Supinator to posterior interosseous nerve transfer to restore hand opening in brachial plexus and spinal cord injury: a systematic review and individual patient-data meta-analysis.

OBJECTIVE: Cervical spinal cord injury (SCI) and lower trunk brachial plexus injury (BPI) commonly result in hand paralysis. Although restoring hand function is complex and challenging to achieve, regaining volitional hand control drastically enhances functionality for these patients. The authors aimed to systematically review the outcomes of hand-opening function after supinator to posterior interosseous nerve (PIN) transfer. METHODS: A systematic literature review was performed according to the PRISMA guidelines. RESULTS: A total of 16 studies with 88 patients and 119 supinator to PIN transfers were included (87 transfers for SCI and 32 for BPI). In most studies, the time interval from injury to surgery was 6-12 months. Finger extension and thumb extension (Medical Research Council grade ≥ 3/5) recovered in 86.5% (103/119) and 78.1% (93/119) of cases, respectively, over a median follow-up of 19 months. The rates of recovery were similar for the SCI and BPI populations (finger extension, 87.3% in SCI and 84.3% in BPI; thumb extension, 75.8% in SCI and 84.3% in BPI). Type of injury (OR 1.05, 95% CI 0.17-6.4, p = 0.95), time from injury to surgery (OR 1.01, 95% CI 0.8-1.29, p = 0.88), and age (OR 0.97, 95% CI 0.90-1.06, p = 0.60) were not associated with odds of a successful outcome. Duration of follow-up was significantly associated with successful finger extension (OR 1.15, 95% CI 1.01-1.30, p = 0.026). No donor-associated supinator weakness was reported postoperatively given that patients had an intact bicep muscle preoperatively contributing to supination. CONCLUSIONS: Supinator to PIN transfer is a safe and effective procedure that can achieve successful restoration of digital extension in the SCI and BPI population at similar rates. Duration of follow-up was associated with superior outcomes, which was expected.

Cervical corpectomy in a pediatric patient with chondrodysplasia punctata and C5 dysplasia with spinal cord compression: illustrative case.

BACKGROUND: Chondrodysplasia punctata (CDP) describes skeletal dysplasia secondary to a variety of genetic underpinnings characterized by cartilaginous stippling from abnormal calcium deposition during endochondral bone formation. Approximately 20%-38% of patients with CDP have cervical spine abnormalities, resulting in stenosis and cord compression. However, approaches to management differ among patients. OBSERVATIONS: The authors present an 18-year-old male with a known history of CDP and cervical kyphosis with worsening paresthesias and increased spasticity. Imaging confirmed dysplastic C4 and C5 vertebra with focal kyphosis, bony retropulsion, spinal cord compression, and myelomalacia. To treat the stenosis and deformity, the patient underwent C4 and C5 vertebrectomies with C3 to C6 anterior fusion with resolution of symptoms. LESSONS: Despite many CDP patients having cervical deformities with spinal cord compression and associated neurological symptoms, there is a paucity of data on surgical management and outcomes. There are only scattered reports, and most authors recommend initial conservative management because of the high risk of operative morbidity and mortality secondary to comorbidities. When surgery is performed, long-term follow-up is recommended because of the high rates of progression of deformity, requiring subsequent operations. The authors hope that their experience adds to the literature describing the surgical management of cervical deformities in these patients.

The Clinical Impact of Vagal Nerve Stimulator Implantation on Laryngopharyngeal Function in Children: A Single-Center Experience.

