Biopsies of Caudal Brainstem Tumors in Pediatric Patients-A Single-Center Retrospective Case Series.

OBJECTIVE: The indication for performing biopsies in patients with diffuse lesions in the brain stem is controversial. The possible risks associated with the technically challenging interventions must be balanced against clarifying the diagnosis and the possible therapeutic options. We reviewed the feasibility, risk profile, and diagnostic yield of different biopsy techniques in a pediatric cohort. METHODS: We retrospectively included all patients aged <18 years who had undergone biopsy of the caudal brainstem region (pons, medulla oblongata) at our pediatric neurosurgical center from 2009 to 2022. RESULTS: We identified 27 children. Biopsies were performed using frameless stereotactic (Varioguide; n = 12), robotic-assisted (Autoguide; n = 4), endoscopic (n = 3), and open biopsy (n = 8) techniques. Intervention-related mortality was not observed. Three patients experienced a transient postoperative neurological deficit. No patient experienced intervention-related permanent morbidity. Biopsy yielded the histopathological diagnosis in all 27 cases. Molecular analysis was feasible for 97% of the cases. The most common diagnosis was H3K27M-mutated diffuse midline glioma (60%). Low-grade gliomas were identified in 14% of patients. Overall survival was 62.5% after 24 months of follow-up. CONCLUSIONS: Biopsies of the caudal brainstem in children were feasible and safe in the presented setup. The amount of tumor material acquired allowing for an integrated diagnosis and was obtained at reasonable risk. The selection of the surgical technique depends on the tumor location and growth pattern. We recommend the performance of brainstem tumor biopsies in children at specialized centers to better understand the biology and enable possible novel therapeutic options.

Extended experience in parieto-occipital expansion surgery by meander technique-clinical and radiological evaluation.

INTRODUCTION: Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity or manifest in syndromic conditions. In severe cases, possible treatment options currently comprise either cranioplasty or osteogenetic distraction. The aim of this paper is to retrospectively review the perioperative course of a series of children treated by posterior meander expansion technique at our institution with focus on the course of postoperative intracranial volume and eventual tonsillar descent evolution. METHODS: Forty-two children received a posterior cranial vault remodeling by means of a posterior meander technique during a 7-year period. Hospital records were reviewed, and pre- and postoperative MRIs were analyzed for intracranial volume, cephalic and asymmetry index, and tonsillar position over time. RESULTS: Median age at surgery was 11.5 months (range 17 days-10 years). Nineteen children had a symmetrical cranial deformity, twenty-three an asymmetrical synostosis. Half of the cohort showed a syndromic condition. Transfusions were administered in the majority (92.2%) of the cases. A significant postoperative increase of intracranial volume was present from 1188.9 ± 370.4 cm3 to 1324.8 ± 352.9 cm3 (p < 0.001). The asymmetry index showed a significant improvement postoperatively: 0.86 ± 0.06 versus 0.91 ± 0.05 (p < 0.001), while the cephalic index showed a non-statistical change (0.91 ± 0.11 versus 0.88 ± 0.08). Tonsillar herniation, bilateral or homolateral, showed no significant changes at early control, while a nonsignificant amelioration of tonsillar descent was seen among children older than 12 months at late imaging follow-up. CONCLUSION: Among the osteoplastic techniques, the posterior meander technique offers several advantages, such as early mobilization of the child, less bony defects, absence of implants, and a small complication rate. However, further comparative studies among different surgical techniques are needed.

First experience with augmented reality neuronavigation in endoscopic assisted midline skull base pathologies in children.

INTRODUCTION: Endoscopic skull base approaches are broadly used in modern neurosurgery. The support of neuronavigation can help to effectively target the lesion avoiding complications. In children, endoscopic-assisted skull base surgery in combination with navigation systems becomes even more important because of the morphological variability and rare diseases affecting the sellar and parasellar regions. This paper aims to analyze our first experience on augmented reality navigation in endoscopic skull base surgery in a pediatric case series. PATIENTS AND METHODS: A retrospective review identified seventeen endoscopic-assisted endonasal or transoral procedures performed in an interdisciplinary setting in a period between October 2011 and May 2020. In all the cases, the surgical target was a lesion in the sellar or parasellar region. Clinical conditions, MRI appearance, intraoperative conditions, postoperative MRI, possible complications, and outcomes were analyzed. RESULTS: The mean age of our patients was 14.5 ± 2.4 years. The diagnosis varied, but craniopharyngiomas (31.2%) were mostly represented. AR navigation was experienced to be very helpful for effectively targeting the lesion and defining the intraoperative extension of the pathology. In 65% of the oncologic cases, a radical removal was proven in postoperative MRI. The mean follow-up was 89 ± 79 months. There were no deaths in our series. No long-term complications were registered; two cerebrospinal fluid (CSF) fistulas and a secondary abscess required further surgery. CONCLUSION: The implementation of augmented reality to endoscopic-assisted neuronavigated procedures within the skull base was feasible and did provide relevant information directly in the endoscopic field of view and was experienced to be useful in the pediatric cases, where anatomical variability and rarity of the pathologies make surgery more challenging.

