scholarly journals Epilepsy surgery in tuberous sclerosis complex: the BC Children’s Hospital experience

2015 ◽  
Vol 42 (S1) ◽  
pp. S14-S14
Author(s):  
C Wilbur ◽  
C Sanguansermsri ◽  
H Chable ◽  
A Mihaela ◽  
P Steinbok ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Tuberous Sclerosis Complex ◽  
Chart Review ◽  
Retrospective Chart Review ◽  
Seizure Outcome ◽  
Corpus Callosotomy ◽  
Age At Surgery ◽  
Second Resection

Background: Epilepsy occurs in up to 90% of patients with Tuberous Sclerosis Complex (TSC) and is often refractory to medications. Our objective was to assess the safety and outcome of epilepsy surgery in children with TSC at our institution. Methods: We performed a systematic, retrospective chart review of children with TSC who underwent epilepsy surgery at our institution. Patients were identified through epilepsy and clinical neurophysiology databases. Results: 19 patients (out of 81 with TSC) underwent surgery between 1995-2014. Median age at surgery was 4.2 (Range 1.1-15.6) years, with patients having failed a median 4 (Range 0-10) anti-seizure medications. Surgery comprised corpus callosotomy in 2 and resection of one or more tubers in 17. 2 patients had a subsequent second resection. Minor neurologic deficits occurred after 14% of surgeries. Median follow-up was 2.4 years (Range 0.3 -13.8 years) following surgery . At last follow-up, 47% were seizure free, including 2 patients off anti-seizure medication. Conclusions: Epilepsy surgery is safe and effective in carefully selected TSC patients, with the majority having a good seizure outcome. Children with epilepsy secondary to TSC should be referred for epilepsy surgery assessment.

scholarly journals The diverse manifestations of tuberous sclerosis complex: the experience of a provincial TSC clinic

2015 ◽  
Vol 42 (S1) ◽  
pp. S14-S14
Author(s):  
C Wilbur ◽  
C Sanguansermsri ◽  
H Chable ◽  
A Mihaela ◽  
MB Connolly
Keyword(s):  
Intellectual Disability ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Chart Review ◽  
Retrospective Chart Review ◽  
Multidisciplinary Care ◽  
Care Needs ◽  
History Of ◽  
Study Population

Background: Recent consensus recommendations for Tuberous Sclerosis Complex (TSC) stress the importance of multidisciplinary follow-up for these patients. The objective of our study was to review the manifestations of TSC seen in our hospital to determine the care needs of this population. Methods: This was a systematic, retrospective chart review of children with TSC treated at our institution. Patients were identified through epilepsy and clinical neurophysiology databases. Results: The study population comprised 81 patients, born between 1987-2014, who were a median 10 (Range 0.2-23.2) years of age at last follow-up. 88% of patients had epilepsy, including 30% with a history of infantile spasms. Developmental delay was reported in 65%, while 40% had intellectual disability. Psychiatric co-morbidities occurred in 49%. The most common psychiatric diagnoses were autism (25%), ADHD (19%), and anxiety (16%). Cardiac rhabdomyomas occurred in 35% of patients and renal angiomyolipomas in 42%, while only 4% had polycystic kidneys. Subependymal giant cell astrocytomas were observed in 14% of patients. 86% had skin manifestations. Conclusions: This study reaffirms the multi-system manifestations of TSC and the need to provide comprehensive, multidisciplinary care. As many children are still very young, the prevalence of autism and intellectual disability is likely underestimated.

Good seizure outcome after epilepsy surgery in young children with tuberous sclerosis complex

2006 ◽  
Vol 37 (03) ◽  
Author(s):  
C Krahn-Peper ◽  
IEB Tuxhorn ◽  
K Ahlbory ◽  
F Behne ◽  
H Pannek
Keyword(s):  
Young Children ◽  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Tuberous Sclerosis Complex ◽  
Seizure Outcome

scholarly journals Tuberous sclerosis complex: Clinical spectrum and epilepsy: A retrospective chart review study

