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Long-Term Endocrine Outcome of Suprasellar Arachnoid Cysts

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Long-Term Endocrine Outcome of Suprasellar Arachnoid Cysts CLINICAL ARTICLE J Neurosurg Pediatr 19:696–702, 2017 Long-term endocrine outcome of suprasellar arachnoid cysts Ji Yeoun Lee, MD, PhD,1,2 Young Ah Lee, MD, PhD,3 Hae Woon Jung, MD,3 Sangjoon Chong, MD,2 Ji Hoon Phi, MD, PhD,2 Seung-Ki Kim, MD, PhD,2 Choong-Ho Shin, MD, PhD,3 and Kyu-Chang Wang, MD, PhD2 1Department of Anatomy and Cell Biology, Seoul National University College of Medicine; and 2Division of Pediatric Neurosurgery, 3Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea OBJECTIVE Due to their distinct location, suprasellar arachnoid cysts are known to cause a wide variety of problems, such as hydrocephalus, endocrine symptoms, and visual abnormalities. The long-term outcome of these cysts has not been elucidated. To find out the long-term outcome of suprasellar arachnoid cysts, a retrospective review of the patients was performed. The neurological and endocrine symptoms were thoroughly reviewed. METHODS Forty-five patients with suprasellar arachnoid cysts, with an average follow-up duration of 9.7 years, were enrolled in the study. A comprehensive review was performed of the results of follow-up regarding not only neurological symptoms but also endocrine status. The outcomes of 8 patients who did not undergo operations and were asymptomat- ic or had symptoms unrelated to the cyst were included in the series. RESULTS Surgery was most effective for the symptoms related to hydrocephalus (improvement in 32 of 32), but en- docrine symptoms persisted after surgery (4 of 4) and required further medical management. More surprisingly, a fairly large number of patients (14 of 40; 1 was excluded because no pre- or postoperative endocrine evaluation was available) who had not shown endocrine symptoms at the time of the initial diagnosis and treatment later developed endocrine ab- normalities such as precocious puberty and growth hormone deficiency. The patients with endocrine symptoms detected during the follow-up included those in both the operated (n = 12 of 32) and nonoperated (n = 2 of 8) groups who had been stable during follow-up since the initial diagnosis. CONCLUSIONS This study implies that patients with suprasellar arachnoid cysts can develop late endocrine problems during follow-up, even if other symptoms related to the cyst have been successfully treated. Hence, patients with these cysts need long-term follow-up for not only neurological symptoms but also endocrine abnormalities. https://thejns.org/doi/abs/10.3171/2017.1.PEDS16404 KEY WORDS arachnoid cyst; suprasellar; endocrine outcome; hormone; congenital UPRASELLAR arachnoid cysts constitute 5%–12% of scopic and endoscopic fenestration; since then, a general intracranial arachnoid cysts.11 They are known to be consensus has been reached that endoscopic fenestration is associated with hydrocephalus and to cause visual preferred when feasible.10 The most recent issue is whether Sand endocrine dysfunction due to their anatomical loca- ventriculocystostomy or ventriculocystocisternostomy tions.2,7 The most controversial topic on suprasellar cysts should be performed.8,13,16,19 Numerous reports have ad- has been the optimal choice of surgical modality. First, dressed this issue, and it appears to be an ongoing debate. operations using only fenestration precluded shunt proce- Despite the detailed discussion regarding surgical mo- dures including ventriculoperitoneal shunting (VPS) and dalities, the long-term outcomes of suprasellar arachnoid cystoperitoneal shunting (CPS) as the surgical modality of cysts have not been thoroughly elucidated. Through a ret- treatment.6 Later a controversy developed between micro- rospective review of a relatively large number of patients ABBREVIATIONS ACTH = adrenocorticotropic hormone; CPS = cystoperitoneal shunting; GH = growth hormone; GnRH = gonadotropin-releasing hormone; VPS = ven- triculoperitoneal shunting. SUBMITTED July 13, 2016. ACCEPTED January 11, 2017. INCLUDE WHEN CITING Published online March 24, 2017; DOI: 10.3171/2017.1.PEDS16404. 