Neutrophil-to-lymphocyte ratio as a predictor of surgical site infection in acute appendicitis.

Background: Surgical site infection (SSI) is one of the most common complications after appendectomy, which carries high associated morbidity. Therefore, it is essential to determine SSI predictive factors in order to prevent its occurrence. The aim of this study is to explore the role of neutrophil-to-lymphocyte ratio (NLR) as a predictor of SSI after appendectomy in children. Methods: A single-center, retrospective cohort study was performed in children who underwent appendectomy between 2017-2020. Demographics, time since symptoms onset, laboratory tests at admission, ultrasound appendiceal diameter, rate of complicated appendicitis, surgical aproach, surgery time and SSI rate were analyzed. Follow-up was performed during hospitalization and at outpatient clinic at 2 weeks and 30 days postoperatively to assess the surgical wound aspect. Diagnostic cut-off values of these markers for SSI prediction were based on the significance in the univariate analysis. Variables with a P value <0.05 in the univariate analysis were then entered into the multivariate analysis. Results: A total of 1,136 patients (710 males; 426 females) were included. SSI was reported in 53 patients (4.7%) during the 30-day follow-up after appendectomy (SSI group), with no demographic differences with the control group. Time since symptoms onset was significantly higher in SSI group (24 vs. 18 hours; P=0.034), as well as ultrasound appendiceal diameter (10.5 vs. 8.5 mm; P=0.010). Complicated appendicitis was observed in about 60% of both groups, without differences in surgical approach between them. Surgery time was statistically higher in the SSI group (62.4 vs. 47.9 min; P<0.001). SSI group presented higher counts of leukocytes, neutrophils and NLR than control group (P<0.001). NLR was the parameter with the highest area under the curve (AUC) (AUC =0.808; P<0.001), with a cut-off point of 9.8 with maximum sensitivity (77.8%) and specificity (72.7%). NLR was an independent predictive factor for SSI in the multivariate analysis [odds ratio (OR) 1.82 (1.13-2.73); P<0.01]. Conclusions: NLR value at admission was the most promising predictive factor for the development of SSI in children undergoing appendectomy. It is an easy, simple, inexpensive, and rapid method to detect patients at high risk for SSI. However, further prospective studies are still needed to confirm these results.

Neutrophil-to-Lymphocyte Ratio as a Predictor of the Need for Surgical Treatment in Children's Intussusception.

OBJECTIVE: Neutrophil-to-lymphocyte ratio (NLR) is an emerging inflammatory marker in abdominal pathologies. Ileocolic intussusception (ICI) involves a progressive intestinal inflammation, and the effectiveness of nonsurgical treatment (enema) might be related to the inflammation degree, although no previous studies have investigated this relationship. Our aim is to identify predictors of the need for surgical treatment in ICI. MATERIALS AND METHODS: A single-center, retrospective, case-control study was performed in children with ICI, who were treated with initial nonsurgical management between 2005 and 2019. Patients were divided in two groups: A (effective enema) and B (need for surgery). Admission demographic and clinical and laboratory data were analyzed. Specificity and sensitivity of the different parameters as predictors of the need for surgical treatment were determined by receiver operating characteristic (ROC) curves. RESULTS: A total of 511 patients were included (410: group A; 101: group B), without statistically significant demographic differences. Group B presented significantly higher frequency of vomiting, bloody stools, and longer median time since symptoms onset (24 vs. 8 hours; p < 0.001). Group B presented higher median laboratory inflammatory markers than group A: NLR (6.8 vs. 1.8; p < 0.001), neutrophils (10,148 vs. 7,468; p < 0.001), and C-reactive protein (CRP; 28.2 vs. 4.7; p < 0.001). In ROC curve analysis, NLR had an area under the curve of 0.925, higher than neutrophil count (0.776; p = 0.001), CRP (0.670; p = 0.001), and time since symptoms onset (0.673; p = 0.001). It was estimated a cut-off point of NLR greater than 4.52 (sensitivity: 73.2%; specificity: 94.5%). CONCLUSION: High NLR values imply a high degree of bowel inflammation and might anticipate the need for surgical treatment in ICI in children. LEVEL OF EVIDENCE: III.

Native Spleen Preservation During Visceral Transplantation Inhibits Graft-Versus-Host-Disease Development: Clinical and Experimental Study.

