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The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years. We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. Twenty seven patients (male/female 5/22), median age 13 days (1–1530) presented with hepatomegaly (24/27), cardiac failure (10/27), cutaneous hemangiomas (8/27), fever and anemia (6/27 each), vomiting (5/27), splenomegaly (4/27). The lesion was focal, multifocal, or diffuse in 9 patients of each group. The management included medical treatment (8/27), embolization (8/27), resection (3/27), observation (6/27), transplantation (2/27). After 16 months’ follow-up (30 days–11 years), 23/27 (85%) were alive. Diffuse lesions (4/4), cardiac failure (4/4), type II histology (4/4), age older than 6 months at diagnosis (3/4) predicted mortality (all p

Background: Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT). Methods: Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (

Background: Despite the fact that Kasai portoenterostomy (KPE) is the primary treatment for biliary atresia (BA), liver transplantation (LT) remains the ultimate surgery for two-thirds of these patients. Their true survival rate with the native liver reflects the original KPE and the burden of post-operative complications. We report an original modification of the adhesion-sparing liver eversion (ASLE) technique during KPE that facilitates the total native hepatectomy at time of transplantation. Methods: All consecutive patients with BA who underwent KPE at our department and subsequent LT at Paediatric Liver Transplant Centre at Papa Giovanni XXIII Hospital between 2010–2018 were retrospectively enrolled. All patients underwent ASLE during KPE. Patients’ demographic data, type of KPE, total transplant time (TTT), hepatectomy time (HT), intra-operative packed red blood cells and plasma transfusions, intra- and post-operative complications were noted. Results: 44 patients were enrolled. Median TTT and HT were 337 and 57 min, respectively. The median volume of packed red blood cell transfusion was 95 mL. No patients presented bowel perforation during the procedure or in the short post-operative course. No mortality after LT was recorded. Conclusions: In addition to the well-known advantages of the standard liver eversion technique, ASLE reduces the formation of intra-abdominal adhesions, lowering significantly the risk of bowel perforation and bleeding when liver transplantation is performed for failure of KPE.

Colostomy is considered by most authors the first step of treatment for neonates affected by high or intermediate anorectal malformations (ARMs). However, for this subset of patients, the debate between total diverting colostomy (TDC) and loop colostomy (LC) is still ongoing among the paediatric surgeons community. The aim of this paper is to present our experience consisting in applying the Brooke technique, until now used only for ileostomy, in order to perform a functionally diverting loop colostomy (FDLC) in patients with high or intermediate ARMs. At our institution, from January 2014 to December 2018, 12 patients (6 males and 6 females) with high or intermediate ARMs underwent colostomy according to the Brooke ileostomy technique. The only complication observed was mild proximal stoma prolapse in 1 patient. Creation and closure of the stomas were easy, and the final cosmetic result was very satisfying in all patients. We believe that this is a promising technique since it might overcome the drawbacks of a TDC as it is less invasive, easier, and quicker to perform. Furthermore, it guarantees final better cosmetic results, albeit functioning as a TDC.

Introduction The use of peripherally inserted central catheters (PICCs) in children is increasingly common due to their certain advantages and low rate of adverse events. Valved PICCs, when displaced, must be removed and new a PICC must be placed in another vein. We present our PICC replacement technique over a Seldinger guidewire using the same entrance vein. Methods This prospective study involved all consecutive patients with a previously inserted Groshong PICC requiring replacement due to displacement managed at our institution between January 2018 and January 2019. All procedures were performed at the patient's bed-side, under local anesthesia. All the steps of the procedure were described. Results The procedure was performed in 19 patients (mean 10.4 years, range 5-18 years) in a mean time of 12 minutes (range 10-35). In all cases, the maneuver was quick and painless. No complications were encountered during the procedure and at follow-up. Conclusions Our preliminary experience suggests that the PICC replacement technique using the same entrance vein is simple, safe and feasible, and provides clear benefits in order to preserve children's venous patrimony.

Introduction: The aim of our study was to present our experience with the use of peripherally inserted central catheters (PICCs) in pediatric patients receiving autologous or allogenic blood stem-cell transplantation. The insertion of the device in older children does not require general anesthesia and does not require a surgical procedure. Methods: From January 2014 to January 2017, 13 PICCs were inserted as a central venous device in 11 pediatric patients submitted to 14 autologous or allogeneic stem-cell transplantation, at the Bone Marrow Transplant Unit of the Children’s Hospital of Brescia. The mean age of patients at the time of the procedure was 11.3 years (range 3-18 years). PICCs remained in place for an overall period of 4104 days. All PICCs were positioned by the same specifically trained physician and utilized by nurses of our stem-cell transplant unit. Results: No insertion-related complications were observed. Late complications were catheter ruptures and line occlusions (1.2 per 1000 PICC days). No rupture or occlusion required removal of the device. No catheter-related venous thrombosis, catheter-related bloodstream infection (CRBSI), accidental removal or permanent lumen occlusion were observed. Indications for catheter removal were completion of therapy (8 patients) and death (2 patients). Three PICCs are currently being used for blood sampling in follow-up patients after transplantation. Conclusions: Our data suggest that PICCs are a safe and effective alternative to conventional central venous catheters even in pediatric patients with high risk of infectious and hemorrhagic complications such as patients receiving stem-cell transplantation.

