1/38. Urethral atresia in a neonate with alveolar capillary dysplasia and pulmonary venous misalignment.Urethral atresia and alveolar capillary dysplasia (ACD) are rare congenital malformations. Urethral atresia is associated with severe pulmonary hypoplasia secondary to oligohydramnios. ACD is associated with pulmonary venous misalignment, results in severe pulmonary hypertension, and is uniformly fatal. We present a case of urethral atresia with successful, early placement of vesicoamniotic shunting, with resolution of the oligohydramnios, in which the neonate rapidly progressed to respiratory failure and death. Postmortem examination confirmed urethral atresia and diagnosed ACD. Given the surprisingly high mortality rate after vesicoamniotic shunting in patients with urethral atresia, we question whether there might be a possible link to ACD.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
2/38. Bilateral sequential lung transplant for ectodermal dysplasia.A case of bilateral sequential lung transplantation for anhidrotic ectodermal dysplasia is presented. The patient was a 16-year-old male with end-stage lung disease secondary to chronic severe respiratory infection. Although a relatively rare disease, the common association of fatal pulmonary compromise in those affected with this disorder warrants consideration of lung transplantation as a viable therapeutic option.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
3/38. Autotransplantation procedure for giant left atrium repair.BACKGROUND: Giant left atrium has been associated with bronchopulmonary and left ventricular compression [Kawazoe 1983]. CASE REPORT: We present a patient with severe congestive heart failure (CHF), respiratory insufficiency and a giant left atrium (GLA) following two previous mitral valve procedures and tricuspid valve annuloplasty in the distant past. Mitral prosthetic function and ventricular systolic function were felt to be normal leading to a tentative diagnosis of diastolic restriction from left ventricular compression and pericardial constriction. A pericardial decortication procedure through left thoracotomy was initially done but proved ineffective. Subsequently, full evidence of hemodynamic failure due to the giant left atrium and its respiratory complication was recognized and the patient underwent cardiac autotransplantation procedure [Kosak 1987], with the aim to reduce the left atrial dimensions to normal. CONCLUSIONS: Calcification of posterior left atrial wall prevented a completely satisfactory reduction of atrial size and the severity of ventricular adhesions from the previous pericardial procedure resulted in very long cardiopulmonary bypass time with severe bleeding complications. This case provides ample evidence that GLA can cause respiratory failure and needs to be surgically corrected.- - - - - - - - - - ranking = 14.242983550169keywords = bronchopulmonary (Clic here for more details about this article) |
4/38. The multiple facets of pulmonary sequestration.PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. methods: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.- - - - - - - - - - ranking = 14.242983550169keywords = bronchopulmonary (Clic here for more details about this article) |
5/38. Congenital acinar dysplasia. Familial cause of a fatal respiratory failure in a neonate.Pulmonary hypoplasia is a rare cause of pulmonary insufficiency, and has a significant rate of morbidity and mortality among affected infants. In most cases, pulmonary hypoplasia is secondary to underlying abnormalities. These may include space occupying lesions, as in infants with congenital diaphragmatic hernia; malformation of chest wall resulting in a small thoracic cavity; severe and prolonged oligohydramnios; and neuromuscular disorders, which prevent normal fetal chest expansion. All lead to poor lung development. Primary pulmonary hypoplasia as a result of congenital acinar dysplasia is exceedingly rare and is diagnosed by exclusion of all known etiologies of secondary pulmonary hypoplasia.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
6/38. cardiovascular abnormalities associated with the Stuve-Wiedemann syndrome.The Stuve-Wiedemann syndrome (SWS) is a congenital bone dysplasia characterized by camptodactyly with ulnar deviation and congenital bowing of the long bones. Affected patients present with respiratory difficulties in the neonatal period or later and recurrent episodes of hyperthermia. The typical radiological findings are bowing of the long bones of the lower limbs, wide metaphyses with decreased density, and abnormal trabecular pattern. Generally, respiratory insufficiency and hyperthermia are reported to be the cause of death. We report on two sibs with SWS, who died from severe pulmonary hypertension with pulmonary artery wall abnormality. We suggest a common pathophysiological process, which could explain the cardiovascular findings that we observed immediately after birth in the two affected sibs. We hypothesize that the severe pulmonary hypertension due to the arterial wall abnormality could explain the neonatal death of these two children.- - - - - - - - - - ranking = 0.2keywords = dysplasia (Clic here for more details about this article) |
7/38. Severe myelodysplasia with monosomies 5 and 7 presenting with rapidly fatal Sweet's syndrome.A 57 year-old Chinese man with anaemia and thrombocytopenia due to monosomies 5 and 7-associated myelodysplasia developed progressive Sweet's syndrome. Recurrent episodes of cutaneous manifestations responded dramatically to corticosteroid therapy. However, progressive pulmonary infiltrates unresponsive to antimicrobial therapy resulted in respiratory failure and death.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
8/38. Multicore myopathy in a patient with anhidrotic ectodermal dysplasia.We report a patient with multicore myopathy, a rare myopathy not previously reported in the anaesthetic literature. It is characterised by a myopathy of proximal muscles which tends to follow a benign course but may be associated with a severe form of cardiomyopathy. The myopathy is related to central core disease so these patients should be considered to have a potential for developing malignant hyperthermia. Complicating this case was an associated anhidrotic type of ectodermal dysplasia resulting in the absence of sweating, febrile episodes, recurrent pulmonary infections, conical and missing teeth, scaly skin and fine, sparse hair. The patient had a scoliosis repair which was uneventful but died three weeks later following a major pulmonary aspiration while on the ward. The cause of the aspiration is thought to have been unsuspected laryngeal incompetence associated with ectodermal dysplasia, the myopathy involving his bulbar muscles and analgesic medication.- - - - - - - - - - ranking = 1.2keywords = dysplasia (Clic here for more details about this article) |
9/38. Acinar dysplasia: a rare cause of neonatal respiratory failure.Acinar dysplasia is a rare cause of death in the first few hours of life, due to an absence of alveoli. This report presents the first case associated with additional major renal malformations. The diagnosis of acinar dysplasia was unexpectedly made at autopsy. Conclusion: Even in the presence of antenatally diagnosed severe anomalies, autopsy may reveal diagnostically important information.- - - - - - - - - - ranking = 1.2keywords = dysplasia (Clic here for more details about this article) |
10/38. life-threatening allergic bronchopulmonary aspergillosis in a well child with cystic fibrosis.Allergic bronchopulmonary aspergillosis (ABPA) is an uncommon condition which may complicate asthma and cystic fibrosis; it is seldom considered life-threatening. We report a well 8-year-old boy with cystic fibrosis and normal lung function who progressed to respiratory failure over several days, attributable to ABPA. He recovered with non-invasive ventilation and oral corticosteroid and antifungal medications, regaining normal lung function within 2 months. To our knowledge, such an acute severe presentation of ABPA in a previously well child has not been reported before.- - - - - - - - - - ranking = 71.214917750846keywords = bronchopulmonary (Clic here for more details about this article) |
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