OBJECTIVE: We aimed to assess whether native spleen preservation during visceral transplantation (VT) affects graft-versus-host-disease (GVHD) incidence. SUMMARY BACKGROUND DATA: GVHD is one of the most severe and frequently lethal hematological complications after VT procedures. Because there is no specific treatment for GVHD, it is imperative to develop a strategy to reduce donor lymphocyte engraftment and proliferation. METHODS: Our study included both clinical and experimental data. A total of 108 patients were divided into 3 groups: a native spleen preservation group, a native spleen removal with no donor spleen group, and a donor spleen included (allogeneic spleen) group. We also used an allogeneic VT rat model, in which recipients were divided into 2 groups: a native spleen preservation (+SP) group and a native spleen removal (-S) group. Skin rash appearance, histopathological changes, chimerism, and spleen effects on circulating allogeneic T-cells were assessed. RESULTS: The patients with native spleen preservation showed a lower rate of GVHD ( P <.001) and better survival ( P <.05) than those in the other groups. Skin and histological signs of GVHD were lower in the rats in the +SP group ( P <.05). The donor T-cell frequency in the bloodstream and skin was also significantly reduced when the native spleen was preserved ( P <.01 and P <.0001, respectively). CONCLUSIONS: The clinical and experimental data indicate that recipient spleen preservation protects against GVHD after VT, and donor cell clearance from the bloodstream by spleen macrophages could be the underlying mechanism. Therefore, spleen preservation should be considered in VT procedures, whenever possible.

European Liver Transplant Registry: Donor and transplant surgery aspects of 16,641 liver transplantations in children.

BACKGROUND AND AIMS: The European Liver Transplant Registry (ELTR) has collected data on liver transplant procedures performed in Europe since 1968. APPROACH AND RESULTS: Over a 50-year period (1968-2017), clinical and laboratory data were collected from 133 transplant centers and analyzed retrospectively (16,641 liver transplants in 14,515 children). Data were analyzed according to three successive periods (A, before 2000; B, 2000-2009; and C, since 2010), studying donor and graft characteristics and graft outcome. The use of living donors steadily increased from A to C (A, n = 296 [7%]; B, n = 1131 [23%]; and C, n = 1985 [39%]; p = 0.0001). Overall, the 5-year graft survival rate has improved from 65% in group A to 75% in group B (p < 0.0001) and to 79% in group C (B versus C, p < 0.0001). Graft half-life was 31 years, overall; it was 41 years for children who survived the first year after transplant. The late annual graft loss rate in teenagers is higher than that in children aged <12 years and similar to that of young adults. No evidence for accelerated graft loss after age 18 years was found. CONCLUSIONS: Pediatric liver transplantation has reached a high efficacy as a cure or treatment for severe liver disease in infants and children. Grafts that survived the first year had a half-life similar to standard human half-life. Transplantation before or after puberty may be the pivot-point for lower long-term outcome in children. Further studies are necessary to revisit some old concepts regarding transplant benefit (survival time) for small children, the role of recipient pathophysiology versus graft aging, and risk at transition to adult age.

Healthcare staff as promoters of parental presence at anesthetic induction: Net Promoter Score survey.

BACKGROUND: Surgical intervention is usually a traumatic event that causes stress and anxiety in the pediatric patient and the family environment. To reduce the harmful effects of presurgical anxiety, parental presence during induction of anesthesia (PPIA) is one of the more notable interventions used in medical centers. However, data on this measure are difficult to evaluate and often face resistance from healthcare staff. AIM: To analyze the perception of the healthcare workers after the implementation of a PPIA program. METHODS: A survey was developed and sent by email to all the healthcare staff working in the children's area of a tertiary hospital. It consisted of 14 items divided into positive aspects of PPIA and negative aspects of PPIA evaluated with the use of a Likert scale (1 to 5). The demographics of the respondents were included in the data collected. The answers to the questions were interpreted through the Net Promoter Score (NPS). The statistical analysis compared the differences in the responses to each question of the survey made by the different groups of health personnel included. RESULTS: A total of 141 surveys were sent out, with a response rate of 69%. Of the total number of responses, 68% were from women and 32% from men. The average age of the participants was 42.3 ± 10.6 years. As for the positive questions about the PPIA, 83% had an NPS > 50, and only one had a score between 0 and 50, which means that the quality of the service was rated as excellent or good by 100% of the respondents. On the other hand, 100% of the negative questions about the PPIA had a negative NPS. Responses to the question "PPIA increases patient safety" were significantly different (P = 0.037), with a lower percentage of pediatric surgeons (70%) thinking that PPIA increased patient safety, compared with anesthesiologists (90%), nursing (92%), and other medical personnel (96%). CONCLUSION: The personnel who participated in the PPIA program at our center were in favor of implementation. There were no validated arguments to support worker resistance to the development of the PPIA.

