scholarly journals Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

2014 ◽  
Vol 13 (4) ◽  
pp. 393-399 ◽  
Author(s):  
Michael Safaee ◽  
Michael C. Oh ◽  
Praveen V. Mummaneni ◽  
Philip R. Weinstein ◽  
Christopher P. Ames ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Radiation Therapy ◽  
Prognostic Factor ◽  
Pediatric Patients ◽  
Extent Of Resection ◽  
Tumor Location ◽  
Grade Ii ◽  
The Mean

Object Ependymomas are a common type of CNS tumor in children, although only 13% originate from the spinal cord. Aside from location and extent of resection, the factors that affect outcome are not well understood. Methods The authors performed a search of an institutional neuropathology database to identify all patients with spinal cord ependymomas treated over the past 20 years. Data on patient age, sex, clinical presentation, symptom duration, tumor location, extent of resection, use of radiation therapy, surgical complications, presence of tumor recurrence, duration of follow-up, and residual symptoms were collected. Pediatric patients were defined as those 21 years of age or younger at diagnosis. The extent of resection was defined by the findings of the postoperative MR images. Results A total of 24 pediatric patients with spinal cord ependymomas were identified with the following pathological subtypes: 14 classic (Grade II), 8 myxopapillary (Grade I), and 2 anaplastic (Grade III) ependymomas. Both anaplastic ependymomas originated in the intracranial compartment and spread to the spinal cord at recurrence. The mean follow-up duration for patients with classic and myxopapillary ependymomas was 63 and 45 months, respectively. Seven patients with classic ependymomas underwent gross-total resection (GTR), while 4 received subtotal resection (STR), 2 received STR as well as radiation therapy, and 1 received radiation therapy alone. All but 1 patient with myxopapillary ependymomas underwent GTR. Three recurrences were identified in the Grade II group at 45, 48, and 228 months. A single recurrence was identified in the Grade I group at 71 months. The mean progression-free survival (PFS) was 58 months in the Grade II group and 45 months in the Grade I group. Conclusions Extent of resection is an important prognostic factor in all pediatric spinal cord ependymomas, particularly Grade II ependymomas. These data suggest that achieving GTR is more difficult in the upper spinal cord, making tumor location another important factor. Although classified as Grade I lesions, myxopapillary ependymomas had similar outcomes when compared with classic (Grade II) ependymomas, particularly with respect to PFS. Long-term complications or new neurological deficits were rare. Among patients with long-term follow-up, those who underwent GTR had a recurrence rate of 20% compared with 40% among those with STR or biopsy only, suggesting that extent of resection is perhaps a more important prognostic factor than histological grade in predicting PFS, which has been suggested by other data in the literature. Given the relative paucity of these lesions, collaborative multiinstitutional studies are needed, and such efforts should also focus on molecular and genetic analysis to refine the current classification system.

Outcomes in treatment for primary spinal anaplastic ependymomas: a retrospective series of 20 patients

2013 ◽  
Vol 19 (1) ◽  
pp. 3-11 ◽  
Author(s):  
XiaoDong Liu ◽  
Bing Sun ◽  
QiWu Xu ◽  
XiaoMing Che ◽  
Jie Hu ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Radiation Therapy ◽  
Cervical Spinal Cord ◽  
Surgical Removal ◽  
Thoracic Spinal ◽  
Grade Ii ◽  
Preoperative Status ◽  
The Mean ◽  
Subtotal Removal

