Original ArticleSurgical Management of Rathke Cleft Cysts
Introduction
Rathke cleft cyst (RCC) is a benign, nonneoplastic cystic lesion that originates from congenital remnants of the Rathke pouch.1 These are predominantly sellar lesions with or without suprasellar extension and occasionally are exclusively suprasellar. Most are asymptomatic, encountered in normal pituitary glands in routine autopsies.2, 3 However, they are occasionally large enough to produce visual or endocrinologic disorders by compressing visual apparatus, pituitary gland, or rarely, hypothalamus. They are clinically significant but uncommon lesions. Various case reports have described their coexistence along with pituitary tumors.4
Over the last few decades, improvement in neuroimaging techniques has increased the diagnosis of RCC, leading to its better understanding. It is thus imperative to differentiate clinically significant from incidental and other cystic lesions of this region, such as craniopharyngioma, cystic pituitary adenoma, and so on. A proper preoperative analysis is essential, especially in incidentally detected asymptomatic patients, because it alters the management protocol.5
The literature has shown that surgery in symptomatic RCCs improves both endocrine and visual function.6 A thorough understanding of the surgical anatomy and pathophysiology is paramount to plan out an optimum management strategy. The aim of our study is to provide a comprehensive review of clinical, imaging, and histopathologic features, as well as operative and nonoperative management strategies in patients with RCCs.
We also attempt to evaluate our evolving surgical techniques over the last decade for symptomatic RCCs and understand the outcome and prognosis of such lesions because the data available for RCC from India are restricted to a few case reports and case series.7, 8, 9, 10
Section snippets
Patient Population
This is a retrospective study of patients with RCC, treated at Bombay Hospital from 2003 to 2015 by the 2 senior authors. The medical records were retrieved from a database of 884 patients with sellar-suprasellar lesions who were either under observation or had surgical treatment. Only cases histologically proved or with neuroimaging features strongly suggestive of RCC were included. The present study was approved by the institutional review board. Data collected included demographics, clinical
Patient Population
Of 884 consecutive patients diagnosed with sellar-suprasellar lesions from 2000 to 2015, 58 (6.56%) had RCC, 27 of whom required intervention. Most patients in the surgical arm were female (63%), with a median age of 37 years (range, 18–58 years) and most of whom were premenopausal. The median duration of follow-up was 38 months, with a minimum follow-up of 18 months.
Presenting Symptoms
Headache was the most common presenting symptom in the surgical group, seen in 25 of 27 patients (92.6%). It was chronic in 19
Discussion
RCC is the second most common benign lesion after pituitary adenoma in the sellar region.13 RCCs are also known as pars intermedia cysts. RCC arises from the remnants of the embryonic Rathke pouch if its lumen is not obliterated by the 12th week of gestation. It is thus a congenital developmental lesion.14, 15
Histologically, RCCs consist of a single pseudostratified ciliated cuboidal or columnar epithelium. Some RCCs show inflammatory contents and squamous metaplasia, and this metaplasia is
Conclusions
We present the first and the largest comprehensive series of surgically managed RCCs from the Indian subcontinent. Symptomatic RCCs account for approximately 10% of surgically treated sellar lesions and mandate early intervention. The common presenting complaints include headache, hyperprolactinemia, menstrual irregularities, sexual dysfunction, and anterior pituitary gland failure. We believe that a timely surgical intervention not only provides significant symptomatic relief but even improves
References (47)
- et al.
Rathke's cleft cyst recurrence after transsphenoidal surgery: a meta-analysis of 1151 cases
Clin Neurosci
(2014) - et al.
Preliminary results of a phase I/II study of simultaneous modulated accelerated radiotherapy for nondisseminated nasopharyngeal carcinoma
Int J Radiat Oncol Biol Phys
(2006) - et al.
Symptomatic Rathke cleft cyst
J Clin Neurosci
(2012) - et al.
Clinical features of symptomatic Rathke's cleft cyst
Clin Neurol Neurosurg
(2001) - et al.
Treatment of Rathke's cleft cysts: experience at a single centre
J Clin Neurosci
(2009) - et al.
Endoscopic transsphenoidal surgery of Rathke's cleft cyst
J Clin Neurosci
(2015) - et al.
Clinical features, management and recurrence of symptomatic Rathke's cleft cyst
J Clin Neurosci
(2009) - et al.
Rathke's cleft cysts in children: clinical, diagnostic, and surgical features
Childs Nerv Syst
(2012) - et al.
Clinical, radiographic, and pathological features of symptomatic Rathke's cleft cysts
J Neurosurg
(1991) - et al.
Incidental pituitary lesions in 1000 unselected autopsy specimens
Radiology
(1994)
Concomitant pituitary adenoma and Rathke's cleft cyst
Neuroradiology
Magnetic resonance imaging, clinical manifestations, and management of Rathke's cleft cyst
Clin Endocrinol (Oxf)
Surgical outcomes in 118 patients with Rathke cleft cysts
J Neurosurg
Is there a need to diagnose Rathke's cleft cyst preoperatively?
Neurol India
An interesting case of Rathke's cleft cyst presenting as bilateral cryptorchidism
J Pediatr Neurosci
Diabetes insipidus as a presenting manifestation of Rathke's cleft cyst
Indian J Endocr Metab
Symptomatic Rathke's cleft cyst with a co-existing pituitary tumor: brief review of the literature
Asian J Neurosurg
The Barts Endocrine Protocols
Endonasal transsphenoidal approach for pituitary adenomas and other sellar lesions: an assessment of efficacy, safety, and patient impressions
J Neurosurg
Rathke cleft cysts
J Neurosurg
Intrasellar epithelial cysts
J Neurosurg
Symptomatic Rathke's cleft cysts located entirely in the suprasellar region: review of diagnosis, management, and pathogenesis
Neurosurgery
Ciliated and goblet cells in craniopharyngioma. Light and electron microscopic studies at surgery and autopsy
Acta Neuropathol
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2022, American Journal of Otolaryngology - Head and Neck Medicine and SurgeryCitation Excerpt :Recurrence of RCCs is correlated with the degree of resection, the presence of squamous metaplasia, inflammation, and infection [3,4]. RCCs are rarely symptomatic, but may present with headaches, visual disturbances, or endocrine dysfunction if they grow large enough to compress surrounding structures, such as the pituitary gland, hypothalamus, optic nerve or chiasm [5,6]. When asymptomatic, RCCs can be followed with serial imaging.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.