Revision of meso-Rex bypass utilizing a collateral vein in a patient with portal steal phenomenon after liver transplant: A case report.

INTRODUCTION: In children with extrahepatic portal vein obstruction or those who develop portal vein thrombosis after liver transplant, the use of Meso-Rex Bypass (MRB) creates a more physiological state by redirecting mesenteric blood flow back into the intrahepatic portal system via a venous conduit. PRESENTATION OF CASE: A 3-year-old female with biliary atresia associated with polysplenia syndrome and a surgical history of Kasai portoenterostomy procedure, and an ABO incompatible whole liver transplant. Within a year after transplant she presented with prehepatic portal hypertension, that was treated with MRB using a deceased donor ABO compatible iliac vein as conduit. Six months later, she was taken to the operating room for bypass revision, during the procedure the MRB showed no flow and no thrombus, and a large splenorenal collateral vein that was causing a portal perfusion steal phenomenon was observed. After dissecting the collateral vein, an 8 cm x8 mm segment of this vessel was used as an autologous conduit to re-do the Rex. DISCUSSION: Failed of MRB can be attributed to portal steal phenomenon, hypercoagulable disorders, bypass contraction or kinking. In this case we believe the culprit to be the former. When there is a history of longstanding portal hypertension, large collaterals develop; thus, intraoperative portal vein flow measurement is critical and ligation of large collaterals during liver transplantation and MRB should be performed to avoid portal steal phenomenon postprocedure. CONCLUSION: Using a collateral vein as an alternative autologous venous conduit is a feasible option that can have durable success.

Ocular dysmotility after intra-arterial chemotherapy for retinoblastoma.

We report the case of a 15-month-old boy with retinoblastoma who developed exotropia secondary to a right medial rectus infarct after intra-arterial chemotherapy. He had unilateral sporadic group C tumor (International Classification of Retinoblastoma) and was treated with intra-arterial melphalan. One week after the first session of intra-ophthalmic arterial melphalan chemotherapy, he was noted to have orbital congestion, exotropia, and right adduction limitation. Magnetic resonance imaging was suggestive of a right medial rectus infarct. The tumor showed a good response to intra-arterial chemotherapy but the exotropia persisted.

Magnamosis: a novel technique for the management of rectal atresia.

We report a case of rectal atresia treated using magnets to create a rectal anastomosis. This minimally invasive technique is straightforward and effective for the treatment of rectal atresia in children.

Bronchial artery pseudoaneurysm as an unsuspected cause of hemoptysis in a pediatric patient.

OBJECTIVE: Hemoptysis in the pediatric population may be caused by foreign body aspiration, cystic fibrosis, bronchiectasis, or infection. Vascular causes are uncommon. We present a rare cause of hemoptysis related to a bronchial artery pseudoaneurysm. METHODS: We report the case of a child with a bronchial artery pseudoaneurysm causing hemoptysis and describe the clinical evaluation, treatment, and outcome. RESULTS: A 12-year-old girl presented to a tertiary children's hospital with a history of daily, intermittent, moderate-volume hemoptysis. Rigid bronchoscopy showed a fresh clot occluding the right bronchus intermedius. Computed tomography angiogram was concerning for mild external vascular compression of the right mainstem bronchus. A bronchial arteriogram showed a right mid-bronchial pseudoaneurysm, which was embolized without complication. On repeat bronchoscopy, thrombus was removed from the bronchus intermedius with no new active bleeding. The patient was discharged in stable condition and did not have any more episodes of hemoptysis. Additional medical work-up did not reveal another source of the patient's bleeding. CONCLUSION: Hemoptysis in the pediatric population can be inflammatory, infectious, or due to systemic disease. Although extremely rare, bronchial artery pseudoaneurysm should be considered in cases of moderate to severe intermittent hemoptysis without another identifiable cause. Bronchial angiography can be both diagnostic and therapeutic.

Sclerotherapy for intra-abdominal lymphatic malformations in children.

PURPOSE: Sclerotherapy is well described as a treatment for lymphatic malformations (LMs) in the head, neck, and other soft tissue areas. This study aims to evaluate the effectiveness of intralesional sclerotherapy as primary treatment for intra-abdominal LMs in children. METHODS: We conducted a retrospective review from 2008 to 2012 of all children with intra-abdominal LMs treated with sclerotherapy at our tertiary children's hospital. RESULTS: In this study, five patients underwent sclerotherapy as a primary intervention for intra-abdominal LMs. The ages of these patients ranged from 12 to 52 months at the time of initial treatment. Doxycycline was used as the primary sclerotherapy agent. The patients required between three and five (median 3) sclerosing treatments over a period that ranged from 5 to 366 days (median 28). No child has required an operation and all LMs have decreased in size. The median decease in maximum diameter is 62% (21-67). Complete resolution has not been attained but all have experienced symptomatic relief with a median follow-up of 3 (1-24) months. CONCLUSIONS: Sclerotherapy is an effective first-line therapy for intra-abdominal LMs in the pediatric population and should be considered when treating these difficult lesions.

