scholarly journals Increase in clivo-axial angle is associated with clinical improvement in children undergoing occipitocervical fusion for complex Chiari malformation: patient series

2021 ◽  
Vol 2 (23) ◽  
Author(s):  
Neelan J. Marianayagam ◽  
John K. Chae ◽  
Ibrahim Hussain ◽  
Amanda Cruz ◽  
Ali A. Baaj ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
Age Groups ◽  
Posterior Fossa Decompression ◽  
Occipitocervical Fusion ◽  
Endoscopic Endonasal ◽  
Presenting Symptoms ◽  
Axial Angle ◽  
Canal Diameter

BACKGROUND The authors analyzed the pre- and postoperative morphometric properties of pediatric patients with complex Chiari malformation undergoing occipitocervical fusion (OCF) to assess clinical outcomes and morphometric properties that might influence postoperative outcomes. OBSERVATIONS The authors retrospectively reviewed 35 patients younger than 22 years with Chiari malformation who underwent posterior fossa decompression and OCF with or without endoscopic endonasal odontoidectomy at their institution (13 with and 22 without odontoidectomy). Clivo-axial angle (CXA), pB-C2, atlantodental interval, basion-dens interval, basion-axial interval, and canal diameter at the level of C1 were measured on preoperative and approximately 3-month postoperative computed tomography or magnetic resonance imaging. The authors further stratified the patient cohort into three age groups and compared the three cohorts. The most common presenting symptoms were headache, neck/shoulder pain, and dysphagia; 80% of the cohort had improved clinical outcomes. CXA increased significantly after surgery. When stratified into those who showed postoperative improvement and those who did not, only the former showed a significant increase in CXA. After age stratification, the significant changes in CXA were observed in the 7- to 13-year-old and 14- to 21-year-old cohorts. LESSONS CXA may be the most important morphometric predictor of clinical outcomes after OCF in pediatric patients with complex Chiari malformation.

scholarly journals Predicting clinical outcomes using morphometric changes in adults with complex Chiari malformation undergoing occipitocervical fusion with or without ventral decompression: patient series

2021 ◽  
Vol 2 (22) ◽  
Author(s):  
John K. Chae ◽  
Neelan J. Marianayagam ◽  
Ibrahim Hussain ◽  
Amanda Cruz ◽  
Ali A. Baaj ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Occipitocervical Fusion ◽  
Endoscopic Endonasal ◽  
Perpendicular Line ◽  
Canal Diameter ◽  
Bulbar Symptoms ◽  
Patient Series ◽  
Atlantodental Interval

BACKGROUND The authors assessed the connection between clinical outcomes and morphometrics in patients with complex Chiari malformation (CM) who have undergone posterior fossa decompression (PFD) and subsequent occipitocervical fusion (OCF) with or without ventral decompression (VD). OBSERVATIONS The authors retrospectively reviewed 33 patients with CM aged over 21 years who underwent PFD and OCF with or without endoscopic endonasal odontoidectomy at the authors’ institution (21 OCF only and 12 OCF + VD). Clivoaxial angle (CXA), pB-C2 (perpendicular line to the line between the basion and C2), atlantodental interval (ADI), basion-dens interval (BDI), basion-axial interval (BAI), and C1 canal diameter were measured on preoperative and approximately 3-month postoperative computed tomography or magnetic resonance imaging scans. Common symptoms included headache, paresthesia, and bulbar symptoms. Clinical improvement after surgery was observed in 78.8% of patients. CXA, ADI, and BDI all significantly increased after surgery, whereas pB-C2 and BAI significantly decreased. OCF + VD had a significantly more acute CXA and longer pB-C2 preoperatively than OCF only. Patients who clinically improved postoperatively showed the same significant morphometric changes, but those who did not improve showed no significant morphometric changes. LESSONS Patients showing improvement had greater corrections in skull base morphometrics than those who did not. Although there are various mutually nonexclusive reasons why certain patients do not improve after surgery, smaller degrees of morphometric correction could play a role.

