Suprasellar and recurrent pediatric craniopharyngiomas: expanding indications for the extended endoscopic transsphenoidal approach.

OBJECTIVE The expanded endonasal endoscopic transsphenoidal approach has become increasingly used for craniopharyngioma surgery in the pediatric population, but questions still persist regarding its utility in younger children, in recurrent and irradiated tumors, and in masses primarily located in the suprasellar region. The narrow corridor, incomplete pneumatization, and fear of hypothalamic injury have traditionally relegated this approach to application in older children with mostly cystic craniopharyngiomas centered in the sella. The authors present a series of consecutive pediatric patients in whom the endonasal endoscopic approach was used to remove craniopharyngiomas from patients of varied ages, regardless of the location of the tumor and previous treatments or surgeries, to ascertain if the traditional concerns about limitations of this approach are worth reevaluating METHODS Eleven consecutive pediatric patients (age ≤ 18 years) underwent surgery via an endoscopic transsphenoidal approach at NewYork-Presbyterian/Weill Cornell Medical Center from 2007 to 2016. The authors recorded the location, consistency, and size of the lesion, assessed for hypothalamic invasion radiographically, calculated skull base measurements, and assessed parameters such as extent of resection, visual function, endocrinological function, weight gain, and return-to-school status. RESULTS The average age at the time of surgery was 7.9 years (range 4-17 years) and the tumor sizes ranged from 1.3 to 41.7 cm3. Five cases were purely suprasellar, 5 had solid components, 4 were reoperations, and 5 had a conchal sphenoid aeration. Nevertheless, gross-total resection was achieved in 45% of the patients and 50% of those in whom it was the goal of surgery, without any correlation with the location, tumor consistency, or the age of the patient. Near-total resection, subtotal resection, or biopsy was performed intentionally in the remaining patients to avoid hypothalamic injury. Anterior pituitary dysfunction occurred in 81.8% of the patients, and 63.3% developed diabetes insipidus . Two patients (18%) had a greater than 9% increase in body mass index. Visual function was stable or improved in 73%. All children returned to an academic environment, with 10 of them in the grade appropriate for their age. There was a single case of each of the following: CSF leak, loss of vision unilaterally, and abscess. CONCLUSIONS The endoscopic transsphenoidal approach is suitable for removing pediatric craniopharyngiomas even in young children with suprasellar tumors, conchal sphenoid sinus, recurrent tumors, and tumors with solid components. The extent of resection is dictated by intrinsic hypothalamic tumor invasiveness rather than the approach. The endoscopic transsphenoidal approach affords the ability to directly inspect the hypothalamus to determine invasion, which may help spare the patient from hypothalamic injury. Irrespective of approach, the rates of postoperative endocrinopathy remain high and the learning curve for the approach to a relatively rare tumor is steep.

Double pituitary adenomas are most commonly associated with GH- and ACTH-secreting tumors: systematic review of the literature.

INTRODUCTION: Double adenomas in the pituitary gland are a rare occurrence. The ability to cure a hormone-producing adenoma with surgery is dependent on the ability to identify and completely remove the correct adenoma. The relative frequency of each subtype of hormone-secreting adenomas confirmed with magnetic resonance image (MRI), surgery and immunohistochemistry is not defined. METHODS: Following PRISMA guidelines, we performed a systematic review of PubMed Central, Google Scholar, Scopus Database, Cochrane database and Science Research, using the key-words: "double pituitary adenomas", "multiple pituitary adenomas" and only papers where multiple or truly separate double pituitary adenomas were identified pre-surgically by MRI and/or confirmed by pathology/immunohistochemistry staining were included. RESULTS: We reviewed papers from 1975 to 2016 and found 17 cases with two pituitary adenomas and 1 with three pituitary adenomas. The ages ranged from 22 to 67 years old, and there were twelve females and five males. Immunohistochemical staining showed that the most common double pituitary adenomas were growth hormone (GH)- followed by adrenocorticotropic (ACTH)-secreting. CONCLUSION: Double pituitary adenomas are rare but most commonly found with GH- or ACTH-producing tumors. It is critical to remove all identified possible adenomas to achieve biochemical cure and clinicians should have heightened sensitivity in patients with acromegaly or Cushing's Disease.

Routine sectioning of the C2 nerve root and ganglion for C1 lateral mass screw placement in children: surgical and functional outcomes.

