Development of a risk score for choledocholithiasis in pediatric patients.

PURPOSE: The prevalence of gallstones in children has increased over the last years. Choledocholithiasis (CD) is present in up to 30% of the cases. There is a scarcity of studies on the management of choledocholithiasis in children. The aim of this study was to develop a score that would allow predicting accurately the risk of CD in children with gallstones and reduce the number of non-therapeutic ERCP. MATERIALS AND METHODS: We conducted a retrospective study in children with gallstones and suspected CD seen between January 2010 and December 2019. The main outcome was the presence of CD confirmed by at least one of the following diagnostic tests: magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), and/or intraoperative cholangiography (IOC). We developed a risk score based on the presence or absence of the following risk factors: acute biliary pancreatitis, ascending cholangitis, elevated liver function tests (AST, ALT, total bilirubin [TB, ≥ 2 mg/dl], conjugated bilirubin, gamma-glutamyl transpeptidase, and alkaline phosphatase), CD on ultrasound (US; this was considered predictive but not confirmatory of CD), and dilation of the common bile duct (> 6 mm) by US. The score was divided into three different categories: low risk (no risk factors), intermediate risk (one risk factor present), high risk (≥ 2 risk factors present or ascending cholangitis). Given the main goal of reducing the number of diagnostic ERCPs, a very-high-risk subgroup (3 risk factors present or ascending cholangitis) was identified. RESULTS: We reviewed 133 patients with gallstones and suspected CD. In 56 (42.1%) patients, the presence of CD was confirmed by one or more of the definitive diagnostic tests (MRCP, ERCP, and IOC). The following variables were found to be the strongest predictors of CD: ascending cholangitis, TB ≥ 2 mg/dl, common bile duct > 6 mm, and the presence of CD by US. The positive predictive value for CD was 7.5% in the low-risk group (OR 0.06, P = < 0.001); 22.9% in the intermediate-risk group (OR 0.31, P = 0.007); 77.6% in the high-risk group (OR 20.14, P = < 0.001); and 95.7% in very-high-risk subgroup (OR 49.18, P = < 0.001). CONCLUSION: The risk score proposed in this study predicts accurately the presence of CD in children with gallstones. It can serve as a helpful tool to triage the need for costly and complex studies in the workup of CD, particularly in centers with limited resources. Finally, due to its high specificity and positive predictive value (PPV), the use of the very-high-risk criteria would allow for an important decrease in the number of non-therapeutic ERCP.

Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia.

PURPOSE: Biliary atresia is managed surgically by the Kasai portoenterostomy (KP). It has been reported by some groups that the outcomes of patients who have an early failed KP requiring a liver transplant (LTx) within the first year of life are worse than the outcomes of patients who undergo a primary LTx. The aim of this study was to identify preoperative parameters that could help predict what patients are at risk for the early failure of the procedure. MATERIALS AND METHODS: We conducted a retrospective chart review of all patients who underwent a KP between January 2008 and May 2018. The following preoperative parameters were analyzed: age at KP, anatomical variant of the biliary atresia, degree of liver fibrosis, CMV status, and PELD score. The main outcome of the study was the early failure of the KP (EF-K), which was defined as the need for LTx before 1 year of age, or BA-related death before 1 year of age. Second, we analyzed the risk factors associated with death without LTx within the first year of life. RESULTS: A total of 58 patients were included in the analysis. The native liver survival (NLS) was 56.5% and 48% at 1 and 5 years post KP, respectively. Overall survival (OS) was 79% and 76% at 1 and 5 years post KP, respectively. Early failure of KP occurred in 23 (39.7%) patients. OS in this group was 47% and 40% at 1 and 5 years, respectively. On the contrary, the OS of the remaining 35 (60.3%) patients was 100% at 1 and 5 years (P < 0.0001). When we compared all preoperative parameters, the only predictor of EF-K was the PELD score. When we analyzed the cases in the EF-K group who died without LTx, we found that the significant predictors were the cystic variant, a degree of liver fibrosis >4, and the PELD score. Nevertheless, on multivariate analysis, only PELD score was found as a statistically significant variable. CONCLUSION: Due to bad prognosis found in EF-K patients, we believe that it could be reasonable to offer them a primary LTx. PELD score was found to be the strongest preoperative parameter that allows predicting which patient will likely have an early failed KP. Further prospective and multicenter studies are needed to reinforce these results.

Hidatidosis en niños: experiencia en un hospital de alta complejidad fuera del área endémica / Echinococcosis in children: Experience in a tertiary care hospital outside the endemic area

La hidatidosis es una zoonosis con una amplia distribución en Argentina y en el mundo. Se adquiere en la infancia, pero se presenta, más frecuentemente, en la edad adulta. La población pediátrica representa el 10%-20% del total de los casos. Se presentan 47 niños con diagnóstico de hidatidosis durante un período de 20 años. La mediana de edad fue de 8 años (rango: 3-17) y, en su mayoría, tenían algún antecedente epidemiológico orientador, como la procedencia de un área endémica, rural y/o la tenencia de perros alimentados con visceras. El 85% fueron quistes únicos localizados en un solo parénquima. Las localizaciones quísticas pulmonares y hepáticas fueron las más frecuentemente encontradas. El tratamiento fue médico-quirúrgico en 45 pacientes (96%). Trece (28%) presentaron alguna complicación posterior, que fue más frecuente en aquellos con localización pulmonar. Tres (6%) recayeron entre el mes y los 24 meses, y requirieron una nueva cirugía. Ninguno falleció a causa de la enfermedad parasitaria.

Echinococcosis is a zoonotic disease that is widely spread across Argentina and worldwide. It is acquired during childhood but it is more common during adulthood. The pediatric population accounts for 10-20% of all cases. This study included 47 children diagnosed with echinococcosis over a 20-year period. Their median age was 8 years old (range: 3-17); most patients had some epidemiological history, such as having lived or living in an endemic or rural area and/or having dogs that are fed with viscera. Findings included 85% of single cysts in only one parenchyma. Cysts were most commonly located in the liver and lungs. Medical/surgical treatment was carried out in 45 patients (96%). Subsequent complications were observed in 13 patients (28%), which were more common in those with lung cysts. Three patients (6%) relapsed 1-24 months later and required a new surgery. No patient died as a result of the parasitic disease.

Echinococcosis in children: Experience in a tertiary care hospital outside the endemic area. / Hidatidosis en niños: experiencia en un hospital de alta complejidad fuera del área endémica.

Echinococcosis is a zoonotic disease that is widely spread across Argentina and worldwide. It is acquired during childhood but it is more common during adulthood. The pediatric population accounts for 10-20% of all cases. This study included 47 children diagnosed with echinococcosis over a 20-year period. Their median age was 8 years old (range: 3-17); most patients had some epidemiological history, such as having lived or living in an endemic or rural area and/ or having dogs that are fed with viscera. Findings included 85% of single cysts in only one parenchyma. Cysts were most commonly located in the liver and lungs. Medical/surgical treatment was carried out in 45 patients (96%). Subsequent complications were observed in 13 patients (28%), which were more common in those with lung cysts. Three patients (6%) relapsed 1-24 months later and required a new surgery. No patient died as a result of the parasitic disease.

La hidatidosis es una zoonosis con una amplia distribución en Argentina y en el mundo. Se adquiere en la infancia, pero se presenta, más frecuentemente, en la edad adulta. La población pediátrica representa el 10%-20% del total de los casos. Se presentan 47 niños con diagnóstico de hidatidosis durante un período de 20 años. La mediana de edad fue de 8 años (rango: 3-17) y, en su mayoría, tenían algún antecedente epidemiológico orientador, como la procedencia de un área endémica, rural y/o la tenencia de perros alimentados con visceras. El 85% fueron quistes únicos localizados en un solo parénquima. Las localizaciones quísticas pulmonares y hepáticas fueron las más frecuentemente encontradas. El tratamiento fue médico-quirúrgico en 45 pacientes (96%). Trece (28%) presentaron alguna complicación posterior, que fue más frecuente en aquellos con localización pulmonar. Tres (6%) recayeron entre el mes y los 24 meses, y requirieron una nueva cirugía. Ninguno falleció a causa de la enfermedad parasitaria.

Laparoscopic leak-free technique for the treatment of choledochal cysts.

OBJECTIVE: The aim of this report was to present our experience with a modified surgical technique designed for the treatment of choledochal cysts (CC) in children. MATERIALS AND METHODS: Between June 2004 and February 2005, we operated on 6 patients with a diagnosis of type I CC by means of a "leak-free" technique that consists of a transient complete sealing of the hepatic duct for the duration of the dissection, and a single- or double laparoscopic running suture to build the end-to-side hepatico-jejuno anastomosis. There were 4 females and 2 males, whose age ranged between 45 days and 7 years (median, 45 months). All cases were performed with three trocars plus the scope, and two or three percutaneous stay-stitches to retract the liver. The end-to-side hepatico-jejuno anastomoses were done with 5.0 or 6.0 PDS. We left no drains. RESULTS: The mean operative time was 335 minutes, and mean postoperative time to oral feeding was 44 hours. The mean hospital stay was 6 days (range, 5-10). No postoperative biliary leak was observed. A cosmetic result was excellent in all patients. In the follow-up (mean, 12 months), all patients were asymptomatic, had no intrahepatic biliary tree dilation, and had normal liver function tests. CONCLUSIONS: Based on the results of our series, we think that the laparoscopic approach is suitable for these patients, but some surgical details should be followed to lower the complication rate. First, a temporary closure of the hepatic duct to prevent bile spillage during the dissection is important for keeping the area clean and thus reducing the operative time. Second, the use of a running suture for the hepatico-jejunostomy, even though it may be technically challenging, should always be attempted to avoid postoperative bile leaks in these high-flow anastomoses.

Percutaneous cecostomy for management of fecal incontinence in pediatric patients.

PURPOSE: To report the authors' experience with percutaneous cecostomy and demonstrate its effectiveness in the management of pediatric patients with fecal incontinence. MATERIALS AND METHODS: Between March 2002 and November 2006, 21 percutaneous cecostomy procedures were performed in 20 patients in whom classical therapeutic approaches for the management of fecal incontinence had failed. Eighteen patients had anorectal malformations, one had myelomeningocele, and one had chronic constipation. All procedures were performed under general anesthesia and fluoroscopic guidance. In all cases, an 8.5-F Dawson-Mueller catheter was placed in the cecum and exchanged after 45 days with a cecostomy button (ie, Trapdoor catheter). Data regarding complications, effectiveness of treatment, satisfaction, and quality of life were obtained by interviewing the patients' parents at follow-up consultation. RESULTS: Nineteen of 20 procedures were technically successful. There were no major complications. All patients' symptoms of incontinence improved. Ninety percent of patients in our series (n = 18) reported satisfaction with the procedure, mainly related to their independence and quality of life. CONCLUSIONS: Percutaneous cecostomy is a safe and effective procedure for the management of pediatric patients with fecal incontinence. Percutaneous cecostomy allows antegrade and more physiologic colon irrigation, avoiding the need for multiple retrograde enemas, achieving more patient independence, and improving quality of life.

Bilhemia: a fatal complication following percutaneous placement of a transhepatic inferior vena cava catheter in a child.

A transhepatic central venous catheter was implanted in a 2-year-old child with a history of multiple venous access procedures and superior and inferior vena cava thrombosis. After 2 weeks, inadvertent dislodgement of the catheter was complicated by a biloma. The biloma was percutaneously drained, but a biliary-venous fistula led to a rapidly progressive and fatal bilhemia. We report this case as an infrequent complication of transhepatic catheterization.

Successful surgical treatment of a 4-month infant after exsanguination for aortoesophageal fistula.

We report on the case of a 4-month infant in hypovolemic shock for severe digestive hemorrhage 38 days after surgical treatment of a double aortic arch who underwent a successful emergency thoracotomy for suspicion of an aortoesophageal fistula.

Percutaneous treatment of osteoid osteoma by CT-guided drilling resection in pediatric patients.

BACKGROUND: Osteoid osteoma is a painful, benign, small osteogenic bone tumor. For a long time, surgery was the only treatment for these lesions. Different minimally invasive therapeutic techniques have been proposed. We report our experience in the treatment of osteoid osteoma by CT-guided drilling resection in pediatric patients. OBJECTIVE: To evaluate the efficacy of CT-guided percutaneous drilling resection as a minimally invasive therapy for osteoid osteoma in children. MATERIALS AND METHODS: Over a 5-year period, 18 patients (age range 6-17 years, mean age 11.6 years) with osteoid osteomas (femur, n=10; tibia, n=5; humerus, n=2; vertebral body, n=1) were treated with this technique. All procedures were performed under general anesthesia. RESULTS: All procedures were technically successful. Clinical success was achieved in 94.5% of patients (17/18). Only one patient had recurrence of symptoms 8 months after percutaneous resection and was surgically retreated. There were no complications. CONCLUSION: CT-guided percutaneous drilling resection is a safe, simple and effective minimally invasive technique for the treatment of osteoid osteoma in pediatric patients.

[Evolution of children one year post liver transplant]. / Evolución de niños post-trasplante hepático luego del primer año de sobrevida.

Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93% and 86% respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69%. De novo hepatitis B was diagnosed in 7 patients (4.8%). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.

Evolución de niños post-trasplante hepático luego del primer año de sobrevida. / [Evolution of children one year post liver transplant]

Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93

and 86

respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69

. De novo hepatitis B was diagnosed in 7 patients (4.8

). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.

Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device.

Two patients with symptomatic congenital portal vein (PV) fistulas (one case of PV-right hepatic vein fistula and one case of PV-inferior vena cava fistula) causing hepatopulmonary syndrome (hepatic dysfunction, lung vascular alteration in the form of arteriovenous shunts, and hypoxemia) were successfully treated with the Amplatzer patent ductus arteriosus (PDA) occlusion device. This device was chosen in light of the anatomic characteristics of the vascular malformations and the specific properties of the prosthesis, especially those related to relocation and retrievability when not properly positioned. Embolization with an Amplatzer PDA occlusion device should be considered as a useful therapeutic alternative in the treatment of congenital portal fistulas that can obviate complex surgeries.

Tumores pancreáticos en pediatría / Primary pancreatic neoplasms in children

Los tumores pancreáticos son infrecuentes en niños. Comunicamos los hallazgos de diagnóstico por imágenes observados en una serie de 9 niños tratados durante el período 1989-2000 con diferentes tipos tumorales (insulinoma, pancreatoblastoma, sólido-quístico-papilar y adenocarcinoma). Utilizamos ultrasonido, tomografía computada, aangiografía y resonancia magnética, que permitieron mostrar la masa tumoral en todos los casos, excepto en uno de insulinoma. No pudo determinarse la localización pancreática en un pancreatoblastoma. Las imágenes fueron de fundamental importancia para la estadificación y un abordaje quirúrgico adecuado para esta rara patología pediátrica (AU)

Tratamiento percutáneo de estenosis recurrente de la vena porta postransplante hepático en un paciente pediátrico, con angioplastía y colocación de stent / Percutaneous angioplasty of portal vein stenosis after hepatic transplantation in a child

Los autores presentan el caso de un paciente de 9 años de edad, receptor de un transplante hepático reducido, consecuencia de una hepatitis fulminante. A los nueve meses presentó repetidos episodios de hemorragia gastrointestinal y melena. La endoscopía colateral, con vena porta permeable y disminución de su calibre en el sitio de la anastomosis. Se realizó una portografía transhepática que confirmó la existencia de una estenosis significativa a nivel de la anastomosis porto-portal, dilatada con resultados satisfactorios. Luego de cuatro meses reaparecieron los síntomas. Una nueva angiografía transhepática demostró restenosis, que fue tratada con angioplastía por vía transhepática, con buenos resultados. Seis meses después, la recurrencia de los síntomas y de la estenosis, llevaron a la realización de una nueva dilatación, esta vez asociada a la colocación de un stent, expandible por balón, de tipo Palmaz. Todos los procedimientos fueron exitosos. No se registraron complicaciones relacionadas con los procedimientos. El seguimiento clínico, por laboratorio y ecografía Doppler, a dos años, no muestra recurrencia de la lesión ni signos de hipertensión portal. Las técnicas intervencionistas, constituyen una opción terapéutica segura y eficaz para el tratamiento de las complicaciones vasculares postransplante hepático (AU)

Tumores pancreáticos en pediatría / Primary pancreatic neoplasms in children

Los tumores pancreáticos son infrecuentes en niños. Comunicamos los hallazgos de diagnóstico por imágenes observados en una serie de 9 niños tratados durante el período 1989-2000 con diferentes tipos tumorales (insulinoma, pancreatoblastoma, sólido-quístico-papilar y adenocarcinoma). Utilizamos ultrasonido, tomografía computada, aangiografía y resonancia magnética, que permitieron mostrar la masa tumoral en todos los casos, excepto en uno de insulinoma. No pudo determinarse la localización pancreática en un pancreatoblastoma. Las imágenes fueron de fundamental importancia para la estadificación y un abordaje quirúrgico adecuado para esta rara patología pediátrica

Tratamiento percutáneo de estenosis recurrente de la vena porta postransplante hepático en un paciente pediátrico, con angioplastía y colocación de stent / Percutaneous angioplasty of portal vein stenosis after hepatic transplantation in a child

Los autores presentan el caso de un paciente de 9 años de edad, receptor de un transplante hepático reducido, consecuencia de una hepatitis fulminante. A los nueve meses presentó repetidos episodios de hemorragia gastrointestinal y melena. La endoscopía colateral, con vena porta permeable y disminución de su calibre en el sitio de la anastomosis. Se realizó una portografía transhepática que confirmó la existencia de una estenosis significativa a nivel de la anastomosis porto-portal, dilatada con resultados satisfactorios. Luego de cuatro meses reaparecieron los síntomas. Una nueva angiografía transhepática demostró restenosis, que fue tratada con angioplastía por vía transhepática, con buenos resultados. Seis meses después, la recurrencia de los síntomas y de la estenosis, llevaron a la realización de una nueva dilatación, esta vez asociada a la colocación de un stent, expandible por balón, de tipo Palmaz. Todos los procedimientos fueron exitosos. No se registraron complicaciones relacionadas con los procedimientos. El seguimiento clínico, por laboratorio y ecografía Doppler, a dos años, no muestra recurrencia de la lesión ni signos de hipertensión portal. Las técnicas intervencionistas, constituyen una opción terapéutica segura y eficaz para el tratamiento de las complicaciones vasculares postransplante hepático