Prognostic impact of pulmonary nodules diagnosed at initial presentation in patients with osteosarcoma.

BACKGROUND: Resection of all lung metastases in patients with osteosarcoma improves survival. The increased computed tomography (CT) scan quality allows detecting smaller nodules. We aimed to evaluate the prognostic impact of those nodules that do not meet the classical criteria for lung metastases. METHODS: A central radiology review (CRR) on lung CT scans performed during the treatment of patients included in OS2006 trial and treated with a high-dose methotrexate-based chemotherapy from 2007 to 2013 was realized in three centers. RESULTS: At trial enrollment, among 77 patients, six (8%) had nodules meeting the trial's criteria for metastatic disease, 46 (60%) were classified as having localized disease, and 25 (32%) as having doubtful nodules. After CRR, 218 nodules were found at diagnosis (all in patients classified as "metastatic or doubtful" and 13 patients classified as "localized") (median two nodules per patient [1-52]). The 5-year event-free survival/overall survival (EFS/OS) of patients with at least one nodule versus no nodule were similar (67.7%/79.2% vs. 81.8%/91%). After histological analysis, two of 46 (4.3%) "localized" and eight of 25 (32.0%) "doubtful" patients were re-classified as "metastatic," whereas there was no change in patients initially "metastatic." The 5-year OS of confirmed histological metastatic versus nonmetastatic patients were different (56% vs. 92%, p < .01). CONCLUSION: Central review of lung CT scan increased the detection of nodules in osteosarcoma. Patients with small lung nodules classified as doubtful had a quite similar outcome as those with a localized disease. However, patients with confirmed metastatic nodules have a poorer prognosis, even if considered as "localized" at diagnosis.

Comparison between US and MRI in the prenatal assessment of lung malformations.

BACKGROUND: The contribution of MRI in the prenatal evaluation of congenital lung abnormalities (CLA) has not been extensively investigated. OBJECTIVE: (1) To compare diagnostic accuracy and assessment of prognostic factors between US and MRI in CLA and (2) to assess the diagnosis agreement between prenatal imaging and postnatal diagnosis. MATERIALS AND METHODS: We included 23 consecutive fetuses who underwent concomitant US and MRI during gestation as well as postnatal CT and surgery (n = 22). RESULTS: US-MRI sets were performed at median gestational age of 26 (n = 16) and 34 (n = 22) weeks. Postnatal diagnoses were 11 congenital pulmonary airway malformations (CPAM), 4 bronchopulmonary sequestrations (BPS), 6 hybrid lesions and 2 cysts. US and MRI agreement was significantly better during the second trimester than during the third one (P = 0.02). Disagreements were related to missed cysts (n = 5), mediastinal shift (n = 6) and vessels (n = 5). US and MRI diagnosis agreement was present in 20 cases, including 5 cases of misdiagnosis. US and MRI were concordant with postnatal diagnosis in 17 and 16 cases, respectively. CONCLUSION: In our series, no clear superiority of MRI over US in the prenatal evaluation of CLA was demonstrated, but US better demonstrated systemic feeding vessels and MRI cysts and normal lung adjacent to the lesion.

Outcome of alimentary tract duplications operated on by minimally invasive surgery: a retrospective multicenter study by the GECI (Groupe d'Etude en Coeliochirurgie Infantile).