OBJECTIVE: A vagal nerve stimulator (VNS) has been established as the treatment of choice for children with refractory epilepsy. The outcomes of the procedure have been well documented in adults but are less clear in children. The goal of our study was to review laryngopharyngeal (LP) function following VNS implantation in children. STUDY DESIGN: Case series with chart review. SETTING: Tertiary-care children's hospital. METHODS: Voice, swallowing, and sleep apnea symptoms were extracted from the charts of children who underwent VNS implantation between 2013 and 2021. A questionnaire was sent to parents of implanted children to ascertain the degree of the social and functional impact of the implant. RESULTS: There were 69 patients, aged 2.3 to 21.4 years old, who met the inclusion criteria. LP symptoms were most common during the first year following implantation; 26 patients (37.6%) demonstrated at least 1 symptom (voice alteration, chronic cough, sleep-disordered breathing, or dysphagia), and 15 patients required adjustments to their implant settings. The incidence of symptoms and the need to adjust VNS settings significantly dropped during years 2 to 5 and 6 to 8 (22% vs 7% and 5%, respectively, p = .0002). The mean score of the Pediatric Voice Handicap Index differed greatly from a normal control group on each subscale and the total score. CONCLUSION: LP dysfunction in children following VNS implantation is comparable to adults, with the most burden noticed during the first year after implantation. The presence of voice alterations did not correlate with the presence of dysphagia and sleep-disordered breathing. Thorough evaluation, preferably by a multidisciplinary team, is required to assess LP dysfunction postoperatively.

An adolescent case of sellar osteochondromyxoma in the setting of spondyloepiphyseal dysplasia.

PURPOSE: Osteochondromyxomas (OMX) are rare congenital bone tumors that have only been described in the context of Carney complex syndrome (CNC). Data on OMX as a separate entity and in association with other disorders remain limited, making both diagnosis and treatment difficult. METHODS: A case report of a 17-year-old female diagnosed with sellar OMX is presented in the setting of spondyloepiphyseal dysplasia (SED). We discuss the radiographic and histopathological interpretations in addition to reviewing the current literature on OMX. RESULTS: A successful gross total resection of the tumor was achieved via an endonasal endoscopic transsphenoidal approach. A diagnosis was established radiographically and pathologically. CONCLUSION: The diagnosis and treatment of OMX are best achieved via tissue biopsy. Following confirmed osteochondromyxoma cases long term for recurrence and outcomes will be essential in understanding its natural tumor history and in establishing standard treatments.

Endoscopic endonasal optic nerve decompression: treatment of fibrous dysplasia in a pediatric population.

OBJECTIVE: Patients with fibrous dysplasia (FD) of the anterior skull base can experience progressive visual loss and impairment. The authors reviewed their experience with endonasal decompression of the optic nerve (ON) in this patient population. Endoscopic ON decompression (EOND) is a feasible surgical approach for children with FD and visual deficit due to structural ON compression. METHODS: Electronic medical records of children between 1 and 17 years of age with unilateral FD of the anterior skull base and concomitant ON compression, who required EOND between 2017 and 2022 (n = 4), were reviewed for demographic data, both pre- and postoperative imaging, and evaluations by an otolaryngologist, neurosurgeon, and ophthalmologist in a multidisciplinary fashion. RESULTS: EOND was found to be a safe and effective surgery for children with FD. Visual acuity was stable in 80% of the eyes postoperatively. Visual fields improved in 40% of the eyes and remained stable in the rest. CONCLUSIONS: EOND is beneficial for progressive optic neuropathy that is unresponsive to steroid therapy and can prevent permanent disability if performed prior to irreversible damage to the nerve. EOND can decompress the edematous ON with proper exposure of the optic canal and orbital apex, without any major complications.

Pediatric pineal region tumors: institutional experience of surgical managements with posterior interhemispheric transtentorial approach.

PURPOSE: Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors resected through craniotomy with posterior interhemispheric occipital transtentorial (OT) approach are reviewed. We present the surgical techniques, pitfalls, and their results. MATERIAL AND METHODS: Eighty patients ranging in age from 3 months to 21 years old, and treated over 3 decades were reviewed. Hydrocephalus caused the main presenting symptoms and was noted in 74 patients. It was treated prior to the craniotomy for tumor resection with endoscopic third ventriculostomy (ETV) in 33, external ventricular drainage in 26, and precraniotomy shunt in 15. Nine patients had ETV together with endoscopic biopsy. All patients had a parieto-occipital craniotomy in a prone position. Through a tentorial section, a gross total resection of the tumor was attempted except for germinomas. RESULTS: The tumor pathology showed 32 germ cell tumors (GCT), 22 benign astrocytomas, 13 pineal parenchymal tumors, 5 ATRTs, 3 papillary tumors, and 5 others. Of GCTs, 18 were teratomas. The extent of resection consisted of 55 gross total resections, 13 subtotal resections, 10 partial, and 2 biopsies with one postoperative death. Hemiparesis in 2, cerebellar ataxia in another 2, and hemiballismus in 1 were transient and improved over time. One had permanent hemisensory loss and another patient had bilateral oculomotor palsy. Postoperative homonymous hemianopia occurred in 2 patients but subsided over a short period of time. Parinaud's sign was noted in 24 patients, of which 16 were transient. CONCLUSION: The posterior interhemispheric OT approach provides a safe route and comfortable access to the pineal region in children. A great majority of postoperative neurological complications are the results of direct manipulations of the midbrain at tumor resection. Identification and preservation of the tumor-brain interface are of paramount importance. GCTs other than teratomas are treated with neoadjuvant chemotherapy and may eliminate the need for craniotomy. Exophytic midbrain JPAs are amenable to resection.

