International Society of Paediatric Oncology (SIOP) Global Mapping Programme: Latin American Society of Pediatric Oncology (SLAOP) country-level report.

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.

Laparoscopic approach of pediatric adrenal tumors.

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.

A novel technique for large lateral enterocutaneous fistula management after intestinal transplantation.

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.

Testis-sparing surgery for testicular tumors in children: a 20 year single center experience and systematic review of the literature.

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.

Impact of the COVID-19 pandemic on surgical skills training in pediatric surgery residents.

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.

Childhood Neuroblastoma: Incidence and Survival in Argentina. Report from the National Pediatric Cancer Registry, ROHA Network 2000-2012.

BACKGROUND: There are reports indicating a low incidence of neuroblastoma (NB) in some developing countries but no conclusive data are available from population-based studies at a national level. PURPOSE: To describe the incidence and survival of 971 patients with NB in Argentina with data from the National Pediatric Cancer Registry (ROHA), and the impact of age, gender, stage, regional, and socioeconomic indicators on outcome. METHODS: All cases of NB reported to ROHA (2000-2012) were the subject of the analysis. Annual-standardized incidence rate (ASR) was calculated using the National Vital Statistics and survival was estimated. The extended human development index (EHDI) was used as the socioeconomic indicator. RESULTS: ASR was 8.3/1,000,000 children (0-14 years) and remained stable along this period. Regional variation in ASR ranged from 3.4 in the Northwest to 9.8 in the Central region, being most marked in the first year of life. Five-year survival rate (SR) was 47%, with no sex difference. For patients older than 18 months, it was 36%, for stage IV 23%, for those born in the Northeast region 38%, and for those with an amplified MYCN 15%. Residents in provinces with a higher EHDI had a better 5-year survival (57% vs. 41% for lower EHDI) and higher ASR (12.3 vs. 5.6 for lower EHDI). Stage and MYCN status showed an independent inferior prognosis. CONCLUSIONS: ASR of NB in Argentina is lower than in developed countries, with considerable regional variation. SRs are also lower than in developed countries.

[Osteosarcoma lung metastases. Survival after chemotherapy and surgery]. / Metástasis pulmonares en osteosarcoma. Neoadyuvancia, tratamiento quirúrgico y supervivencia.

Five years overall survival in osteosarcoma patients is around 70%, although in patients with metastatic disease it is only 10-30%. The objective of this study was to analyze overall survival and prognostic factors in a group of patients with metastatic osteosarcoma treated with surgical removal of the lung metastases. A retrospective review from our oncology data base revealed 38 patients treated between 1992 and 2006. The mean age at diagnosis was 18 ± 9.4 years (3-45) and mean follow-up was 57 ± 53.8 months (12-231). All patients were treated with chemotherapy and oncologic resection of the primary tumor and surgical removal of the lung metastases. We analyzed overall survival and prognostic factors: age, gender, site, time of metastasis, local recurrences, number of lung metastasis and chemotherapy response (necrosis). Overall survival of the entire series was 29% at 5 years (CI 95%: 14.5-43.5) and 26% at 10 years (CI 95%: 12-40). Significant difference in 5 year overall survival was found between good and bad responders to chemotherapy, 53% (IC 95%: 28-78) vs. 8% (IC 95%: 0-20) (p = 0.0008). No statistically significant relationship between other prognostic factors analyzed was observed. Five and ten years overall survival rates in osteosarcoma patients with lung metastasis treated with chemotherapy and surgically resection is poor. Patients with good response to chemotherapy have better prognosis.

International Society of Paediatric Surgical Oncology (IPSO): A Short History, Developments, and Current Status.

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.

Major surgical conditions of childhood and their lifelong implications: comprehensive review.

BACKGROUND: In recent decades, the survival of children with congenital anomalies and paediatric cancer has improved dramatically such that there has been a steady shift towards understanding their lifelong health outcomes. Paediatric surgeons will actively manage such conditions in childhood and adolescence, however, adult surgeons must later care for these 'grown-ups' in adulthood. This article aims to highlight some of those rare disorders encountered by paediatric surgeons requiring long-term follow-up, their management in childhood and their survivorship impact, in order that the adult specialist may be better equipped with skills and knowledge to manage these patients into adulthood. METHODS: A comprehensive literature review was performed to identify relevant publications. Research studies, review articles and guidelines were sought, focusing on the paediatric management and long-term outcomes of surgical conditions of childhood. The article has been written for adult surgeon readership. RESULTS: This article describes the aforementioned conditions, their management in childhood and their lifelong implications, including: oesophageal atresia, tracheo-oesophageal fistula, malrotation, short bowel syndrome, duodenal atresia, gastroschisis, exomphalos, choledochal malformations, biliary atresia, Hirschsprung disease, anorectal malformations, congenital diaphragmatic hernia, congenital lung lesions and paediatric cancer. CONCLUSION: The increasing survivorship of children affected by surgical conditions will translate into a growing population of adults with lifelong conditions and specialist healthcare needs. The importance of transition from childhood to adulthood is becoming realized. It is hoped that this timely review will enthuse the readership to offer care for such vulnerable patients, and to collaborate with paediatric surgeons in providing successful and seamless transitional care.

Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with embolization and vincristine in two newborns.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor and has a high mortality in newborns when associated with Kasabach-Merritt syndrome (KMS). In two newborns with KHE and severe KMS refractory to medical treatment, emergency embolization led to clinical improvement in the acute neonatal setting by reducing tumor volume, increasing the platelet count, and improving other clotting parameters. Systemic vincristine treatment was added for further tumor control. Both patients remained symptom-free at long-term follow-up.

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines.

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.

Thyroid cancer in children: A multicenter international study highlighting clinical features and surgical outcomes of primary and secondary tumors.

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%).

Choledochal cyst in pediatric patients: a 10-year single institution experience.

BACKGROUND: Choledochal cysts (CCs) are rare congenital dilatations of the biliar tree. The incidence is 1:150,000. A correct diagnosis and surgical treatment are important because of the long term risks of infection and neoplasia. We report our experience with CCs in children. PATIENTS AND METHODS: Patients with CC disease, who were seen at the Hospital Italiano de Buenos Aires between 1999 and 2009, were identified retrospectively from our prospectively acquired hepato-pancreatico-biliary database. Prenatal, clinical, surgical and pathological records were analysed. RESULTS: We included 12 children (10 girls and 2 boys), all of them affected by a cystic dilatation type I of Todani classification. Between the 4 patients with antenatal diagnosis only one presented symptoms before surgery, expressed in acolic feces. Between the 8 patients with postnatal diagnosis, the most common clinical presentation was abdominal pain, occurring in all patients. Ultrasound was used for the diagnosis of all patients. Mean age at surgery was 74 months. All patients had intraoperative cholangiograms to definitively confirm the diagnosis. Five patients underwent open surgery and 7 laparoscopic approach. From patients with a laparoscopic approach, 1 had to be converted to open surgery due to intraoperative complications and 2 underwent an initial surgery for a cholecystectomy and a CC cyst excision. The complications observed were: anastomotic biliary fistula 1, residual lithiasis in the pancreatic portion of the duct 1 and anastomotic stenosis 1. The last 2 cases required reoperation. CONCLUSIONS: Once CCs are diagnosed, careful treatment decisions need to be taken because both intra hepatic and extra hepatic CCs, if untreated, have an increased risk of cancer. After a careful study of the cyst's characteristics, we recommend a laparoscopic approach when possible, as the best choice, after 3 months of life to increase the success of the treatment according to the size of the anatomic structure. We recommend early treatment, before 3 months of life, only in case of severe symptoms.

[Duodenal hematoma after upper gastrointestinal endoscopy in pediatric graft vs host disease: Report of two cases]. / Hematoma duodenal posvideoendoscopia digestiva alta para diagnóstico de enfermedad de injerto contra huésped en pediatría: a propósito de dos casos.

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.

Impact of the COVID-19 pandemic on the pediatric population with acute appendicitis: Experience at a general, tertiary care hospital. / Impacto de la pandemia por COVID-19 en la población pediátrica con apendicitis aguda: experiencia en un hospital general de tercer nivel.

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.

[Facial nerve schwannoma as differential diagnosis of parotid tumors in pediatrics]. / Schwannoma del nervio facial como diagnóstico diferencial en tumores de la región parotídea en pediatría.

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.

Successful Treatment of Juvenile Polyposis of Infancy With Sirolimus.

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.

[Fetal ascites as clinical presentation of inferior vena cava agenesis]. / Ascitis fetal como forma de presentación de agenesia de la vena cava inferior.

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.

[Minimally invasive treatment of biliary tract injury secondary to closed abdominal trauma: Clinical case]. / Tratamiento mínimamente invasivo de una lesión de la vía biliar secundaria a un traumatismo abdominal cerrado: Caso clínico.

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.

[Post traumatic diaphragmatic rupture in children: Case report]. / Ruptura diafragmatica postraumatica en pediatria: A proposito de un caso.

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.

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.