Non-operative treatment versus percutaneous drainage of pancreatic pseudocysts in children.

PURPOSE: The objective of this study was to characterize the clinical course and outcomes of children with pancreatic pseudocysts that were initially treated non-operatively or with percutaneous drainage. METHODS: A retrospective review of children with pancreatic pseudocysts over a 12-year period was completed. Categorical variables were compared using Fischer's exact method and the Student's t test was used to compare continuous variables. Analysis was done using logistic and linear regression models. RESULTS: Thirty-six children met the criteria for pancreatic pseudocyst and 33 children were treated either non-operatively or with percutaneous drainage. Of the 22 children managed non-operatively, 17 required no additional intervention (77 %) and five required surgery. Operative procedures were: Frey procedure (3), distal pancreatectomy (1), and cystgastrostomy (1). Eight of the 11 children treated with initial percutaneous drainage required no additional treatment (72 %). The other three children underwent distal pancreatectomy. Success of non-operative management or percutaneous drainage was not dependent on size or complexity of the pseudocyst Logistic regression did not identify any patient demographic (gender, age, and weight), etiologic (trauma, non-traumatic pancreatitis) or pseudocyst characteristic (size, septations) that predicted failure of non-operative therapy. CONCLUSIONS: In children, pancreatic pseudocysts can frequently be managed without surgery regardless of size or complexity of the pseudocyst. When an intervention is needed, percutaneous drainage can be performed successfully, avoiding the need for major surgical intervention in the majority of patients.

Efficacy and safety during formulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease.

Von Willebrand disease (VWD) is an inherited bleeding disorder caused by the quantitative or qualitative deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived VWF/factor VIII (FVIII) concentrates is required in patients unresponsive to desmopressin. To assess the efficacy, safety and ease of use of a new, volume-reduced (VR) formulation of VWF/FVIII concentrate Haemate(®) P in patients requiring treatment for bleeding or prophylaxis for recurrent bleeding or for invasive procedures. Pharmacoeconomic variables were also recorded. Data were analysed using descriptive statistics. This was a multicentre, prospective, observational study. Consecutively enrolled patients received Haemate(®) P VR according to their needs, and were followed for 24 months. Of the 121 patients enrolled, 25.6% had type 3 VWD and more than 40% had severe disease. All patients were followed for 2 years, for a total of 521 visits. On-demand treatment was given to 61.9% of patients, secondary long-term prophylaxis to 25.6% and prophylaxis for surgery, dental or invasive procedures to 45.5%. The response to treatment was rated as good to excellent in >93-99% of interventions. The new formulation was well tolerated by all patients with no report of drug-related adverse events. The switch to volume-reduced Haemate(®) P was easy to perform and infusion duration was decreased twofold compared with the previous formulation. Volume-reduced Haemate(®) P was at least as effective and well-tolerated as the previous formulation.

Safe treatment of pulmonary hypertension with bosentan in a patient with moyamoya disease and cerebral ischemia.

A 7-year-old girl with Down syndrome and moderately severe pulmonary hypertension experienced a stroke while being treated with a calcium channel blocker. Angiography identified bilateral stenosis of the supraclinoid internal carotid arteries, stenosis or occlusion of the proximal anterior and middle cerebral arteries, and occlusion of the left posterior cerebral artery. She underwent surgery to enhance collateral blood flow to vulnerable areas of the brain. Her pulmonary hypertension therapy was changed to an oral endothelin receptor antagonist. She developed excellent collateral blood flow through external carotid arteries to each cerebral hemisphere and an improvement in blood flow through the right internal carotid artery. This case suggests that bosentan can be used safely in children with moyamoya disease. Additional studies are needed to determine whether endothelin receptor antagonists may influence the progression of moyamoya disease or the development of collateral cerebral blood flow following surgery.

Current treatment of pediatric empyema.

Pneumonia with complicated parapneumonic effusion and empyema is increasing in incidence and continues to be a source of morbidity in children seen in our institution. Current diagnostic modalities include chest radiographs and CT scanning with ultrasound being helpful in some situations. Exact management of empyema remains controversial. Although open thoracotomy drainage is well accepted in children, video-assisted thoracoscopic surgery (VATS) drainage has become more prevalent in the current era. Over the last 4 years, we have treated 58 children with intrapleural placement of pigtail catheters and administration fibrinolytics consisting of tissue plasminogen activator (tPA). Successful drainage and resolution of 54 of the 58 effusions was achieved with percutaneous methods alone. There was no mortality or 30-day recurrence. Mean hospital stay was 9.1 days (range 5 to 21) and mean chest catheter removal was 6 days post placement (range 1.5 to 20). Of the four patients that failed percutaneous tube therapy, 3 underwent video assisted thoracic surgery (VATS), and one had open thoracotomy with decortication. Based on our experience, tPA administered through a small bore chest tube for drainage of complicated parapneumonic effusions has become our standard practice. We reserve VATS for treatment failures and open thoracotomy and decortication for patients with VATS failure.

Management of complicated parapneumonic effusions in children.

Pneumonia with complicated parapneumonic effusion is a significant source of morbidity in children seen in our institution. This affords us the opportunity to evaluate new treatment options. In an effort to ensure that we provide quality care to these pediatric patients presenting with complicated parapneumonic effusions, we performed a retrospective review of patient records as well as our interventional radiology database. Fifty-eight patients were identified who were treated with intrapleural placement of pigtail catheters and administration of tPA. Successful drainage and resolution of 54 of the 58 effusions were achieved with percutaneous methods alone. There was no mortality or 30-day recurrence. Mean hospital stay was 9.1 days (range 5-21). On average, the chest catheter was removed on day 6 postplacement (range 1.5-20). tPA was administered intrapleurally, utilizing a standardized hospital protocol developed conjointly by Interventional Radiology and Thoracic Surgery. Patients were afebrile within 72 hours. In most patients, one catheter was placed. However, five patients had more than one catheter placed initially. Of the four patients that failed percutaneous tube therapy, three underwent video-assisted thoracic surgery (VATS) and one had open thoracotomy with decortication. The complication associated with this treatment was an average drop in hemoglobin of 2 g/mL. Based on our experience, tPA administered through a small-bore chest tube for drainage of complicated parapneumonic effusions has become our standard practice.

Methylene blue-stained autologous blood for needle localization and thoracoscopic resection of deep pulmonary nodules.

BACKGROUND/PURPOSE: Video-assisted thoracoscopic surgery (VATS) has used a variety of preoperative techniques to localize deep pulmonary nodules including wires, plain methylene blue, colored collagen, indigo carmine, India ink, and barium. The authors describe their experience with a computed tomography (CT)-guided localization technique using autologous blood stained with methylene blue. METHODS: The authors reviewed retrospectively children who had pulmonary nodules localized using CT guidance with a mixture containing 3 mL autologous blood stained with 0.3 mL methylene blue. Nodules were resected by standard VATS technique. Postoperative chest tube drainage was performed selectively. RESULTS: Nineteen procedures were performed in 17 children (average age, 11 years). Operating time (range, 21 to 171 minutes) varied depending on the number of nodules resected. All resections were diagnostic, and 80% represented malignancy. Lesions averaged 0.9 cm in size (range, 0.3 to 3 cm) with an average pulmonary depth of 0.8 cm (range, 0.1 cm to 1.8 cm). One patient required conversion to an open thoracotomy because of malfunction of the endoscopic stapler. Forty percent of the children received chest tubes, and 53% were discharged home the same day. CONCLUSION: VATS diagnostic resection of deep pulmonary nodules preoperatively localized with methylene blue stained autologous blood is safe and effective.

[Problems of codification of cause of death: comparison of the mortality data of the ISTAT and the Regional Mortality Registry of Tuscany]. / Problemi di codifica della causa di morte: confronto tra i dati di mortalità dell'ISTAT e del Registro di Mortalità Regionale della Toscana.

A two-part study was undertaken to assess the comparability of the coding of underlying cause of death between ISTAT (Central Statistics Office providing "national" mortality statistics) and RMR (Mortality Registry of Tuscany Region providing "local" mortality statistics). In Part I was compared mortality data of the Province of Florence (years 1985-1986) from the files of ISTAT with those of RMR. The source of the cause of death is the same for both systems (ISTAT certificate), but the data collection and coding of RMR are different from those of ISTAT. In Part II was compared a set of 219 Tuscany death certificates (year 1988) coded by ISTAT and RMR. The results showed an high degree of completeness of RMR (only--0.65% vs. ISTAT) and a satisfactory level of correspondence in the number of deaths for circulatory diseases, for neoplasms and for cancers of most important sites (lung, stomach, intestine, pancreas, breast). Discrepancies were found for some other diseases; for some of these causes of death, also age-adjusted mortality rates showed discrepancies (for example ischaemic heart disease). The cause of these differences have been analyzed.

[Avoidable deaths in the evaluation of the performance of health services. II. European Atlas of avoidable deaths: initial data and several reflections]. / Le morti evitabili nella valutazione dell' attività del servizio sanitario. II. Atlante Europeo sulle morti evitabili: primi dati ed alcune riflessioni.

The second European Atlas of Avoidable deaths, which will be shortly issued, concerns the period 1979-83 for Italy. The causes of deaths included were: tuberculosis, neoplasms of the cervix uteri and those of the uterus with no specified site, Hodgkin's disease, chronic rheumatic heart disease, hypertensive and cerebrovascular disease, childhood respiratory infections, asthma, appendicitis, abdominal hernia, cholecystitis and cholelithiasis, maternal and perinatal deaths. In Italy marked excesses for cholecystitis-cholelithiasis, Hodgkin's disease, hypertensive and cerebrovascular disease and perinatal mortality were observed. A remarkable decrease was observed in comparison with the first Atlas (1974-78); but the European countries generally maintained the differences amongst them and their rank. Proposals to assess the causes of the observed differences are suggested (death certificate quality evaluation studies, case-reference studies, cohort studies) and the role of confidential enquiries is discussed.