Journal of Pathology and Translational Medicine

Unilateral Pulmonary Agenesis Combined with other Unusual Anomalies: An autopsy report.: Ik Su Kim, Sang Han Lee, In Soo Shu; Korean J Pathol. 1996;30(2):166-168.

Abstract PDF: Pulmonary agenesis is a very rare anomaly. It is defined as total absence of the pulmonary parenchyma, vascular structures, and bronchi beyond the carina. We experienced a case of right pulmonary agenesis in association with other congenital defects who died at 1 day of age. The other defects included: esophageal atresia, tracheoesophageal fistula, cardiac malformation, anal atresia and a malformed left thumb. The cardiac malformations were a type of Pentalogy of Fallot, composed of right ventricular hypertrophy, ventricular septal defect, an overiding of aorta, pulmonary atresia, and an atrial septal defect. Hand roentgenograms of the malformed left thumb showed an unarticulated metacarpopharyngeal joint. This unique combination of anomalies is extremely rare.

Laryngeal Atresia with Tracheoesophageal Fistula: 1 case report.: Eun Kyung Kim, Je G Chi; Korean J Pathol. 1993;27(5):504-508.

Abstract PDF: Laryngeal atresia is a very rare congenital anomaly requiring immediate tracheotomy. We present a case of laryngeal atresia with tracheoesophageal fistula who showed immediate respiratory difficulty after ligation of umbilical cord and died of aspiration pneumonia at 8 days of age. The atretic portion of larynx is composed of irregulary arranged cartilaginous tissue, bundles of intrinsic muscle and soft tissue without epithelium-lined lumen. The lungs show normal development and evidences of aspiration pneumonia.

Congenital Bronchopulmonary Foregut Malformation: Analysis of the surgical and autopsy cases.: Sung Hye Park, Je G Chi; Korean J Pathol. 1993;27(5):459-467.

Abstract PDF: Because early embryonic development of the tracheobronchial tree and foregut are closely associated, there is a wide spectrum of congenital anomalies involving either one or both organ systems. We analysed a total of 89 surgical and autopsy cases that are assumed to belong to congenital bronchopulmonary foregut malformation from the files of Seoul National University Hospital and Children's Hospital during the periord of 1961~1990. We also reviewed the serial sections of the embryos and fetuses from 3 weeks to fifteen weeks fertilization age for the observation of tracheobronchial and esophageal trees. Intralobar sequestrations(25 cases) and extralobar pulmonary sequestrations(4 cases) with patent, involuted-partial or complete-communication with the alimentary tract, tracheoesophageal fistula(30 cases) with or without esophageal atresia, esophageal atresia, esophageal stenosis due to tracheobroncheal remnant(4 cases), foregut duplication cysts(3 cases), esophageal or gastric diverticulum(1 cases), and bronchogenic cysts(22 cases) are included in this analysis(Table 1). Through this study, we confirmed the unifying concept of "bronchopulmonary forgut malformations". We believe a common embryologic pathogenesis leads to the formation of a previously described spectrum of malformations.

Congenital Subglottic Stenosis of the Larynx Associated with Tracheoesophageal Fistula: 1 autopsy case.: In Sook Kim, Tae Jung Kwon, Dong Wha Lee; Korean J Pathol. 1989;23(3):350-354.

Abstract PDF: Congenital subglottic stenosis of the larynx is one of the most common cause of chronic airway obstruction im infancy and childhood. It is defined as narrowing of the space bounded inferiorly by the inferior margin of the cricoid cartilage amd superiorly by the insertion of the fibers of the conus elasticus into the true vocal cords. In case we experienced was a female full-term baby delivered by Cesarean section. The stenosis was believed by hypertrophy of stromal soft tissue and cricoid cartilage in the subglottic area. The lesion was associated with tracheoesophageal fistula of H1 type. A brief review of the literature was done.

Espihageal Atresia with Tracheoesophageal Fistula and Other Multiple Congenital Anomalies: An autopsy case.: Mee Soo Chang, Hye Ju An, Kyo Young Lee, Won Il Kim, Sang In Shim, Sun Moo Kim; Korean J Pathol. 1989;23(1):145-148.

Abstract PDF: We experienced an autopsy case of esophageal atresia with tracheoesophageal fistula and other multiple congenital anomalies in a 4 days old male infant. Esophageal atresia with tracheoesophageal fistula was type C by Gross Classification. Combined anomalies were malrotation of left kidney, one left accessory renal artery form right renal artery and one left accessory renal vein from right renal vein, duodenal atresia and annular pancreas. Until now, the esophageal atresia with tracheoesophageal fistula and other multiple congenital anomalies (7 congenital anomalies) are not reported in the korean literature, except our case. Our autopsy case, it suggests that esophageal atresia with tracheoesophageal fistula has often close relation-ship with other multiple congenital anomalies.

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