Reduced rates of infection after myelomeningocele closure associated with standard perioperative antibiotic treatment with ampicillin and gentamicin.

PURPOSE: Postnatal closure of a myelomeningocele (MMC) is a complex procedure with frequent complications following surgery. Bacterial colonization of the placode may cause infection and subsequent complications. The objectives of this study were to determine the preoperative bacterial colonization rates, to assess the antibiotic regimen, and to evaluate the overall postoperative infection rate. METHODS: All consecutive patients undergoing MMC closure in our hospital from January 2010 to January 2020 were evaluated. Epidemiological data, surgical data, complication characteristics, and microbiological results were documented. RESULTS: A total of 45 patients were evaluated; in 41 patients, a wound swab of the placode was performed directly before MMC closure (91%). All patients received a prophylactic antibiotic treatment for a mean of 5.6 ± 2.7 days around the performed MMC closure. In three patients with a wound swab (7.3%), a bacterial colonization could be detected-none of the patients developed a subsequent infection. Overall, 7 other patients developed an infection (15.6%), three local surgical site infections, and four shunt-related infections. After applying a standardized perioperative prophylactic antibiotic treatment with ampicillin and gentamicin, the infection rate was observed to be lower compared with that of a non-standardized treatment (6% vs. 45%; p = 0.019). CONCLUSIONS: In neonates who undergo MMC closure in the first 48 h after birth, the colonization rate of the placode was lower than previously reported. While the data presented cannot proof the benefit of a perioperative antibiotic prophylaxis, as compared with no prophylaxis, infection rates are low with a standardized antibiotic regime comprising ampicillin and gentamicin.

Super Giant Growth Hormone-Secreting Pituitary Adenoma in Young Woman: From Ventricles to Nose.

BACKGROUND: Giant pituitary adenomas are rare tumors that can have a devastating impact on a patient's life. They require a well-studied therapeutic approach that often combines different strategies. CASE DESCRIPTION: A 29-year-old woman was diagnosed with the largest GH-secreting pituitary adenoma reported in the literature, to the best of the author's knowledge. The tumor was removed with a combined approach: endoscopic endonasal transsphenoidal and transcortical transventricular. All available acromegaly drugs were used. After 2 surgeries, a large part of the tumor was removed. Both postoperative courses were uneventful. Because the disease was still active, medical therapy was initiated. The combination of pasireotide, pegvisomant, and cabergoline permitted satisfactory control of hormonal levels. CONCLUSIONS: Giant adenomas, >4 cm, are rare pituitary tumors. Therefore in order to achieve the best clinical results, they require complex management that involves a multidisciplinary team of ear, nose, and throat surgeons; endocrinologists; radiation therapists; ophthalmologists; and neurosurgeons.

Nasal Functions in Three-Dimensional Endoscopic Skull Base Surgery.

OBJECTIVES: Endoscopic transnasal transsphenoidal surgery has become the standard procedure for the majority of skull base diseases, including sellar, parasellar, and clival pathologies. The aim of this study was the objective evaluation of nasal airflow resistances and olfactory function in 3-dimensional (3D) endoscopic transnasal transsphenoidal surgery. METHODS: One hundred patients who underwent 3D transnasal endoscopic surgery for sellar, parasellar, and clival diseases were enrolled. Active anterior rhinomanometry and Sniffin' Sticks tests were performed before endoscopic surgery and at 3 and 6 months postoperatively. RESULTS: No significant difference about nasal airflow resistance and olfactory function was observed between preoperative and postoperative subjective and objective scores. In the group of patients with sellar and parasellar diseases, a worst nasal respiratory function was seen when crusting was present, and a worst olfactory function was observed in patients with synechiae. Nasal functions returned to previous levels when crusting or synechiae solved. No statistically significant correlation was observed between the evaluated nasal functions and the reconstruction with flaps. CONCLUSIONS: The 3D endoscopic transnasal transsphenoidal surgery represents a more and more important tool in skull base surgery. It does not determine nasal respiratory and olfactory alterations after the treatment, without an increase in nasal complaints that could worsen quality of life.

