Bronchogenic Cyst: Lessons Learned in 20 Years of Experience at a Tertiary Pediatric Center.

BACKGROUND: Bronchogenic cysts are benign congenital malformations of the primitive ventral foregut. The aim of this study is to analyze and report 20 years of experience in the diagnosis and treatment of bronchogenic cysts at a tertiary pediatric center. METHODS: A retrospective review was conducted of all patients diagnosed with a bronchogenic cyst between 2000-2020. Presence of symptoms, cyst location, surgical technique, postoperative complications, need for pleural drainage, and recurrence were reviewed. RESULTS: Forty-five children were included in the study. In 37 patients a partial resection of the cyst was done, followed by cauterization or chemical obliterateration with iodopovidone of the mucosa of the remaining cyst wall that was adherent to the airway. A lobectomy was done in patients who had intrapulmonary cysts (n = 8). Cyst location was subcarinal in 23 (51.1%), paratracheal in 14 (31.1%), and intrapulmonary in eight patients (17.8%). The majority of subcarinal and paratracheal cysts (90%) were approached by thoracoscopy. Complications occurred in seven patients (15%): subcutaneous emphysema after pleural drain removal in one, extubation failure in two, reoperation due to bleeding in one, surgical site infection in one, bronchopleural fistula in one, and pneumothorax in one. Reoperation due to cyst recurrence was necessary in two patients (4.4%). Mean follow-up was 56 months (range, 0-115). CONCLUSION: A minimally invasive approach is a safe option for the management of paratracheal and subcarinal bronchogenic cysts with no history of infection in specialized pediatric surgery center. Thoracoscopic partial resection is a feasible option in most patients with subcarinal and paratracheal bronchogenic cysts with a low complication and reoperation rate. LEVEL OF EVIDENCE: IV.

Clinical Utility of Definitive Diagnostic Tests for Choledocholithiasis in Pediatric Patients with Mild Gallstone Pancreatitis.

BACKGROUND: Gallstone pancreatitis was historically considered a risk factor for choledocholithiasis (CD). However, recent studies of adult patients evidenced a weak association between gallstone pancreatitis and CD. The aim of this study was to analyze this association in pediatric patients. METHODS: We conducted a retrospective study on patients with mild gallstone pancreatitis who underwent any definitive testing for CD (MRCP, ERCP, IOC), managed between March 2010 and September 2022. Patients were classified according to the presence or absence of risk factors for CD (total bilirubin ≥2 mg/dl; common bile duct >6 mm on ultrasound; and/or CD on ultrasound). We evaluated the diagnosis of CD on definitive testing in both groups and analyzed the predictive capacity of the presence of risk factors. RESULTS: Eighty-four patients were included in the final analysis. Seventy-nine percent were females. The median age was 13 (4-17) years. Forty-seven (55.9%) patients had one or more risk factors. The definitive testing confirmed 13 (15.5%) cases of CD, 12 (25.5%) in the group of patients with risk factors and 1 (2.7%) in those without risk factors. The sensitivity, specificity, positive predictive value and negative predictive value of the presence of associated risk factors were 92.3, 50.7, 25.5 and 97.3%, respectively. CONCLUSION: Pediatric patients with gallstone pancreatitis without associated risk factors have a very low incidence of CD. In these patients we suggest performing a laparoscopic cholecystectomy without intraoperative cholangiography or any other definitive test for CD. LEVEL OF EVIDENCE: Level II, retrospective study.

Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso / Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años. Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada. Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1­0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years. Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report. / Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso.

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1-0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years. Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años. Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada. Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo.

In a Setting of Esophageal Replacement, Total Gastric Pull-Up has Fewer Complications than Partial Gastric Pull-Up.

