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At least 487 records · Page 27Linked to original sources
PubMed2004

[Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)].

Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.

Adult↗
PubMedDate not supplied

Congenital intestinal lymphangiectasia: CT demonstration in a young child.

There have been few reports of the CT findings of intestinal lymphangiectasia in adults, and no CT descriptions have been reported in the literature in children. We describe the CT appearance of a biopsy-proven case of primary small intestinal lymphangiectasia in a 3-year-old boy, and we will review the limited literature on the CT findings in this disorder in adults.

Adult↗
PubMedDate not supplied

Lymphangiectasia within angiomyolipoma in a tuberous sclerosis patient.

This is a presentation of a case of tuberous sclerosis with multifocal hamartomas in the retroperitoneum, both kidneys, and the liver. Lymphangiectasia and numerous ectatic lymphatic channels penetrating through the retroperitoneal angiomyolipoma were demonstrated by lymphography and computed tomography. The findings in our study may document the presence of degenerative change in the lymphatic system--not just in elastic vessels--an association which has not yet been reported in the radiological literature.

Adult↗
PubMed2002

[Long-term followup of primary intestinal lymphangiectasia in the child. Six case reports].

UNLABELLED: Primary intestinal lymphangiectasia induce symptoms of protein-losing gastroenteropathy. Only very few studies evaluate the long term follow up of such patients. We reviewed six children diagnosed at 17 +/- 12 months and followed for 11 +/- 4.9 years. CASE REPORTS: As soon as the diagnosis was made the patients were submitted to a strict low fat diet with added medium chain triglycerides and intermittent liposoluble vitamins perfusions. The diet allowed the disappearance of symptoms for all the patients but laboratory findings indicated continuing chyle leak for most of the children. Only one child who had normal biological parameters tolerates a normal diet since four years. Relaxation of the diet by two patients who had moderate hypoalbuminemia and lymphopenia led to severe clinical relapses 14 and 17 years after the diagnosis period with therapeutic difficulties. Three patients with long term strict low fat diet remain asymptomatic. CONCLUSION: In most asymptomatic patients, the underlying lymphatic defect remains with permanent biological abnormalities. Clinical relapses may be severe and difficult to treat; thus the need for dietary treatment appears to be permanent.

Adolescent↗
PubMed2003

[Congenital pulmonary lymphangiectasia revealed by cardiac arrest].

UNLABELLED: Congenital pulmonary lymphangiectasia is a rare cause of respiratory distress in the neonatal period. Cardiac arrest may be its first manifestation. CASE REPORT: We report the case of a full term newborn who suffered at 30 min of life a sudden cardiac arrest. Despite intensive support, the patient died 5 h later. Lung examination showed pulmonary lymphangiectasia. CONCLUSION: Congenital pulmonary lymphangiectasia may be revealed by a sudden neonatal cardiac arrest. Pulmonary lymphangiectasia should be suspected in any newborn who develops early in life an unexplained refractory hypoxemia with radiographic reticulonodular images and uni or bilateral pneumothorax. The diagnosis is established at lung microscopy.

Fatal Outcome↗
PubMed1998

[Hennekam syndrome].

UNLABELLED: Hennekam syndrome is a disorder comprising intestinal lymphangiectasia, facial anomalies and moderate mental retardation. Eight cases have been previously reported. CASE REPORT: A 17-month-old girl was admitted to hospital for peripheral edema. On physical examination, she presented with a normal mental development. Facial anomalies were noted including a flat face, depressed and broad nasal bridge, puffy eye lids, mild down-slanting palpebral fissures, hypertelorism, epicanthal folds, bulbous nasal tip, small mouth, and low set ears. A simian line and haemangiomas on the arms, trunk and left limb were also noted. There was no organomegaly. Laboratory investigations showed iron deficiency anemia, hypoproteinemia, hypogammaglobulinemia and an elevated level of alpha-1 antitrypsin excreted in the feces. Endoscopic investigation and the small bowel biopsy showed findings consistent with lymphangiectasia. The patient did well on 24 hour enteral nutrition including medium-chain triglyceride rich diet and infusion of human albumin. CONCLUSION: We have aimed to remind that Hennekam syndrome should be included in differential diagnosis when intestinal lymphangiectasia are associated with facial anomalies.

Diagnosis, Differential↗
PubMed1998

Ultrasonographic features of long-standing primary intestinal lymphangiectasia.

This article describes a case of primary intestinal lymphangiectasia associated with protein-losing enteropathy and lower right leg lympoedema in a 20-year-old woman. Echographic findings showed dilation of the intestinal loops, regular and diffuse thickening of the walls, plical hypertrophy and impressive mesenteric oedema. Although diagnosis of the disease is substantially histological, the ultrasonographic characteristics of the lesions are fairly indicative and of certain diagnosis usefulness in patients with protein-losing enteropathy.

Adult↗
PubMedDate not supplied

Hypoplastic left heart syndrome with intact atrial septum associated with deletion of the short arm of chromosome 18.

We report on a female newborn with deletion of the short arm of the chromosome 18 (del 18p) and hypoplastic left heart syndrome (HLHS) with intact atrial septum. Several forms of congenital heart disease (CHD) are found in 10% of patients with this chromosomal abnormality, although HLHS has not been reported yet. Interesting coronary artery anomalies, as well as the presence of pulmonary lymphangiectasia, were found in our patient and were contributors to her fatal outcome. Del 18 p must be considered when evaluating a patient with characteristic phenotypical anomalies and HLHS with intact atrial septum.

Abnormalities, Multiple↗
PubMed1985

Lymphangiectatic fibrous polyp of the palatine tonsil. A report of three cases.

Three cases of lymphangiectatic fibrous polyp of the palatine tonsil are reported. The polyp arose from the upper pole of the tonsil in two cases and from the middle-to-lower part in one case. They were all asymptomatic until the polyps grew larger. The polyps were removed, with or without the tonsil. The pertinent literature is reviewed.

Adolescent↗
PubMed2004

[A special case of swollen lower limb].

We report on a case of a 31-year-old patient suffering from long-standing peripheral edema with severe hypoalbuminemia, but without proteinuria. Differential diagnosis, diagnostic work-up and the therapeutic options in this unusual case are discussed. The general practitioner must keep in mind a broad range of causes when seeing every-day-patients with peripheral edema, although the correct etiology can be found easily in most cases.

Adult↗
PubMed1998

Lymphangiectasia haemorrhagica conjunctivae.

PURPOSE: To re-describe a condition that has not been mentioned in the literature for more than four decades and to outline a new method of treatment of the pathology using an argon laser. METHODS: Nine patients were identified over a 2 year period who fitted a clinical picture consistent with the features of lymphangiectasia haemorrhagica. Three were treated for frequent recurrent haemorrhages with a newly developed technique using the argon laser and followed for up to 2 years. RESULTS: None of the three treated patients has had a recurrence of the condition to date. In the six untreated patients the condition resolved spontaneously after repeated attacks over between 8 months and 8 years. CONCLUSIONS: Lymphangiectasia haemorrhagica conjunctivae is probably more common than expected and may resolve spontaneously, but is readily amenable to treatment with the argon laser if attacks become frequent, uncomfortable or unsightly.

Adult↗
PubMed2002

Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia.

Primary lymphoedema associated with chylous reflux is a very rare clinical entity. We report a 3-year-old girl with unilateral lymphoedema, xanthomatosis and vaginal lymphorrhoea. Biopsy also revealed intestinal lymphangiectasia. This paper also presents a brief review of the literature and draws attention to the significance of the xanthomatous eruption in the diagnosis of a chylous reflux.

Child, Preschool↗