Pediatric Neurosurgery最新文献

{"title":"Cyclic Hypertonia and Hypotonia Associated with Intrathecal Baclofen Pumps: Findings and Treatments.","authors":"Megan V Ryan, Sophia Blasco, Kaitlin E Olson, Kim Sawyer, Joyce Oleszek, Swetha Sundar, Scott LeBeau, C Corbett Wilkinson","doi":"10.1159/000546529","DOIUrl":"10.1159/000546529","url":null,"abstract":"<p><strong>Introduction: </strong>Intrathecal baclofen therapy and baclofen pumps can lead to complications, including recurrent cycles of alternating hypertonia and hypotonia. The causes and optimal treatments for this issue remain unclear. This study reviews presentations, radiologic and surgical findings, treatments, and outcomes in cases of cyclic hypertonia/hypotonia.</p><p><strong>Methods: </strong>We reviewed patients with baclofen pumps treated at our hospital from 1998 to 2024 who developed cyclic hypertonia/hypotonia. Data collected included patient sex, etiology and type of tone abnormality, age at pump placement, age and weight at symptom onset, infusion rate and type at onset, side port access and catheter dye study results, treatments, surgical findings, and outcomes.</p><p><strong>Results: </strong>We identified 15 cases in 14 patients (10 females, 71%; 4 males, 29%). Among 248 patients with baclofen pumps, 38% were female. Females were significantly more likely to develop cyclic hypertonia/hypotonia (p < 0.01). The mean age at pump implantation was 10.2 years, and the mean time from last pump surgery to symptom onset was 645 days. All patients received enteric baclofen initially; 14 also had pump rate adjustments. Three patients improved without surgery, and one died during a hypotonia episode. Side port access showed patent catheters in 7 of 8 cases, with 6 of these showing good intrathecal dye dispersion. Surgery was performed in 11 cases, including two surgeries in 6 cases. Nine patients had pump replacements; 8 also had catheter revisions. Surgical findings included catheter kinks in 4 cases (36% of surgical cases) and leaks in 5 cases (45%), with 4 leaks at the pump-connector catheter connection. Six of 11 partial revisions (55%) and all 5 complete catheter replacements (100%) resulted in symptom resolution without recurrence. Replacing the pump-connector segment resolved symptoms without recurrence in 4 of 5 patients with leaks at this site. Overall, all patients undergoing surgery experienced symptom improvement without recurrence after one or two revisions.</p><p><strong>Conclusion: </strong>Adjusting pump rates and adding enteric baclofen may help some patients with cyclic hypertonia/hypotonia. If a leak at the pump-connector catheter segment is identified, segment replacement should be considered. When partial revisions fail, complete catheter replacement appears more effective, especially as a second surgery. Cyclic hypertonia/hypotonia is a treatable complication of baclofen pumps.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"51-63"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144152810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Combined Transtemporal and High Parietal Approach for Large Trigonal Intraventricular Tumor: A Case Report.","authors":"Vich Yindeedej, Anusorn Mungmee, Thitirat Lokhoonsombut, Supaporn Konmun, Panusorn Chiensumai, Putch Phairintr, Kosuke Nakajo","doi":"10.1159/000548854","DOIUrl":"10.1159/000548854","url":null,"abstract":"<p><strong>Introduction: </strong>Surgical removal of trigonal intraventricular tumors presents significant challenges. These tumors are primarily supplied by branches of the choroidal artery group arising from the inferior side. To access the vascular pedicles, the shortest and most direct surgical corridor to the trigone is generally preferred, making the transcortical transparietal approach a common choice. However, this approach carries a high risk of damaging critical subcortical white matter tracts. Injury to these tracts can result in language deficits (on the left side), spatial neglect syndrome (on the right side), as well as sensory disturbances and visual field deficits. To minimize disruption to these white matter tracts, the high parietal approach is often recommended. However, this technique presents its own challenges, particularly in achieving early control of arterial feeders, which becomes even more critical in larger trigonal tumors.</p><p><strong>Case presentation: </strong>We present the case of a 13-year-old boy with a large trigonal intraventricular tumor. Given his lack of significant symptoms and his young age, our surgical strategy focused on maximizing the preservation of his neurological function. We developed a novel combined transtemporal and high parietal approach. The small transtemporal approach allows early devascularization, followed by the high parietal approach, which facilitates tumor resection while preserving the vital white matter tracts along the lateral wall of the trigone.