AIM: The main indications for an esophageal replacement (ER) are unresolved complex esophageal atresia (EA) and caustic strictures (CS). The use of different organs for replacement has been described. When the stomach is chosen, there are two ways to do a gastric pull-up: a partial (PGP) or a total pull-up (TGP). Few studies have been published comparing the different techniques. The aim of this study was to compare the outcomes of patients who underwent ER by PGP or by TGT. METHODS: The medical records of all patients who underwent gastric pull-up for ER in the last 18 years at the National Pediatric Hospital Prof. Dr. Juan P. Garrahan were reviewed. The study is comparative, retro-prospective and longitudinal. Patients were divided in two groups according to the ER technique (PGP or TGP). We compared the following outcomes: duration of the operation, days of hospitalization in the intensive care unit (ICU), days of total hospitalization, time to initiation of oral feedings and rate of anastomosis dehiscence, incidence of anastomotic stenosis, need for re-operations, incidence of gastroesophageal reflux disease (GERD), incidence of tracheo-esophageal fistulas (TEF), incidence of dumping syndrome, incidence of gastric necrosis and mortality. RESULTS: There were 92 patients included in the study: 70 in the PGP group (76%) and 26 in the TGP group (24%). The two groups were demographically equivalent. Patients in the TGP group had a statistically significant lower incidence of anastomotic dehiscence (22,7% versus 54,3%; p = 0.01) and dumping syndrome (13,6% versus 37,1%; p = 0.038). Patients in the TGP had lower incidence of anastomotic stenosis, although the difference was not statistically significant. There were no statistically significant differences between the groups in terms of duration of the operation, postoperative days in the ICU, time to oral feedings, GERD, TEF or overall hospital stay. There were no cases of gastric necrosis. There were 3 deaths in the PGP group and one in the TGP group. CONCLUSIONS: We observed benefits in the TGP group versus the PGP approach in terms of anastomotic dehiscence and dumping syndrome, as well as a trend toward a lower incidence of anastomotic stenosis. Based on this experience, we recommend the TGP approach for patients who need an esophageal replacement by a gastric pull-up. LEVELS OF EVIDENCE: According to the Journal of Pediatric Surgery this research corresponds to type of study level III for retrospective comparative study.

[Pulmonary tuberculosis presenting as a lung mass in children: case report]. / Masa pulmonar como manifestación primaria de tuberculosis en pediatría: reporte de un caso.

Tuberculosis is one of the main causes of death due to infection around the world. Although tuberculosis frequently involves lung parenchyma, tuberculoma is a rare complication. We describe an atypical pulmonary presentation of tuberculosis. A 15-month-old girl, previously healthy, was referred to the pulmonology department due to fever, poor weight gain, and a 2-months persistent lung image on chest x-ray despite antibiotic therapy. She had been in frequent contact with a respiratory symptomatic subject. She was admitted to the hospital with a TST of 13 mm and a heterogeneous bulky mass in the left upper lobe at chest computed tomography. Three gastric lavages were Masa pulmonar como manifestación primaria de tuberculosis en pediatría: reporte de un caso Pulmonary tuberculosis presenting as a lung mass in children: case report done and the patient underwent exploratory thoracoscopy and lung biopsy, with positive cultures for Mycobacterium tuberculosis. The diagnosis of pulmonary tuberculoma was confirmed, and the patient received standard anti- tuberculosis therapy with a favorable evolution.

La tuberculosis es una de las principales causas infecciosas de muerte en el mundo y es endémica en Argentina. La mayoría de los casos de tuberculosis son de localización pulmonar; el tuberculoma una complicación infrecuente. Se describe un caso clínico de presentación pulmonar atípica de tuberculosis. Se trata de una niña de 15 meses, previamente sana, derivada a neumología por fiebre, mal progreso de peso e imagen persistente por 2 meses en la radiografía de tórax a pesar de haber recibido antibioticoterapia. Antecedente de contacto estrecho con persona sintomática respiratoria. Se internó para estudio, mostró una PPD de 13 mm y una masa voluminosa heterogénea en el lóbulo superior izquierdo en la tomografía computada de tórax. Se realizaron tres lavados gástricos y toracoscopia exploratoria con biopsia pulmonar con rescate de Mycobacterium tuberculosis en cultivos. Se diagnosticó tuberculoma pulmonar y realizó tratamiento estándar con fármacos antituberculosos con evolución favorable.