3D HD Endoscopy in Skull Base Surgery: From Darkness to Light.

The introduction of modern endoscopy in neurosurgery brought light to one of the darkest areas: the skull base. In particular, the trans-nasal route allowed skull base surgeons to visualize and dominate the entire midline skull base. We analyzed our surgical series of 199 patients that were affected by several pathologies (pituitary adenomas, clivalchordomas, craniopharyngioma, Rathke's cleft cysts, tuberculum sellaemeningiomas, and craniovertebral junction pathologies with bulbar compression) and treated each by using a 3D-HD endoscope between December 2012 and December 2015 and reviewed the literature. We present our results in terms of tumor resections and decompression inpatients affected by craniovertebral junction pathologies. Analyzing our direct experience, as well as the literature, we can assert that the amount and accuracy of necessary movements in order to achieve a determined target are affected by the screen clarity and image resolution of the device. Additionally, the experience of the surgeon has an important role in the surgical outcome. Moreover, depth perception is critical in order to obtain precise and accurate movements. Our observations and the experts' opinion indicate that this modality provides improved surgical dexterity by affording the surgeon with depth perception while manipulating tissue and maneuvering the endoscope in the endonasal corridor, which is especially crucial in reducing the learning curve of young neurosurgeons.

Three-Dimensional Endoscopic Endonasal Approach and Outcomes in Sellar Lesions: A Single-Center Experience of 104 Cases.

BACKGROUND: Pituitary tumors account for approximately 15% of primary intracranial neoplasms and often are diagnosed incidentally. Common sellar lesions are pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. Currently endoscopic transsphenoidal approaches are largely used, and many centers also use 3-dimensional (3D) technology, although additional results of long-term follow-up are still being accumulated. METHODS: We present a retrospective analysis of 104 patients with sellar lesions (57 male and 47 female, mean age 52 years) who underwent 3D endoscopic transsphenoidal surgery in our center from December 2011 to March 2015 and were followed up for a mean time of 18 months. RESULTS: A total of 16 patients were diagnosed with growth hormone-secreting adenomas, 9 with adrenocorticotropic hormone-secreting, 5 with prolactin-secreting, and 2 with thyroid-stimulating hormone-secreting tumors and 58 with nonsecreting pituitary adenomas. Five patients had Rathke cleft cysts, 5 craniopharyngiomas, 2 fibrous solitary tumors, 1 a metastasis, and 1 a chordoma. At baseline, 47 patients (45.2%) had hormonal changes and 52 (50%) had visual field changes. Complete resection was achieved in 73 patients (70.1%). Follow-up evaluation detected hormonal remission in 7 patients with Cushing disease (77.7%) and in 11 patients with acromegaly (68.7%). Complications were cerebrospinal fluid leak in 5 cases (4.8%) and transient diabetes insipidus in 6 cases (5.7%). A total of 55 patients (52.9%) were discharged less than 72 hours after surgery. CONCLUSIONS: The use of an endoscopic endonasal approach with 3D technology provides several advantages relating to patient length of stay, rate of complications, postoperative recovery, and novice surgeons' training. Advantages of 3D endoscopy and long-term follow-up still need further elucidation.

Three-dimensional endoscopy in transnasal transsphenoidal approach to clival chordomas.

BACKGROUND: The purpose of this prospective, observational study was to evaluate the management of skull base chordomas surgically resected via a 3D-endoscopic transnasal approach. METHODS: Thirteen consecutive patients were observed and only 9 were surgically treated using a 3D-endoscopic transnasal approach assisted by a novel 3D visualization system. RESULTS: Nine consecutive male patients (mean age, 57.4 years) underwent exclusive 3D-endoscopic transnasal transsphenoidal resection of clival chordomas; gross total resection was achieved in 66.6% of cases (6 of 9 patients), near-total resection in 11.2% (1 of 9 patients), and partial resection in 22.2% (2 of 9 patients). The complications observed were 2 cases of postoperative cerebrospinal fluid (CSF) leaks and 1 case of temporary VI cranial nerve palsy. CONCLUSION: No discomfort was recorded; when a dura opening was required, 3D vision allowed an accurate intradural sharp dissection and a precise repair of the skull base. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1814-E1819, 2016.