Weight loss in a patient with polycystic kidney disease: when liver cysts are no longer innocent bystanders.

OBJECTIVE AND IMPORTANCE: Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disorder, and liver involvement represents one of its major extra-renal manifestations. Although asymptomatic in most patients, polycystic liver disease (PLD) can lead to organ compression, severe disability and even become life-threatening, thereby warranting early recognition and appropriate management. CLINICAL PRESENTATION: We report the case of a 56-year-old woman with ADPKD and severe weight loss secondary to a giant hepatic cyst compressing the pylorus. Partial hepatectomy was required after failure of cyst aspiration and sclerotherapy, and patient's condition improved rapidly. DISCUSSION AND CONCLUSIONS: We discuss the presentation and classification of compressing liver cysts, and the available therapeutic alternatives for this potentially severe complication of ADPKD.

Hepatocyte transplantation using the domino concept in a child with tetrabiopterin nonresponsive phenylketonuria.

Phenylketonuria is a metabolic disease caused by phenylalanine hydroxylase deficiency. Treatment is based on a strict natural protein-restricted diet that is associated with the risk of malnutrition and severe psychosocial burden. Oral administration of tetrahydrobiopterin can increase residual enzyme activity, but most patients with severe clinical phenotypes are nonresponders. We performed liver cell transplantation in a 6-year-old boy with severe tetrahydrobiopterin nonresponsive phenylketonuria who failed to comply with diet prescriptions. The transplanted hepatocytes were obtained in part from an explanted glycogen storage type 1b liver. Following two infusions, blood phenylalanine levels returned within the therapeutic target while the phenylalanine half-life assessed by loading tests decreased from 43 to 19 h. However, 3 months later, blood phenylalanine concentrations increased and the phenylalanine intake had to be reduced. Cell-based therapy is a promising therapeutic option in phenylketonuria, and the domino concept may solve the issue of cell sources for hepatocyte transplantation.

[Indications and surgical treatments for failed coiled aneurysms]. / Indications et traitement chirurgical des anévrismes intracrâniens après recanalisation ou embolisation incomplète.

The possibility of treating intra-cranial aneurysms (ICA) through an endovascular approach is a great progress. But, as any technique, it has its own limitations. Multidisciplinary neurovascular teams are regularly confronted with ICA where embolization is a poor option or even failed (a residue of more than 5% at six months follow-up or after recanalization). Another potential failure is a coil extrusion into the parent vessel with thrombo-embolic risks. Our team and others in the world developed strategies to manage these complex cases. After a brief review of the literature, we describe our experience and present a modified Gurian classification. This classification allows a better identification of the various failed coiled aneurysms types and their potential surgical treatments.

One year clinical outcomes of renal artery stenting: the results of ODORI Registry.

The safety, efficacy and long term clinical benefits of renal artery revascularization by stenting are still a matter of debate. The aim of our study was to define the safety and efficacy of renal artery stenting with the Tsunami peripheral stent (Terumo Corporation, Tokyo, Japan). The ODORI was a prospective, multicentre registry which enrolled 251 consecutive patients, (276 renal arteries) in 36 centres across Europe. The primary endpoint was acute procedural success defined as <30% residual stenosis after stent placement. Secondary endpoints included major adverse events, blood pressure control, serum creatinine level, and target lesion revascularization (TLR) at 6 and 12 months. Patients were 70 +/- 10 years old, 59% were male, 33% had diabetes, and 96% hypertension. The main indications for renal stent implantation were hypertension in 83% and renal salvage in 39%. Direct stent implantation was performed in 76% of the cases. Acute success rate was 100% with residual stenosis of 2.5 +/- 5.4%. Systolic/diastolic blood pressure decreased from a mean of 171/89 at baseline to 142/78 mmHg at 6 months (p < 0.0001 vs. baseline), and 141/80 mmHg at 12 months (p < 0.0001 vs. baseline). Mean serum creatinine concentration did not change significantly in the total population. However, there was significant improvement in the highest tercile (from 283 micromol/l at baseline to 205 and 209 micromol/l at 6 and 12 months respectively). At 12-months, rates of restenosis and TLR were 6.6 and 0.8% respectively. The 12 month cumulative rate of all major clinical adverse events was 6.4% while the rate of device or procedure related events was 2.4%. In hypertensive patients with atherosclerotic renal artery stenosis Tsunami peripheral balloon-expandable stent provides a safe revascularization strategy, with a potential beneficial impact on hypertension control and renal function in the highest risk patients.

Neurosurgical management of inadequately embolized intracranial aneurysms: a series of 17 consecutive cases.