Object Little is known regarding the anaplastic variant of primary ependymomas that involve the spinal cord. The aim of this study was to evaluate the clinical characteristics and treatment outcomes of primary spinal anaplastic ependymomas (PSAEs). Methods Medical records were reviewed in 20 patients with pathologically proven PSAEs who underwent surgical treatment at the Department of Neurosurgery in Huashan Hospital between 1999 and 2008. Results This series included 7 women and 13 men between the ages of 2 and 67 years (mean 31.9 years). The mean preoperative course was 9.3 months (range 20 days to 48 months). The most common PSAE locations were the cervical and thoracic spinal cords. The most common presenting symptom was weakness, followed by numbness, bowel or bladder dysfunction, and pain. Gross-total resection (GTR) was achieved in 17 patients, and a subtotal removal was performed in 3 patients. Nine patients received radiation therapy and/or chemotherapy. The mean follow-up duration was 83.5 months. Functional assessment of the 10 patients available at the latest follow-up evaluation showed that 2 had worsened and 8 remained unchanged from their preoperative status. There were 2 local recurrences and 1 lung metastasis. Conclusions Patients with PSAEs presented with a much shorter preoperative course than patients with Grade II ependymomas in previous studies. Patients with tumors that involved the cervical spinal cord experienced a worse outcome. Surgical removal of PSAEs, with the goal of GTR, is beneficial to patients. The role of radiation therapy and chemotherapy in PSAEs remains to be determined in further studies.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

scholarly journals Second Allogeneic Hematopoietic Stem Cell Transplantation for Post-Transplant Relapsed Acute Leukemia in Children - an EBMT PDWP Retrospective Study

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 912-912
Author(s):  
Isaac Yaniv ◽  
Aviva C. Krauss ◽  
Eric Beohou ◽  
Arnaud Dalissier ◽  
Selim Corbacioglu ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Prognostic Factor ◽  
Acute Leukemia ◽  
Pediatric Patients ◽  
Multivariate Analyses ◽  
Acute Leukemias ◽  
Hematopoietic Stem ◽  
Entire Cohort

Abstract Introduction Using the EBMT registry, we retrospectively analyzed outcomes for 373 pediatric patients who underwent second allogeneic transplant for relapsed acute leukemia at 120 centers in 32 countries, between the years 2004 and 2013, in an attempt to assess relapse, survival, GVHD and other outcomes, as well as identify factors correlating with prognosis in this cohort of patients. To our knowledge, this is the largest analysis of pediatric patients undergoing second allogeneic HSCT for relapsed acute leukemia to date. This allowed for an independent analysis of each disease, including 214 patients with ALL and 159 with AML. Patients and Methods Centers received a questionnaire completing data already available in the ProMISe database on patients between 0-18 years of age treated between 2004 and 2013. Results A total of 387 patients received a second SCT after relapse. 373 have been included in the analysis, 214 for ALL and 159 for AML. Detailed data were available for 201 patients from 48 centers; for the remainder, analysis was based on the registry. For the entire cohort overall survival (OS) at 2 and 5 years were 38% and 29%, and leukemia free survival (LFS) 30% and 25% respectively. ALL: With a median follow up from 2nd SCT of 36.4 months, OS at 1 and 5 years were 47% and 28% respectively. LFS was 39% and 28% respectively. NRM at 2 years was 22%. In multivariate analyses favorable prognostic factors for both OS and LFS were: CR prior to 2nd SCT (p=0.0001), interval > 12 months between transplants (p=0.0007), use of myeloablative conditioning (p=0.039) and the presence of cGvHD after the first SCT (p=0.0001). Good prognostic factor for low NRM was interval of more than 12 months between transplants (p=0.0002). AML: With a median follow up from 2nd SCT of 50 months, OS at 1 and 5 years were 44% and 15% respectively. LFS was 28% and 15% respectively. NRM at 2 years was 18%. In multivariate analyses, favorable prognostic factors for OS as well as LFS were: CR prior to 2nd SCT (p=0.031;0.044 respectively), interval > 6 months between transplants (p=0.0003;0.0001 respectively), and having cGvHD after the first SCT (p=0.0001). Most patients experience disease relapse or NRM within the first year after their second transplant. This observation seems to be more consistent in patients transplanted for ALL, with more changes over time in patients with AML. For ALL in particular, the 2-year incidences of relapse, NRM and LFS were not different from those at 5-years. Even in the relapse setting, survival rates for patients with ALL remain superior to patients with AML, consistent with the prognostic differences at diagnosis. Our findings, consistent for the AML and ALL subgroups, suggest that cGHVD prior to second HSCT is associated with better outcome. The identification of cGHVD prior to second transplant has not been heretofore described as a favorable prognostic factor. This strong correlation merits further study, specifically as to the underlying biology for this association. Conclusion Children with relapsed acute leukemias have a substantial chance to become long term survivors following a second SCT. CR prior to second SCT, longer interval between transplants and the presence of cGvHD after the first transplant, are favorable prognostic factors for ALL and AML. Our findings may help physicians in discussing the risk-benefit of a second transplant. These results are particularly relevant in an era where an explosion of new therapies, specifically targeted therapies and those that modulate the immune response, behoove us to carefully identify subpopulations of patients for whom specific therapies are appropriate. Novel approaches are needed to minimize relapse risk as well as short and long term morbidity in these pediatric patients while considering a second SCT for relapsed acute leukemia. Disclosures Corbacioglu: Jazz Pharmaceuticals: Consultancy, Honoraria. Bader: Novartis, Medac, Amgen, Riemser, Neovii: Consultancy, Honoraria, Research Funding.

