Association of multiple liver cell adenomas with spontaneous intrahepatic portohepatic shunt

The association of multiple liver cell adenomas containing foci of focal nodular hyperplasia with a spontaneous intrahepatic portohepatic venous shunt is reported in a 13-year-old male patient. At least eight nodules less than 10 cm in diameter were recognized and proved by means of surgical resection or surgical biopsies. These lesions were heterogeneous and hypodense on precontrast computed tomographic (CT) scans, and were slightly enhanced after injection of contrast medium. At magnetic resonance (MR) imaging, the signal intensity of these nodules varied. It was either hyperintense or hypointense on T1-weighted SE images. Sonography and angiography demonstrated a portohepatic venous shunt and hepatic arterialization was observed. These findings emphasize the hypothesis that hepatic arterialization may cause the development of liver cell adenomas. Moreover, it is suggested that liver cell adenoma and focal nodular hyperplasia have a common pathogenesis.     

Hepatopulmonary syndrome: a complication of type 1 Gaucher disease

Gaucher's disease is a not exceptional lysosomial disease in Tunisia. Type 1 is by far the most common one. Pulmonary involvement is considered to be rare in type 1 Gaucher's disease. Pulmonary hypertension, infiltration of the lungs with Gaucher cells, and severe hypoxemia due to intrapulmonary arterial-venous shunts, have been described in case reports and small case series. We reported the case of hepatopulmonary syndrome in a 14-year-old boy with type 1 Gaucher disease. The diagnosis of Gaucher disease was established, at 2 years age, by enzyme assay of leucocyte β-glucosidase. The patient presented dyspnoea, digital clubbing and cyanosis of the lips. The arterial blood gas found severe hypoxaemia with PaO(2) at 56.9 mmHg. The diagnosis of hepatopulmonary syndrome, in our patient, was confirmed by demonstration of the intrapulmonary shunting using contrast-enhanced echocardiography and the technetium-99m-labeled macroaggregated albumin. The patient was treated by symptomatic measure, long term oxygen therapy because the insufficiency of the enzyme replacement therapy. Screening for hypoxemia in children with liver disease should be considered.     

Early Pleural Effusions Related to the Myocardial Injury after Open-heart Surgery for Congenital Heart Disease

Objectives. The degree of effusion immediately after cardiopulmonary bypass (CPB) can vary and may reflect several factors including the degree of myocardial injury. We compared the degree of pleural effusions after CPB to the overall myocardial injury as determined by serum cardiac troponin I (cTnI) levels after elective repair of a variety of congenital heart defects, including univentricular surgeries via cavopulmonary shunts. Methods. Serum was collected pre-CPB, post-CPB, and daily after that and cTnI level measured. The postoperative pleural effusion was measured each day until the chest tube was removed. Results. The 21 study patients were of average age of 5.5 years (±5.6). The duration of chest-tube drainage after open-heart surgery was 4.3 days (±3.5) and the amount was 2.4 mL/kg/hour (±2.9). For the biventricular repairs, cTnI levels on the postoperative day (POD) 1 best correlated with amount of effusion (n = 16, r = 0.5, P = 0.02) and the average (POD 0–3) cTnI levels with the total duration (n = 16, r = 0.4, P = 0.01) and also the amount (n = 16, r = 0.5, P = 0.02) of effusions. For the cavopulmonary shunts, the post-CBP cTnI level best correlated with the duration (n = 5, r = 0.8, P = 0.02) and amount (n = 5, r = 0.9, P = 0.02) of effusions. A cTnI level on the first postoperative day ≥15 µg/L was associated with effusions >2 days (sensitivity of 81% and specificity of 80%). Conclusion. We found that higher the cTnI released, especially ≥15 µg/L, longer the duration and greater the amount of early pleural effusions for a variety of congenital heart surgeries including cavopulmonary shunts. A number of factors may lead to excessive pleural effusions and the degree of myocardial injury may be one of them.     

Transjugular intrahepatic portosystemic shunt in a patient with cavernomatous portal vein occlusion

A 23-year-old woman with liver cirrhosis secondary to primary sclerosing cholangitis was referred to us for the treatment of recurrent bleeding from esophageal varices that had been refractory to endoscopic sclerotherapy. Her portal vein was occluded, associated with cavernous transformation. A transjugular intrahepatic portosystemic shunt (TIPS) was performed after a preprocedural three-dimensional computed tomographic angiography evaluation to determine feasibility. The portal vein system was recanalized and portal blood flow increased markedly after TIPS. Esophageal varices disappeared 3 weeks after TIPS. Re-bleeding and hepatic encephalopathy were absent for 3 years after the procedure. We conclude that with adequate preprocedural evaluation, TIPS can be performed safely even in patients with portal vein occlusion associated with cavernous transformation.     

Cardiac Perforation and Tamponade During Transjugular Intrahepatic Portosystemic Shunt Placement

A patient developed acute severe hemodynamic compromise during a transjugular intrahepatic portosystemic shunt (TIPS) procedure for intractable ascites. Rapid clinical and radiographic evaluation of the patient disclosed pericardial blood and cardiac tamponade as the cause, probably due to right heart perforation from guidewire and catheter manipulation. The tamponade was successfully treated percutaneously, and the patient survived. Cardiac tamponade should be considered in the differential diagnosis of patients who develop hypotension during TIPS placement.     