Highlights • Hypothermic cardiopolmunary bypass is safe for prolonged total vascular exclusion. • Ante situm liver resection is feasible for hepatoblastoma considered unresectable. • Inferior vena cava replacement with aortic graft from cadaveric donor is feasible. • Preoperative and intraoperative assessment are essential to achieve good outcome., Introduction Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear. Presentation of case A 11-months-old boy was referred for a right hepatic lobe mass(90 × 78 mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795 IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61 × 64 mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft. Conclusions We report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.

Study Objective In a pediatric setting, laparoscopic management of large cystic ovarian neoplasms with low malignancy probability is not suitable, because of the mass size; nevertheless, an effort should be made to be as minimally invasive as possible, without violating the principles of oncologic surgery. We describe our experience in managing these neoplasms with leak-proof extracorporeal drainage through mini laparotomy, followed by cyst excision or oophorectomy. Design Case series study, describing interventions and outcomes. Setting Department of pediatric surgery in a tertiary pediatric and adult university hospital. Participants Pediatric patients affected by large cystic ovarian mass. Interventions Hybrid minimally invasive approach using leak-proof extracorporeal drainage. Main Outcome Measures Data on demographic characteristics, tumor marker values, and imaging findings were collected and analyzed. Outcome of surgical technique was evaluated and reported. Results Between 2011 and 2018, 17 patients (mean age, 10.2 years; range, 2-14 years) affected by large cystic ovarian mass, were eligible for this technique. All patients had negative preoperative tumor markers. Of the seventeen subjects, 13/17 girls (76%) underwent pelvic magnetic resonance imaging. No sign of lymphadenopathy or metastasis was found. Surgery was successful in all patients, with ovarian preservation in 5/17 cases (29.4%). Mean surgical time was 98 minutes; no intra-abdominal leakage of neoplasm content or postoperative complications occurred. Mature cystic teratoma was the most frequent histopathological diagnosis (71%). Conclusion After a thorough patient selection, the management of large cystic ovarian neoplasms with leak-proof extracorporeal drainage performed through a mini laparotomy is a feasible and safe approach, with excellent cosmetic results. When achievable, ovarian-sparing surgery has to be considered.

Introduction: The management of primary spontaneous pneumothorax (PSP) in children still remains controversial. The current literature shows a lack of scientific evidence supporting a standardized management. Materials and Methods: A multicenter retrospective study was performed, patients admitted for PSP in five Pediatric Surgical Units over a 9-year period (from 2008 to 2017) were included. Patient data were reviewed for each case. Management and outcomes were statistically analyzed. Results: Overall 159 patients (135 males and 24 females) were included in this study. During the first hospital admission, 62/159 patients (39%) were conservatively managed with oxygen therapy; 95/159 patients (60%) required a chest drain insertion; 2/159 patients (1%) underwent surgery because of a bilateral pneumothorax. Thoracoscopy was performed in 75/159 (47%) patients after the second hospital admission or for persistent air leak during the first access. Postoperatively, 8/75 (11%) patients developed recurrent pneumothorax requiring chest drain insertion in 3/8 (37%) cases or aspiration in 1/8 (13%), whereas 4/8 (50%) patients were conservatively managed. All of the patients but one, were confirmed to be affected by emphysematous-like changes at histology. Conclusions: Despite the limitations of being a retrospective study, we suggest that the early surgical management in children with PSP is feasible and safe and it seems to significantly reduce the recurrence rate. To the best of our knowledge there are no pediatric guidelines for the management of PSP, therefore, we support the need for prospective studies to create the evidence-based pillars for correct and standardized management of this condition.

Minimally invasive surgery (MIS) is now common in the paediatric age group, again highlighting the relevance of ‘clean’ retrieval of surgical specimens. Since disposable tissue bags can be expensive, we have developed a low-cost method for specimen retrieval using a modified finger from a surgical glove. We have used this technique mostly for laparoscopic appendectomy. There was no spillage of contents or surrounding tissue contamination and all wounds healed well without any infection from exit wound. There was no break in the glove or dispersion of glove fragments within the peritoneum. We believe that the Reverse Glove-finger Technique is a safe, quick, easy and low-cost technique that is freely available to all, for retrieval of small specimens during MIS procedures.