scholarly journals Syringobulbia in pediatric patients with Chiari malformation type I

2018 ◽  
Vol 22 (1) ◽  
pp. 52-60 ◽  
Author(s):  
Arnold H. Menezes ◽  
Jeremy D. W. Greenlee ◽  
Brian J. Dlouhy
Keyword(s):  
Posterior Fossa ◽  
Cranial Nerve ◽  
Chiari Malformation ◽  
Fourth Ventricle ◽  
Pediatric Patients ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Presenting Symptoms ◽  
Cranial Nerve Palsies

OBJECTIVESyringobulbia (SB) is a rare entity, with few cases associated with Chiari malformation type I (CM-I) in the pediatric population. The authors reviewed all pediatric cases of CM-I–associated SB managed at their institution in order to better understand the presentation, treatment, and surgical outcomes of this condition.METHODSA prospectively maintained institutional database of craniovertebral junction abnormalities was analyzed to identify all cases of CM-I and SB from the MRI era (i.e., after 1984). The authors recorded presenting symptoms, physical examination findings, radiological findings, surgical treatment strategy, intraoperative findings, and outcomes. SB cases associated with tumors, infections, or type II Chiari malformations were excluded.RESULTSThe authors identified 326 pediatric patients with CM-I who were surgically treated. SB was identified in 13 (4%) of these 326 patients. Headache and neck pain were noted in all 13 cases. Cranial nerve abnormalities were common: vagus and glossopharyngeal nerve dysfunction was the most frequent observation. Other cranial nerves affected included the trigeminal, abducens, and hypoglossal nerves. Several patients exhibited multiple cranial nerve palsies at presentation. Central sleep apnea was present in 6 patients.Syringomyelia (SM) was present in all 13 patients. SB involved the medulla in all cases, and extended rostrally into the pons and midbrain in 2 patients; in 1 of these 2 cases the cavity extended further rostrally to the cerebrum (syringocephaly). SB communicated with the fourth ventricle in 7 of the 13 cases.All 13 patients were treated with posterior fossa decompression with intradural exploration to ensure CSF egress out of the fourth ventricle and through the foramen magnum. The foramen of Magendie was found to be occluded by an arachnoid veil in 9 cases. Follow-up evaluation revealed that SB improved before SM. Cranial nerve palsies regressed in 11 of the 13 patients, and SB improved in all 13.CONCLUSIONSThe incidence of SB in our surgical series of pediatric patients with CM-I was 4%, and all of these patients had accompanying SM. The SB cavity involved the medulla in all cases and was found to communicate with the fourth ventricle in 54% of cases. Posterior fossa decompression with intradural exploration and duraplasty is an effective treatment for these patients.

scholarly journals Expansile duraplasty and obex exploration compared with bone-only decompression for Chiari malformation type I in children: retrospective review of outcomes and complications

2021 ◽  
Vol 27 (1) ◽  
pp. 1-8
Author(s):  
Chibawanye I. Ene ◽  
Anthony C. Wang ◽  
Kelly L. Collins ◽  
Robert H. Bonow ◽  
Lynn B. McGrath ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Posterior Fossa ◽  
Retrospective Review ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Symptom Resolution ◽  
Presenting Symptoms ◽  
Significant Difference