BACKGROUND: Alimentary tract duplications (ATD) are a rare cause of intestinal obstruction in childhood. There are many case reports but few series about laparoscopy or thoracoscopy for ATD. The aim of our study was to report the outcome of minimally invasive surgery (MIS) for ATD. METHODS: This was a retrospective multicenter study from the GECI (Groupe d'Etude en Coeliochirurgie Infantile). We reviewed the charts of 114 patients operated on by MIS for ATD from 1994 to 2009. RESULTS: Sixty-two patients (54 %) had a prenatal diagnosis. Forty-nine patients (43 %) were symptomatic before surgery: 33 of those patients (63 %) with postnatal diagnosis compared to 16 (25 %) with prenatal diagnosis (P < 0.01). In this last group, the median age at onset of symptoms was 16 days (range = 0-972). One hundred and two patients had laparoscopy (esophageal to rectal duplications) and 12 patients had thoracoscopy for esophageal duplications. The mean operative time was 90 min (range = 82-98). There were 32 (28 %) resection anastomoses, 55 (48 %) enucleations, and 27 (24 %) unroofings. The conversion rate was 32 %, and in a multivariate analysis, it was significantly higher, up to 41 % for patients weighing <10 kg (P < 0.01). Ten patients (8 %) had unintentional perioperative opening of the digestive tract during the dissection. Eight patients had nine postoperative complications, including six small bowel obstructions. The median length of hospital stay was 4 days (range = 1-21) without conversion and 6 days (range = 1-27) with conversion (P = 0.01). The median follow-up was 3 months (range = 1-120). Eighteen of the 27 patients who underwent partial surgery had an ultrasound examination during follow-up. Five (18 %) of them had macroscopic residue. CONCLUSION: This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.

Efficacy of blebs detection for preventive surgery in children's idiopathic spontaneous pneumothorax.

BACKGROUND: This retrospective, single-center study was designed to assess our management strategy based on blebs detection on the initial CT scan. METHODS: Children younger than aged 18 years presenting with a primary spontaneous pneumothorax (PSP) between 2000 and 2007 in a University Children's Hospital (hospital Armand Trousseau, Paris, France) were included in this study. RESULTS: Twenty-five children who presented with PSP were included. The mean age was 14.2 +/- 1.9 years, and the sex ratio was 2.1. There was no significant difference between patients with or without blebs with regard to the anthropomorphic data or the side of the pneumothorax. Six patients had recurrence, which, in most cases, was a grade 1 pneumothorax. Fourteen (56%) children showed blebs on CT scan, which was ipsilateral or bilateral in 13 cases and contralateral in 1 case. Eleven of these children had surgery, and all the remaining patients (n = 3) had recurrence. All the patients, except one, presenting blebs on the preoperating CT scan, showed blebs on the subsequent surgery (predictive positive value = 92%), and the CT-scan sensibility for blebs was 75%. CONCLUSIONS: In children, blebs detection on CT scan has a good sensitivity and may be a useful tool to determine the indication of lung surgery to prevent PSP recurrence.

Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT).

OBJECTIVE: Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. METHODS: Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered. RESULTS: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). CONCLUSION: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments. LEVEL OF EVIDENCE: NA Laryngoscope, 130:E243-E251, 2020.

Analysis of Clinical and Ultrasound Determinants of Adnexal Torsion in Children and Adolescents.

STUDY OBJECTIVE: We aimed to assess the performance of transabdominal ultrasonography (US) for diagnosis of adnexal torsion (AT) in children and adolescents and evaluate its clinical and US determinants. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: We retrospectively (2004-2014) studied data for children referred for acute or subacute pelvic pain who underwent US and included children 3 months to 18 years old who underwent surgical exploration and/or clinical and radiological follow-up for at least 3 months. The evaluation of US diagnostic performance was on the basis of the proposed diagnosis after US: AT or no AT with or without mass. Clinical and US predictors of AT were identified using regression analysis. RESULTS: Among 65 girls included (mean age 11.75 ± 4.49 years), 33 (50.8%) had AT. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of US for AT were 90.9%, 68.7%, 75%, 88%, and 80%, respectively. Overall, 20 of 33 (60.6%) AT patients had a mass, including 33% with mature teratomas. Patients with AT versus no AT more frequently showed vomiting (64% vs 28%; P = .008), ovarian edematous stroma with a peripheral distribution of follicles (45.5% vs 9.4%; P = .002) and a mass of pluritissular aspect (40% vs 0%; P < .0001). The quantitative US predictors of AT were total area ratio (ovary and mass)/area of the contralateral ovary 4.9 or greater (P = .0002) and surface area 18.5 cm2 or greater (P = .0003). CONCLUSION: US has high sensitivity but low specificity for the diagnosis of AT in children. The predictive criteria are vomiting, presence of a pluritissular mass, 4.9 area ratio or greater, or surface area 18.5 cm2 or greater.

