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At least 19 recordsLinked to original sources

Congenital testicular lymphangiectasis in children with otherwise normal testes.

Two cases of testicular lymphangiectasis are reported, occurring in stillborns. The disease is a congenital malformation consisting of an abnormal expansive development of lymphatic vessels in both testes. Autopsy study revealed the absence of pulmonary, intestinal or systemic lymphangiectasis. The testes showed normal tubular development with normal germ cell numbers and also normal Leydig cell numbers. The epididymis and spermatic cord appeared normal. In contrast with the two previously reported cases of testicular lymphangiectasis, the present cases were not associated with cryptorchidism or other malformations.

Humans

Benign transient lymphangiectasis (sclerosing lymphangitis) of the penis.

The literature on benign transient lymphangiectasis of the penis (BTLP) was reviewed; reports of 45 cases were collected and a further series, comprising 21 cases, is presented. Both the published and present series support the view that BTLP is associated with recent sexual activity; little support is given for an infective cause and it is confirmed that BTLP is a benign, self-limiting condition presenting in the sexually active man between 20 and 40 years old. There can be recurrences, and susceptibility to them may be encouraged by circumcision in which disturbances of lymphatic drainage may play a part. As so few cases have been reported and few were diagnosed in the present series these comments can only be pointers to further studies. The descriptions in six histological examinations, the reports as `phlebitis' of apparently similar lesions, the report of `sclerosing lymphangitis' in other sites, and the known histological similarity between veins and lymphatics suggest that `sclerosing lymphangitis' is too specific a term. Until further knowledge is available, we suggest that this condition is best regarded as simple dilatation of a lymphatic vessel-namely, lymphangiectasis. As the condition is both benign and transient we propose the term benign transient lymphangiectasis of the penis for the condition formerly known as sclerosing lymphangitis.

Adolescent

Late presentation of primary pulmonary lymphangiectasis.

Pulmonary lymphangiectasis can occur either as a primary change or secondary to chronic congestion. Primary lymphangiectasis is generally considered to occur exclusively in young children. This report describes three male patients, aged 13, 16, and 19 years, in whom the changes are limited to one lobe or to two lobes and the mediastinum. So far as could be ascertained these patients are the oldest reported. Lymphangiectasis should be considered in the clinical differential diagnosis of "pulmonary cysts" in children and young adults.

Adolescent

[Primary intestinal lymphangiectasis or Waldmann's disease (author's transl)].

The authors report the observation of a primary intestinal lymphangiectasy diagnosed on a young girl sent for isolated edema of her inferior members, recently appeared. Clinical examination was normal. Biology found a low protein rate at 33 g/l and a low lymph rate : 183 L/mm3. Hepatic and renal records were normal. Test to marked albumin asserted the exsudative enteropathy with a fecal radio-activity of 3.6% (N 1%). Biopsy of the small intestines set out lymphangiectasies of the intestinal mucosa. Referring to this observation and to literature date, the authors realise a clinical and physiopathological analysis of Waldmann's disease or primary intestinal lymphangiectasy.

Adult

[Congenital pulmonary lymphangiectasis].

Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patients die, usually within the first 24 hours of life. It may present as an isolated anomaly or it may be associated with other congenital abnormalities, most commonly cardiac in origin, as is the case of total obstruction of the pulmonary venous drainage. Congenital pulmonary lymphangiectasis may be also associated with lymphatic anomalies of other portions of the body or with other non-lymphatic, non-cardiac congenital malformations. Chest radiographs show more commonly that the lungs are hyperexpanded and present a diffuse interstitial reticulonodular pattern. The differential diagnosis from the radiological standpoint should include total anomalous pulmonary venous drainage and pneumonia. We present the case of a newborn infant with congenital pulmonary lymphangiectasis who died within the first 24 hours of life, emphasizing clinical, radiological and pathological findings.

Female

Congenital testicular lymphangiectasis.

Testicular lymphangiectasis are described for the first time in a patient with bilateral inguinal cryptorchidism. A great number of irregular lymphatic channels was observed within the parenchyma and the tunica vasculosa in both testes. Large and numerous anastomosis between the lymphatic vessels of these two areas could also be seen. The MTD and the TFI of the left testis were normal. Both parameters were very low in the right testis. The association of this fact with the greater development of the lymphatic vessels in this testis strongly supports the idea that testicular lymphangiectasis interfere mechanically with the testis tubular development.

Biopsy

[Congenital unilobar pulmonary lymphangiectasis].

A left upper lobectomy was performed on a 27-day-old male infant with clinically suspected congenital lobar emphysema. At operation a cranial pericardial hiatus was also noted. The infant died ten days postoperatively of a septicaemia of extrapulmonary origin. An autopsy was not performed. Histologic examination of the left upper lobe revealed the changes to be pulmonary lymphangiectasis, which is only rarely unilateral or unilobar. In this infant alveolar rupture is thought to have provided air entry into the primarily ectatic lymphatic vessels with further spread of air. The clinical symptoms were due to this complication. This interpretation is supported by the finding of numerous foreign-body giant cells in the lymph vessels, an extraordinary finding in congenital pulmonary lymphangiectasis.

Diagnosis, Differential

Conjunctival hemorrhagic lymphangiectasis.

Conjunctival hemorrhagic lymphangiectasis is a benign condition resulting from an abnormal communication between conjunctival lymphatics and conjunctival blood vessels. In this paper, two cases of conjunctival hemorrhagic lymphangiectasis are presented and the possible mechanisms giving rise to this entity are discussed. To aid in clinical differential diagnosis, a short discussion of conjunctival lymphangioma is also presented.

Conjunctival Diseases

[Non-Hodgkin lymphoma arising in a case of Waldmann's intestinal lymphangiectasis].

A case of high grade malignant lymphoma in a young woman with congenital disseminated lymphangiectasis, exudative enteropathy and immunodeficiency is reported. Lymphoid malignancies occur with a high frequency in the course of this rare disease, possibly as a consequence of immunodeficiency. In this case, a selective defect of the OKT4+ peripheral blood lymphocyte subset has been shown. The relationships between congenital lymphangiectasis, immunodeficiency and malignant lymphoma are discussed.

Adult

Acquired vulvar lymphangiectasis in a child.

A unique case of extensive acquired vulvar lymphangiectasis without lymphedema in a child is described. This lesion developed after pelvic exenteration, lymphadenectomy, and radiation therapy for rhabdomyosarcoma of the bladder. These acquired etiologic factors simulated the pathophysiology of congenital lymphangioma. She was successfully treated with surgical excision and vulvar reconstruction.

Child

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

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

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

[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

[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

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

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