1/10. Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of two affected siblings.Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/10. Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia.Three unrelated female term infants died when less than 1 month old from intractable pulmonary hypertension associated with deficient capillaries in airspace walls, anomalous small pulmonary veins in bronchiolar-arterial rays, and medial thickening in small pulmonary arteries together with peripheral muscularization. This complex vascular abnormality in the lungs has been termed alveolar capillary dysplasia and/or misalignment of lung vessels in seven previously reported cases. Each infant also showed abnormally immature parenchymal development in the lungs, as was noted in four of the seven prior cases. One had phocomelia; four of the seven prior cases had a variety of congenital anomalies. The primary pulmonary vascular anomaly is likely to be a failure of fetal lung vascularization dating from the second trimester and to be due to action of an unknown teratogen. Centroacinar veins may represent bronchial veins that do not normally develop beyond the ends of cartilaginous bronchi. Pulmonary arterial occlusive changes are interpreted as reactive to obstruction at the level of pulmonary arterioles.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/10. Fatal persistent pulmonary hypertension presenting late in the neonatal period.Two cases of fatal idiopathic persistent pulmonary hypertension presented late in the neonatal period. Lungs were examined histologically by light and electron microscopy, and immunocytochemical studies were used to identify nerves. There was extension of medial smooth muscle distally along the arterial pathway so that most precapillary arteries had completely muscular walls, which in some cases completely obliterated the vessel lumen. Enlarged endothelial cells also contributed to the reduction in the size of the lumen. Nerve fibres accompanying muscular arteries were found in the alveolar region, more distal than is normal. The predominant neuropeptide was the vasoconstrictor tyrosine. Possible aetiological factors in persistent pulmonary hypertension of the newborn are increased muscularity of the peripheral pulmonary arteries antenatally, an increase in the number of vasoconstrictor nerves, or an imbalance in the production of leukotrienes and prostacyclins in the perinatal period.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/10. Basilar arterio-venous pseudoparallelism due to persistence of embryonal venous pattern.A vascular malformation, consisting of a venous vessel bridgeing the right inferior petrosal sinus and the anterior spinal veins, was found in the posterior fossa. The vessel presented a ring-like course around the right trigeminal root, and it was parallel and dorsal to the basilar artery. The malformation was associated with cutaneous and hepatic angiomas and peri-osteal lipomas. It had been clinically silent for 52 years, when it thrombosed causing death. The authors think that, within a general mesenchymopatic state, this is a result of the persistence of an embryonal cerebral venous pattern.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/10. Misalignment of lung vessels and alveolar capillary dysplasia: a cause of persistent pulmonary hypertension.Two infants with fatal persistent pulmonary hypertension are described. Morphologically there was misalignment of the lung vessels, with the veins and the arterioles anomalously related, often sharing the same adventitial sheet. The capillaries did not make contact with the alveolar epithelium. The arterioles had increased medial muscle, and there was extension of the arteriolar muscularization to the precapillary level. The fraction of the parenchyma that was septal and connective tissue was increased. The acini had a decreased complexity, with immature alveoli and with a decreased radial alveolar count. The cause appeared to be related to abnormal capillary and venous plexus formation and migration. This syndrome seems to be identical with that described in three previous reports and probably represents a specific cause of persistent pulmonary hypertension.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
6/10. Misalignment of lung vessels: a syndrome causing persistent neonatal pulmonary hypertension.The cases of two female infants with persistent neonatal pulmonary hypertension are described. Combinations of pulmonary parenchymal lesions were present, including underdevelopment of alveoli and interstitial fibrosis as well as misalignment of lung vessels. As a result of the misalignment, the pulmonary veins joined the pulmonary arteries, rather than following a course away from them. One of the infants had additional congenital malformations. This syndrome should be considered in the evaluation of infants with persistent fetal circulation.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
7/10. Quantitative morphometric analysis of the pulmonary arteries in two patients with D-transposition of the great arteries and persistence of the fetal circulation.Quantitative morphometric studies were performed on the pulmonary arteries of two newborns who died at 1 and 3 days of age with both transposition of the great arteries and persistence of the fetal circulation. Similar studies were performed on two normal control subjects (age 2 and 6 days), one newborn infant (age 1 day) with secondary persistence of the fetal circulation, and one newborn infant (age 2 days) with isolated D-transposition of the great arteries, as compared to those without it. When wall thickness was related to external diameter of the vessel, the greatest difference in thickness was observed in the smallest vessels (less than 150 mu in diameter). Extension of smooth muscle peripherally and to the smallest vessels (less than 50 mu) was similarly most marked in those infants with persistence of the fetal circulation may coexist but at the same time be unrelated in a single patient. Furthermore when such coexistence is unrecognized, pharmacologic manipulation of the ductus arteriosus may be hazardous.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
8/10. Congenital misalignment of pulmonary vessels: an unusual syndrome associated with PPHN.We report a case of misalignment of pulmonary vessels and review the clinical features of all 13 cases reported to date. All were term infants dying from severe persistent pulmonary hypertension of the newborn. We have identified a triad of features that will alert neonatal clinicians to the possibility of this diagnosis: association with other non-lethal congenital malformations; delayed onset of presentation (especially after 12h); and severe hypoxaemia refractory to conventional therapy. We recommend that any autopsy on newborn infants include a specific search for misalignment of pulmonary vessels to outline the pathophysiology and clinical significance of this disorder.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
9/10. The use of a second course of extracorporeal membrane oxygenation in neonatal patients.extracorporeal membrane oxygenation (ECMO) has been successful in rescuing near term or term infants in cardio-respiratory failure that results from a reversible disease process. In most cases, only one course of ECMO is needed to save these infants. However, a second course of ECMO may be beneficial in a select group of infants when recurrent persistent pulmonary hypertension develops. Other than abstract form, this is the first report of the use of a second course of ECMO in the literature. The authors report on three infants, two with recurrent persistent pulmonary hypertension secondary to congenital diaphragmatic hernia and one with necrotizing tracheobronchitis after Group B streptococcal sepsis who were treated at their institution with a second course of ECMO. Technical considerations in using a second course of ECMO depend upon the initial vessel cannulation site, time elapsed between cannulations, and the condition of the original artery and vein. By adopting a stenting procedure in those infants whose initial trial off was equivocal, a second cannulation may be prevented in neonatal patients with recurrent persistent pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/10. Familial persistent pulmonary hypertension of the newborn resulting from misalignment of the pulmonary vessels (congenital alveolar capillary dysplasia).Misalignment of the pulmonary veins with congenital alveolar capillary dysplasia, although rare, has been reported as a cause of persistent pulmonary hypertension of the newborn. Reported cases have been mainly sporadic. Familial occurrence has been reported in only three instances. We present affected sibs with this condition. In addition to pulmonary abnormalities, urogenital abnormalities, including ureteric and urethral obstruction, seem to be common. Autosomal recessive inheritance is suggested.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
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