Pediatric Neurosurgery最新文献

{"title":"Management of Pediatric Patient with Multiple Cranial, Intracranial, and Spinal Manifestations of Penttinen Syndrome: A Case Report.","authors":"Megan V Ryan, Reinier Alvarez, Ellen R Elias, Chinonye Ihekweazu, C Corbett Wilkinson","doi":"10.1159/000540756","DOIUrl":"10.1159/000540756","url":null,"abstract":"<p><strong>Introduction: </strong>Penttinen premature aging syndrome is caused by mutations in the PDGFRB gene. We describe the case of a 10-year-old girl with a de novo c.1994T&gt;C variant in PDGFRB who developed multiple cranial, intracranial, and spinal manifestations, including macrocephaly, enlarged convexity subarachnoid spaces crossed by numerous vascularized arachnoid trabecule, hydrocephalus, spinal epidural lipomatosis, a low conus medullaris, calvarial thinning with large anterior fontanelle, and a skull fracture with bilateral epidural hematomas. Vascularized arachnoid granulations, spinal epidural lipomatosis, and low conus medullaris have not been previously described in Penttinen syndrome.</p><p><strong>Case presentation: </strong>A female with Penttinen syndrome diagnosed at 9 years of age initially presented as an infant with cutaneous hemangiomas and macrocephaly; imaging showed enlarged convexity subarachnoid spaces. Her convexity subarachnoid spaces continued to expand, leading to subdural shunt placement. At surgery, her enlarged subarachnoid spaces were found to contain numerous abnormally thick, vascularized arachnoid trabecule. Eventually, her subdural shunt failed and her ventricles enlarged, leading to ventricular shunt placement. A large, sunken anterior fontanelle which did not diminish in size led to cranioplasty with a custom implant. She later developed chronic back pain and imaging revealed spinal epidural lipomatosis, a low conus medullaris, and mild scoliosis. At 10 years of age, a fall from a chair resulted in a depressed skull fracture and bilateral parietal epidural hematomas. Emergency left parietal craniotomy was performed for evacuation of the left hematoma, and the patient recovered without complications. Intraoperatively, it was noted that her skull was extremely thin.</p><p><strong>Conclusion: </strong>This case report highlights the clinical presentation and multifaceted neurosurgical management of a patient with Penttinen syndrome. The patient exhibited characteristic features including hypertrophic skin lesions, macrocephaly, and skeletal abnormalities. Our patient's vascularized arachnoid trabecule, spinal epidural lipomatosis, and low conus medullaris have not previously been reported in Penttinen syndrome. Her thin skull potentially contributed to the extent of her depressed skull fracture after her backwards fall and predisposed her toward developing epidural hematomas. Patients with Penttinen syndrome can have multiple cranial, intracranial, and spinal manifestations which may need the attention of a neurosurgeon.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"165-172"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141972303","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":"Symptomatic Multi-Nodular Fourth Ventricular Choroid Plexus Papillary Fibrosis in the Context of Chiari II Malformation: Case Report and Literature Review.","authors":"Brandon Edelbach, Rasha G Elbadry, Jeremy Deisch, Brian W Hanak","doi":"10.1159/000541699","DOIUrl":"10.1159/000541699","url":null,"abstract":"<p><strong>Introduction: </strong>Chiari II malformation (CM-II) is a congenital malformation characterized by the caudal displacement of the cerebellar tonsils, inferior cerebellar vermis, and fourth ventricle through the foramen magnum. Although rare, the association between CM-II and the development of a variety of mass lesions within the fourth ventricle and craniocervical junction has been described in the literature.</p><p><strong>Case presentation: </strong>We present the case of a 9-year-old boy with a history of mid-lumbar myelomeningocele, CM-II, and ventriculoperitoneal shunt-dependent hydrocephalus who was discovered to have a syringomyelia and numerous spherical/ovoid masses within the caudal fourth ventricle and dorsal cervicomedullary subarachnoid space on a surveillance brain MRI. On questioning, the patient endorsed a longstanding history of poor bilateral hand dexterity and grip strength. After further imaging workup, the patient underwent suboccipital craniectomy and C1 laminectomy for resection of the mass lesions arising from the fourth ventricular choroid plexus (CP) and performance of an expansile pericranial duraplasty. The patient tolerated surgery well and had progressive improvement in hand dexterity/strength as well as radiographic improvement in the cervical cord syrinx after surgery. Pathologic analysis of the resected mass lesions demonstrated the lesions to be predominantly characterized by dense nodular fibrosis of the CP stromal cores which we are describing as choroid plexus papillary fibrosis. A minority of the lesional stromal cores were noted to be edematous, imparting a more \"reticular\" appearance. There were no features to suggest a neoplastic or infectious process.