Imaging of the fetal bony pelvis by computed tomography in a case of bladder exstrophy.

We describe the findings on computed tomography (CT) in a prenatally diagnosed case of bladder exstrophy, and compare them with the findings on two- and three-dimensional sonography. The CT data of the affected fetus were compared with the CT findings of 14 fetuses with normal bony pelvises. The CT images showed differences in the structure of the bony pelvis in the case of bladder exstrophy, with a wide gap between the iliopubic and ischiopubic rami and a widening of the iliac bones. CT scanning was useful in confirming the sonographic diagnosis of bladder exstrophy, and it may also be helpful for planning early surgery following delivery.

Pulmonary veno-occlusive disease.

Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.

[Postembolic pulmonary hypertension]. / Hypertension pulmonaire postembolique.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease characterized by the persistence of thromboemboli obstructing the pulmonary arteries as an organized tissue. The consequence is an increase in pulmonary vascular resistance resulting in pulmonary hypertension (PH) and progressive right heart failure. BACKGROUND: It is difficult to recognize the postembolic nature of PH because there is no known history of thromboembolic disease in more than 50% of cases. Diagnosis is based on the presence of mismatched segmental defects in the ventilation-perfusion scanning. When CTEPH is suspected, pulmonary angiography and high-resolution CT scan are required to establish the diagnosis and to assess the operability. Pulmonary angiography is always performed in conjunction with a diagnostic right heart catheterization, which is required to confirm the diagnosis of PH and to determine the degree of hemodynamic impairement. If there is a good correlation between the pulmonary vascular resistance and the anatomical obstruction, pulmonary endarterectomy (PEA) must be proposed. Otherwise, vasodilator and antiproliferative treatments and lung transplantation represent interesting alternatives. VIEWPOINT AND CONCLUSION: PEA remains the treatment of choice for eligible patients. Nevertheless, there is a need to conduct randomized trials to assess the efficacy of novel medical therapies in some situations: (1) in inoperable CTEPH due to distal lesions, (2) before PEA (therapeutic bridge) in patients who are considered "high risk" due to extremely poor hemodynamics, (3) in patients with persistent pulmonary hypertension after surgery.

[Hilar adenopathy compressing the pulmonary arteries in the course of sarcoidosis]. / Adénopathies hilaires compressives sur les artères pulmonaires au cours d'une sarcoïdose.

INTRODUCTION: In pulmonary sarcoidosis, vascular involvement is usually limited to the small and medium-sized vessels. Enlarged hilar lymph nodes are usually considered to be soft and hence unlikely to cause pressure on adjacent bronchi or blood vessels. CASE REPORT: We report a rare symptomatic compression of a major pulmonary artery by lymphadenopathy in a case of sarcoidosis. Our patient presented with exertional dyspnoea, with enlarged hilar lymph nodes responsible for segmental pulmonary arterial hypertension, without other thoracic abnormalities. The symptoms and lymphadenopathy regressed with corticosteroid therapy. CONCLUSION: Enlarged hilar lymph nodes can compress major pulmonary arteries and cause segmental pulmonary hypertension. The prognosis can be grave in the absence of treatment.

[Surgical treatment of chronic thromboembolic pulmonary hypertension]. / Traitement chirurgical de la maladie thromboembolique pulmonaire chronique.

Chronic thromboembolic pulmonary hypertension is a condition that has long remained in the shadows, a kind of orphan disease, because of the lack of any curative treatment. The renewal of interest by pulmonary specialists, cardiologists and thoracic surgeon is due to the development over the past 20 years of major new treatments: lung transplantation, continuous prostacyclin infusion, and pulmonary endarterectomy, in chronological order. Most patients with postembolic pulmonary arterial hypertension (PEPAH) in a sufficiently proximal location can benefit from curative surgical treatment by bilateral endarterectomy of the pulmonary arteries. This complex surgery, performed under deep hypothermic circulatory arrest, clears out the pulmonary vascular bed down through its subsegmental branches and results in a frank reduction in pulmonary vascular resistance and normalization of cardiopulmonary function. It is a curative procedure with a perioperative mortality rate less than 7% and a definitive result, unlike pulmonary and cardiopulmonary transplantation, which have a postoperative mortality rate of 20% and a 5-year survival rate of 50%. It is difficult to recognize the postembolic nature of pulmonary hypertension because there is no known history of venous thrombosis or embolic phenomena in more than 50% of cases. Diagnosis is based on the presence of mismatched segmental defects in the radioisotopic ventilation-perfusion scanning. To be accessible to endarterectomy, lesions must involve the main, lobar, or segmental arteries. When conducted by experienced operators according to specific protocols, pulmonary (frontal and lateral views of each lung) and multislice CT angiography optimize assessment of the lesion site. When the pulmonary vascular resistance evaluated by catheterization is correlated with the anatomical obstruction visible on the images, pulmonary endarterectomy has a mortality rate below 4% and offers the patient a substantial chance to regain normal cardiorespiratory function. In cases of pulmonary arterial hypertension due to older embolisms, major arteriolitis occurs in the nonobstructed areas and aggravates the pulmonary hypertension, which may become suprasystemic. The endarterectomy mortality rate is then higher, and in specific cases justifies preoperative medical treatment. Pulmonary or cardiopulmonary transplantation is indicated in this disease only when the lesions are too distal and thus inaccessible to endarterectomy.

[Pulmonary hypertension: CT findings in pulmonary veno-occlusive disease]. / Hypertension pulmonaire: aspect tomodensitométrique de la maladie veino-occlusive.

PURPOSE: To describe the CT findings of pulmonary veno-occlusive disease. MATERIALS AND METHODS: Pre-therapeutic CT of 15 patients suffering from pulmonary veno-occlusive disease were retrospectively reviewed. Pathologic evaluation of pulmonary veno-occlusive disease was obtained after post mortem examination or pulmonary transplantation. CT protocol always included intravenous helical CT and high resolution CT. RESULTS: The most frequent CT-findings in pulmonary veno-occlusive disease were the following: ground glass opacity with poorly defined nodular opacities (73%), septal lines (93%), and adenopathy (80%). Pericardial (60%) and pleural effusions (27%) were also noted. Other parenchymal findings were unusual. CONCLUSION: HRCT must be systematically included in the initial CT evaluation of pulmonary hypertension. Combination of poorly defined nodular opacities, septal lines, and adenopathy are indicative of pulmonary veno-occlusive disease.

Management in intractable obstetric haemorrhage: an audit study on 61 cases.

OBJECTIVE: To refine the indications of bilateral hypogastric artery ligation (BHAL) and angiographic selective embolisation (ASE) in intractable obstetric haemorrhage. DESIGN: an audit study. SETTING: Tertiary care university hospital. POPULATION AND METHODS: Retrospective analysis of 61 cases of obstetric intractable post partum haemorrhage (PPH) initially managed either by hysterectomy or a conservative approach in a tertiary referral centre between 1983 and 1998. Procedures were reviewed as a primary (P) or secondary (S) attempt to arrest the haemorrhagic process. RESULTS: Ten hysterectomies (5 P, 5 S), 49 BHAL (48 P, 1 S) and 9 ASE (8 P, 1S) were successfully performed in arresting the haemorrhagic process. There were 7 maternal deaths, 5 following hysterectomy and 2 following a conservative approach. Atony of the uterus was the main cause of haemorrhage (n=21) and genital tract laceration was associated with the worst prognosis. Time-elapse between delivery and surgery appears to be the main prognostic factor. Nine patients became pregnant 1 to 4 years later following a conservative approach. CONCLUSIONS: ASE seems to be indicated in haemodynamically stable patients with birth canal trauma or uterine atony and clotting anomalies. BHAL is indicated when haemorrhage occurs after a cesarean section or when the patient is haemodynamically unstable. BHAL should be taught to Junior doctors in an attempt to decrease the number of patients transferred in tertiary referral centers for intractable PPH. This might also decrease the number of hysterectomies in intractable PPH.

High prevalence of detectable deep venous thrombosis in patients with acute pulmonary embolism.

