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At least 19 recordsLinked to original sources
PubMed1981

[Congenital lymphangiectasis of the lungs (so-called primary and secondary forms) and lymph vessel dilation in hyaline membrane syndrome].

In five cases of congenital pulmonary lymphangiectasis, light microscopic features of the lungs and measurements of their lymph vessel lumina are presented. All lung sections show the same histologic characteristics: a network of partly tubular, partly cystically enlarged lymph vessels within large areas of connective tissue. The lung sections in four cases with pulmonary vein outflow obstruction, the so-called secondary form according to NOONAN et al. [28], do not differ from those in primary lymphangiectasis without obstruction in the pulmonary vein outflow area. The author's own observations are discussed together with 57 cases from the literature. Among the 26 cases of secondary lymphangiectasis are 15 children with aberrant pulmonary veins, and 11 children with hypoplasia of the left side of the heart. Clinically, both forms present with neonatal asphyxia and massive respiratory distress, usually with a fatal outcome within the first few days of life. Hyaline membrane disease may be accompanied by pulmonary lymphangiectasis to a degree that renders its distinction from congenital lymphangiectasis difficult. The lungs in 5 cases with hyaline membrane disease are compared to those in 5 cases of congenital lymphangiectasis. In the former, distended lymphatics are primarily interlobular in location, while in congenital lymphangiectasis they are also found in the subpleural and periarterial tissues. In addition, they are widened to a lesser degree than in the congenital form. In the latter the lymphatic vessel walls are markedly thickened, and the pulmonary lesions, particularly the signs of immaturity, typical for hyaline membrane disease, are lacking.

Diagnosis, Differential↗
PubMed1999

[Initial manifestation of primary intestinal lymphangiectasis as acute abdomen].

HISTORY: A 32-year-old man had ten years previously undergone several laparotomies for recurrent ileus of the small intestine. They revealed severe intestinal oedema and histology showed hyperplasia of the lymphatic system but the aetiology was unclear. After a 10-year interval free of symptoms he presented with marked hypoproteinaemic oedema and exudative enteropathy the cause of which was to be clarified by exploratory laparotomy with excision of lymph nodes and a small section of small intestine. INVESTIGATIONS: Histology revealed intestinal lymphangiectasis with partly hyperplastic lymphoid tissue. Lymphangiography demonstrated several lymph nodes in the region of the aortic bifurcation and renal vessels with a central filling defect. It is thought likely that obstruction to lymphatic flow in this region resulted in oedema of the intestinal wall which caused the recurrent episodes of ileus. DIAGNOSIS: Retrospectively it is assumed that primary intestinal lymphangiectasis was responsible for the initial manifestation of an acute abdomen. For treatment of hypoproteiaemia with human albuminea prot system was implanted. The course was complicated by recurrent inflammation and thrombosis of the port catheter. Because of immune deficiency risk of carcinoma is high in primary intestinal lymphangiectasis. CONCLUSION: Primary intestinal lymphangiectasis, even though a rare condition, should be considered in the differential diagnosis of otherwise unclear acute abdomen.

Abdomen, Acute↗
PubMed1984

[Congenital pulmonary lymphangiectasis].

3 cases of congenital pulmonary lymphangiectasis are described. A girl, now 3 years old, underwent at the age of 4 weeks a resection of the left superior lobe of the lung which was singularly involved. A male newborn died 4 h after birth and showed the typical affection of both lungs. A second male infant died 19 d after birth and had an isolated involvement of the left lung. Post-mortem examination additionally revealed in both boys a cardiovascular malformation. Histologically, we especially observed a papillar endothelial hyperplasia in a dilated lymph vessel in case 1 and multinuclear giant cells of the foreign-body type in case 3. In the literature, there are reported 99 cases of congenital pulmonary lymphangiectasis. Including our case, a unilobar or unilateral involvement is described in only 8 cases. The congenital pulmonary lymphangiectasis occurs more often in males than in females (1.8 : 1). 90 out of 93 life-born children suffering from this malformation died, 57 of them during the perinatal period. However, cardiovascular malformations, which were observed in 53 cases, are playing an important role as the cause of death. The aetiology of the congenital pulmonary lymphangiectasis remains unknown. There are 4 different theories concerning the pathogenesis, namely, a persistence of early-fetal lymphatic vessels, a missing connection between primitive lymphatic vessels, a hyperplasia of lymphatic ducts and, last but not least, a passive dilatation of the lymph vessels because of venous or lymphatic congestion.

Female↗
PubMed1980

[Congenital pulmonary lymphangiectasis. Report of four cases (author's transl)].