Long-Term Follow-Up of Patients with Metastatic Epidural Spinal Cord Compression from Solid Tumors Submitted for Surgery Followed by Radiation Therapy

2018 ◽  
Vol 115 ◽  
pp. e681-e687 ◽  
Author(s):  
Federico Pessina ◽  
Pierina Navarria ◽  
Giulio Alberto Carta ◽  
Giuseppe Roberto D'Agostino ◽  
Elena Clerici ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Radiation Therapy ◽  
Solid Tumors ◽  
Spinal Cord Compression ◽  
Cord Compression ◽  
Long Term Follow Up

Long-term outcomes and prognostic factors in pediatric patients with severe traumatic brain injury and elevated intracranial pressure

2008 ◽  
Vol 2 (4) ◽  
pp. 240-249 ◽  
Author(s):  
Jay Jagannathan ◽  
David O. Okonkwo ◽  
Hian Kwang Yeoh ◽  
Aaron S. Dumont ◽  
Dwight Saulle ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Intracranial Pressure ◽  
Pediatric Patients ◽  
Elevated Intracranial Pressure ◽  
The Mean

Object The management strategies and outcomes in pediatric patients with elevated intracranial pressure (ICP) following severe traumatic brain injury (TBI) are examined in this study. Methods This study was a retrospective review of a prospectively acquired pediatric trauma database. More than 750 pediatric patients with brain injury were seen over a 10-year period. Records were retrospectively reviewed to determine interventions for correcting ICP, and surviving patients were contacted prospectively to determine functional status and quality of life. Only patients with 2 years of follow-up were included in the study. Results Ninety-six pediatric patients (age range 3–18 years) were identified with a Glasgow Coma Scale score < 8 and elevated ICP > 20 mm Hg on presentation. The mean injury severity score was 65 (range 30–100). All patients were treated using a standardized head injury protocol. The mean time course until peak ICP was 69 hours postinjury (range 2–196 hours). Intracranial pressure control was achieved in 82 patients (85%). Methods employed to achieve ICP control included maximal medical therapy (sedation, hyperosmolar therapy, and paralysis) in 34 patients (35%), ventriculostomy in 23 patients (24%), and surgery in 39 patients (41%). Fourteen patients (15%) had refractory ICP despite all interventions, and all of these patients died. Seventy-two patients (75%) were discharged from the hospital, whereas 24 (25%) died during hospitalization. Univariate and multivariate analysis revealed that the presence of vascular injury, refractory ICP, and cisternal effacement at presentation had the highest correlation with subsequent death (p < 0.05). Mean follow-up was 53 months (range 11–126 months). Three patients died during the follow-up period (2 due to infections and 1 committed suicide). The mean 2-year Glasgow Outcome Scale score was 4 (median 4, range 1–5). The mean patient competency rating at follow-up was 4.13 out of 5 (median 4.5, range 1–4.8). Univariate analysis revealed that the extent of intracranial and systemic injuries had the highest correlation with long-term quality of life (p < 0.05). Conclusions Controlling elevated ICP is an important factor in patient survival following severe pediatric TBI. The modality used for ICP control appears to be less important. Long-term follow-up is essential to determine neurocognitive sequelae associated with TBI.