Masa pulmonar como manifestación primaria de tuberculosis en pediatría: reporte de un caso / Pulmonary tuberculosis presenting as a lung mass in children: case report

La tuberculosis es una de las principales causas infecciosas de muerte en el mundo y es endémica en Argentina. La mayoría de los casos de tuberculosis son de localización pulmonar; el tuberculoma una complicación infrecuente. Se describe un caso clínico de presentación pulmonar atípica de tuberculosis. Se trata de una niña de 15 meses, previamente sana, derivada a neumología por fiebre, mal progreso de peso e imagen persistente por 2 meses en la radiografía de tórax a pesar de haber recibido antibioticoterapia. Antecedente de contacto estrecho con persona sintomática respiratoria. Se internó para estudio, mostró una PPD de 13 mm y una masa voluminosa heterogénea en el lóbulo superior izquierdo en la tomografía computada de tórax. Se realizaron tres lavados gástricos y toracoscopia exploratoria con biopsia pulmonar con rescate de Mycobacterium tuberculosis en cultivos. Se diagnosticó tuberculoma pulmonar y realizó tratamiento estándar con fármacos antituberculosos con evolución favorable.

Tuberculosis is one of the main causes of death due to infection around the world. Although tuberculosis frequently involves lung parenchyma, tuberculoma is a rare complication. We describe an atypical pulmonary presentation of tuberculosis. A 15-month-old girl, previously healthy, was referred to the pulmonology department due to fever, poor weight gain, and a 2-months persistent lung image on chest x-ray despite antibiotic therapy. She had been in frequent contact with a respiratory symptomatic subject. She was admitted to the hospital with a TST of 13 mm and a heterogeneous bulky mass in the left upper lobe at chest computed tomography. Three gastric lavages were done and the patient underwent exploratory thoracoscopy and lung biopsy, with positive cultures for Mycobacterium tuberculosis. The diagnosis of pulmonary tuberculoma was confirmed, and the patient received standard anti- tuberculosis therapy with a favorable evolution.

Surgical treatment of thoracic outlet syndrome in pediatrics.

AIM: Thoracic outlet syndrome (TOS) is a condition that occurs when the cervical neuro-vascular bundle becomes compressed at one of the three narrow areas of the thoraco-cervico-axillary region. Conservative management is the first line of treatment. Patients who do not respond to conservative management should be treated surgically. The aim of this review is to present our experience with the surgical management of TOS in pediatric patients. METHODS: We retrospectively reviewed the outcomes of all patients with TOS operated at our Hospital between 2001 and 2020. We collected all demographic data, clinical features, imaging data, type of operation performed, intraoperative findings, complications and recurrence. RESULTS: We operated 9 patients within the study period. The median age at surgery was 14 (7 to 17) years. A transaxillary approach was used in 7 patients and a supraclavicular approach in 2. There was only one minor intraoperative complication (violation of the pleural space). There were no postoperative complications. The median length of stay was 3 (2 to 4) days. All patients were extubated in the operating room. Two patients developed symptoms on the contralateral side. One of these underwent a successful contralateral transaxillary Roos operation. The follow-up was 4 months to 20 years. All patients are asymptomatic. CONCLUSION: We believe that the Roos operation is a safe and effective treatment with excellent long-term outcomes for children with TOS that fail conservative management.

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.