Graft infusion of adipose-derived mesenchymal stromal cells to prevent rejection in experimental intestinal transplantation: A feasibility study.

BACKGROUND: Mesenchymal stromal cells (MSC) have been proposed as a promising complement to standard immunosuppression in solid organ transplantation because of their immunomodulatory properties. The present work addresses the role of adipose-derived MSC (Ad-MSC) in an experimental model of acute rejection in small bowel transplantation (SBT). MATERIAL/METHODS: Heterotopic allogeneic SBT was performed. A single dose of 1.5x106 Ad-MSC was intra-arterially delivered just before graft reperfusion. Animals were divided into CONTROL (CTRL), CONTROL+Ad-MSC (CTRL_MSC), tacrolimus (TAC), and TAC+Ad-MSC (TAC_MSC) groups. Each Ad-MSC groups was subdivided in autologous and allogeneic third-party groups. RESULTS: Rejection rate and severity were similar in MSC-treated and untreated animals. CTRL_MSC animals showed a decrease in macrophages, T-cell (CD4, CD8, and Foxp3 subsets) and B-cell counts in the graft compared with CTRL, this decrease was attenuated in TAC_MSC animals. Pro- and anti-inflammatory cytokines and some chemokines and growth factors increased in CTRL_MSC animals, especially in the allogeneic group, whereas milder changes were seen in the TAC groups. CONCLUSION: Ad-MSC did not prevent rejection when administered just before reperfusion. However, they showed immunomodulatory effects that could be relevant for a longer-term outcome. Interference between tacrolimus and the MSC effects should be addressed in further studies.

Routine Ultrasound Control after Successful Intussusception Reduction in Children: Is It Really Necessary?

INTRODUCTION: Recurrence of ileocolic intussusception (ICI) has been related to residual bowel wall edema after enema reduction. Early oral tolerance has been associated with a higher risk of re-intussusception, so an imaging test (ultrasound) has traditionally been performed before restarting oral tolerance. Our aim is to analyze the cost-effectiveness of performing a routine ultrasound in patients who remain asymptomatic after successful enema reduction. MATERIALS AND METHODS: A retrospective observational study was performed in patients with ICI who underwent a successful enema reduction between 2005 and 2019 and distributed in two groups according to whether or not a routine ultrasound was performed before restarting oral tolerance: group A (ultrasound) or B (no ultrasound). We analyzed demographic, clinical and laboratory variables, length of hospital stay, and recurrence rate. RESULTS: We included 366 patients who presented 373 ICI episodes (165 in group A and 208 in group B), without significant differences in gender and age. Group A patients presented a higher percentage of vomiting and bloody stools than those in group B without differences in the other clinical features studied, time of evolution, or laboratory variables. Group A presented a higher length of hospital stay than group B (36 vs. 24 hours), although it was not statistically significant (p = 0.30). No statistically significant differences were observed in the recurrence rate between both groups (10.3% A vs. 10.8% B; p = 0.83). CONCLUSION: Performing routine ultrasound before restarting oral tolerance in asymptomatic patients after successful ICI reduction does not decrease the risk of re-intussusception and should not be routinely encouraged.

Implant of mesenchymal cells decreases acute cellular rejection in small bowel transplantation.

OBJECTIVE: The objective of the study was to show adipose tissue-derived mesenchymal stem cells (AD-MSCs) immunomodulatory effects in small bowel transplantation (SBTx). MATERIALS AND METHODS: Forty Wistar Han rats (age: 10-12 weeks): were allogenic receptor rats and were allotted in 2 groups. Control group: rats undergoing orthopic SBTx ; AD-MSCs group: rats undergoing orthotopic SBTx plus AD-MSCs. Male Lewis rats were allogeneic small bowel donors. Rejection was confirmed by histological study of the explanted intestine, enterocyte apoptosis was determined in crypts and the lamina propria of the small bowel. Cytokine concentration levels (enzyme-linked immunosorbent assay) (interleukin [IL]-4, IL-10, IL-12, IL-17, IL-21, IL-23, tumor necrosis factor-alpha, and transforming growth factor [TGF]-b1) and cell percentages (flow cytometry) (CD3+ CD4+, CD8+, CD4+/25+, CD8+/25+, CD4+/25+/Foxp3+, and CD8+/25+/Foxp3+) were assessed in peripheral blood preoperatively and after death. RESULTS: Treatment with AD-MSCs produced a significantly lower risk of rejection in the first 7 post-operative days (five rejection cases among 20 rats in the control group and only one case in the AD-MSCs group). Treg cells and TGFb1 levels showed a significant increase in the AD-MSCs group. CONCLUSIONS: The local implantation of AD-MSC in the anastomosis and the intestinal lumen can induce a regulatory immune response, by increasing the percentages of Treg cells and TGb-1 levels, leading to a lower risk of acute rejection by cell mediation, in the first 7 days of the intestinal transplant. We think that the implantation of AD-MSCs, in the anastomoses and in the lumen of the donor intestine, could give rise to a chimera of donor-recipient cells.