Four cases of congenital pulmonary lymphangiectasis were observed during a five year period. This represents an incidence of 1.11 per 10,000 cases among alive newborns and of 53 per 10,000 cases among pediatric necropsias performed during the same period. One case was observed in the clinical context of a generalized hemangiolymphangiomatosis, another was associated to tetralogy of Fallot and the remaining two cases were associated to obstruction of pulmonary venous return. Although one of the pathogenic theories generally accepted in the formation of pulmonary lymphangiectasis points to the presence of either hypertension or obstruction of pulmonary venous drainage, it is possible that such situation is only circumstantial. Among 40 personal cases of obstruction of pulmonary venous return proved anatomically, only in the two cases presented were pulmonary lymphangiectasis demonstrated. The presence of dysplastic elements in pulmonary tissue in cases of lymphangiectasis suggests that a more possible mechanism is a primary defect in the development of pulmonary lymphatics.

Female↗
PubMed2003

Surgical management of conjunctival lymphangiectasis by conjunctival resection.

PURPOSE: To report the treatment of symptomatic conjunctival lymphangiectasis with conjunctival resection. DESIGN: Interventional case series. METHODS: Retrospective review of three cases of patients with symptomatic unilateral conjunctival lymphangiectasis who had conjunctival resection of the involved tissue down to bare sclera. RESULTS: The surgical area reepithelialized without recurrence of the lymphatics. In all cases the patients have remained asymptomatic for at least 14 months. CONCLUSION: Conjunctival resection for symptomatic lymphangiectasis can be a successful therapeutic option for this condition.

Conjunctival Diseases↗
PubMed1990

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↗
PubMed1977

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↗
PubMed1978

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↗
PubMedDate not supplied

[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↗
PubMedDate not supplied

[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↗
PubMed2003

Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care.

Primary pulmonary lymphangiectasis is a rare disease, often described as fatal within the neonatal period. We report on an infant who developed severe bilateral chylous pleural effusions with computed tomography (CT) features of pulmonary lymphangiectasis. She recovered spontaneously with supportive treatment only. We discuss aspects of her intensive care which might have contributed to the good outcome, and conclude that modern intensive care may allow spontaneous recovery in conditions described in the literature as having a very poor outcome.

Female↗
PubMed1977

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↗
PubMed2002

Chylous reflux into localized pulmonary lymphangiectasis.

Spontaneous chylothorax is uncommon and may originate from different etiologies either pleural, pulmonary, or mediastinal. Chyloptysis is a still rarer clinical problem and always of pulmonary origin. We report 2 cases: the first, a 63-year-old woman presenting with a chylothorax, and the second, a 28-year-old man with chyloptysis. Both were successfully treated with a medium chain triglyceride diet. Lymphangiograms demonstrated an identical origin for the 2 cases: reflux from the thoracic duct into right lower lobe lung lymphangiectasis. In our experience, chylous reflux into pulmonary lymphangiectasis is not as rare as believed and many cases probably remain undiagnosed.

Adult↗
PubMed1993

Epididymal lymphangiectasis.

We report on a 59-year-old man with epididymal lymphangiectasis. The patient had noticed somewhat intermittent intrascrotal painless swelling. The interstitium of the right caput epididymis exhibited a number of large, dilated lymphatic vessels forming irregular channels among and around the epididymal ducts. The afferent epididymal ducts showed dilatation similar to that of the lymphatic vessels except for focal cuboid epithelial linings. Spermatogenesis in the right testis was preserved. Lymphangiectasis in the epididymis is infrequent and needs to be differentiated from other intrascrotal lesions.

Diagnosis, Differential↗
PubMed1980

[Haemodynamics in congenital pulmonary lymphangiectasis (author's transl)].

Respiratory insufficiency is the clinical principal sign of cystic pulmonary lymphangiectasis. Clarification of cardiovascular morphology and haemodynamics is the basic prerequisite for the classification and therapy of the disease pattern. The prognosis is unfavourable if it is found that the pulmonary circulation does not flow through the lungs, reaching instead the systemic circulation via the persisting ductus arteriosus. The histopathological findings as well as the results of dissection of the cardiovascular system clarify the question as to the pathogenesis of this disease and allow subdivision of the disease into a primary and a secondary manifestation of cystic pulmonary lymphangiectasis.

Ductus Arteriosus, Patent↗
PubMed1986

[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↗
PubMed1999

Acquired lymphangiectasis.

Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histologic features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculo-bullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe two patients who had these lesions after treatment for cancer and review the pertinent literature. Although a number of treatment options are available, we have found CO2 laser ablation particularly effective.

Adenocarcinoma↗