</p><p><strong>Conclusion: </strong>We propose a novel combined technique that offers a balance between optimal tumor resection and functional preservation, particularly in cases of large and hypervascularized trigonal tumors.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"120-126"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145245540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Better Understanding the Orthopaedic Burden of Neurosurgical Intervention for Drug-Resistant Epilepsy in Paediatric Patients.","authors":"Adrian J Lin, Nakul S Talathi, Nicholas Gajewski, Rachel M Thompson","doi":"10.1159/000545112","DOIUrl":"10.1159/000545112","url":null,"abstract":"<p><strong>Introduction: </strong>Hemispherectomies, hemispherotomies, and lobectomies of the brain are neurosurgical techniques used to treat drug-resistant epilepsy (DRE). While effective for seizure control, these neurosurgical interventions can produce significant functional deficits including hemiparesis and iatrogenic cerebral palsy. In this study, we aimed to evaluate the resulting MSK pathology following surgery for DRE so that we may establish the incidence of new MSK pathology, identify risk factors for developing MSK pathology, and guide orthopaedic follow-up care.</p><p><strong>Methods: </strong>A retrospective chart review of 168 paediatric patients who underwent a brain hemispherectomy, hemispherotomy, or lobectomy between 2009 and 2018 was performed. Data including pre-existing neurological and orthopaedic conditions, presence of MSK pathology that emerged post-neurosurgical procedure, documented referral to orthopaedics, and post-operative interventions were collected. A multivariate logistic regression model was used to correlate predictive variables with the risk for developing new MSK pathology.</p><p><strong>Results: </strong>Of the 168 patients included, 45.2% (n = 76) developed a new MSK condition post-operatively. Of those with new MSK pathology, 30.3% (23) received orthopaedic follow-up. Of those, 34.8% (8) underwent a subsequent orthopaedic surgery. The median time to diagnosis of emerging MSK pathology following neurosurgical intervention was 0.7 months (range: 0-128 months), while the median time from emergence of symptoms to orthopaedic follow-up was 9.5 months (range: 2-161 months). Of the 28 patients who had MSK pathology prior to neurosurgical intervention, 42.8% (n = 12) were seen by orthopaedic providers following neurosurgery, of which 58.3% (n = 7) required orthopaedic surgery. Older age at the time of initial neurological surgery was significantly associated with decreased risk for developing new post-operative MSK pathology (OR 0.985, 95% CI: 0.979-0.911, p < 0.001), while repeat or revision neurosurgery was associated with increased risk (OR 3.728, 95% CI: 1.530-9.083, p < 0.01).</p><p><strong>Conclusion: </strong>Paediatric patients who undergo hemispherectomies, hemispherotomies, or lobectomies for DRE are subject to a significant post-operative burden of MSK disease, yet less than 1/3 of newly-affected patients receive orthopaedic follow-up - highlighting a gap between the need for and provision of orthopaedic care in this population.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"9-16"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143588293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary Gamma Knife Radiosurgery as a Treatment Option for Hamartoma of Floor of Fourth Ventricle: A Case Report of Pediatric Hemifacial Spasm.","authors":"Onam Verma, Manjul Tripathi, Adnan Hussain Shahid, Chirag Ahuja, Narendra Kumar, Arushi Gahlot Saini, Jitendra Kumar Sahu","doi":"10.1159/000543470","DOIUrl":"10.1159/000543470","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric hemifacial spasm (HFS) is rare, presenting early in infancy, and often fraught with subsequent psychomotor and intellectual deficits. Fourth ventricular hamartoma (FVH) is a rare cause of HFS with only 5 cases reported in literature. While gamma knife radiosurgery (GKRS) has been used to treat hypothalamic hamartomas, this is the first case of FVH treated with primary GKRS.</p><p><strong>Case presentation: </strong>A two-year-old female presented with persistent episodes of HFSs and dystonic posturing with an early resistance to medication. Thorough radiological profiling of the fourth ventricular tumor is the suggested tentative diagnosis of FVH. The patient's guardians refused surgical intervention and gave consent for GKRS aware of the lack of literature on its use in FVH. She underwent frame-based GKRS covering a total target volume of 0.986 cc with 13 Gy@50% with Leksell Perfexion. The patient showed a phasic response to GKRS with remarkable seizure control at a 1.5-year follow-up.