2018 ◽  
Vol 9 (1) ◽  
pp. 154-160
Author(s):  
Sulaiman Almobarak ◽  
Mohammad Almuhaizea ◽  
Musaad Abukhaled ◽  
Suad Alyamani ◽  
Omar Dabbagh ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Gene Mutation ◽  
Tuberous Sclerosis Complex ◽  
Chart Review ◽  
Clinical Presentation ◽  
Retrospective Chart Review ◽  
Neurocutaneous Disorder ◽  
Development Delay ◽  
Review Study ◽  
New Onset

Abstract Tuberous sclerosis complex (TSC) is an autosomal dominant genetic neurocutaneous disorder, with heterogeneous manifestations. We aimed to review the clinical presentation of TSC and its association with epilepsy among Saudi population. This was a retrospective chart review study of 88 patients diagnosed with TSC with or without epilepsy. In 38.6% of patients, symptoms began before 1 year of age. The most frequent initial manifestations of TSC were new onset of seizures (68.2%), skin manifestations (46.6%) and development delay (23.9%). During the evolution of the disease 65.9% had epilepsy, 17% facial angiofibromas, 13.6% Shagreen patch, 18.2% heart rhabdomyomas and 12.5% retinal hamartomas. The genetic study for TSC diagnosis was done for 44 patients, 42 (95,4%) of them were genetically confirmed, for whom 13 patients had TSC1 mutation (29.5%), 29 patients were carrying TSC2 gene mutation (65.9%), Genetic test for TSC 1 and TSC 2 were negative for 2 patients (4.5%) despite positive gene mutation in their relative with TSC. The most common manifestations were central nervous system (predominantly epilepsy) and dermatological manifestations. Most of the patients develop epilepsy with multiple seizure types. TSC 2 mutation is more common than TSC 1 mutation.

scholarly journals Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions

2015 ◽  
Vol 15 (1) ◽  
pp. 26-33 ◽  
Author(s):  
Ravindra Arya ◽  
Jeffrey R. Tenney ◽  
Paul S. Horn ◽  
Hansel M. Greiner ◽  
Katherine D. Holland ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Tuberous Sclerosis Complex ◽  
Refractory Epilepsy ◽  
Full Scale ◽  
Secondary Outcome ◽  
Resective Surgery ◽  
Class 1 ◽  
Medically Refractory Epilepsy

OBJECT Tuberous sclerosis complex (TSC) with medically refractory epilepsy is characterized by multifocal brain abnormalities, traditionally indicating poor surgical candidacy. This single-center, retrospective study appraised seizurerelated, neuropsychological, and other outcomes of resective surgery in TSC patients with medically refractory epilepsy, and analyzed predictors for these outcomes. METHODS Patients with multilesional TSC who underwent epilepsy surgery between 2007 and 2012 were identified from an electronic database. All patients underwent multimodality noninvasive and subsequent invasive evaluation. Seizure outcomes were classified using the International League Against Epilepsy (ILAE) scale. The primary outcome measure was complete seizure remission (ILAE Class 1). Secondary outcome measures included 50% responder rate, change in full-scale IQ, electroencephalography improvement, and reduction in antiepileptic drug (AED) burden. RESULTS A total of 37 patients with TSC underwent resective surgery during the study period. After a mean follow-up of 5.68 ± 3.67 years, 56.8% achieved complete seizure freedom (ILAE Class 1) and 86.5% had ILAE Class 4 outcomes or better. The full-scale IQ on follow-up was significantly higher in patients with ILAE Class 1 outcome (66.70 ± 12.36) compared with those with ILAE Class 2 or worse outcomes (56.00 ± 1.41, p = 0.025). In 62.5% of the patients with ILAE Class 2 or worse outcomes, the number of AEDs were found to be significantly reduced (p = 0.004). CONCLUSIONS This study substantiates the evidence for efficacy of resective epilepsy surgery in patients with bilateral multilesional TSC. More than half of the patients were completely seizure free. Additionally, a high proportion achieved clinically meaningful reduction in seizure burden and the number of AEDs.

