Rathke cleft cysts: a review of clinical and surgical management

Abstract

<jats:p>The aim of this paper is to provide a comprehensive review of clinical, imaging, and histopathological features, as well as operative and nonoperative management strategies in patients with Rathke cleft cysts (RCCs).</jats:p> <jats:p>A literature review was performed to identify previous articles that reported surgical and nonsurgical management of RCCs. Rathke cleft cysts are often incidental lesions found in the sellar and suprasellar regions and do not require surgical intervention in the majority of cases. In symptomatic RCCs, the typical clinical presentation includes headache, visual loss, and/or endocrine dysfunction. Visual field testing and endocrine laboratory studies may reveal more subtle deficiencies associated with RCCs. When indicated, the transsphenoidal approach typically offers the least invasive and safest method for treating these lesions. Various surgical strategies including cyst wall resection, intralesional alcohol injection, and sellar floor reconstruction are discussed. Although headache and visual symptoms frequently improve after surgical drainage of RCCs, hypopituitarism and diabetes insipidus are less likely to do so. A subset of more aggressive, atypical RCCs associated with pronounced clinical symptoms and higher recurrence rates is discussed, as well as the possible relationship of these lesions to craniopharyngiomas.</jats:p> <jats:p>Rathke cleft cysts are typically benign, asymptomatic lesions that can be monitored. In selected patients, transsphenoidal surgery provides excellent rates of improvement in clinical symptoms and long-term cyst resolution. Complete cyst wall resection, intraoperative alcohol cauterization, and sellar floor reconstruction in the absence of a CSF leak are not routinely recommended.</jats:p>

Individualized surgical strategies for Rathke cleft cyst based on cyst location

“…However, the range of recurrence rates in previous reports is quite large (0%-42%). 1,8,11,13,15,[23][24][25]28,[34][35][36]47,53 Several factors, including sample size, surgical modalities, duration of follow-up, and definitions of recurrence, may account for the variation. For example, the radiological recurrence rate in our series was 13%, while the symptomatic recurrence rate was 8%.…”

Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma

“…RCCs can also cause delayed or precocious puberty, amenorrhea/galactorrhea, growth retardation, weight gain, and hypogonadism [9][10][11]. As the peak age of patients presenting with RCCs symptoms is typically between 30 and 50 years, it is unusual to detect RCCs by magnetic resonance imaging (MRI) in asymptomatic children [7,12,13]. In addition, even among symptomatic patients, the rate of RCC prevalence is much lower in children compared to adults [14,15].…”

“…The mean age of these patients was 12.2 years (range, [5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Of the 15 patients with CP that were reviewed, 8 were male and 7 were female.…”

“…Although they are reported in as many as 3-22% of all autopsy studies, they are seldom symptomatic during life [5,6]. The most common clinical symptoms associated with RCCs are headache, impaired visual function, and endocrinological insufficiency [7,8]. RCCs can also cause delayed or precocious puberty, amenorrhea/galactorrhea, growth retardation, weight gain, and hypogonadism [9][10][11].…”

“…It is well known that patients with CP require total removal of the tumor including the cyst wall. The rate of postoperative complications, such as hypopituitarism, diabetes insipidus, and recognition insufficiency is remarkably high [7,16]. Therefore, indications for the surgical treatment of symptomatic pituitary cysts should include histological confirmation of the surgical specimen, as well as confidence the surgery will lead to the relief of the symptoms.…”