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Congenital infantile fibrosarcoma (CIF) is a rare tumor in children that occurs in the first years of life. It usually arises in the extremities but some cases affect the trunk, neck, abdomen, or retroperitoneum. Surgical resection has been traditionally the treatment of choice but the development of genomic analysis and targeted therapies has shed light on new therapeutic options. We present two patients with a congenital mass, one in the abdominal cavity (1-month-old) and the second in the left lower extremity respectively (2-months-old). In both cases, the clinical and radiological findings showed heterogeneous masses with rapidly progressive growth. MRI in the first patient exhibited an abdominal mass surrounding the aorta and inferior vena cava associated with a giant infrarenal aortic aneurysm. CT-guided biopsy was performed with pathological findings of fibrosarcoma and ETV6-NTRK3 gene fusion. The second patient underwent open biopsy also with histopathological diagnosis of fibrosarcoma and the same mutation in the TRK gene (NTRK3). Targeted therapy with a specific TRK inhibitor, larotrectinib, was started in both patients. Periodical controls were made by ultrasound or MRI, and after a few weeks of treatment, both children showed significant decrease in the mass. By the second and third months after starting the treatment, both tumors disappeared. The first patient is now 15-months-old and the second one is 8-months-old. Larotrectinib is a novel targeted therapy with excellent results in CIF but long-term outcomes are limited to establish it as a gold standard treatment.

Abstract Introduction Minimally invasive repair for pectus excavatum (MIRPE) is controversial in extremely severe cases of pectus excavatum (PE) and an open repair is usually favored. Our aim is to describe a case of a patient with an extremely severe PE that underwent a minimally invasive approach. Case report An 8-year-old girl with severe sternum depression was assessed. She had a history of exercise intolerance, nocturnal dyspnea, fatigue, and shortness of breath. Chest computed tomography showed that sternum depression was posterior to the anterior vertebral column; therefore, Haller and correction index could not be measured. Spirometry indicated an obstructive ventilation pattern (forced expiratory volume in 1 second = 74.4%), and echocardiogram revealed a dilated inferior vena cava, mitral valve prolapse with normal ventricular function. After multidisciplinary committee evaluation, a MIRPE approach was performed. All symptoms had disappeared at the 3-month postoperative follow-up; the desired sternum shape was achieved and normalization of cardiopulmonary function was observed. The Nuss bars were removed after a 2-year period. After 18-month follow-up, the patient can carry out normal exercise and is content with the cosmetic result. Conclusion Nuss procedure is feasible in our 8-year-old patient. In this case, both the Haller and correction index were not useful to assess the severity of PE. Therefore, under these circumstances, other radiologic parameters have to be taken into consideration for patient evaluation.

Abstract Introduction The penis eventually needs specific cutaneous coverage in the context of reconstructive procedures following trauma or congenital anomalies. Local flaps are the first choice but are not always available after multiple previous procedures. In these cases, skin graft and dermal matrices should be considered. Materials and Methods This study was a retrospective review of the past 4 years of four patients with severe loss of penile shaft skin who underwent skin reconstruction. Dermal matrices and skin grafts were utilized. Dermal matrices were placed for a median of 4.5 weeks (3.0–6.0 weeks). The skin graft was harvested from the inner thigh region for split-thickness skin graft (STSG) and the inguinal region for full-thickness skin graft (FTSG). Results The four patients presented with complete loss of skin in the penile shaft. One patient had a vesical exstrophy, one had a buried penis with only one corpus cavernosum, one had a wide congenital lymphedema of the genitalia, and one had a lack of skin following circumcision at home. They underwent reconstruction with three patients undergoing split-thickness skin graft; two dermal matrices; and one full-thickness graft, respectively, thereby achieving a good cosmetic and functional result. There were no complications, and all the patients successfully accepted the graft. Conclusion Dermal matrices and skin grafts may serve as effective tools in the management of severe penile skin defects unable to be covered with local flaps.

Objective: The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D-dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation., Methods: With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan-1 was determined by ELISA., Results: In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan-1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium., Conclusions: For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles., (© 2024 Wiley Periodicals LLC.)

