Pub Date : 2025-01-06DOI: 10.1007/s00381-024-06718-9
Lukasz Antkowiak, Jerzy Luszawski, Wieslawa Grajkowska, Joanna Trubicka, Marek Mandera
Purpose: This study aimed to summarize the existing English-language literature on central nervous system (CNS) meningeal melanocytomas in children, and additionally describe our institutional case report.
Methods: PubMed database was screened on September 2, 2024, for English-language papers reporting on pediatric patients with CNS meningeal melanocytoma.
Results: A total of 17 papers reporting on 18 patients with 19 CNS meningeal melanocytomas were found in the literature. Additionally, we reported on a 15-year-old male patient with C2-C6 meningeal melanocytoma. Pediatric cohort analysis showed nearly equal sex distribution and a mean age at diagnosis of 11.9 years. There were fifteen intracranial (75%) and five spinal tumors (25%). Four lesions (20%) were diagnosed as intermediate-grade melanocytomas, while the remaining sixteen (80%) were benign meningeal melanocytomas. Most tumors were hyperintense on T1-weighted imaging (85%) and hypointense on T2-weighted imaging (73%). All tumors showed positivity for S100 and Melan-A. Most tumors were characterized by a lack of CNS invasion (91%). Gross-total resection (GTR) was performed in 61% of tumors. Adjuvant radiotherapy (RT) was applied in 50% of patients with incomplete tumor resection. Postoperatively, 62% of patients achieved a favorable outcome. We found 1, 2, 3, and 4-year overall survival of 80%, 71%, 71%, and 50%, respectively. The recurrence rate was 15% after a mean time of 10 months.
Conclusions: Meningeal melanocytomas constitute a rare subgroup of CNS tumors. Surgical tumor removal aiming at maximally safe GTR remains a standard approach, resulting in favorable postoperative outcomes. Considering high recurrence rate, long-term follow-up is needed.
{"title":"Meningeal melanocytoma of the central nervous system in children.","authors":"Lukasz Antkowiak, Jerzy Luszawski, Wieslawa Grajkowska, Joanna Trubicka, Marek Mandera","doi":"10.1007/s00381-024-06718-9","DOIUrl":"10.1007/s00381-024-06718-9","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to summarize the existing English-language literature on central nervous system (CNS) meningeal melanocytomas in children, and additionally describe our institutional case report.</p><p><strong>Methods: </strong>PubMed database was screened on September 2, 2024, for English-language papers reporting on pediatric patients with CNS meningeal melanocytoma.</p><p><strong>Results: </strong>A total of 17 papers reporting on 18 patients with 19 CNS meningeal melanocytomas were found in the literature. Additionally, we reported on a 15-year-old male patient with C2-C6 meningeal melanocytoma. Pediatric cohort analysis showed nearly equal sex distribution and a mean age at diagnosis of 11.9 years. There were fifteen intracranial (75%) and five spinal tumors (25%). Four lesions (20%) were diagnosed as intermediate-grade melanocytomas, while the remaining sixteen (80%) were benign meningeal melanocytomas. Most tumors were hyperintense on T1-weighted imaging (85%) and hypointense on T2-weighted imaging (73%). All tumors showed positivity for S100 and Melan-A. Most tumors were characterized by a lack of CNS invasion (91%). Gross-total resection (GTR) was performed in 61% of tumors. Adjuvant radiotherapy (RT) was applied in 50% of patients with incomplete tumor resection. Postoperatively, 62% of patients achieved a favorable outcome. We found 1, 2, 3, and 4-year overall survival of 80%, 71%, 71%, and 50%, respectively. The recurrence rate was 15% after a mean time of 10 months.</p><p><strong>Conclusions: </strong>Meningeal melanocytomas constitute a rare subgroup of CNS tumors. Surgical tumor removal aiming at maximally safe GTR remains a standard approach, resulting in favorable postoperative outcomes. Considering high recurrence rate, long-term follow-up is needed.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"82"},"PeriodicalIF":1.3,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142930633","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}
Pub Date : 2025-01-04DOI: 10.1007/s00381-024-06726-9
Rafael Tiza Fernandes, Rui Sobrinho, Miguel Azevedo, Mário Matos, Amets Sagarribay, Dalila Forte
Background: Subdural hematoma (SDH) typically occurs due to traumatic brain injury but can arise as a rare complication of procedures like endoscopic third ventriculostomy (ETV).
