Clinical course of untreated pediatric brainstem cavernous malformations: hemorrhage risk and functional recovery

2014 ◽  
Vol 13 (5) ◽  
pp. 471-483 ◽  
Author(s):  
Da Li ◽  
Shu-Yu Hao ◽  
Jie Tang ◽  
Xin-Ru Xiao ◽  
Gui-Jun Jia ◽  
...  

Object The aim of this study was to investigate the clinical appearance of untreated pediatric brainstem cavernous malformations (CMs) and to identify the hemorrhage risks and functional outcomes. Methods All pediatric patients with a diagnosis of brainstem CM between 1985 and 2012 were registered. The clinical chart and radiographs were recorded, and follow-up evaluations were obtained prospectively. Results A total of 85 patients (69.4% male) were included with a mean age of 12.7 years. Sixty-seven patients (78.8%) had prior hemorrhage, and 6 patients (7.1%) were asymptomatic. There were 15 midbrain lesions, 53 pons lesions, and 17 medulla lesions. The mean lesion size was 1.9 cm. During a total of 401.6 patient-years of follow-up, 47 hemorrhages occurred in 37 patients, and the annual hemorrhage rate was 11.7% per patient-year. The mean hemorrhage interval was 47.8 months. The hemorrhage risk declined over time, especially after the first 2 years. Both a lesion size ≥ 2 cm (hazard ratio [HR] 2.122, p = 0.037) and the presence of perilesional edema (HR 2.192, p = 0.039) predicted future hemorrhage and were associated with a high annual hemorrhage rate. The hemorrhage-free survival at 6 months was 85.7%, and at 1, 5, 10, and 15 years was 71.5%, 49.4%, 27.5%, and 13.7%, respectively. At the most recent functional evaluation, 33 patients (38.8%) had improved, 32 (37.6%) had stabilized, and 20 (23.5%) had worsened, without any deaths. Twenty-two patients (25.9%) obtained a full recovery. Prospective hemorrhage (HR 0.191, p = 0.003) was the adverse predictor for full recovery. Full recovery primarily occurred within the first 12 months, after which the chance of full recovery decreased. The cumulative percentage of complete recovery at 6 months was 32.7%, and at 1, 3, and 5 years was 40.8%, 43.6%, and 49.2%, respectively. Conclusions In this study the hemorrhage rate was relatively high in pediatric brainstem CMs, although the functional outcome was acceptable. The decline in hemorrhage risk and the identified adverse predictors in this study were helpful for clinicians and patients when deciding on treatment. Referral bias and the insufficient follow-up period of the study were highlighted as limitations.

2013 ◽  
Vol 71 (4) ◽  
pp. 220-228 ◽  
Author(s):  
Marcelo Campos Moraes Amato ◽  
João Flávio Gurjão Madureira ◽  
Ricardo Santos de Oliveira

Objectives: To determine the clinical presentation and treatment outcome of pediatric intracranial cavernous malformation (CM) in a single-centered institution. Methods: Clinical data review of 30 patients under 18 years-old who had undergone surgery for cavernous malformation from January 1993 to December 2011. Results: The Study Group included 18 males and 12 females (mean age: 8.7 years-old). Symptoms at presentation were seizures (16/30, 53.3%), headache (15/30, 50.0%), and focal neurological deficits (11/30, 36.6%). Multiple cavernous malformations were found in 5/30 (16.6%). According to location, patients were classified in groups: (G1) brain-steam in 5/30 (16.6%), (G2) cerebellum in 2/30 (6.6%), (G3) supratentorial associated with seizures in 16/30 (53.3%), and (G4) supratentorial without seizures in 7/30 (23.3%). Surgical resection was performed in 26 out of 30 (86.6%) patients. The mean follow-up period was 4.1 years. Of 15 children followed-up with preoperative seizures, all were rendered seizure-free after surgery. Conclusions: For symptomatic solitary cavernous malformation, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation. For multiple cavernous malformation or asymptomatic patients, the treatment modalities must be cautiously considered.


2014 ◽  
Vol 13 (5) ◽  
pp. 484-502 ◽  
Author(s):  
Da Li ◽  
Shu-Yu Hao ◽  
Jie Tang ◽  
Xin-Ru Xiao ◽  
Gui-Jun Jia ◽  
...  

Object The goal of this study was to evaluate surgical outcomes of pediatric brainstem cavernous malformations (CMs) and identify the risk factors associated with postoperative full recovery and rebleeding. Methods The clinical charts and radiographs from a series of 52 pediatric patients (37 male and 15 female; mean age 12.2 years; range 1–17 years) who underwent surgery for brainstem CMs between 1996 and 2011 were reviewed. Follow-up evaluation measures were obtained retrospectively. Neurological function was evaluated using the modified Rankin Scale (mRS) score. Results The lesion locations among the 52 patients included the midbrain (n = 7, 13.5%), pons (n = 38, 73.1%), and medulla (n = 7, 13.5%). The mean duration of symptoms was 18.5 months, and the preoperative annual hemorrhage and rebleeding rates were 12.3% and 32.5% per patient-year, respectively. The mean lesion size was 2.1 cm. Gross-total resection without surgery-related death was achieved in 49 patients (94.2%). Immediate postoperative reduced neurological function was observed in 17 patients (32.7%). Surgical morbidities developed in 25 patients (48.1%) and remained in 11 patients (21.2%) after 7.9 years of follow-up. The mean mRS scores at admission, discharge after surgery, 3 and 6 months postsurgery, and recent evaluation were 2.0, 2.3, 2.0, 1.5, and 1.0, respectively. The postoperative mRS scores at 6 months (p < 0.001) and on recent evaluation (p < 0.001) were significantly lower than those at admission. Postoperative rebleeding occurred in 2 patients, and the postoperative annual rebleeding rate was 0.5% per patient-year. By the most recent evaluation, 10 patients (19.2%) had achieved full recovery and all patients were either improved (n = 32, 61.5%) or unchanged (n = 20, 38.5%). The adverse predictors for full recovery included age ≥ 12 years (HR 0.230, p = 0.021), ≥ 2 preoperative hemorrhages (HR 0.124, p = 0.048), and poor preoperative status (HR 0.197, p = 0.040). An HR < 1 predicted poor complete recoveries. The single risk factor predicting postoperative rebleeding was incomplete resection (χ2 = 4.340, p = 0.037). Conclusions Fair outcomes for pediatric brainstem CMs could be obtained through surgery, but only a few patients achieved full recovery. Thus, to minimize surgical morbidity, surgical planning must be tailored to individual patients in all cases in which an operation is warranted. Complete resection must be attempted to reduce the risk of postoperative rebleeding. The predictors associated with complete postoperative recovery were referential for determining treatment.


2017 ◽  
Vol 127 (3) ◽  
pp. 480-491 ◽  
Author(s):  
Kai-Bing Tian ◽  
Jing-Jie Zheng ◽  
Jun-Peng Ma ◽  
Shu-Yu Hao ◽  
Liang Wang ◽  
...  

OBJECTIVEThe natural history of cerebral cavernous malformations (CMs) has been widely studied, but the clinical course of untreated thalamic CMs is largely unknown. Hemorrhage of these lesions can be devastating. The authors undertook this study to obtain a prospective hemorrhage rate and provide a better understanding of the prognosis of untreated thalamic CMs.METHODSThis longitudinal cohort study included patients with thalamic CMs who were diagnosed between 2000 and 2015. Clinical data were recorded, radiological studies were extensively reviewed, and follow-up evaluations were performed.RESULTSA total of 121 patients were included in the study (56.2% female), with a mean follow-up duration of 3.6 years. The overall annual hemorrhage rate (subsequent to the initial presentation) was calculated to be 9.7% based on the occurrence of 42 hemorrhages over 433.1 patient-years. This rate was highest in patients (n = 87) who initially presented with hemorrhage and focal neurological deficits (FNDs) (14.1%) (χ2 = 15.358, p < 0.001), followed by patients (n = 19) with hemorrhage but without FND (4.5%) and patients (n = 15) without hemorrhage regardless of symptoms (1.2%). The initial patient presentations of hemorrhage with FND (hazard ratio [HR] 2.767, 95% CI 1.336–5.731, p = 0.006) and associated developmental venous anomaly (DVA) (HR 2.510, 95% CI 1.275–4.942, p = 0.008) were identified as independent hemorrhage risk factors. The annual hemorrhage rate was significantly higher in patients with hemorrhagic pres entation at diagnosis (11.7%, p = 0.004) or DVA (15.7%, p = 0.002). Compared with the modified Rankin Scale (mRS) score at diagnosis (mean 2.2), the final mRS score (mean 2.0) was improved in 37 patients (30.6%), stable in 59 patients (48.8%), and worse in 25 patients (20.7%). Lesion size (odds ratio [OR] per 0.1 cm increase 3.410, 95% CI 1.272–9.146, p = 0.015) and mRS score at diagnosis (OR per 1 point increase 3.548, 95% CI 1.815–6.937, p < 0.001) were independent adverse risk factors for poor neurological outcome (mRS score ≥ 2). Patients experiencing hemorrhage after the initial ictus (OR per 1 ictus increase 6.923, 95% CI 3.023–15.855, p < 0.001) had a greater chance of worsened neurological status.CONCLUSIONSThis study verified the adverse predictors for hemorrhage and functional outcomes of thalamic CMs and demonstrated an overall annual symptomatic hemorrhage rate of 9.7% after the initial presentation. These findings and the mode of initial presentation are useful for clinicians and patients when selecting an appropriate treatment, although the tertiary referral bias of the series should be taken into account.


2014 ◽  
Vol 121 (1) ◽  
pp. 32-41 ◽  
Author(s):  
Da Li ◽  
Shu-Yu Hao ◽  
Gui-Jun Jia ◽  
Zhen Wu ◽  
Li-Wei Zhang ◽  
...  

Object Cerebral cavernous malformations have been studied widely, but the natural history of brainstem cavernous malformations (CMs) is not well defined, and hemorrhages caused by brainstem CMs are devastating. The goal of this study was to quantify the hemorrhage risks and functional outcomes of patients with brainstem CMs. Methods This prospective, longitudinal, cohort study included patients with brainstem CMs diagnosed between 1985 and 2012. The clinical courses of all patients were recorded. Predictors of hemorrhage and the overall untreated outcomes were evaluated. Results A total of 331 patients (46.5% female) were included, with a mean follow-up duration of 6.5 years. The annual hemorrhage rates in patients initially presenting with hemorrhage with (n = 215) or without (n = 34) focal neurological deficits were 15.9% and 12.4%, respectively. However, the annual hemorrhage rate was 8.7% in patients initially presenting without hemorrhage (n = 82). The risk factors for hemorrhage were female sex (hazard ratio [HR] 1.445, p = 0.041), prior hemorrhage (HR 1.277, p = 0.029), and perilesional edema (HR 1.830, p = 0.002). Overall, neurological function at the most recent assessment was improved compared with neurological function at diagnosis. Additionally, 307 patients (92.7%) improved or stabilized, 268 (81.0%) lived independently, and 95 (28.7%) completely recovered. Predictors favoring complete recovery were no prospective hemorrhage (HR 1.958, p = 0.001), younger age (HR 1.268, p = 0.001), and small lesion size (HR 1.578, p = 0.004). Conclusions Patients' initial presentation predicts their prospective annual hemorrhage rate. This study suggests that several strong risk factors for hemorrhage and predictors of brainstem CM outcomes may enable clinicians to evaluate the potential hemorrhage risks of their patients and design personalized treatments.


2014 ◽  
Vol 17 (3) ◽  
pp. 146
Author(s):  
Osman Tansel Darcin ◽  
Mehmet Kalender ◽  
Ayse Gul Kunt ◽  
Okay Guven Karaca ◽  
Ata Niyazi Ecevit ◽  
...  

<p><b>Background:</b> Thoracoabdominal aortic aneurysms (TAAA) present a significant clinical challenge, as they are complex and require invasive surgery. In an attempt to prevent considerably high mortality and morbidity in open repair, hybrid endovascular repair has been developed by many authors. In this study, we evaluated the early-term results obtained from this procedure.</p><p><b>Methods:</b> From November 2010 to February 2013, we performed thoracoabdominal hybrid aortic repair in 18 patients. The mean age was 68 years (12 men, 6 women). All of the patients had significant comorbidities. Follow-up computed tomography (CT) scans were performed at 1 week, 3 months, 6 months, and annually thereafter.</p><p><b>Results:</b> All patients were operated on in a staged procedure and stent graft deployment was achieved. Procedural success was achieved in all cases. All patients were discharged with complete recovery. No endoleaks weres detected in further CT examination.</p><p><b>Conclusion:</b> Our results suggests that hybrid debranching and endovascular repair of extensive thoracoabdominal aneurysms represents a suitable therapeutic option to reduce the morbidity and mortality of TAAA repair, particularly in those typically considered at high risk for standard repair.</p>


Author(s):  
Gildas Patet ◽  
Andrea Bartoli ◽  
Torstein R. Meling

AbstractRadiation-induced cavernous malformations (RICMs) are delayed complications of brain irradiation during childhood. Its natural history is largely unknown and its incidence may be underestimated as RCIMS tend to develop several years following radiation. No clear consensus exists regarding the long-term follow-up or treatment. A systematic review of Embase, Cochrane Library, PubMed, Google Scholar, and Web of Science databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was performed. Based on our inclusion/exclusion criteria, 12 articles were included, totaling 113 children with RICMs, 86 were treated conservatively, and 27 with microsurgery. We were unable to precisely define the incidence and natural history from this data. The mean age at radiation treatment was 7.3 years, with a slight male predominance (54%) and an average dose of 50.0 Gy. The mean time to detection of RICM was 9.2 years after radiation. RICM often developed at distance from the primary lesion, more specifically frontal (35%) and temporal lobe (34%). On average, 2.6 RICMs were discovered per child. Sixty-seven percent were asymptomatic. Twenty-one percent presented signs of hemorrhage. Clinical outcome was favorable in all children except in 2. Follow-up data were lacking in most of the studies. RICM is most often asymptomatic but probably an underestimated complication of cerebral irradiation in the pediatric population. Based on the radiological development of RICMs, many authors suggest a follow-up of at least 15 years. Studies suggest observation for asymptomatic lesions, while surgery is reserved for symptomatic growth, hemorrhage, or focal neurological deficits.


Cartilage ◽  
2021 ◽  
pp. 194760352110219
Author(s):  
Danielle H. Markus ◽  
Anna M. Blaeser ◽  
Eoghan T. Hurley ◽  
Brian J. Mannino ◽  
Kirk A. Campbell ◽  
...  

Objective The purpose of the current study is to evaluate the clinical and radiographic outcomes at early to midterm follow-up between fresh precut cores versus hemi-condylar osteochondral allograft (OCAs) in the treatment of symptomatic osteochondral lesions. Design A retrospective review of patients who underwent an OCA was performed. Patient matching between those with OCA harvested from an allograft condyle/patella or a fresh precut allograft core was performed to generate 2 comparable groups. The cartilage at the graft site was assessed with use of a modified Magnetic Resonance Observation of Cartilage Repair Tissue (MOCART) scoring system and patient-reported outcomes were collected. Results Overall, 52 total patients who underwent OCA with either fresh precut OCA cores ( n = 26) and hemi-condylar OCA ( n = 26) were pair matched at a mean follow-up of 34.0 months (range 12 months to 99 months). The mean ages were 31.5 ± 10.7 for fresh precut cores and 30.9 ± 9.8 for hemi-condylar ( P = 0.673). Males accounted for 36.4% of the overall cohort, and the mean lesion size for fresh precut OCA core was 19.6 mm2 compared to 21.2 mm2 for whole condyle ( P = 0.178). There was no significant difference in patient-reported outcomes including Visual Analogue Scale, Knee Injury and Osteoarthritis Outcome Score for Joint Replacement, and Tegner ( P > 0.5 for each), or in MOCART score (69.2 vs. 68.3, P = 0.93). Conclusions This study found that there was no difference in patient-reported clinical outcomes or MOCART scores following OCA implantation using fresh precut OCA cores or size matched condylar grafts at early to midterm follow-up.


2018 ◽  
Vol 46 (4) ◽  
pp. 900-907 ◽  
Author(s):  
Luis E.P. Tírico ◽  
Julie C. McCauley ◽  
Pamela A. Pulido ◽  
William D. Bugbee

Background: Cartilage repair algorithms use lesion size to choose surgical techniques when selecting a cartilage repair procedure. The association of fresh osteochondral allograft (OCA) size with graft survivorship and subjective patient outcomes is still unknown. Purpose: To determine if lesion size (absolute or relative) affects outcomes after OCA transplantation. Study Design: Cohort study; Level of evidence, 3. Methods: The study included 156 knees in 143 patients who underwent OCA transplantation from 1998 to 2014 for isolated femoral condyle lesions. The mean age was 29.6 ± 11.4 years, and 62.9% were male. The majority of patients (62.2%) presented for cartilage repair because of osteochondritis dissecans. The mean graft area, used as a surrogate for absolute size of the lesion, was 6.4 cm2 (range, 2.3-11.5 cm2). The relative size of the lesion was calculated as the tibial width ratio (TWR; ratio of graft area to tibial width) and affected femoral condyle ratio (AFCR; ratio of graft area to affected femoral condyle width) using preoperative radiographs. All patients had a minimum follow-up of 2 years. Further surgical procedures were documented, and graft failure was defined as revision OCA transplantation or conversion to arthroplasty. International Knee Documentation Committee (IKDC) pain, function, and total scores were obtained. Satisfaction with OCA transplantation was assessed. Results: The mean follow-up among patients with grafts remaining in situ was 6.0 years (range, 1.9-16.5 years). The OCA failure rate was 5.8%. Overall survivorship of the graft was 97.2% at 5 years and 93.5% at 10 years. No difference in postoperative outcomes between groups was found in absolute or relative size. Change in IKDC scores (from preoperative to latest follow-up) was greater for knees with large lesions compared to knees with small lesions, among all measurement methods. Overall satisfaction with the results of OCA transplantation was 89.8%. Conclusion: The size of the lesion, either absolute or relative, does not influence outcomes after OCA transplantation for isolated femoral condyle lesions of the knee.


2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Hyeong Seok Nam ◽  
Hyung Wook Kim ◽  
Dae Hwan Kang ◽  
Cheol Woong Choi ◽  
Su Bum Park ◽  
...  

Background. Endoscopic ultrasound- (EUS-) guided drainage is generally performed under fluoroscopic guidance. However, improvements in endoscopic and EUS techniques and experience have led to questions regarding the usefulness of fluoroscopy. This study aimed to retrospectively evaluate the safety and efficacy of EUS-guided drainage of extraluminal complicated cysts without fluoroscopic guidance.Methods. Patients who had undergone nonfluoroscopic EUS-guided drainage of extraluminal complicated cysts were enrolled. Drainage was performed via a transgastric, transduodenal, or transrectal approach. Single or double 7 Fr double pigtail stents were inserted.Results. Seventeen procedures were performed in 15 patients in peripancreatic fluid collections (n=13) and pelvic abscesses (n=4). The median lesion size was 7.1 cm (range: 2.8–13.0 cm), and the mean time spent per procedure was26.2±9.8minutes (range: 16–50 minutes). Endoscopic drainage was successful in 16 of 17 (94.1%) procedures. There were no complications. All patients experienced symptomatic improvement and revealed partial to complete resolution according to follow-up computed tomography findings. Two patients developed recurrent cysts that were drained during repeat procedures, with eventual complete resolution.Conclusion. EUS-guided drainage without fluoroscopic guidance is a technically feasible, safe, and effective procedure for the treatment of extraluminal complicated cysts.


2018 ◽  
Vol 6 (7_suppl4) ◽  
pp. 2325967118S0013 ◽  
Author(s):  
Kelsey Davidson ◽  
Nathan L. Grimm ◽  
Melissa A. Christino ◽  
S. Clifton Willimon ◽  
Michael T. Busch

Objectives: Osteochondritis dissecans (OCD) is an idiopathic alteration of the subchondral bone which may lead to early osteoarthritis. This condition is commonly seen in the knee and is more common in young, athletic individuals. Several techniques have been developed to address the variable presentation of OCD in the knee, each with varying results. To our knowledge the use of autogenous bone marrow supplementation to an OCD lesion has not been described. The purpose of this study was to determine the radiographic healing of OCD lesions of the knee in a cohort of young, active patients who underwent retroarticular drilling of an OCD lesion with supplemental back-filling with bone marrow aspirate concentrate as an adjuvant to facilitate healing. Methods: We evaluated adolescent patients who were diagnosed with OCD of the knee who had previously failed non-operative, conservative treatment. All subjects underwent retroarticular drilling of the knee with supplemental back-filling of the lesion with bone marrow aspirate concentrate (BMAC®), harvested from the ilium, by two Orthopaedic Surgeons from a single institution. Based off previously published definitions, lesions were assigned as small if < 320 mm2 or large >320 mm2. All lesions were then followed radiographically for evidence of healing. Healing was graded based off the ROCK (Research in OsteoChondritis of the Knee) group radiographic healing criteria, which has shown excellent reliability. The healing was rated by two independent reviewers and when disagreements were noted a third reviewer was brought in to facilitate group consensus on healing. Results: 52 lesions in 49 patients were included in the study. 26 right knees, 26 left knees with 41 lesions located on the medial femoral condyle and 11 lesions located on the lateral femoral condyle. The mean age of the subjects was 12.5 (10 - 17) years old. 30 subjects were male, 19 were female. All lesions were categorized as immobile lesions based off the ROCK Arthroscopy Classification. The average OCD lesion size was 407 mm2 (132 - 899 mm2), respectively. The mean amount of BMAC® used per OCD lesion was 60 mL (45 - 120 mL). 40 lesions (76.9%) were rated as healed at a mean of 10.6 (2 - 32) months. 10 lesions (18.9%) were rated as 25-75% healed and 3.8% were rated at 0 - 25% healed at latest follow-up. 76.5% of large lesions were rated as healed at final follow-up. Whereas 77.8% of small lesions were rated as healed at final follow-up. There were three complications (1 rash from skin glue, 1 suture abscess, and 1 contact dermatitis from surgical cleansing prep) however no complications were noted from the surgical intervention or BMAC®. Conclusion: Overall healing rates with surgical treatment of OCD lesions that have failed non-operative interventions are variable. For immobile lesions, mainstay treatment options include retroarticular and transarticular drilling. Based off previous definitions of lesion size, the mean lesion size in our cohort would be categorized as “large” with 65% of our lesions falling into this category. Previously published series have shown that large lesions are significantly less likely to heal. Despite this we showed a 76.5% healing rate for large lesions. Overall, the adjuvant supplementation of BMAC® as back-fill for retrograde drilling of large OCD lesions of the knee has shown excellent results with no serious complications associated with its use. Future randomized trials are necessary to evaluate the comparative effectiveness of this treatment method.


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