Pediatric Endoscopic Endonasal Skull Base Surgery: A Retrospective Review Over 11 Years.

OBJECTIVE: To show the safety and efficacy of the endoscopic endonasal approach (EEA) for skull base surgery in pediatric patients through descriptive analysis of cases over an 11-year period. METHODS: The study comprised 94 patients undergoing EEA for skull base surgery, between January 2007 and June 2018, at 2 tertiary pediatric hospitals. Descriptive statistics are presented regarding the presentation, intraoperative details, and complications. RESULTS: Over the study period, 130 surgeries were performed in 94 patients: 94 primary surgeries and 36 reoperations. The mean patient age was 13.8 years and 48.9% of patients were female. Presenting signs/symptoms included endocrinopathies (56.4%), vision abnormalities (37.2%), and cranial nerve deficits (20.2%). EEA alone was used in 95.7% of primary surgeries and 91.7% of reoperations. Diseases treated included craniopharyngioma (18.1%), pituitary adenoma (17.0%), Rathke cleft cyst (13.8%), chordoma (9.6%), osteosarcoma (5.3%), juvenile nasopharyngeal angiofibroma (4.3%), skull base fracture (4.3%), and encephalocele (3.2%). A lumbar drain was used in 20.2% of primary surgeries and 25% of reoperations. A nasoseptal flap was used in 36.2% of primary surgeries and 25% of reoperations. Postoperative complications included cerebrospinal fluid leak (12.8%), sinusitis (7.4%), bacterial meningitis (3.2%), and carotid artery injury in 1 reoperation. CONCLUSIONS: EEA for anterior cranial base disease is safe and efficacious in pediatric patients and can be used to treat many of the rare and heterogenous diseases that arise in this anatomic location. Management strategies and rates of sinonasal and intracranial complications including cerebrospinal fluid leak rate are similar to those reported in adult cohorts.

Pediatric benign triton tumor of trigeminal nerve: a case report and literature review.

PURPOSE: Benign triton tumors (BTTs) in the pediatric population are extremely rare occurrences. Paucity of data on BTTs poses both diagnostic and therapeutic challenges, particularly when found intracranially. METHODS: A case report of a 10-year-old male diagnosed with incidental maxillary trigeminal (V2) BTT is presented. We discuss radiographic and histopathological interpretations. Furthermore, we provide a brief review of current literature and historical background on pediatric trigeminal BTT diagnosis, histopathology, and management. RESULTS: Successful gross total resection of the tumor was achieved via Dolenc approach to the cavernous sinus. Management options with consideration of outcomes from the few prior cases reported in the literature are presented. CONCLUSION: Treatment of trigeminal nerve tumors requires a broad differential diagnosis and understanding rare tumors is essential in the diagnosis and treatment algorithm.

Presentation, management and outcome of surgically managed pediatric thoracic outlet syndrome.

