[Surgical results of video-assisted thoracoscopic thymectomy for treatment of Juvenile Myasthenia Gravis]. / Resultados quirúrgicos de la timectomía por videotoracoscopía en el tratamiento de la Miastenia Gravis Juvenil.

INTRODUCTION: Juvenile myasthenia gravis (JMG) is an autoimmune disease affecting the neuromuscular junction that appears before 19 years of age with varying degrees of weakness of different muscle groups. The main treatment is pharmacological, but thymectomy has also demonstrated to improve remission rates. OBJECTIVE: To describe the clinical characteristics and postoperative course of pediatric patients with JMG who underwent video-assisted thoracoscopic (VATS) thymectomy. Clinical Serie: Six pa tients who underwent VATS thymectomy between March 2011 and June 2019. The age range at diag nosis was between 2 and 14 years and the average age at surgery was 7 years. All patients were under treatment with pyridostigmine bromide associated with immunosuppression with corticosteroids before surgery. The interval between diagnosis and thymectomy was 21.5 months on average. VATS was performed by left approach, and there was no perioperative morbidity or mortality. The average hospital stay was 2 days. Three patients remain with no symptoms and without corticotherapy. Two patients were on corticosteroids, but in smaller doses than previous to surgery. One patient presented a crisis requiring hospitalization and ventilatory support during follow-up. CONCLUSION: VATS thy mectomy is part of the treatment for JMG. In this series, it appears as a safe approach and its results were favorable.

Per oral endoscopic myotomy (POEM) in pediatric patients with esophageal achalasia: First Latin-American experience.

BACKGROUND: Achalasia is the most common primary motor disorder of the esophagus, but its incidence in pediatric patients is low. Laparoscopic Heller myotomy (LHM) is the current surgical standard of care treatment. Per-oral endoscopic myotomy (POEM) has emerged as a safe and effective therapeutic alternative in adult patients. We herein report the outcomes of a cohort of pediatric patients with achalasia treated by POEM at a Chilean medical center. METHODS: This is a retrospective analysis of prospectively collected data on children who underwent POEM for esophageal achalasia. Clinical follow-up was evaluated by recording the Eckardt score, a high-resolution esophageal manometry (HREM) three months after the procedure, and an annual upper gastrointestinal endoscopy. RESULTS: Five patients with esophageal achalasia confirmed by HREM and with a mean age of 11 (5 to 15) years underwent POEM between 2017 and 2019. One patient had a previous LHM. No morbidity or mortality was observed. All patients resolved their dysphagia and no patient required further interventions. Mean Eckardt score reduced from 10 points preoperatively to 1 point postoperatively. Two patients currently have mild esophagitis (confirmed by endoscopy). CONCLUSION: Our results support the previously reported safety and effectiveness of POEM. Longer follow-up and larger cohorts will be important to confirm its role in the treatment of children with esophageal achalasia. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level IV.

Intracisternal BioGlue injection in the fetal lamb: a novel model for creation of obstructive congenital hydrocephalus without additional chemically induced neuroinflammation.

OBJECTIVE: The authors hypothesized that new agents such as BioGlue would be as efficacious as kaolin in the induction of hydrocephalus in fetal sheep. METHODS: This study was performed in 34 fetal lambs randomly divided into 2 studies. In the first study, fetuses received kaolin, BioGlue (2.0 mL), or Onyx injected into the cisterna magna, or no injection (control group) between E85 and E90. In the second study, fetuses received 2.0-mL or 2.5-mL injections of BioGlue into the cisterna magna between E85 and E90. Fetuses were monitored using ultrasound to assess lateral ventricle size and progression of hydrocephalus. The fetuses were delivered (E120-E125) and euthanized for histological analysis. Selected brain sections were stained for ionized calcium binding adaptor 1 (Iba1) and glial fibrillary acidic protein (GFAP) to assess the presence and activation of microglia and astroglia, respectively. Statistical comparisons were performed with Student's t-test for 2 determinations and ANOVA 1-way and 2-way repeated measures for multiple determinations. RESULTS: At 30 days after injection, the lateral ventricles were larger in all 3 groups that had undergone injection than in controls (mean diameter in controls 3.76 ± 0.05 mm, n = 5). However, dilatation was greater in the fetuses injected with 2 mL of BioGlue (11.34 ± 4.76 mm, n = 11) than in those injected with kaolin (6.4 ± 0.98 mm, n = 7) or Onyx (5.7 ± 0.31 mm, n = 6) (ANOVA, *p ≤ 0.0001). Fetuses injected with 2.0 mL or 2.5 mL of BioGlue showed the same ventricle dilatation but it appeared earlier (at 10 days postinjection) in those injected with 2.5 mL. The critical threshold of ventricle dilatation was 0.1 for all the groups, and only the BioGlue 2.0 mL and BioGlue 2.5 mL groups exceeded this critical value (at 30 days and 18 days after injection, respectively) (ANOVA, *p ≤ 0.0001). Moderate to severe hydrocephalus with corpus callosum disruption was observed in all experimental groups. All experimental groups showed ventriculomegaly with significant microgliosis and astrogliosis in the subventricular zone around the lateral ventricles. Only kaolin resulted in significant microgliosis in the fourth ventricle area (ANOVA, *p ≤ 0.005). CONCLUSIONS: The results of these studies demonstrate that BioGlue is more effective than Onyx or kaolin for inducing hydrocephalus in the fetal lamb and results in a volume-related response by obstructive space-occupancy without local neuroinflammatory reaction. This novel use of BioGlue generates a model with potential for new insights into hydrocephalus pathology and the development of therapeutics in obstructive hydrocephalus. In addition, this model allows for the study of acute and chronic obstructive hydrocephalus by using different BioGlue volumes for intracisternal injection.