</p><p><strong>Conclusion: </strong>This combination of dysplastic/reactive CP histologic findings has not been previously reported in an extramedullary location. The unique pathology of this patient's CP lesions will be discussed, and previously reported fourth ventricular mass lesions seen in association with CM-II will be reviewed.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"193-201"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142513114","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":"Traumatic Head Injuries from Ceiling Fans at a Pediatric Level I Trauma Center in the United States.","authors":"Logan Muzyka, Holly Hughes Garza, Diala Merheb, Julie Sanchez, Elizabeth Tyler-Kabara, Karla A Lawson","doi":"10.1159/000535335","DOIUrl":"10.1159/000535335","url":null,"abstract":"<p><strong>Background/objective: </strong>Several studies describe traumatic head injuries caused by ceiling fans in Australia, the Middle East, and Malaysia. Some injuries required neurosurgical intervention, especially those caused by metallic ceiling fans. This study describes traumatic head injuries caused by ceiling fans at a single pediatric level 1 trauma center in the Southern USA.</p><p><strong>Methods: </strong>Medical records were retrospectively reviewed for patients under 18 years of age who presented with a traumatic injury to the head from a ceiling fan from January 1, 2008, through December 31, 2021. The cohort of patients meeting all inclusion criteria was identified by querying multiple free-text fields derived from the electronic medical record, followed by a manual record review.</p><p><strong>Results: </strong>Of 60 children treated for traumatic head injury from a ceiling fan, the median age was 5.7 years and 53% were female. Laceration was the most common injury (80%), followed by scalp swelling/hematoma (20%), contusion (8%), and skull fracture (7%). Two patients (3%) with intracranial hemorrhage and fracture underwent neurosurgery. One neurosurgical case involved a metal ceiling fan and the other involved an outdoor ceiling fan. Nearly half of the injuries involved bunk or loft beds (47%) and young children were often injured while being lifted up by a caregiver (18%).</p><p><strong>Conclusion: </strong>Although most pediatric traumatic head injuries from ceiling fans resulted in minor injuries, our center saw a similar proportion of cases with skull fractures to what has been reported in Australia (5%). The effects of fan construction and blade material on the severity of head injury may warrant further study. Understanding the most common mechanisms for these injuries may guide injury prevention efforts.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"14-19"},"PeriodicalIF":0.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138048811","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":"Middle Meningeal Artery Embolization in Pediatric Patients.","authors":"Sima Vazquez, David A Zuckerman, Chirag Gandhi, Fawaz Al-Mufti, Carrie Muh, Justin Santarelli, Jared M Pisapia","doi":"10.1159/000534895","DOIUrl":"10.1159/000534895","url":null,"abstract":"<p><strong>Background: </strong>Middle meningeal artery (MMA) embolization has been increasingly applied in adult populations for the treatment of chronic subdural hematomas (cSDH). There is a paucity of literature on the indications, safety, and outcomes of MMA embolization in the pediatric population.</p><p><strong>Summary: </strong>A systematic literature review on pediatric patients undergoing MMA embolization was performed. We also report the case of successful bilateral MMA embolization for persistent subdural hematomas following resection of a juvenile pilocytic astrocytoma. Persistent bilateral subdural hematomas following resection of a large brain tumor resolved following MMA embolization in a 13-year-old male. Indications for MMA embolization in the pediatric literature included cSDH (6/13, 46.2%), treatment or preoperative embolization of arteriovenous fistula or arteriovenous malformation (3/13, 23.1%), preoperative embolization for tumor resection (1/13, 7.7%), or treatment of acute epidural hematoma (1/13, 7.7%). Embolic agents included microspheres or microparticles (2/13, 15.4%), Onyx (3/13, 23.1%), NBCA (3/13, 23.1%), or coils (4/13, 30.8%).