PURPOSE: Thoracic outlet syndrome (TOS) is a rare disorder involving compression of the brachial plexus, subclavian artery, and subclavian vein. There is a paucity of data for this pathology's surgical treatment within pediatrics. The objective of this study is to explore the presentation, management, and outcome of pediatric TOS. METHODS: A retrospective chart review was conducted for 44 patients at a single institution undergoing surgery for TOS. Data was collected on demographics, pre- and postoperative factors, and outcomes. RESULTS: Forty-four patients underwent 50 surgeries (8 bilaterally). The average age was 15.5 years with 72% female. The most common symptoms were numbness (72%) and pain (66%), with a normal exam in 58%. The average symptom duration prior to surgery was 35.2 months. A supraclavicular approach was performed in all patients, with anterior scalene section (90%), rib resection (72%), neurolysis (92%), and intraoperative EMG (84%) commonly used. Two patients had a lymphatic leak. All patients reported subjective improvement of preoperative symptoms of numbness (26%), pain (22%), and weakness (6%). Differences between vTOS (n = 9) and nTOS (n = 35) included higher preop swelling (p < 0.012), decreased symptom duration (p < 0.022), higher venogram usage (p < 0.0030), and higher preoperative thrombolytics/angioplasty (p < 0.001) in vTOS compared to nTOS. A comparison of soft tissue and soft tissue with bone decompression did not reveal any outcome differences. CONCLUSION: Pediatric TOS benefits from a multidisciplinary approach, showing good outcomes in postoperative symptom resolution. In our cohort, a supraclavicular approach provided an effective window for decompression with a low complication rate.

The role of neurosurgery in the management of tuberous sclerosis complex-associated epilepsy: a systematic review.

OBJECTIVE: Tuberous sclerosis complex (TSC) is an autosomal dominant, multisystem neurocutaneous disorder associated with cortical tubers, brain lesions seen in nearly all patients with TSC, which are frequently epileptogenic. Seizures are often the earliest clinical manifestation of TSC, leading to epilepsy in over 70% of patients. Medical management with antiepileptic drugs constitutes early therapy, but over 50% develop medically refractory epilepsy, necessitating surgical evaluation and treatment. The objective of this study was to summarize the literature and report seizure outcomes following surgical treatment for TSC-associated epilepsy. METHODS: A systematic literature review was performed in accordance with the PRISMA guidelines. The PubMed and Embase databases were searched for journal articles reporting seizure outcomes following epilepsy surgery in TSC patients. Included studies were placed into one of two groups based on the surgical technique used. Excellent and worthwhile seizure reductions were defined for each group as outcomes and extracted from each study. RESULTS: A total of 46 studies were included. Forty of these studies reported seizure outcomes following any combination of resection, disconnection, and ablation on a collective 1157 patients. Excellent and worthwhile seizure reductions were achieved in 59% (683/1157) and 85% (450/528) of patients, respectively. Six of these studies reported seizure outcomes following treatment with neuromodulation. Excellent and worthwhile seizure reductions were achieved in 34% (24/70) and 76% (53/70) of patients, respectively. CONCLUSIONS: Surgery effectively controls seizures in select patients with TSC-associated epilepsy, but outcomes vary. Further understanding of TSC-associated epilepsy, improving localization strategies, and emerging surgical techniques represent promising avenues for improving surgical outcomes.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report.

A 6-year-old girl presented with a 1-week history of progressive upper and lower extremity weakness and bilateral upper extremity dysesthesia. Imaging demonstrated a 4.7 × 1.2-cm enhancing intramedullary lesion in the cervical spine from level C2 to C5 with associated cystic components and syringomyelia. The patient underwent a C2-C5 laminoplasty, with gross total resection of the intramedullary lesion. Histological analysis showed small to medium-sized epithelioid cells, with predominantly a solid architecture focally infiltrating into the adjacent spinal cord tissue. Focal papillary differentiation was present along with peri-vascular pseudorosettes, mucin microcysts, and globules of dense collagen. Focal anaplasia was noted with mitosis (5/10 HPF), focal necrosis, and elevated Ki67 10-15%. These findings were consistent with a myxopapillary ependymoma with anaplastic features. CSF cytology was negative for tumor cells. MYCN amplification was not present. She was treated with targeted proton-beam radiation therapy. This is the fourth case of an intramedullary anaplastic myxopapillary ependymoma to date, and the first case in the cervical spine reported in the literature.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

OBJECTIVE: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS: A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS: Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

OBJECTIVE: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS: The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS: A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS: In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

Posterior Cranial Vault Distraction (PCVD): Common Complication Rates and Strategies of Prevention and Management.