Introduction: In severe cases of pectus excavatum (PE) the sternal depression may cause distortion of the cardiac chambers and great vessels. The aim of our study was to determine if the sternal impingement causes significant inferior vena cava (IVC) compression., Methods: Retrospective study of patients with severe PE assessed between 2015-2017. The antero-posterior (AP) and transverse diameters of the suprahepatic IVC were measured on a cardiac-MRI at the level of the diaphragmatic hiatus. Results were compared with patients that had a thoracic image study performed for other causes, adjusting for age and sex., Results: Among the 81 patients, 28 cases and 53 controls, 63% were males and had a mean age of 12.9±0.5 yrs. Significant differences were found between groups in both AP and transverse diameter of the IVC: 13.3±0.75 mm vs. 15.8±0.76 mm (p=0.001) and 28.8±1.34 mm vs. 27.1±0.89mm (p=0.045) respectively. After adjusting for age and sex, these differences were only statistically significant for AP IVC diameter in males 12.7±0.5 mm (95% CI 11.66-13.79 mm) vs. 16.6±0.5 mm (95% CI 15.69-17.56 mm) (p=0.000). The Pearson correlation coefficient for the Haller index was r=0.471 (p=0.01)., Conclusion: Male patients with severe sternal depression show changes in the IVC diameter that may correspond to compression. These changes are correlated with the severity of the deformity and can justify certain clinical symptoms and cardiac function abnormalities in patients with severe PE.

Aim:  Hepatoblastoma is the most frequent hepatic tumor in children, and its initial presentation will affect treatment and prognosis. The aim of this study is to evaluate long-term results of liver transplantation in children with unresectable hepatoblastoma., Patients and Methods:  This is a retrospective review of patients with hepatoblastoma who underwent liver transplantation, analyzing risk factors, tumor presentation, treatment, and long-term survival to identify prognostic factors., Results:  Thirty-one patients underwent liver transplantation in the context of unresectable hepatoblastoma, mainly males (67%) and with risk factors such as prematurity (12.9%), maternal smoking (6.5%), and familial adenomatous polyposis (3.2%). Most frequent presentation was multifocal PRETEXT III (51.6%) and PRETEXT IV (45.2%), with metastasis at diagnosis in 12.9% and vascular involvement in 54.8%.Twenty-one patients received a living-donor (67.7%) and 10 a cadaveric graft (32.2%), at 31.7 months of age (5-125). Most transplants were primary, and only two were performed as rescue therapy after an attempt of surgical resection of the tumor.Overall survival 1 and 5 years after transplantation were 93.3% ± 4.6% and 86.4% ± 6.3%, respectively. We could not find any statistically significant differences between risk factors, tumor presentation, type of graft, or type of transplant., Conclusion:  Liver transplantation has increased hepatoblastoma survival in unresectable tumors. Probably due to these good results, we have not been able to find significant prognostic factors in this cohort., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction:  Central venous port (CVP) placement is traditionally performed under fluoroscopy guidance. However, efforts for radiation dose reduction in children have allowed the introduction of ultrasound guidance (USG) and anatomic landmarks as an alternative technique for CVP placement. The aim of this study is to determine whether intraoperative fluoroscopy (IF) is required to confirm the correct position of the catheter tip in children., Patients and Methods:  A prospective, single-center study was performed between July and December 2017. Standard venous access site was the right internal jugular vein under USG. Estimated catheter length (ECL) was measured using anatomic landmarks. Ideal catheter length (ICL) was measured after placement under fluoroscopy guidance in the same patient. Age, sex, radiation dose, and complications were also analyzed. A t -test for paired samples and intraclass correlation coefficient were performed to analyze results., Results:  A total of 30 consecutive patients aged 7 ± 2 years underwent CVP placement. The mean ECL was 17.1 ± 1.8 cm, while the mean ICL was 17.7 ± 1.8 cm. The mean difference between measurements was 0.28 cm (95% confidence interval [CI], -0.29 to 0.86; p  = 0.324). Intraclass correlation coefficient analysis showed an agreement of 0.95 (95% CI, 0.91-0.98) between measurements. Mean radiation exposure during the procedure was 1.060.78 mGym 2 during 0.34 ± 0.6 minutes. There were no complications registered during CVP placement., Conclusion:  The correlation between IF and USG and anatomically guided catheter tip placement is optimal. These results suggest that fluoroscopy and the radiation exposure it entails can be safely avoided in selected children., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction:  There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity., Methods:  Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS)., Results:  Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1-5) episodes during an 8.3-year (2-15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1-28). A HAEC score of 9 (2-16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score ( r  = 0.3, p  = 0.014)., Conclusion:  There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim:  Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery., Patients and Methods:  Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery., Results:  Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery ( p  = 0.0015) as well as prothrombin activity ( p  = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt ( p  = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them., Conclusion:  Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim of the Study: The aim of this study is to identify potential perinatal risk or protective factors associated with NEC., Materials and Methods: Single-center, retrospective case-control study of newborns admitted to the neonatal intensive care unit with NEC from 2014 to 2015. Clinical charts were reviewed recording maternal factors (fever, positive recto-vaginal swab and signs of corioamnionitis or fetal distress), and neonatal factors analyzed were: birth-weight and weeks gestation, umbilical vessel catheterization, time of enteral feedings and the use of probiotics, antibiotics and antifungal agents. Cases and controls were matched for all of these factors. Parametric tests were used for statistical analysis and p < 0.05 deemed significant., Results: We analyzed 500 newborns of which 44 developed NEC (cases) and 456 controls. Univariate analysis did not identify any maternal risk factors for NEC. We did not found statistical differences between patients either time of enteral feedings or probiotics. Nevertheless, patients with signs of fetal distress and early sepsis had a higher risk of NEC (p < 0.0001)., Conclusions: Infants with history of fetal distress and signs of early sepsis are at a higher risk of NEC. The use of prophylactic catheter infection or orotracheal intubation with antifungal treatment seemed to elevate the incidence of NEC. However, antibiotic treatment couldn´t be demonstrated to increase the risk of NEC.

Aim of the Study: Recommendation of early pulmonary resection in asymptomatic congenital pulmonary airway malformations (CPAMs) is based on the presumed compensatory lung growth during the first months of life. Our aim is to analyze the long-term pulmonary function after lobectomy before and after one year of age using spirometry., Methods: We performed a retrospective review of children who underwent pulmonary lobectomy for CPAM between 2001 and 2016. Patients who were old enough (>5 years) to carry out a spirometry were included in the study and were divided into 2 groups (surgery before or after 12 months of age). Pulmonary function testing values were considered normal if they were >80% of predicted., Main Results: Forty-seven patients underwent pulmonary lobectomy for CPAM, 23 of them met the inclusion criteria and prospectively performed a spirometry. Among them, 7 had surgery before and 16 after one year of age (0.1 vs. 2); being both groups comparable in terms of sex, type of CPAM and surgical approach. Time from surgery until pulmonary function testing was longer in patients who had surgery before one year of age (9.1 vs. 4.6 years, p = 0.003). After correcting results by time from surgery until spirometry, a better FEV1/FVC was found in patients who had surgery after one year of age (90% vs. 77%, p = 0.043)., Conclusion: Although spirometry may be influenced by many other variables, these preliminary results do not support the current recommendation of performing early lobectomy in CPAMs. Further studies are required in order to resolve the best age to perform pulmonary lobectomy.

Aim:  The onset of inflammatory bowel disease (IBD) in patients younger than the age of 5 years is rare. Moreover, when there is exclusive colonic inflammation, diagnosis can be challenging. Our aim was to describe the difficulties and pitfalls in the diagnosis of early-onset IBD (eoIBD) and their repercussions in treatment decisions., Patients and Methods:  Patients with eoIBD (Crohn's disease [CD], ulcerative, and unclassified colitis) treated at our center between 1990 and 2016 were studied retrospectively. Demographic, clinical, medical, and surgical treatment data were analyzed., Results:  A total of 19 patients were diagnosed with eoIBD at 21 (1-46) months of age. Bloody diarrhea, growth failure, and abdominal pain were present in 90, 42, and 16%, respectively. After 9 (1-62) months from the onset, patients were classified as ulcerative colitis (nine), CD (two), and unclassified colitis (eight). Unresponsiveness to medical treatment was observed in 10 and prompted surgical assessment. A partial colectomy was performed in one, and nine underwent a total colectomy (one end stoma and eight ileoanal anastomosis [IAA]). At least one surgical complication occurred in 80% and ultimately six patients with an IAA required an end stoma. Overtime, final diagnosis of 5 out of the 10 surgical patients changed due to biopsy findings, unresponsiveness to medical treatment, or extraintestinal and perianal manifestations. After a 12-year (1-22) follow-up, 57.9% of the diagnoses of all patients were modified., Conclusion:  EoIBD poses a challenge due to ambiguous presentation and absence of specific diagnostic tests. Surgical evaluation is often needed and surgeons must be mindful of possible initial misdiagnosis, in addition to short- and long-term outcomes before deciding aggressive surgical measures as well as intestinal reconstruction., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim:  Cardiac function can be impaired in patients with pectus excavatum (PE) due to anatomic and dynamic compression of the heart. Efforts for radiation dose reduction in imaging techniques have allowed cardiac magnetic resonance imaging (c-MRI) to play a major role in PE assessment. The aim of our study is to describe the findings of c-MRI 18 months after we changed the PE assessment protocol from chest computed tomography to c-MRI., Patients and Methods:  Since mid-2015 all patients with severe PE (suspected Haller's index > 3.2) were assessed with inspiratory and expiratory c-MRI. A retrospective analysis of these patients was performed evaluating the following parameters: (1) Radiologic PE indexes (Haller's, correction and asymmetry indexes; and sternal rotation) and (2) cardiac function (including left and right ventricle ejection fraction)., Results:  A total of 20 patients met the inclusion criteria. Dynamic imaging showed a significant difference during inspiration and expiration of the Haller's index 3.85 (range: 3.17-7.3) versus 5.10 (range: 3.85-10.8) ( p  < 0.05), and correction index (26.86% vs. 36.84%, respectively, p  < 0.05). The sternal rotation was 14.5 (range: 0-36). c-MRI analysis disclosed a right ventricle ejection fraction of 50.3%. (normal range: 61% [54-71%]). Echocardiographic imaging underestimated the functional repercussion of PE in all patients., Conclusion:  Initial results show that PE assessment by c-MRI allows a radiation-free image of the chest wall deformity during the entire breathing process. Also, it permitted the evaluation of the influence of sternum impingement on cardiac function. These findings allowed us a careful surgical evaluation and preoperative planning., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Objective:  One of the principal objectives in treatment of facial nevus is to minimize psychological damage and encourage the child's schooling by the best possible cosmetic result. There are several therapeutic techniques: grafts, flaps, dermal regenerator, and tissue expanders., Materials and Methods:  We reviewed 10 patients with facial nevus higher than 10 cm treated in the past 8 years. Our treatment protocol includes serial expander implant to remove everything that does not involve the eyelid and nasal pyramid. Ten patients were included, between 8 and 36 months of age. A median of 4 (2-6) surgeries were conducted, and the number of implanted expanders was 1 to 3 in each session. There were only minor complications in two patients, infection that responded to antibiotic therapy and minimal dehiscence of incision forcing resuturing. The median follow-up was 1.6 (1.3-3.4) years., Conclusion:  Tissue expansion has become in recent years the treatment of choice for facial nevus in children, obtaining the best aesthetic result because the defect is covered with similar characteristics in color, texture, and relief skin. It is essential that the reconstruction is done by specialized units and we recommend complete surgical excision before starting school., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction/aim of the Study: Gastroschisis is a congenital malformation with an easy and early prenatal diagnosis, however, it has a variable post-natal outcome. Our aim was to determine if certain ultrasound markers or early delivery were related with a worse postnatal outcome., Patients and Methods: Retrospective study of a cohort of patients with gastroschisis diagnosed between 2005-2014, with emphasis on prenatal ultrasounds, gestational age at delivery and post-natal outcome. Oligohydramnios, peel, mesenteric edema, fixed and dilated bowel with loss of peristalsis and small wall defect were considered ultrasonographic markers associated with poor prognosis. Outcome variables included: length-of-stay, complications, nutritional and respiratory factors. Non-parametric statistical analysis were used with p < 0,05 regarded as significant., Results: Clinical charts of 30 patients with gastroschisis were reviewed (17M/13F). Gestational age at diagnosis was 20 (12-31) and at delivery 36 (31-39) weeks (33% of the patients over 36+3 weeks). A 73% of the patients presented at least one ultrasonographic marker factor during follow-up. Univariate analysis showed that mesenteric edema was associated with poor outcome variables: short-bowel syndrome (p= 0,000), PN-dependence (p= 0,007) and intestinal atresia (p= 0,02). The remaining risk factors analysed, including late delivery (> 36+3 weeks) were not associated with length-of-stay, ventilatory support, digestive autonomy, complications or mortality., Conclusions: Neither the presence of ultrasonographic markers classically associated with unfavorable outcomes, nor early delivery (< 36 weeks) resulted in worse postnatal outcome. Mesenteric edema was the only alarming ultrasound marker and that may suggest the need of closer follow-up.

Introduction/aim of the Study: In patients with PE, cardiovascular alterations ensue as a result of the mediastinum compression caused by sternum impingement and is responsible of many of the symptoms. Anatomical and functional assessment is of the utmost importance for a comprehensive understanding of the disease and an adequate treatment plan. Our aim was to describe the use of magnetic resonance image (MRI) in the study of PE and whether it is comparable to imaging techniques., Patients and Methods: A retrospective study of the first 10 patients in which an MRI was performed as part of PE assessment within august 2015-2016. PE indexes were evaluated: Haller, correction, asymmetry, as well as sternal rotation. An analysis of right ventricular function was carried out comparing echocardiogram and MRI., Results: MRI scan on 10 patients showed the following findings: Haller index: inspiration: 3.75 (3.5-7.3) and expiration 4,9 (3.9-10.8), correction index of 24% (5-37%) and a sternal rotation of 12º (0-31º). The cardiovascular study showed a median ejection fraction of the right ventricle (EFRV) of 50% (38-64%), with 9 of the 10 patients under the normal value of 61% (54-71%). Echocardiographic findings underestimated functional alterations in all of the patients., Conclusion: This initial study suggests that the use of MRI as a test of choice in the evaluation of PE subject to surgical correction is feasible. Absence of radiation offers the capacity of a complete and dynamic anatomical as well as cardiovascular study.

