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.

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.

Tumores raros en pediatría. Primer reporte en la Argentina / Rare tumors in pediatrics. First report in Argentina

Introducción. Los ensayos clínicos cooperativos han aumentado el conocimiento sobre los tumores pediátricos; sin embargo, no es el caso de los tumores raros (TR). Objetivo. Describir prevalencia, características clínicas y evolución de los TR en la edad pediátrica diagnosticados en el Hospital Garrahan. Material y métodos. Estudio descriptivo retrospectivo de pacientes entre 0 y 18 años con diagnóstico de TR ingresados entre enero de 2007 y diciembre de 2017.Resultados. De 1 657 pacientes con diagnóstico de tumores sólidos, 164 (9,9 %) correspondieron a TR, 71,95 % (118) eran menores de 14 años y 81,7 % (130) eran varones. En orden de frecuencia, los TR fueron: carcinoma de tiroides (60), carcinoma suprarrenal (14), tumores pulmonares (14), melanoma (13), carcinoma de glándulas salivales (11), tumores gastrointestinales (8), tumores gonadales no germinales (7), tumores pancreáticos (7), carcinomas renales (6), carcinomas nasofaríngeos (5), feocromocitoma y paraganglioma (5) y carcinoma de timo en 1 paciente. El tratamiento recibido dependió del tipo de tumor y del estadio. Con una mediana de seguimiento de 34,9 meses (rango: 1-128,5 meses), 133 pacientes (78,7 %) están vivos y solo 10 pacientes (6 %) se perdieron durante el seguimiento. Conclusión. La prevalencia de TR fue del 9,9 %. El 27 % se presentaron en adolescentes. Los tumores más frecuentemente diagnosticados fueron carcinoma de tiroides, carcinoma suprarrenal y melanoma. El tratamiento y la evolución varió según el tipo histológico. Se hallaron alteraciones moleculares predisponentes en el 5,3 % de los pacientes, el 3,5 % tenían antecedente de patología oncológica.

Introduction. Collaborative clinical trials have enlarged the knowledge base about pediatric tumors; however, this is not the case for rare tumors (RT). Objective. To describe the prevalence, clinical characteristics, and course of RT in pediatric patients diagnosed at Hospital Garrahan. Material and methods. Descriptive, retrospective study of patients aged 0-18 years diagnosed with a RT and admitted between January 2007 and December 2017. Results. Out of 1657 patients diagnosed with solid tumors, 164 (9.9 %) were RT; 71.95 % (118) of patients were younger than 14 years and 81.7 % (130) were males. In order of frequency, RT were thyroid carcinoma (60), adrenal carcinoma (14), lung tumors (14), melanoma (13), salivary gland cancer (11), gastrointestinal tumors (8), non-germ cell gonadal tumors (7), pancreatic tumors (7), renal carcinomas (6), nasopharyngeal carcinomas (5), pheochromocytoma and paraganglioma (5), and thymic carcinoma in 1 patient. Treatment depended on tumor type and stage. The median follow-up was 34.9 months (range: 1-128.5 months);133 patients (78.7 %) are alive and only 10 patients (6 %) were lost-to-follow-up. Conclusion. The prevalence of RT was 9.9 %. Twenty-seven percent occurred in adolescents. The most frequent tumors included thyroid carcinoma, adrenal carcinoma, and melanoma. Treatment and course varied based on tumor histology. Predisposing molecular alterations were found in 5.3 % of patients; 3.5 % had a history of cancer.

Rare tumors in pediatrics. First report in Argentina. / Tumores raros en pediatría. Primer reporte en la Argentina.