Case presentation: We report an unusual case in a 9-year-old male with previous resection of a fourth-ventricle ependymoma at 2 years of age. Seven years post-surgery, he presented with worsening hydrocephalus and underwent ETV. One month later, he developed severe headaches and motor difficulties. Imaging revealed a significant right SDH, necessitating urgent drainage. Postoperative recovery was uneventful, and follow-up imaging showed resolution of the hematoma.
Literature review and discussion: ETV is generally preferred for obstructive hydrocephalus due to lower complication rates compared to shunt procedures. However, cases of SDH post-ETV remain reported, albeit rarely. Potential mechanisms include altered cerebrospinal fluid dynamics and intraoperative vessel injury. This case aligns with literature findings and reinforces the importance of postoperative monitoring and prompt intervention in symptomatic cases to prevent complications.
Conclusion: Clinicians should consider SDH in pediatric patients with new symptoms post-ETV. Further research should focus on understanding the risk factors and mechanisms for SDH development.
{"title":"Subdural hematoma as a complication of endoscopic third ventriculostomy in a pediatric patient: a case report and literature review.","authors":"Rafael Tiza Fernandes, Rui Sobrinho, Miguel Azevedo, Mário Matos, Amets Sagarribay, Dalila Forte","doi":"10.1007/s00381-024-06726-9","DOIUrl":"https://doi.org/10.1007/s00381-024-06726-9","url":null,"abstract":"<p><strong>Background: </strong>Subdural hematoma (SDH) typically occurs due to traumatic brain injury but can arise as a rare complication of procedures like endoscopic third ventriculostomy (ETV).</p><p><strong>Case presentation: </strong>We report an unusual case in a 9-year-old male with previous resection of a fourth-ventricle ependymoma at 2 years of age. Seven years post-surgery, he presented with worsening hydrocephalus and underwent ETV. One month later, he developed severe headaches and motor difficulties. Imaging revealed a significant right SDH, necessitating urgent drainage. Postoperative recovery was uneventful, and follow-up imaging showed resolution of the hematoma.</p><p><strong>Literature review and discussion: </strong>ETV is generally preferred for obstructive hydrocephalus due to lower complication rates compared to shunt procedures. However, cases of SDH post-ETV remain reported, albeit rarely. Potential mechanisms include altered cerebrospinal fluid dynamics and intraoperative vessel injury. This case aligns with literature findings and reinforces the importance of postoperative monitoring and prompt intervention in symptomatic cases to prevent complications.</p><p><strong>Conclusion: </strong>Clinicians should consider SDH in pediatric patients with new symptoms post-ETV. Further research should focus on understanding the risk factors and mechanisms for SDH development.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"81"},"PeriodicalIF":1.3,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142926567","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}
Pub Date : 2025-01-03DOI: 10.1007/s00381-024-06734-9
Fırat Demir, Suat Demir, Fikret Baskan
A 5-month-old male patient presented with obstructive hydrocephalus caused by pineal cyst apoplexy. Ventriculoperitoneal shunt surgery was performed for hydrocephalus treatment. During follow-up, spontaneous shrinkage of the cyst was observed. At 24 months of follow-up, no complications were reported. Pediatric cases of pineal cyst apoplexy are rare, and little is known about its conservative management. This case report emphasizes the effectiveness of conservative treatment and close monitoring, aiming to contribute to the understanding of pineal cyst apoplexy management in the pediatric population.
{"title":"Conservative management of pediatric pineal cyst apoplexy: a case report and literature review.","authors":"Fırat Demir, Suat Demir, Fikret Baskan","doi":"10.1007/s00381-024-06734-9","DOIUrl":"10.1007/s00381-024-06734-9","url":null,"abstract":"<p><p>A 5-month-old male patient presented with obstructive hydrocephalus caused by pineal cyst apoplexy. Ventriculoperitoneal shunt surgery was performed for hydrocephalus treatment. During follow-up, spontaneous shrinkage of the cyst was observed. At 24 months of follow-up, no complications were reported. Pediatric cases of pineal cyst apoplexy are rare, and little is known about its conservative management. This case report emphasizes the effectiveness of conservative treatment and close monitoring, aiming to contribute to the understanding of pineal cyst apoplexy management in the pediatric population.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"80"},"PeriodicalIF":1.3,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920913","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}
Pub Date : 2025-01-03DOI: 10.1007/s00381-024-06735-8
Sanjana R Salwi, Sierra D Land, Taryn Gallagher, Tom A Reynolds, Deborah M Zarnow, Angela Viaene, Julie S Moldenhauer, N Scott Adzick, Tracy M Flanders, Gregory G Heuer
Purpose: We sought to evaluate the incidence, natural history, and management of cystic spinal lesions following myelomeningocele/myeloschisis closure.