BACKGROUND Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. METHODS A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (

Bowel dilatation is a common adaptive mechanism after intestinal resection. The symptomatic dilated dysmotile duodenum is difficult to manage, since conventional bowel tailoring and lengthening techniques are potentially hazardous because of the anatomy of the duodenal blood supply, the proximity to the pancreas, and the risk of injury to the common bile duct. A 2-month-old child with short bowel and a symptomatic massively dilated duodenum was treated with a Transverse Flap Duodenoplasty (TFD). The duodenum was opened longitudinally along its antimesenteric border preserving an intact strip of tissue overlying the pancreatic head. Three full thickness vascularized pedicle flaps were cut on both the anterior and posterior walls and were spirally rotated and sutured to create a uniform propulsive duodenum without diverticulae. Healing was complicated by a soft anastomotic duodeno-ileal stenosis that resolved after three elective balloon dilatations. Oral feeding established rapidly. The child is growing, does not vomit, and passes 1–2 semiformed motions daily. TFD is a safe and versatile technique that preserves all duodenal absorptive mucosa and that removes any risk to the pancreas, bile duct, and ampulla of Vater. Our experience, although limited, has been encouraging and leads us to suggest TFD for the management of the difficult symptomatic dysmotile dilated duodenum.

Wandering spleen is a condition in which an incomplete fusion of the splenic ligaments allows the spleen to move within the abdomen, predisposing it to splenic torsion along its vascular pedicle. Torsion of a wandering spleen is an uncommon occurrence, especially in children, and associated torsion of the distal pancreas is even more unusual, with only four cases having been reported in adults. Non-specific clinical presentation makes radiologic evaluation essential in order to obtain a diagnosis and to send the patient for early surgery before life-threatening complications arise. Here we present a rare case of torsion of wandering spleen together with volvulus of the distal pancreas in a 13-year-old girl. In this case, prompt radiological assessment allowed for an early diagnosis, and the patient was successfully treated with urgent laparoscopic derotation of both the spleen and the distal pancreas as well as mesh splenopexy. To the best of our knowledge, this procedure has never been described in a pediatric setting.

Background and aims Duodenal atresia is a relatively common anomaly and its frequency ranges from 1 in 3000 to 5000 live births. Association of duodenal atresia with situs viscerum inversus is rare and fewer than 20 patients have been reported in literature. Methods We present a sub-Saharan 1 years old female baby, born at 31 weeks with prenatal diagnosis of situs viscerum inversus with dextrocardia, ventricular septal defect, ductus venous agenesis, duodenal atresia and intestinal malrotation. We considered risk factors, clinical and surgical management, complications and evolution of this rare association. Results A 31 weeks female baby was born by emergency caesarean section for cardiotocographic changes. Her mother history was positive for pre-gestational diabetes on insulin therapy and for Streptococcus beta-haemolytic infection. Incomplete intrapartum amplicillin prophylaxis was made. The baby weighed 2300 g and her Apgar scores were 5 and 5, needing non-invasive ventilation with nCPAP. Because of suspected intestinal obstruction, parental nutrition was started one day after birth and a radiological examination was performed showing the mirror image of a double-bubble configuration of duodenal atresia. She underwent laparotomy at 6 days of life, confirming situs inversus and intestinal necrosis secondary to volvulus on common mesentery (apple peel type 3b): duodeno-cecal anastomosis with saving the ileocecal valve was performed. Histology of the surgical specimen confirmed the transmural necrosis. Six days after, enteral nutrition was progressively introduced with good tolerance and weight gain; parental nutrition was continued with subsequent cholestasis needing the modification of the lipid composition. At 3 months of age, she underwent a Bianchi intestinal lengthening procedure and cholecystectomy. The onset of post-surgical duodenal stenosis two months after required one endoscopic pneumatic dilation (8–10 mm) and a gastrostomy button was placed too. Upper endoscopies performed 1 month and 6 months later were normal. Enteral and parental nutrition was continued and the oral feeding was gradually introduced. Conclusions Duodenal atresia and situs inversus are rare combinations. Outcome is good with appropriate multidisciplinary management and follow-up. Maternal diabetes could be factor risk of laterality, as suggested in literature.

We report a child with post-surgical short bowel state who underwent bowel expansion followed by spiral intestinal lengthening and tailoring (SILT) at 10 months of age. Growth at 1-year follow-up is along the 15-25th centile on 82 % oral calories as normal diet and 18 % as parenteral nutrition, and he is passing 2-3 semisolid motions daily. SILT is a versatile technique for reconstructing dilated bowel towards improved propulsion and absorption, and has a role in the management of the short bowel state.

Fig 1. Abdominal radiograph showing massive dilatation of the whole colon, without signs of intestinal perforation. A 10-YEAR-OLD GIRL was evaluated at the Emergency Department because of a 2-day history of abdominal distension with pain and nonbilious vomiting. She had a 5-year history of chronic constipation, previously diagnosed as functional constipation. On physical examination, her abdomen was massively distended with voluntary guarding. Laboratory evaluation showed an elevated C-reactive protein (24 mg/dL, normal range 0–5 mg/dL). Abdominal radiograph showed a massively dilated colon (Fig 1). A nasogastric tube was placed, and a water-soluble contrast enema was obtained (Fig 2). The enema showed the “twisted taper” or “bird’s beak” configuration of the twisted colon, in which the dilated proximal segment represents the bird’s head, and the tapered distal end its beak. These findings were consistent with the diagnosis of sigmoid volvulus. After an unsuccessful attempt at endoscopic decompression and derotation, the patient underwent an emergency exploratory operation. At laparotomy, 1808 counterclockwise torsion of the sigmoid colon was confirmed. The dilated sigma had a maximal diameter of 17 cm, without sign of ischemia or necrosis. Colonic irrigation with saline solution was performed, and the patient underwent derotation and resection of 25 cm of dilated sigma and primary end-to-end colocolonic anastomosis with sigmoidopexy. Sigmoid volvulus is extremely rare in children and is usually associated with a long-standing history of constipation or pseudo-obstruction. In these patients, redundant sigmoid colon could