Introduction/purpose: There is current debate about the need of hospitalization of patients with enema-reduced intussusception. The purpose of this study is to describe intussusception recurrence in a tertiary care children's hospital in order to evaluate the feasibility of ambulatory treatment., Patients and Methods: Retrospective review of children diagnosed with intussusception from January 2009 to December 2013, identifying early recurrences as those that occurred between 12-72 hours after successful enema reduction and comparing the results with current literature., Results: A total of 121 children (77 male - 44 female), with a mean age of 18,9±2,7 months and weight of 10,77±0,57 kg (CI 95%) were treated for intussusception. Enema reduction was attempted in 90,7% (n= 88) of the cases, with a success rate of 76,1% (n= 67). Early recurrence rate was 6% (n= 4), without associated complications, which is similar to recent meta-analysis results (5,4%); however, three patients required surgical exploration. Mean length of stay was 2 days for enema-reduced intussusception, which resulted in a total cost of 2,076.67 euro per patient., Conclusion: The low recurrence rate and scarce risk of complications suggests that an 8 to 12 hour observation is a feasible alternative to hospital admission, which results in social advantages including family welfare as well as management costs. These results are a starting point for prospective randomized controlled trials comparing both treatment modalities.

Objectives: To analyze and describe the association between the development of venous aneurysms of the mayor vessels in patients with lymphatic malformations., Material and Methods: Retrospective review of patients diagnosed with both VA and LM from 1993 to 2014 and a descriptive analysis of clinical course was performed., Results: We found 6 patients, 50% females, who had LM and VA confirmed with imaging techniques. All were diagnosed between 20 weeks- 12 years of age. LM were found within the thoracic cavity (n= 4), intrathoracical-cervical area (n= 1) and lower extremities (n= 1). In most cases the dilated veins were near the LM and thus, the mediastinal vessels were most commonly affected (superior vena cava-innominated (n= 1), venous jugulosubclavian confluence (n= 2), superior vena cava (n= 2) and popliteal vein (n= 1)). A total of 4 patients required surgical treatment of the LM with complete excision in 2 of the cases. Patients with a prenatal diagnosis of lymphatic malformation were most likely to present venous aneurysms at birth (n= 2), however the remaining patients (without prenatal diagnosis) developed them later on (average 6 years). Unlike lower extremity aneurysms, none of these aneurysms grew or required antiplatelet therapy; local thrombosis developed in one which resulted in pulmonary thromboembolism and one developed mitral valve insufficiency (regurgitation?) which required valve replacement., Conclusion: VA is extremely rare, and there is not therapeutic algorithm, therefore treatment should be individualized.

Aim of the Study: Optimal surgical treatment of patients with gastroschisis remains controversial. Recent studies suggest better outcomes with secondary closure techniques (surgical or preformed silo). The purpose of the study is to identify differences in outcome of infants treated with traditional primary closure (PC) versus surgical silo (SS)., Patients and Methods: Retrospective study of patients primarily treated of gastroschisis between 2004 and 2014. Patients were divided in PC and SS according to abdominal wall closure. Non-parametric statistical analysis was used with p< 0.05 regarded as significant., Results: Twenty-seven patients were included (14M/13F). Primary closure was performed on 17 and 10 underwent surgical silo placement with a median of 6 (5-26) days till secondary closure. Prenatal diagnosis was available in most patients (74%) by the 20th week of gestation. There were no significant differences regarding sex, gestational age or birthweight between groups. Fewer ventilation days were required in PC group compared to SS (4 vs 13, p< 0,05), however, there was no difference in type of ventilation or oxygen needs. Sedation and parenteral nutrition requirements were also lower in PC patients 4 vs 10 and 12 vs 20 days respectively (p< 0,05). Post-operative complications (5vs6) and median length of stay (36vs43 days) were also similar in PC and SS patients. One patient ultimately died due to catheter-related sepsis. Mean length of stay in hospital was 42 days (20-195)., Conclusion: Patients with gastroschisis who underwent primary closure showed shorter ventilator support and PN dependency than those treated with surgical silo. However, SS is as safe and effective technique as PC and led to similar outcome regarding digestive autonomy and hospital length of stay.