Showing papers on "Alveolar capillary dysplasia published in 2011"

Alveolar capillary dysplasia.

Abstract: Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare, fatal developmental lung disorder of neonates and infants. This review aims to address recent findings in the etiology and genetics of ACD/MPV and to raise awareness of this poorly known disease, which may also present as milder, unclassified forms. Successively discussed are what is known about the epidemiology, pathogenesis, pathophysiology, diagnostic indicators and approaches, genetic testing, treatment, and cases of delayed onset. The review concludes with suggestions for future directions to answer the many unknowns about this disorder.

Developmental and Pediatric Lung Disease

Abstract: This chapter provides an overview of the major forms of lung disease affecting neonates, infants, children, and adolescents; where appropriate, it highlights the distinctions from diseases more commonly seen in adults. A standard approach for tissue handling is presented to maximize the diagnostic yield for pediatric lung biopsies and lobectomies. Congenital lung malformations and acquired cystic lesions are discussed, followed by a review of diffuse disorders of lung development, including pulmonary hypoplasia, pulmonary hyperplasia, acinar dysplasia, and congenital alveolar dysplasia. Developmental disorders of the vasculature are also reviewed, including alveolar capillary dysplasia, primary lymphangiectasia, and lymphangiomatosis. Acute and chronic complications of premature birth remain a significant cause of morbidity and mortality in neonates. Pathologic features of hyaline membrane disease, bronchopulmonary dysplasia, and pulmonary interstitial emphysema are summarized. Diffuse lung disease in children includes a variety of patterns of alveolar and interstitial disease including some diseases unique to this age group, such as pulmonary interstitial glycogenosis, neuroendocrine cell hyperplasia of infancy, and the genetic disorders of surfactant metabolism. The differential diagnosis of diffuse lung disease in children also includes entities such as hypersensitivity pneumonitis, eosinophilic pneumonia, aspiration, and obliterative bronchiolitis. These diseases are reviewed briefly and discussed in more detail in other chapters. A practical approach to pathologic diagnosis is emphasized, by way of integration of clinical features, gross pathology, microscopic findings, and use of special studies when necessary.

A novel association of alveolar capillary dysplasia, atypical duodenal atresia, and subglottic stenosis

Abstract: Alveolar capillary dysplasia (ACD), which is a rare and lethal congenital pulmonary anomaly found in newborns, begins its onset or causes deterioration of the infant's condition some time after birth. Various congenital anomalies in combination with ACD have been reported, except for subglottic stenosis. Therefore, we aim to report a novel association in a case of ACD with the combination of atypical duodenal atresia and subglottic stenosis. The male infant was scheduled for duodeno-duodenostomy because a double-bubble sign was observed on a chest radiograph. He arrived at the operating theater without any symptoms. After induction of general anesthesia, although mask ventilation was performed without difficulties throughout the entire procedure, oxygen saturation values of the upper and lower extremities dissociated after several attempts of intubation. Surgery was canceled because of instability of the respiratory condition. Respiratory insufficiency worsened progressively, and the infant died at 5 days of age. An autopsy confirmed ACD and revealed cartilaginous subglottic stenosis, which had made intubation difficult. This report highlights the hazards of the onset and worsening of ACD, and the importance of thorough echocardiography before surgery when atypical duodenal atresia is suspected. Anesthesiologists should also be prepared for the difficulty of intubation.

Misalignment of Lung Vessels and Alveolar Capillary Dysplasia: A Case Report With Autopsy

Abstract: Misalignment of lung vessels (MLV) with or without alveolar capillary dysplasia (ACD) is a rare cause of idiopathic persistent pulmonary hypertension of the neonate. This report describes a full-term infant with severe and intractable pulmonary hypertension. The patient’s condition progressively deteriorated despite high-frequency oscillatory ventilation, infusion of magnesium sulfate, dopamine, and dobutamine to control blood pressure, and nitric oxide inhalation therapy. The infant died at 5 days of age. The diagnosis of MLV with ACD was established by autopsy. Histopathologic analysis revealed a failure of formation and an ingrowth of alveolar capillaries, thickening of the alveolar walls, poor contact of capillaries with alveolar epithelium, small intra-acinar muscularized arterioles, and anomalous pulmonary veins within bronchovascular bundles. The low rate of diagnosis of MLV with or without ACD may be because of the early high mortality rate or patchy involvement in some cases. Increasing awareness of this clinical entity may prevent the use of costly, invasive, and probably ineffective procedures. Short-term improvement after inhalation of nitric oxide does not lead to long-term survival but merely provides time for potential lung transplantation.

Alveolar capillary dysplasia associated with duodenal atresia: Ultrasonographic findings of enlarged, highly echogenic lungs and gastric dilatation in a third-trimester fetus

Abstract: We report a case of alveolar capillary dysplasia, wherein duodenal atresia was diagnosed during the third trimester. A 36-year-old mother was referred to our hospital for polyhydramnios at 31 weeks' gestation. Duodenal atresia was suspected from the ultrasonographic findings, which showed gastric dilation. Other findings noted were enlarged, highly echogenic lungs, a spherical heart and an increased lung-thorax transverse area ratio. A male infant was born at 37 weeks' gestation. The findings of serial radiography of the infant's upper gastrointestinal tract were compatible with the diagnosis of duodenal atresia; however, he developed persistent pulmonary hypertension of the newborn eight hours after birth and died at five days of age. The autopsy revealed alveolar capillary dysplasia and duodenal obstruction. We propose that the detection of duodenal atresia should prompt the physician to conduct a thorough ultrasonographic examination to rule out associated anomalies, such as alveolar capillary dysplasia, which can be detected by the presence of highly echogenic lungs.

Alveolar capillary dysplasia with anorectal anomaly.

Abstract: Alveolar capillary dysplasia (ACD) is an uncommon cause of irreversible persistent pulmonary hypertension in full-term newborn. In ACD there is a failure of formation of air - blood barrier in addition to misalignment of pulmonary veins. The etiology of the disease is still not understood. We present a case report of a full-term newborn with ACD associated with anorectal anomaly.