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PubMedDate not supplied

Short-term survivors of pediatric heart transplantation: an autopsy study of their pulmonary vascular disease.

BACKGROUND: The most common indications for heart transplantation in children are congenital heart disease and cardiomyopathy. Intracardiac lesions that vary widely in morphology may have a similar impact on pulmonary vascular morphology; for example, any lesion that increases left-sided intracardiac pressure will affect pulmonary venous pressure and morphology and, if long-standing, may affect pulmonary arteries also. METHODS: The lungs of eight children who died 2 days to 7 months after receiving a heart transplant at ages 8 days to 4 years were examined at autopsy with particular regard to the pulmonary arteries and veins. Arteries were evaluated for medial thickness, intimal proliferation, and peripheral extension of muscle; veins for medial thickness and mural elastic tissue configuration; and lymphatics for degree of dilation. RESULTS: The main pulmonary arteries of the children with congenital heart disease showed an abnormal elastic tissue pattern, similar to the pattern of the aorta, indicating that pulmonary arterial hypertension was present in these children at birth. In contrast, the pulmonary trunk of a child who had myocarditis beginning at age 2 years had a normal pulmonary elastic tissue pattern, consistent with normal neonatal pulmonary pressure. Five children with morphologic evidence of early pulmonary arterial hypertension had a left-to-right atrial shunt before the operation and elevated pulmonary artery pressure immediately after transplantation. The pulmonary arteries of all five patients had medial hypertrophy or peripheral extension of muscle; the most advanced pathologic condition occurred in the arteries of the oldest child, who was 5 months of age, at the time of transplantation who died 2 days after the operation with pulmonary arterial hypertension. In the lungs of all eight patients, pulmonary veins showed medial hypertrophy and excess elastic tissue fibers (arterialization), consistent with venous hypertension. The most impressive venous changes in the lungs of the children with hypoplastic left heart syndrome occurred in the two children who had the smallest left ventricles (4 x 6 mm and 8 x 8 mm), and in another child with hypoplastic and anomalous extrapulmonary pulmonary veins. The most impressive lymphangiectasis occurred in the lungs of the child with hypoplastic left heart syndrome who was oldest at the time of transplantation and in the lungs of a child with obstructed venous drainage. CONCLUSIONS: Pulmonary vasculopathy in children who require heart transplantation because of congenital heart disease or long-standing congestive heart failure may involve arteries, veins, and lymphatics. Among the eight patients in this autopsy study of children who died less than a year after heart transplantation, arterial vasculopathy associated with congenital heart disease appeared more pronounced in the five who survived less than 30 days compared with the two longer survivors. The lack of morphologic progression in short-term survivors of pulmonary arterial vasculopathy after heart transplantation correlates with clinical follow-up of long-term survivors who do not have problematic pulmonary arterial hypertension. Pulmonary venous vasculopathy is determined by the degree of pretransplantation left-sided obstruction caused by congenital or acquired disease and may be more persistent morphologically than arterial vasculopathy. This morphologic study of the pulmonary vasculature of short-term survivors of heart transplantation supports the concept that palliative surgical procedures may benefit children awaiting heart transplantation, especially infants with hypoplastic left heart syndrome for whom suitable donors are scarce.

Cardiomyopathies↗
PubMedDate not supplied

Peripheral corneal disorders.

The peripheral cornea is anatomically and physiologically distinct from its central counterpart. The major differences relate to the gradual transition of corneal tissues to those of the conjunctiva, episclera, and sclera; furthermore, the vascular structures, lymphatics, and inflammatory cells from these neighboring structures are intimately associated with the limbus and periphery of the cornea. The peripheral cornea is thereby predisposed to three main classes of disorders which do not normally involve the central cornea. First, local conditions affecting the sclera and conjunctiva may secondarily spread to involve the limbus and peripheral cornea. These include several infectious diseases, as well as hypersensitivity conditions, mass lesions, and degenerations. Second, due to the associated blood vessels and lymphatics, the peripheral cornea may be involved in a variety of systemic diseases, including vasculitides, autoimmune disorders, and abnormal metabolic conditions. Finally, there are several conditions, such as the noninflammatory peripheral degenerations, which primarily affect the peripheral cornea without associated ocular or systemic changes. In this review, we present a classification and discussion of the various disorders which may involve the peripheral cornea.

Aging↗
PubMed1987

Lymphangioma. An otolaryngologic perspective.

Lymphangiomas are relatively rare congenital hamartomas of the lymphatic system usually diagnosed in infancy and early childhood. Although the precise pathogenesis has not been clearly defined, abnormal development leads to formation of fluid-filled sinuses lined by vascular endothelium. In the head and neck, lymphangioma may encroach on vital structures and infiltrate surrounding tissues making complete surgical removal difficult. A number of children (n = 137) with lymphangioma have been treated at Texas Children's Hospital from 1972-1985. In 45 (33%), the tumors were diagnosed at birth and in an additional 51 (37%), by two years of age. Commonly affected areas included the head and neck (45%), trunk (26%), and axilla (17%). Mediastinal involvement occurred in 4%. Surgical removal was the preferred treatment method and with complete removal the recurrence rate was 6%; known incomplete removal increased this rate to 35%. Although lymphangiomas are slow growing, early removal was preferable: recurrences increased as the time interval between tumor identification and surgical intervention lengthened. Size fluctuation with a history of infection, another unfavorable prognostic factor, also increased recurrence rate.

Child, Preschool↗
PubMed1985

Preoperative lymph node evaluation in prostatic cancer patients who are surgical candidates: the role of lymphangiography and computerized tomography scanning with directed fine needle aspiration.

A total of 53 consecutive patients who were candidates for surgical treatment of prostatic cancer underwent preoperative evaluation of the lymph node status by computerized tomography scanning and/or lymphangiography combined with skinny needle aspiration biopsy of any abnormal lymph nodes. In 7 of 14 patients (50 per cent) ultimately found to have stage D1 disease lymphatic metastases were confirmed histologically with needle biopsy alone, thus, obviating the need for pelvic lymph node dissection. Over-all sensitivity, specificity and accuracy rates were 50, 100 and 91.4 per cent, respectively, for computerized tomography scanning with biopsy and 53.8, 100 and 84.1 per cent, respectively, for lymphangiography with biopsy. Computerized tomography scanning and lymphangiography with aspiration biopsy are cost-effective means to identify approximately 50 per cent of the patients who ultimately have lymphatic metastases.

Biopsy, Needle↗
PubMed2001

Acute leukemia cytogenetics: an evaluation of combining G-band karyotyping with multi-color spectral karyotyping.

We have, during a 12-month period, evaluated the adjuvant effect of combining G-band karyotyping and multi-color spectral karyotyping (SKY) in acute leukemia patients. Forty-four cases were evaluated; fewer cases than those routinely analyzed by G-band cytogenetics had mitoses left for SKY analysis. Of the 44 patients, 35 were acute myeloid leukemia (AML) and 9 acute lymphatic leukemia (ALL) cases. Twenty-seven of 35 AML and 7 of 9 ALL patients had an abnormal G-band karyotype. Thirteen of these 34 abnormal cases had a simple clonal chromosome aberration, and the remaining 21 cases had a complex karyotype. The SKY confirmed the simple karyotype in 11 and in 7 with a complex karyotype. In 13 of the cases with a complex karyotype, ambiguous structural aberrations were classified, in 6 of these, SKY disclosed cryptic translocations. Thus, SKY either extended or confirmed G-band karyotypes in 31 of 34 analyzed abnormal cases. Cases where SKY did not reveal the abnormal clone showed only few abnormal mitoses by G-banding (2/23, 2/25, and 4/27). Additional or confirmatory information was therefore obtained in 91% of analyzed cases, and SKY proved to be a valuable additional tool for hematologic cytogenetics.

Acute Disease↗
PubMed1997

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↗
PubMed2000

Bilateral upper limb lymphoedema associated with psoriatic arthritis: a case report and review of the literature.

Lymphoedema is an unusual extra-articular feature of rheumatoid arthritis and has rarely been described in psoriatic arthritis. We report a 41-year-old man with psoriasis and psoriatic arthritis who developed bilateral lymphoedema of the upper extremities. Lymphoscintigraphy showed absent lymphatic drainage in the right arm and a subnormal increase in lymphatic flow under manual exertion in both arms. Colour Doppler ultrasound studies did not reveal venous or arterial abnormalities. Conservative management and therapy with cyclosporin (for worsening arthritis) resulted in partial resolution of the lymphoedema and improvement of flow parameters on the right side upon repeat lymphoscintigraphy.

Adult↗
PubMed2001

Prenatal diagnosis from cystic hygroma fluid: the value of fluorescence in situ hybridization.

OBJECTIVE: We sought to determine the optimal approach to the prenatal chromosome analysis of cystic hygroma fluid using traditional cytogenetic analysis and fluorescence in situ hybridization. STUDY DESIGN: A retrospective evaluation of our experience with traditional cytogenetic and fluorescence in situ hybridization analysis on cystic hygroma fluid was performed through a systematic review of the Genzyme Genetics database from January 1995 to July 2000. Information on gestational age, sample volume, clinical ultrasound findings (including fetal viability), cytogenetic results, fluorescence in situ hybridization results, and turn-around-time were queried. RESULTS: Eighty-three specimens were included in the investigation. The mean gestational age was 18.1 weeks (range, 13-27 weeks), and the mean sample volume was 20.7 mL (range, 0.1-101 mL). Of the 72 samples in which > 5 mL of cystic hygroma fluid was available, the success rate for cytogenetic analysis was 76% (55/72 samples). In 11 specimens of < or = 5 mL of cystic hygroma fluid, cytogenetic analysis was successful in only 1 case (9%). Fluorescence in situ hybridization was attempted on 23 samples, 18 of which were successful (78%), including 6 of 9 cases of cell culture failure (67%). Both traditional cytogenetic analysis and fluorescence in situ hybridization were performed in 21 instances when a sample of > 5 mL was available. A successful result was obtained by either cytogenetic testing or fluorescence in situ hybridization analysis or both in 19 of 21 of these cases (90%). Samples of > 5 mL from viable fetuses had a higher cytogenetic success rate (80%) and fluorescence in situ hybridization success rate (89%) than samples from fetuses with intrauterine death (38% and 50% cytogenetic and fluorescence in situ hybridization success rates, respectively.) The mean turn-around time was 8.2 days (range, 4-17 days). Results were available in < or = 12 days in 91% of cases. There was a 91% aneuploidy rate identified, with 45,X occurring in 86% of the samples. CONCLUSION: We conclude that the optimal approach for the prenatal diagnosis of chromosome abnormalities from cystic hygroma samples is to perform both traditional cytogenetic studies and interphase prenatal fluorescence in situ hybridization evaluation for the most common aneuploidies that involve chromosomes 13, 18, 21, X, and Y. With this combined approach, our data indicate that, in viable pregnancies with a fluid sample of >5 mL, a 90% diagnostic success rate can be achieved.

Congenital Abnormalities↗
PubMed1982

The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy.

Sinus histiocytosis with massive lymphadenopathy is a benign pseudolymphomatous disease with distinctive microscopic features. Painless cervical adenopathy is the most characteristic clinical finding, although other node groups and extranodal sites may be involved. Of 200 patients in a case registry, 8 had neurologic symptoms. The lesions included vertebral canal and intracranial infiltrates and a destructive vertebral body lesion. Two patients had both cord compression and intracranial disease; one of them died after 10 years of disease. Seven patients were alive 1 to 15 years after disease onset.

Adolescent↗
PubMed1992

[Pulmonary lymphangiomatosis associated with renal angiomyolipoma. Presentation of a case and review of the literature].

The case concerned is one of lymphangiomyomatosis (LAM) in a woman aged 39, that after a episode of spontaneous pneumothorax was subject to medical research for progressive dyspnea of medium efforts and bloodstained sputum. High resolution CT showed numerous thin-walled cystic airspaces of varying sizes distributed diffusely throughout the lungs. The abdominal CT and ultrasound developed multiple right renal angiomyolipomas. The histopathological study of the sample obtained by open lung biopsy was conclusive of LAM. The main feature of this rare disease, which occurs almost exclusively in women of reproductive age, is the abnormal proliferation of immature smooth muscle at the level of the distal airway, small blood vessels and lung lymphatic system, including sometimes the mediastinal and retroperitoneal lymphatic system. The clinical, functional, radiological and differential diagnostic aspects with entities histopathologically similarities are discussed and the therapeutic possibilities are reviewed.

Adult↗
PubMedDate not supplied

[Mediastinal-pulmonary lymphangioleiomyomatosis. The anatomicoclinical aspects].

The lymphangioleiomyomatosis is a rare disease which has a severe course and prognosis and occurs exclusively in females. The symptomatology is not characteristic and the radiological picture is that of a reticular and micronodular diffuse interstitial process. The morphological examination is the only which elucidates the diagnosis. Two cases are reported, the diagnosis of which was established postmortem. In the first case, the site was predominantly pulmonary; in the second case, in addition to the lung, the mediastinal lymph system was also strongly damaged. The morphological changes are characterized by an abnormal proliferation of muscular tissue at the level of the pulmonary interstitium and of the pulmonary and mediastinal lymphatics. The process develops towards destructive lesions of the bronchiolo-alveolar and vascular structures. The pathogenesis is unknown, but genetic and endocrine factors seem to be involved.

Female↗
PubMed1987

[Cystic hygroma of the neck and non-immunologic hydrops fetalis].

Fetal cystic hygromas are a manifestation of early lymphatic obstruction. They are mostly associated with nonimmune hydrops fetalis. They often occur in a number of chromosome abnormalities (Turner syndrome and Down syndrome). We report on a prenatally detected case with nuchal cystic hygroma and nonimmune hydrops fetalis without chromosome aberration and without further major malformations. Postnatally hygroma and hydrops regressed.

Ascites↗