Tethered cord syndrome in adults

2011 ◽  
Vol 15 (3) ◽  
pp. 258-270 ◽  
Author(s):  
Jörg Klekamp
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Neurological Deficits ◽  
Short Term ◽  
Group A ◽  
The Mean ◽  
Group B

Object The treatment of tethered cord syndromes in adults is discussed regarding the natural history and surgical indications. The author analyzes data obtained in patients who were diagnosed with a tethered cord in adulthood and either underwent surgical or conservative therapy between 1991 and 2009. Methods Since 1991, data obtained in 2515 patients with spinal cord pathologies were entered into the spinal cord database, and prospective follow-up was performed through outpatient visits and questionnaires. Of the 2515 patients, 85 adults with a tethered cord syndrome formed the basis of this study. The tethering effect was caused either by a split cord malformation, a thick filum terminale, a conus medullaris lipoma with extradural extension, or various combinations of these mechanisms. The mean age of the patients was 46 ± 13 years (range 23–74 years) and the mean follow-up duration was 61 ± 62 months. Two groups were distinguished based on the absence (Group A, 43 patients) or presence (Group B, 42 patients) of an associated lipoma or dysraphic cyst (that is, dermoid, epidermoid, or neurenteric cyst). Surgery was recommended for patients with symptoms only. Short-term results were determined within 3 months of surgery, whereas long-term outcomes (clinical recurrences) were evaluated using Kaplan-Meier statistics. Results For all patients, pain was the most common major complaint. Severe neurological deficits were rare. In Group A, 20 of 43 patients underwent surgery, whereas in Group B 23 of 42 patients underwent surgery. Among individuals who did not undergo surgery, 17 patients refused surgery and 25 patients underwent recommended conservative treatment. Short-term postoperative results indicated a significant improvement of pain and a stabilization of neurological symptoms. Long-term results showed a good prognosis in patients in whom first-time (that is, nonrevision) surgery achieved successful untethering, with a 10-year rate of neurological stabilization in 89% of Group A and a 10-year rate of neurological stabilization in 81% of Group B patients. The benefit of secondary operations in Group B was limited, with eventual clinical deterioration occurring in all patients within 10 years. For patients treated conservatively, follow-up information could be obtained in 33 of 42 patients. Twenty-eight patients remained in stable clinical condition. Only 5 of the conservatively treated patients experienced clinical deterioration over time; in 4 of these individuals with deterioration, surgery had been recommended but was refused by the patient. The clinical recurrence rate in all conservatively treated patients was 21% after 10 years. With a recommendation for surgery this figure rose to 47% within 5 years. Conclusions Surgery in adult patients with a tethered cord syndrome should be reserved for those with symptoms. In surgically treated patients, pain relief can often be achieved, and long-term neurological stabilization tends to persist more often than it does in conservatively treated patients. A conservative approach is warranted, however, in adult patients without neurological deficits. Revision surgery in patients with complex dysraphic lesions should be performed in exceptional cases only.

Spondylolysis of C-2 in children 3 years of age or younger: clinical presentation, radiographic findings, management, and outcomes with a minimum 12-month follow-up

2014 ◽  
Vol 13 (2) ◽  
pp. 196-203 ◽  
Author(s):  
Loyola V. Gressot ◽  
Sudhakar Vadivelu ◽  
Steven W. Hwang ◽  
Daniel H. Fulkerson ◽  
Thomas G. Luerssen ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Young Children ◽  
Neurological Deficits ◽  
Radiographic Findings ◽  
Children's Hospital ◽  
Very Young Children ◽  
The Mean ◽  
Neurologically Intact

Object Cervical spondylolysis is a rare condition that results from a pars interarticularis defect. The C-6 level is the most frequently involved site in the cervical spine. Its clinical presentations range from incidental radiographic findings to neck pain and, rarely, neurological deficits. Although 150 patients with subaxial cervical spondylolysis have been reported, a mere 24 adult and pediatric patients with C-2 spondylolysis have been described. The long-term outcomes of very young children with bilateral C-2 spondylolysis are of great interest, yet only a few longitudinal studies exist. Methods The authors retrospectively reviewed 5 cases of bilateral C-2 spondylolysis at Texas Children's Hospital and Riley Children's Hospital; these were combined with 5 other cases in the literature, yielding a total of 10 patients. Data regarding the patients' age, sex, C2–3 angulation and displacement, associated spine anomalies, neurological deficits, treatment, and most recent follow-up were recorded. Results The patients' ages ranged from 3 to 36 months (mean 12.9 months). There were 6 boys and 4 girls. The C2–3 angulation, displacement, and width of pars defect were measured when available. The mean C2–3 angulation was 9.5° (range 1–34°), the mean C2–3 displacement was 4.78 mm (range 1.1–10.8 mm), and the mean width of the pars defect was 4.16 mm (range 0.9–7 mm). One patient developed myelopathy and spinal cord injury. All 10 of the patients were treated initially with conservative therapy: 3 with close observation alone, 1 with a rigid cervical collar, 4 with a Minerva jacket, 1 with a sternal-occipital-mandibular immobilizer, and 1 with a halo vest. Three patients ultimately underwent surgery for internal fixation due to progressive instability or development of neurological symptoms. All patients were neurologically intact at the last follow-up (mean 44.3 months, range 14–120 months). Conclusions Based on the literature and the authors' own experience, they conclude that most very young children with C-2 spondylolysis remain neurologically intact and maintain stability in long-term follow-up despite the bony defect. This defect is often an asymptomatic incidental finding and may be managed conservatively. More aggressive therapy including surgery is indicated for those patients with a neurological deficit from spinal cord compromise secondary to stenosis and local C-2 kyphosis, progressive deformity, or worsening C2–3 instability.

Retethering risk in pediatric spinal lipoma of the conus medullaris

2021 ◽  
pp. 1-8
Author(s):  
Toshiaki Hayashi ◽  
Tomomi Kimiwada ◽  
Reizo Shirane ◽  
Teiji Tominaga
Keyword(s):  
Risk Factors ◽  
Growth Rate ◽  
Pediatric Patients ◽  
Conus Medullaris ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
The Mean ◽  
Untethering Surgery

OBJECTIVE Lipoma of the conus medullaris (LCM) causes neurological symptoms known as tethered cord syndrome (TCS). The symptoms can be seen at diagnosis and during long-term follow-up. In this report, pediatric patients with LCMs who underwent untethering surgery, under the policy of performing surgery if diagnosed regardless of symptoms, were retrospectively reviewed to evaluate long-term surgical outcomes. Possible risk factors for retethered cord syndrome (ReTCS) were evaluated in the long-term follow-up period. METHODS A total of 51 consecutive pediatric patients with LCMs who underwent a first untethering surgery and were followed for > 100 months were retrospectively analyzed. The surgery was performed with the partial removal technique. Pre- and postoperative clinical and radiological data were reviewed to analyze the outcomes of surgery and identify potential risk factors for ReTCS. RESULTS During follow-up, 12 patients experienced neurological deterioration due to ReTCS. The overall 10-year and 15-year progression-free survival rates were 82.3% and 75.1%, respectively. On univariate analysis, a lipoma type of lipomyelomeningocele (OR 11, 95% CI 2.50–48.4; p = 0.0014), patient age at the time of surgery (OR 0.41, 95% CI 0.14–1.18; p = 0.0070), and the mean patient growth rate after surgery (OR 2.00, 95% CI 1.12–3.41; p = 0.0040) were significant factors associated with ReTCS. Cox proportional hazard models showed that a lipoma type of lipomyelomeningocele (HR 5.16, 95% CI 1.54–20.1; p = 0.010) and the mean growth rate after surgery (HR 1.88, 95% CI 1.00–3.50; p = 0.040) were significantly associated with the occurrence of ReTCS. CONCLUSIONS More complex lesions and a high patient growth rate after surgery seemed to indicate increased risk of ReTCS. Larger prospective studies and registries are needed to define the risks of ReTCS more adequately.

Encephaloduroarteriosynangiosis for Pediatric Moyamoya Disease: A Single-Center Experience With 67 Cases in China

2018 ◽  
Vol 33 (14) ◽  
pp. 901-908 ◽  
Author(s):  
Chengjun Wang ◽  
Meng Zhao ◽  
Jia Wang ◽  
Shuo Wang ◽  
Dong Zhang ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Pediatric Patients ◽  
Positive Impact ◽  
Case Series ◽  
Stroke Recurrence ◽  
Long Term Outcomes ◽  
Single Center Experience ◽  
The Mean

The purpose of this study is to investigate the surgical results and long-term outcomes of encephaloduroarteriosynangiosis for moyamoya disease in pediatric patients. We performed a retrospective analysis of 67 pediatric patients with moyamoya disease who underwent encephaloduroarteriosynangiosis in Beijing Tiantan Hospital. The case series included 36 boys and 31 girls. All the patients underwent surgical revascularization, and a total of 93 encephaloduroarteriosynangiosis procedures were performed (41 unilateral, 26 bilateral). The mean follow-up period after surgery was 30 months. During follow-up, ischemic stroke events were detected in 5 patients and the stroke rate for pediatric patients who underwent encephaloduroarteriosynangiosis procedure was 7.1% per patient-years. At the last follow-up, favorable outcomes (modified Rankin Scale score ≤ 2) were observed in 65 cases (97.0%). Our study suggests that long-term surgical outcome of encephaloduroarteriosynangiosis in pediatric moyamoya disease patients is satisfactory, and this technique has a positive impact on the prevention of stroke recurrence.

Long-term outcome of 114 children with cerebral aneurysms

2012 ◽  
Vol 9 (6) ◽  
pp. 636-645 ◽  
Author(s):  
Päivi Koroknay-Pál ◽  
Hanna Lehto ◽  
Mika Niemelä ◽  
Riku Kivisaari ◽  
Juha Hernesniemi
Keyword(s):  
Carotid Artery ◽  
Pediatric Patients ◽  
Internal Carotid ◽  
De Novo ◽  
Anterior Circulation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
The Mean

Object Population-based data on pediatric patients with aneurysms are limited. The aim of this study is to clarify the characteristics and long-term outcomes of pediatric patients with aneurysms. Methods All pediatric patients (≤ 18 years old) with aneurysms among the 8996 aneurysm patients treated at the Department of Neurosurgery in Helsinki from 1937 to 2009 were followed from admission to the end of 2010. Results There were 114 pediatric patients with 130 total aneurysms during the study period. The mean patient age was 14.5 years (range 3 months to 18 years). The male:female ratio was 3:2. Eighty-nine patients (78%) presented with subarachnoid hemorrhage. The majority of the aneurysms (116 [89%]) were in the anterior circulation, and the most common location was the internal carotid artery bifurcation (36 [28%]). The average aneurysm diameter was 11 mm (range 2–55 mm) with 16 giant aneurysms (12%). Eighty aneurysms (62%) were treated microsurgically, and 37 (28%) were treated conservatively due to poor medical and neurological status of the patient or due to technical reasons during the early years of the patient series. No connective tissue disorders common to pediatric aneurysm patients were diagnosed in this series, with the exception of 1 patient with tuberous sclerosis complex. The mean follow-up duration was 24.8 years (range 0–55.8 years). At the end of follow-up, 71 patients (62%) had a good outcome, 3 (3%) were dependent, and 40 (35%) had died. Twenty-seven deaths (68%) were assessed to be aneurysm-related. Factors correlating with a favorable long-term outcome were good neurological condition of the patient on admission, aneurysm location in the anterior circulation, complete aneurysm closure, and absence of vasospasm. Six patients developed symptomatic de novo aneurysms after a median of 25 years (range 11–37 years). Fourteen patients (12%) had a family history of aneurysms. There was no increased incidence for cardiovascular diseases in long-term follow-up. Conclusions Most aneurysms were ruptured and of medium size. Internal carotid artery bifurcation was the most frequent location of the aneurysms. There was a male predominance of pediatric patients with aneurysms. Most patients experienced good recovery, with 91% of the long-term survivors living at home independently without assistance and meaningfully employed. Altogether, almost a third of these patients finished high school and one-fifth had a college or university degree. Pediatric patients had a tendency to develop de novo aneurysms.

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