Complications of Central Venous Access Devices in Patients With Sickle Cell Disease and Thalassemia Major.

Pediatric patients with sickle cell disease and thalassemia major present clinical characteristics that could lead to a higher incidence of central venous access devices-associated complications (CVAD-C). With the objective of analyzing the safety of the use of CVAD in these patients, a retrospective review including all pediatric patients with these pathologies who required the implantation of a CVAD between 2004 and 2019 was performed. In all, 54 patients with 100 CVAD (65 totally implantable venous access port with subcutaneous reservoir, 35 single-lumen or double-lumen partially tunneled catheter) were included. During 60,410 days at risk of suffering a CVAD-C, 55 complications (complication rate [CR]/1000 catheter-days at risk=0.91) were reported in 46 CVAD: 19 mechanicals (CR=0.32), 32 infectious (CR=0.53), and 4 thrombotic complications (CR=0.066). Incidence of mechanical and infectious complications was significantly higher in double-lumen partially tunneled catheter than in totally implantable venous access port with subcutaneous reservoir (P<0.001). Lower age at insertion was related with a higher incidence of any complication (odds ratio=0.88/y, P=0.02). Patients who required a stem cell transplantation (31 patients and 65 CVAD) had no significant higher incidences of CVAD-C. In conclusion, our study supports the safety of using CVAD in these patients, with a low incidence of infectious, thrombotic, and mechanical complications.

Evaluation of proadrenomedullin as a diagnostic or prognostic biomarker of acute appendicitis in children.

INTRODUCTION: A delay in the diagnosis of acute appendicitis (AA), with the added complication of symptoms that mimic other self-limited causes of abdominal pain, can lead to an increase in ruptured appendices and morbimortality. None of the serum biomarkers evaluated to date have shown a predictive value for early diagnosis. OBJECTIVE: The objective of this study was to evaluate the usefulness of proadrenomedullin (MR-proADM) in the diagnosis of AA in children presenting with acute abdominal pain. METHODS: A single-center prospective observational study was conducted in 136 children who presented to the emergency department with suspected AA. RESULTS: Forty-four (32.5%) children had AA, and 9 (20.5%) had perforated appendicitis. The mean concentration of MR-proADM was significantly higher in children with AA than in children with nonspecific abdominal pain (NAP) (0.54 nmol/L; 95% confidence interval, 0.46-0.55 and 0.37 nmol/L; 95% confidence interval, 0.35-0.40, respectively). Performance characteristics of MR-proADM alone were not optimal. However, after combining best cutoff points, the combination of a C-reactive protein level of <0.3 mg/dL and a MR-proADM level of <0.34 nmol/L showed 100% sensitivity and negative predictive value, with 61% specificity. CONCLUSIONS: Although MR-proADM values are higher in children with AA than in children with nonspecific abdominal pain, these values do not help in the early diagnosis of AA. The combination of low C-reactive protein and low MR-proADM levels is useful for the identification of children with a low risk of AA.

Long Term Outcome of 112 Pediatric Patients With Ureteroplevic Junction Obstruction Treated by Endourologic Retrograde Balloon Dilatation.

Purpose: To analyze the effectiveness, complications and long-term outcome of the patients with ureteropelvic junction obstruction (UPJO) treated by endoscopic retrograde balloon dilatation (ERBD) in the largest series reported. Materials and Methods: Between years 2004 and 2018, 112 patients with primary unilateral UPJO were treated by ERBD. Endoscopic treatment consisted on a retrograde balloon dilatation of the ureteropelvic junction (UPJ), through cystoscopy and under fluoroscopic guidance, using high-pressure balloon catheters. In case of persistence in the balloon notch, a Cutting Balloon™ catheter was used. Double-J stent was placed after dilatation. Results: Mean age at surgery was 13.1 ± 21.3 months, 92 cases being younger than 18 months. Mean operative time was 24.4 ± 10.3 min; hospital stay was 1 day in 82% of patients. No intraoperative complications occurred. UPJ was calibrated at time of stent removal with cystoscopy 39.1 ± 13.7 days after dilatation. ERBD was not possible in 11 cases. An additional procedure was needed in 24 cases: second ERBD (n = 11, seven during the stent withdrawal), a third dilatation (n = 3) due to persistent hydronephrosis, and percutaneous endopyelotomy (n = 3) or open pyeloplasty (n = 7) in cases of technical failure. Significant improvement in postoperative ultrasound measures were observed (p < 0.05, T-test). Long-term success rate was 76.8% after one dilatation, and 86.6% in those who required up to 2 dilatations. Mean follow-up was 66.7 ± 37.5 months. Conclusions: ERBD is a feasible and safe option for the minimally invasive treatment of UPJ obstruction in infants. Long-term outcome is acceptable with a very low complication rate.

Juvenile Xanthogranuloma as Differential Diagnosis of a Vulvar Mass: A Case Report.

Vulvar masses in children are an unusual finding but their differential diagnosis is extensive. In case of solid masses, rhabdomyosarcoma (RMS) must always be considered due to the fact that it is the most common tumor in external genitals during childhood. However, RMS has a radiological appearance very similar to juvenile xanthogranuloma (JXG). We present a 16-month-old girl with a 2 cm solid mass on her left labia majora, with four overlying cutaneous papules. After imaging tests, an excisional biopsy was programmed due to high malignancy suspicion. Histopathology of the mass and one of the papules was diagnostic for JXG. After a 12-month follow-up, the patient shows no signs of relapse or complication. Deep JXG is an uncommon entity in childhood and exceptional in the genital area. Therefore, it must be included in the differential diagnosis of a solid vulvar mass, especially if accompanying yellowish xanthomatous cutaneous lesions are present.

Management and results of thyroidectomies in pediatric patients with MEN 2 syndrome.

AIM OF THE STUDY: To evaluate the outcome of prophylactic thyroidectomies (PT) in patients with MEN 2 syndrome in a tertiary center. METHODS: A retrospective study was designed, including all patients with MEN 2 syndrome who underwent PT between 2000 and 2019. Demographics, gene mutation, postoperative complications and histopathological findings were registered. MAIN RESULTS: 30 patients were included (29 MEN 2A and 1 MEN 2B) with a median age at surgery time of 7.0 ± 3.2 years. Familiar history was present in all but 3 patients. A therapeutic thyroidectomy was performed in 2 patients due to evidence of medullary thyroid carcinoma (MTC, both were late diagnosis), and in the other 28 cases, a PT was performed. 8 patients had a RET mutation ranked as Moderate Risk (American Thyroid Association): median age at surgery was 7.2 ± 4.2 years, and histological findings were C-cell hyperplasia (n = 6) and no alterations (n = 2). 16 patients had a high risk mutation; median age at surgery time was 6.9 ± 2.8 years and histological findings were normal thyroid gland (n = 1), C Cell Hyperplasia (n = 8), microcarcinoma (n = 6), and MTC (n = 1). The mean hospital stay was 1.4 ± 0.68 days. No intraoperative complications or recurrent laryngeal nerve injuries were registered. 7 patients presented a transient hypoparathyroidism and 1 patient had permanent hypoparathyroidism. CONCLUSIONS: Early PT in patients with MEN 2 syndrome is a safe procedure when performed by an experienced team of Pediatric Surgeons and with a multidisciplinary approach. Early genetic analysis and familial counselling is essential to prevent the development of a MTC.

[Ingestion of magnetic foreign bodies during the paediatric age. Case report, location and endoscopic extraction aided by a magnet]. / Ingesta de cuerpos extraños magnéticos en la edad pediátrica. Caso clínico de localización y extracción endoscópica con ayuda de un imán.

The ingestion of more than one magnet can cause multiple complications. Current protocols recommend endoscopic extraction if possible. We report a patient who swallowed two magnets and the endoscopic extraction technique. An 11-yearold boy presented at the Emergency Room after ingesting two small magnets, being asymptomatic. In the abdominal x-ray two radiopaque bodies were identified at the gastric chamber, apparently together. A gastroscopy was done in the operating room under general anaesthesia. To enable the extraction, a neodymium magnet was placed externally at the abdominal wall. In the endoscopic image, the two magnets were fixed to the anterior gastric wall. Once located, the neodymium magnet was removed and the two magnets were retrieved with an endoscopic basket.

La ingesta accidental de más de un imán puede producir complicaciones graves. Los protocolos actuales recomiendan la extracción endoscópica de ser posible. El objetivo de este trabajo es presentar un caso clínico de una ingesta de dos imanes y la técnica endoscópica de extracción. Niño de 11 años que acudió a Urgencias tras la ingesta de dos imanes, asintomático. En la radiografía de abdomen, se identificaron dos cuerpos extraños radiopacos, aparentemente unidos, en la cámara gástrica. Se realizó una endoscopía bajo anestesia general en quirófano. Para facilitar la extracción, se colocó, encima de la pared abdominal, a nivel gástrico, un imán de neodimio. En la endoscopía, se observaron dos pequeños imanes localizados y fijados en la cara anterior del estómago. Una vez localizados, se retiró el imán externo y se procedió a su extracción con cesta.

Digital thoracic drainage: a new system to monitor air leaks in pediatric population.

