Superficial femoral vein as substitute for pulmonary artery reconstruction after resection for bronchovascular fistula

Post-lobectomy bronchovascular fistula (BVF) associated with massive hemoptysis is a rare but life-threatening complication. Surgical options include completion pneumonectomy or BVF resection with end-to-end anastomosis of the airways and reconstruction of the pulmonary artery (PA) by interposition of an appropriate substitute. We report PA resection and successful reconstruction by interposition of an autologous reversed superficial femoral vein (SFV) segment for this purpose.

15-OR: Multi-phase postmortem CT angiography of pulmonary embolism: technique-related artefacts or real findings?

Multi-phase postmortem CT angiography (MPMCTA) is recognized as a valuable tool to explore the vascular system, with higher sensitivity than conventional autopsy. However, a limitation is the impossibility to diagnose pulmonary embolism (PE) due to post-mortem blood clots situated in pulmonary arteries. The purpose of this study was to explore an eventual possibility to distinguish between real PE and artefacts mimicking PE. Our study included 416 medico-legal cases. All of them underwent MPMCTA, conventional autopsy and histological examination. We selected cases presenting arterial luminal filling defects in the pulmonary arteries. Their radiological interpretation was confronted to the one of autopsy and histological examination. We also investigated an eventual correlation between artefacts in pulmonary arteries and those in other parts of the vascular system. In 123 cases, filling defects of pulmonary arteries were described during MPMCTA. In 57 cases, this was interpreted as artefact and in 4 cases as suspected PE. In 62 cases only a differential diagnosis was made. Autopsy and histology could clearly identify the artefacts as such. Only one case of real PE was radiologically misinterpreted as artefact. In 6 of the 62 cases with no interpretation a PE was diagnosed. In 3 out of 4 suspected cases, PE was confirmed. We found out that filling defects in pulmonary arteries are nearly always associated to other vascular artefacts. Therefore, we suggest following some rules for radiological interpretation in order to allow a reliable diagnosis of pulmonary embolism after MPMCTA.

Pulmonary hypertension complicating isolated atrial septal defect.

Atrial septal defect (ASD) typically is asymptomatic in infancy and early childhood and elective defect closure usually is performed at an age between 4 - 6 years. Severe pulmonary hypertension (PHT) complicating an ASD is seen in adulthood and has only occasionally been reported in small children. A retrospective study was undertaken to evaluate the incidence of severe PHT complicating an isolated ASD and requiring early surgical correction in the fi rst year of life. 355 pediatric patients underwent treatment for an isolated ASD either surgically or by catheter intervention during a 10 year period (1996 - 2006) at two tertiary referral centers. 297 patients had secundum ASD and 58 primum ASD with mild to moderate mitral regurgitation. 8 infants were found with isolated ASD (six with secundum and two with primum ASD) associated with signifi cant PHT, accounting for 2.2% of all ASD patients in our centers. These 8 infants had invasively measured pulmonary artery pressures between 50 and 100% of systemic pressure. Median size of the ASD at the time of surgery was 14mm (7 - 20). They were operated in the fi rst year of life and had complicated postoperative courses requiring specifi c treatment for PHT for up to 16 weeks (median 12) postoperatively. Compared to ASD patients without PHT these infants had prolonged postoperative ICU stay of 5 - 9 days (median 8) and prolonged perioperative overall hospital stay of 8 - 32 days (median 15). Ultimate outcome in all 8 infants was good with persistent normalization of pulmonary pressures during mid-term follow-up of between 8 to 60 months (median 28). All other ASD patients had normal pulmonary pressures and mean age at defect closure was higher being 6.2 years for secundum ASD and 3.2 years for primum ASD. In conclusion, ASD is rarely associated with signifi cant PHT in infancy but then requires early surgery to normalize the prognosis of the patients.

Axial spondylometaphyseal dysplasia: Additional reports.

Axial spondylometaphyseal dysplasia (SMD) (OMIM 602271) is an uncommon skeletal dysplasia characterized by metaphyseal changes of truncal-juxtatruncal bones, including the proximal femora, and retinal abnormalities. The disorder has not attracted much attention since initially reported; however, it has been included in the nosology of genetic skeletal disorders [Warman et al. (2011); Am J Med Genet Part A 155A:943-968] in part because of a recent publication of two additional cases [Isidor et al. (2010); Am J Med Genet Part A 152A:1550-1554]. We report here on the clinical and radiological manifestations in seven affected individuals from five families (three sporadic cases and two familial cases). Based on our observations and Isidor's report, the clinical and radiological hallmarks of axial SMD can be defined: The main clinical findings are postnatal growth failure, rhizomelic short stature in early childhood evolving into short trunk in late childhood, and thoracic hypoplasia that may cause mild to moderate respiratory problems in the neonatal period and later susceptibility to airway infection. Impaired visual acuity comes to medical attention in early life and function rapidly deteriorates. Retinal changes are diagnosed as retinitis pigmentosa or pigmentary retinal degeneration on fundoscopic examination and cone-rod dystrophy on electroretinogram. The radiological hallmarks include short ribs with flared, cupped anterior ends, mild spondylar dysplasia, lacy iliac crests, and metaphyseal irregularities essentially confined to the proximal femora. Equally affected sibling pairs of opposite gender and parental consanguinity are strongly suggestive of autosomal recessive inheritance. © 2011 Wiley-Liss, Inc.

Fibrose pulmonaire idiopathique: nouveautés diagnostiques et thérapeutiques [Idiopathic pulmonary fibrosis: recent diagnostic and therapeutic advances].