</p><p><strong>Key messages: </strong>Whereas MMA embolization has primarily been applied in the adult population for subdural hematoma in the setting of cardiac disease and anticoagulant use, we present a novel application of MMA embolization in the management of persistent subdural hematoma following resection of a large space-occupying lesion. A systematic review of MMA embolization in pediatric patients currently shows efficacy; a multi-institutional study is warranted to further refine indications, timing, and safety of the procedure.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"44-53"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71415312","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":"Evolution of Surgical Management of Pineal Region Tumors in the Pediatric Population: A 17-Year Experience at a Single Institution.","authors":"Nirali Patel, Alexandria Marino, Tianzhi Tang, Kelsi Chesney, Jean Paul Bryant, Trae Robison, Robert F Keating, John S Myseros, Hasan R Syed","doi":"10.1159/000538745","DOIUrl":"10.1159/000538745","url":null,"abstract":"<p><strong>Introduction: </strong>Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these patients, and this is well demonstrated by our single institution's experience over a 17-year-period in which the evolution of diagnosis, treatment, and outcomes of pineal tumors in pediatric patients will be outlined.</p><p><strong>Methods: </strong>We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children's National Hospital (CNH) from 2005 to 2021. Variables analyzed included presenting symptoms, presence of hydrocephalus, diagnostic and surgical approach, pathology, and adverse events, among others. IRB approval was obtained (IRB: STUDY00000009), and consent was waived due to minimal risk to patients included.</p><p><strong>Results: </strong>A total of 43 pediatric patients with pineal region tumors were treated during a 17-year period. Most tumors in our series were germinomas (n = 13, 29.5%) followed by pineoblastomas (n = 10, 22.7%). Twenty seven of the 43 patients (62.8%) in our series received a biopsy to establish diagnosis, and 44.4% went on to have surgery for resection. The most common open approach was posterior interhemispheric (PIH, transcallosal) - used for 59.3% of the patients. Gross total resection was achieved in 50%; recurrence occurred in 20.9% and mortality in 11% over a median follow-up of 47 months. Endoscopic third ventriculostomy (ETV) was employed to treat hydrocephalus in 26 of the 38 patients (68.4%) and was significantly more likely to be performed from 2011 to 2021. Most (73%) of the patients who received an ETV also underwent a concurrent endoscopic biopsy. No difference was found in recurrence rate or mortality in patients who underwent resection compared to those who did not, but complications were more frequent with resection. There was disagreement between frozen and final pathology in 18.4% of biopsies.</p><p><strong>Conclusion: </strong>This series describes the evolution of surgical approaches and outcomes over a 17-year-period at a single institution. Complication rates were higher with open resection, reinforcing the safety of pursuing endoscopic biopsy as an initial approach. The most significant changes occurred in the preferential use of ETVs over ventriculoperitoneal shunts. Though there has been a significant evolution in our understanding of and treatment for these tumors, in our series, the outcomes for these patients have not significantly changed over that time.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"66-77"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11232948/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140866217","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":"Draining Vein Thrombosis of Developmental Venous Anomaly in Sickle Cell Trait Patients: A Case Report and a Literature Review.","authors":"Muhammed Amir Essibayi, Genesis Liriano, Kaitlin L Strumph, Deepa Manwani, Mandana Behbahani, David J Altschul","doi":"10.1159/000540929","DOIUrl":"10.1159/000540929","url":null,"abstract":"<p><strong>Introduction: </strong>Cerebral venous sinus thrombosis (CVST) is a rare but serious condition in both adults and children. Risk factors include thrombophilias, dehydration, and certain inherited conditions like sickle cell trait (SCT). We present a case of CVST in a pediatric patient with SCT to highlight key considerations in diagnosis and management.