</p><p><strong>Conclusion: </strong>Previous studies have suggested that gelastic seizures of hypothalamic hamartoma are comparable to HFSs of FVH. Our case exemplifies another key similarity between the two, i.e., a near-congruent phasic response to GKRS. This hints at the underlying pathophysiology of HFS in similar pathologies and GKRS as a treatment option in select patients.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"25-31"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142959072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multimodality Approach to a Pediatric Craniopharyngioma with Mixed Histological Features.","authors":"Sharon Y Y Low, Sharmila Devi, Enrica E K Tan, Sze Jet Aw, Khurshid Z Merchant, Ngee Lek, Marielle V Fortier, Lee Ping Ng, Sharon Y Y Low","doi":"10.1159/000546046","DOIUrl":"10.1159/000546046","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric craniopharyngiomas (CPGs) are histologically benign but clinically complex tumors. Traditional mainstays of treatment include surgical resection and radiotherapy. Molecular insights report that children tend to have the adamantinomatous subtype that is driven by the CTNNB1 pathway, while papillary CPGs prevalent in the adult population are characterized by BRAFV600E mutations. Mixed histological subtypes are rare. We report an unusual case of a pediatric CPG with both histological subtypes and discuss the management strategies in corroboration with contemporary literature.</p><p><strong>Case presentation: </strong>A 11-year-old female presented with symptoms of panhypopituitarism, optic atrophy, and bitemporal hemianopia. Magnetic resonance imaging (MRI) of her brain demonstrated a lobulated cystic-solid sellar-suprasellar lesion. The patient had emergent stereotactic aspiration of the lesion and insertion of an Ommaya reservoir. Intraoperative cyst fluid cytology confirmed wet keratin nodules, characteristic of adamantinomatous CPG. Following that, intracystic interferon-alpha therapy was commenced with good response for approximately 7 months. However, tumor progression was noted on subsequent MRI scans, with difficulty aspirating from the Ommaya reservoir. In view of this, the patient underwent an uneventful transsphenoidal resection of the tumor. Histology reported a craniopharyngioma with mixed adamantinomatous and papillary features with BRAFV600E positivity. MRI scans performed 2 months after surgery showed tumor recurrence. Decision was made for a trial of a dabrafenib - a BRAF inhibitor. After commencement of dabrafenib monotherapy for 1 month, radiological evaluation showed good tumor response. At 24 months posttreatment, the patient was well with her tumor in remission. In addition, no treatment-related adverse side effects were observed.</p><p><strong>Conclusion: </strong>We report a unique case of pediatric craniopharyngioma with mixed histological features that were managed successfully via a multimodality approach. Emphases are on molecular profiling of the said lesion, minimizing permanent morbidity and maintenance of quality of life for a growing child. In the context of tumors with BRAFV600E mutations, the use of targeted monotherapy can be considered.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"85-94"},"PeriodicalIF":1.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060352","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complex Craniosynostosis in Pitt-Hopkins Syndrome: Case Report in Twins.","authors":"José Roberto Tude Melo, Ana Rita de Luna Freire Peixoto, Danilo Marden de Lima Souza","doi":"10.1159/000536380","DOIUrl":"10.1159/000536380","url":null,"abstract":"<p><strong>Introduction: </strong>Pitt-Hopkins syndrome (PTHS) is a rare genetic syndrome associated with neurodevelopmental disorders and craniofacial dysmorphisms caused by variations in the TCF4 transition factor. The aim of this article was to report the case of two twin infants diagnosed with PTHS, confirmed by the identification of a heterozygous pathogenic variant in the TCF4 gene through DNA extracted from a buccal swab.</p><p><strong>Case presentation: </strong>Both infants presented with craniofacial asymmetry with a metopic crest and cranial deformity. During the diagnostic investigation, computed tomography with three-dimensional reconstruction of the skull showed premature fusion of the left coronal and metopic sutures in both twins. They underwent craniofacial reconstruction at the 9th month of age using a combination of techniques. The postoperative outcomes were satisfactory in both cases.