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Background Leukoencephalopathy with brain calcifications and cysts (LCC) is neuroradiologically characterized by leukoencephalopathy, intracranial calcification, and cysts. Coats plus syndrome is also characterized by the same neuroradiological findings together with defects in retinal vascular development. Indeed, LCC and Coats plus were originally considered to be the same clinical entity termed cerebroretinal microangiopathy with calcifications and cysts, but evidence suggests that they are genetically distinct. Mutations in CTS telomere maintenance complex component 1 (CTC1) and small nucleolar RNA, C/D box 118 (SNORD118) genes have been found to cause Coats plus and LCC, respectively. Materials and Methods Eight unrelated families with LCC were recruited. These patients typically showed major neuroradiological findings of LCC with no signs of extra-neurological manifestations such as retinal abnormality, gastrointestinal bleeding, or hematological abnormalities. SNORD118 was examined by Sanger sequencing in these families. Results Seven out of eight probands carry compound heterozygous mutations, suggesting that SNORD118 mutations are the major cause of LCC. We identified a total of eight mutation, including four that were novel. Some of the variants identified in this study present heterozygously in public databases with an extremely rare frequency (

Background Hypophysitis is a rare disease entity mimicking a pituitary adenoma. Despite crucial disease involving a pituitary gland which is a main component of the hormonal axis, there have been minimal researches regarding the hypophysitis. In this study, we described the constellation of the preoperative findings including clinical, radiologic, and endocrinologic features, as well as postoperative outcomes of the hypophysitis. Methods The authors reviewed the data retrospectively of a total of 2814 patients who underwent microsurgery at our institution to treat a pituitary mass lesion. Among them, the patients proven as hypophysitis in histologic examination were enrolled, followed by stratification into tumor-associated hypophysitis and isolated hypophysitis depending on the existence of associated tumor lesion. As a result, a total 21 cases were enrolled consisting of the 13 cases of tumor-associated and the 8 cases of isolated hypophysitis. In this study, the isolated hypophysitis was focused on the analysis. All patients with isolated hypophysitis were evaluated the clinical, radiologic, and endocrinologic tests preoperatively. After microsurgery, all patients had a minimum follow up of 6 months (6∼72 months). The clinical symptoms, endocrinologic tests, and brain MRI were checked at every 6 months during first 2 year, since then, at every 12 months. Results Among 2814 cases of pituitary mass lesion underwent microsurgery, the hypophysitis were found 21 cases (0.74 %). The 13 cases were the tumor-associated hypophysitis, consisting of rathke's cleft cyst (7 cases), craniopharyngioma (3 cases), pituitary adenoma (2 cases), and germinoma (1 case). The 8 cases were the isolated hypophysitis including lymphocytic (4 cases), granulomatous (3 cases), and IgG4-related (1 case) hypophysitis. The patients presented a wide range of clinical symptoms from general weakness to panhypopituitarism. In addition, the most remarkable radiologic features of the isolated hypophysitis were infundibular thickening and the low signal intensity in T1WI and T2WI. All 8 patients with isolated hypophysitis presented endocrinologic deficiency with various extent in a preoperative biochemistry. After surgery, the hormonal status were not changed in 4 patients (50%), however, aggravated in 4 patients (50%). The 3 patients (37.5%) complained visual field defects in preoperative ophthalmologic test, but improved all after surgery. Conclusion In this study, the preoperative clinical manifestations, radiologic and endocrinologic features of hypophysitis were described comprehensively. In addition, we also revealed the long-term outcomes of hypophysitis in the aspect of visual function and endocrinologic outcomes. Preexisting visual field defects were improved in all patients (3 cases). However, all patients presented endocrinologic deficiency in preoperative biochemistry, among them, the 4 patients (50 %) were unchanged and the 4 patients (50 %) were exacerbated after surgery. In conclusion, the visual outcomes were satisfactory, however, the endocrinologic outcomes were vice versa, because the preoperative endocrinologic deterioration was unchanged or even aggravated after surgery in some cases.

A 69-year-old woman presented with cognitive impairment related to attentive, executive, and mnemonic functions; progressive worsening of walking, speaking, writing, and reading ability; and double sphincter incontinence. Leukoencephalopathy, cystic lesions, and calcifications, suspected for Labrune syndrome, were observed at MRI and CT brain images. Generalized wave abnormalities were also visible at electroencephalogram. Functional brain imaging performed with F-FDG PET/CT demonstrated a decreased glucose metabolism in impaired brain regions, in accordance with MRI findings. Genetic testing confirmed a mutation of SNORD118.

How mutations in the non-coding U8 snoRNA cause the neurological disorder leukoencephalopathy with calcifications and cysts (LCC) is poorly understood. Here, we report the generation of a mutant U8 animal model for interrogating LCC-associated pathology. Mutant U8 zebrafish exhibit defective central nervous system development, a disturbance of ribosomal RNA (rRNA) biogenesis and tp53 activation, which monitors ribosome biogenesis. Further, we demonstrate that fibroblasts from individuals with LCC are defective in rRNA processing. Human precursor-U8 (pre-U8) containing a 3′ extension rescued mutant U8 zebrafish, and this result indicates conserved biological function. Analysis of LCC-associated U8 mutations in zebrafish revealed that one null and one functional allele contribute to LCC. We show that mutations in three nucleotides at the 5′ end of pre-U8 alter the processing of the 3′ extension, and we identify a previously unknown base-pairing interaction between the 5′ end and the 3′ extension of human pre-U8. Indeed, LCC-associated mutations in any one of seven nucleotides in the 5′ end and 3′ extension alter the processing of pre-U8, and these mutations are present on a single allele in almost all individuals with LCC identified to date. Given genetic data indicating that bi-allelic null U8 alleles are likely incompatible with human development, and that LCC is not caused by haploinsufficiency, the identification of hypomorphic misprocessing mutations that mediate viable embryogenesis furthers our understanding of LCC molecular pathology and cerebral vascular homeostasis., SCOPUS: ar.j, info:eu-repo/semantics/published

Background and purpose Although Labrune syndrome is a well-known disorder characterized by a typical neuroradiological triad, namely leukoencephalopathy, intracranial calcifications and cysts, there are no reports of systemic involvement in this disorder. This paper attempts to describe a peculiar clinical manifestation related to a novel mutation in the SNORD118 gene. Methods Clinical examination, brain and total-body imaging, and neurophysiological and ophthalmological investigations were performed. Amplification of the SNORD118 gene and Sanger sequencing were integrated to investigate potential causative mutations. Results A 69-year-old woman, with a long history of episodes of vertigo and gait imbalance, was referred to our hospital for progressive cognitive and motor deterioration. Computed tomography and magnetic resonance imaging disclosed diffuse bilateral leukoencephalopathy in periventricular and deep white matter, widespread calcifications and numerous cysts in the brain, liver, pancreas and kidneys. The genetic analysis revealed two biallelic variants in the SNORD118 gene, one of which is novel (n.60G>C). Conclusions This is the first report of adult-onset Labrune syndrome with an unusual systemic involvement presenting a novel mutation in the SNORD118 gene.