</p><p><strong>Case presentation: </strong>A 14-year-old male with SCT presented with worsening headache and vomiting after prolonged sun exposure and dehydration during athletic camp. Imaging revealed right occipital hemorrhage, hydrocephalus, right CSVT, and bilateral cerebellar developmental venous anomalies. Hypercoagulability testing was normal. Diagnostic evaluation included computed tomography, magnetic resonance imaging, MR venography (MRV), and hypercoagulability testing. The patient was treated with an external ventricular drain, platelet transfusion, and anticoagulation. Management also involved hydration, platelet transfusion, supportive care, and multidisciplinary follow-up. Follow-up MRV showed recanalization.</p><p><strong>Conclusion: </strong>This case highlights SCT as a potential CVST risk factor. Timely recognition, evaluation of precipitants like dehydration, supportive care including anticoagulation, and multidisciplinary management are important. An individualized approach is needed to balance thrombosis recurrence and bleeding risks. Patients with SCT require education on risks and prompt evaluation of neurological symptoms to allow early diagnosis and care of CVST.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"173-180"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142005919","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":"Neurosurgical Management of Rubinstein-Taybi Syndrome: An Institutional Experience.","authors":"Regan M Shanahan, Joseph S Hudson, Martin G Piazza, Faith Kehinde, Sharath Kumar Anand, Damara Ortiz, Suneeta Madan-Khetarpal, Stephanie Greene","doi":"10.1159/000540931","DOIUrl":"10.1159/000540931","url":null,"abstract":"<p><strong>Introduction: </strong>Rubinstein-Taybi syndrome (RTS) is a rare genetic condition with a distinctive set of physical features. This case series reports a single institutional experience of RTS cases, highlighting the role of neurosurgery in the comprehensive management of RTS patients.</p><p><strong>Methods: </strong>A retrospective review of patients with genetically confirmed RTS presenting between 2010 and 2023 at Children's Hospital of Pittsburgh was performed. Patient demographics, genetic profile, clinical symptoms, radiographic characteristics, and neurosurgical management were recorded for all patients.</p><p><strong>Results: </strong>Twenty-one patients (13 females, 8 males) aged 0 to 22 years presented for formal genetic counseling and diagnosis. Twenty patients (95%) had CREBBP pathogenic variants (RTS type 1), and 1 patient (5%) had EP300 pathogenic variants (RTS type 2). Ten patients (48%) had a low-lying conus medullaris, and 3 patients (30%) underwent subsequent spinal cord detethering. Four patients (19%) had a Chiari malformation, and three (75%) underwent Chiari decompression surgeries. One patient (5%) had Chiari-associated syringomyelia.</p><p><strong>Conclusions: </strong>RTS patients have an increased rate of tethered cord syndrome requiring detethering. The incidence of symptomatic Chiari I malformation requiring decompression has not been previously reported. The RTS series presented here demonstrates a high incidence of symptomatic Chiari I malformation in addition to tethered cord syndrome.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"181-192"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142005941","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":"A Case Report of Infant-Type Hemispheric Glioma with a Novel GAB1-ABL2 Kinase Fusion Treated with Dasatinib.","authors":"Emery Buckner-Wolfson, Geena Jung, Timothy Kim, Ryan Fatemi, Genesis Liriano, Nagma Dalvi, Mandana Behbahani, Steven Chin, Allison Martin, Andrew Kobets","doi":"10.1159/000535842","DOIUrl":"10.1159/000535842","url":null,"abstract":"<p><strong>Introduction: </strong>Infant-type hemispheric glioma (IHG) is a rare form of cancer that affects newborns and infants. It is classified as a pediatric-type high-grade glioma and typically harbors receptor tyrosine kinase (RTK) gene fusions. Here, we present the finding of a novel gene fusion IHG treated with a targeted therapy that has yet to be implemented for any other IHG case to date.</p><p><strong>Case presentation: </strong>We report the case of a 12-month-old boy with IHG who presented with obstructive hydrocephalus due to a large mass in the right frontal lobe. The patient initially underwent mass resection, but subsequent imaging showed rapid interval progression of the residual tumor. Comprehensive molecular analysis of the tumor tissue revealed a novel GAB1-ABL2 gene fusion, and the patient was started on dasatinib, an ABL kinase inhibitor. Shortly after initiation of dasatinib treatment, there was a significant reduction in tumor size and enhancement, followed by stabilization of disease.