An idiopathic congenital chylothorax: surgery or conservative management?

A 3-month-old infant was admitted to the respiratory unit for dyspnoea and vomiting after her second DTaP-Polio vaccine shot. The chest X-rays showed a white right lung with a left mediastinal shift. A pleural aspiration assessed the diagnosis of chylothorax. A conservative treatment was initiated with a fat-free diet and pleural aspirations. As this treatment was ineffective, a total parenteral nutrition was started at day 11, plus increasing doses of Octreotide. As the chylothorax persisted at day 50, a pleuroperitoneal shunting was performed but a pleurodesis was finally necessary. The child was discharged from the hospital 6 weeks after the surgery. Ten months later, her physical and biological conditions were normal and her chest X-rays dramatically improved. This case highlights the difficult management of infant chylothorax. Although conservative treatment has to be tried first, surgical procedures as pleuroperitoneal shunting and rarely pleurodesis have to be discussed.

[Bronchial carcinoid tumors in children]. / Tumeurs carcinoïdes bronchiques de l'enfant.

Carcinoid tumors are the most common endobronchial tumors in the pediatric population, and represent a rare cause of airway obstruction. Clinical manifestations are unspecific, and diagnosis is often delayed due to low clinical suspicion. These tumors are considered low-grade malignant neoplasms, and their evolution is usually favorable after surgery. However, local recurrence and/or metastases can occur with both typical and atypical carcinoid tumors, justifying the need of prompt diagnosis and long-term follow-up.

Percutaneous retrograde endovascular occlusion for pediatric varicocele.

BACKGROUND/PURPOSE: The aim of this study was to assess whether percutaneous retrograde endovascular occlusion (PREVO) is effective and safe for the treatment of varicocele in pediatric patients. METHODS: We retrospectively studied 71 children who underwent PREVO for left-sided varicocele. The primary outcome was the proportion of varicocele-free patients 6 months after PREVO as assessed by ultrasonography. RESULTS: Seventy-one boys with left-sided grade III varicocele underwent PREVO at a mean age of 13.2 years. PREVO was performed under local anesthesia in all boys but 2, who required general anesthesia. The procedure was technically feasible in 68 (96%) patients. In the remaining 3 patients, the internal spermatic vein could not be catheterized. Minor short-term complications occurred in 6 patients and resolved fully. No major complications or deaths were recorded. The proportion of varicocele-free patients 6 months after PREVO was 93% (66/71) overall and 97% (66/68) in the patients whose PREVO procedure was feasible. No clinical recurrence was observed during the mean follow-up of 17.5 months. CONCLUSIONS: Percutaneous retrograde endovascular occlusion is an effective minimally invasive approach for varicocele treatment in pediatric patients. It can be safely performed on an outpatient basis under local anesthesia.

Therapeutic strategies for idiopathic chylothorax.

