Elsevier

World Neurosurgery

Volume 107, November 2017, Pages 276-284
World Neurosurgery

Original Article
Surgical Management of Rathke Cleft Cysts

https://doi.org/10.1016/j.wneu.2017.07.164Get rights and content

Background

The diagnosis of Rathke cleft cysts (RCC) has increased in recent times as a result of improvements in imaging techniques; however, symptomatic patients are uncommon and accurate preoperative diagnosis may sometimes be difficult. The indications of surgical management protocol are evolving. We aim to provide a comprehensive review of clinical, imaging, and histopathologic features with operative management strategies along with outcome and prognosis in RCC.

Methods

A retrospective analysis (2003–2015) was performed of 58 consecutive cases of RCC seen in a surgical unit. Twenty-seven surgically treated symptomatic RCCs were further evaluated for their clinical presentation, imaging characteristics, surgical approaches, and intraoperative findings.

Results

Headache was the most common presenting complaint followed by visual deficit. Hormonal abnormality was observed in 13 patients. On magnetic resonance imaging, the characteristic intracystic nodule was identified in 6 patients. Transsphenoidal surgery for cyst excision was performed in all 27 patients with an endoscopic route in 25 patients and radical excision was performed in 17 patients. The pituitary stalk and the normal gland were preserved in all patients. Headache improved in 96% of patients and visual field defect resolved in all. Around 46% had improvement of the anterior pituitary axis. New permanent hormone deficiency was not observed. The recurrence rate was 3.7% after a minimum of 18 months follow-up.

Conclusions

RCCs are an uncommon disease with a wide spectrum of clinical and radiologic features. Endonasal endoscopic transsphenoidal surgery provides excellent clinical and endocrinologic improvement. We believe that radical excision does not necessarily result in endocrinologic impairment and may have a better impact on recurrence and cyst resolution.

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

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    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.

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