We assessed whether oxidant-stress and inflammation in beta-thalassemia could be controlled by the novel oral iron chelator deferasirox as effectively as by deferoxamine.Forty-nine subjects were enrolled from seven sites and studied at baseline, and after 1, 6, and 12 months of therapy. Malondialdehyde, protein carbonyls, vitamins E and C, total non-transferrin bound iron, transferrin saturation, C-reactive protein, cytokines, serum ferritin concentration and liver iron concentration were measured.Liver iron concentration and ferritin declined significantly in both treatment groups during the study. This paralleled a significant decline in the oxidative-stress marker malondialdehyde (deferasirox -22%/year, deferoxamine -28%/year, average decline p=0.006). The rates of decline did not differ between treatment groups. Malondialdehyde was higher in both treatment groups than in a group of 30 non-thalassemic controls (p0.001). The inflammatory marker high-sensitivity C-reactive protein decreased significantly only in the group receiving deferasirox (deferasirox -51%/year, deferoxamine +8.5%/year, p = 0.02). This result was confounded by a chance difference in the level of high-sensitivity C-reactive protein between the two groups at baseline, but analyses controlling for this difference suggested an equally large treatment effect.Iron chelation therapy with deferoxamine or with deferasirox was equally effective in decreasing iron burden and malondialdehyde. The possible differential effect of the two chelators on inflammation warrants further investigation.

Aim of the study The management of newborns with esophageal atresia (EA) and right aortic arch (RAA) is still an unsolved problem. This study provides a systematic review of epidemiology, diagnosis, management and short-term results of children with EA and RAA. Materials and methods The PubMed database was searched for original studies on children with EA and RAA. In each study, data were extracted for the following outcomes: number of patients, associated anomalies, type of surgical repair, morbidity and mortality rate. Results Eight studies were selected, including 54 patients with EA and RAA. RAA was encountered in 3.6% of infants. Preoperative detection of RAA was reported in 7 of them. In these patients, primary anastomosis was achieved through the right approach in 3 (thoracotomy in 2 and thoracoscopy in 1) while the left approach was the primary choice in 4 (thoracotomy in 2 and thoracoscopy in 2). No significant differences were found between the right and left approaches with regard to leaks (P = 0.89), strictures (P = 1) or mortality (P = 1). In 47/54 patients (87%) RAA was noted during right thoracotomy, and primary anastomosis was achieved through the same approach in 29 (61.7%); conversion to other approaches (left thoracotomy or esophageal substitution) was performed in 15 children (38.3%). No significant differences were found between primary left thoracotomy (LT) and LT after RT with regard to leaks (P = 0.89), strictures (P = 1) or mortality (P = 1). Conclusions Skills and preferences of the surgeon still guide the choice of surgical approach even when preoperatively faced with RAA. A multicenter, prospective randomized study is strongly required.

To study that inflammatory fibroid polyps (IFPs) in children are extremely uncommon tumors that may occur throughout the gastrointestinal tract.A systematic review of the pediatric literature and a report of a new case of IFP is also presented. The PubMed database was searched for original studies on pediatric IFPs since 1960, according to "Preferred reporting items for systematic reviews and meta-analyses" guidelines for systematic reviews.Five studies were finally enclosed, encompassing 6 children with IFPs (mean age 64 mo). Tumors were located in the stomach (2 patients), in the small bowel (2 patients), in the rectum (1 patient) and in the colon (1 patient). Open surgery was performed in all patients and complete excision of the mass was achieved in all cases. All patients are alive and free of symptom. Authors described a further case of a 3-year-old boy with a large duodenal IFP, in whom the tumor was removed by "en block resection". The presence of IFP throughout the gastrointestinal tract and its variable clinical appearances make it difficult to diagnose. An accurate pre-operative assessment is fundamental in order to differentiate IFP from other more aggressive gastrointestinal tumor, enabling unnecessary demolitive surgery.When complete resection of the IFP is achieved, the prognosis is excellent.

Two infants affected by intestinal vascular anomalies causing lower gastrointestinal hemorrhage are reported. Upper and lower gastrointestinal tract endoscopy and radionuclide scan were negative. The authors found laparoscopic exploration very useful in detecting the cause and the site of bleeding. Because the usual investigations for gastrointestinal bleeding have been proven to be time-consuming and often unreliable in small infants, the authors suggest considering laparoscopic exploration as a first-line diagnostic tool in this subset of patients. A new diagnostic algorithm for gastrointestinal bleeding in infants is proposed.

