Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>Rathke cleft cysts (RCCs) are sellar lesions that are commonly encountered in adults but infrequently diagnosed in the pediatric population. As a result, the optimal management of pediatric RCCs remains a subject of controversy. Only 2 prior surgical series have been published on pediatric RCCs and no study has compared the presentation and outcomes of surgically versus conservatively managed cases. The authors therefore performed a comparative analysis of pediatric cases of RCC in which patients were treated with surgery or managed in a conservative manner.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>All cases involving pediatric patients diagnosed with an RCC at the University of Virginia between 2000 and 2016 were included in this study. Patient medical records, operative notes, and neuroimaging findings were reviewed. Patients who developed visual field deficits, radiographic evidence of chiasmal compression, or medically refractory headaches were considered candidates for surgical intervention. All patients who were selected for surgery underwent an endoscopic endonasal approach with cyst fenestration.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>A total of 24 pediatric patients were diagnosed with an RCC over a 16-year period. Seven patients ultimately underwent transsphenoidal cyst fenestration, and 17 were managed conservatively. The patients’ age at diagnosis, cyst size, and pituitary function at the time of RCC diagnosis were similar in the conservatively and surgically managed cohorts. At diagnosis, 19 of 24 patients endorsed headaches that led to neuroimaging. All patients in the surgical cohort endorsed severe headaches at diagnosis compared with 71% in the conservative group. For the 7 patients treated with surgery, complete cyst evacuation was achieved in 86% of cases. Transient postoperative endocrinopathy occurred in 4 (57%) of 7 surgically treated individuals and resolved in all cases. In the conservative cohort, 1 patient developed a delayed pituitary-related endocrinopathy. Headache resolution occurred in 5 (71%) of the 7 patients who underwent surgery and 7 (58%) of the 12 who were treated without surgery. Cyst recurrence was documented in 1 individual in the surgical cohort who underwent a subtotal cyst fenestration that ultimately required re-intervention. In the conservative cohort, spontaneous cyst shrinkage occurred in 35% of patients with a median time to regression of 23.5 months.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Pediatric RCCs are benign sellar lesions that often present with headaches. While cyst fenestration mitigates headaches in most patients, the majority of conservatively managed pediatric patients with RCCs will have spontaneous headache resolution. Furthermore, spontaneous RCC regression occurs in a substantial number of individuals. Thus, in the absence of optic compression, visual field deficit, or diagnostic uncertainty, many pediatric cases of RCC can be managed conservatively.</jats:p></jats:sec>

Endonasal Endoscopic Surgery for Pediatric Sellar and Suprasellar Lesions: A Systematic Review and Meta‐analysis

“…A total of 25 studies met inclusion criteria for qualitative and quantitative analysis. 1,7,11,21-42 See Figure 1 for a summary of search results. A funnel plot indicating less likelihood of publication bias across studies is presented in Figure 2 .…”

A clinical approach to parasellar lesions in the transition age

“…Several retrospective studies have investigated the clinical presentation of RCCs in a paediatric population, including at least 5% of patients in the transition age. In these patients, as in adults, of non‐endocrine symptoms, headache is the most frequent manifestation with an incidence of 15%‐85%, whereas activity loss, fatigue and anorexia are reported in 13%‐18% of cases, and visual disturbances are reported in 3%‐18%, occurring more rarely than in adults 192,193,197‐205 . Pituitary hormone deficits, involving one or more axes, are common.…”

“…Pituitary hormone deficits, involving one or more axes, are common. Specifically, growth retardation occurs in 7%‐57% of cases, symptoms and signs of hypothyroidism in 5%‐50%, delayed puberty in 1%‐37%, and adrenal insufficiency in 1%‐26% 192,193,197‐205 . Furthermore, precocious puberty, occurring in 8%‐35% of patients, is another characteristic endocrine manifestation in the paediatric‐adolescent population, whereas DI (3%‐26%) and hyperprolactinemia (2%‐20%) are less frequent 192,193,197‐205 .…”

“…Specifically, growth retardation occurs in 7%‐57% of cases, symptoms and signs of hypothyroidism in 5%‐50%, delayed puberty in 1%‐37%, and adrenal insufficiency in 1%‐26% 192,193,197‐205 . Furthermore, precocious puberty, occurring in 8%‐35% of patients, is another characteristic endocrine manifestation in the paediatric‐adolescent population, whereas DI (3%‐26%) and hyperprolactinemia (2%‐20%) are less frequent 192,193,197‐205 . Rare presenting manifestations in adolescents include seizures, 192,197,198,203 syncope, 197,206 weight gain, 192,198 aseptic meningitis, 192 pathological laughter, 207 ataxia, 207 dizziness 197 and tinnitus 197 …”

“…Currently, there is no serum or CSF biomarker that is helpful for the diagnosis of a RCC. Pituitary hormone deficits, involving one or more axes, are the most common endocrine manifestations in the transition age, with an incidence widely ranging from 8% to 68% 192,193,197‐205 . The most common anterior pituitary alterations reported are represented by GHD, hypothyroidism, low gonadotrophin levels, secondary adrenal insufficiency and hyperprolactinaemia, whereas signs and symptoms of DI are typical of posterior pituitary involvement 165,192,193,197‐205 …”