RESUMO
Subependymal giant cell astrocytoma (SEGA) is a rare circumscribed astrocytic glioma that occurs in approximately 25% of all tuberous sclerosis (TSC) cases. Herein, we discuss an atypical presentation of SEGA, including the genetic alterations, impact on clinical presentation, and the determinants of each medical and surgical treatment option. A 14-year-old girl presented with intermittent headache and a right intraventricular mass originating near the foramen of Monro. The tumor's proximity to critical structures necessitated maximum safe resection, which improved her symptoms. Histological findings indicated SEGA, and genetic sequencing revealed a TSC2 mutation. However, complete clinical and radiological evaluations failed to reveal TSC. Two months later, a new subependymal nodule was incidentally found. She had a recurrent left occipital horn lesion and diffuse smooth leptomeningeal enhancement with no spine drop metastases. She was administered everolimus as the tumor was considered unresectable. Subsequent imaging revealed a reduction in both residual and new lesions.
Assuntos
Astrocitoma , Mutação , Proteína 2 do Complexo Esclerose Tuberosa , Humanos , Feminino , Astrocitoma/genética , Astrocitoma/diagnóstico por imagem , Astrocitoma/patologia , Proteína 2 do Complexo Esclerose Tuberosa/genética , Adolescente , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Esclerose Tuberosa/genética , Esclerose Tuberosa/diagnóstico por imagem , Esclerose Tuberosa/complicaçõesRESUMO
PURPOSE: Revisional bariatric surgeries following laparoscopic sleeve gastrectomy (LSG) have demonstrated a dramatic increase worldwide. Recently, one-anastomosis gastric bypass (OAGB) has become a contender as an appropriate revisional procedure; however, no long-term data currently exist on the effectiveness of it as a revisional surgery post-LSG. METHODS: A retrospective analysis was performed on all patients who underwent LSG at a public hospital in Kuwait from 2008 to 2017. A list was obtained of those who underwent revisional OAGB surgery after initial LSG, after which a phone survey was performed and demographics were analyzed. RESULTS: A total of 29 patients underwent revisional OAGB post-initial LSG, of which 89.7% were female. Prior to LSG, the mean weight of the patients was 127.5 kg, and the mean BMI was 49.0 kg/m2. The mean weight loss after initial LSG was 43.8 kg, while the average duration until patients underwent revisional OAGB was 5.3 years. The cause for revision was weight regain (86.2%) or inadequate weight loss (13.8%). Prior to undergoing revisional OAGB, the weight and BMI of the patients was 110.9 kg and 42.4 kg/m2, respectively. Revisional OAGB demonstrated a %excess weight loss of 14.5%, 31.9%, 48.0%, 56.3%, 57.2%, and 54.7% at 2 weeks, 3 months, 6 months, 1 year, 4 years, and 5 years, respectively. Twelve morbidities were reported during the follow-up period. CONCLUSION: Revisional bariatric surgery is technically demanding and may be associated with a high complication rate. However, OAGB as a revisional procedure has proven to be safe and effective in the long-term outcomes of revisional OAGB patients post-LSG.
Assuntos
Derivação Gástrica , Laparoscopia , Obesidade Mórbida , Humanos , Feminino , Masculino , Derivação Gástrica/efeitos adversos , Derivação Gástrica/métodos , Obesidade Mórbida/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Laparoscopia/métodos , Reoperação/efeitos adversos , Gastrectomia/efeitos adversos , Gastrectomia/métodos , Redução de PesoRESUMO
BACKGROUND: There are limited data on utility of magnetic resonance imaging (MRI) in the assessment of suspected neonatal hemochromatosis (NH). OBJECTIVES: The aim of the study was to present our experience with utilization of multi-echo sequence MRI technique in the evaluation of NH and to compare MRI findings in infants with and without NH. METHODS: MRI performed for suspected NH were retrospectively reviewed to note the presence and severity of iron deposition (ID) in liver, spleen, pancreas, and kidneys on multi-echo sequences. Findings were compared in infants with and without NH. RESULTS: Of 20 infants (9 boys and 11 girls; median age of 12.5 days) included in the study, 7 of 20 had NH and 13 of 20 were assigned to the non-NH group. Higher degree of pancreatic ID was seen in the NH group (Pâ=â0.001) with 4 of 7 evaluable pancreas showing moderate-to-severe degree and 1 of 7 showing mild degree of ID whereas none of the 13 infants in non-NH group showed moderate or severe degree of pancreatic ID. Even though the severity of hepatic ID was higher in NH group (Pâ=â0.033), variable severity of hepatic ID was seen in both groups with most infants in both groups showing moderate-to-severe degree of ID. The severity of splenic ID was not particularly associated with any group (Pâ=â0.774) but there was no moderate or severe degree of ID in NH. Renal ID was seen in two infants in non-NH group. CONCLUSIONS: A moderate-to-severe degree of pancreatic ID seen on MRI tends to be associated with NH and should be sought to establish a timely diagnosis of NH. Presence and severity of hepatic ID cannot be used for differentiation of NH from other causes of neonatal liver failure.