696 J Neurosurg Pediatr Volume 19 • June 2017 ©AANS, 2017 Unauthenticated | Downloaded 09/30/21 10:10 PM UTC Long-term endocrine outcome of suprasellar arachnoid cysts TABLE 1. Demographics and clinical features of patients in the TABLE 2. Preoperative clinical manifestation of suprasellar study arachnoid cysts Clinical Features (n = 45) Value (range) Symptom No. of Patients M/F 24/21 Motor development delay 8 Mean age at initial operation in yrs 2.8 (0.5–18.0) Fetal diagnosis 8 Mean follow-up duration in yrs 9.7 (0.1–20.9) Incidental 6 Mean age at last follow-up in yrs 14.0 (1.6–27.4) Headache 6 Treatment Nausea/vomiting 6 Operation 37 Seizure 3 Microscopic fenestration 6 Strabismus/EOM limitation 4 Shunting (CPS or VPS) ± fenestration 9 Large head 3 Endoscopic fenestration 22 Decrease of visual acuity 2 No operation 8 Endocrine disorders (3 PP, 1 HH) 4 Hydrocephalus 32 Head bobbing 1 Reoperation 13 Lethargy 1 Staring 1 with suprasellar arachnoid cysts and long follow-up dura- EOM = extraocular movement (caused specifically by cranial nerve dysfunc- tions, this study uncovered new aspects of this disease that tion); HH = hypogonadotropic hypogonadism; PP = precocious puberty. are particularly related to endocrine outcomes. The long- term outcomes of patients who have not been treated with surgery are also described. was approved by the Institutional Review Board of Seoul National University Hospital. Methods Data Collection Results Initial Presentation and Surgical Indication We searched the electronic medical records of the pa- tients who visited the outpatient clinic of Seoul National A total of 45 patients (24 boys and 21 girls) were in- University Children’s Hospital between 2000 and 2014. cluded in this study (Table 1). The median age at the initial The patients with either the registered diagnosis of “supra- operation or visit was 2.8 years (range 0.5–18.0 years) with sellar arachnoid cyst,” “arachnoid cyst,” or those with the a mean follow-up duration of 9.7 years. Thirty-seven of words “suprasellar cyst” on the radiology report of brain the patients underwent operations, and 8 were not treated imaging (using a free-text search) were listed. The brain surgically. Twenty-two patients were treated with endo- imaging of all patients was reviewed manually to select scopic fenestration, 6 with microscopic fenestration, and those with arachnoid cysts located in the suprasellar loca- 9 received shunt insertions with (n = 4) or without (n = 5) tion. A retrospective review of the medical records was fenestration as the initial operation. performed for the demographic data, operational proce- Of the 45 patients, 6 (13%) were diagnosed incidentally dures, and symptomatic changes during follow-up. As the (Table 2). The most common symptom at diagnosis was operational procedure, CPS or VPS was used during the motor developmental delay (n = 8, 18%). In this series, early period, but since the 2000s, fenestration procedures hydrocephalus was detected in all patients who presented became the primary choice, and endoscopic fenestration with motor developmental delay. Equal numbers of pa- gradually became the method used most often. Currently, tients were diagnosed during fetal screening (n = 8). Endo- endoscopic fenestration is the treatment of choice for su- crine abnormalities at diagnosis were found in 4 patients prasellar arachnoid cysts, and ventriculocystocisternos- (9%). Two patients presented with decreased visual acuity. tomy is performed if possible. The indication for surgery Head bobbing was noted in 1 patient. Some patients had varied between surgeons. The only absolute indication more than 1 symptom at presentation. for surgical intervention was hydrocephalus. Incidental, All patients with hydrocephalus (n = 32) underwent op- asymptomatic lesions without hydrocephalus were not erations. Hydrocephalus was defined as ventriculomegaly operated on and were observed in the outpatient clinic. with signs of increased intracranial pressure such as head- Imaging follow-up was performed for all patients. Oph- ache, nausea, vomiting, papilledema, bulged fontanelle, thalmological evaluation was conducted in symptomatic etc. The surgical indications for the 5 patients without hy- patients. Postoperative endocrine evaluation was also not drocephalus included chronic, nonprojectile vomiting (n = performed routinely, but recently we have changed our 1, age 6.1 years), nonspecific staring not related to seizure strategy to allow patients to undergo regular evaluation by (n = 1, age 1.1 years), strabismus (n = 1, age 5.6 years), and pediatric endocrinologists. enlargement of the cyst (n = 2, ages 0.8 and 2.5 years; vol- ume enlargement by 10.1-fold and 5.6-fold). Vomiting and Statistical Analysis nonspecific staring which were transient or not supported For statistical analysis, the Fisher’s exact test and by neuroimaging studies were regarded as unrelated to the Mann-Whitney U-test were used accordingly. This study cyst. J Neurosurg Pediatr Volume 19 • June 2017 697 Unauthenticated | Downloaded 09/30/21 10:10 PM UTC J. Y. Lee et al. TABLE 3. Details of patients with endocrine dysfunction Age at Diagnosis of Age (yrs) Endocrine Problem (yrs) At Diagnosis of Age at At Diagnosis At 1st Endocrine GH Last FU Case No. Sex of SSAC Op Problem PP Deficiency HYG (yrs) HC Type of Op Treatment Initially 5 F 8.8 8.8 8.8 8.8 — — 13.0 Yes CPS None 1 M 9.7 9.7 9.7 9.7 — NA 19.4 Yes EF GnRH analog 43 M 8.4 8.5 8.4 8.4 — NA 9.5 Yes
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