</p><p><strong>Conclusion: </strong>To the best of our knowledge, this is the first case report to describe the occurrence of complex craniosynostosis (CCS) in children with PTHS. Further studies are needed to determine whether the co-occurrence of PTHS and CCS described here indicates an association or is explained by chance.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"109-114"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139514220","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Factors for Pediatric Intracranial Neoplasms in the Kids' Inpatient Database.","authors":"Anthony Price, Sean O'Leary, Hannah Parker, Liliana Camarillo-Rodriguez, Adam Husain, Mason Bartman, Ariadna Robledo, Oliver Zhou, Aaron Mohanty, Patrick Karas","doi":"10.1159/000539686","DOIUrl":"10.1159/000539686","url":null,"abstract":"<p><strong>Introduction: </strong>In children and adolescents, brain and central nervous system (CNS) tumors are the leading types of cancers. Past studies have found differing rates of intracranial cancers among races and identified additional cancer risk factors. This study aimed to see if these differences can be substantiated with further investigation of the latest version (2019) of the Kids' Inpatient Database (KID).</p><p><strong>Methods: </strong>A total of 7,818 pediatric patients &lt;21 years old in KID with ICD-10 codes consisting of malignant neoplasms of the brain, brainstem, and cerebral meninges (C700, C709-C719) were queried. Modifiable risk factors evaluated include: hospital region, insurance type, hospital city size, the average income of patient zip code, and location/teaching status of a hospital. Non-modifiable risk factors were race and sex at birth. Dependent variables were tested in Excel and GraphPad Prism 9 using a χ2 test with Yates' continuity correction and Tukey's one-way and two-way ANOVAs.</p><p><strong>Results: </strong>Mortality rates of females (2.88%) compared to males (1.99%) were significant (p &lt; 0.05). Mortality was (4.17%) in black patients compared to (1.68%) for white (p &lt; 0.0001), Hispanic mortality (2.95%) compared to white (p &lt; 0.01), and mortality of Asian/Pacific Islander (3.86%) compared to white (p &lt; 0.01). Black patients had significantly higher mortality than white, Hispanic, Asian/Pacific Islander, Native American, and other races overall (p &lt; 0.01). There was no significant difference in the mortality rates between children's hospitals and large hospitals for any race. After accounting for patient race, mortality was still not significantly different for patients with Medicaid insurance compared to non-Medicaid insurance types. Of the children treated at children's hospitals, the most transferred in from outside hospitals were Native American (20.00%) followed by Asian/Pacific Islander (15.09%) then Hispanic patients (13.67%). A significant difference between races was also seen regarding length of stay (p &lt; 0.001) and number of charges (p &lt; 0.001).</p><p><strong>Conclusion: </strong>These findings confirm prior studies suggesting gender and race are significant factors in mortality rates for children with intracranial neoplasms. However, the findings do not identify the root causes of these discrepancies but may serve as an impetus for clinicians, healthcare administrators, and governmental leaders to improve national resource allocation to better care for pediatric patients with intracranial neoplasms.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"130-142"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141297296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-Term Follow-Up of a Child with EWSR1-BEND2 Fused Spinal Astroblastoma.","authors":"Isabel Fernandes Arroteia, Maria Licci, Jürgen Hench, Andrea Bartoli, Marc Ansari, Pavlo Plavsky, Axel Terrier, Andre Oscar von Bueren, Stephan Frank, Jehuda Soleman, Raphael Guzman","doi":"10.1159/000542050","DOIUrl":"10.1159/000542050","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Spinal astroblastoma is a rare highly malignant tumor that mainly affects children. We review the few cases described in the literature and highlight the challenges of managing this neoplasm by illustrating a case recently treated at our institutions. To our knowledge, this is the first published case of EWSR1-BEND2 fused spinal astroblastoma with long-term follow-up.