OBJETIVO: Mostrar el efecto inmunomodulador de las células madre mesenquimales (AD-MSCs) en el trasplante de intestino delgado (SBTx). MÉTODO: 40 ratas Wistar Han (edad: 10-12 semanas): grupo control (SBTx) y grupo AD-MSCs (SBTx + AD-MSCs implantadas en las anastomosis distal y proximal del intestino delgado y en la luz intestinal). El intestino delgado provino de ratas Lewis. El rechazo se confirmó histológicamente. Se estudió la apoptosis de los enterocitos en las criptas y en la lámina propia del intestino delgado. Se determinaron por ELISA las citocinas (IL-4, IL-10, IL-12, IL-17, IL-21, IL-23, TNF-α, TGF-b1) en sangre periférica y por citometría de flujo los porcentajes celulares (CD3+ CD4+, CD8+, CD4+/25+, CD8+/25+, CD4+/25+/Foxp3+, CD8+/25+/Foxp3+) en el preoperatorio y después de la muerte. RESULTADOS: El empleo de AD-MSCs se asoció a una disminución significativa del riesgo de rechazo en los primeros 7 días posoperatorios (cinco casos de rechazo de 20 ratas en el grupo control y un solo caso en el grupo AD-MSCs). Las células Treg y los valores de TGFb1 mostraron un incremento significativo en el grupo AD-MSCs. CONCLUSIONES: El implante local de AD-MSCs en las anastomosis del trasplante de intestino delgado podría disminuir el rechazo celular agudo. Pensamos que la implantación de AD-MSCs, en las anastomosis y en el lumen del intestino donante, podría dar lugar a un quimera de células donante-receptor.

Congenital hepatic hemangiomas: Clinical, histologic, and genetic correlation.

BACKGROUND: The guide for monitoring and treatment of congenital hepatic hemangiomas (CHH) will depend on the subtype and the postnatal clinical behavior. Our aim is to present a series of CHH and characterize its clinical, histologic and genetic correlation, compared to cutaneous congenital hemangiomas (CCH). MATERIAL AND METHODS: A retrospective review of CHH patients diagnosed between 1991 and 2018 was performed. Clinical, morphological and histological data were analyzed and deep high-throughput sequencing was performed. MAIN RESULTS: Sixteen patients with CHH were included. Five patients were followed up with serial ultrasounds while pharmacological treatment (corticosteroids and propranolol) was decided in five. Surgical resection was performed in five owing to hemorrhage and suspicion of malignancy, and the last patient underwent embolization. Histologic analysis was available in 7 patients and confirmed CHH, showing two different histological patterns that could be associated with the presence of somatic pathogenic variants in GNAQ and/or PIK3CA detected in the genetic testing. Review of 7 samples of CCH revealed some histologic differences compared to CHH. CONCLUSION: CHH resemble its cutaneous homonym with similar clinical behavior. Histologic analysis can differentiate two subgroups while genetic testing can confirm mutations in GNAQ and in PIK3CA in a subset of CHH. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: IV.

Reliability of the Hirschsprung-Associated Enterocolitis Score in Clinical Practice.

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.

Long-Term Results after Diversion Surgery in Extrahepatic Portal Vein Obstruction.

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.

Prognostic Factors for Liver Transplantation in Unresectable Hepatoblastoma.

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.

Nuss Procedure for a Patient with Negative Haller Index.

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.

Long-term results of surgery for bowel lengthening: how many transplants are avoided, for which patients?

PURPOSE OF REVIEW: One of the biggest successes of intestinal rehabilitation programs is that more patients achieve enteral autonomy without transplantation. Many factors are responsible of this accomplishment including new parenteral formulas, better catheter management, surgical management, and the experience of the teams. The purpose of this review is to analyze recent published papers regarding intestinal lengthening procedures trying to find out how many transplantations are avoided and for which patients. RECENT FINDINGS: A trend towards performing less intestinal transplants has been identified in the last years. The general improvement of intestinal rehabilitation accounts for this step forward. However, the role of intestinal lengthening has not been clarified. SUMMARY: Surgical techniques for autologous reconstructive surgery are not limited to bowel lengthening. Longitudinal intestinal lengthening and tailoring and serial transverse enteroplasty offered good results in terms of intestinal adaptation, long-term survival, and subsequent need of intestinal transplantation. In recent series, less than one quarter of patients who underwent intestinal lengthening required salvage intestinal transplantation.