STUDY OBJECTIVES: Because specific studies are unavailable, the exact prevalence of detectable "residual" deep venous thrombosis (DVT) in patients with acute pulmonary embolism (PE) is unknown. DESIGN: Review of clinical records and radiologic documents of consecutive patients. SETTING: Pulmonary diseases and radiology departments at a university hospital. PATIENTS: All patients hospitalized in the Department of Pulmonary Diseases with a diagnosis of acute PE during a 5-year period (1984 to 1988). During this period, the diagnosis of PE was based exclusively on pulmonary angiography, and bilateral lower limb venography was routine in patients with proven acute PE. MEASUREMENTS AND RESULTS: Among 228 consecutive patients with angiography-proven PE, 213 underwent bilateral lower limb venography within 48 h of the diagnosis. Venography demonstrated DVT in 174 patients (81.7%; 95% confidence interval, 76.5 to 86.9%), including 128 patients (60%) with proximal DVT. Signs or symptoms of DVT were present in only 72 patients (42%) with DVT. The prevalence of detectable DVT was significantly lower in patients with recent pelvic surgery or delivery (6 of 12, 50%) than in the other patients, whatever their individual risk factors (p < 0.05). The mean pulmonary vascular obstruction was significantly lower in patients with normal venography than in patients with detectable DVT (37.6 +/- 20.9% vs 48.4 +/- 21.7%; p = 0.007). CONCLUSIONS: Lower limb venography demonstrates a high prevalence (82%) of residual DVT in patients with angiography-proven PE. These data should be taken into account in the diagnostic and therapeutic management of patients with suspected or proven PE.

High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease.

OBJECTIVE: Clinical differentiation of isolated pulmonary hypertensive arteriopathy from pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease can be difficult on a clinical basis alone. Differentiation is important because misdiagnosis of pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease may lead to severe vasodilator-induced pulmonary edema. The objective of our study was to determine whether high-resolution CT of the chest could distinguish pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease from isolated pulmonary hypertensive arteriopathy. CONCLUSION: Pulmonary hypertension in patients who also have pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease shows characteristics on high-resolution CT that are not seen in patients with isolated pulmonary hypertensive arteriopathy.

Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis.

Continuous intravenous epoprostenol (prostacyclin) produces hemodynamic and symptomatic responses and improves survival in patients with severe primary pulmonary hypertension refractory to conventional medical therapy. However, it has been recently shown that short-term infusion of epoprostenol can produce pulmonary edema in pulmonary veno-occlusive disease, presumably because of increased pulmonary perfusion in the presence of downstream vascular obstruction. We describe two additional cases of pulmonary edema complicating continuous intravenous epoprostenol in patients displaying severe pulmonary hypertension and pulmonary capillary hemangiomatosis, a rare condition characterized by the proliferation of thin-walled microvessels in the alveolar walls. This report indicates that epoprostenol therapy should not be used in patients with severe pulmonary hypertension secondary to pulmonary capillary hemangiomatosis.

Post-traumatic membranous obstruction of the inferior vena cava associated with a hypercoagulable state.

It has been hypothesized that abdominal trauma may be one of the factors involved in membranous obstruction of the inferior vena cava. We present two cases of membranous obstruction of the inferior vena cava associated with trauma. One asymptomatic case, associated with an occult myeloproliferative disorder, developed within 3 years of a violent abdominal trauma. The other case, associated with familial plasminogen deficiency, was discovered at surgery 3 days after a road accident with obvious abdominal trauma, since superimposed extensive thrombosis of the inferior vena cava caused acute Budd-Chiari syndrome. We conclude that underlying prothrombotic conditions are probably necessary for the development of membranous obstruction of the inferior vena cava and that minor trauma may contribute to the development of thrombosis through indirect mechanisms.

Prevalence of cholelithiasis according to alcoholic liver disease: a possible role of apolipoproteins AI and AII.

Moderate alcohol intakes decreases the risk of gallstones; in contrast, the prevalence of gallstones is increased in patients with alcoholic cirrhosis. The aims of this prospective study were to assess the prevalence of cholelithiasis among drinkers according to the histological severity of liver disease, and to estimate the importance of serum apolipoproteins AI and apolipoprotein AII as risk factors for gallstones. Among the 320 drinkers included, 53 patients had cholelithiasis. The prevalence increased (p < 0.0001) from 5% in patients with normal liver (1 of 22) and 6% in patients with steatosis only (3 of 47) to 13% in patients with fibrosis (7 of 54), reaching 27% among patients with biopsy-proven cirrhosis (28 of 103). Among patients with clinically obvious cirrhosis on whom biopsy was not performed mainly because of the severity of liver disease, the prevalence of cholelithiasis reached a maximum of 46% (6 of 13). Among drinkers with nonsevere liver disease, patients with cholelithiasis were older (59 +/- 9 years, mean +/- SD vs. 45 +/- 11, p = 0.003) with lower apolipoprotein AI (118 +/- 37 vs. 163 +/- 45 mg/dl; p = 0.002) and apolipoprotein AII (30 +/- 12 vs. 53 +/- 20 mg/dl; p = 0.0002) in comparison with patients without cholelithiasis. These differences persisted after considering by multiple logistic regression analysis, sex, and ideal body weight. Alcohol consumption during the last 5 years was lower in patients with cholelithiasis (83 g/day) in comparison with patients without cholelithiasis (142 g/day; p = 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)

[Pulmonary arterial hypertension of chronic thromboembolic origin; therapeutic indications]. / Hypertension artérielle pulmonaire d'origine thrombo-embolique chronique; indications thérapeutiques.