Introduction: Collaborative clinical trials have enlarged the knowledge base about pediatric tumors; however, this is not the case for rare tumors (RT). Objective: To describe the prevalence, clinical characteristics, and course of RT in pediatric patients diagnosed at Hospital Garrahan. Material and methods: Descriptive, retrospective study of patients aged 0-18 years diagnosed with a RT and admitted between January 2007 and December 2017. Results: Out of 1657 patients diagnosed with solid tumors, 164 (9.9 %) were RT; 71.95 % (118) of patients were younger than 14 years and 81.7 % (130) were males. In order of frequency, RT were thyroid carcinoma (60), adrenal carcinoma (14), lung tumors (14), melanoma (13), salivary gland cancer (11), gastrointestinal tumors (8), non-germ cell gonadal tumors (7), pancreatic tumors (7), renal carcinomas (6), nasopharyngeal carcinomas (5), pheochromocytoma and paraganglioma (5), and thymic carcinoma in 1 patient. Treatment depended on tumor type and stage. The median follow-up was 34.9 months (range: 1-128.5 months); 133 patients (78.7 %) are alive and only 10 patients (6 %) were lost-to-follow-up. Conclusion: The prevalence of RT was 9.9 %. Twenty-seven percent occurred in adolescents. The most frequent tumors included thyroid carcinoma, adrenal carcinoma, and melanoma. Treatment and course varied based on tumor histology. Predisposing molecular alterations were found in 5.3 % of patients; 3.5 % had a history of cancer.

Introducción. Los ensayos clínicos cooperativos han aumentado el conocimiento sobre los tumores pediátricos; sin embargo, no es el caso de los tumores raros (TR). Objetivo. Describir prevalencia, características clínicas y evolución de los TR en la edad pediátrica diagnosticados en el Hospital Garrahan. Material y métodos. Estudio descriptivo retrospectivo de pacientes entre 0 y 18 años con diagnóstico de TR ingresados entre enero de 2007 y diciembre de 2017. Resultados. De 1 657 pacientes con diagnóstico de tumores sólidos, 164 (9,9 %) correspondieron a TR, 71,95 % (118) eran menores de 14 años y 81,7 % (130) eran varones. En orden de frecuencia, los TR fueron: carcinoma de tiroides (60), carcinoma suprarrenal (14), tumores pulmonares (14), melanoma (13), carcinoma de glándulas salivales (11), tumores gastrointestinales (8), tumores gonadales no germinales (7), tumores pancreáticos (7), carcinomas renales (6), carcinomas nasofaríngeos (5), feocromocitoma y paraganglioma (5) y carcinoma de timo en 1 paciente. El tratamiento recibido dependió del tipo de tumor y del estadio. Con una mediana de seguimiento de 34,9 meses (rango: 1-128,5 meses), 133 pacientes (78,7 %) están vivos y solo 10 pacientes (6 %) se perdieron durante el seguimiento. Conclusión. La prevalencia de TR fue del 9,9 %. El 27 % se presentaron en adolescentes. Los tumores más frecuentemente diagnosticados fueron carcinoma de tiroides, carcinoma suprarrenal y melanoma. El tratamiento y la evolución varió según el tipo histológico. Se hallaron alteraciones moleculares predisponentes en el 5,3 % de los pacientes, el 3,5 % tenían antecedente de patología oncológica.

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.

Simplified surgical approach to congenital megaprepuce: fixing, unfurling and tailoring revisited.

PURPOSE: Megaprepuce is a congenital penile malformation that includes phimosis and severe ballooning of the internal prepuce. We combined the main technical details of 2 previously reported surgical techniques to simplify the surgical approach. We report the key points of this revisited surgical correction for megaprepuce and the long-term results. MATERIALS AND METHODS: Penile reconstruction was performed between March 2004 and March 2009 in 26 children 2 to 19 months old (median age 6) with congenital megaprepuce. Surgical repair consisted of limited preputial resection of the phimotic ring, wide dissection of the outer prepuce to the base of the penis, fixation of the proximal outer prepuce to Buck's fascia to create a new penoscrotal junction, and unfurling and wide tailoring of the inner prepuce in the ventral midline to resurface the whole elongated penile shaft. A bladder catheter was left in place for 5 or 6 days. Only patients with at least 6 months of followup were included in analysis. RESULTS: All patients underwent surgery on an outpatient basis. Operative time was between 50 and 85 minutes. Median followup was 17.6 months (range 6 to 59). Two patients had slight dehiscence of the ventral suture, which healed spontaneously without a scar. In 1 patient transient urethral meatal stenosis developed. The final cosmesis result was adequate in all patients. CONCLUSIONS: It is possible to correct megaprepuce and achieve an adequate genital aspect with a simple approach of resurfacing the penis with the unfurled, tailored inner prepuce.