OBJECTIVE: Inadequately embolized aneurysms (IEA) are coiled aneurysms with a significant remnant (>5%), initially or after recanalisation, or with a coil extrusion deemed too thrombogenic or threatening the blood flow in the parent vessel. Our objective is to report our experience with the surgical clipping (SC) of a consecutive series of 17 IEA considered as not appropriate for an additional endovascular procedure. METHODS: Between February 1996 and April 2006, we evaluated 523 ICA in 380 patients of whom 192 underwent coil embolisation (CE), 117 with complete occlusion (61%), 47 with near complete occlusion (> or = 95%), 9 with partial occlusion (<95%), and 19 without any coil delivery (attempted embolisation). Of the 173 ICA embolized one or two times, at their radiological follow-up 15 (8.6%) were considered as IEA and not appropriate for an additional CE. Two IEA treated endovascularly before February 1996 were added to this series. The female/male ratio was 0.47 with an average age of 54 years (range, 37-65). All cases were located on the anterior circulation except the last one. The 17 IEA were treated by SC either because of an aneurysm remnant deemed not accessible to a further CE and large enough for direct clipping or because the risks of a thromboembolic event related to extruded coils was too high. RESULTS: SC was complete in all 17 cases, confirmed angiographically. Postoperatively, the clinical status of two patients deteriorated slightly but transiently. Our surgical experience with this series led us to classify IEA into five types, in three groups: group A (with one type: type A) was the most important group (n = 11) with IEA characterized by an aneurysm residue allowing direct SC, as assessed preoperatively; group B (n = 4) comprised aneurysms with a residue smaller than predicted and showing parent vessel stenosis when a clip was applied to the neck residue requiring the fundus full of coils to be removed followed by either clip application to the neck residue (type B1, n = 3) or suture if the remnant was too small (type B2, n = 1); and group C (n = 2) grouping cases requiring coil extraction through the parent vessel (type C2, n = 1) or through the fundus (type C2, n = 1). CONCLUSIONS: With this series of IEA, we observed that open surgery of type A and C aneurysms can be a straightforward procedure. Our experience with type B IEA encourages us to wait for a sufficient aneurysm residue before performing SC because of the potential difficulties that may be encountered by the surgeon, particularly in type B2. SC of IEA was very effective with complete occlusion and no permanent morbidity in all 17 cases.

Bronchobiliary fistula and cholangiocarcinoma: a case report and principles of management.

A 64-year-old woman was admitted with fever and cough. At admission, she had jaundice, hepatomegaly, and green-stained sputum. Computed tomography (CT) showed an intrahepatic abscess located near the dome, multiple hepatic metastases, biliary tract dilatation, and a right pleural effusion. Percutaneous transhepatic cholangiography demonstrated a communication between the intrahepatic biliary ducts and the bronchial tree. The patient was treated with antibiotic therapy, pleural and biliary drainages and a percutaneous drainage of the hepatic abscess.

Carotid stenting for impending carotid blowout: suitable supportive care for head and neck cancer patients?

BACKGROUND: Carotid blowout (CB) represents a dramatic end-of-life situation for palliative head and neck cancer patients, their relatives and caregivers. Recently, endovascular therapy has been proposed for head and neck surgical patients. Preliminary reports showed a better outcome with less morbidity compared to the previous treatment modalities. However, the specific use of such techniques for palliative head and neck cancer patients has not been previously described. METHOD: Retrospective review of two cases of head and neck cancer patients receiving palliative care, presenting with a CB, managed with endovascular stent placement. RESULTS: Bleeding was effectively stopped by the procedure in both cases. Both patients developed a post-procedure thromboembolism, which was immediately treated by appropriate anticoagulation therapy. Neurological symptoms resolved within 24 hours allowing rapid hospital discharge. One patient died at home seven months later. The second patient is alive five months after the procedure. No recurrence of CB occurred in either patient. CONCLUSIONS: Endovascular stent placement for CB allows a rapid arrest of bleeding and permits the use of anticoagulation therapy in order to avoid long-term neurological injury. In our view, carotid stenting should be considered as valid supportive care for palliative head and neck patients presenting with a CB.

Adult liver transplantation at UCL: update 2002.

The authors present the results of a single centre study of 587 liver transplants performed in 522 adults during the period 1984-2002. Results have improved significantly over time due to better pre-, peri- and post-transplant care. One, five, ten and fifteen year actuarial survivals for the whole patient group are 81.2; 69.8; 58.9 and 51.2%. The high incidence of de novo tumors (12.3%), of cardiovascular diseases (7.5%) and of end-stage renal function (3.6%) should be further incentives to tailor the immunosuppression to the individual patient and to direct the attention of the transplant physician to the long-term quality of life of the liver recipient.

[Imaging of intestinal ischemia]. / Imagerie des ischémies intestinales.

Ischemic bowel disease includes acute and chronic mesenteric ischemia, and colon ischemia. Cross-sectional imaging, and more particularly computed tomography, has an increasing role in the detection of acute and chronic mesenteric ischemia. Vascular obstructions or stenoses and changes in the bowel wall can be observed. Functional information can be added with MRI by using sequences that are sensitive to oxygen saturation in the superior mesenteric vein. Arteriography remains the reference examination in patients with acute mesenteric ischemia.

Severe hypoplasia of the abdominal aorta and its branches in a patient and his daughter.

We report the history of a patient and his daughter, both affected with hypoplasia of the abdominal aorta and its branches, leading to early and dramatic complications. In the index patient, renal ischaemia as a result of severe hypoplasia of the abdominal aorta and the origin of renal arteries led to progressive renal failure and end-stage renal disease at the age of 32 years. Other vascular abnormalities included hypoplasia of the celiac trunk (CT) and superior mesenteric artery (SMA). After a successful kidney transplantation at the age of 40 years, he eventually deceased following an episode of possibly ischaemic acute pancreatitis at 47 years. The patient's daughter suffered from an haemorrhagic stroke at the age of 7 years, which led to the discovery of severe hypertension caused by bilateral narrowing of renal arteries, as well as hypoplasia of CT, SMA, subclavian and pulmonary arteries. Biopsy of the narrowed renal artery of the daughter showed a particular form of fibrodysplasia characterized by an unusual fibrosis of the inner part of the media, just beneath the internal elastic lamina. To our knowledge, this is the first report of familial hypoplasia of the abdominal aorta. It might be the cardinal manifestation of a familial form of fibromuscular dysplasia (FMD). Interestingly, the histological lesions described in the daughter's renal artery differ from the classical form of medial FMD.

[Cardiac imaging by means of four-detector row computed tomography and cardiac gating]. / Imagerie cardiaque en tomodensitométrie à quatre canaux d'acquisition et synchronisation cardiaque.

Electrocardiographically-assisted imaging is a recent development in multislice spiral computed tomography. In this article, we summarize the principles of four-detector row CT for cardiac applications. Following is an overview of the potential of this technique to evaluate the heart, the thoracic aorta, and the paracardiac pulmonary parenchyma. Technical considerations for optimal imaging are highlighted.

[True isolated atherosclerotic aneurysm of the axillary artery]. / Anévrisme vrai isolé d'origine athéromateuse de l'artère axillaire.

The true atherosclerotic aneurysm of the axillary artery is a rare condition. It either presents as a pulsatile axillary mass or arterial emboli in the hand and fingers. We report the case of a 70-year-old man with a 5 cm aneurysm of the axillary artery presenting with embolic disease to the hand. Angiography is helpful and provides valuable preoperative anatomic details. Surgery remains the treatment of choice but endovascular treatment can also be considered.

Demonstration of pulmonary embolism with gadolinium-enhanced spiral CT.

The authors report a case of successful detection of pulmonary embolism using gadolinium-enhanced spiral CT (Gadodiamide, 0.4 mmol/kg, 2 ml/s, delay 18 s) in a 77-year-old woman, with previous allergy to iodinated contrast medium, and renal failure, who presented with pulmonary arterial hypertension. Doppler ultrasound of the lower limbs was first performed and revealed a deep venous thrombosis of the right lower limb. To establish if venous thrombosis was the cause of pulmonary hypertension and to confirm that pulmonary endarterectomy was not indicated in this situation, several imaging modalities were performed. Lung scintigraphy and MRI were non-diagnostic. Gadolinium-enhanced spiral CT demonstrated a large thrombus located proximally and in a segmental artery of the right lower lobe. This case illustrates the potential usefulness of gadolinium as alternative contrast agent with spiral CT to diagnose pulmonary embolism and elucidate the cause of pulmonary arterial hypertension in a patient with some contraindications for iodinated contrast medium injection.

Hepatic vein obstruction due to hypertrophy of right split-liver adult allograft.

The increasing shortage of liver allografts has led to the development of split liver transplantation. More frequent applications have permitted the resolution of most of its technical complications. A hitherto unreported complication of split liver transplantation, hepatic vein obstruction caused by hypertrophy of a right split-liver adult allograft, is discussed. As surgical correction was impossible, the outflow obstruction was treated successfully with interventional radiology.