. Results All patients were followed clinically, endocrinologically and radiologically. The endoscope was shown to be more useful in patients with large residual cavities or descent of the suprasellar cistern. Endoscopic exploration with the 0° and angled optics allowed better visualization so that the dorsum sellae and the suprasellar cistern could be seen easily. The follow-up period ranged from 3 months to 36 months. Postoperative improvement occurred in all patients with pre-operative headaches and seven of the nine patients with pre-operative visual symptoms (39% of all patients). Three of the six patients with pituitary dysfunction (26% of all patients) initially had post-operative improvement. After surgery, one patient developed temporary diabetes insipidus and two patients had a post-operative infection.
RCC are ectodermal remnants of the craniopharyngeal duct and they are located between the adenohypophysis and neurohypophysis.7 Histologically, RCC consist of a single layer or pseudostratified epithelium with an underlying layer of connective tissue.8 These cysts can be found as incidental lesions or associated with headache, pituitary dysfunction and/or visual disturbances. Headache is the presenting symptom in most patients2–6 and the etiology of headaches may be the local irritation of surrounding structures or an inflammatory foreign body reaction caused by the mucinous material within the cyst.9 In our series, mild and chronic headache was the main symptom and all patients had postoperative improvement. We believe that the endoscope allowed better visualization and therefore facilitated a wide opening of the cyst and complete evacuation. Visual disturbance was the second most common symptom in our series, possibly because most cysts were located in the sellar–suprasellar region. These RCC can compress the optic chiasm. New-onset hormonal deficits occurred in 4% to 30% of patients in our series post-operatively, which was similar to the results of Madhok et al.6 We believe that the endoscope can provide a better view of the perforating vessels that maintain the blood supply to the normal neurovascular structures. When the cyst was removed, the suprasellar arachnoid membrane caved into the diaphragmatic opening (Fig. 2C). We could see the optic chiasm through the pulsing arachnoid membrane (Fig. 2B).
Fig. 2. (A) Pre-operative post-contrast sagittal T1-weighted MRI showing a rim-enhancing sellar–suprasellar mass. (B) Intraoperative endoscopic view showing the sella after sphenoidotomy. (C) Intraoperative endoscopic view showing the suprasellar arachnoid membrane caved into the diaphragmatic opening after removal of the cyst. The optic chiasm can be seen through the pulsing arachnoid membrane. AM = arachnoid membrane, APG = anterior lobe of pituitary gland, C = clivus, Ch = optic chiasm, CP = carotid prominence, CR = clival recess, Ds = diaphragm a sella, LOCR = lateral opticocarotid recess, PS = pituitary stalk, S = sella, SS = sphenoidal septum. (This figure is available in color at www.sciencedirect.com.)
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diabetes insipidus and CSF leak after the operation, likely due to manipulation of the normal gland. The recurrence rate of RCC has been cited as 0% to 33%.12 Squamous epithelium, MRI enhancement and the extent of cyst removal may be related to recurrence. Other factors correlated with higher recurrence rate include the use of fat and/or fascial graft for sellar fossa closure. In our series, two patients had recurrence of the cyst contents. Regretfully, neither patient agreed to re-operation. Longer follow-up periods will be needed to assess the recurrence rate more accurately. 5. Conclusions Fig. 3. (A) Pre-operative MRI showing an intrasellar mass with suprasellar expansion and an intracystic nodule having high signal intensity on this T1weighted MRI. (B) Postoperative T1-weighted post-contrast MRI showing complete resolution of the cyst.
RCC can be difficult to differentiate from other intrasellar or suprasellar masses in routine radiologic studies. However, MRI can reveal typical features of RCC, which supports a quick and accurate diagnosis. These features include a smooth contour, a sellar epicenter, the lack of internal enhancement, a homogeneous intensity within the lesion, an ovoid shape, a small tumor volume and either the absence of, or thin, cyst wall enhancement. Two patients in our series had an intracystic nodule with high signal intensity on T1-weighted MRI and low signal intensity on T2weighted MRI; these features in an RCC can be helpful for the diagnosis (Fig. 3). The transsphenoidal approach remains the preferred method for surgical intervention to remove symptomatic RCC but the extent of cyst wall removal has been debated. Although Aho et al., Benveniste et al. and Frank et al.3,10,4 recommend a simple drainage of the cyst with biopsy of its wall, Madhok et al. advised not to strip the cyst capsule when it adhered to the underlying pituitary gland and neurovascular structures.6 Other authors have recommended total resection of the cyst wall, as many RCC relapse following incomplete resection or decompression and biopsy alone.5,11 In our series, we performed fenestration and aspiration of the RCC with partial excision of the cyst wall. Extensive removal of the cyst wall should be carried out only when loose adhesions allow non-traumatic peeling from the pituitary gland and its stalk. As the endoscope allows good magnification and angled vision, it offers the possibility of minimizing gland manipulation and maximizing sellar exposure. For a suprasellar cyst, Madhok et al.6 recommended the endoscopic transtuberculum or transplanum approach to reach the cyst. In our series, one suprasellar cyst was treated following a standard EEA. The patient developed transient
Surgical treatment is necessary for removal of symptomatic RCC. EEA is a safe and effective approach for such treatment. Fenestration and aspiration of the cyst with partial excision of the cyst wall is usually sufficient. EEA leads to good post-operative outcomes especially in the improvement of pituitary dysfunction. Acknowledgments This study was supported by the Shanghai Committee of Science and Technology, China (Grant No. 08ZR1403300). References 1. Shuangshoti S, Netsky MG, Nashold Jr BS. Epithelial cysts related to sella turcica. Proposed origin from neuroepithelium. Arch Pathol 1970;90:444–50. 2. El-Mahdy W, Powell M. Transsphenoidal management of 28 symptomatic Rathke’s cleft cysts with special reference to visual and hormonal recovery. Neurosurgery 1998;42:7–17. 3. Aho CJ, Liu C, Zelman V, et al. Surgical outcomes in 118 patients with Rathke cleft cysts. J Neurosurg 2005;102:189–93. 4. Frank G, Sciarretta V, Mazzatenta D, et al. Transsphenoidal endoscopic approach in the treatment of Rathke’s cleft cyst. Neurosurgery 2005;56:124–8. 5. Koutourousiou M, Grotenhuis A, Kontogeorgos G, et al. Treatment of Rathke’s cleft cysts: experience at a single centre. J Clin Neurosci 2009;16:900–3. 6. Madhok R, Prevedello DM, Gardner P, et al. Endoscopic endonasal resection of Rathke cleft cysts: clinical outcomes and surgical nuances. J Neurosurg 2010;112:1333–9. 7. Couldwell WT, Weiss MH. Pituitary Disorders. Baltimore: Lippincott Williams& Wilkins; 1999. 8. Matsushima T, Fukui M, Fujii K, et al. Epithelial cells in symptomatic Rathke’s cleft cysts: a light-and electron-microscopic study. Surg Neurol 1988;30: 197–203. 9. Abe T, Matsumoto K, Kuwazawa J, et al. Headache associated with pituitary adenomas. Headache 1998;38:782–6. 10. Benveniste RJ, King WA, Walsh J, et al. Surgery for Rathke cleft cysts: technical considerations and outcomes. J Neurosurg 2004;101:577–84. 11. Laws ER, Kanter AS. Rathke cleft cyst. J Neurosurg 2004;101:571–2. 12. Kim JE, Kim JH, Kim OL, et al. Surgical treatment of symptomatic Rathke cleft cysts: clinical features and results with special attention to recurrence. J Neurosurg 2004;100:33–40.