Use of a compact intraoperative low-field magnetic imager in pediatric neurosurgery

Object The majority of investigations on the utility of and indications for intraoperative magnetic resonance imaging (iMRI) have been in adult patients. We report our initial experience utilizing low-field iMRI in pediatric patients.Methods We performed 21 procedures on 20 patients aged 2 months to 18 years (mean 8.9 years) utilizing the PoleStar –10 iMRI system. The procedures included 15 craniotomies, 2 shunts, and 1 each of the following surgeries: transsphenoidal, craniotomy/transsphenoidal, cranioplasty, and endoscopic biopsy and fenestration. Treated lesions included low-grade astrocytoma (5), craniopharyngioma (3), cortical dysplasia (3), hydrocephalus (2), and others (8). The number of scans ranged from 2 to 5 with a mean of 3.2. Intraoperative imaging and navigation provided valuable information on the extent of resection and catheter placement. In eight procedures it influenced the surgical strategy. No untoward events attributable to the system occurred.Conclusions The low-field PoleStar –10 iMRI system can safely assist pediatric neurosurgeons treating a variety of diseases. In addition to neuronavigation it provides information on extent of resection, real-time guided catheter placement, and avoidance of complications.A commentary on this paper is available at     

Bidirectional glenn operation in infancy

The ideal age for bidirectional Glenn shunt (BDGS) as the first stage of staged Fontan is still not clear. Because of the concerns regarding relatively high pulmonary vascular resistance during infancy, many centres would bridge through a systemic to pulmonary artery shunt in this age group. Patients and Methods We did a retrospective analysis of 28 infants who had undergone bidirectional Glenn shunt at our institute from February 200. Results The mean age was 5 months (2.5–11) and the mean weight was 6.5 Kg (3.4–8.7). Boys dominated the group (25∶3). 7 infants had previous procedures. In 3 patients, BDGS was done as a salvage procedure. Formal Cardiopulmonary bypass (CPB) was used in all but 4 patients, in whom a right heart bypass was used. Superior Vena Cava (SVC) or innominate vein was cannulated in 12 patients and the rest were managed with temporary occlusion of SVC under deep hypothermic low flow bypass. 9 infants had bilateral BDGS. The main pulmonary artery was interrupted in 12 and atrial septectomy was done in 10 patients. Additional procedures with BDGS included Patent Ductus Arterious (PDA) interruption, Blalock Taussig (BT) shunt interruption, Left pulmonary arterioplasty, Stansel procedure and redo TAPVC repair. The mean SVC pressure post operatively was 14 (10–24) and only 2 patients needed pulmonary vasodilators in the post-oprative period. There is only one mortality in this series and the duration of chest tube drainage and Intensive Care Unit (ICU) stay is comparable with the older age group. Conclusion BDGS can be performed safely in infants more than 2 months of age electively or as a salvage procedure. It helps to avoid one step in the form of aortopulmonary shunt and hence the ventricular volume overload associated with it. Further studies are required to establish the growth potential of pulmonary arteries following an early BDGS. Presented at the 50th annual meeting of IACTS, New Delhi, Feb. 2004.     

Transcaval Transjugular Intrahepatic Portosystemic Shunt: Preliminary Clinical Results

Objective

To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins.

Materials and Methods

Transcaval TIPS, performed in six patients, was indicated by active variceal bleeding (n=2), recurrent variceal bleeding (n=2), intractable ascites (n=1), and as a bridge to liver transplantation (n=1). The main reasons for transcaval rather than classic TIPS were the presence of an unusually acute angle between the hepatic veins and the level of the portal bifurcation (n=3), hepatic venous occlusion (n=2), and inadequate small hepatic veins (n=1).

Results

Technical and functional success was achieved in all patients. The entry site into liver parenchyma from the inferior vena cava was within 2 cm of the atriocaval junction. Procedure-related complications included the death of one patient due to hemoperitoneum despite the absence of contrast media spillage at tractography, and another suffered reversible hepatic encephalopathy.

Conclusion

In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is feasible.     

Cerebrospinal fluid flow in children with normal and dilated ventricles studied by MR imaging

Purpose: To quantify the cerebrospinal fluid (CSF) dynamics in the aqueduct of children with normal and dilated ventricles using MR phase-contrast technique.Material and Methods: Eighteen patients (6 months to 17 years of age) with various neurological symptoms underwent routine brain MR imaging and CSF flow measurement in the aqueduct. Nine patients had normal ventricles, 5 had dilated ventricles and 4 had a ventriculoperitoneal shunt.Results: The CSF velocity and flow rates in the aqueduct in patients with normal and dilated ventricles showed marked inter-individual variation and clear overlap. In a patient with tight aqueductal stenosis and increased ventricular pressure, pronounced CSF flow in the aqueduct was measured. Absence of flow in another patient with aqueductal stenosis was detected. Measurable although low flow in the aqueduct in 4 patients with a ventriculoperitoneal shunt was found.Conclusion: Quantitative phase MR flow measurement in the aqueduct demonstrated aqueductal stenosis; these patients had either pronounced flow or no flow in the aqueduct.     

The treatment of Candida albicans shunt infections

Cerebrospinal fluid shunting procedures are performed for the treatment of hydrocephalus. Infection of ventriculoperitoneal shunts may create significant clinical management issues in these patients. The majority of these infections are bacterial, but occasionally a Candida albicans shunt infection may occur. We report two patients who acquired Candida albicans shunt infection and discuss their clinical presentation, management, and successful outcome. The treatment with or without removal of the shunt and the correct dosage and route of administration of the antifungal agents is not well documented. The dilemma of treatment of Candida albicans shunt infections in these patients and review of the limited literature on this subject are the subjects of this report. Received: 22 February 1999 Revised: 15 July 1999