Ectrodactyly, retrognathism, abnormal ears, highly arched palate, spina bifida, congenital heart defect, single umbilical artery.
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Abnormalities, Multiple↗
Biomedical subjects
J Kasznica
Publications and source records attributed to J Kasznica.
Tonsillar polypoid lymphangioma in a small child.
The authors report a case of an unusual tonsillar polypoid lymphangioma in a three-year-old child. A discussion of the possible mechanisms of its pathogenesis is included, as well as a review of the literature of benign lymphangiomatous tonsillar lesions.
Child, Preschool↗
The completeness of the afterbirth: a medical/pathological discussion.
Practical problems (with possible medicolegal implications) regarding the interpretation of the completeness of the afterbirth are discussed. The case material was obtained during surgical pathological examination and, later, at the time of autopsy of the same patient. The mother (three weeks following emergency delivery) succumbed to life-threatening complications of an epidural anesthetic accident.
Adolescent↗
Lymph node hemangioma.
Lymph node hemangiomas are rare lesions. There have been two previous articles on the subject in the literature. We describe another case in which a lymph node was surgically removed from the inguinal region of a 4 1/2-year-old boy, with a five-month history of a right-sided groin mass. The literature was reviewed, regarding some vasoformative benign lymph node lesions, and a general working classification of these lesions is listed, as we attempted to recognize possible patterns in reactive-proliferative processes and separate them from true neoplasms with hamartomatous features.
Child, Preschool↗
Herrmann-Opitz syndrome: report of an affected fetus.
In 1969, Herrmann and Opitz described a syndrome of acrocephaly, oligosyndactyly, hypertelorism, and mental retardation. We report on a second case, a fetus with cleft palate, urethral astresia, oligohydramnios, and intrauterine death.
Abnormalities, Multiple↗
Iatrogenic causes of large pleural fluid collections in the premature infant: ultrasonic and radiographic findings.
Ten cases of large pleural collections, all of which were iatrogenically induced in premature infants, are presented. There were five instances of hypopharyngeal tears communicating with the right pleural space, three of chylothorax secondary to superior vena caval obstruction in patients undergoing total parenteral nutrition catheterization (TPN), and two of direct erosion by the inferior vena caval TPN catheter into the pleural space. Ultrasonography was most helpful in detecting these pleural collections. It is important to recognize the presence of pleural fluid in these infants as a marker of iatrogenic injury.
Catheterization↗
Abnormalities of the coronary circulation in pulmonary atresia and intact ventricular septum.
To identify developmental aspects and establish morphologic criteria useful for clinical recognition of sinusoids and coronary artery abnormalities in pulmonary atresia and intact ventricular septum, we studied 17 autopsy hearts with this lesion. Hearts from age- and weight-matched infants served as controls. There were two stillborn fetuses, 12 full-term neonates, and 3 infants. None of the eight hearts with normal or large-sized right ventricular chambers had sinusoids or coronary artery abnormalities. Five of nine hearts with small right ventricular chambers had sinusoids; all five lacked trabecular and outlet portions of the ventricle. Four of these five contained major coronary artery abnormalities: atrophy of one or both coronaries proximal to a communication between a sinusoid and the coronary artery. We conclude (1) there is a strong association between the presence of sinusoids and coronary anomalies, supporting the developmental concept of abnormal persistence of embryonic right ventricular sinusoid--coronary artery communications in pulmonary atresia and intact ventricular septum; (2) the absence of trabecular and outlet portions of the small right ventricle indicates a strong possibility of coronary artery abnormalities; and (3) this initial report of a fully-developed lesion in a mid-gestation fetus documents that the coronary artery problem may arise early in fetal life.
Coronary Vessel Anomalies↗
Benign scrotal tumors masquerading as expanding varicoceles.
Vascular lesions of the scrotum though most often varicoceles, can occasionally represent either benign or malignant tumors containing vascular elements. Two pediatric patients presenting with vascular scrotal masses resembling varicoceles are reported. Histologic interpretation of the operative specimens demonstrated benign tumors of the scrotum. Hemangiomas, hamartomas, or mesenchymal tumors should be especially suspect in the presence of any vascular right scrotal lesion. Because of the infrequency of prepubertal varicoceles, any vascular lesion of the scrotum in this age group should undergo biopsy before simple excision is performed to rule out the presence of malignancy. Early excision of benign mesenchymal tumors is especially important if, as some believe, they eventually dedifferentiate to more malignant tumors later in the lifetime of an individual.
Child, Preschool↗
Sclerosing adenosis in the breast of a man with pulmonary oat cell carcinoma: report of a case.
A case of sclerosing adenosis of the male breast is reported. This very unusual lesion was found at autopsy in a man with pulmonary oat cell carcinoma. In females, sclerosing adenosis is a well-characterized entity. In males, however, it does not normally occur because of the physiologic lack of lobular development. The possible pathomechanism of this lesion is briefly discussed, with an emphasis on presumed lobular stimulation by tumor-elaborated ectopic hormone.
Adult↗
Cystic lung disease in Down's syndrome: a report of two cases.
Previously unreported lung disease found at autopsy in 2 young infants with Down's syndrome and congenital heart disease (complete atrioventricular canal malformation with left-to-right shunt) is described. The perinatal and neonatal period was unremarkable, and there was no history of mechanical ventilation or administration of high concentration of oxygen for extended periods. In 1 of the cases respiratory symptoms and hyperinflation with focal cystic changes in the lung fields on chest X-ray were noted at 5-7 months of age. Pathologically there was cystic dilatation of alveoli with focal cuboidal metaplasia of alveolar epithelium and mild to moderate focal alveolar septal fibrosis. Wilson-Mikity syndrome, congenital pulmonary lymphagiectasia, bronchopulmonary dysplasia, and idiopathic interstitial fibrosis of lungs were ruled out on clinical and/or pathologic grounds. Factors such as compression of bronchi by enlarged pulmonary arteries or cardiac chambers, peribronchiolar accumulation of fluid, pulmonary hypoplasia occurring in Down's syndrome, and episodes of pulmonary arterial hypoperfusion associated with severe congenital heart disease may be related to the pathogenesis of the lesion.
Cysts↗
Atypical mesoblastic nephroma. Pathologic characterization of a potentially aggressive variant of conventional congenital mesoblastic nephroma.
A case of an aggressive variant of conventional congenital mesoblastic nephroma (CMN) occurred in a 10-month-old male infant. The tumor proved to be fatal, with two abdominal recurrences, but no metastases were found. Of the various terms used to designate the tumor, atypical mesoblastic nephroma (AMN) is preferred. We reviewed 17 previously reported cases to attempt pathologic characterization of AMN. The features that distinguish AMN from CMN are as follows: (1) atypical gross features consisting of one or more of the following: fleshy areas, foci of hemorrhage, necrosis, involvement of adjacent structures other than connective tissue; and (2) high cellularity and mitotic index. Aggressive behavior was noted at surgery or on follow-up in seven of the 18 cases. It was characterized by one or more of the following: (1) invasion of adjacent structures, such as adrenal gland, spleen, colon, and diaphragm (three cases); (2) one or more recurrences (four cases); and (3) metastasis (two cases). Atypical mesoblastic nephroma should be recognized as a potentially aggressive lesion separate from CMN.
Combined Modality Therapy↗