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[Pulmonary lymphangiectasis with spontaneous chylothorax in Noonan syndrome].

We report a case of Noonan syndrome associated with pulmonary stenosis and major lymphedema of the lower extremities. At the age of 15 yr spontaneous chylothorax with increasing dyspnea occurred> Chest-x-ray demonstrated increased interstitial markings restricted to the right lower lobe representing pulmonary lymphangiectasia. The chylothorax did not respond to repeated thoracocentesis and medium-chain-triglyceride diet. When a chest tube was inserted and total parenteral nutrition was supplied, the chylous effusion decreased within 32 days. The patient is still on diet and asymptomatic effusion remained during 12 months follow up. In conclusion, pulmonary lympgangiectasia should be considered in patients with Noonan syndrome and an abnormal interstitial pulmonary pattern similar to pulmonary congestion (without any hemodynamic abnormalities). In case of pleural effusion, chylothorax should be considered.

Adolescent↗
PubMed1995

[Bowenoid papulosis, Bowen's disease and squamous cell carcinoma of the anus and vulva in congenital intestinal lymphangiectasis].

A 32-year-old woman with congenital intestinal lymphangiectasia (CIL) and warts (condylomata acuminata) was found to have bowenoid papulosis, Bowen's disease and squamous-cell carcinoma, at first in the anus, later also in the vulva. Limited surgical measures and laser vaporization with systemic and topical administration of interferon controlled the tumour development for some time. But after 7 years the squamous-cell carcinoma recurred with infiltration of the outer anogenital region. The patient then had an episode of thrombotic cerebral ischemia, which prevented a planned abdominoperineal resection with radical vulvectomy. Instead she received chemotherapy with bleomycin, mitomycin and cisplatin. But she died 8 weeks later, from tumour cachexia. Occurrence of a squamous-cell carcinoma of the anus and vulva in this young patient suggests a high oncogenic potential of the papilloma virus (HPV) infection. The latent period was probably shortened by a cellular immune deficiency as part of CIL. Treatment of the various carcinomatous manifestations should as long as possible be by local measures and interferon administration.

Adult↗
PubMed1991

[Therapy-refractory primary intestinal lymphangiectasis].

A 20-year-old man with severe physical weakness (from which he soon recovered spontaneously) was found to have hypoproteinaemia and lymphocytopenia (768/microliters). Ten years later, after having been free of symptoms in the meantime, oedema, ascites and abdominal pain occurred, associated with loss of physical capacity. Enteric loss of protein (alpha 1-antitrypsin clearance increased about thirtyfold) with a total serum protein concentration of only 3.7 g/dl, as well as histological evidence of lymphangiectasia of the small and large intestines provided the diagnosis of primary intestinal lymphangiectasia. In the course of the disease cholelithiasis and (after treatment with tranexamic acid) thrombosis of the axillary vein occurred. No treatment has been of any avail and the patient has been unable to work for three years.

Adult↗
PubMed1995

Unilateral lymphangiectasis of the kidney: an unusual cause of renal enlargement in an infant.

We report on an infant with unilateral renal lymphangiectasia and the absence of small cysts in the renal parenchyma or renal hilum. We suggest that renal lymphangiectasia be part of the differential diagnosis in children who present with unilateral renal enlargement, especially when contrast enhanced computerized tomography shows preservation of a rim of renal cortex with decreased attenuation of the medulla and/or when ultrasound demonstrates increased echogenicity without displacement of the caliceal system.

Humans↗