OBJECTIVEWhile a select population of pediatric patients with Chiari malformation type I (CM-I) remain asymptomatic, some patients present with tussive headaches, neurological deficits, progressive scoliosis, and other debilitating symptoms that necessitate surgical intervention. Surgery entails a variety of strategies to restore normal CSF flow, including increasing the posterior fossa volume via bone decompression only, or bone decompression with duraplasty, with or without obex exploration. The indications for duraplasty and obex exploration following bone decompression remain controversial. The objective of this study was to describe an institutional series of pediatric patients undergoing surgery for CM-I, performed by a single neurosurgeon. For patients presenting with a syrinx, the authors compared outcomes following bone-only decompression with duraplasty only and with duraplasty including obex exploration. Clinical outcomes evaluated included resolution of syrinx, scoliosis, presenting symptoms, and surgical complications.METHODSA retrospective review was conducted of the medical records of 276 consecutive pediatric patients with CM-I operated on at a single institution between 2001 and 2015 by the senior author. Imaging findings of tonsillar descent, associated syrinx (syringomyelia or syringobulbia), basilar invagination, and clinical assessment of CM-I–attributable symptoms and scoliosis were recorded. In patients presenting with a syrinx, clinical outcomes, including syrinx resolution, symptom resolution, and impact on scoliosis progression, were compared for three surgical groups: bone-only/posterior fossa decompression (PFD), PFD with duraplasty (PFDwD), and PFD with duraplasty and obex exploration (PFDwDO).RESULTSPFD was performed in 25% of patients (69/276), PFDwD in 18% of patients (50/276), and PFDwDO in 57% of patients (157/276). The mean follow-up was 35 ± 35 months. Nearly half of the patients (132/276, 48%) had a syrinx. In patients presenting with a syrinx, PFDwDO was associated with a significantly higher likelihood of syrinx resolution relative to PFD only (HR 2.65, p = 0.028) and a significant difference in time to symptom resolution (HR 2.68, p = 0.033). Scoliosis outcomes did not differ among treatment groups (p = 0.275). Complications were not significantly higher when any duraplasty (PFDwD or PFDwDO) was performed following bone decompression (p > 0.99).CONCLUSIONSIn this series of pediatric patients with CM-I, patients presenting with a syrinx who underwent expansile duraplasty with obex exploration had a significantly greater likelihood of syrinx and symptom resolution, without increased risk of CSF-related complications, compared to those who underwent bone-only decompression.

Chiari malformation I and autism spectrum disorder: an underrecognized coexistence

2015 ◽  
Vol 15 (1) ◽  
pp. 96-100 ◽  
Author(s):  
Mayur Jayarao ◽  
Kristin Sohl ◽  
Tomoko Tanaka
Keyword(s):  
Autism Spectrum Disorder ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
Autism Spectrum ◽  
Spectrum Disorder ◽  
Symptom Improvement ◽  
Type I ◽  
Presenting Symptoms ◽  
Children With Asd ◽  
Cranial Mri

OBJECT Patients with symptomatic Chiari malformation Type I (CM-I) frequently present with headaches, neck pain, difficulty swallowing, and balance disturbances. In children with autism spectrum disorder (ASD), diagnosing CM-I can be a challenging task. Moreover, even if symptomatic, some patients do not undergo further evaluation or management, as their presentations are attributed to autism and its myriad symptoms. Therefore, cranial MRI findings were reviewed after evaluating and treating patients with coexisting ASD and CM-I. In this paper, the authors report on 5 children with ASD and symptomatic CM-I, including their clinical presentation, imaging studies, management, and outcomes, and discuss the likely underrecognized coexistence of these conditions. METHODS All pediatric patients with ASD and cranial MRI conducted for any reason in the period from 1999 to 2013 were considered for analysis. All cases with concomitant symptomatic CM-I were eligible for this retrospective analysis. RESULTS One hundred twenty-five pediatric patients diagnosed with ASD had undergone MRI, and 9 of them had evidence of cerebellar tonsillar herniation. Five patients were symptomatic and underwent suboccipital craniectomy, a C-1 or a C-1 and C-2 laminectomy, and duraplasty with bovine pericardium or Type I collagen allograft. There were no intraoperative complications. All patients showed symptom improvement and/or resolution of presenting symptoms, which included headache, dysphasia, speech, and irritability. CONCLUSIONS There is no identified cause of autism. Children with ASD can be difficult to assess specifically in a neurological examination. Thus, cranial MRI considered when completing a comprehensive diagnostic evaluation. While cranial MRI is not a routine part of ASD evaluation, this study demonstrates that CM-I and ASD may coexist and be underrecognized. The study reinforces the importance of a comprehensive medical evaluation designed to elucidate neurological findings in children with impaired communication abilities and suggests the judicious use of neuroimaging.

