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Results for “Lymphangiectasis”

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At least 55 records · Page 3Linked to original sources
PubMed1984

Testicular lymphangiectasis in Noonan's syndrome.

We describe a boy with testicular lymphangiectasis and Noonan's syndrome. Both testes showed seminiferous tubules with a reduced tubular diameter, containing few spermatogonia. The testicular interstitium exhibited a number of large, dilated lymphatic vessels forming irregular channels among the seminiferous tubules and surrounding them. Since there was no accompanying pathological condition to indicate an obstruction to the lymphatic flow at the level of the spermatic cord or in the regional lymph nodes, the abnormal development of testicular lymphatic vessels suggests a congenital malformation.

Child↗
PubMed1983

Airway obstruction due to epiglottic lymphangiectasis: a case report.

Airway obstruction caused by the epiglottis was seen in a 4-month-old boy. Biopsy showed multiple dilated lymphatic vessels, and spontaneous resolution occurred over a period of one year. The possible causes for lymphangiectasis of the epiglottis are discussed.

Airway Obstruction↗
PubMedDate not supplied

Hypoplastic left heart syndrome with restrictive atrial septal defect and congenital pulmonary lymphangiectasis.

The presence of a restrictive atrial septal defect in hypoplastic left heart syndrome represents a surgical emergency and may negatively affect survival after operation. A neonate with such a disease association, requiring septectomy upon birth developed intractable respiratory failure due to congenital pulmonary lymphangiectasis. The therapeutic implications of this rare pathologic condition are discussed.

Fatal Outcome↗
PubMed1979

Congenital pulmonary lymphangiectasis.

We have described an unusual case of congenital pulmonary lymphangiectasis which does not conform to Felman's classification. We suggest establishing a third category in the group with non-cardiac-associated CPL, entitled "noncardiac, intermediate onset."

Female↗
PubMed1986

Chronic lymphangiectasis in Turner's syndrome.

A 3 1/2-year-old female presented with Turner's syndrome and Nonne-Milroy-Meige disease. Ocular findings included strabismus and bilateral chemosis which was unchanging and persisted throughout the four years the patient was followed up. Histopathological findings included diffuse lymphangiectasia and dense connective tissue surrounding the dilated lymph channels. Although the association between congenital lymphoedema and Turner's syndrome is common, the lymphoedema usually disappears by the first year of life. The persistence of the lymphoedema beyond this age is rare, as is the presence of the persistent chemosis. This report represents the first histopathological documentation of congenital lymphangiectasis in association with Turner's syndrome.

Child, Preschool↗
PubMed1999

Lymphangioma and congenital pulmonary lymphangiectasis: a histologic, immunohistochemical, and clinicopathologic comparison.

Lymphangioma (LA) and congenital pulmonary lymphangiectasis (CPL) are part of a spectrum of lymphatic disorders less well characterized than other vascular tumors and malformations. Recent studies showed proliferative and involutional growth phases for hemangiomas that distinguish them from malformations. We investigated immunohistochemical reactivity and proliferative activity to determine whether a similar diagnostically/prognostically useful pattern exists for LA, comparing LA with CPL as a malformative lesion. Immunohistochemical tests for vimentin, Factor VIII-related protein, CD31, CD34, CD45RO, smooth muscle actin, Type IV collagen, MIB-1, bcl-2, and topoisomerase IIalpha were performed on 20 LAs and 10 cases of CPL. Giemsa staining was also performed to quantitate mast cells. Clinicopathologic correlation was performed by medical record review. LA and CPL shared a similar immunohistochemical profile for vimentin, Factor VIII-related protein, CD31, CD34, smooth muscle actin, CD34, and, to a lesser extent, CD45RO. CD31 and CD34 displayed the most uniform pattern of endothelial reactivity, although CD34 had high background staining. bcl-2 was negative. Four LAs exhibited focal low reactivity for MIB-1 and topoisomerase IIalpha; recent infection and thrombosis were associated conditions. LAs displayed seven-fold more mast cells and more reactive T lymphocytes than did cases of CPL. LA and CPL had similar immunohistochemical profiles; LA resembled vascular malformations more than hemangiomas. CD31 and CD34 were useful for detection of small lymphatics at resection margins of LA, a feature associated with recurrence. MIB-1 and topoisomerase IIalpha expression were associated with inflammatory, thrombotic, or reactive processes and were not diagnostically useful. Abundant mast cells, which also were noted in other soft tissue neoplasms, prompt speculation concerning their role in the growth of LAs.

Adolescent↗
PubMed1992

Diffuse pulmonary lymphangiectasis with heart defect discovered 4 months post-natally.

Congenital pulmonary lymphangiectasia is a rare abnormality with dilatation of pulmonary lymphatics and the radiological pattern of a pulmonary interstitial syndrome. It is usually symptomatic at birth and is almost always lethal. We report the case of an infant with congenital pulmonary lymphangiectasis and congenital heart disease who is still alive at 11 years. This case is interesting from the pathogenic, nosologic and prognostic point of view.

Child↗
PubMedDate not supplied

[Primary intestinal lymphangiectasis. A case treated surgically].

The authors describe a rare case of primary intestinal lymphangiectasis resolved with surgical treatment. Usually the natural course of the disease is relatively mild and medical nutritional treatment can be sufficient. In this case the lymphatic intestinal anomaly was generalized to the entire small intestine but a distal ileal segment was particularly involved. The surgical resection of this intestinal tract resolved the symptomatology.

Age Factors↗
PubMed1989

[Chyluria caused by congenital retroperitoneal lymphangiectasis].

A Dutch woman with non-parasitic chyluria is described. Lymphangiography and abdominal CT demonstrated retroperitoneal lymphangiectasis, dilated and tortuous lymphatic ducts around the right pyelocaliceal system and contrast in the bladder. The thoracic duct was patient. There were no metabolic or immunological abnormalities. The aetiology was probably congenital malformation of the lymphatic system. The treatment was conservative.

Adult↗
PubMed1985

Neonatal pneumothorax, a rare complication of pulmonary cystic lymphangiectasis.

An unusual case of neonatal pneumothorax in a full-term female infant is reported. The collapse was caused by congenital pulmonary lymphangiectasis and rapidly led to death; the lesion was discovered at autopsy. Multiple cardiac malformations were also associated. The literature and pathogenesis are reviewed and discussed.

Female↗
PubMed1979

Dysplasia of the lymphatics with lymphoedema, generalized lymphangiectasis, chylothorax and "pseudo-storage-disease".

A patient showing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous angle. Chylous effusions and subcutaneous chyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The "pseudo"-storage disease is regarded as secondary to the backflow of chylus into the tissues.

Adolescent↗
PubMedDate not supplied

[Congenital pulmonary lymphangiectasis and hyaline membrane. Report of a case].

The case of a newborn with pathological confirmation of congenital pulmonary lymphangiectasis and hyaline membrane is reported. The lymphagiectasis corresponded to type III, with histological characteristics that make it different from other types of lymphatic dilatation. This association is quite uncommon and should be considered in the differential diagnosis of acute respiratory failure in the newborn period.

Diagnosis, Differential↗
PubMed1983

[Lymphangiectasis of bone. Apropos of a case].

A case of osseous lymphangiectasis in a girl is presented and a review of existing literature is made. We include this illness in the group of complex vascular displasis. We mainly focus our attention on radiographic examination which is characteristic, even if not specific. Diagnosis was confirmed by osseous biopsy which is not easy to perform successfully but produced a positive result in our case. It was not possible to perform lymphography.

Bone Diseases↗
PubMed1995

[Congenital pulmonary lymphangiectasis].

Six rare cases of congenital pulmonary lymphangiectasis were presented. The macroscopic characteristic of the lungs was honeycomb appearance due to extreme dilatation of the lymphatic spaces in the subpleural, interlobular, peribronchial and perivascular connective tissue. They had thin walls lined by endothelium. The dilated lymphatics may be due to congenital developmental defects or obstruction of major lymphatics, but they appeared to be associated only with pulmonary venous hypertension.

Female↗
PubMed1996

Acquired lymphangiectasis following mastectomy and radiation therapy--report of a case and review of the literature.

A woman noted vesicles and papules on her left upper extremity fifteen years after she underwent mastectomy and received radiation treatment for left breast carcinoma. The vesicles showed clinical and pathologic features of acquired lymphangiectasis. The skin lesions were successfully treated with shave excision followed by electrodesiccation and sequential peristaltic compressions twice daily.

Adult↗
PubMedDate not supplied

[Idiopathic intestinal lymphangiectasis. Evolution with M.C.T. (author's transl)].

A case of idiopathic intestinal lymphangiectasis is reported in a three month old child. Clinical course and laboratory findings are given in relation to administration of three diets containing different concentrations and types of fat. Short term improvement was only noticed with diets containing low concentrations of long chain triglycerides supplement with medium chain triglycerides. Clinical manifestations related to fat malabsortion improved greatly but there was no relationship with serum protein level. No effect on low level of gamma-globulins and lymphatic displasia was found as sawn in an intestinal biopsy performed after three months of treatment. Nevertheless, long-term results were poor and only were evident in a diminution of steatorrhea and normalization of stools.

Dietary Fats↗