Chronic thromboembolic pulmonary hypertension is a rare complication of acute pulmonary embolism. The therapeutic approach to the disease is addressed, based on a series of 81 patients referred to our department between 1984 and 1993. Medical treatment associates long-term anticoagulant therapy and, in some cases, inferior vena cava interruption. Two surgical procedures are available in selected patients: lung transplantation and pulmonary thrombo-endareterectomy. Lung transplantation has been performed in 8 patients since 1988 with 6 survivors with a maximal follow-up of 5 years. Twelve patients underwent pulmonary thromboendareterectomy with in 9 patients a dramatic functional and haemodynamic improvement. Chronic thromboembolism should be systematically investigated as the cause of pulmonary hypertension considering that in selected cases, curative surgery is possible.

Magnetic resonance imaging of the fetus: a study of 20 cases performed without curarization.

Twenty patients underwent magnetic resonance imaging (MRI) at a mean gestational age of 32 weeks. There were 12 patients with suspected fetal brain abnormality and four with intrauterine growth retardation (IUGR), while the remaining four cases were studied for other reasons. The MRI examinations were performed on a 0.5 Tesla machine, with surface coils. One minute acquisition time T1 sequences were used. All the studies were performed without fetal curarization, and only under maternal sedation using flunitrazepam given per os 1 h before MRI examination. Three examinations were incomplete because of fetal movement artefacts. In the remaining cases, MRI allowed the examination of fetal brain anatomy. In five cases, it helped to differentiate isolated hydrocephalus and corpus callosum agenesis. Sub-ependymal nodules were depicted in a case of fetal tuberous sclerosis. One suspected arachnoid cyst was proved to be an ultrasound artefact. Decreased fetal fat on MR images was correlated with low birth weight in cases of IUGR. Due to its better spatial resolution, ultrasonography was more accurate for the diagnosis of facial and lumbar anomalies. Fetal MRI may be performed without curarization. Surface coils allow the detailed analysis of brain parenchyma, and thus MRI is especially useful in the difficult prenatal diagnosis of fetal brain abnormalities.

Role of CT in chronic pulmonary embolism: comparison with pulmonary angiography.

To assess the value of CT in chronic pulmonary embolism (CPE), CT scans and pulmonary angiograms of 21 consecutive patients were reviewed. Computed tomography was better than angiography in assessing proximal clots (three thrombi not seen by angiography, three angiographic false-positives confirmed by surgery). Furthermore, CT was able to analyze pulmonary arteries distal to angiographic amputations. Computed tomography was less sensitive than angiography for vascular distortions (38 vs. 50%) and stenosis (35 vs. 71.8%). Pulmonary infarctions were better detected and characterized by CT than by angiography. Isolated parenchymal ground-glass opacities were seen by CT in 18 patients, especially in those with right cardiomegaly. High resolution CT delineated them better than did standard CT. Computed tomography may be a useful adjunct to angiography in the assessment of CPE.

[Tomodensitometry imaging and MRI of primary lung cancers]. / Imagerie tomodensitométrique et IRM des cancers bronchiques primitifs.

Accurate determination of the extent of lung cancer is essential to decide on its surgical treatment and to evaluate its prognosis. Modern medical imaging is there to spare the patients an unnecessary thoracotomy, knowing that the only satisfactory treatment is surgical resection. Computed tomography (CT) and magnetic resonance imaging (MRI) are part of the preoperative evaluation, side by side with the patient's general condition, his cardiovascular and respiratory status and the histological type of his tumor. It seems rational to ask for these imaging methods for all tumors seemed to be operable after radiology and fibroscopy, except perhaps, for some authors, in cases of peripheral T1 NO tumors not in contact with the chest wall. CT remains the reference examination for preoperative evaluation of lung cancer extension, provided it is performed with technical rigor and is interpreted on the basis of strict criteria. The opposition of MRI in this field has not yet been definitely established, largely because of possible improvements of this new method in the years to come. In certain cases, it seems to be a useful complement to CT or even a possible alternative to this examination.