Intradiploic occipital pseudomeningocele in a patient with remote history of surgical treatment of Chiari malformation

2014 ◽  
Vol 21 (5) ◽  
pp. 769-772 ◽  
Author(s):  
Kelly B. Mahaney ◽  
Arnold H. Menezes
Keyword(s):  
Older Adults ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Rare Entity ◽  
Good Recovery ◽  
Occipitocervical Fusion ◽  
Delayed Complication ◽  
History Of ◽  
Dorsal Hindbrain

An intradiploic CSF pseudocyst is a rare entity that has been described in association with trauma, as a sequela of untreated hydrocephalus, or occasionally as a congenital finding in older adults. The authors present the case of a woman with a remote history of a posterior fossa intradural procedure, in which she underwent Chiari malformation decompression, Silastic substitute–assisted duraplasty, and occipitocervical fusion; she presented 19 years later with recurrent symptoms of Chiari malformation. She was found to have an occipital intradiploic pseudomeningocele, arising within her dorsal occipitocervical fusion mass and resulting in dorsal hindbrain compression. She underwent a posterior fossa decompression and revision of her failed duraplasty, and she had a good recovery. This case demonstrates intradiploic CSF pseudomeningocele as a rare potential delayed complication of an intradural procedure for the treatment of Chiari malformation with occipitocervical fusion.

scholarly journals Divergence insufficiency alleviated by posterior fossa decompression with duraplasty in a patient with Chiari type 1.5 malformation

2018 ◽  
Vol 22 (5) ◽  
pp. 504-507 ◽  
Author(s):  
Allison Strickland ◽  
Cordell M. Baker ◽  
R. Michael Siatkowski ◽  
Timothy B. Mapstone
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Chiari Type ◽  
Presenting Symptoms ◽  
Dural Opening ◽  
Adequate Decompression ◽  
Divergence Insufficiency ◽  
Repeat Imaging

The authors present a case of Chiari type 1.5 malformation with the uncommon presenting symptoms of esotropia and diplopia due to divergence insufficiency in a 12-year-old girl. Imaging at initial diagnosis revealed cerebellar herniation with extension of the tonsils to the C2 vertebral body, a retroflexed odontoid, and a small cervical syrinx. The patient was initially treated with an uncomplicated Chiari malformation decompression without dural opening. Repeat imaging revealed an adequate decompression. Three months postoperatively the patient’s diplopia recurred and she underwent repeat posterior fossa decompression with dural opening and duraplasty. Following repeat decompression with dural opening and duraplasty, the patient’s diplopia had not recurred by the 2-year follow-up.https://thejns.org/doi/abs/10.3171/2018.5.PEDS1886

scholarly journals Multidimensional, patient-reported outcome after posterior fossa decompression in 79 patients with Chiari malformation type I

2019 ◽  
Vol 10 ◽  
pp. 242 ◽  
Author(s):  
Jan De Vlieger ◽  
Joost Dejaegher ◽  
Frank Van Calenbergh
Keyword(s):  
Patient Satisfaction ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
Patient Reported Outcome ◽  
Symptom Improvement ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Important Improvement ◽  
Patient Reported