Assuntos
Hemocromatose/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Feminino , Hemocromatose/patologia , Humanos , Lactente , Recém-Nascido , Ferro/metabolismo , Fígado/diagnóstico por imagem , Fígado/patologia , Masculino , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Estudos Retrospectivos , Baço/diagnóstico por imagem , Baço/patologiaRESUMO
BACKGROUND: Elevated intracranial pressure is a potentially catastrophic complication of neurologic injury in children. Successful management of elevated intracranial pressure requires prompt recognition and therapy directed at both reducing intracranial pressure and reversing its underlying cause. A rare condition that causes elevated intracranial pressure is childhood primary angiitis of the central nervous system, which is a rare inflammatory central nervous system disease that poses diagnostic and therapeutic challenges. To our knowledge, this is the first reported case of angiography-positive progressive childhood primary angiitis of the central nervous system requiring decompressive hemicraniectomy for refractory elevated intracranial pressure in children. CASE PRESENTATION: We report the case of a 5-year-old Saudi girl who presented to the pediatric emergency department with fever and new-onset status epilepticus. She had elevated inflammatory markers with radiological and histopathological evidence of angiography-positive progressive childhood primary angiitis of the central nervous system, complicated by elevated intracranial pressure. Despite medical management for both childhood primary angiitis of the central nervous system and elevated intracranial pressure, her neurological status continued to deteriorate and the elevated intracranial pressure became refractory. She developed right uncal, right subfalcine, and tonsillar herniation requiring decompressive hemicraniectomy with a favorable neurological outcome. CONCLUSION: Decompressive craniectomy might be considered in cases of angiography-positive progressive childhood primary angiitis of the central nervous system with elevated intracranial pressure refractory to medication. A multidisciplinary approach for the decision of decompressive craniectomy is advised to ensure patient safety and avoid possible morbidities and mortality.
Assuntos
Craniectomia Descompressiva , Hipertensão Intracraniana , Vasculite do Sistema Nervoso Central , Criança , Pré-Escolar , Feminino , Humanos , Hipertensão Intracraniana/cirurgia , Pressão IntracranianaRESUMO
BACKGROUND: Bariatric surgery has been shown to produce the most predictable weight loss results, with laparoscopic sleeve gastrectomy (SG) being the most performed procedure as of 2014. However, inadequate weight loss may present the need for a revisional procedure. OBJECTIVES: The aim of this study is to compare the efficacy of laparoscopic resleeve gastrectomy (LRSG) and laparoscopic Roux-en-Y gastric bypass in attaining successful weight loss. SETTING: Public hospital following SG. METHODS: A retrospective analysis was performed on all patients who underwent SG from 2008-2019. A list was obtained of those who underwent revisional bariatric surgery after initial SG, and their demographic characteristics were analyzed. RESULTS: A total of 2858 patients underwent SG, of whom 84 patients (3%) underwent either a revisional laparoscopic Roux-en-Y gastric bypass (rLRYGB) or LRSG. A total of 82% of the patients were female. The mean weight and body mass index (BMI) before SG for the LRSG and rLRYGB patients were 136.7 kg and 49.9 kg/m2 and 133.9 kg and 50.5 kg/m2, respectively. The mean BMI showed a drop from 42.0 to 31.7 (P < .001) 1 year post revisional surgery for the LRSG group and 42.7 to 34.5 (P < .001) for the rLRYGB group, correlating to an excess weight loss (EWL) of 61.7% and 48.1%, respectively. At 5 years post revisional surgery, LRSG patients showed an increase in BMI to 33.8 (EWL = 45.3%), while those who underwent rLRYGB showed a decrease to 34.3 (EWL = 49.2%). Completeness of follow-up at 1, 3, and 5 years for rLRYGB and LRSG were 67%, 35%, and 24% and 45%, 21%, and 18%, respectively. CONCLUSIONS: Revisional bariatric surgery is a safe and effective method for the management of failed primary SG. LRSG patients tended to do better earlier on; however, it leveled off with those who underwent rLRYGB by 5 years.
