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Hypoplasia of the small pulmonary arteries in total anomalous pulmonary venous connection with obstructed pulmonary venous drainage.

OBJECTIVE: Preoperative pulmonary venous obstruction has been reported to be a risk factor negatively impacting survival in total anomalous pulmonary venous connection. We examined lung tissue from total anomalous pulmonary venous connection patients with pulmonary venous obstruction and demonstrated hypoplasia of small pulmonary arteries to elucidate the mechanism underlying the poor outcome. METHODS: Ten total anomalous pulmonary venous connection patients with preoperative pulmonary venous obstruction between the ages of 2 days and 10 months were studied. As histological parameters, we assessed the size of small pulmonary arteries in relation to the size of accompanying bronchioles to identify small pulmonary artery underdevelopment. Other parameters, such as the radial alveolar count, which reflects alveolar maturity, intimal lesions, lymphangiectasia, and the medial thickness of small pulmonary arteries and small pulmonary veins, were also examined. As a control group, we examined 24 autopsy cases with no congenital heart or pulmonary disease. RESULTS: When the radius of the accompanying bronchiole was 100 microm, the radius of small pulmonary artery in the control group was found to enlarge for the first 2 months and then remain stable at approximately 80 microm from 2 to 10 months. In total anomalous pulmonary venous connection with preoperative pulmonary venous obstruction, the radius was significantly lower than in the control (47.0 +/- 21.8 microm versus 75.9 +/- 9.8 microm, P <.001), and the difference between dead and surviving patients was significant at P <.001 (33.0 +/- 14.6 microm versus 68.2 +/- 9.2 microm). Examination of the alveoli yielded an radial alveolar count of 4.6 +/- 1.5 in the control group and 4.4 +/- 0.8 in the total anomalous pulmonary venous connection patients, and the difference was not significant (P =.71). CONCLUSIONS: The small pulmonary arteries of total anomalous pulmonary venous connection patients with preoperative pulmonary venous obstruction were underdeveloped compared with controls but their alveolae were not hypoplastic. These results suggested that the small pulmonary artery hypoplasia may be responsible for the poor outcome of these patients.

Age Factors↗

Pseudopolyps of the small intestine in Crohn's disease.

The morphologic features of two types of pseudopolyps of the terminal ileum in a patient with Crohn's disease are described. One form of pseudopolyp is indistinguishable from that observed in the large intestine in Crohn's colitis, while the second, nodular lymphangiectasia, seems to be unique to the small bowel.

Adult↗

[Acquired lymphangiectasias or mammary dermolytic blisters with spontaneous resolution].

INTRODUCTION: Spontaneous occurrence of hemorrhagic blisters on the lower side of the breasts is an unusual clinical presentation of acquired lymphangiectasia. CASE REPORT: A 64 Year-old and a 85 Year-old woman had developed recurrent bleeding blisters in the sub-mammary region for several Months. Histological, immunohistochemical and electron microscopic examination revealed a subpapillary dermal bullous dehiscence, parallel to the epidermis, which was connected to lymphangiectasias of the superficial dermis. Their extreme dilatation and rupture were probably responsible for the clinical inflammatory and bleeding aspect of the lesions, which have not recurred after 1 and 3 Years, respectively. DISCUSSION: The presence of subpapillary inflammatory lymphangiectasias might be responsible for recurrent dermal blister formation. The reason for the presence of these hemorrhagic lymphangiectasias, restricted to the sub-mammary location and their spontaneous regression after several flare-ups, remain unclear. The unusual clinical presentation of the lesions observed in these two women constitutes a differential diagnosis of acquired dermolytic blisters.

Aged↗

[Necrolytic migratory erythema in Waldmann's disease].

BACKGROUND: We report a case of necrolytic migratory erythema in a patient with Waldmann's disease. PATIENTS AND METHODS: A 55-year-old male patient with a history of Waldmann's disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient's cutaneous lesions. DISCUSSION: Waldmann's disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. Increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann's disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.

