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CLAPO syndrome (Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial or generalized Overgrowth) is a nonfrequent pathology. This syndrome is characterized by the capillary malformation (CM) of the lower lip, a very important clinical sign when diagnosing CLAPO. The aim of our report is to demonstrate that rapamycin could be a reliable and safe targeted therapy in lymphatic malformations (LMs). This drug is useful in reducing the LM's size before final surgical treatment. The clinical and radiological evolution of a patient with CLAPO syndrome is reported in this article, before and after the treatment with rapamycin., (© 2019 Wiley Periodicals, Inc.)

Introduction: Resection of bronchial tumors usually needs resection of the lung parenchyma ventilated by the ill bronchus. Surgery over a developing child must preserve the biggest amount of lung parenchyma as possible. We show a complete resection of a benign tumor from the left main-stem bronchus, without pneumonectomy., Materials and Methods: Case report, Results: A 5 month girl presented with left hypoventilation. No significant previous dates. After bronchoscopy (with negative biopsy), chest radiograph and CT, she was diagnosed of a tumor in the mainstem left bronchus (20 x 15 mm) without metastatic extension. A sleeve resection of the tumor, with carinal reconstruction was performed. It was done under unipulmonary ventilation, with selective bronchial intubation. The patient was extubated in the operating room and the hospital stay was of 5 days. Follow up bronchoscopies shows no anastomotic stricture neither leakage. The patient is doing well 2 years after surgery., Conclusions: With this report and others in literature we can say that is possible to resect bronchial tumors in infancy without parenchymal resections.

BACKGROUND/PURPOSE The International Society of Paediatric Oncology (SIOP) protocol for Wilms tumor (WT) includes preoperative chemotherapy as the initial approach. However, an inadequate treatment may be performed in case of histological misdiagnosis. We evaluated the impact of fine-needle aspiration cytopathology (FNAC) in the diagnosis of unilateral WT in our group of patients. MATERIALS AND METHODS A retrospective descriptive study of patients with diagnosis of unilateral WT who underwent FNAC prior to neoadjuvant chemotherapy between 1993 and 2009 was performed. We reviewed the cytological diagnosis obtained by ultrasound-guided FNAC and the histological correlation with the resected specimens. RESULTS FNAC was performed in 66 patients with unilateral WT. In 57 of the 58 patients with positive FNAC for WT, the final diagnosis was correct (PPV: 98.2%). In 8 cases with negative FNAC for WT, the final diagnosis was positive for WT in 3 patients (NPV: 62.5%). Sensitivity was 95% and specificity was 83.3%. No complications were found associated with the procedure, except for an episode of haematuria, which resolved spontaneously. CONCLUSIONS FNAC is a useful and feasible technique in children that may confirm the suspected diagnosis of unilateral WT, avoiding inadequate preoperative chemotherapy in case of a non-Wilms renal tumor.

Aim: To present our experience in the management of three cases of tracheobronchial iatrogenic injuries and the literature revision about this pathology., Material and Methods: We present 3 patients treated in our center with tracehobronchial iatrogenic injuries since 2005., Results: Case 1. 8 year old boy who present cervical, mediastinal and facial emphysema after foreign body extraction. After 9 days of conservative management without endotracheal intubation all emphysema remited, and the bronchial injury healed. Case 2. 1 month old toddler who presented after cardiovascular surgery and endotracheal tube extraction dyspnea and hypoxia. Fibrobronchoscopy showed subglotic posterior tracheal disrupture. Fifteen-day endotracheal intubation throughout the injury was enough management on this patient. One month later the tracheal injury was completely healed. Case 3. 5 year old girl with tracheobronchial iatrogenic injury after orotracheal intubation who developed neumothorax and subcutaneous emphysema. After fiteen-day conservative management without intubation the injury healed., Conclusion: After iatrogenic tracheobronchial injury suspicion there were confirmed by fibrobronchoscopy. Conservative treatment in this patients was successful. Orotracheal intubation prevented air leaking through the tracheal injury allowing complete healing of the trachea. Bronchial injuries healed without needing intubation or mechanical ventilation. Articles reviewed recommend surgical treatment in those cases who had complete or large airway disrupture or in those who were misdiagnosed.