BACKGROUND: Posterior cranial vault distraction (PCVD) is a well-established procedure to treat infants with craniosynostosis. Craniofacial surgeons have seen an evolution in the complications following PCVD. This report aims to demonstrate the rates of common complications from PCVD, and to identify strategies for prevention and management of these complications. METHODS: A formal literature review of studies on postoperative complications from PCVD was conducted to identify the breadth of reported complications from PCVD and rates of occurrence. RESULTS: A total of 776 articles were captured by our search strategy. Nineteen original articles met inclusion criteria, with a total of 342 patients. The average rate of reported complications was 25%, range (0%-100%). The most commonly reported complication was postoperative infection (23.7%), followed by cerebrospinal fluid leak (20.3%) and device failure (15.3%). Postoperative infection, device exposure, iatrogenic vascular injury, cerebrospinal fluid leak, and other complications have the potential to disrupt distraction and increase morbidity and mortality. CONCLUSIONS: By continuing to identify and evaluate complications, prevention strategies can be determined and standardized to decrease complications from PCVD. These efforts uphold the ultimate goal for surgeons involved in the treatment of craniosynostosis: to provide safe, effective surgical care.

Pediatric Suprasellar Germ Cell Tumors: A Clinical and Radiographic Review of Solitary vs. Bifocal Tumors and Its Therapeutic Implications.

Suprasellar germ cell tumors (S-GCTs) are rare, presenting in either solitary or multifocal fashion. In this study, we retrospectively examine 22 solitary S-GCTs and 20 bifocal germ cell tumors (GCTs) over a 30-year period and demonstrate clinical, radiographic, and prognostic differences between the two groups with therapeutic implications. Compared to S-GCTs, bifocal tumors were almost exclusively male, exhibited higher rate of metastasis, and had worse rates of progression free and overall survival trending toward significance. We also introduce a novel magnetic resonance (MR) imaging classification of suprasellar GCT into five types: a IIIrd ventricle floor tumor extending dorsally with or without an identifiable pituitary stalk (Type Ia, Ib), ventrally (Type III), in both directions (Type II), small lesions at the IIIrd ventricle floor extending to the stalk (Type IV), and tumor localized in the stalk (Type V). S-GCTs almost uniformly presented as Type I-III, while most bifocal GCTs were Type IV with a larger pineal mass. These differences are significant as bifocal GCTs representing concurrent primaries or subependymal extension may be treated with whole ventricle radiation, while cerebrospinal fluid (CSF)-borne metastases warrant craniospinal irradiation (CSI). Although further study is necessary, we recommend CSI for bifocal GCTs exhibiting high-risk features such as metastasis or non-germinomatous germ cell tumor histology.

Infratentorial choroid plexus tumors in children.

OBJECTIVE: Choroid plexus tumors (CPTs) are rare pediatric intracranial neoplasms, and mostly occur in the lateral ventricle. CPTs located in the infratentorial location are considered to be rare in the pediatric population. We present a series of eight patients treated in the last decade at our institution focusing on clinical presentations and their outcome after excision. METHODS: We performed an institutional retrospective review of patients who underwent surgical resection of infratentorial CPTs during the period from 2008 to 2017. Patients' charts were reviewed for demographic data, clinical presentation, surgical treatment, and follow-up. RESULTS: There were eight patients (6 females and 2 males), with mean age for the cohort at presentation was 9.0 years. They represent 75% of 12 CPTs of all locations treated at the same period in our institution. These 8 infratentorial CPTs were in the fourth ventricle in seven, and in the cerebellopontine angle (CPA) in one. Seven patients had choroid plexus papillomas (WHO grade I) and 1 had an atypical choroid plexus papilloma (WHO grade II). Gross total resection was attempted in all patients. However, two of 3 patients with fourth ventricle floor invasion had subtotal resection with a thin layer of tumor left on the floor. The remaining 6 had a gross total resection. Six patients with preoperative hydrocephalus had a perioperative external ventricular drainage but none required permanent shunting after tumor resection. None showed recurrence/tumor progression without adjuvant therapy during the follow-up period of 20 months to 11 years. CONCLUSION: Infratentorial dominance among pediatric CPTs in this series contradicts previous reports. Infratentorial CPTs are amenable to surgical resection. Unresected small residuals due to invasion to the fourth ventricle floor showed no regrowth during 2 to 3 years follow-up without adjuvant therapy. However, these patients with incomplete resection need watchful observations.

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established. METHODS: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length. RESULTS: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate. CONCLUSIONS: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

Nasal dermoid cysts with intracranial extension: avoiding coronal incision through midline exposure and nasal bone osteotomy.

OBJECTIVE: Up to 10% of midline nasal dermoid cysts have intracranial extension. Previous techniques of excision include frontal and frontonasal craniotomies via a coronal approach, combined with a direct cutaneous excision of the dermoid cyst. While the coronal incision allows for wide visualization, it carries significant risks of transfusion, blood loss, and scarring. The authors present an alternative technique in which access is gained through a midline extension of the dermoid cyst excision that provides direct access for a keyhole frontal craniotomy. METHODS: The authors utilize a nasal bone osteotomy, pericranial flap, and keyhole-type craniotomy performed through a nasal midline incision for the treatment of nasal dermoid cysts with intracranial extension. They performed a retrospective chart review of all patients with nasal dermoid cysts treated at the Ann & Robert H. Lurie Children's Hospital of Chicago from 2009 to 2017. Patient demographic data, operative data, and in- and outpatient complication data were collected. RESULTS: In 10 patients with cyst extension near or into the intracranial cavity (7 with true intracranial extension), the nasal osteotomy technique was performed. The mean blood loss was 13 ml, with a 0% transfusion rate. The mean length of inpatient stay was 1 day. A durotomy was made and repaired as part of the dermoid cyst dissection in 3 patients. One patient underwent intraoperative placement of a lumbar drain. The mean operative time was 228 minutes. There were no intraoperative or postoperative complications, including the need for a reoperation. No patients had any long-term complications, and no patients have had dermoid cyst recurrence. The appearance of the scar was acceptable in all cases. CONCLUSIONS: The midline approach to nasal dermoid cysts with intracranial extension is safe and results in limited blood loss, short operative times, and short lengths of inpatient hospital stay. This is a viable technique for the treatment of this challenging pathology.

Pediatric primary spinal atypical teratoid rhabdoid tumor: a case series and review of the literature.

Atypical teratoid rhabdoid tumors (ATRTs) are rare malignant central nervous system tumors, commonly occurring before 3 years of age. Median overall survival (OS) of patients with these tumors is about 1 year, despite aggressive multimodal therapy. Pediatric primary spinal ATRTs are even more rare, with fewer than 50 cases reported. The authors present a series of four patients who were treated at Ann and Robert H. Lurie Children's Hospital of Chicago in the period from 1996 to 2017.These patients, with ages 2-11 years, presented with pain and a decline in motor functions. They were found to have lesions in the lumbar, thoracic, and/or cervical spine. One patient's tumor was intramedullary with exophytic components, while another patient's tumor had both intra- and extradural components. All patients underwent resection followed by chemotherapy (systemic and intrathecal). Two patients had fractionated radiation therapy and one had an autologous stem cell transplant. Three patients are known to be deceased (OS 8.5-45 months). The fourth patient was in remission 19 years after her initial diagnosis. To the authors' knowledge, this is the largest series of pediatric primary spinal ATRTs documented at a single institution. These cases illustrate a variety of presentations of spinal ATRT and add to the body of literature on this aggressive pathology.A systematic MEDLINE search was also conducted using the keywords "atypical teratoid rhabdoid tumor," "pediatric spinal rhabdoid tumor," and "malignant rhabdoid tumor spine." Reports were included for patients younger than 21 years, without evidence of intracranial or systemic disease at the time of diagnosis. Clinical characteristics and outcomes of the four institutional cases were compared to those in the literature. This review yielded an additional 48 cases of primary pediatric spinal ATRTs reported in the English-language literature. Patients (ages 2 months to 19 years) presented with symptoms of pain, regression of motor function, and spinal cord compression. The majority of tumors were intradural (14 extramedullary, 8 intramedullary, 1 both). Eleven cases in the literature described tumors limited to extradural structures, while 10 tumors involved the intra- and extradural spine. Four reports did not specify tumor location. Although rare, spinal ATRT should be considered in the differential diagnosis of pediatric patients presenting with a new spinal mass.