PURPOSE: Adult studies have shown that sectioning the C2 nerve root and ganglion may facilitate placement of C1 lateral mass screws and lead to decreased operative time and blood loss. We report the functional outcomes and complications following routine sectioning of the C2 nerve root and ganglion, which have not been reported in pediatric patients. METHODS: Fifteen consecutive pediatric patients underwent C1 lateral mass screw insertion and bilateral C2 nerve root and ganglion sectioning. Clinical and radiographic assessments were performed at follow-up. Numbness in the C2 distribution and/or occipital neuralgia, operative times, estimated blood loss (EBL), length of stay (LOS), and complications were recorded. RESULTS: Average follow-up time was 35.7 months. Overall mean operative time was 250.5 min, LOS was 8.46 days, and EBL was 337 cc. When considering only atlantoaxial fusions, mean operative time was 180.7 min and EBL was 97.1 cc. There were no intraoperative complications, and no patient reported new onset occipital neuralgia or numbness in the C2 distribution that would interfere with daily living. Of the patients, 93 % achieved Lenke fusion grade A; one achieved Lenke fusion grade B. CONCLUSIONS: Routine C2 nerve root sectioning and ganglionectomy enhanced surgical exposure of the C1 lateral mass and C1-2 facet joint, potentially maximizing fusion rate and minimizing intraoperative complications. This technique may yield favorable operative times, EBL, and LOS in children undergoing C1 lateral mass screw insertion without negatively affecting functional outcome.

Endoscopic endonasal odontoidectomy in a child with chronic type 3 atlantoaxial rotatory fixation: case report and literature review.

INTRODUCTION: Although the transoral transpharyngeal approach has been the standard approach to decompress the odontoid process, it bears some disadvantages including risk of infection, prolonged intubation or tracheostomy, need for nasogastric tube feeding, extended hospitalization, and possible effects of phonation. The endoscopic transnasal approach is a viable alternative, managing to avoid some of the pitfalls of the more accepted transoral transpharyngeal approach. However, there have only been a handful of adult cases and only three pediatric cases. CASE REPORT: We present the case of a 10-year-old girl with a chronic type 3 atlantoaxial rotator fixation and significant spinal cord compression from basilar invagination and a displaced odontoid process. We performed an endoscopic endonasal odontoidectomy prior to posterior occiptocervical fusion on the patient. She was neurologically intact with a well-healed wound at 7-month follow-up.

Neurogenic claudication associated with posterior vertebral rim fractures in children.

OBJECT: Outside of the patient population with achondroplasia, neurogenic claudication is rare in the pediatric age group. Neurogenic claudication associated with posterior vertebral rim fracture is even more uncommon but nonetheless causes pain and disability in affected children and adolescents. The purpose of this study was to describe the surgical results of 3 adolescents presenting with neurogenic claudication and posterior vertebral rim fracture when treated with laminectomy alone. METHODS: The medical and operative records of the 3 pediatric patients were retrospectively reviewed. Presenting signs and symptoms and CT findings, such as the interpedicular distances between T-12 and L-5, were obtained. Perioperative results were assessed, including operative time, blood loss, length of hospital stay, and complications. Findings at latest follow-up were also recorded, including a patient satisfaction survey. RESULTS: The 3 patients (1 girl and 2 boys) had a mean age of 14.7 years (range 14-15 years) and underwent follow-up for a mean of 11.3 months (range 5-18 months). Notable preoperative signs and symptoms included back pain (all patients), leg pain (all patients), leg numbness (1 patient), and leg weakness (1 patient). No patient presented with bowel and/or bladder dysfunction. The mean blood loss during laminectomy was 123 ml (range 20-300 ml), and the mean length of hospital stay was 4.3 days (range 3-6 days). On average, decompression was performed at 2.2 levels (range 2-2.5 levels). All 3 patients reported at most recent follow-up that they were "satisfied" with the surgery. There was 1 complication of instability from an iatrogenic pars fracture, which required reoperation and posterior instrumented fusion. CONCLUSIONS: To the best of the authors' knowledge, this report represents the first surgical series of pediatric neurogenic claudication associated with posterior vertebral rim fractures. Pediatric neurosurgeons may infrequently encounter neurogenic claudication associated with a posterior vertebral rim fracture in children. To treat children with neurogenic claudication associated with posterior vertebral rim fractures, a simple laminectomy may be a safe and efficacious alternative to discectomy and removal of fracture fragments.