Methods: We performed a single-center retrospective review of all patients who underwent myelomeningocele/myeloschisis closure from 2013 to 2018 with follow-up to 5 years old.
Results: We analyzed 100 fetal repairs and 81 postnatal closures from 305 total surgeries. Patients within this cohort systematically underwent serial MRI scans of the lumbar spine and had clinical follow-up until at least 5 years of age. Ninety-three (51.2%) developed radiographic evidence of cystic lesions with 28 (30.1%) requiring surgical intervention. Presence of cysts was higher in fetal repair (67/100, 67%) compared with postnatal (26/81, 32.1%; p < 0.01). Of the 93 patients with radiographic cysts, 28 (30.1%) underwent surgical resection at a median age of 27.6 months old ([Q1, Q3], [13.0, 48.6 months]). Fetal repair patients had a higher rate (26/67, 38.8%) of cysts requiring surgical resection compared with postnatal closure (2/26, 7.7%, p value < 0.01). Pathology demonstrated 18 of resected cysts were dermoid, 8 were epidermoid, and 2 were fibrous tissue. Post-operatively, no patients experienced a worsened ambulation status. Bladder compliance showed a non-significant trend toward improvement.
Conclusions: Cystic lesions in myelomeningocele/myeloschisis patients are common findings that result in nerve root tethering. We propose regular screening in both symptomatic and asymptomatic patients to circumvent nerve injury. Most cystic lesions do not require surgical resection though fetal repair is associated with a higher incidence of operative cysts. However, these lesions can be safely surgically resected with maintenance of ambulation and urologic function.
{"title":"Detailed analysis of cystic lesions in patients after open fetal repair and postnatal myelomeningocele closure.","authors":"Sanjana R Salwi, Sierra D Land, Taryn Gallagher, Tom A Reynolds, Deborah M Zarnow, Angela Viaene, Julie S Moldenhauer, N Scott Adzick, Tracy M Flanders, Gregory G Heuer","doi":"10.1007/s00381-024-06735-8","DOIUrl":"10.1007/s00381-024-06735-8","url":null,"abstract":"<p><strong>Purpose: </strong>We sought to evaluate the incidence, natural history, and management of cystic spinal lesions following myelomeningocele/myeloschisis closure.</p><p><strong>Methods: </strong>We performed a single-center retrospective review of all patients who underwent myelomeningocele/myeloschisis closure from 2013 to 2018 with follow-up to 5 years old.</p><p><strong>Results: </strong>We analyzed 100 fetal repairs and 81 postnatal closures from 305 total surgeries. Patients within this cohort systematically underwent serial MRI scans of the lumbar spine and had clinical follow-up until at least 5 years of age. Ninety-three (51.2%) developed radiographic evidence of cystic lesions with 28 (30.1%) requiring surgical intervention. Presence of cysts was higher in fetal repair (67/100, 67%) compared with postnatal (26/81, 32.1%; p < 0.01). Of the 93 patients with radiographic cysts, 28 (30.1%) underwent surgical resection at a median age of 27.6 months old ([Q1, Q3], [13.0, 48.6 months]). Fetal repair patients had a higher rate (26/67, 38.8%) of cysts requiring surgical resection compared with postnatal closure (2/26, 7.7%, p value < 0.01). Pathology demonstrated 18 of resected cysts were dermoid, 8 were epidermoid, and 2 were fibrous tissue. Post-operatively, no patients experienced a worsened ambulation status. Bladder compliance showed a non-significant trend toward improvement.</p><p><strong>Conclusions: </strong>Cystic lesions in myelomeningocele/myeloschisis patients are common findings that result in nerve root tethering. We propose regular screening in both symptomatic and asymptomatic patients to circumvent nerve injury. Most cystic lesions do not require surgical resection though fetal repair is associated with a higher incidence of operative cysts. However, these lesions can be safely surgically resected with maintenance of ambulation and urologic function.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"78"},"PeriodicalIF":1.3,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11698761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920918","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}
Pub Date : 2025-01-03DOI: 10.1007/s00381-024-06741-w
Tracy M Flanders, Misun Hwang, Nickolas W Julian, Christina E Sarris, John J Flibotte, Sara B DeMauro, David A Munson, Lauren M Heimall, Yong C Collins, Jena M Bamberski, Meghan A Sturak, Eo V Trueblood, Gregory G Heuer