INTRODUCTION: New digital thoracic drainage systems allow an objective measurement of air leakage. They have proven their usefulness in the postoperative thoracic surgery in adults, but there is little experience with its use in the pediatric population. The objective of our study is to analyze their safety and effectiveness in the postoperative period of the pediatric patients. METHOD: A prospective consecutive observational study was done. All patients submitted to pulmonary resection between 2011 and 2017 and in whom digital thoracic drainage system was used (Thopaz Chest Drain System, Medela, Switzerland) were prospectively enrolled in this study. We analyzed variables: duration of chest tube (CT), days of hospitalization and radiographs in the immediate postoperative period related to the presence of CT. This group was compared with a historical cohort of patients (from 2011 to 2015) with a pulmonary resection in whom the traditional thoracic drainage was used. For the statistical analysis, the Mann-Whitney U-Test was used for independent samples. RESULT: Twenty-six patients were included, Digital drainage system was used in13 patients and traditional drainage was used in 13 patients. The median age was 18 months (12 days-14 years). The mean number of days with the chest tube was 1.69 ±â€¯0.6 in digital drainage group versus 5.38 ±â€¯4 days in traditional drainage group (p < 0.05) The mean number of postoperative radiographs was 2.8 ±â€¯1.1 in digital drainage group versus 6.23 ±â€¯5.2 radiographs in traditional drainage group (p < 0.05). The average hospital stay in digital drainage group was 5.69 ±â€¯2.7 days versus 7 ±â€¯4.7 days in the traditional drainage group (p > 0.05). No complications related to the use of digital drainage group were registered. CONCLUSION: The digital thoracic drainage systems provide an objective measurement of air leakage, allowing early chest tube removal and decreasing the number of radiographs performed postoperatively. Its use in the pediatric population appears to be safe and potentially beneficial. LEVEL OF EVIDENCE: II.

Long-Term Outcomes in Primary Obstructive Megaureter Treated by Endoscopic Balloon Dilation. Experience After 100 Cases.

Aim: To assess long-term effectiveness, complications, and outcomes of primary obstructive megaureter (POM) treated by endoscopic balloon dilation (EBD) in the largest series reported. Patients and Methods: Hundred POM in 92 consecutive patients were treated by EBD between years 2004 and 2016. A total of 79 POM (73 patients) with more than 18 months of follow-up after treatment have been analyzed. EBD of the vesicoureteral junction was performed with semicompliant high-pressure balloon catheters (2.7FG) with minimum balloon diameter of 5 mm, followed by temporary Double-J stent placement. Follow-up protocol included periodical clinical reviews, US and MAG-3 renogram scans. Results: Median age at surgery was 4 months (15 days-3.6 years), with median operating time of 20 min (10-60) and hospital stay of 1 day (1-7). Initial renal function was preserved in all patients with significant improvement in renal drainage on the MAG-3 diuretic renogram after endoscopic treatment (p < 0.001 T-test). Significant post-operative differences were observed in hydronephrosis grade and ureteral diameter that were maintained in the long-term (p < 0.001 T-test). Endoscopic approach of POM had a long-term success rate of 87.3%, with a mean follow-up of 6.4 ± 3.8 years. Secondary VUR was found in 17 cases (21.5%), being successfully treated by endoscopic subureteral injection in 13 (76.4%). Nine cases developed long-term re-stenosis (12.2%) that were successfully treated with a new EBD in 8. Endoscopic management of POM failed in 10 cases (12.7%) that required ureteral reimplantation. Five were early failures (4 intraoperative technical problems and 1 double-J stent migration with severe re-stenosis), and 5 long-term (4 persistent VUR and 1 re-stenosis recurrence). Conclusion: EBD has shown to be an effective treatment of POM with few complications and good outcomes at long-term follow up. Main complication was secondary VUR that could also be treated endoscopically with a high success rate. In our opinion, EBD may be considered first-line treatment in POM.

Retrograde Endopyelotomy with Cutting Balloon™ for Treatment of Ureteropelvic Junction Obstruction in Infants.

PURPOSE: The aim of this study is to analyze results of retrograde endopyelotomy with cutting balloon for treatment of ureteropelvic junction obstruction (UPJO) in infants. METHODS: We routinely treat patients with UPJO under 18 months of age with retrograde high-pressure balloon dilatation of the pelviureteric junction (PUJ). During the procedure, in these cases where narrowing at the PUJ persists, endopyelotomy with cutting balloon is performed. Endopyelotomy is performed over guidewire with 5-mm Cutting Balloon™ under fluoroscopic control. Double-J stents is left in situ for 4 weeks. We retrospectively analyzed the postoperative, clinical, and radiological outcome infants treated with cutting balloon endopyelotomy between 2007 and 2015. RESULTS: Sixteen patients required cutting balloon endopyelotomy to achieve complete resolution of narrowing of the waist observed during high-pressure balloon dilatation of the PUJ. Mean operative time was 35 ± 21 min (mean ± SD) and hospital stay was <24 h in all patients. Complete resolution of the narrowing at the PUJ under fluoroscopy was achieved in all cases, with no perioperative complications. One patient presented with urinary tract infection, postoperatively (Clavien grade II). Preoperatively, all cases had grade IV SFU hydronephrosis with parenchymal thinning. During follow-up, resolution of the hydronephrosis was observed in 11 patients (grade I SFU). In four infants, there was an improvement of the hydronephrosis (grade II SFU) and the renogram curve. In one case, an open pyeloplasty was required due to persistent hydronephrosis and obstructive curve. CONCLUSION: We believe that endopyelotomy with cutting balloon could be a valid and safe option in minimally invasive management of UPJO in infants.

Three-Dimensional Custom-Made Titanium Ribs for Reconstruction of a Large Chest Wall Defect.

Reconstruction of large chest wall defects always demand surgeons of having lots of means available (both materials and resourceful) to apply a cover to chest wall defects which can range from a few centimeters to the lack of a few entire ribs. In this study, we present the case of a teenager who suffered from a complete resection of three ribs because of Ewing sarcoma dependent on the sixth rib. Given the size of the defect, a multidisciplinary approach was chosen to provide rigid and soft tissue coverage and minimal functional and aesthetic impact. Custom-made titanium implants were designed based on three-dimensional computed tomography scan reconstruction. The surgical specimen via a left lateral thoracotomy (fifth, sixth, and seventh entire ribs) was resected, leaving a defect of 35 × 12 × 6 cm. A Gore-Tex patch (W. L. Gore & Associates, Arizona, United States) was placed and, after that, the implants were anchored to the posterior fragment of the healthy ribs and to the costal cartilage anteriorly. Finally, the surgical site was covered with a latissimus dorsi flap. The postoperative course was uneventful. After 9 months of follow-up, the patient has full mobility. This case shows that the implant of custom-made ribs, combined with other techniques, is a good surgical choice for reconstruction of large chest wall defects. The implant of custom-made ribs, combined with other techniques, is a good surgical choice for reconstruction of large chest wall defects.

Acute gastric volvulus and congenital diaphragmatic hernia, case report and review.

Congenital diaphragmatic hernia (CDH) is the result of the incomplete fusion and closure of the pleuroperitoneal canal during the fetal development. CDH is usually diagnosed prenatally but, if undiagnosed, the clinical presentation ranges from asymptomatic children to serious respiratory or gastrointestinal symptoms. Acute gastric volvulus associated with CDH is a rare surgical emergency in children. We report two cases of acute gastric volvulus associated with CDH and review the literature.

Ingesta de cuerpos extraños magnéticos en la edad pediátrica. Caso clínico de localización y extracción endoscópica con ayuda de un imán / Ingestion of magnetic foreign bodies during the paediatric age. Case report, location and endoscopic extraction aided by a magnet

La ingesta accidental de más de un imán puede producir complicaciones graves. Los protocolos actuales recomiendan la extracción endoscópica de ser posible. El objetivo de este trabajo es presentar un caso clínico de una ingesta de dos imanes y la técnica endoscópica de extracción. Niño de 11 años que acudió a Urgencias tras la ingesta de dos imanes, asintomático. En la radiografía de abdomen, se identificaron dos cuerpos extraños radiopacos, aparentemente unidos, en la cámara gástrica. Se realizó una endoscopía bajo anestesia general en quirófano. Para facilitar la extracción, se colocó, encima de la pared abdominal, a nivel gástrico, un imán de neodimio. En la endoscopía, se observaron dos pequeños imanes localizados y fijados en la cara anterior del estómago. Una vez localizados, se retiró el imán externo y se procedió a su extracción con cesta

The ingestion of more than one magnet can cause multiple complications. Current protocols recommend endoscopic extraction if possible. We report a patient who swallowed two magnets and the endoscopic extraction technique. An 11-yearold boy presented at the Emergency Room after ingesting two small magnets, being asymptomatic. In the abdominal x-ray two radiopaque bodies were identified at the gastric chamber, apparently together. A gastroscopy was done in the operating room under general anaesthesia. To enable the extraction, a neodymium magnet was placed externally at the abdominal wall. In the endoscopic image, the two magnets were fixed to the anterior gastric wall. Once located, the neodymium magnet was removed and the two magnets were retrieved with an endoscopic basket.