</p><p><strong>Discussion: </strong>The patient's robust response to treatment suggests that dasatinib is an effective targeted therapy for IHG harboring a GAB1-ABL2 gene fusion. This finding may inform future investigations into the disease processes of IHG and help guide the diagnosis and treatment of IHG in the absence of previously identified gene fusions, improving clinical management of this vulnerable patient population.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"27-34"},"PeriodicalIF":0.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138812586","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":"Paediatric Rhabdoid Meningioma: Clinical and Therapeutic Features Findings - Case Series of 3 Patients.","authors":"Ghassen Gader, Abdelhafidh Slimane, Firas Sliti, Mohamed Badri, Ihsèn Zammel","doi":"10.1159/000535715","DOIUrl":"10.1159/000535715","url":null,"abstract":"<p><strong>Introduction: </strong>Paediatric rhabdoid meningioma (RM) is the rarest but most aggressive subtype of meningioma, related to a severe prognosis. They account for 1-3% of all intracranial meningiomas.</p><p><strong>Case presentations: </strong>We report an institutional experience of 3 cases through which we discuss clinical, histological, and therapeutic features of this tumour. Two of our patients were female-gendered (3 years old and 1 year and 6 months old), and one was male-gendered (16 years old). Revealing symptoms were related to intracranial hypertension, cerebellar syndrome, cranial nerve palsy, and skull tumefaction. Imaging showed extra-axial tumour located in the right ponto-cerebellar angle in the first case, in the left occipital region in the second case, left parietal tumour in the third case. All patients underwent a surgical intervention with a gross total resection. Histological evaluation supported by immunohistochemistry confirmed the diagnosis of RM. Tumour recurrence was observed at 45 days in the first case with a fatal outcome. Despite adjuvant radiotherapy, both second and third cases had local recurrence after a mean follow-up of 1 month following the radiotherapy.</p><p><strong>Conclusions: </strong>RM is very aggressive tumours. Standardized therapeutic guidelines are still under debate as actual approaches are still inefficient to prevent quick recurrence and fatal outcome.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"20-26"},"PeriodicalIF":0.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138812644","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":"Coexistence of Trigonocephaly and Sylvian Arachnoid Cysts: A Coincidence?","authors":"Federico Bianchi, Ludovico Agostini, Paolo Frassanito, Luca Massimi, Gianpiero Tamburrini","doi":"10.1159/000538559","DOIUrl":"10.1159/000538559","url":null,"abstract":"<p><strong>Introduction: </strong>The association between trigonocephaly and Sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature. However, the real incidence of this association and its clinical relevance remain unknown.</p><p><strong>Methods: </strong>The authors collected and retrospectively reviewed all clinical charts and CT scans of patients surgically treated for trigonocephaly at the Pediatric Neurosurgical Department of Fondazione Policlinico Universitario \"Agostino Gemelli\" IRCCS from January 2014 to June 2023.</p><p><strong>Results: </strong>During the study period, 136 patients with trigonocephaly underwent surgery. Analysis of the clinical charts revealed that in 39.7% of the cases (54/136), preoperative CT scan depicted the presence of a Sylvian fissure AC. Of these, AC was bilateral in 23 cases and unilateral in the remaining 31. All unilateral ACs were on the left side. The ACs were classified as Galassi grade I in 52 cases (96.3%) and Galassi grade II in 2 cases (3.7%). Interestingly, in 1 case we reported a Galassi grade I AC enlargement during follow-up, thereby necessitating surgical fenestration.</p><p><strong>Conclusion: </strong>ACs and trigonocephaly are well-known conditions for pediatric neurosurgeons; however, their association is poorly defined. Despite the lack of reports on the incidence and clinical significance of this association, it is worth knowing that radiological follow-up is essential in monitoring AC evolution.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":" ","pages":"115-120"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140295365","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}