{"title":"Correction: Technique and protocol for bedside neuroendoscopic lavage for post-hemorrhagic hydrocephalus: technical note.","authors":"Tracy M Flanders, Misun Hwang, Nickolas W Julian, Christina E Sarris, John J Flibotte, Sara B DeMauro, David A Munson, Lauren M Heimall, Yong C Collins, Jena M Bamberski, Meghan A Sturak, Eo V Trueblood, Gregory G Heuer","doi":"10.1007/s00381-024-06741-w","DOIUrl":"https://doi.org/10.1007/s00381-024-06741-w","url":null,"abstract":"","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"79"},"PeriodicalIF":1.3,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920915","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}
Pub Date : 2025-01-02DOI: 10.1007/s00381-024-06740-x
Min Xi, Hangyu Shi, Gang Zhang
Objective: Investigate the clinical features and treatment outcomes of neonatal cephalohematoma and ossified cephalohematoma.
Methods: A retrospective review was conducted on the clinical features of 281 children with neonatal cephalohematoma and ossified cephalohematoma managed over the previous 10 years.
Results: Of the neonatal cephalohematomas, 75 underwent puncture and aspiration, while 98 neonatal ossified cephalohematomas were treated with neurosurgery without cranioplasty. The majority (83%) required only a single puncture, with no cases of infection or ossification in this group. Pathological examination revealed that ossified cephalohematomas resulted from the progression of neonatal cephalohematomas and were characterized by subperiosteal osteogenesis.
Conclusion: Our findings suggest that a more aggressive therapeutic approach and early surgical intervention for cephalohematoma are warranted. Puncture and aspiration of neonatal cephalohematoma is a safe and effective procedure that can prevent ossification. Early neurosurgery for ossified cephalohematoma is recommended as it is technically less challenging and can minimize the risk of severe surgical trauma.
{"title":"Management of neonatal cephalohematoma and ossified cephalhematoma -281 cases of personal 10-year experience.","authors":"Min Xi, Hangyu Shi, Gang Zhang","doi":"10.1007/s00381-024-06740-x","DOIUrl":"https://doi.org/10.1007/s00381-024-06740-x","url":null,"abstract":"<p><strong>Objective: </strong>Investigate the clinical features and treatment outcomes of neonatal cephalohematoma and ossified cephalohematoma.</p><p><strong>Methods: </strong>A retrospective review was conducted on the clinical features of 281 children with neonatal cephalohematoma and ossified cephalohematoma managed over the previous 10 years.</p><p><strong>Results: </strong>Of the neonatal cephalohematomas, 75 underwent puncture and aspiration, while 98 neonatal ossified cephalohematomas were treated with neurosurgery without cranioplasty. The majority (83%) required only a single puncture, with no cases of infection or ossification in this group. Pathological examination revealed that ossified cephalohematomas resulted from the progression of neonatal cephalohematomas and were characterized by subperiosteal osteogenesis.</p><p><strong>Conclusion: </strong>Our findings suggest that a more aggressive therapeutic approach and early surgical intervention for cephalohematoma are warranted. Puncture and aspiration of neonatal cephalohematoma is a safe and effective procedure that can prevent ossification. Early neurosurgery for ossified cephalohematoma is recommended as it is technically less challenging and can minimize the risk of severe surgical trauma.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"77"},"PeriodicalIF":1.3,"publicationDate":"2025-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920922","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}
Pub Date : 2024-12-31DOI: 10.1007/s00381-024-06745-6
Matthew I Sanders, Veejay Bagga, Patricia de Lacy, Shungu Ushewokunze
Cerebral cavernous malformations (CCMs) are angiographically occult vascular lesions that present with a variety of neurological symptoms, including seizures, features of raised intracranial pressure and focal neurological deficits. In extremely rare circumstances, CCMs have presented with concomitant brain abscess formation. To date, five cases have previously been reported, the majority of which have affected patients aged 16 years or older. We present a case of a 7-year-old child with recent chickenpox infection, who presented with headache and left hemiparesis and was diagnosed with a large, deep-seated right-sided CCM with concurrent infection caused by Streptococcus pyogenes, an organism which itself is an extremely rare cause of brain abscess and may be a complication of recent chickenpox infection. The patient underwent surgical aspiration of the infected collection, completed a prolonged course of antibiotic therapy and made a good clinical recovery. Based on our review of the literature, this case represents the first reported case of Strep. pyogenes as a cause of infected CCM and only the second in a patient aged under 16 years.