Transfusional iron overload is a potentially fatal complication of the treatment of thalassaemia. We aimed to investigate short-term efficacy, pharmacokinetic/pharma- codynamic (PK/PD) relations, and safety of ICL670, a novel, tridentate, orally active iron chelator.We enrolled 24 patients and divided them into three cohorts consisting of a minimum of seven individuals. Patients were admitted to a metabolic unit and consumed a diet with a defined content of iron. Two patients in each cohort were randomly allocated placebo. Five or more patients received one daily dose of ICL670 at 10, 20, or 40 mg x kg(-1) x day(-1), from day 1 to 12. Net iron excretion (NIE) was measured between days 1 and 12. Primary objectives included assessment of safety and tolerability (measured by adverse events and clinical laboratory monitoring), pharmacokinetics (measured as drug and drug-iron complex), and cumulative net iron excretion (measured by faecal and urine output minus food input). Analysis was for efficacy.ICL670 was absorbed promptly and was detectable in the blood for 24 h. Exposure (area under the curve of plasma concentration) to ICL670 at pharmacokinetic steady state was proportional to dose. All three doses resulted in positive NIE. The NIE achieved at 20mg x kg(-1) day(-1) would prevent net iron accumulation in most patients transfused with 12-15 mL packed red-blood-cells kg(-1) month(-1), equivalent to 0.3-0.5 mg iron kg(-1) x day(-1). A linear relation (PK/PD) was recorded between exposure to ICL670 and total iron excretion, by contrast with placebo (r2=0.54, p0.0001). Skin rashes were noted in four patients treated at 20 and 40 mg x kg(-1) x day(-1), and one patient also developed grade 2 transaminitis.ICL670 given once daily at 20 mg/kg seems to be an effective orally active iron chelator and is reasonably well tolerated. Long-term studies are now necessary to establish the practical contribution of this drug.

Summary Objective To evaluate the functional and cosmetic result of modified-triple incision preputial plasty for surgical management of phimosis in children. Materials and methods This prospective study involved all consecutive children (age 3–15) with phimosis, managed at our Institution in a one–year period. All patients unable to retract the foreskin and symptomatic with episodes of posthitis or balanoposthitis or ballooning of the foreskin with voiding were initially included. After a two-month trial of topic corticosteroids treatment, for patients refractory to conservative ointment treatment, “trident” preputial plasty was offered. This procedure combines two lateral Z-plasty and one middle Y–V plasty. All the procedures were performed as a day care, in general anaesthesia by two senior surgeons. Outpatient follow-up controls were scheduled at 1 and 2 week, 1, 6 and 12 months after surgery, respectively. Results A total of 41 patient were enrolled. Mean operative time was 24 minutes (range 15–43). At 12-month follow-up, all but one patients (97.6%) was able to retract the prepuce. Cosmetic and functional results were satisfactory. Conclusion In selected group of children with phimosis, the “trident” preputial plasty provided excellent cosmetic and functional result. Adequate retraction of the prepuce by the patients soon after surgery is mandatory.

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Esophageal atresia is a relatively common congenital malformation occurring in 1:3000-4500 live births. Improvement in surgical, anesthetic and neonatal care has achieved a survival rate near 100% in infants weighing over 1500 g and having no major cardiac problem. To achieve these outcomes, as well as a good surgical technique, careful preoperative management (early diagnosis, investigation of associated anomalies, suction of the upper pouch, prevention and treatment of gastric and bowel distension) and accurate postoperative care (postoperative analgesia and ventilation, management of trans-anastomotic and chest tube, prevention, early recognition and treatment of complications) are mandatory.

Background Imatinib mesylate is the first line treatment for chronic myeloid leukemia. In patients with advanced phase of the disease, the advent of imatinib significantly increased survival. However, few long-term data, based on large, prospective and controlled trials are available on the outcome of these patients. Design and Methods We conducted a phase II trial of imatinib 600 mg daily in patients with chronic myeloid leukemia in blast crisis. The return to chronic phase was defined as

Preoperative tracheobronchoscopy (TBS) in the diagnostic assessment of newborns affected by esophageal atresia (EA) was described in 1981. Nevertheless, the value of the procedure is actually much debated; only a few studies have clearly explored the advantages of TBS and this procedure is not yet routinely included in the diagnostic and therapeutic assessment in many international pediatric surgery settings. Routine preoperative TBS is a safe procedure that enables the accurate examination of the tracheobronchial tree, the visualization of tracheoesophageal fistula and the diagnosis of tracheomalacia or associated respiratory anomalies. When a distal fistula is found, its occlusion with a Fogarty balloon catheter improves mechanical ventilation and facilitates surgical repair. This review provides a detailed overview on the use of TBS in newborns with EA, focusing on technical aspects, anesthesiological management, indications and limits. The benefits and risks of the procedure are also compared with alternative diagnostic tools, such as an esophageal contrast study, computed tomography scan and ultrasound.

BACKGROUND:: The management of extrahepatic portal vein obstruction (EHPVO) in children is controversial. We report our experience with a prospective evaluation of a stepwise protocol based on severity of portal hypertension and feasibility of mesoportal bypass (MPB). METHODS:: After diagnosis, children with EHPVO underwent surveillance endoscopies and received nonselective β-blockers (NSBBs) or endoscopic variceal obliteration (EVO) when large varices were detected. In patients who failed NSBBs and EVO, we considered MPB as first-line and shunts or transjugular intrahepatic portosystemic shunt (TIPS) as second-line options. RESULTS:: Sixty-five children, median age 12.5 (range 1.6-25.8), whose age at diagnosis was 3.5 (0.2-17.5) years, were referred to our unit. Forty-three (66%) had a neonatal illness, 36 (55%) an umbilical vein catheterisation. Thirty-two (49%) presented with bleeding at a median age of 3.8 years (0.5-15.5); during an 8.4-year follow-up period (1-16), 43 (66%) had a bleeding episode, 52 (80%) were started on NSBBs, 55 (85%) required EVO, and 33 (51%) required surgery or TIPS. The Rex recessus was patent in 24 of 54 (44%), negatively affected by a history of umbilical catheterisation (P=0.01). Thirty-four (53%) patients underwent a major procedure: MPB (13), proximal splenorenal (13), distal splenorenal (2), mesocaval shunt (3), TIPS (2), and OLT (1). At the last follow-up, 2 patients died, 53 of 57 (93%) are alive with bleeding control, 27 of 33 (82%) have a patent conduit. CONCLUSIONS:: Children with EHPVO have a high rate of bleeding episodes early in life. A stepwise approach comprising of medical, endoscopic, and surgical options provided excellent survival and bleeding control in this population.

Children affected with chronic granulomatous disease develop severe infections, which frequently evolve to abscess. In most instances the liver is involved. We report a case of liver abscess successfully treated by percutaneous transhepatic alcoholization after antibiotic therapy and percutaneous drainage had failed. This procedure avoided the need for hepatic resection, which is associated with high morbidity in such patients.

We report successful fluoroscopically guided balloon dilatation of an early postoperative stricture of a jejunoileal anastomosis in a 3-month-old infant who underwent serial transverse enteroplasty for type IIIb apple peel intestinal atresia.

Sigmoid volvulus (SV) is extremely uncommon in children and is usually associated with a long-standing history of constipation or pseudo-obstruction. An early diagnosis and management are crucial in order to prevent the appearance of hemorrhagic infarction of the twisted loop, avoiding further complications such as necrosis, perforation and sepsis. In patients with no evidence of peritonitis or ischemic bowel, treatment starts with resuscitation and detorsion of the SV, accomplished by means of sigmoidoscopy and concomitant rectal tube placement. The bowel is then prepared and surgery is undertaken electively during the same hospitalization. We report a detailed review of the literature focusing on technical details, risks and benefits of endoscopic management of SV in childhood.

Congenital tracheal stenosis is a rare disorder, characterized by the presence of focal or diffuse complete tracheal cartilage rings, resulting in a fixed tracheal narrowing. The entity may be seen in isolation or in conjunction with other malformations that include an anomalous right upper lobe bronchus. We here report the case of a 12-month-old girl who had severe bi-segmental congenital tracheal stenosis with the interposition of a tracheal bronchus between the two stenotic segments.

Deferasirox is a once-daily, oral iron chelator developed for treating transfusional iron overload. Preclinical studies indicated that the kidney was a potential target organ of toxicity. As patients with sickle cell disease often have abnormal baseline renal function, the primary objective of this randomised, open-label, phase II trial was to evaluate the safety and tolerability of deferasirox in comparison with deferoxamine in this population. Assessment of efficacy, as measured by change in liver iron concentration (LIC) using biosusceptometry, was a secondary objective. A total of 195 adult and paediatric patients received deferasirox (n = 132) or deferoxamine (n = 63). Adverse events most commonly associated with deferasirox were mild, including transient nausea, vomiting, diarrhoea, abdominal pain and skin rash. Abnormal laboratory studies with deferasirox were occasionally associated with mild non-progressive increases in serum creatinine and reversible elevations in liver function tests. Discontinuation rates from deferasirox (11·4%) and deferoxamine (11·1%) were similar. Over 1 year, similar dose-dependent LIC reductions were observed with deferasirox and deferoxamine. Once-daily oral deferasirox has acceptable tolerability and appears to have similar efficacy to deferoxamine in reducing iron burden in transfused patients with sickle cell disease.

We evaluated safety and efficacy of imatinib (600 mg) in 36 c-KIT+ acute myeloid leukemia patients not amenable to receive conventional chemotherapy. No patient achieved complete remission. One patient obtained a hematologic improvement (platelet increase with transfusion independence). Median overall survival was 3 months (0.5-44+). Non-hematologic toxicity was overall mild.

Alagille syndrome (AS) is a dominantly inherited, multisystem disorder involving the liver, heart, eyes, face, and skeleton. From October 1997 through July 2004, 260 pediatric orthotopic liver transplantations (OLTx) were performed in 231 patients. This report describes 21 patients of median age 1.95 years (range, 0.7-16.7) who had alagille syndrome. We present the technical features of the OLTx, incidence and type of complications, medical conditions related to the syndrome, need for retransplantation, as well as patient and graft survival rates. A split liver technique was used in 16 patients (76%) who received a left lateral segment (LLS) graft whereas 7 patients (33%) received a whole liver. Only cadaveric donors were used. The major surgical complications requiring reintervention in 11 patients (52%) included biliary problems (19%) and vascular complications (17%). One case of hepatic artery thrombosis required retransplantation. Three recipients (14%) died. All other patients are alive with an actuarial survival rate of 90% at 1 year and 80% at 5 years. The actuarial graft survival rate is 85% at 1 year and 75% at 5 years. Patients with AS, despite the associated cardiovascular anomalies, can be treated successfully by a combined approach between cardiologist, radiologist, cardiothoracic, and liver transplant surgeons. With careful planning and operative management, the results are comparable with those obtained with other more common cholestatic diseases.

In this study we analyzed the features of 12 patients who underwent liver transplantation for progressive familial intrahepatic cholestasis (Byler's disease [BD]) in view of the technical features of the OLTx, incidence and type of complications, need for retransplantation, as well as patient and graft survivals. BD was the indication in 12 patients of median age 1.32 years and median weight 10 kg. Median follow-up was 670 days. Major surgical complications requiring reintervention occurred in three patients. No thrombosis of the hepatic artery was observed. Infections with positive blood cultures were diagnosed in four patients. One patient had a biliary anastomotic stenosis successfully treated by percutaneous techniques. Four patients had episodes of acute rejection treated with steroids. Two patients were retransplanted, both of whom died in the early postoperative period due to hepatic vein thrombosis and venoenteric fistula. The actuarial patient and graft survival was 83% at 1 year and 83% at 5 years. Split-liver grafts represent an excellent organ supply for these patients, achieving good results with no mortality on the waiting list.

Early portal vein thrombosis (PVT) represents a serious complication after liver transplantation (OLTx). From October 1997 through July 2004, 260 OLTx were performed in 231 children, including 189 of left lateral segments (LLS). We retrospectively analyzed the incidence and the outcome of early PVT in this group. A daily doppler US scan was performed during the first week after transplantation. Early PVT occurred in 14 patients (8%), 10 males and four females of median age 0.77 years. The main indication for primary transplantation was biliary atresia (10), followed by Byler's disease (2), acute liver failure on cryptogenetic cirrhosis (1), and Alagille syndrome (1). Four children underwent retransplantation; three cases of thrombectomy and revision of the anastomosis, two children were treated with beta blockers, one of whom had a later failed attempt at percutaneous revascularization and eventually a meso-caval shunt. Five patients were followed with observation and no treatment. Among the four patients who died, three were in the retransplantation group and one in the thrombectomy and revision of the anastomosis group; the overall mortality was 28%. With a median follow up of 399 days, 10 patients are alive with an actuarial survival at 1 and 5 years of 72%, and graft survival rates at 1 and 5 years of 64%. PVT represents a serious complication after pediatric OLTx with LLS grafts. Prompt detection and aggressive surgical treatment in selected cases are required to reduce the mortality and graft loss.

Torsion of a lung or a lobe (LT) is a severe, sometimes life-threatening event that may occur spontaneously, after trauma, or after cardiac or thoracic surgery. The authors report on 2 prematurely born neonates who had LT after cardiac surgery. Both patients successfully underwent pulmonary lobectomy, which seems to be the best surgical approach. Given that careful anatomic unfolding of the lung and its reinflation under vision at the end of a cardiac or thoracic operation is deemed crucial to avoid LT, the authors suggest that, in case of a complete pulmonary fissure and/or free long bronchovascular pedicle, lobe fixation should be accomplished, too. Because of its rarity, we could find only 6 well-documented reports of LT diagnosed in children, whereas another 3 cases were quoted without clinical details. The pediatric literature is reviewed.

Background. End-stage cholestatic liver disease (ESCLD) is the main indication for liver replacement in children. Pediatric cadaver-organ-donor shortage has prompted the most important evolutions in the technique of liver transplantation, in particular living-donor liver transplantation (LDLT) and split-liver transplantation (SLT). Methods. Between November 1997 and June 2001, 127 children with ESCLD were evaluated for liver transplantation, and 124 underwent 138 liver transplantations after a median time of 40 days. Causes of liver disease were congenital biliary atresia (n=96), Alagille's syndrome (n=12), Byler's disease (n=8), and other cholestatic diseases (n=8). Results. Ninety (73%) patients received a split-liver graft, 28 (23%) a whole liver, and 6 (4%) a reduced-size liver. Overall 2- and 4-year patient survival rates were 93% and 91%, respectively; the 2- and 4-year graft-survival rates were 84% and 80%, respectively. In split-liver recipients, 4-year patient and graft-survival rates were 91% and 83%, respectively; these were 93% and 78%, respectively, in whole-liver recipients and 67% and 63%, respectively, in reduced-size liver recipients. Retransplantation rate was 11%, whereas mortality rate was 8%. Overall incidence of vascular and biliary complication were 16% and 27%, respectively. Conclusions. SLT can provide liver grafts for children with ESCLD with an outcome similar to the one reported following LDLT, eliminating mortality while they are on a transplantation wait list. The need for pediatric LDLT should be reevaluated and programs of SLT strongly encouraged and supported at a national and international level.

Objective: To achieve hepatic portal revascularisation and decompression of extrahepatic portal hypertension in children with cavernoma and obstruction caused by idiopathic portal vein thrombosis, Design: Selected cases. Setting: Teaching hospitals, Belgium and Italy. Subjects: 11 children who weighed between 5.9 and 54 kg (2 emergencies) with symptomatic extrahepatic portal hypertension. Intervention: Interposition of venous autograft between the superior mesenteric vein and the distal (umbilical) portion of the left portal vein. Main outcome measures: Improvements in symptoms and endoscopic appearance after operation. Results: 2 bypasses had to be redone because they stenosed; all 11 were patent at the time of writing (median follow-up 6 months, range 1-32 months). Conclusion: The bypass effectively relieved symptoms of extrahepatic portal hypertension by restoring normal hepatic portal blood flow.

The Wiskott-Aldrich syndrome (WAS) is a X-linked hematologic disorder characterized by thrombocytopenia, eczema, and immunodeficiency of variable severity. Reported here are the results of a morphologic, morphometric, and immunophenotypic analysis of splenic lymphoid tissue in 12 WAS patients with documented molecular defect and with different disease severity. Spleens from 29 age-matched patients with different diseases were used as controls. Paraffin-embedded tissue (from all cases) and fresh-frozen samples (from 5 WAS patients and 4 control subjects) were used to study the different white pulp compartments by classic morphologic, immunophenotyping, and image analysis techniques. Data were statistically analyzed by both parametric and nonparametric tests. Spleens from WAS patients showed a significant depletion of the total white pulp (p = 0.0008), T cell (p < 0.05), and B cell (p = 0.0002) areas and marginal zone (MZ) thickness (p < 0.0001). Among WAS patients, a negative correlation was found between the score of severity of the disease and all variables considered (Spearman's rank correlation coefficient, r = -0.79, r = -0.73, r = -0.68, and r = -0.56, respectively). In conclusion, this study shows that in WAS a general depletion of the splenic white pulp occurs, supporting the evidence that WAS is characterized by a combined immune defect. The significant reduction of the MZ may explain the inability of WAS patients to mount a response to T-independent antigens.

Adenomyomatosis of the gallbladder (ADMG) is defined as an acquired disease characterized by localized or diffuse hyperplastic extensions of the mucosa into, and often beyond, the thickened gallbladder muscular layer (Rokitansky-Aschoff's sinuses). In recent years, attention has been drawn to its malignant potential. The occurrence of ADMG has never been reported in children. The authors report the case of a 5-year-old boy with symptomatic ADMG, who was successfully treated by laparoscopic cholecystectomy.

Background: Decompression of extrahepatic portal hypertension by directly bypassing the thrombosed portal vein has never been reported in cases of children with idiopathic (or neonatal) portal vein obstruction and cavernoma. Methods: Seven children (15 years or younger) with portal vein obstruction requiring surgical decompression (urgently in two cases), and in whom preoperative Doppler had shown that the intrahepatic portal branches were hypoplastic but free of thrombus, were included in a pilot study. The cavernoma was bypassed by interposing a venous jugular autograft between the superior mesenteric vein and the distal portion of the left portal vein. Patients received follow-up using routine clinical parameters, upper gastrointestinal endoscopy, and Doppler ultrasound. Results: The mesenterico-portal bypass restored a direct (physiological) hepatopetal portal flow. The operation resulted in effective portal decompression as demonstrated by decrease of the pressure gradient, rapid regression of clinical signs of portal hypertension, and definitive control of bleeding. Conclusions: This study shows that direct bypassing of portal cavernoma is possible and results in effective portal decompression. Restoration of the hepatic portal flow is a major advantage compared with conventional surgical shunting procedures. This new technique is potentially applicable to two thirds of children with portal vein thrombosis and should be considered when shunting procedures are indicated. Copyright (C) 1998 by W.B. Saunders Company.

The oral bioavailability of Sandimmun can be impaired by cholestasis, external biliary diversion, and diarrhea. We report two cases of pediatric liver transplant recipients who experienced chronic rejection and diarrhea secondary to proximal bowel resection. These conditions resulted in poor oral absorption of Sandimmun; the children were converted to the new oral microemulsion formulation Neoral, which significantly improved oral absorption, allowing intravenous cyclosporine weaning and patient discharge. Comparative pharmacokinetic studies were performed in both cases, and the relative Neoral/Sandimmun bioavailabilities were 32.9 and 5.4, respectively. Accordingly, Neoral may constitute to good alternative to ensure the effectiveness of oral cyclosporine administration, particularly in liver-transplanted children with severe cholestasis or shortened small bowel.