A single centre experience with an evolving approach for the repair of coarctation of the aorta

2019 ◽  
Vol 29 (7) ◽  
pp. 885-887
Author(s):  
Kelly Costopoulos ◽  
Joseph Philip ◽  
Dalia Lopez-Colon ◽  
Giridhar Kaliki ◽  
Arun Chandran ◽  
...  
Keyword(s):  
Chart Review ◽  
Median Sternotomy ◽  
Coarctation Of The Aorta ◽  
Retrospective Chart Review ◽  
Single Centre ◽  
Vocal Cord Paresis ◽  
Age At Surgery ◽  
Single Centre Experience ◽  
Lateral Thoracotomy

AbstractBackground:Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention.Methods:Retrospective chart review of 66 patients aged 0–1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4–232) and median weight was 3.08 kg (1.25–8.0). Forty-one (62%) patients underwent median sternotomy.Results:There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%.Conclusion:We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.

scholarly journals Surgical and seizure outcome in children with DNETs who underwent epilepsy surgery

2015 ◽  
Vol 42 (S1) ◽  
pp. S22-S22
Author(s):  
AM Bueckert ◽  
J Pugh ◽  
T Snyder ◽  
M Wheatley ◽  
F Jacob ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Epilepsy Surgery ◽  
Tumor Resection ◽  
Retrospective Chart Review ◽  
Benign Tumors ◽  
Seizure Outcome ◽  
Low Grade ◽  
Partial Seizures ◽  
Complex Partial Seizures

Background: Dysembryoblastic neuroepithelial tumors (DNETs) are benign tumors of the cerebral cortex that most commonly occur in children or young adults. Seizures are a frequent presenting feature, with an incidence of 80-100%, and are often an indication for surgical resection. Methods: We performed a retrospective chart review of children with DNETs who underwent epilepsy surgery between 1998 and 2014. Results: A total of 12 subjects were identified (6 males, 6 females), all of whom had seizures prior to surgical resection. Of these patients, 1 had infantile spasms, 2 had simple partial seizures and 10 had complex partial seizures. Tumors were located in the temporal (n=7), frontal (n=3) or parietal (n=2) cortex. These patients went on to have surgery on average 15 months after seizure onset, 3 had incomplete resections. At an average follow up of 6 years 4 months, all patients were class 1 on Engel’s Classification. All but one subject with rare non-disabling seizures were seizure free, with only 6 on medication. Follow up MR imaging revealed tumor recurrence in 1 subject. Conclusions: Despite differing seizure seminology and tumor location, surgical resection of these low-grade tumors resulted in excellent seizure outcome even in the setting of incomplete tumor resection.

scholarly journals Resective epilepsy surgery in tuberous sclerosis complex: a nationwide multicentre retrospective study from China

Brain ◽  
2020 ◽  
Vol 143 (2) ◽  
pp. 570-581 ◽  
Author(s):  
Shiyong Liu ◽  
Tao Yu ◽  
Yuguang Guan ◽  
Kai Zhang ◽  
Ping Ding ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Tuberous Sclerosis Complex ◽  
Intelligence Quotient ◽  
Influence Factors ◽  
Seizure Freedom ◽  
Postoperative Seizure

Abstract At least 50% of patients with tuberous sclerosis complex present with intractable epilepsy; for these patients, resective surgery is a treatment option. Here, we report a nationwide multicentre retrospective study and analyse the long-term seizure and neuropsychological outcomes of epilepsy surgery in patients with tuberous sclerosis complex. There were 364 patients who underwent epilepsy surgery in the study. Patients’ clinical data, postoperative seizure outcomes at 1-, 4-, and 10-year follow-ups, preoperative and postoperative intelligence quotients, and quality of life at 1-year follow-up were collected. The patients’ ages at surgery were 10.35 ± 7.70 years (range: 0.5–47). The percentage of postoperative seizure freedom was 71% (258/364) at 1-year, 60% (118/196) at 4-year, and 51% (36/71) at 10-year follow-up. Influence factors of postoperative seizure freedom were the total removal of epileptogenic tubers and the presence of outstanding tuber on MRI at 1- and 4-year follow-ups. Furthermore, monthly seizure (versus daily seizure) was also a positive influence factor for postoperative seizure freedom at 1-year follow-up. The presence of an outstanding tuber on MRI was the only factor influencing seizure freedom at 10-year follow-up. Postoperative quality of life and intelligence quotient improvements were found in 43% (112/262) and 28% (67/242) of patients, respectively. Influence factors of postoperative quality of life and intelligence quotient improvement were postoperative seizure freedom and preoperative low intelligence quotient. The percentage of seizure freedom in the tuberectomy group was significantly lower compared to the tuberectomy plus and lobectomy groups at 1- and 4-year follow-ups. In conclusion, this study, the largest nationwide multi-centre study on resective epilepsy surgery, resulted in improved seizure outcomes and quality of life and intelligence quotient improvements in patients with tuberous sclerosis complex. Seizure freedom was often achieved in patients with an outstanding tuber on MRI, total removal of epileptogenic tubers, and tuberectomy plus. Quality of life and intelligence quotient improvements were frequently observed in patients with postoperative seizure freedom and preoperative low intelligence quotient.

Late endoscopic evaluation of the ostium size after external dacryocystorhinostomy

2020 ◽  
pp. 112067212097604
Author(s):  
Reem R Al Huthail ◽  
Yasser H Al-Faky
Keyword(s):  
Chart Review ◽  
Retrospective Chart Review ◽  
Tertiary Hospital ◽  
Nasal Endoscope ◽  
Long Term Follow Up ◽  
Mean Size ◽  
External Dacryocystorhinostomy ◽  
The Mean

Objective: To evaluate the effect of chronicity on the size of the ostium after external dacryocystorhinostomy (DCR) with intubation. Methods: Design: A retrospective chart review of patients who underwent external DCR with intubation over 10 years from January 2003 at a tertiary hospital. All patients were recruited and examined with rigid nasal endoscope. Results: A total number of 66 (85 eyes) patients were included. The mean age at the time of evaluation was 53.1 years with gender distribution of 54 females (81.8 %). The mean duration ±SD between the date of surgery and the date of evaluation was 33.2 ± 33.6 (6–118 months). Our study showed an overall anatomical and functional success of 98.8% and 95.3%, respectively. The mean size of the ostium (±SD) was 23.0 (±15.7) mm2 (ranging from 1 to 80.4 mm2). The size of the ostium was not a significant factor for failure ( p = 0.907). No statistically significant correlation was found between the long-term duration after surgery and the size of the ostium ( R: 0.025, p = 0.157). Conclusions: Nasal endoscopy after DCR is valuable in evaluating the ostium with no observed potential correlation between the long-term follow-up after surgery and the size of the ostium.

Cholecystectomy for Biliary Hyperkinesia

2019 ◽  
Vol 85 (2) ◽  
pp. 219-222 ◽  
Author(s):  
Joshua Gazzetta ◽  
Betty Fan ◽  
Paul Bonner ◽  
John Galante
Keyword(s):  
Laparoscopic Cholecystectomy ◽  
Chart Review ◽  
Gallbladder Disease ◽  
Retrospective Chart Review ◽  
Biliary Colic ◽  
Study Cohort ◽  
Final Pathology ◽  
Pathology Reports ◽  
Histologic Changes

Patients with classic biliary colic symptoms and documented gallbladder ejection fractions on the higher end of the spectrum on hepatobiliary iminoacetic acid scans with cholecystokinin stimulation are presently understudied and the benefits of cholecystectomy are unclear. To determine whether patients with biliary-type pain and biliary hyperkinesia (defined as a gallbladder ejection fractions of 80% or greater) benefit from laparoscopic cholecystectomy, a retrospective chart review encompassing five community hospitals was performed. Patients 16 years and older with diagnosed biliary hyperkinesia who underwent laparoscopic cholecystectomy between January 1, 2010 and May 31, 2015 were included. Pathology reports were reviewed for histologic changes indicating cholecystitis. Resolution of biliary colic symptoms was reviewed one to three weeks after surgery in their postoperative follow-up documentation. Within our study cohort, we found 97 patients who underwent laparoscopic cholecystectomy for biliary hyperkinesia. Within this population, 84.5 per cent of patients undergoing laparoscopic cholecystectomy for biliary hyper-kinesia had positive findings for gallbladder disease on final pathology. Of the 77 patients with data available from their first postoperative visit, 70 (90.9%) reported improvement or resolution of symptoms. Our findings suggest that symptomatic biliary hyperkinesia may be treated successfully with surgery.

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