{"title":"Cerebral cavernous malformation with associated Streptococcus pyogenes abscess in a child: a case report and literature review.","authors":"Matthew I Sanders, Veejay Bagga, Patricia de Lacy, Shungu Ushewokunze","doi":"10.1007/s00381-024-06745-6","DOIUrl":"10.1007/s00381-024-06745-6","url":null,"abstract":"<p><p>Cerebral cavernous malformations (CCMs) are angiographically occult vascular lesions that present with a variety of neurological symptoms, including seizures, features of raised intracranial pressure and focal neurological deficits. In extremely rare circumstances, CCMs have presented with concomitant brain abscess formation. To date, five cases have previously been reported, the majority of which have affected patients aged 16 years or older. We present a case of a 7-year-old child with recent chickenpox infection, who presented with headache and left hemiparesis and was diagnosed with a large, deep-seated right-sided CCM with concurrent infection caused by Streptococcus pyogenes, an organism which itself is an extremely rare cause of brain abscess and may be a complication of recent chickenpox infection. The patient underwent surgical aspiration of the infected collection, completed a prolonged course of antibiotic therapy and made a good clinical recovery. Based on our review of the literature, this case represents the first reported case of Strep. pyogenes as a cause of infected CCM and only the second in a patient aged under 16 years.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"76"},"PeriodicalIF":1.3,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909164","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}
Pub Date : 2024-12-30DOI: 10.1007/s00381-024-06742-9
Zubayer Shams, Ismail Bozkurt, Bipin Chaurasia
{"title":"Role of global neurosurgery in tackling pediatric brain tumors: an insight.","authors":"Zubayer Shams, Ismail Bozkurt, Bipin Chaurasia","doi":"10.1007/s00381-024-06742-9","DOIUrl":"https://doi.org/10.1007/s00381-024-06742-9","url":null,"abstract":"","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"75"},"PeriodicalIF":1.3,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909167","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}
Pub Date : 2024-12-30DOI: 10.1007/s00381-024-06744-7
Victor M Lu, James V Boddu, Allan D Levi
Wolcott-Rallison Syndrome is an extremely rare syndrome characterized by infantile non-autoimmune diabetes, extensive skeletal dysplasia, and multi-organ failure requiring transplant. Prognosis is very poor, and as such, surgical intervention for symptomatic cervical spine compromise in pediatric patients has not been widely reported in part due to their high fragility. We report a complex case of Wolcott-Rallison Syndrome that presented with cervical myelopathy due to cervicomedullary compression and the exceptional surgical considerations required for successful intervention.
{"title":"Cervical spinal decompression and fusion in the setting of Wolcott-Rallison Syndrome: a rare pediatric indication and its surgical considerations.","authors":"Victor M Lu, James V Boddu, Allan D Levi","doi":"10.1007/s00381-024-06744-7","DOIUrl":"10.1007/s00381-024-06744-7","url":null,"abstract":"<p><p>Wolcott-Rallison Syndrome is an extremely rare syndrome characterized by infantile non-autoimmune diabetes, extensive skeletal dysplasia, and multi-organ failure requiring transplant. Prognosis is very poor, and as such, surgical intervention for symptomatic cervical spine compromise in pediatric patients has not been widely reported in part due to their high fragility. We report a complex case of Wolcott-Rallison Syndrome that presented with cervical myelopathy due to cervicomedullary compression and the exceptional surgical considerations required for successful intervention.</p>","PeriodicalId":9970,"journal":{"name":"Child's Nervous System","volume":"41 1","pages":"74"},"PeriodicalIF":1.3,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909166","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}