Background: We studied patient-reported outcome among patients who underwent posterior fossa decompression (PFD) for Chiari malformation type I (CM-I). Methods: We interviewed patients who underwent PFD for CM-I from 1995 to 2016. Results: A total of 79 patients were interviewed. The median age at surgery was 30 years (range 5–72 years) with 27 pediatric patients. Forty-six patients had syringomyelia (36 adults and 10 pediatric patients). Fifty-four patients (68%) reported at least some improvement, 46 (58%) important improvement, 13 (16%) worsening, and 12 stabilization (15%). Any improvement as well as important improvement were significantly more often reported in the nonsyringomyelia group (85% vs. 57%, P = 0.01 and 76% vs. 46%, P = 0.01, respectively). Of the 47 patients reporting preoperative neck pain, 31 (66%) reported at least some improvement after surgery and 9 (19%) worsening after surgery. Of the 59 patients experiencing headaches before surgery, 45 (76%) reported at least some improvement after surgery and 4 (7%) worsening. Quality of life was mostly affected by pain and discomfort in all groups. Sixty-two patients (78%) were satisfied or very satisfied with the results of surgery and 8 (11%) were unsatisfied or very unsatisfied. Up to 71 patients (90%) would consent to surgery again. Conclusion: In CM-I patients, PFD offers symptom improvement in about two-thirds of patients with high patient satisfaction. Symptom improvement is significantly higher in patients without associated syringomyelia, but patient satisfaction is similar. Symptom worsening is more frequent in the adult than in the pediatric population, with similar rates of postoperative improvement and patient satisfaction.

scholarly journals Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature

2018 ◽  
Vol 22 (4) ◽  
pp. 426-438 ◽  
Author(s):  
Andrew C. Vivas ◽  
Nir Shimony ◽  
Eric M. Jackson ◽  
Risheng Xu ◽  
George I. Jallo ◽  
...  
Keyword(s):  
Literature Review ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Pediatric Patients ◽  
High Dose ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Dural Graft ◽  
Csf Diversion

OBJECTIVEHydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.METHODSA retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.RESULTSOver the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.CONCLUSIONSDe novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

scholarly journals Treatment Outcomes of Pediatric Patients With Ewing Sarcoma in a War-Torn Nation: A Single-Institute Experience From Iraq

2019 ◽  
pp. 1-9
Author(s):  
Sazgar S. Majeed ◽  
Hawzheen A. Muhammad ◽  
Jalil S. Ali ◽  
Hassanain H. Khudhair ◽  
Ayah Said ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Clinical Outcomes ◽  
Ewing Sarcoma ◽  
Pediatric Patients ◽  
Lumbar Vertebrae ◽  
Related Factors ◽  
Palpable Mass ◽  
Presenting Symptoms ◽  
Developed Nations ◽  
Time Of Presentation

PURPOSE Ewing sarcoma (ES) is a relatively rare, highly malignant tumor of the musculoskeletal system. It is the second most common malignant bone tumor in children and adolescents in the age group of 5 to 20 years. The aim of this study was to identify the treatment outcomes of pediatric patients with ES in Sulaimani governorate, Iraq. PATIENTS AND METHODS This was a retrospective study that reviewed the medical records of pediatric patients with ES who were managed between 2009 and 2015, with follow-up until late 2017. Patient- and tumor-related factors were correlated with clinical outcomes. RESULTS A total of 31 pediatric patients with ES were included in this study. All the patients received chemotherapy and radiotherapy, whereas only 14 patients underwent surgical resection and just eight had free surgical margins. The median age at diagnosis was 13 years, 58% were male, and 42% were female. The presenting symptoms at diagnosis were mostly pain (67.7%) and palpable mass (25.8%). The primary tumor was located in the extremities (51.6%), the thoracic cage (19.4%), the pelvis (16.1%), and the lumbar vertebrae (12.9%). Approximately two thirds of the patients (61.3%) had localized disease at the time of presentation. The 5-year overall survival was 19%, and the 5-year recurrence-free survival was 34%. CONCLUSION Clinical outcomes of ES in pediatric patients in our war-torn nation, Iraq, are still markedly inferior to the published outcomes from stable, developed nations. Additional large and multicenter national studies are required. Diagnostic and therapeutic measures need improvement, and multidisciplinary and comprehensive cancer-integrated approaches are vital for better outcomes.

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