Comparative study of intracisternal kaolin injection techniques to induce congenital hydrocephalus in fetal lamb.

PURPOSE: Kaolin (aluminum silicate) has been used to generate hydrocephalus by direct cisterna magna injection in animal models. The aim of the present study is to compare which method of Kaolin injection into fetal cisterna magna is feasible, safer, and more effective to induce hydrocephalus in fetal lambs. METHODS: Twenty-five well-dated pregnant ewes at gestational 85-90 days (E85-90) were used to compare three different kaolin injection puncture techniques into the fetal cisterna magna. Group 1, ultrasound guidance in a maternal percutaneous transabdominal (TA); group 2, without opening the uterus in a transuterine (TU) technique; group 3, by occipital direct access after exteriorizing fetal head (EFH); and group 4, control group, was normal fetal lambs without injection. The fetal lambs were assessed using lateral ventricle diameter ultrasonographic measurements prior the kaolin injection and on the subsequent days. We analyzed the effectivity, mortality, and fetal losses to determine the best technique to create hydrocephalus in fetal lamb. RESULTS: After fetal intracisternal kaolin (2%, 1mL) injection, lateral ventricle diameters increased progressively in the three different interventional groups compared with the normal values of the control group (p ≤ 0.05). We observed that the transabdominal method had a 60% of fetal losses, considering failure of injection and mortality, compared with the 12.5% in the open group (EFH), and 0% for the transuterine group. CONCLUSIONS: Based on our study, we believe that both, open uterine (EFH) and transuterine approaches are more effective and safer than the transabdominal ultrasound-guided method to induce hydrocephalus.

[Treatment of Familial Adenomatous Polyposis and family screening]. / Poliposis familiar: alternativas terapéuticas y estudio de los familiares.

BACKGROUND: To reduce the mortality associated to Familial Adenomatous Polyposis (FAP), screening of close relatives of patients with the disease is crucial. AIM: To analyze the results of the surgical treatment of patients with FAP, and to evaluate the family screening. PATIENTS AND METHODS: Clinical records of patients operated in our institution since 1977, were reviewed analyzing surgical and pathological results, and follow up. In their family members, we evaluated and analyzed the performance of screening tests, former surgeries, history of disease-related cancer and mortality, all due to FAP. RESULTS: Between January 1977 and August 2002, 15 patients were operated on. Of these, only 33% consulted on the setting of a familial screening. A proctocolectomy and terminal ileostomy was performed in 27% of patients; 20% had a proctocolectomy and ileal pouch, and 53% underwent a total colectomy with ileo-rectal anastomosis. Morbidity and mortality were 7% and 0%, respectively. Twenty percent had a colorectal cancer. During a median of 68 months follow-up, the disease-related survival was 92%; no cancer of the rectal stump was detected. Of the 122 family members identified, only 33% with clear indication of screening underwent a colonoscopy. Twenty-nine percent had a confirmed FAP and were operated: in 61% of them a colorectal cancer was found, and 91% of these died. CONCLUSIONS: The results of the surgical treatment of FAP are satisfactory. Nevertheless, family screening should be improved to reduce the high rates of mortality revealed in the study of other family members.

Estudio comparativo entre drenaje biliar interno y sonda T para la descompresión de la vía biliar después de una coledocotomía / Comparative study between internal biliary drainage and T catheter for decompresing biliary tract after choledocotomy

La sonda de Kehr (sonda T) es en la actualidad el procedimiento de elección para la descompresión de la vía biliar después de una coledocotomía. Sin embargo, su uso no está exento de morbilidad e incomodidad para el paciente. Una alternativa efectiva a la sonda T podría ser el drenaje biliar interno (DBI) puesto en el momento de la coledocotomía. Sin embargo, no se cuenta con estudios comparativos para evaluar su real efectividad. El objetivo de este trabajo es comparar la efectividad del DBI respecto a la sonda T para la descompresión de la vía biliar en un modelo de coledocolitiasis residual simulada en perros. Bajo anestesia general e intubación orotraqueal se operaron 22 perros. En todos ellos se efectuó una laparotomía media seguido de una colecistectomía. Se introdujo una cánula en el conducto cístico para el registro de presión de la vía biliar. Posteriormente se efectuó una coledocotomía, se introdujeron 4 perdigones (1,5 mm de diámetro) en la vía biliar simulando una coledocolitiasis y luego se seleccionó al azar el uso de DBI o sonda T. Las presiones de la vía biliar fueron evaluados en condición basal, posterior a la introcucción de los perdigones y después de realizada la descompresión. Paralelamente se realizó un seguimiento de los niveles plasmáticos de bilirrubina, fosfatasas y transaminasas durante 6 días o hasta el momento en que se realizó la autopsia de los animales. No se encontraron diferencias en las presiones de la vía biliar ni en los valores de las pruebas de colestasia entre los perros descomprimidos con sonda T y DBI. Se concluye que el uso de drenaje biliar interno es una alternativa válida para la descompresión de la vía biliar aun en condiciones extremas (coledocolitiasis residual)