Dietary Fats↗

Lymphangioma circumscriptum following mastectomy and radiation therapy.

We describe a woman who developed clear, tense vesicles in an area of chronic lymphedema 8 years following mastectomy and radiation therapy. The vesicles showed the clinical and pathologic features of lymphangioma circumscriptum, which can develop as a late sequelae of the lymphatic damage inherent in some forms of cancer therapy.

Breast Neoplasms↗

Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels.

Classification of cutaneous vascular anomalies is difficult because conceptual confusion persists between vascular neoplasms and malformations. However, hemangiomas of the infancy fulfill criteria both for hyperplasia and neoplasm because they result from proliferation of endothelial cells, but often undergo complete regression. Despite these pitfalls we have classified cutaneous vascular anomalies into the following categories: hamartomas, malformations, dilatations of preexisting vessels, hyperplasias, benign neoplasms, and malignant neoplasms. In this first part of our clinicopathologic review of vascular anomalies, hamartomas, malformations, and dilatation of preexisting vessels are covered. Hamartomas include several combined vascular and melanocytic proliferations grouped as phakomatosis pigmentovascularis and the so-called eccrine angiomatous hamartoma that consists of proliferations of both eccrine glands and blood vessels. Vascular malformations result from anomalies of embryologic development, and in some of them the abnormalities of the involved vessels are more functional than anatomic, as is the case of nevus anemicus. In contrast, other cutaneous vascular malformations show striking morphologic abnormalities of the vascular structures. These anatomic vascular malformations are subdivided into the following groups: capillary, venous, arterial, lymphatic, and combined anomalies. Spider angioma, capillary aneurysm-venous lake, and telangiectases are not vascular proliferations at all, but dilations of preexisting vessels. In our opinion, most of the lesions described with the generic term of "angiokeratoma" are not authentic vascular neoplasms, but hyperkeratotic malformations of capillaries and venules or acquired telangiectases of preexisting blood vessels of the papillary dermis. Therefore the first group of these "angiokeratomas" are included in the vascular malformations section, and the second group are covered in the section of dilation of preexisting vessels. Lymphangiectases are considered the lymphatic counterpart of angiokeratomas because they result from ectasia of preexisting lymphatic vessels of the papillary dermis.

Aneurysm↗

Chylothorax in the dog and cat.

The etiology, pathogenesis, and treatment of chylothorax are discussed in this article. A detailed discussion of thoracic duct anatomy, physiology, and methods of lymphangiography is included. The information presented is a review of previous literature, an update on recently completed studies, and speculation about where future research is needed.

Animals↗

Intestinal lymphangiectasia in an adolescent. A case study of edema.

We report a patient who presented with bilateral leg swelling, in whom a diagnosis of intestinal lymphangiectasia was made by small bowel biopsy. Failure to consider the differential diagnosis of edema and lymphedema in an adolescent could cause a considerable delay in diagnosis. We present a brief review and approach to the edematous patient.

Adolescent↗

Lymphangiomatous hamartoma of the spleen.

A 72 year old female was admitted for an operation of gastric cancer. At the operation, a 3 x 3 cm-sized, whitish and multilocular nodule was incidently found at the left side of the spleen. This nodule was adjoining the splenic capsule and was characterized with sparsely found multilocular lymphatic cysts with varying sizes. The walls of these cysts were composed of partly thin fibrous tissue, or partly thin to thick splenic trabeculae. Abnormally elongated, nodular or fragmentary trabeculae were also present in the nodule. White and red splenic pulp was found widely dispersed among the cysts. The surrounding splenic tissue was not compressed. We considered the essence of this tumor to be an abnormal mixture of normal splenic elements such as lymphatics, splenic trabeculae, and red and white pulp, and regarded this tumor as a focal malformation (lymphangiomatous hamartoma) rather than a true neoplasm such as a lymphangioma.

Aged↗