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Case presentation: &lt;/strong&gt;An 8-year-old girl was transferred from her home country to Switzerland for treatment of a recurrent intramedullary tumor of the cervical spine extending from C2-C7. The tumor was primarily diagnosed as an ependymoma of the spinal cord. Prior to her transfer to our department, the patient had undergone subtotal resection of the lesion, radiation therapy, multiple chemotherapy regimens, and biopsy of the recurrent tumor. Clinically, the patient presented with tetraparesis and had recently experienced worsening upper extremity weakness with complete loss of hand function. We performed a near total resection of the recurrent tumor. Ultra-fast Nanopore seq® based DNA methylome profiling allowed confirmation of the molecular diagnosis of a high-grade neuroepithelial tumor (HGNET-MN1) consistent with astroblastoma in less than 2 h, with subsequent molecular workup revealing a EWSR1-BEND2 fusion. After surgery, the patient gradually regained function in her hands. She was sent to a specialized pediatric rehabilitation center, and while the tumor was being followed radiologically with no adjuvant treatment planned, the patient presented with a relapse of the tumor in only 3 months. Given the acute worsening of radiating pain and sudden respiratory failure, a cervical decompression was performed. MRI of the cervical spine showed infiltration of the lower aspects of the brainstem. The patient was offered palliative comfort care.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;Spinal astroblastoma is a rare and highly aggressive tumor affecting children and young adults with a high recurrence rate and thus far not well-defined prognosis. The molecular signature of astroblastoma needs to be further characterized to establish a treatment-relevant classification and to allow a better prognostication. Currently, gross-total resection combined with radiotherapy remains the mainstay of treatment for spinal astroblastoma.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Introduction: &lt;/strong&gt;Spinal astroblastoma is a rare highly malignant tumor that mainly affects children. We review the few cases described in the literature and highlight the challenges of managing this neoplasm by illustrating a case recently treated at our institutions. To our knowledge, this is the first published case of EWSR1-BEND2 fused spinal astroblastoma with long-term follow-up.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Case presentation: &lt;/strong&gt;An 8-year-old girl was transferred from her home country to Switzerland for treatment of a recurrent intramedullary tumor of the cervical spine extending from C2-C7. The tumor wa","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"202-209"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11825131/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142481098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy.","authors":"Felipe Chavelas-Ochoa, Ma de la Luz Bermúdez-Rojas, Virginia Medina-Jiménez, Antonio Helue-Mena, Savino Gil-Pugliese, Ivan Gutiérrez-Gómez, Miguel Martínez-Rodríguez, Carmen Julia Gaona-Tapia, Rosa Villalobos-Gómez, Karla Aguilar-Vidales, Rogelio Cruz-Martínez","doi":"10.1159/000537723","DOIUrl":"10.1159/000537723","url":null,"abstract":"<p><strong>Introduction: </strong>Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases.</p><p><strong>Case presentation: </strong>We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting.</p><p><strong>Conclusion: </strong>This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"87-93"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139718048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric Cervical Spine Trauma: Injury Patterns, Diagnosis, and Treatment.","authors":"Taemin Oh, Kasey J Han, Vardhaan S Ambati, John K Yue, John F Burke, Alex Y Lu, Peter P Sun","doi":"10.1159/000541483","DOIUrl":"10.1159/000541483","url":null,"abstract":"<p><strong>Background: </strong>Traumatic injuries to the cervical spine or spinal cord are uncommon pathologies in the pediatric population. As injury severity is disproportionately higher among children due to significant risk for debilitating long-term disability, traumatic spinal fractures in children raise greater clinical concern than comparable injuries in adults.</p><p><strong>Summary: </strong>Unlike adults, children possess unique features such as incomplete ossification of vertebrae, synchondroses, pseudo-subluxation, horizontal alignment of ligaments, and absence of lordosis, which results in greater mobility and flexibility in the pediatric spine. These features are prominent in the cervical spine, which accounts for the most common area of traumatic spinal injuries in children.</p><p><strong>Key messages: </strong>In this review, we summarize injury patterns, diagnosis, and treatment of traumatic cervical spine injuries in the pediatric population.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"210-228"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142301148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}