Idiopathic pulmonary fibrosis (IPF) is the most frequent of the idiopathic interstitial pneumonias. It is a progressive disorderwith a poor prognosis. Its diagnosis requires the careful exclusion of potential causes, and a pattern of usual interstitial pneumonia at high-resolution computed tomography or video-assisted surgical lung biopsy. Several recent randomized trials have profoundly modified the therapeutic management of IPF. The combination of prednisone and azathioprine, often prescribed until recently, has been shown to be harmful and is no longer indicated. N-acetylcystein, also used in the past decade, failed to show an efficacy. However, two new antifibrotic drugs, pirfenidone and nintedanib, have for the first time proven effective in slowing disease progression.

Frequency of severe pulmonary hypertension complicating "isolated" atrial septal defect in infancy.

Atrial septal defects (ASDs) are typically asymptomatic in infancy and early childhood, and elective defect closure is usually performed at ages of 4 to 6 years. Severe pulmonary hypertension (PH) complicating an ASD is seen in adulthood and has only occasionally been reported in small children. A retrospective study was undertaken to evaluate the incidence of severe PH complicating an isolated ASD and requiring early surgical correction. During a 10-year period (1996 to 2006), 355 pediatric patients underwent treatment for isolated ASDs either surgically or by catheter intervention at 2 tertiary referral centers. Two hundred ninety-seven patients had secundum ASDs, and 58 had primum ASDs with mild to moderate mitral regurgitation. Eight infants were found with isolated ASDs (6 with secundum ASDs and 2 with primum ASDs) associated with significant PH, accounting for 2.2% of all patients with ASDs at the centers. These 8 infants had invasively measured pulmonary artery pressures of 50% to 100% of systemic pressure. They were operated in the first year of life and had complicated postoperative courses requiring specific treatment for PH for up to 16 weeks postoperatively. The ultimate outcomes in all 8 infants were good, with persistent normalization of pulmonary pressures during midterm follow-up of up to 60 months (median 28). All other patients with ASDs had normal pulmonary pressures, and the mean age at defect closure was significantly older, at 6.2 years for secundum ASDs and 3.2 years for primum ASDs. In conclusion, ASDs were rarely associated with significant PH in infancy but then required early surgery and were associated with excellent midterm outcomes in these patients.

Acampomelic Form of Campomelic Dysplasia with SOX9 Missense Mutation.

Campomelic dysplasia is a skeletal dysplasia characterized by flat face, Pierre Robin sequence, shortening and bowing of long bones and club feet. The authors describe a case of "acampomelic" campomelic dysplasia that differs from classical campomelic dysplasia by the absence of bone bowing. This condition is among the most common skeletal dysplasias but is often misdiagnosed in the absence of overt campomelia.

Surfactant palmitoylmyristoylphosphatidylcholine is a marker for alveolar size during disease.

Two common lung-related complications in the neonate are respiratory distress syndrome, which is associated with a failure to generate low surface tension at the air-liquid interface because of pulmonary surfactant insufficiency, and bronchopulmonary dysplasia (BPD), a chronic lung injury with reduced alveolarization. Surfactant phosphatidylcholine (PC) molecular species composition during alveolarization has not been examined. Mass spectrometry analysis of bronchoalveolar lavage fluid of rodents and humans revealed significant changes in surfactant PC during alveolar development and BPD. In rats, total PC content rose during alveolarization, which was caused by an increase in palmitoylmyristoyl-PC (16:0/14:0PC) concentration. Furthermore, two animal models of BPD exhibited a specific reduction in 16:0/14:0PC content. In humans, 16:0/14:0PC content was specifically decreased in patients with BPD and emphysema compared with patients without alveolar pathology. Palmitoylmyristoyl-PC content increased with increasing intrinsic surfactant curvature, suggesting that it affects surfactant function in the septating lung. The changes in acyl composition of PC were attributed to type II cells producing an altered surfactant during alveolar development. These data are compatible with extracellular surfactant 16:0/14:0PC content being an indicator of alveolar architecture of the lung.

Moderate postnatal hyperoxia accelerates lung growth and attenuates pulmonary hypertension in infant rats after exposure to intra-amniotic endotoxin.

To determine the separate and interactive effects of fetal inflammation and neonatal hyperoxia on the developing lung, we hypothesized that: 1) antenatal endotoxin (ETX) causes sustained abnormalities of infant lung structure; and 2) postnatal hyperoxia augments the adverse effects of antenatal ETX on infant lung growth. Escherichia coli ETX or saline (SA) was injected into amniotic sacs in pregnant Sprague-Dawley rats at 20 days of gestation. Pups were delivered 2 days later and raised in room air (RA) or moderate hyperoxia (O₂, 80% O₂ at Denver's altitude, ∼65% O₂ at sea level) from birth through 14 days of age. Heart and lung tissues were harvested for measurements. Intra-amniotic ETX caused right ventricular hypertrophy (RVH) and decreased lung vascular endothelial growth factor (VEGF) and VEGF receptor-2 (VEGFR-2) protein contents at birth. In ETX-exposed rats (ETX-RA), alveolarization and vessel density were decreased, pulmonary vascular wall thickness percentage was increased, and RVH was persistent throughout the study period compared with controls (SA-RA). After antenatal ETX, moderate hyperoxia increased lung VEGF and VEGFR-2 protein contents in ETX-O₂ rats and improved their alveolar and vascular structure and RVH compared with ETX-RA rats. In contrast, severe hyperoxia (≥95% O₂ at Denver's altitude) further reduced lung vessel density after intra-amniotic ETX exposure. We conclude that intra-amniotic ETX induces fetal pulmonary hypertension and causes persistent abnormalities of lung structure with sustained pulmonary hypertension in infant rats. Moreover, moderate postnatal hyperoxia after antenatal ETX restores lung growth and prevents pulmonary hypertension during infancy.