Spinal ganglioneuroma: a rare and challenging tumor in the pediatric population.

BACKGROUND: "Spinal ganglioneuroma" is a rare entity of neuroblastic tumors, frequent among children, that has been sparsely reported in articles involving both the pediatric and adult populations. These tumors mostly arise from the sympathetic ganglia located in the paravertebral region, near the intervertebral foramina of the spine. This makes their extension into the spinal canal possible but quite rare, in a dumbbell fashion, producing radicular or medullary symptoms. We provide an extensive review of the pediatric cases found in the literature; while reporting a challenging case we have recently got to treat at the CHRU de Brabois in Nancy, France. METHODS: We searched PubMed's database for articles and abstracts related to "spinal ganglioneuromas," from inception until June 1, 2024. We combined every case among patients under 18 years of age and divided them between the different spinal areas. We excluded those that originated from outside of the spine. We particularly focused our attention on the thoraco-lumbar region, which involved our case report, since the surgical management is completely different from other regions of the spine. RESULTS: As per June 1, 2024, 21 patients aged between 3 and 17 years were identified with "spinal ganglioneuromas," with a predominance of females (81%). The thoracic region of the spine seems to be the area of predilection for these tumors (42.85%), although some have been found in the cervical (9.5%), cervico-thoracic (4.76%), thoraco-lumbar (19.04%), lumbar (19.04%), and sacral (4.76%) regions. DISCUSSION: Our specific case included long-date abdominal pain and recent progressive paraparesis as the main symptoms, with radiological manifestations of renal atrophy and hydronephrosis, due to the compression and complete displacement of the kidney, as well as scoliosis. To our knowledge, this combination of symptoms has never been seen with spinal ganglioneuromas before in the pediatric population, given the gigantic size of the tumor, and its significant extension to both the spinal canal and the retroperitoneal space, which is quite uncommon. CONCLUSION: Spinal ganglioneuromas are very rare, benign lesions, that should be included in the differential diagnosis of dumbbell tumors that can cause in the pediatric population, alongside neurological symptoms in the limbs and back pain, thoracic, abdominal, and even urinary symptoms.

Excitability of the radiculo-medullary circuitry in spastic cerebral palsy: An intraoperative neurophysiological study in children undergoing selective dorsal rhizotomy.

AIM: To explore - through intraoperative neurophysiology mapping and recordings - the comparative distribution of the reflexive excitability of the L2 to S2 radiculo-metameric segments of the spinal cord in a series of children with bilateral spastic cerebral palsy (CP) who underwent selective dorsal rhizotomy (SDR). METHOD: Our series included 46 consecutive children (36 males, 10 females; aged 5-16 years, mean 8 years) who underwent SDR, using keyhole interlaminar dorsal rhizotomy. The procedure allowed access to all L2 to S2 roots independently, while preserving the posterior architecture of the lumbar spine. Dorsal roots were stimulated selectively to test reflexive excitability of the corresponding radiculo-metameric levels. Stimulation parameters were identical for all roots for optimal comparison between root levels, with an intensity just above threshold to avoid excessive diffusion. The responses in the main muscular groups in each lower limb were clinically observed and electromyograms recorded. Degrees of excitability were quantified according to Fasano's scale. RESULTS: The difference between root levels was highly significant. Median values of excitability were 1, 2, 3, 3, 3, and 3 for the L2, L3, L4, L5, S1, and S2 levels respectively. Lower root levels exhibited significantly more excitability. INTERPRETATION: In addition to insight into the spasticity of children with CP, the profile of segmental excitability can be useful in establishing surgical planning when programming SDR. WHAT THIS PAPER ADDS: Keyhole interlaminar dorsal rhizotomy modality allowed selective stimulation of all L2-S2 dorsal roots for testing excitability. There were significant differences in reflexive excitability of L2-S2 radiculo-medullary segments. Lower segments of L2-S2 medullary levels have higher excitability. Interindividual variability in excitability of lumbosacral segments justifies intraoperative neurophysiology. This original article is commented on by Young on pages 9-10 of this issue.

Postural control in Chiari I malformation: protocol for a paediatric prospective, observational cohort - potential role of posturography for surgical indication.

INTRODUCTION: Chiari I malformation (CM1) is an anatomical abnormality characterised by the cerebellar tonsils descending at least 5 mm below the foramen magnum. CM1 causes obstruction of cerebrospinal fluid (CSF) circulation as well as direct compression on the brainstem, thus causing typical consequences (syringomyelia), and typical clinical features (characteristic headaches and neurological impairment). Surgery is the only available treatment, indicated when symptomatology is present. However, sometimes patients have atypical complaints, which are often suggestive of otolaryngological (ears, nose and throat, ENT) involvement. This may be difficult for a neurosurgeon to explain. Our study aims to investigate the relationship between one of these atypical symptoms, for example, postural instability, in a paediatric population using a Computerised Dynamic Posturography (Equitest, NeuroCom, Clackamas, OR). To our knowledge, there are no previously published studies carried out on children with CM1, using dynamic posturography. METHODS AND ANALYSIS: Forty-five children aged 6-18 years old presenting with radiologically confirmed CM1 and presenting ENT clinical complaints will be included in the study for a duration of 3 years. As primary endpoint, posturographic results will be described in the population study. Second, posturographic results will be compared between patients with and without indication for surgery. Finally, preoperative and postoperative posturographic results, as well as CSF circulation quality at foramen magnum level, syringomyelia, sleep apnoea syndrome, scoliosis and behaviour will be compared in the operated patient group. ETHICS AND DISSEMINATION: This protocol received ethical approval from the Clinical Research Delegation of Nancy University Hospital, in accordance with the National Commission on Informatics and Liberties (Commission Nationale de l'Informatique et des Libertés) (protocol number 2019PI256-107). Our data treatment was in accordance with the Methodology of reference Methodology Reference-004 specification for data policy. The study findings will be disseminated via peer-reviewed publications and conference presentations, especially to the Neurosphynx's rare disease healthcare network. TRIAL REGISTRATION NUMBER: NCT04679792; Pre-results.

How I do it: Selective dorsal rhizotomy, using interlaminar approaches, for spastic diplegia/quadriplegia in children with cerebral palsy.

BACKGROUND: Dorsal rhizotomy is considered the gold standard for treating spastic diplegia/quadriplegia in children with cerebral palsy, when rehabilitation programs reveal insufficient to control excess of spasticity. METHOD: The Keyhole Interlaminar Dorsal rhizotomy modality has been developed to access-individually-all L2-S2 roots, intradurally at the corresponding dural sheath, and preserve the posterior spine architecture. Intraoperative neuromonitoring consists of stimulating each ventral root, to verify its myotomal innervation, and dorsal roots, to explore their reflexive muscular responses in order to help determination of the proportion of rootlets to be cut. CONCLUSION: This modality, which requires 5 ± 1 h duration, offers tailored accuracy.

Usefulness of external anal sphincter EMG recording for intraoperative neuromonitoring of the sacral roots-a prospective study in dorsal rhizotomy.

BACKGROUND: In conus medullaris and cauda equina surgery, identification of the sacral nerve roots may be uncertain in spite of their anatomical/radiological landmarks. Mapping the sacral roots by recording the muscular responses to their stimulation may benefit from EMG recording of the External Anal sphincter (EAS) in addition to the main muscular groups of the lower limbs. METHOD: In a consecutive series of 27 lumbosacral dorsal rhizotomy (DRh), authors carried out a prospective study on the reliability of the EMG recording of the EAS for identification of the S1 and S2 sacral roots. RESULTS: An EAS-response was recorded in all the 27 (bilaterally) explored individuals, testifying good sensitivity and selectivity of the method. EAS-responses were obtained in 96.3% of the 54 stimulated sides of the S2 root versus in only 16.66% for the S1 root, so that an absence of response would indicate S1 rather than S2 level. Furthermore, comparison between myotomal distribution of the S1 and S2 roots showed a significant difference (p < 0.00001), so that myotomal profile may help to identify root level. CONCLUSIONS: EMG recording of the EAS can be recommended for current intraoperative neuromonitoring. This simple method also provides-indirectly by extrapolation-information on the sacral motor pathways of the external urethral sphincter (EUS), as the later has the same somatic innervation via the pudendal nerve and related S2, S3, and S4 roots. Method can be helpful not only for DRh, of all varieties, but also for spine surgery, correction of dysraphisms, lipomas and/or tethered cord, and tumor resection.

Early surgical management of antenatal diagnosed cystic lesions of the foramen of Monro causing monoventricular hydrocephalus.

INTRODUCTION: After diagnosis of an antenatal monoventricular hydrocephalus caused by a cystic lesion of the foramen of Monro, treatment modality and time frame may be difficult to assess. Previously, this type of hydrocephalus was often treated with internal shunting. The advent of neuroendoscopy has changed the surgical management of this pathology. MATERIALS AND METHODS: We report on two cases of cystic lesions of the foramen of Monro discovered on antenatal ultrasonography. A cavum veli interpositi in one case and a choroid plexus cyst in the other were responsible for unilateral hydrocephalus. In both cases, endoscopic treatment before 1 month of age was performed as the primary procedure with a successful outcome. CONCLUSIONS: We advise early endoscopic treatment for newborns presenting with progressive unilateral hydrocephalus caused by a cystic lesion of the foramen of Monro. It has been, in our hands, a safe and efficient procedure.