Advantages of Cardiac Magnetic Resonance Imaging for Severe Pectus Excavatum Assessment in Children.

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.

Pitfalls in Diagnosis of Early-Onset Inflammatory Bowel Disease.

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.

Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus.

Background Kaposiform hemangioendothelioma (KHE) is a vascular tumor frequently associated with Kasabach-Merritt phenomenon (KMP), characterized by severe thrombocytopenia and consumptive coagulopathy. Visceral involvement in KHE is rare. In our recent experience, sirolimus has shown to be an effective treatment in cutaneous KHE, becoming indeed the treatment of choice in KMP. We report a case of pancreatic KHE associated with KMP and refractory to sirolimus. Case Report A 4-month-old infant is referred for obstructive jaundice (10 mg/dL conjugated bilirubin) secondary to vascular pancreatic tumor. Magnetic resonance (MR) and immunohistochemistry were compatible with KHE, but the tumor was considered unresectable. We initiated sirolimus (0.8 mg/m 2 /12 h) to treat KMP, and interventional radiology was performed for percutaneous biliary diversion. This procedure prompted KMP (platelets: 51,000/µL). Sirolimus treatment for 7 days showed no effect; therefore, we started our VAT protocol (vincristine/aspirine/ticlopidin) with great response after 10 days (platelets: 3,70,000/µL). Three months later, percutaneous biliary diversion was replaced by a biliary stent. The tumor disappeared leaving fibrosis and dilatation of biliary tract needing hepaticojejunostomy 6 months later. Discussion It is difficult to establish protocols for an unusual presentation of a tumor with different targets. This is a reason collaborative multicenter studies should be performed. Management of obstructive jaundice secondary to a tumor that usually regresses in 10 years is an added challenge; therefore, the management should be led by a multidisciplinary team. Sirolimus treatment in cutaneous KHE has been described as successful in the literature, as well as in our own experience; however, it failed in our first patient with visceral KHE. We need to investigate the different response to pharmacological agents in tumors with similar histopathology, but with visceral involvement.

Portal Cavernoma in the Era of Mesoportal Shunt (Rex) and Liver Transplant in Children.

OBJECTIVE: The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO. MATERIAL AND METHODS: A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time. RESULTS: Of the 73 children with EHPVO, 39 were operated (12 posttransplant and 27 idiopathic). The median age at surgery was 9.36 years (range, 1.60-17.42 years). The MPS was the technique of choice; it was offered in 21 patients but eventually could be performed in only 18 (9 posttransplant and 9 idiopathic). The results of MPS were better in idiopathic (just one thrombosis successfully converted into mesocaval bypass). In the MPS after LT (n = 9), six shunts are permeable, two became thrombotic (one patient requiring retransplantation), and one late thrombosis occurred and the patient died eventually because of gastrointestinal bleeding. In the remaining patients (21 out of the 39), MPS was not performed because of intrahepatic cavernoma, portal vein hypoplasia, or insufficient length of vascular graft (especially in left lateral segment graft LT, since the portal vein is usually located in the right upper quadrant). They underwent 10 distal splenorenal shunts (DSRS) (1 posttransplant and 9 idiopathic), 5 proximal splenorenal shunts (PSRS) (1/4), 6 mesocaval shunts (1/5), and 1 modified Sugiura procedure. The results with DSRS have been favorable (one thrombosis, converted into mesocaval bypass). In the PSRS no thrombosis was identified and in the mesocaval shunt one early thrombosis occurred. Posttransplantation group had higher risk of shunt thrombosis, regardless of the surgical technique (chi-square, 0.021). The total increase of platelets after 6 months was not different in MPS as compared with other surgical techniques (analysis of variance, 0.110). CONCLUSIONS: The MPS is the technique of choice in EHPVO for idiopathic thrombosis as well as secondary to LT. Not all cases are favorable for MPS, so the surgeon must consider the possibility of alternative techniques for EHPVO. The results in terms of shunt patency are much better in idiopathic cavernoma that posttransplant patients.

Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient.

INTRODUCTION: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition. MATERIAL AND METHODS: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given. RESULTS: According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt. CONCLUSIONS: Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers.