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1.
Pediatr Blood Cancer ; 71(6): e30973, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38556746

RESUMO

BACKGROUND: Latin American countries are improving childhood cancer care, showing strong commitment to implement the Global Initiative for Childhood Cancer, but there are scant publications of the situation at a continental level. METHODS: As part of the International Society of Paediatric Oncology Global Mapping project, delegates of each country participating in the Latin American Society of Pediatric Oncology (SLAOP) and chairs of national pediatric oncology societies and cooperative groups were invited to provide information regarding availability of national pediatric cancer control programs (NPCCP), pediatric oncology laws, pediatric oncology tumor registries, and training programs and support to diagnosis and treatment. RESULTS: Nineteen of the 20 countries participating in SLAOP responded. National delegates reported nine countries with NPCCP and four of them were launched in the past 5 years. National pediatric tumor registries are available in eight countries, and three provided published survival results. Fellowship programs for training pediatric oncologists are available in 12 countries. National delegates reported that eight countries provide support to most essential diagnosis and treatments and 11 provide partial or minimal support that is supplemented by civil society organizations. Seven countries have a pediatric oncology law. There are three international cooperative groups and four national societies for pediatric oncology. CONCLUSION: Despite many challenges, there were dramatic advances in survivorship, access to treatment, and availability of NPCCP in Latin America. Countries with highest social development scores in general provide more complete support and are more likely to have NPCCP, training programs, and reported survival results.

2.
J Pediatr Surg ; 2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38485536

RESUMO

The International Society of Paediatric Surgical Oncology (IPSO) was officially inaugurated in 1991 through the creativeness and inspiration of a collective dynamic group of paediatric surgeons committed to advancing childhood cancer. This article traces the origins and birth of IPSO tracking its modern day development to a growing world community of paediatric surgeon oncology members. LEVEL OF EVIDENCE: 5.

3.
Front Pediatr ; 10: 914942, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35935364

RESUMO

Background: Thyroid gland malignancies are rare in pediatric patients (0.7% of tumors); only 1.8% are observed in patients aged <20 years, with a higher prevalence recorded in women and adolescents. Risk factors include genetic syndromes, MEN disorders, autoimmune diseases, and exposure to ionizing radiation. Radiotherapy is also associated with an increased risk of secondary thyroid cancer. This study describes the clinical features and surgical outcomes of primary and secondary thyroid tumors in pediatric patients. Methods: Institutional data were collected from eight international surgical oncology centers for pediatric patients with thyroid cancer between 2000 and 2020. Statistical analyses were performed using the GraphPad Prism software. Results: Among 255 total cases of thyroid cancer, only 13 (5.1%) were secondary tumors. Primary thyroid malignancies were more likely to be multifocal in origin (odds ratio [OR] 1.993, 95% confidence interval [CI].7466-5.132, p = 0.2323), have bilateral glandular location (OR 2.847, 95% CI.6835-12.68, p = 0.2648), and be metastatic at first diagnosis (OR 1.259, 95% CI.3267-5.696, p > 0.999). Secondary tumors showed a higher incidence of disease relapse (OR 1.556, 95% CI.4579-5.57, p = 0.4525) and surgical complications (OR 2.042, 95% CI 0.7917-5.221, p = 0.1614), including hypoparathyroidism and recurrent laryngeal nerve injury. The overall survival (OS) was 99% at 1 year and 97% after 10 years. No EFS differences were evident between the primary and secondary tumors (chi-square 0.7307, p = 0.39026). Conclusions: This multicenter study demonstrated excellent survival in pediatric thyroid malignancies. Secondary tumors exhibited greater disease relapse (15.8 vs. 10.5%) and a higher incidence of surgical complications (36.8 vs. 22.2%).

4.
Pediatr Surg Int ; 38(10): 1435-1444, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35876904

RESUMO

PURPOSE: To analyze the role of laparoscopic surgery for the management of pediatric adrenal tumors (AT). METHODS: Retrospective analysis of children diagnosed with AT, operated laparoscopically during 2003-2020. The strategy differed according to tumor extension. AT < 6 cm were resected. Locally advanced tumors (L2) or > 6 cm were biopsied. RESULTS: N = 28. Complete tumor resection (R0) in 20 (71%), tumor biopsy in 8 (R2). Age (median): 28.8 months (2 months-18 years). 14/28 left-sided, 2 bilateral. Median operating time: 78 min (45-180). Mean tumor size (for resections): 4 cm (2.5-6). Tumor pathology: neuroblastoma (n = 17), Ganglioneuroma (n = 7), Adrenocortical carcinoma (n = 1), Osteosarcoma metastasis (n = 1), Pheochromocytoma (n = 1), Venous malformation (n = 1). Mean hospital stay: 2.5 days (1-3). Mean follow up: 65.5 months (24-192). Overall survival and event-free survival were 86 and 75%, respectively (5 years event-free survival for neuroblastoma: 33% [intermediate risk], 16.6% [high risk]. No surgery-related mortality. CONCLUSION: Laparoscopic surgery for adrenal tumors is safe. Laparoscopic biopsy is useful for unresectable tumors when a percutaneous approach is not possible. With the proposed selection criteria, the laparoscopic approach should be the first option for resection of small and localized AT in pediatrics.


Assuntos
Neoplasias das Glândulas Suprarrenais , Laparoscopia , Neuroblastoma , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Criança , Humanos , Neuroblastoma/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
5.
Ecancermedicalscience ; 16: 1356, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35510137

RESUMO

Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries. The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High- (HICs) and Low- and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer. We hope that this initiative will benefit children worldwide in the best way possible. Simone Abib, IPSO President Justin T Gerstle, IPSO Education Committee Chair Chan Hon Chui, IPSO Secretary.

6.
Arch Argent Pediatr ; 119(5): e513-e517, 2021 10.
Artigo em Espanhol | MEDLINE | ID: mdl-34569753

RESUMO

Graft versus host disease is a serious complication that occurs following bone marrow transplant with significant morbidity and mortality. The gold standard to diagnose gastrointestinal graft versus host disease is upper and lower gastrointestinal endoscopy with histological validation. The development of intramural duodenal hematoma is a rare complication associated with this procedure. We present two cases of intramural duodenal haematoma after duodenal biopsies in bone marrow transplant patients that presented clinically with severe abdominal pain and intestinal bleeding. In both cases, CT scans confirmed the diagnosis and they were treated conservatively with favorable outcomes. Final diagnosis of gastrointestinal graft versus host disease was based on the colonic samples with normal duodenal histoarchitecture, which could lead to avoiding duodenal samples in future patients in order to prevent this serious complication and thus diminish morbidity.


La enfermedad de injerto contra huésped es una complicación grave que se presenta después del trasplante de médula ósea, con morbilidad y mortalidad elevadas. El patrón de oro para evaluar su compromiso gastrointestinal es la endoscopia digestiva alta y baja con toma de biopsia. El desarrollo de hematoma duodenal intramural es una complicación poco frecuente asociada con este procedimiento. Se presentan dos casos de hematoma duodenal intramural posendoscopia en pacientes con trasplante y sospecha de enfermedad injerto contra huésped que presentaron un cuadro agudo de dolor abdominal y sangrado intestinal. El diagnóstico se realizó por tomografía y recibieron tratamiento conservador, con un resultado favorable. En ambos casos, el diagnóstico de enfermedad injerto contra huésped gastrointestinal se hizo a través de las biopsias colónicas con histología duodenal normal, lo que sugiere evitar la toma de muestras duodenales para prevenir esta grave complicación en pacientes de alto riesgo y, de este modo, disminuir la morbilidad.


Assuntos
Duodenopatias , Doença Enxerto-Hospedeiro , Criança , Duodenopatias/diagnóstico , Duodenopatias/etiologia , Endoscopia Gastrointestinal , Hemorragia Gastrointestinal , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/etiologia , Hematoma/diagnóstico , Hematoma/etiologia , Humanos
7.
Arch. argent. pediatr ; 119(4): 224-229, agosto 2021. tab, ilus
Artigo em Inglês, Espanhol | LILACS, BINACIS | ID: biblio-1280889

RESUMO

Introducción. La apendicitis constituye la principal causa de abdomen agudo quirúrgico en pediatría. Durante la pandemia por COVID-19, se replantearon las estrategias de manejo ydisminuyeron las consultas en las guardias, lo que podría asociarse a diagnósticos tardíos y complicaciones. El objetivo de este estudio fue analizar el impacto de la pandemia en los niños con apendicitis aguda. Métodos. Estudio analítico retrospectivocomparativo de pacientes pediátricos conapendicitis aguda durante los cinco meses del confinamiento por COVID-19 versus los meses equivalentes del año previo. Se analizaron la incidencia, la clínica, el estadio, el abordajequirúrgico y las complicaciones. Resultados. Los casos totales de apendicitisse redujeron un 25 % (n = 67 versus n = 50 en 2020). El tiempo medio hasta la consulta fue de 24 horas en ambos períodos (p = 0,989). La incidencia de peritonitis fue del 44 % (n = 22) versus el 37 % (n = 22) (p = 0,22) en 2019. No se evidenció diferencia en los estadios deenfermedad de acuerdo con lo informado en los partes quirúrgicos. En 2019, todas las cirugías se realizaron por vía laparoscópica; en 2020, solo un42 % (n = 21). La incidencia de complicaciones fue del 6 %, contra 7,5 % en el período previo (p = 0,75). Un paciente fue COVID-19 positivo. Conclusión. A pesar de la reducción en el númerode casos de apendicitis, no se evidenció una demora en la consulta en nuestra población. El mayor impacto se asoció a la readecuación del manejo, evitando el abordaje laparoscópico para reducir la diseminación del virus.


Introduction. Appendicitis is the leading cause of surgical acute abdomen in pediatrics. During the COVID-19 pandemic, management strategies were reassessed and the number of visits to the emergency department dropped down, which may be associated with delayed diagnoses and complications. The objective of this study was to analyze the impact of the pandemic on children with acute appendicitis. Methods. Analytical, retrospective, comparative study of pediatric patients with acute appendicitis in the 5 months of COVID-19 lockdown versus the same period in the previous year. Incidence, clinical data, stage, surgical approach, and complications were analyzed. Results. The total number of appendicitis cases went down by 25 % (n = 67 versus n = 50 in 2020). The mean time to consultation was 24 hours in both periods (p = 0.989). The incidence of peritonitis was 44 % (n = 22) versus 37 % (n = 22) (p = 0.22) in 2019. No differences were  observed in terms of appendicitis stage based on surgery reports. In 2019, all surgeries were laparoscopic; while in 2020, only 42 % (n = 21). The incidence of complications was 6 % versus 7.5 % in the previous period (p = 0.75). One patient was COVID-19 positive. Conclusion. Although in our population the number of appendicitis cases dropped down, consultation was not delayed. The greater impact was associated with the reformulation of management strategies, in which the laparoscopic approach is avoided to reduce virus transmission.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Apendicectomia/tendências , Apendicite/cirurgia , Apendicite/diagnóstico , Apendicite/epidemiologia , Padrões de Prática Médica/tendências , Diagnóstico Tardio/tendências , COVID-19/prevenção & controle , Acesso aos Serviços de Saúde/tendências , Apendicectomia/métodos , Argentina/epidemiologia , Doença Aguda , Incidência , Estudos Retrospectivos , Laparoscopia/tendências , Pandemias/prevenção & controle , Centros de Atenção Terciária , COVID-19/diagnóstico , COVID-19/epidemiologia , Hospitais Gerais
8.
Arch Argent Pediatr ; 119(4): 224-229, 2021 08.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34309297

RESUMO

INTRODUCTION: Appendicitis is the leading cause of surgical acute abdomen in pediatrics. During the COVID-19 pandemic, management strategies were reassessed and the number of visits to the emergency department dropped down, which may be associated with delayed diagnoses and complications. The objective of this study was to analyze the impact of the pandemic on children with acute appendicitis. METHODS: Analytical, retrospective, comparative study of pediatric patients with acute appendicitis in the 5 months of COVID-19 lockdown versus the same period in the previous year. Incidence, clinical data, stage, surgical approach, and complications were analyzed. RESULTS: The total number of appendicitis cases went down by 25% (n = 67 versus n = 50 in 2020). The mean time to consultation was 24 hours in both periods (p = 0.989). The incidence of peritonitis was 44% (n = 22) versus 37% (n = 22) (p = 0.22) in 2019. No differences were observed in terms of appendicitis stage based on surgery reports. In 2019, all surgeries were laparoscopic; while in 2020, only 42% (n = 21). The incidence of complications was 6% versus 7.5% in the previous period (p = 0.75). One patient was COVID-19 positive. CONCLUSION: Although in our population the number of appendicitis cases dropped down, consultation was not delayed. The greater impact was associated with the reformulation of management strategies, in which the laparoscopic approach is avoided to reduce virus transmission.


Introducción. La apendicitis constituye la principal causa de abdomen agudo quirúrgico en pediatría. Durante la pandemia por COVID-19, se replantearon las estrategias de manejo y disminuyeron las consultas en las guardias, lo que podría asociarse a diagnósticos tardíos y complicaciones. El objetivo de este estudio fue analizar el impacto de la pandemia en los niños con apendicitis aguda. Métodos. Estudio analítico retrospectivo comparativo de pacientes pediátricos con apendicitis aguda durante los cinco meses del confinamiento por COVID-19 versus los meses equivalentes del año previo. Se analizaron la incidencia, la clínica, el estadio, el abordaje quirúrgico y las complicaciones. Resultados. Los casos totales de apendicitis se redujeron un 25% (n = 67 versus n = 50 en 2020). El tiempo medio hasta la consulta fue de 24 horas en ambos períodos (p = 0,989). La incidencia de peritonitis fue del 44% (n = 22) versus el 37% (n = 22) (p = 0,22) en 2019. No se evidenció diferencia en los estadios de enfermedad de acuerdo con lo informado en los partes quirúrgicos. En 2019, todas las cirugías se realizaron por vía laparoscópica; en 2020, solo un 42% (n = 21). La incidencia de complicaciones fue del 6%, contra 7,5% en el período previo (p = 0,75). Un paciente fue COVID-19 positivo. Conclusión. A pesar de la reducción en el número de casos de apendicitis, no se evidenció una demora en la consulta en nuestra población. El mayor impacto se asoció a la readecuación del manejo, evitando el abordaje laparoscópico para reducir la diseminación del virus.


Assuntos
Apendicectomia/tendências , Apendicite , COVID-19/prevenção & controle , Diagnóstico Tardio/tendências , Acesso aos Serviços de Saúde/tendências , Padrões de Prática Médica/tendências , Doença Aguda , Adolescente , Apendicectomia/métodos , Apendicite/diagnóstico , Apendicite/epidemiologia , Apendicite/cirurgia , Argentina/epidemiologia , COVID-19/diagnóstico , COVID-19/epidemiologia , Criança , Pré-Escolar , Feminino , Hospitais Gerais , Humanos , Incidência , Lactente , Recém-Nascido , Laparoscopia/tendências , Masculino , Pandemias/prevenção & controle , Estudos Retrospectivos , Centros de Atenção Terciária
9.
Pediatr Surg Int ; 37(10): 1415-1420, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34272598

RESUMO

PURPOSE: To analyze the initial impact of the SARS-CoV-2 pandemic on surgical skills training and performance of Pediatric Surgery Residents. METHODS: Retrospective study considering the modifications on the Pediatric Surgery Residency training from March 1st-May 31st, 2020. Exposure to OR learning opportunities was compared to the same 2018-2019 trimesters. An anonymous survey about self-perception on surgical skills development was also performed. RESULTS: Residents performed 209 procedures as leading surgeons during the 2020 trimester with a mean number of surgeries per resident of 20.9, representing a reduction of 46% and 56.8% compared to the 2018-2019 averages, respectively. Reduction in both the number and the percentage of total procedures (n: 209, 56.8%) compared to both 2019 (n: 354, 68.7%, p: 0.000272) and 2018 (n: 420, 76.1%, p < 0,00,001) showed statistical correlation with no changes in their complexity pattern. From the survey (response rate: 100%), hours dedicated to simulation-based training were highly increased. More time was spent studying, but only 60% achieved better preparation for surgery and 70% perceived a decrease in surgical confidence. CONCLUSIONS: Even though the pandemic promoted new teaching strategies and the use of simulation-based training, it drastically reduced "on-the-job" learning opportunities with potential effects on residents' performance and self-confidence during surgery.


Assuntos
COVID-19/prevenção & controle , Cirurgia Geral/educação , Internato e Residência/organização & administração , Pandemias , COVID-19/epidemiologia , COVID-19/psicologia , Criança , Competência Clínica , Humanos , Pandemias/prevenção & controle , Estudos Retrospectivos , SARS-CoV-2 , Inquéritos e Questionários
10.
Pediatr Transplant ; 25(8): e14105, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34328249

RESUMO

BACKGROUND: Enterocutaneous fistula (ECF) is a serious and complex problem when affecting children, being responsible for a high morbidity burden, with an estimated mortality rate of 10 to 20%. There are many therapeutic options, including surgery and a wide variety of nonoperative strategies. Prognosis of ECF closure depends on the output and also on the patency of distal bowel. Spontaneous closure without operative intervention occurs in approximately 50% of patients with lateral ECF and distal bowel transit, but this drastically decreases in high output fistulas. High-volume fistula output and consequent skin damage are a great challenge for the health-care team. METHODS: We describe a postoperative complication that required a new nonoperative technique for the transient management of a lateral high-output ECF, involving the insertion of an occlusive device in order to redirect intestinal content to the distal bowel, reducing the fistula output. RESULTS AND CONCLUSIONS: The main benefit of this nonoperative technique is the ability to occlude a high-output fistula, allowing the distal flow to be restored and reducing abdominal wall damage, as a bridge to definitive surgical closure.


Assuntos
Fístula Intestinal/cirurgia , Intestino Delgado/transplante , Complicações Pós-Operatórias/cirurgia , Síndrome do Intestino Curto/cirurgia , Criança , Drenagem/instrumentação , Desenho de Equipamento , Humanos , Fístula Intestinal/etiologia , Masculino , Complicações Pós-Operatórias/etiologia
11.
Arch. argent. pediatr ; 119(5): e513-e517, oct. 2021. tab, ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1292683

RESUMO

La enfermedad de injerto contra huésped es una complicación grave que se presenta después del trasplante de médula ósea, con morbilidad y mortalidad elevadas. El patrón de oro para evaluar su compromiso gastrointestinal es la endoscopia digestiva alta y baja con toma de biopsia. El desarrollo de hematoma duodenal intramural es una complicación poco frecuente asociada con este procedimiento .Se presentan dos casos de hematoma duodenal intramural posendoscopia en pacientes con trasplante y sospecha de enfermedad injerto contra huésped que presentaron un cuadro agudo de dolor abdominal y sangrado intestinal. El diagnóstico se realizó por tomografía y recibieron tratamiento conservador, con un resultado favorable. En ambos casos, el diagnóstico de enfermedad injerto contra huésped gastrointestinal se hizo a través de las biopsias colónicas con histología duodenal normal, lo que sugiere evitar la toma de muestras duodenales para prevenir esta grave complicación en pacientes de alto riesgo y, de este modo, disminuir la morbilidad.


Graft versus host disease is a serious complication that occurs following bone marrow transplant with significant morbidity and mortality. The gold standard to diagnose gastrointestinal graft versus host disease is upper and lower gastrointestinal endoscopy with histological validation. The development of intramural duodenal hematoma is a rare complication associated with this procedure. We present two cases of intramural duodenal haematoma after duodenal biopsies in bone marrow transplant patients that presented clinically with severe abdominal pain and intestinal bleeding. In both cases, CT scans confirmed the diagnosis and they were treated conservatively with favorable outcomes.Final diagnosis of gastrointestinal graft versus host disease was based on the colonic samples with normal duodenal histoarchitecture, which could lead to avoiding duodenal samples in future patients in order to prevent this serious complication and thus diminish morbidity.


Assuntos
Humanos , Masculino , Lactente , Criança , Duodenopatias/diagnóstico , Duodenopatias/etiologia , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/etiologia , Endoscopia Gastrointestinal , Hematoma/diagnóstico , Hematoma/etiologia , Hemorragia Gastrointestinal
12.
Pediatr Surg Int ; 37(5): 607-616, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33454815

RESUMO

PURPOSE: Although surgical therapy for testicular tumors (TT) is often radical orchidectomy, tumor resection with preservation of healthy testicular parenchyma has been proposed. This study herein reports a 20 year single center experience applying testicular sparing surgery (TSS) as a primary operative strategy in pediatric patients. A systematic literature review summarizes the utility and outcomes of TSS in appropriately selected patients. METHODS: Pediatric patients with TT who underwent TSS between 1997 and 2018 were studied. TSS was indicated if patients presented evidence of adequately spared healthy testicular parenchyma on preoperative ultrasound and negative serum tumor markers. A systematic review of the literature was also performed. RESULTS: 12 cases met full inclusion criteria with 10 of 12 subjects in the prepubertal age group. Follow-up was 73 months (range 18-278 months). Only a single male patient (GSCCT) presented with early recurrence and orchidectomy was then performed. No cases of postoperative testicular atrophy were identified. Sexual maturation (Tanner stage) expected for age in each patient was documented. Review of the literature identified 34 published studies including 269 patients (94% prepubertal). Pathologic lesions here were mainly mature teratoma(s)-(62%) with a follow-up period of 4 years. Recurrent tumors were observed in only three patients (1.1%) notably two Leydig Cell Tumors and one Teratoma. Testicular atrophy reportedly occurred in only one single case (0.37%). DISCUSSION: TSS is a feasible alternative to radical orchidectomy in pediatric male patients with localized TT and negative tumor markers. Long term follow-up is essential to monitor testicular growth, puberty with sexual development and psychological male health.


Assuntos
Tumor de Células de Leydig/cirurgia , Neoplasias Testiculares/cirurgia , Adolescente , Biomarcadores Tumorais , Criança , Pré-Escolar , Humanos , Lactente , Tumor de Células de Leydig/patologia , Masculino , Recidiva Local de Neoplasia/cirurgia , Orquiectomia , Teratoma/cirurgia , Ultrassonografia
13.
Pediatrics ; 144(2)2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31366686

RESUMO

Juvenile polyposis syndrome is a rare autosomal dominant condition characterized by multiple hamartomatous polyps throughout the gastrointestinal tract. Juvenile polyposis of infancy is a generalized severe form of juvenile polyposis syndrome associated with a poor prognosis. A 47-month-old female infant presented initially with gastrointestinal bleeding and protein-losing enteropathy at 4 months of age. At the age of 12 months, the condition worsened, requiring albumin infusions every 24 to 48 hours and red blood cell transfusions every 15 days. Upper gastrointestinal endoscopy, colonoscopy, and small-bowel enteroscopy revealed diffuse polyposis that was treated with multiple endoscopic polypectomies. Despite subtotal colectomy with ileorectal anastomosis, protein-losing enteropathy and bleeding persisted, requiring continued blood transfusions and albumin infusions. A chromosomal microarray revealed a single allele deletion in chromosome 10q23, involving both the PTEN and BMPR1A genes. Loss of PTEN function is associated with an increased activation of the protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway involved in cell proliferation. Treatment with sirolimus, an mTOR inhibitor, was initiated with the aim of inhibiting polyp growth. Soon after initiation of treatment with sirolimus, blood and albumin infusions were no longer needed and resulted in improved patient growth and quality of life. This case represents the first detailed report of successful drug therapy for life-threatening juvenile polyposis of infancy.


Assuntos
Imunossupressores/uso terapêutico , Polipose Intestinal/congênito , Síndromes Neoplásicas Hereditárias/diagnóstico , Síndromes Neoplásicas Hereditárias/tratamento farmacológico , Sirolimo/uso terapêutico , Pré-Escolar , Feminino , Seguimentos , Humanos , Polipose Intestinal/diagnóstico , Polipose Intestinal/tratamento farmacológico , Polipose Intestinal/cirurgia , Síndromes Neoplásicas Hereditárias/cirurgia , Resultado do Tratamento
14.
Arch. argent. pediatr ; 117(3): 301-304, jun. 2019. ilus, tab
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1001207

RESUMO

El schwannoma es un tumor benigno originado de las células de Schwann y puede producirse a lo largo de cualquier nervio en el que estas células formen parte de su vaina. Los schwannomas del nervio facial extratemporales son infrecuentes y se presentan como masas indoloras en la región parotídea, de lento crecimiento y con compromiso del nervio facial. Se los debe tener en cuenta como diagnóstico diferencial en masas parotídeas en los niños, aunque sean raros. La utilización de la punción aspirativa con aguja fina y la resonancia magnética nuclear evidencia la mejor aproximación diagnóstica. El tratamiento quirúrgico de elección en la localización intraparotídea es la parotidectomía superficial. Otra opción es la tumorectomía completa con electroestimulación intraoperatoria y preservación del nervio facial. Se presenta un caso de schwannoma intraparotídeo en una paciente pediátrica operada con esta última técnica.


Schwannomas are benign tumors which arise from Schwann cells and take place along peripheral nerves. Extra-temporal facial nerve schwannomas are infrequent and present as painless masses in the parotid region, slow-growing and involvement of the facial nerve. Although rare, they should be taken into account as a differential diagnosis in parotid masses in children. Surgical strategies include superficial parotidectomy and surgical tumor resection with electrical nerve stimulation and nerve preservation. The following case describes a paediatric patient treated with the previously mentioned nerve sparing surgical technique.


Assuntos
Humanos , Feminino , Adolescente , Glândula Parótida , Pediatria , Nervo Facial , Neoplasias , Neurilemoma
15.
Arch Argent Pediatr ; 117(3): e301-e304, 2019 06 01.
Artigo em Espanhol | MEDLINE | ID: mdl-31063322

RESUMO

Schwannomas are benign tumors which arise from Schwann cells and take place along peripheral nerves. Extra-temporal facial nerve schwannomas are infrequent and present as painless masses in the parotid region, slow-growing and involvement of the facial nerve. Although rare, they should be taken into account as a differential diagnosis in parotid masses in children. Surgical strategies include superficial parotidectomy and surgical tumor resection with electrical nerve stimulation and nerve preservation. The following case describes a paediatric patient treated with the previously mentioned nerve sparing surgical technique.


El schwannoma es un tumor benigno originado de las células de Schwann y puede producirse a lo largo de cualquier nervio en el que estas células formen parte de su vaina. Los schwannomas del nervio facial extratemporales son infrecuentes y se presentan como masas indoloras en la región parotídea, de lento crecimiento y con compromiso del nervio facial. Se los debe tener en cuenta como diagnóstico diferencial en masas parotídeas en los niños, aunque sean raros. La utilización de la punción aspirativa con aguja fina y la resonancia magnética nuclear evidencia la mejor aproximación diagnóstica. El tratamiento quirúrgico de elección en la localización intraparotídea es la parotidectomía superficial. Otra opción es la tumorectomía completa con electroestimulación intraoperatoria y preservación del nervio facial. Se presenta un caso de schwannoma intraparotídeo en una paciente pediátrica operada con esta última técnica.


Assuntos
Neoplasias dos Nervos Cranianos/diagnóstico , Nervo Facial/patologia , Neurilemoma/diagnóstico , Neoplasias Parotídeas/diagnóstico , Adolescente , Neoplasias dos Nervos Cranianos/patologia , Neoplasias dos Nervos Cranianos/cirurgia , Diagnóstico Diferencial , Nervo Facial/cirurgia , Feminino , Humanos , Neurilemoma/patologia , Neurilemoma/cirurgia , Neoplasias Parotídeas/patologia , Neoplasias Parotídeas/cirurgia
16.
Arch. argent. pediatr ; 116(6): 778-781, dic. 2018. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-973697

RESUMO

Las lesiones hepáticas y de las vías biliares por causa traumática son poco usuales en la edad pediátrica. Del total de pacientes con trauma abdominal cerrado, menos del 9 % presentan lesión hepática, y la frecuencia varía entre el 2 % y el 3 % de la lesión de las vías biliares. Actualmente, el tratamiento recomendado para el trauma abdominal cerrado con lesión hepática sin repercusión hemodinámica es conservador; en caso de presentar lesión de la vía biliar intra- o extrahepática, los abordajes de preferencia son mínimamente invasivos, como la cirugía percutánea o endoscópica, y se utiliza la laparotomía en casos seleccionados. Se presenta el caso de un paciente con trauma abdominal cerrado y trauma hepático, inicialmente laparotomizado en 2 ocasiones por inestabilidad hemodinámica y hemoperitoneo; presentó un bilioma subdiafragmático y coleperitoneo, en el que se realizó un manejo mínimamente invasivo por drenaje percutáneo bajo control tomográfico.


Hepatic and biliary tract injuries due to traumatic causes are rare in pediatric patients. Of the total number of patients with closed abdominal trauma, less than 9 % have liver injury, and the frequency varies between 2 and 3 % of biliary tract lesions. Currently, the recommended treatment for closed abdominal trauma with liver injury without hemodynamic repercussion is conservative. In case of presenting intra or extrahepatic biliary tract lesion, the preferred approaches are minimally invasive, such as percutaneous or endoscopic surgery, using laparotomy in selected cases. We present the case of a patient with closed abdominal trauma and liver trauma, initially laparotomized on 2 occasions due to hemodynamic instability and hemoperitoneum; presented a subdiaphragmatic and coleperitoneal bilioma; it was performed minimally invasive percutaneous drainage under tomographic control.


Assuntos
Humanos , Masculino , Pré-Escolar , Ferimentos não Penetrantes/terapia , Sistema Biliar/lesões , Drenagem/métodos , Traumatismos Abdominais/terapia , Ferimentos não Penetrantes/complicações , Tomografia Computadorizada por Raios X , Hemoperitônio/etiologia , Hemoperitônio/terapia , Laparotomia/métodos , Traumatismos Abdominais/complicações , Fígado/lesões
17.
Arch Argent Pediatr ; 116(6): e778-e781, 2018 12 01.
Artigo em Espanhol | MEDLINE | ID: mdl-30457736

RESUMO

Hepatic and biliary tract injuries due to traumatic causes are rare in pediatric patients. Of the total number of patients with closed abdominal trauma, less than 9 % have liver injury, and the frequency varies between 2 and 3 % of biliary tract lesions. Currently, the recommended treatment for closed abdominal trauma with liver injury without hemodynamic repercussion is conservative. In case of presenting intra or extrahepatic biliary tract lesion, the preferred approaches are minimally invasive, such as percutaneous or endoscopic surgery, using laparotomy in selected cases. We present the case of a patient with closed abdominal trauma and liver trauma, initially laparotomized on 2 occasions due to hemodynamic instability and hemoperitoneum; presented a subdiaphragmatic and coleperitoneal bilioma; it was performed minimally invasive percutaneous drainage under tomographic control.


Las lesiones hepáticas y de las vías biliares por causa traumática son poco usuales en la edad pediátrica. Del total de pacientes con trauma abdominal cerrado, menos del 9 % presentan lesión hepática, y la frecuencia varía entre el 2 % y el 3 % de la lesión de las vías biliares. Actualmente, el tratamiento recomendado para el trauma abdominal cerrado con lesión hepática sin repercusión hemodinámica es conservador; en caso de presentar lesión de la vía biliar intra- o extrahepática, los abordajes de preferencia son mínimamente invasivos, como la cirugía percutánea o endoscópica, y se utiliza la laparotomía en casos seleccionados. Se presenta el caso de un paciente con trauma abdominal cerrado y trauma hepático, inicialmente laparotomizado en 2 ocasiones por inestabilidad hemodinámica y hemoperitoneo; presentó un bilioma subdiafragmático y coleperitoneo, en el que se realizó un manejo mínimamente invasivo por drenaje percutáneo bajo control tomográfico.


Assuntos
Traumatismos Abdominais/terapia , Sistema Biliar/lesões , Drenagem/métodos , Ferimentos não Penetrantes/terapia , Traumatismos Abdominais/complicações , Pré-Escolar , Hemoperitônio/etiologia , Hemoperitônio/terapia , Humanos , Laparotomia/métodos , Fígado/lesões , Masculino , Tomografia Computadorizada por Raios X , Ferimentos não Penetrantes/complicações
18.
Arch. argent. pediatr ; 116(4): 621-625, ago. 2018. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-950055

RESUMO

La vena cava inferior (VCI) está constituida por tres segmentos de diferente origen embriológico. De su mala fusión, surge un amplio espectro de anomalías. La prevalencia de anomalías de la VCI es de 0,07-8,7% de la población. Generalmente, se diagnostica como hallazgo incidental en la vida adulta. Representa el 5-9,5% de las trombosis venosas profundas idiopáticas en menores de 30 años sin factores de riesgo asociados. Se presenta a una recién nacida a término con diagnóstico prenatal de ascitis en la semana 20 de gestación. Se diagnosticó, mediante angiotomografía abdominal, la agenesia de VCI. El tratamiento de pacientes con agenesia de la VCI se basa en el manejo de las complicaciones. Debido al mayor riesgo que presentan de sufrir un evento trombótico, se debe considerar la profilaxis antitrombótica a largo plazo. Se recomienda iniciar profilaxis anticoagulante en la pubertad.


Inferior Vena Cava (IVC) is composed of three segments from different embryological origin. Its lack of fusion originates a wide spectrum of anomalies of the IVC. These malformations are present in 0.07-8.7% of the population. It is generally diagnosed as an incidental finding in adult life. It represents between 5 and 9.5% of idiopathic deep vein thrombosis in patients younger than 30 years old without associated risk factors. We present a case of a term newborn with prenatal diagnosis of ascites during the 20th week of gestation. IVC Agenesis was diagnosed with the use of abdominal angiotomography. The treatment of patients with IVC Agenesis is based on the management of its complications. Due to the increased thrombotic risk of these patients, we should consider lifelong anticoagulation. We suggest initiating it during puberty.


Assuntos
Humanos , Feminino , Recém-Nascido , Diagnóstico Pré-Natal/métodos , Ascite/etiologia , Veia Cava Inferior/anormalidades , Veia Cava Inferior/diagnóstico por imagem , Gravidez , Angiografia por Tomografia Computadorizada/métodos
19.
Arch Argent Pediatr ; 116(4): e621-e625, 2018 08 01.
Artigo em Espanhol | MEDLINE | ID: mdl-30016044

RESUMO

Inferior Vena Cava (IVC) is composed of three segments from different embryological origin. Its lack of fusion originates a wide spectrum of anomalies of the IVC. These malformations are present in 0.07-8.7% of the population. It is generally diagnosed as an incidental finding in adult life. It represents between 5 and 9.5% of idiopathic deep vein thrombosis in patients younger than 30 years old without associated risk factors. We present a case of a term newborn with prenatal diagnosis of ascites during the 20th week of gestation. IVC Agenesis was diagnosed with the use of abdominal angiotomography. The treatment of patients with IVC Agenesis is based on the management of its complications. Due to the increased thrombotic risk of these patients, we should consider lifelong anticoagulation. We suggest initiating it during puberty.


La vena cava inferior (VCI) está constituida por tres segmentos de diferente origen embriológico. De su mala fusión, surge un amplio espectro de anomalías. La prevalencia de anomalías de la VCI es de 0,07-8,7% de la población. Generalmente, se diagnostica como hallazgo incidental en la vida adulta. Representa el 5-9,5% de las trombosis venosas profundas idiopáticas en menores de 30 años sin factores de riesgo asociados. Se presenta a una recién nacida a término con diagnóstico prenatal de ascitis en la semana 20 de gestación. Se diagnosticó, mediante angiotomografía abdominal, la agenesia de VCI. El tratamiento de pacientes con agenesia de la VCI se basa en el manejo de las complicaciones. Debido al mayor riesgo que presentan de sufrir un evento trombótico, se debe considerar la profilaxis antitrombótica a largo plazo. Se recomienda iniciar profilaxis anticoagulante en la pubertad.


Assuntos
Ascite/etiologia , Diagnóstico Pré-Natal/métodos , Veia Cava Inferior/anormalidades , Angiografia por Tomografia Computadorizada/métodos , Feminino , Humanos , Recém-Nascido , Gravidez , Veia Cava Inferior/diagnóstico por imagem
20.
Arch. argent. pediatr ; 116(2): 292-295, abr. 2018. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-887471

RESUMO

La ruptura diafragmática es una entidad relativamente infrecuente en pediatría que se puede presentar como consecuencia de un traumatismo de alto impacto. Solo entre el 25% y el 50% de los casos es detectado en la evaluación inicial del paciente, lo que aumenta el riesgo de complicaciones. En este trabajo, se presenta el caso de una paciente asintomática, de 8 años de edad, que, tras un accidente vehicular y la evaluación inicial en otro centro, fue derivada a nuestra Institución para el control evolutivo. A las 36 horas del ingreso, presentó hipoventilación pulmonar izquierda. La tomografía demostró un ascenso intratorácico del ángulo esplénico del colon y asas de intestino delgado. Se efectuó la exploración y el tratamiento quirúrgico. Los controles radiográficos diferidos no mostraron signos de recidiva.


Diaphragmatic rupture is a relatively uncommon entity in pediatrics that can occur as a result of a high-impact trauma. Only between 25 and 50% of the cases are detected in the initial evaluation of the patient, which increases the risk of complications. This paper presents the case of an asymptomatic 8-year-old patient who was referred to our institution after a vehicular accident. A day and a half after admission, a left pulmonary hypoventilation was detected. Computed tomography scan showed intrathoracic elevation of the splenic angle of the colon and the small bowel. Surgical exploration and treatment were performed. Deferred radiographic controls showed no signs of relapse.


Assuntos
Humanos , Masculino , Criança , Diafragma/lesões , Ruptura/diagnóstico por imagem , Diafragma/diagnóstico por imagem
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