Nefroma mesoblastico congénito: reporte de un caso / Congenital mesoblastic nephroma: case report

El nefroma mesoblastico congénito es un tumor renal infrecuente, pero común en el periodo neonatal. De comportamiento benigno y pronóstico excelente en su variedad clásica pero en el subtipo celular puede presentar recurrencia local, invasión retroperitoneal y metástasis pulmonares y cerebrales. El tratamiento es la nefrectomía radical. Los factores pronósticos son la edad menor a tres meses y la resección quirúrgica completa. Paciente de 17 días nacida a las 35 semanas por polihidramnios, con diagnóstico prenatal de masa abdominal. Al examen físico masa en flanco derecho e hipertensión arterial (121/79 mm Hg, TAM 77mm Hg). Ecografía abdominal con riñón derecho aumentado de tamaño y voluminosa formación sólida, de 4,4 x 4,3 x 4,8 cm, heterogénea y vascularizada al Doppler en polo inferior. Tomografía de abdomen con formación nodular heterogénea de 5,0 x 4,1 x 5,1 cm en polo inferior del riñón derecho. Se realizó nefrectomía radical derecha sin complicaciones. Evolución en los últimos 20 meses satisfactoria y libre de enfermedad.

Congenital mesoblastic nephroma is an uncommon renal tumor, but frequent in neonates. Classical variety usually benign with excellent prognosis. Cellular cases may be recurrent with retroperitoneal invasion, brain and lung metastasis. Radical nephrectomy is the treatment of choice. Relevant prognostic factors are diagnosis before three months of age and complete resection. A 17 day old patient, borned at 35 weeks due to polyhydramnios, with prenatal diagnosis of abdominal mass. On examination, right flank abdominal mass and arterial hypertension (121/79 mm Hg, MAP 77 mm Hg). Abdominal ultrasound showed an enlarged right kidney with a voluminous solid heterogeneous mass, 4.4 x 4.3 x 4.8 cm in the lower pole, vascularized on Doppler scan. Computed tomography showed a big 5.0 x 4.1 x 5.1 cm, nodular, heterogeneous mass in the lower pole of the right kidney. Right radical nephrectomy was performed with an uneventful recovery. Follow up for the last 20 months satisfactory and free of disease.

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.

Biological versus synthetic patch for the repair of congenital diaphragmatic hernia: 8-year experience at a tertiary center.

PURPOSE: The aim of this study is to compare outcome between small intestinal submucosal (SIS) and polytetrafluoroethylene (PTFE) patches in patients with congenital diaphragmatic hernia (CDH) with large muscular defects. MATERIAL AND METHODS: We performed a retrospective review of prospectively collected data of patients who underwent CDH repair from January 2011 to June 2019, with a minimum follow-up of 1 year. Data were collected regarding length of stay (LOS), mortality and recurrence. Kaplan-Meier analyses was used. RESULTS: In the study period, 195 patients with CDH were admitted, 176 underwent surgical repair and 19 died before surgery. Sixty-two (35%) required a patch. Over the last 5 years, our institution transitioned from the use of all SIS to all PTFE. SIS was used in 18 cases and PTFE in 25. Overall survival rate was 89% (174/195). There was no significant difference regarding LOS and mortality. Nine patients (50%) of SIS and 1 (4%) of PTFE repairs recurred (P < 0.01; log-rank test P <  0.01), with a time course of recurrence of 18 (SD = 13) and 8 months, respectively. Median follow-up was 60 months (SD: 23) for the SIS group and 26 (SD: 10) for the PTFE group. CONCLUSION: A significant lower recurrence was found using PTFE patches instead of SIS for the repair of large CDH. LEVEL OF EVIDENCE: Level 3. Retrospective comparative study.

Series de Pediatría Garrahan. El niño con problemas respiratorios / Garrahan Pediatrics Series. The child with respiratory problems

La patología respiratoria presenta un gran desafío para las instituciones de salud, por su frecuencia, su complejidad diagnóstica y terapéutica y la carga que representa en costos económicos y vitales. Estos conceptos se extienden a todos los grupos etarios y sus características han ido cambiando a lo largo del tiempo ante los avances producidos en las inmunizaciones, los métodos diagnósticos y los tratamientos. Aún así, las infecciones respiratorias bajas son todavía la causa más frecuente de consulta, internación, morbilidad crónica, discapacidad y mortalidad en pediatría. Este nuevo volumen aborda esta temática especial y entre sus características se destacan: El estudio de las patologías más frecuentes en los diferentes ámbitos de atención pero en particular en el primer nivel, con una exposición centrada en los aspectos que facilitan el diagnóstico rápido y el tratamiento adecuado, con el menor uso de recursos y con pautas que fijan la derivación oportuna hacia el especialista o hacia una institución de mayor complejidad. El desarrollo de importantes temas, como la patología obstructiva de la vía aérea superior, su estudio diagnóstico y sus formas recurrentes; bronquiolitis; las intercurrencias respiratorias en pacientes con condiciones clínicas especiales; y la supuración pleuropulmonar. La inclusión, en todos los capítulos, de casos clínicos con su evolución y desenlace, textos destacados con los principales conceptos y puntos claves para recordar. Una obra sólida y práctica, que transmite la experiencia de los profesionales de una institución del prestigio internacional del Hospital dePediatría Prof. Dr. Juan P. Garrahan, dedicada a todos los pediatras, dondequiera que trabajen al servicio de la salud de los niños.

Risk factors for morbidities and mortality in children following pneumonectomy.

BACKGROUND AND OBJECTIVE: Pneumonectomy (PNE) is a procedure infrequently performed in children. A high morbidity/mortality rate associated with PNE has been described. Few series have been published in the last 15 years. Risk factors associated with morbidity/mortality after PNE were evaluated. Indications, course, survival and complications of PNE in children were also analized. METHODS: In a case series of 51 children who underwent PNE, death within 30 days of surgery, pneumonia, empyema, sepsis, adult respiratory distress syndrome, bronchopleural fistula, bleeding, pneumothorax and post-PNE syndrome were considered major morbidities. Scoliosis, wound infection and atelectasis were considered minor morbidities. RESULTS: Median age at PNE was 7.4 years; 45% were males. Indications of pneumonectomy were postinfectious bronchiectasis (61%), tumours (17%), pulmonary malformations (17%), aspiration syndrome (14%), cystic fibrosis (6%), immunodeficiency (4%) and trauma (2%). Mortality rate was 4% at 1 month. Major and minor morbidities were present in 23% and 27% of patients, respectively. Risk factors for development of morbidities after PNE were age ≤ 3 years (OR: 16.7; 95% CI: 2.4-117) and the need for mechanical ventilation for at least 4 days (OR: 8; 95% CI: 1.5-43.6). CONCLUSION: Children are at high risk of death, major and minor morbidities following PNE. Caution is recommended for this group of patients.

Primary lung tumors in children: 24 years of experience at a referral center.

PURPOSE: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years. METHODS: A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990-2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome. RESULTS: Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91-16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology. CONCLUSION: Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.

[Right postpneumonectomy syndrome: case report]. / Síndrome postneumonectomía derecha: informe de un caso.

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.

Síndrome postneumonectomía derecha: informe de un caso / Right postpneumonectomy syndrome: Case report

El síndrome postneumonectomía es una entidad poco frecuente en pediatría. Comunicamos un caso secundario a una neumonectomía realizada por un tumor intratorácico. Niño de 4 años con antecedente de exéresis pulmonar derecha y dificultad respiratoria progresiva, con obstrucción extrínseca de la vía aérea por estructuras mediastínicas desplazadas. Hubo intentos frustros de tutorización con dispositivos endoluminales de la vía aérea, e imposibilidad de retiro de la asistencia ventilatoria mecánica. Ante la imposibilidad de reposicionamiento mediastinal, se realiza puente (bypass) aórtico con tubo protésico, con evolución favorable y egreso hospitalario.(AU)

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.(AU)

Síndrome postneumonectomía derecha: informe de un caso / Right postpneumonectomy syndrome: Case report

El síndrome postneumonectomía es una entidad poco frecuente en pediatría. Comunicamos un caso secundario a una neumonectomía realizada por un tumor intratorácico. Niño de 4 años con antecedente de exéresis pulmonar derecha y dificultad respiratoria progresiva, con obstrucción extrínseca de la vía aérea por estructuras mediastínicas desplazadas. Hubo intentos frustros de tutorización con dispositivos endoluminales de la vía aérea, e imposibilidad de retiro de la asistencia ventilatoria mecánica. Ante la imposibilidad de reposicionamiento mediastinal, se realiza puente (bypass) aórtico con tubo protésico, con evolución favorable y egreso hospitalario.

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures. There were failure to intubate the airway with endoluminal devices and impossibility of withdrawing from mechanical ventilation. As mediastinal repositioning was not possible, an aortic bypass with a prosthetic tube graft was performed, with favorable outcome and hospital discharge.

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases.

OBJECTIVE: Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital. METHODS: Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years (1986-2011). RESULTS: Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure (OR = 25.7 [95%CI 3.2-221]; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008). CONCLUSIONS: Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications.

Malformação adenomatoide cística congênita: características clínicas, conceitos patológicos e tratamento em 172 casos / Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

OBJETIVO: A malformação adenomatoide cística congênita (MACC) é a malformação pulmonar ressecada cirurgicamente mais comum em crianças. Este estudo retrospectivo foi realizado para apresentar a experiência de 172 casos de MACC em um hospital pediátrico. MÉTODOS: Séries publicadas com um pequeno número de pacientes relatam detalhes de lesões, evolução e tratamento. Como este estudo lida com características clínicas, evolução e procedimentos cirúrgicos em 172 crianças com diagnóstico de MACC, a população inclui casos tratados e acompanhados em um hospital pediátrico ao longo de 25 anos (1986-2011). RESULTADOS: A idade média ao diagnóstico foi de 48 meses (r = 0,03-213), 52% (n = 90) eram do sexo masculino. Os sintomas de apresentação mais comuns foram dificuldade respiratória em crianças com menos de 6 meses de idade (40%) e pneumonia recorrente nas que tinham mais idade (75%; p = 0.001). Lobectomia foi o procedimento de escolha na maioria dos casos. Todos os tipos histológicos foram encontrados: 1 (70%), 2 (24%), 4 (4%) e 0 e 3 (n = 1). Foi observado um padrão misto em nove pacientes. Foram encontradas anomalias associadas em 47% das crianças. A mais frequente foi sequestro (71%), mais presente na MACC tipo 2 (p = 0,001). As anomalias mais graves se relacionaram principalmente com o tipo 2 (p = 0,008). Também foram observados um blastoma pleuropulmonar e um carcinoma broncoalveolar. A mortalidade foi de 5% (n = 9). Os fatores de risco para mortalidade foram falência respiratória (OR = 25,7 [IC95% 3,2-221]; p = 0,03), sepse (OR = 9,9 [IC95% 8,2-12]; p = 0,002), necessidade de assistência respiratória (OR = 9,5 [IC95% 2,3-37]; p = 0,04) e diversas comorbidades associadas (OR = 3,3 [IC95% 1,2-22]; p = 0,008). CONCLUSÕES: Foram observadas anomalias relacionadas em quase metade da população. Devido à possibilidade de infecção recorrente ou desenvolvimento de neoplasias, deve-se considerar a ressecção cirúrgica quando MACC for diagnosticada. O desfecho cirúrgico é favorável e apresenta complicações tratáveis.

OBJECTIVE: Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital. METHODS: Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years (1986-2011). RESULTS: Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure (OR = 25.7 [95%CI 3.2-221]; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008). CONCLUSIONS: Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications.