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The resection of malignant tumors often poses the challenge of reconstruction. The objective of this reconstruction is the restoration of a function. We present the case of a chondroblastic osteosarcoma of the ulna in a 27-year-old patient. The resection sacrificed part of the ulna. The conservation of part of the olecrane allowed an innovative reconstruction to be carried out by bringing the radial head into contact with the humeral trochlea. The preservation of elbow function was satisfactory.

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: 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.

Omphalopagus twins are joined ventrally in the umbilical region. In omphalopagus twins, liver's fusion is very frequent, being present in about 80% of cases. Two conjoined twins born in our Hospital were evaluated using plain x-rays, ultrasound (US), gastrointestinal xrays, Tc-99 hepatobiliary scintigraphy (SC) and contrast enhanced computed tomography (CT). There was no bony connection on conventional xrays, neither bowel communication on GI x-rays study. US demonstrated the fusion of left hepatic lobes and a not-quantifiable vascular shunt. Normal biliary function was demonstrated by Tc-99 SC. The first CT scan (twin A) showed irregular lobules in the site of hepatic fusion, a portoportal shunt and a venous vessel to the inferior vena cava of twin B. Only the second CT scan (twin B) showed an arterio-arterial partiallyextrahepatic shunts. The double CT scan allows to increase the probability of a correct individuation and description of vascular shunts for an effective preoperatory assessment. The omphalopagus twins in our case underwent planned surgical separation at 5 months of age. The surgery lasted 5 h and was not affected by any intraoperative complications.

Neuromyelitis optica spectrum disorder (NMOSD) is not defined as a classical paraneoplastic neurological syndrome, however there are growing evidences that NMOSD may be rarely associated with cancer. Older (>45 years old) male patients with longitudinally extensive transverse myelitis (LETM) or patients with “area postrema” syndrome (intractable vomiting and hiccups) at onset are at higher risk for neoplasm-associated NMOSD. We report the case of 79-years old man who developed, a month after radiotherapy for prostatic adenocarcinoma, an area postrema syndrome rapidly followed by a LETM involving the whole spinal cord (from C2 to the conus). Aquaporin-4-IgG antibodies were positive in serum.

Status Epilepticus (SE) is a condition resulting either from the failure of seizure termination mechanisms or from the abnormal activation of prolonged seizure mechanisms. It represents a life-threatening medical emergency associated with high morbidity and mortality that requires prompt diagnosis and treatment. SE treatment begins with a quick-acting benzodiazepine, followed by intravenous (IV) loading and a continuous infusion of antiseizure medications (ASMs). The failure of first- and second-line treatments is defined as “refractory SE” (RSE) and requires infusion of IV anaesthetics. If anaesthetics fail or SE recurs after 24 hours, SE is defined as “super refractory status epilepticus” (SRSE).

Highlights • Autoimmune encephalitis and status epilepticus in COVID-19 can be challenging. • Immunotherapy could be useful for anti-NMDAr encephalitis and SARS-Cov-2 infection. • Cytokines storm could play a role for NORSE presentation in NDMA-R encephalitis.

LGI1 encephalitis is an autoimmune disorder characterized by cognitive symptoms and seizures, which rarely respond to common antiepileptic drugs (AEDs). Rituximab (RTX) is a CD-20-depleting monoclonal antibody which has been used for the treatment of LGI1 encephalitis, however, its efficacy remains controversial. A 54-year-old woman came to our attention due to memory loss and gambling. Brain MRI revealed areas of bilateral hippocampal hyperintensity and LGI1 antibodies were found in both serum and cerebrospinal fluid. Immunotherapy with steroids was started, followed by intravenous immunoglobulins with partial improvement. The patient developed multiple generalized tonic-clonic seizures. She was then administered intravenous rituximab with significant improvement for both cognitive symptoms and seizure control. High-density EEG was recorded before treatment, seven days after the first dose and seven days after the second dose. Topoplot and power spectrum analysis were performed for each recording. Interictal epileptiform discharges, as well as theta power bands, were significantly reduced after each dose, while topoplot analysis showed reduced spreading over posterior and frontal electrodes for interictal epileptiform discharges of temporal origin. Our experience indicates that rituximab is a valid treatment for LGI1 encephalitis, demonstrating efficacy for both cognitive symptoms and seizure control. High-density EEG could represent a novel, safe and reproducible method to study epileptogenesis in autoimmune limbic encephalitis.

Introduction The risk of acquiring SARS-CoV-2 in a hospital setting and the need of reorganizing the Emergency Departments (EDs) to cope with infected patients have led to a reduction of ED attendances for non-infectious acute conditions and to a different management of chronic disorders. Methods We performed a retrospective study evaluating the frequency and features of ED attendances for seizures during the lockdown period (March 10th–April 30th 2020) in the University Hospital of Trieste, Italy. We studied the possible pandemic impact on the way patients with seizures sought for medical assistance by comparing the lockdown period to a matched period in 2019 and to a period of identical length preceding the lockdown (January 18th–March 9th 2020). Results A striking decrease in total ED attendances was observed during lockdown (4664) compared to the matched control (10424) and to the pre-lockdown (9522) periods. A similar reduction, although to a lesser extent, was detected for seizure attendances to the ED: there were 37 during lockdown and 63 and 44 respectively during the two other periods. Intriguingly, during the lockdown a higher number of patients attended the ED with first seizures (p = 0.013), and more EEGs (p = 0.008) and CT brain scans (p = 0.018) were performed; there was a trend towards more frequent transport to the ED by ambulance (p = 0.061) in the lockdown period. Conclusions Our data suggest that the pandemic has affected the way patients with seizures access the Health Care System.

Objectives Non-cirrhotic portal vein thrombosis (PVT) is a main cause of portal hypertension in children. We describe the characteristics at presentation and outcome of a cohort of patients with PVT to determine clinical features and predictors of outcome. Methods We recorded: (1) Associated factors: prematurity, congenital malformations, neonatal illnesses, umbilical vein catheterization (UVC), deep infections, surgery; (2) congenital and acquired prothrombotic disorders; (3) features at last follow up including survival rate and need for surgery. Results 187 patients, mean age at diagnosis 4 ± 3.7 years, had a history of prematurity (61%); UVC (65%); neonatal illnesses (79%). The diagnosis followed the detection of splenomegaly (40%), gastrointestinal bleeding (36%), hypersplenism (6%), or was incidental (18%). Of 71 patients who had endoscopy at presentation 62 (87%) had oesophageal varices. After 11.3 years’ follow up 63 (34%) required surgery or TIPS. Ten-year survival rate was 98%, with 90% shunt patency. Spleen size, variceal bleeding and hypersplenism at presentation were predictors of surgery or TIPS (p Conclusion PVT is associated with congenital and acquired co-morbidities. History of prematurity, neonatal illnesses and UVC should lead to rule out PVT. Large spleen, variceal bleeding and hypersplenism at presentation predict the need for eventual surgery in a third of cases.

Background: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia].Methods: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes.Results: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean.Conclusions: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO. (C) 2019 Elsevier Inc. All rights reserved.

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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.