Background and importance Cavum septum pellucidum (CSP) and cavum vergae (CV) cysts are common incidental findings on imaging studies. However, they may rarely present with symptoms related to the obstruction of the foramen of Monro by the cyst leaflets. There is no consensus regarding the management of symptomatic CSP and CV cysts. We present an original transcavum interforniceal endoscopic fenestration technique. The step-by-step surgical procedure and two illustrative cases are presented. Clinical presentation A 31-yr-old male and a 24-yr-old woman presented with symptomatic CSP and CV cysts. For both patients, neuronavigation was used to plan the procedure. An endoscope was introduced into the cyst through a right frontal burr-hole. After an examination of the endoscopic anatomy, a communication between the cyst and the third ventricle was performed using an endoscopic forceps. In both cases, directly after the fenestration, cerebrospinal fluid (CSF) passed through the communication, and the collapse of the cyst was appreciated. Symptoms were relieved in both patients, and neuropsychological assessment improved. Postoperative imaging showed a reduction in the cyst bulge, and patent foramen of Monro. Conclusion Endoscopic fenestration of CSP and CV cysts to the third ventricle through an interforniceal navigated approach is a feasible and efficient surgical procedure. Theoretical advantages include a single tract through noneloquent brain, a perpendicular trajectory to the membrane for fenestration, and a large CSF space beyond the fenestration point.

The efficacy of the endoscopic transcortical transventricular approach (ETTA) for craniopharyngioma in the third ventricle with hydrocephalus has been reported focusing on its reduced invasiveness. On the other hand, suprasellar craniopharyngioma without ventriculomegaly is generally surgically managed by craniotomy or the endoscopic endonasal approach (EEA). Here, we report an elderly patient who received cyst fenestration and Ommaya reservoir placement in ETTA for recurrent suprasellar cystic craniopharyngioma without ventriculomegaly. The ETTA as a less invasive procedure is feasible in patients not only with intraventricular craniopharyngioma but also with suprasellar craniopharyngioma without hydrocephalus provided a navigational system is applied and the surgeon has ample experience with transcranial endoscopic procedures.

Rathke's cleft cysts are benign cystic lesions of the sellar region, which may cause headache, pituitary deficiencies and visual disturbances from mass effect. Their management is not standardized yet. This study is about establishing a consensus for medical care of RCC.We performed a retrospective observational study of all patients that were diagnosed or followed for RCC between 2008 and 2018 (11 years), in the neurosurgical and the adult endocrine departments of our institution. The study's average time length of follow-up is 72.9 months (from 2 to 385 months).The 57 included patients were divided into 2 groups: group A, which included 39 patients that were conservatively managed and group B, which included 18 surgically treated patients. Group A showed either an improvement or a spontaneous resolution of headaches in 56.1% of the cases (P0.01); a resolution of hyperprolactinemia in 70% of the cases (P=0.21); and of hypogonadism, ACTH deficiency, growth hormone deficiency in 100% of the cases. There was no spontaneous improvement of visual disturbances (P0.01) or diabetes insipidus (P=0.29) during follow-up. Regarding group B, surgery allowed improvement or complete resolution of headaches in 60% of the cases; visual troubles in 100% of the cases (P0.01); and hyperprolactinemia in 100% of the cases. Pituitary deficiencies were not improved by surgery.This study offers guidance in decision-making regarding the management of RCC patients. Surgery is particularly suitable for treating visual disturbances caused by RCC. Regular follow-up is more appropriate than surgery concerning headaches, hyperprolactinemia, endocrine disruptions and diabetes insipidus.

Background Malignant craniopharyngioma is a rare clinical entity; most patients with this disease show transformation from an initially benign craniopharyngioma. The rare prevalence of the disease, nonspecific presenting symptoms, and imaging features that overlap with benign craniopharyngiomas make preoperative identification challenging. Methods In this study, we retrospectively evaluated the data of 7 patients diagnosed with malignant craniopharyngioma within a 5-year period in a single center. A thorough review of patient records from Beijing Tiantan Hospital, Capital Medical University, between 2013 and 2018 was performed. After identifying these 7 patients with malignant craniopharyngioma, we evaluated their demographic and clinical characteristics, including symptoms at presentation, imaging characteristics, treatment, and outcomes. Results Five patients showed transformation from benign craniopharyngiomas, and 2 had de novo malignancy. Two patients were children at diagnosis of malignancy. Patients' average age was 22 years. Headache was the most common presenting symptom, followed by visual field defects and adenohypophyseal dysfunction. Imaging characteristics showed that all patients had giant mass in the sellar, suprasellar, and/or parasellar regions that had invaded surrounding anatomic structures. Of the 5 patients who showed transformation from benign craniopharyngioma, 3 had undergone radiologic treatment before the diagnosis of malignant transformation. Conclusions Malignant craniopharyngioma should not be overlooked in patients with a giant sellar mass, especially in young patients who have experienced multiple recurrences of craniopharyngioma and patients who received radiologic therapy.

Background Diabetes insipidus (DI) is a recognized transient or permanent complication following transsphenoidal surgery (TSS) for pituitary tumors. Objective To describe significant experience with the incidence of DI after TSS, identifying predictive characteristics and describing our diagnosis and management of postoperative DI. Methods A retrospective analysis was performed of 700 patients who underwent endoscopic TSS for resection of pituitary adenoma (PA), Rathke cleft cyst (RCC), or craniopharyngioma. Inclusion criteria included at least 1 wk of follow-up for diagnosis of postoperative DI. Permanent DI was defined as DI symptoms and/or need for desmopressin more than 1 yr postoperatively. All patients with at least 1 yr of follow-up (n = 345) were included in analyses of permanent DI. Multivariable logistic regression models were constructed to identify predictors of transient or permanent postoperative DI. Results The overall rate of any postoperative DI was 14.7% (103/700). Permanent DI developed in 4.6% (16/345). The median follow-up was 10.7 mo (range: 0.2-136.6). Compared to patients with PA, patients with RCC (odds ratio [OR] = 2.2, 95% CI: 1.2-3.9; P = .009) and craniopharyngioma (OR = 7.0, 95% CI: 2.9-16.9; P ≤ .001) were more likely to develop postoperative DI. Furthermore, patients with RCC (OR = 6.1, 95% CI: 1.8-20.6; P = .004) or craniopharyngioma (OR = 18.8, 95% CI: 4.9-72.6; P ≤ .001) were more likely to develop permanent DI compared to those with PA. Conclusion Although transient DI is a relatively common complication of endoscopic and microscopic TSS, permanent DI is much less frequent. The underlying pathology is an important predictor of both occurrence and permanency of postoperative DI.

Objective: Pineal cysts are common benign incidental findings in adults. There are no commonly accepted criteria for follow up or indications for intervention. We looked at our outcomes for this condition to explore their natural history and review our surveillance criteria.Method: Retrospective review of multidisciplinary team meetings at a tertiary neurosurgical centre over 10 years. Data relating to demographics, presenting symptoms, maximum diameter, duration of surveillance, final diagnosis and overall outcome were extracted from electronic patient records and available MRI. Data were analysed using IBM SPSS version 24.Result: Seventy-seven pineal cysts were identified. Female to male ratio was 1.43, female mean age was 38.6 and male mean age was 50.4. An increase in referral frequency was observed over the study period (mean increase of 1.4 cases per year). Presenting symptoms of headache in 45% and visual and hearing symptoms in 38.5% were recorded and baseline mean maximum diameter was 13.4mm. 20 patients were discharged on presentation, 54 were booked for at least one follow-up scan with a median follow up period of 14 months. The mean change in maximum diameter was 0.04mm over 18 months. Three patients (3.9%) underwent endoscopic biopsy and CSF diversion for cysts all more than 20mm with radiological evidence of hydrocephalus. In 100% of cases, the initial MDT diagnosis and final diagnosis were concordant.Conclusions: No patient under surveillance required surgical treatment and those managed surgically were symptomatic with large cysts and hydrocephalus on presentation. A majority of pineal cysts remained unchanged during the MRI follow-up, therefore our review suggests that routine follow-up of pineal cysts is not necessary in the absence of unusual radiological characteristics or related clinical symptoms.

We describe a surgical case of aseptic meningitis and secondary hypophysitis due to rupture of a Rathke's cleft cyst(RCC)and report on literature considerations.A 36-year-old woman with a past history of menstrual irregularity who had been taking a low-dose contraceptive for five years visited our hospital. She had discomfort in her right eye four years earlier. A waxy nodule observed on MRI was diagnosed to be an asymptomatic RCC. She experienced periodic headaches and retrobulbar pain without any prior history. MRI showed dural thickening on gadolinium-T1-weighted(GdT1)imaging, disappearance of the waxy nodule on T1/T2-weighted imaging, signal change of the cyst contents, and suspected cerebrospinal fluid in the pre-pontine cistern. Aseptic meningitis or hemorrhagic adenoma due to RCC rupture was suspected, and endoscopic transsphenoidal surgery was performed on day 26. The pathological results indicated that the ruptured RCC had resulted in aseptic meningitis and secondary hypophysitis.If the rupture of an RCC is suspected and subsequent secondary hypophysitis and optic neuritis are observed, early diagnosis and surgery with informed consent are essential. This is because of the high risk for panhypopituitarism with vision loss and visual field disturbance.

Background Bobble-head doll syndrome is a rare neurological syndrome presenting with repetitive anteroposterior head movements. It is usually associated with expansile cystic lesions in the third ventricular region. Case Description An 8-year-old boy presented with involuntary bobbling head movements. Magnetic resonance imaging of the brain revealed an extensive suprasellar cyst resulting in obstructive hydrocephalus. Endoscopic ventriculo-cysto-cisternostomy resulted in improved clinical outcome. Conclusions Endoscopic ventriculo-cysto-cisternostomy is an effective, less-invasive technique in the treatment of suprasellar cysts that results in resolution of the bobbling head movements.

Objective Delayed postoperative hemorrhage (DPOH) was a severe postoperative complication after intracranial epidermoid cyst (EC) surgery. This study was designed to investigate possible clinical data and image features related to DPOH in patients with EC. Methods We retrospectively investigated 186 cases of EC, and 8 cases presented an appearance of DPOH. Preoperative magnetic resonance imaging (MRI) and clinical data (age, sex, chief surgeon, tumor size, tumor location, and degree of resection) were reviewed and analyzed. The Student t test and the χ2 test were used, and statistical significance was defined as P Results A total of 8 patients suffered from DPOH. Seven patients (87.5%) with DPOH showed atypical mixed signal intensity on preoperative T1 MRI compared with typical low intensity in another DPOH case. Only 1 case of mixed signal intensity on T1 MRI was found in 178 patients without DPOH. The mixed signal intensity on preoperative T1 MRI was highly related to DPOH and mortality (both P Conclusions Mixed signal intensity on preoperative T1 MRI might be associated with DPOH in patients with EC. The surgeon should pay more attention to this rarely atypical imaging before surgery. Further observations are necessary to illustrate the relationship between mixed signal intensity and DPOH in EC and to guide reasonable therapy.

Background The purpose of this study was to evaluate the preoperative clinical data and postoperative endocrinologic improvement, recurrence, and complications of Rathke's cleft cysts (RCCs) in a large series of endoscopic transsphenoidal surgery patients, operated in a single center. Methods Between 1997 and April 2018, 84 patients underwent endoscopic transsphenoidal surgery for RCCs. The presentation symptoms, endocrinologic results by localization and content of the cysts, surgical resections, postoperative complications, and recurrences were evaluated retrospectively. Results Chronic headache was reported in 66.6% (56/84), visual field defects were present in 20.2% (17/84), and 23.8% (20/84) of the cases had pituitary dysfunction. Among the endocrinopathies, 17 of 17 patients had hyperprolactinemia, 2 of 7 patients had cortisol deficiency, and 2 of 13 had hypogonadism, which resolved after surgery. Preoperative pituitary hormone deficiency was associated with gelatinous tumor content (P = 0.044). However, there was no significant relationship between tumor localization and preoperative hormone insufficiency. Postoperative hypocortisolemia was seen more frequently in intrasellar RCCs (P = 0.048). Three new pituitary hormonal deficiencies were identified in postoperative patients (1 hypocortisolism, 1 diabetes insipidus, 1 hypogonadism). Gross total resection was achieved in 20 cases (23.8%), 9 of which were performed using an extended approach. Postoperative complications included 3 cerebrospinal fluid leakages and 1 epistaxis. Two patients presented with cyst recurrence at follow-up at 12 and 26 months. Conclusions Preoperative hormone deficiency was found to be associated with tumor content, independent of size or localization. The surgical aim for symptomatic RCCs should be decompression and this is effective for endocrinologic improvement. An extended endoscopic approach was adequate for total cyst wall removal in suprasellar cysts. Endoscopic transsphenoidal surgery was successful, with low complication rates, for decompression of intrasellar and suprasellar cysts in both complete cyst wall resection and cyst fenestration.

The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients. Retrospective review of 30 patients between the ages of two and 24 who underwent endoscopic endonasal resection of sellar or suprasellar lesions between January 2010 and December 2015. Endocrinological and ophthalmological outcomes, as well as extent of resection and complications were all evaluated. Gross total resection was achieved in eight of the nine pituitary adenomas, nine of the 12 craniopharyngiomas, and six of the nine Rathke cleft cysts. Of the 30 patients, 22 remained disease free at last follow-up. A total of six patients developed hypopituitarism and five developed diabetes insipidus. Eleven patients experienced improved vision, sixteen experienced no change, and one patient experienced visual worsening. Postoperative cerebrospinal fluid leak was seen in a single case and later resolved, vasospasm/stroke was experienced by 10% of patients, and new obesity was recorded in 10% of patients. There were no perioperative deaths. Endoscopic endonasal resection is a safe and effective surgical alternative for the management of sellar and suprasellar pathologies in pediatric populations with excellent outcomes, minimal complications, and a low risk of morbidity.

Leukoencephalopathy, cerebral calcifications, and cysts (LCC) is an extremely rare neurological disease, also known as Labrune syndrome. The disease more commonly affects children and young adults and the characteristic triple imaging findings are leukoencephalopathy, calcifications and multiple cysts, presenting with a variety of supra- and infratentorial symptoms but lacking for extra-neurological manifestations. Coats plus syndrome and cerebroretinal microangiopathy with calcifications and cysts (CRMCC) share similar neurological findings with LCC, but additionally involves other extra-neurological organs. Tumoral excision is usually required due to mass effect to the eloquent brain of multiple growing cysts or hemorrhages, but the outcome of surgery varies. Here we demonstrate an 8-year neuroimaging study of a rare adult-onset case of LCC with gradual headache, hemiparesis, hand tremors, unstable gait, and seizure attacks despite several times of tumoral excision. Neuroimaging revealed multiple microbleeds and microcalcification in the leukoencephalopathic areas, with increasing calcifications, recurrent previously excised cysts and new cyst formation in the longitudinal neuroimaging follow-ups within the eight years. We believe that LCC involves microangiopathy, which causes blood-brain barrier disruption, myelin serum collection and subsequent growing cysts and dystrophic calcification formation. We provide histopathological correlation in the illustration. Due to the underlying pathomechanism and long-term recurrence nature, patients with a combination of cysts and calcifications on CT scan should be follow up carefully and postoperative recurrence after years may occur.

Purpose To compare postoperative macular thickness measurements of inner and outer retinal layers in eyes of patients with chiasmal compression with or without visual field (VF) recovery and healthy controls using optical coherence tomography (OCT). Methods Macular spectral-domain OCT has been used for the auto-segmentation of images obtained from 100 eyes affected with chiasmal compression compared with 100 healthy controls enrolled in this study. We have divided eyes with chiasmal compression into two groups: group 1 characterized by VF recovery after tumour excision and group 2 showing partial or no recovery of VF. The thickness of the macular retinal nerve fibre layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer (INL), outer plexiform layer (OPL), outer nuclear layer (ONL), photoreceptor layer (PRL), inner retinal layer (IRL) and outer retinal layer (ORL) was segmented. The correlation between macular RNFL (mRNFL) and functional parameters has been analysed. Results Both groups 1 and 2 showed significant thinning of RNFL, GCL, IPL and IRL in all quadrants. However, no significant changes have been detected in PRL and ORL of patients in either group compared with healthy controls. A significant thickening was detected in INL and ONL of group 2 compared with healthy controls and group 1. Postoperative mRNFL thickness is significantly correlated with VF defects and visual acuity except temporal quadrant. Conclusions Eyes with chiasmal compression showed thinning of the inner retinal layers with thickening of the INL and ONL in patients with partial to no recovery of VF. The changes in retinal microstructures are well-correlated with functional recovery. Further studies are needed to reveal the clinical implications of these findings in patients with chiasmal compression.

Low-grade gliomas (LGG) are among the most common types of brain tumors in children and young adults. These tumors often consist of solid and cystic components. Bevacizumab is a documented treatment for progressive LGG, yet the impact of therapy on the cystic component of these tumors is unknown. We present four patients with prominently cystic LGG treated with bevacizumab at the time of progression. In each case, the cystic component responded to treatment. This is the first known study to investigate bevacizumab's impact on the cystic component of low-grade gliomas.

Rathke’s cleft cysts (RCCs) are common sellar lesions. Their management remains controversial, particularly when small or asymptomatic. Herein we review a consecutive series of RCC patients managed with surgery or observation. All patients with a new diagnosis of presumed RCC, based on MRI, from February 2012–March 2018 were retrospectively divided into observational and surgical cohorts based on an intent-to-treat model. The cohorts were compared for clinical presentation, and cyst volume. The observational cohort was followed for change in cyst size. The surgical cohort was followed for changes in endocrinopathy, visual symptoms, headache and recurrence. Of 90 patients (mean age 36.7 ± 19.4 years; 68% female), 60% (n = 54) were in the observational cohort and 40% (n = 36) in the surgical cohort. Average follow-up was 13 ± 23 months in the observational cohort and 24 ± 19 months in the surgical group. In comparing the cohorts, mean ages were similar with more women in the surgical group (81% vs. 56%, p = 0.04). Most patients in the observational cohort had incidentally-discovered RCCs (n = 50, 88%) as opposed to the surgical cohort (n = 6, 17%). The surgical cohort had higher rates of headache (89% vs 26%, p

Background Intraspinal degenerative cysts in the cervical region are rare disorders that may cause myelopathy or radiculopathy. Most of the intraspinal degenerative cysts reported are extradural cysts. This case report includes the neuroimaging, intraoperative, pathologic, and postoperative findings obtained in a patient with a degenerative intradural cyst at the craniovertebral (CV) junction. Case Description We report a patient presenting with progressive spastic quadriparesis resulting from compressive myelopathy due to intradural degenerative cyst at the CV junction. Preoperative magnetic resonance imaging showed the intradural cyst at the cervicomedullary junction with degenerative changes causing myelopathy. We performed decompression of the CV junction, and histopathology of the cyst revealed degenerative cyst. Postoperatively the symptoms were relieved with no apparent complication. Intraspinal degenerative cysts causing compressive myelopathy are rare. To the best of our knowledge, this is the first case of intradural degenerative cyst at the CV junction. Conclusions In this case report, we demonstrated the clinical, imaging, intraoperative, and pathologic features of a degenerative intraspinal cyst at the CV junction that was intradural in location. Compression of the spinal cord resulted in a gradually progressive myelopathy that showed remarkable improvement immediately after decompression by cystectomy.

Introduction Leukoencephalopathy with calcifications and cysts (LCC) is a rare autosomal recessive cerebral angiomatous-like microangiopathy characterized by diffuse and asymmetric white-matter lesions associated with multiple calcifications and cysts. The disease is caused by SNORD118 mutations. The entire clinical spectrum of LCC is not yet fully determined. Material and methods To define the clinical spectrum of LCC, we analyzed data from recently diagnosed cases and from the litterature. Both clinical and imaging features from our five LCC cases harboring compound heterozygous SNORD118 mutations were presented and all cases reported in the litterature reviewed. Results Ninety-two LCC cases including our five patients were identified. Consanguinity was rare (4%), and 97% of cases were symptomatic. Mean age of first clinical manifestations was 16.1 ± 16.1 years (range 1 month–71 years) and was earlier in men (10.3 ± 14.3 years) than in women (20.2 ± 22.8 years) (P = 0.02). The main inaugural symptoms were seizures (36%; mean age at onset: 5.2 ± 9.5 years) and progressive neurological symptoms including ataxia, dystonia and spasticity (26%; 27.8 ± 23.6 years). Intracranial hypertension was less frequently observed (14%), mostly in adults (mean age 31.5 ± 13.2 years). Ischemic or hemorrhagic strokes were inaugural symptoms in two adults (2%). During follow-up, most patients developed progressive extrapyramidal, cerebellar and pyramidal signs (83%), cognitive decline (56%), seizures (37%), intracranial hypertension (30%) or stroke (2%). Conclusion In LCC, the clinical spectrum is largely heterogeneous and the course of the disease appears highly variable in contrast to other hereditary cerebral small vessel diseases.

To report a Rathke's cleft cyst (RCC) presenting as unilateral incomplete cavernous sinus syndrome and disc edema METHOD: Case report with literature review RESULTS: We present an 18-year-old girl who has been experiencing intermittent binocular diplopia for 1 month. Upon examination, the best corrected visual acuity was 20/20 (OU). Pronounced limitation of ocular motility with ptosis in her right eye was noted. Her pupil constricted normally. However, a relative afferent pupillary defect in the right eye was observed. Fundus examination showed disc edema in the right eye without peripapillary hemorrhage. Moreover, her thyroid function and acetylcholine receptor antibody levels were normal. Brain magnetic resonance imaging revealed a 1.6-cm mass at the suprasellar fossa and optic nerve sheath of right eye enhancement. She underwent transsphenoidal surgery, and pus-like formation was noted. Pathological findings were consistent with RCCs. After the cyst was removed, her ocular motility remarkably improved and ptosis subsided.A case of RCC presenting as multiple cranial nerve palsy was reported. RCCs are benign and usually asymptomatic. Symptomatic RCCs often manifest as headache or visual problems, such as reduced visual acuity and visual field defect. Multiple cranial nerve involvement is uncommon and indicates inflammation or compression of the cavernous sinus and optic nerve. Neuropathy may resolve after surgery; therefore, surgery is indicated for symptomatic RCCs. Further, RCC should be included in the differential diagnosis of patients with disc edema.

Background The benefit of prophylactic antibiotic use in endoscopic endonasal transsphenoidal surgery (EETS) for pituitary lesions is controversial. Many surgeons administer antibiotics perioperatively not based on clear guidelines but to be safe. The purpose of this study was to determine if antibiotic prophylaxis use reduces the risk of infection (e.g., meningitis, sinusitis) within 30 days after the surgery in adult patients with pituitary lesions undergoing EETS. Methods A systematic review was performed to assess the effectiveness of perioperative antibiotic use in preventing infectious complications in patients undergoing EETS. Data sources included Ovid Databases, Scopus, PubMed, Cochrane Library, and Grey Literature. The inclusion criteria were randomized controlled trials, systematic reviews, observational studies, and case series of prophylactic antibiotic perioperative use for EETS. The study end points were the rates of meningitis and sinusitis as infectious complications after EETS. Results A total of 282 articles were identified by the initial literature search. Four studies met the inclusion criteria: 3 retrospective cohort and 1 prospective case series studies. All patients included in each study received different antibiotic regimens perioperatively. The quality of studies did not permit performance of a meta-analysis. Conclusions Even though there are no clear practice guidelines regarding the antibiotic prophylaxis need in EETS, various antibiotic regimens have been used by surgeons. Our systematic review identified a limited number of published studies assessing this question, all observational. Randomized controlled trials are needed to evaluate the effectiveness of prophylactic antibiotic use in patients with pituitary lesions undergoing EETS.

To investigate the effectiveness of endoscopic fenestration in the patients with prior failed ventriculoperitoneal (VP) shunt treatment of suprasellar arachnoid cysts (SACs). Between 2012 and 2018, four pediatric patients of SACs with previous failed VP shunt treatment were surgically treated using endoscopic ventriculocystocisternostomy (VCC) in our hospital. The clinical symptoms, imaging data, and surgical outcomes were collected and analyzed retrospectively. A literature review is provided with regard to the reasons of shunt failure and surgical outcome of further endoscopic fenestration in the previously reported patients of SACs with prior failed VP shunt. For the 4 cases, the initial clinical symptoms relieved or even disappeared after shunt placement, but, respectively, recurred 2, 6, 11, and 6 months later. MR scans were conducted when the clinical symptoms reappeared and showed a cyst had greatly enlarged after shunt placement. Furthermore, VP shunt-related slit ventricle was also demonstrated in 3 cases. Clinical improvement and cysts shrinkage occurred in all 4 patients after VCC. Slit ventricle and hydrocephalus were also resolved. Three patients had their shunt apparatus removed after VCC, and another patient’s guardian refused to remove the shunt apparatus. Subdural hematoma occurred in one case after shunt apparatus removal. Four patients have been stable during follow-up period (mean follow-up 26.5 months). All the three patients whose VP shunt were removed were shunt independence. There were 24 patients who underwent endoscopic fenestration as an alternative to the failed VP shunt treatment in the published reports. Added our 4 patients to the published group, the effective rate of endoscopic fenestration for SACs following previous failed VP shunt treatment was approximately 93% (26/28). Of the 24 patients, the shunt apparatuses were in situ or reimplantation in 9 patients due to shunt dependence. The correction to recognize the SAC is the first condition to select the optimal management philosophy. The analysis of the series suggests endoscopic operation is still an effective and safe option in the SAC patients with previous failed VP shunt, and the shunt apparatus can be removed for some patients. The short interval time between shunt operation and endoscopic fenestration is conductive to return patients to the shunt-free state.

Objective Posterior fossa cystic malformations are diversely classified with considerable overlap without therapeutic relevance. These cysts posterior to the cerebellum, presenting in children younger than 5 years, are labeled developmental retrocerebellar cysts (DRCCs) under a new classification in relation to neuroendoscopy. Methods DRCC was categorized as type 0 for asymptomatic enlarged cistern magna and was not treated. Among symptomatic cases, cysts with a compressed fourth ventricle were labeled type 1, whereas cysts in continuity with the fourth ventricle were termed type 2. They were further categorized as subtype B if hydrocephalus was greater relative to the cyst, or otherwise as subtype A. The literature was reviewed according to PRISMA guidelines. Results There were 13 children aged 3–48 months. Type 1A DRCC was noted in 5 patients, with onset before 6 months, 4 of whom (80%) had intracranial hypertension. All underwent suboccipital endoscopic deroofing and cisternostomy (SEDC), a new technique. Type 1B DRCC was seen in 2 patients, with onset at 8–9 months, who underwent endoscopic third ventriculostomy (ETV) + endoscopic ventriculocystostomy (EVC). Type 2A DRCC was observed in 4 patients, with onset at 5–47 months, who underwent SEDC. Type 2B DRCC was noted in 2 patients, with onset 6–8 months, who underwent ETV. With a mean follow-up of 32 months, all showed clinicoradiologic improvement. The application of our classification to other studies showed ETV/EVC to be successful in only 67% of type 1A DRCC and 72% of type 2A DRCC, compared with 100% efficacy of SEDC in our series. Conclusions This is probably the first ever endoscopic classification of pediatric posterior fossa cyts, elucidating pathophysiology, presentation, and treatment. Patients with type 1 DRCC present early because of extraneous compression. Among patients with type 2 DRCC, posterior fossa compliance determines the degree of hydrocephalus. The newly described SEDC seems more appropriate for types 1A and 2A DRCC. ETV is adequate in type 2B DRCC and effective with EVC in type 1B.

Background Symptomatic cysts of the septum pellucidum (CSP) are extremely rare in children and surgical indications are not well defined. A very careful clinical and neuroradiologic evaluation is necessary to consider a patient for surgical indication. Methods We present a surgical series of 7 pediatric patients. Clinical and radiological features of the patients, including clinical presentation, previous treatment, pre, and post-operative MRI, immediate postoperative, neuropsychiatric assessment, and outcomes were reviewed. Results There were 5 males and 2 females (mean age 8 yrs). Five patients presented a history of severe intermittent headaches, two of them were admitted with acute symptoms of raised intracranial pressure. One patient presented Epilepsy and ADHD and one patient had severe psychosis. Overall, psychiatric disorders were diagnosed in six patients, three patients had Intellectual Disability (ID). In all cases, the cyst presented a ballooning feature, with a mean volume of 18,36 cm3 (range 10,62–28,5) and significant lateral bulging of both layers. All were operated on endoscopically without complications. After surgery, a very significant decrease in cyst volume was observed (mean volume 5,68 cm3; range 3,18–10,1) with complete disappearance of the ballooning aspect. Headaches resolved in all patients. In two patients operated in emergency papilloedema and vision improved in the first week after surgery. No recurrence of the cysts was noted during follow-up in all patients. Conclusions CSP may be associated with behavioral or psychiatric problems also in children. Neuroendoscopic surgery is a safe and effective therapeutic modality to treat CSP presenting with symptoms and signs of intracranial hypertension with good clinical results.

An 80-year-old woman with a background of hypothyroidism, poor mobility and previous deep vein thrombosis of the lower limb presented to hospital following a fall and hypoglycaemia. Further investigations found her to have adrenal insufficiency and low or undetectable serum thyroid stimulating

Xanthomatous hypophysitis is a rare inflammatory disease of the pituitary gland that can mimic a neoplastic lesion clinically and radiologically. Its pathogenesis remains largely unknown, although recent evidence suggests that pituitary inflammation may occur as a secondary reaction to mucous content released from a ruptured cyst. In a series of 1221 pituitary specimens, we identified seven cases of xanthomatous hypophysitis. Six patients had complete radiological and biochemical workup preoperatively: a cystic-appearing pituitary mass was identified in all six patients (100%) with a mean size of 2.0 cm (range 1.4-2.5 cm) on imaging, and pituitary endocrine dysfunction was noted in five patients (83.3%). In all cases, the pituitary mass was resected through an endoscopic transsphenoidal approach. Pathological examination revealed the presence of foamy macrophages admixed with variable amounts of giant cells and chronic inflammatory cells, confirming the diagnosis of xanthomatous hypophysitis. Additionally, all cases presented with concurrent findings of ruptured Rathke's cleft cyst, with the exception of one patient who had previous surgery for a Rathke's cleft cyst, followed by recurrence and diagnosis of xanthomatous hypophysitis. While accurate distinction of hypophysitis from a pituitary neoplasm can be problematic in the preoperative setting, the identification of a cystic lesion in the sella turcica should raise the possibility of such an entity in the clinical and radiological differential diagnosis. The current series provides further evidence that xanthomatous hypophysitis predominantly occurs as a secondary reaction to a ruptured Rathke's cleft cyst; thus, it is best classified as a secondary (reactive) hypophysitis.

Rathke’s cleft cysts are congenital lesions seen in sellar–suprasellar regions. We report a case of giant Rathke cyst in a 43-year-old patient with multi-compartmental involvement who presented with seizures with almost normal visual acuity. We discuss radiological and pathological features of various differential diagnoses for the giant suprasellar Rathke’s cyst.

A 21-year-old woman underwent surgery for a pituitary tumor. On the 11th postoperative day, blood examination revealed severe hyponatremia, with a serum sodium level of 111 mEq/l, and two days later this increased rapidly to 137 mEq/l. On the 20th postoperative day, the patient developed dysarthria and gait disturbance. Head MRI on the 30th postoperative day demonstrated intense high-signal lesions in the pons and bilateral corpus striatum on FLAIR and DWI, and central pontine and extrapontine myelinolysis was diagnosed. The patient's symptoms improved gradually after rehabilitation and antispasticity treatment. It was suggested that the changes in serum sodium levels after pituitary surgery were due to impaired secretion of antidiuretic hormone due to degeneration of nerve terminals in the posterior pituitary. As pituitary surgery may trigger changes in serum sodium leading to myelinolysis, this possibility should always be borne in mind when treating such patients.

Cholesterol granulomas in the sella are rare and can mimic the appearance of craniopharyngioma or Rathke’s cleft cysts. Information regarding the clinical presentation, imaging characteristics, and clinical course of sellar cholesterol granulomas can help clinicians to differentiate these lesions from other sellar cystic lesions. We present three cases of sellar cholesterol granulomas. A literature review was performed for all cases of sellar cholesterol granulomas with individual patient data reported. We identified 24 previously reported cases in addition to our three cases. Mean age was 36.6 years (range 5–68). There were 16 (59%) females. The most common (74%) presenting symptom was endocrinological deficits, typically either isolated diabetes insipidus (DI) or panhypopituitarism. Location was intrasellar in 3 (11%), suprasellar in 6 (22%), and intrasellar/suprasellar in 18 (67%) patients. Lesions were most commonly (83%) T1 hyperintense. Gross total resection was achieved in 16 (64%) and subtotal resection in 9 (36%) patients. Of the seventeen (63%) patients presenting with varying degrees of bitemporal hemianopsia, all had improvement in vision postoperatively. It is worth noting that no cases of preoperative hypopituitarism or DI improved postoperatively. Even though gross total resection was only achieved in 64%, there was only one recurrence reported. Sellar cholesterol granulomas are characterized by T1 hyperintensity, younger age, and more frequent and severe endocrinological deficits on presentation. Our review demonstrates high rates of improvement of visual deficits, but poor rates of endocrine function recovery. Recurrence is uncommon even in cases of subtotal resection.

Background Leukoencephalopathy with calcifications and cysts (LCC) is a rare disease in which parenchymal cysts and calcifications within a widespread leukoencephalopathy can cause a broad spectrum of neurological symptoms. We present cases with adult LCC and discuss previously described entities in relevant literature. Case presentation Two cases of adult-onset LCC confirmed by clinical presentations, typical neuroimaging and neuropathological findings are reported. Literature review A detailed search of all relevant reports published in the English language between 1996 and 2015 via PubMed ( http://www.ncbi.nlm.nih.gov/pubmed ) was performed, with “Leukoencephalopathy”, “cerebral calcifications” and “cysts” as keywords. Including the current cases, we summarized the clinical presentations, neuroimaging features, biopsy features, and genetic features of 38 LCC patients. Conclusion Our findings suggested that LCC could be diagnosed by clinical presentations, neuroimaging and gene detection, and biopsy might not be necessary. Therefore, we propose a diagnostic flow chart for neuroimaging in leukoencephalopathy, cerebral calcifications and cysts.

Background The collection of the greatest possible amount of pathologic tissue is of paramount importance in neurosurgery to achieve the most accurate histopathologic diagnosis, to perform all of the necessary biomolecular tests on the pathologic specimen, and to collect biological material for basic or translational science studies. This problem is particularly relevant in pituitary surgery because of the possible small size and soft consistency of tumors, which make them suitable for removal through suction, reducing the amount of available pathologic tissue. To solve this issue, we adopted a filter connected to the suction tube, which allows the surgeon to collect all of the tissue aspirated during surgery. Methods Our experience of 1734 endoscopic endonasal procedures, performed adopting this device since 1998 to December 2015, has been revised to assess its advantages and limitations. Results This system is easy-to-use, does not impair the surgical maneuvers, and does not add any relevant cost to the surgery. The tissue collected through the filter proved useful for diagnostic histologic and biomolecular analyses and for research purposes, without any relevant artifacts as a result of this method of collection. Conclusions The use of a filter has allowed us to obtain the greatest amount possible of pathologic tissue at each surgery. This surgical material has revealed to be helpful both for diagnostic and basic science purposes. The use of the filter has proven to be of particular importance for microadenomas, soft tumors, and supradiaphragmatic or skull base lesions with heterogeneous features, improving the accuracy of histopathologic diagnosis.

STUDY DESIGN A cadaveric study OBJECTIVE.: The aim of this study was to analyze the in situ macro- and microscopic configuration of the type II cyst and its anatomic relationship with surrounding structures. SUMMARY OF BACKGROUND DATA The lack of consensus of surgical strategy to manage symptomatic type II meningeal cysts (Tarlov cysts) is because our knowledge of type II cyst anatomy remains incomplete. It has been assumed that the cyst communicates with the subarachnoid space via microconnections. Till date, however, no direct evidence demonstrates the existence of the microconnections, although delayed contrast filling of type II cysts is commonly observed in CT myelography. METHODS Three type II meningeal cysts analyzed in this study were incidentally found in one of 16 plastinated spines. The spine was from an 89-year-old female cadaver and plastinated as a set of 164 transverse sections with a thickness of 2.5 mm. The sections were examined under a stereomicroscope. RESULTS Three type II cysts were in the thoracic spine and had a common feature that a clearly identifiable cyst neck connected the cyst body to the subarachnoid space. The dorsal root of the spinal nerve was centered in the cyst neck but spread over the cyst body or traversed the cyst cavity. The meningeal opening of the cyst was located above the inferior border of the vertebral pedicle, thus, the cyst neck hugged around the pedicle and sharply turned inferolaterally into the intervertebral foramen. The cyst body was halted by the dorsal ganglion. CONCLUSION This study reveals in situ macro- and microscopic configuration of the type II cyst and its relationship with the structures and suggests that it may be feasible to localize and ligate the cyst neck for surgical management of type II cysts. LEVEL OF EVIDENCE 3.

OBJECTIVE Patients with symptomatic Rathke's cleft cysts (RCCs) managed by surgical treatment often experience recurrence. The authors attempted to clarify the outcome of surgically treated RCCs over a long-term follow-up period. METHODS Ninety-one consecutive RCC patients with a follow-up period of more than 12 months (mean 80.2 months, range 12–297 months) were retrospectively studied. The authors examined the clinical features and postoperative course of patients who experienced a reaccumulation of cyst contents visible on MRI after the initial surgery, and they investigated data from the patients who underwent reoperation for symptomatic recurrent RCCs. RESULTS Reaccumulation of cyst contents occurred in 36 patients (39.6%). In 34 of these patients, a reaccumulation occurred in the first 5 years after surgery. The initial cysts in these patients were most often large, with squamous metaplasia in the cyst walls. Thirteen patients (14.3%) with recurrent symptoms underwent a reoperation, and 10 of the 13 patients had a reaccumulation of RCCs within the 1st year after surgery. The reoperations were performed in the 1st year (61.5%) or several years later (23.1%). Patients were likely to initially have had a visual disturbance and the cyst walls likely included squamous metaplasia. However, no association was observed between the incidence of reaccumulation/reoperation of RCCs and the surgical procedure for RCCs. CONCLUSIONS The reaccumulation rate of RCC is high in the long-term period, and it is associated with the histological findings but not with the surgical procedure. Long-term monitoring, for a period of at least 5 years, should therefore be conducted to identify and assess any RCC reaccumulation.

Cavum septi pellucidi (CSP) cysts have a very low incidence (0.04%). Symptomatic patients usually present aspecific symptoms. For this reason, the management of these patients is still debated.We selected the case of a ten year old patient, with a clinical history of frontal morning headaches and difficulty in concentration. Brain MRI documented a septum pellucidum cyst and a moderate biventricular dilation. We submitted the case, and a questionnaire concerning indications to surgery and management options to an international group of 54 pediatric neurosurgeons, analyzing the results and comparing them with the current literature.The majority of the participants (50%) indicated as appropriate at the early stage only a clinical observation. In case of persistence of clinical symptoms, 58% opted for intracranial pressure (ICP) monitoring, which, if raised, was considered by 91% as an adequate indication to proceed with surgical treatment. A total of 98% of the participants indicated endoscopic fenestration of the cyst as the preferred surgical strategy.The management of symptomatic patients with CSP cyst is controversial. Our results suggest that in most of the patients with aspecific symptoms, clinical observation and eventually ICP monitoring are adequate to identify patients for surgery.

Zika virus (ZIKV) infection acquired during pregnancy is associated with congenital microcephaly. We describe 2 cases of ZIKV infection in women in their 36th week of pregnancy whose fetuses had preserved head circumference at birth and findings of subependymal cysts and lenticulostriate vasculopathy in postnatal imaging. These represent the first signs of congenital brain injury acquired due to ZIKV in the third trimester.

Introduction: Symptomatic Rathke cleft cysts (RCCs) are rarely detected in neuroradiological screening and are less commonly found in children than in adults. However, when RCCs are observed in children, it is important to carefully distinguish a RCC from a cystic craniopharyngioma (CP) even if surgically treated or conservatively followed up. Methods: We conducted a retrospective review of clinical data from 11 patients with symptomatic RCCs whose ages were under 18 years and compared the data with data from 15 age- and sex-matched patients with cystic CP who were treated at our institute. Results: The mean age of the patients with RCCs was 12.2 years (range, 6–18). There were six males and five females. As initial symptoms, nine patients presented with headache, while two each had impaired visual function, diabetes insipidus, and activity loss. The 14 patients with CP suffered from impaired visual function. Magnetic resonance imaging (MRI) mainly showed hyperintensity on T1-weighted images (WIs) and hypointensity on T2-WI in patients with RCC. However, patients with CP had characteristic hyperintensity on T2-WI. The average maximum diameter of the RCCs was 19.0 mm on average (range, 8–33 mm). The RCCs were thus significantly smaller than CPs (34.9 mm; range, 21–54 mm). The RCCs were usually oval or dumbbell-shaped and regular in appearance, while the larger CPs were lobular and irregular. A preoperative endocrinological evaluation revealed insufficiencies in four axes in five patients with RCC. Postoperative endocrinological status improved in three patients, remained unchanged in three, and worsened in one. The gonadotropin axis was damaged in a majority (nine) of the patients with CP preoperatively. Postoperative evaluation revealed deficits in five axes in 14 patients with CP, which is a significantly different trend than observed in patients with RCC. Eight patients underwent surgical procedures (transsphenoidal surgery (TSS) in four, craniotomy in four). Two of these patients experienced a recurrence of the cysts. One of these patients subsequently underwent two craniotomies followed by radiation and other underwent TSS. Among the three conservatively treated patients, two experienced a transient worsening of their symptoms along with cyst enlargement. However, none of the three conservatively treated patients required an operation. Conclusions: When RCCs become symptomatic in children, the most common symptom they lead to is headache. The cysts are commonly small, regular, and oval in shape. Hypointensity of cyst contents on MRI is a characteristic of RCCs, which distinguishes them from CPs. Surgical intervention can be effective and lead to the relief of symptoms without a high rate of complications. However, there seems to be a relatively high recurrence rate following surgery. Thus, if the patient’s symptoms remain minor, the surgical treatment option should be used only when prudent, as the patient’s symptoms may improve over time. © 2016 Springer-Verlag Berlin Heidelberg, In Press / Embargo Period 12 months