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At least 325 records · Page 18Linked to original sources
PubMed1998

Comparison of endoscopy and radiographic fluoroscopy in the evaluation of pediatric congenital airway abnormalities.

In order to assess the accuracy of conventional dynamic radiographic studies compared to endoscopy in the diagnosis of congenital airway abnormalities, we performed a retrospective chart review for the period between July 1991 to June 1996. A total of 186 patients were identified who required hospitalization for their respiratory status as a result of a congenital airway abnormality. Of these, 19% had both endoscopy and conventional dynamic radiographic evaluation (airway fluoroscopy, barium esophagography, or both). Eleven percent had fluoroscopy only, 62% had endoscopy only, and 9% had neither. In those patients who underwent both endoscopic and conventional dynamic radiographic evaluation, endoscopy was considered to be the definitive procedure for diagnosis. Laryngomalacia was present in 94% of these patients. Thirteen patients had multiple sites of airway abnormalities on endoscopy, and a total of 51 abnormalities were identified. Dynamic radiographic evaluation was correct in four, was suggestive of the abnormality in 12, did not recognize an abnormality in 33, and suggested a different diagnosis (not corresponding to endoscopy) in two. Thus, airway fluoroscopy and/or barium esophagography were correct or suggestive in 16 of 51 abnormalities (31%). We conclude that endoscopy is required in the majority of hospitalized patients for the precise diagnosis of a congenital airway abnormality. Conventional dynamic radiographic studies are helpful to confirm a suspected diagnosis in patients with a strong clinical history and physical examination. When the diagnosis is not clear based on the patient presentation, endoscopy is more definitive than conventional dynamic radiography in identifying pediatric congenital airway abnormalities.

Child, Preschool↗
PubMed2005

Persistent vascular defects in lung allografts attributed to defective endogenous endothelial progenitors.

BACKGROUND: A major pathological finding in human newborns with pulmonary hypoplasia and congenital diaphragmatic hernia is the presence of vascular abnormalities in lungs. Vasculogenesis/angiogenesis are crucial to lung development. To study lung alveolar development, including microvascular formation in fetal lung implants, Schwarz et al. [1] developed a subcutaneous allograft model. We adopted their model to assess the influence of neovascularization or the "host-graft vascular development" on hypoplastic lung structure and growth. MATERIALS AND METHODS: Normal and hypoplastic lungs at pseudoglandular stage were implanted subcutaneously under the dorsolateral fold of immunocompromised nude mice (athymic, nu/nu). Lung allografts were removed and assessed at 2, 4, 6, and 8 weeks postimplantation. RESULTS: Neovascularization of implanted lungs from subcutaneous vasculature of nude mice resulted in varying degrees of maturation of implanted normal and hypoplastic lungs. By 4 weeks, implanted normal lungs contained Type 2-like cells and by 7 to 8 weeks, Type 2 and Type 1-like cells, air spaces had enlarged, and surfactant secretion was observed. Despite some differentiation and maturation of hypoplastic lungs, there was more mesenchymal tissue, no secondary septa, and smaller air spaces compared to normal lungs. CONCLUSIONS: (a) Neovascularization or host-graft vascular development occurs in both normal and hypoplastic lung allografts. (b) Development and maturation of implanted normal and hypoplastic lungs follow the establishment of the vascular connections between the host and grafts. (c) The host-graft vascular connections do not improve the growth of normal or hypoplastic lungs. (d) Neovascularization failed to overcome the embryonic defects in vascular formation and the pulmonary vasculogenesis remained defective in hypoplastic lung allografts, which may be attributed to the defective endogenous endothelial progenitor cells.

Animals↗
PubMed1975

Congenital respiratory tract defects in lambs.

One hundred and forty-seven newborn lambs with congenital defects of the respiratory tract were examined during a 3-year investigation into the causes of perinatal lamb mortality. Respiratory defects were found in 3.3% of the 4,417 lambs examined and in one-third (36.6%) of the 401 congenitally malformed lambs. All the defects were secondary to other more extensive malformations. The upper respiratory tract was involved 3 times more than the lower tract. Males were involved more than females (4:3). The upper tract defects were harelip, cleft palate, atelorrhinia, arrhinia, proboscis, fused nostrils and choanal atresia. One of the facial clefts was a rare median harelip associated with cleft palate and fused nostrils. The lower tract defects were pulmonary agenesis, hypoplasia and duplication. The 2 most common defects were choanal atresia and secondary pulmonary hypoplasia. It was concluded that primary defects of the respiratory tract of sheep are rare and that secondary defects are relatively common.

Abnormalities, Multiple↗
PubMed2001

Combination of renal agenesis with respiratory and alimentary tract atresia results in normal lung development.

The VACTERL complex comprises renal agenesis and atresias of the alimentary and respiratory tracts. We report on a case with this combination causing severe oligohydramnios but with normal lung development. The likely protective mechanism for pulmonary development was an increase in alveolar pressure and reduced alveolar fluid loss due to the esophageal-tracheal malformation. This suggests the possible treatment of oligohydramnios by tracheal occlusion.

Abnormalities, Multiple↗
PubMed2003

Bronchopulmonary foregut malformations: embryology, radiology and quandary.

Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM.

Abnormalities, Multiple↗
PubMedDate not supplied

Congenital tracheal stenosis.

Twenty-one cases of congenital tracheal stenosis seen at the Royal Alexandra Hospital for Children 1971 through 1980 were reviewed with regard to the clinical features, associated anomalies, endoscopic findings and radiological evaluation. Congenital tracheal stenosis was usually a serious problem often associated with other major anomalies of the respiratory tract, esophagus, or skeleton. There was no set pattern of presentation. The presenting features included: persistent wheeze or stridor, atypical "respiratory distress," and atypical "croup" or "bronchiolitis." Documentation of the nature and extent of the stenosis by endoscopy and, if necessary, tracheobronchogram was important for prognosis and to direct treatment. Congenital tracheal stenosis must be considered in infants who present with atypical respiratory tract obstruction, especially where there is an H-type tracheoesophageal fistula, pulmonary hypoplasia or skeletal abnormalities such as hemivertebrae or a thumb abnormality.

Child↗
PubMed2004

Tracheal agenesis: management of the first 10 months of life.

Tracheal agenesis is a potentially lethal congenital anomaly, appearing only at birth. We describe a newborn preterm infant who presented with immediate respiratory distress and no audible cry. There was almost complete tracheal agenesis with a very short segment of distal trachea (only two tracheal rings) arising from the anterior wall of the esophagus, before dividing into the mainstem bronchi. The anomaly was unsuspected prenatally, as the scan showed pyloric atresia and complex congenital cardiac disease. Despite the patient's difficult course, with correction of the rare-associated malformations (cardiac and gastrointestinal tract anomalies), the fact that the child is lively and neurologically normal for her age, requires that we now consider the patency of the airway and the possibility of surgical correction, in accordance with a good quality of life.

Abnormalities, Multiple↗