Objective: To analyze advantages and disadvantages between Seldinger's technique (ST) and surgical dissection (SD) on intravenous port-a-caths comparing surgical parameters as time and complications., Material and Method: An analytic retrospective study involving historic cohorts was realized, comparing our surgical experience on port-a-cath implantation with Seldinger technique or surgical dissection. Statistical analysis was made reflecting clinical and surgical parameters, such as surgical time length and intra/postoperative complications., Results: 193 Port-a-caths were analyzed (119 SD, 74 ST), mainly placed for chemotherapy treatment (83.41%). Surgical time length expended at single procedures was 72.85 +/- 29.35 minutes for SD and 62.83 +/- 20.08 minutes for ST (p < 0.05). There were none operator-dependent differences. Statistically significant differences were not found between the two cohort's populations, neither at complications percentages. Greater-sized port-a-caths presented a higher average of skin necrosis (p > 0.05) however, lower-sized port-a-caths showed a higher average of infection (16% upon 7.7% p > 0.05). Both ST (51.67 vs. 98.14 min) and SD (78.56 vs. 123.61 min) showed lower surgical time length at left venous accesses (d = 171 vs. i = 19). Average in days for the extraction of port-a-caths with regard to complications was 121 days., Conclusion: Seldinger technique reduced the definitive lost of surgical dissected venous accesses, being possible further utilization of the same vein for subsequent port-a-caths. Seldinger technique reduces surgical time length without increasing complication's rate. Left venous access does not imply higher surgical time length. Complications may be related with port-a-cath's size.

Aim: To evaluate our experience in sigmoid augmentation to manage renal upper tract dilatation and urinary incontinence., Material and Methods: Retrospective descriptive study of the augmentation cystoplasties with sigmoid patch. We analyze clinical, urodinamic and surgical parameters., Results: We have make 30 cistoplasties with a mean age of 10.24 years (3-15 years) with a mean follow up of 8 years (9 months-15 years). The main diagnosis was neurogenic bladder (63,3%) being the main indication for the augmentation the progressive dilatation of the renal upper tract (43,3%). We associate to the procedure the collocation of an artificial sphinter (30%), Mitrofanoff or ureteral reimplantation. There was no major complication. In the urodinamics, the bladders had low capacity, high pression and low compliance. After surgery, urodinamic parameters improve. During follow up, continence improve with more intermittent clean catheterism (median 4/day). Vesical lithiasis was more common late complication with nor acute abdominal surgical illness nor malignancy; 2 patients finished in renal transplantation. Evolution was positive in 66%, and negative in 18,5%., Conclusions: Sigmoid augmentation cystoplasty is a good technique, that allows urodinamic and continence improvement. The conscience in the importance of intermittent catheterisms reduces the incidence of complications.

Unlabelled: The acid and non acidic gastroesophageal reflux can trigger apnoea, desaturation and bradicardia events, as well as chronic pulmonary pathology due to microaspirations, whereas the acute or chronic airway closing increase the intrathoracic pressure, triggering the reflux. Our aims were to measurement in preterms newborn the correlations between cardiorespiratory events and gastroesophageal reflux, find out the direction of this relation, identify the patients with association GER->CRE and decide the suitability of antireflux surgery., Method: The study was made in the Motility Unit and in the Intensive Neonatal Care Unit, to preterms newborns without associated pathology except apnoea and/or bradicardia and/or desaturation., Material: 3 hours manometry study in the Motility Unit. 24 hours impedance, pH and cardiorespiratory parameters monitoring (respiratory and cardiac frequent, O2 saturation and CO2) in Intensive Neonatal Care Unit. We characterised the gastroesophageal barrier, all the reflux events and the association between GER and CRE., Results: We made 28 records to 28 patients with CRE. The average of the total number of reflux was 61 (22,25-103,00), 29,2% acid reflux and 70,8% weakly acidic. 12 patients had some GER associated with CRE but in only 2 cases was statistically significant (Sympton index: SI; Sympton Sensitivity Index: SSI) (SI > or = 50%; SSI > or = 10%). The surgical management was successfully in these two babies and nowadays they are asymptomatic., Conclusion: There is not any general association between GER and CRE, nevertheless, in a little percentage of patients, this relationship is fulfilled and it is possible to measurement with impedance, pH and cardiorespiratory parameters. In these cases, the surgical management is the right treatment.