STUDY OBJECTIVES: The objectives of the study were to present our institutional experience of idiopathic chylothorax in children and to propose therapeutic strategies. DESIGN: This was a retrospective, single-center study. PATIENTS: Patients were 6 children (4 boys, 2 girls) presenting with an idiopathic chylothorax diagnosed from the presence of a chylous pleural effusion with triglycerides greater than 1.2 mmol/L and a cellularity greater than 1000 cells/mL with a predominance of lymphocytes. RESULTS: Median age of onset was 7 years (range, 2-14 years). Initial symptoms included cough (n = 4), tachypnea (n = 4), asthenia (n = 5), abdominal pain (n = 2), and bronchitis (n = 1). Chest radiography showed 2 left, 2 right, and 2 bilateral pleural effusions. Serum biology assessment was normal in all children. Respiratory function assessment at diagnosis revealed a decrease in functional residual capacity in 3 children and a decrease in lung diffusing capacity in 2 children. Initially, all patients received a medium-chain triglyceride diet for 29 months (range, 10-50 months). Total parenteral nutrition was required for 4 patients (for 1-4 months), and somatostatin was tried in one child. Two children required pleuroperitoneal shunting, bilateral in one case. During the follow-up (median duration, 6 years; range, 2-16 years), chylothorax stabilized in all patients and 5 patients were able to return to a normal diet. CONCLUSION: A medium-chain triglyceride diet associated in some cases with total parenteral nutrition may stabilize idiopathic chylothorax in children. In cases where conservative treatment has failed, pleuroperitoneal shunting may be useful.

Fetal and neonatal ovarian cysts: is surgery indicated?

OBJECTIVES: To evaluate the frequency of ovarian torsion in neonates with ovarian cysts (OCs) and to analyze the outcome after surgical treatment. METHODS: A retrospective review of all infants with OCs managed in our institution during 20 years, was conducted. Our management included a follow-up (FU) of prenatal serial ultrasound (US) scan without prenatal cyst aspiration and early postnatal US scan. Neonatal surgery was indicated for cysts that appeared complex regardless of size and for simple cysts larger than 20 mm. RESULTS: Sixty-seven OCs detected through prenatal diagnosis were identified. Cysts were detected at a median gestational age (GA) of 33 weeks. At diagnosis, median size of the OCs was 40 mm, and 18% were complex. On the postnatal US scan, 55% were complex. Neonatal surgery was performed for 64 OCs : 56% were torsed, 6% were hemorrhagic, and 38% were uncomplicated. Proportions of complex- and simple-appearing cysts on prenatal or postnatal US scan were the only significantly different parameters found between infants with torsed or not torsed OC. CONCLUSION: Our study demonstrated that OC bears a high risk of ovarian loss. These findings have implications regarding information and treatment offered to pregnant women bearing an affected fetus.

Carcinoid and mucoepidermoid bronchial tumours in children.

UNLABELLED: The aim of the study was to determine the characteristic features and outcome of carcinoid or mucoepidermoid tumours in children. A retrospective analysis of all patients treated for a carcinoid or mucoepidermoid tumour in France between 1984 and 2001 was performed. There were 11 cases of carcinoid tumour and 6 cases of mucoepidermoid tumour. The mean age of the patients was 10.5+/-3.0 years, with a range of 5 to 15 years. Twelve and 6 patients presented with evidence of bronchial obstruction and haemoptysis, respectively. Fibre optic bronchoscopy confirmed the presence of a bronchial tumour in all cases and endobronchial biopsies were diagnostic in 11 of 12 cases. A chest CT scan revealed the presence of a hypervascular tumour in 8 of 12 patients. The distribution of the location of the tumours was equal between the right and the left lung, and, in 9 cases, the airways were totally occluded by the tumour. Complete surgical resection (lobectomy in 15 patients and pneumonectomy in 2 patients) was performed in all cases without pre-operative chemotherapy or radiotherapy. The mean duration of follow-up was 4.0+/-3.0 years. In 2 patients, auscultation assymetry and an episode of haemoptysis revealed the recurrence of a mucoepidermoid tumour, successfully cured by removal of the tumour and chemotherapy and radiotherapy in one child. No death was observed. CONCLUSION: Pulmonary carcinoid and mucoepidermoid tumours are rare in children. Bronchoscopic removal should not be performed. With aggressive surgical therapy, the prognosis is excellent. Fibre optic bronchoscopy confirms the presence of an endobronchial mass. A biopsy is needed for diagnosis and complete surgical removal is the treatment of choice. Long-term results are excellent but a clinical follow-up is recommended.