Assuntos
Cirurgia Bariátrica , Derivação Gástrica , Laparoscopia , Obesidade Mórbida , Feminino , Gastrectomia , Humanos , Masculino , Obesidade Mórbida/cirurgia , Reoperação , Estudos Retrospectivos , Resultado do TratamentoRESUMO
INTRODUCTION: Laparoscopic sleeve gastrectomy (LSG) has become the most performed bariatric procedure as of 2015. However, inadequate weight loss may present the need for revisional procedures. OBJECTIVES: The objective of this study was to analyze the efficacy of single-anastomosis gastric bypass (SAGB) in attaining successful weight loss following an initial LSG. METHODS: A retrospective analysis was performed on all patients who underwent LSG at Amiri Hospital, Kuwait from 2008 to 2017. A list was obtained of those who underwent revisional SAGB surgery after initial LSG, and their demographics were analyzed. RESULTS: A total of 31 patients underwent revisional SAGB bariatric surgery after initial LSG, of which 87.1% were female. Prior to LSG, the mean weight of the patients was 127.5 kg and the mean BMI was 49.0 kg/m2. The mean weight loss after initial LSG was 43.8 kg, while the average duration until patients underwent revisional SAGB was 5.1 years. The cause for revision was weight regain (86.2%), inadequate weight loss (10.3%), or the development of a complication (reflux) (6.5%). Prior to undergoing revisional SAGB, the weight and BMI of the patients were 110.9 kg and 42.6 kg/m2, respectively. This demonstrated a %excess weight loss of 14.5, 31.9, 48.0, and 58.9% 2 weeks, 3 months, 6 months, and 1-year post-SAGB, respectively. Three (10.3%) morbidities were reported and thereafter successfully resolved. CONCLUSION: Revisional SAGB bariatric surgery is a safe and effective method in the short-term basis for the management of inadequate primary LSG.
Assuntos
Gastrectomia/efeitos adversos , Derivação Gástrica , Obesidade Mórbida/cirurgia , Reoperação/métodos , Aumento de Peso , Adulto , Anastomose Cirúrgica/métodos , Índice de Massa Corporal , Feminino , Gastrectomia/métodos , Derivação Gástrica/métodos , Humanos , Kuweit/epidemiologia , Laparoscopia/métodos , Masculino , Pessoa de Meia-Idade , Obesidade Mórbida/epidemiologia , Reoperação/efeitos adversos , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Laparoscopic sleeve gastrectomy (LSG) is increasing worldwide; however, long-term follow-up results included insufficient weight loss and weight regain. This study aims at assessing the outcomes of converting LSG to laparoscopic Roux-en-Y gastric bypass (LRYGB) and laparoscopic re-sleeve gastrectomy (LRSG). METHODS: A total of 1300 patients underwent LSG from 2009 to 2012, of which 12 patients underwent LRYGB and 24 patients underwent LRSG in Al-Amiri Hospital alone. Data included length of stay, percentage excessive weight loss (EWL%), and body mass index (BMI). RESULTS: Twenty-four patients underwent conversion from LSG to LRSG, and 12 patients underwent conversion from LSG to LRYGB due to insufficient weight loss and weight regain. Eighty-five percent were females. The mean weight and BMI prior to LSG for the LRYGB and LRSG patients were 136.5 kg and 52, and 134 kg and 50, respectively. The EWL% after the initial LSG was 37.9 and 43 %, for LRYGB and LRSG, respectively. There were no complications recorded. Results of conversion of LSG to LRYGB involved a mean EWL% 61.3 % after 1 year (p value 0.009). Results of LRSG involved a mean EWL% of 57 % over interval of 1 year (p value 0.05). Comparison of the EWL% of LRYGB and LRSG for failed primary LSG was not significant (p value 0.097). CONCLUSION: Following our algorithm, revising an LSG with an LRSG or LRYGB for poor weight loss is feasible with good outcomes. Larger and longer follow-up studies are needed to verify our results.