Cases reported "Lymphangiectasis"

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1/53. Pleuroperitoneal shunt in the management of chylothorax caused by thoracic lymphatic dysplasia.

    Three cases of intractable chylothorax secondary to thoracic lymphatic dysplasia were treated by pleuroperitoneal shunt insertion. These cases included one with Gorham's syndrome, and one case with a bilateral chylothorax and chylous ascites. Pleuroperitoneal shunts allowed an adequate internal drainage in all cases, alleviating protein and lymphocyte losses caused by recurrent pleural taps. In the third case with chylous ascites, valved shunts were used to avoid reflux between the peritoneal cavity and the pleural space. Such palliative therapy did not change the bad prognosis of these patients with lymphatic disorders but improved the children's quality of life.
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2/53. Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites.

    BACKGROUND: Lymphangiectasia results from acquired dilation of lymphatic vessels. Areas of skin affected by obstruction or destruction of lymphatic drainage are said to be prone to the development of lymphangiectasia. Cirrhosis is a cause of alterations of lymph flow. methods: Case report. RESULTS: We report a case of acquired, late-onset, lymphangiectasia associated with alcoholic hepatic cirrhosis. Lesions were scattered over the right, lower, anterior abdominal wall, a region that is drained by a common group of lymphatic vessels that were probably disordered. CONCLUSION: We think that this is the first reported case of lymphangiectasia associated with altered lymph flow in cirrhosis and ascites.
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3/53. 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.
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4/53. A case of noonan syndrome with pulmonary and abdominal lymphangiectasia.

    noonan syndrome is characterised by a Turner-like phenotype and a normal karyotype. Although it is reported to be associated with abnormalities of the lymphatic system, involvement of the pulmonary lymphatics is rare. We present a case of noonan syndrome where a whole body scintigraphy revealed lymphangiectasia of the lower extremities, abdomen and lungs.
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5/53. An imaging evaluation of angiodysplasia syndromes.

    Current imaging techniques such as magnetic resonance, magnetic resonance angiography, computer tomography, ultrasound, plain x-rays, and lymphangioscintigraphy have enhanced the ability to define blood and lymph vascular malformations in more precise pathophysiologic terms. Not only can these imaging modalities distinguish arterial anomalies from lymphatic and venous angiodysplasia, but they also readily differentiate edema in the epifascial as opposed to the subfascial peripheral compartments. Moreover, visceral lymphangiectasia (e.g., chylous and non-chylous reflux), bone and muscle overgrowth, agenesis, and fat deposits can also be delineated. Clinical examples are provided including an algorithm for approaching these conditions.
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6/53. Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades.

    A 28-year old woman presented with Leri's disease (melorheostosis) and the rare combination of complex vascular malformations and lymphatic anomalies. Multifocal melorheostosis was segmental and unilateral, located in the left axial and peripheral skeleton, fifth thoracic vertebral body, fifth rib. left upper limb and lumbosacral spine (third lumbar body to first sacral segment). Sacral involvement was associated with spinal canal stenosis. Additionally the patient had multiple nevi and had suffered from left hemiplegia since birth. Lymphangiectasia of the mesentery and thorax led to chylothorax resistant to therapy for which the patient underwent a pleuropericardiectomy. death ensued due to respiratory failure.
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7/53. Lymphangiectatic fibrolipomatous polyp of the palatine tonsil.

    A rare benign polypoid tumor of the right palatine tonsil is described in a 23 year old male. It contained dilated lymphatic channels surrounded by fibrous tissue and foci of mature fat. The features of this lesion add support to the hypothesis that benign tumors of tonsil may be hamartomas of tonsil rather than true neoplasms. The differential diagnosis of polypoid lesions of the tonsil is discussed.
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8/53. Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy.

    Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.
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9/53. lymphedema-lymphangiectasia-mental retardation (Hennekam) syndrome: a review.

    The Hennekam syndrome is an infrequently reported heritable entity characterized by lymphedema, lymphangiectasia, and developmental delay. Here we add an additional 8 patients, and compare their findings to the 16 cases from the literature. The lymphedema is usually congenital, can be markedly asymmetrical, and, often, gradually progressive. Complications such as erysipelas are common. The lymphangiectasias are present in the intestines, but have also been found in the pleura, pericardium, thyroid gland, and kidney. Several patients have demonstrated congenital cardiac and blood vessel anomalies, pointing to a disturbance of angiogenesis in at least some of the patients. Facial features are variable, and are chiefly characterized, in a typical patient, by a flat face, flat and broad nasal bridge, and hypertelorism. Facial features are thought to mirror the extent of intrauterine facial lymphedema, or may be caused by lymphatic obstruction that affects the early migration of neural crest tissue. Other anomalies have included glaucoma, dental anomalies, hearing loss, and renal anomalies. The psychomotor development varies widely, even within a single family, from almost normal development to severe mental retardation. Convulsions are common. The existence of 10 familial cases, equal sex ratio, increased parental consanguinity rate (4/20 families), and absence of vertical transmission are consistent with an autosomal recessive pattern of inheritance. It seems likely that most (but not all) manifestations of the entity can be explained as sequences of impaired prenatal and postnatal lymphatic flow, suggesting that the causative gene(s) should have a major function in lymphangiogenesis.
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10/53. Thoracic lymphangiectasis presenting with chyloptysis and bronchial cast expectoration.

    A 70-year-old man with recurrent undiagnosed episodes of bronchial cast expectoration and pulmonary infiltrates on chest radiography for 15 years is described. The diagnosis of chyloptysis was established by chemical analysis of the bronchial aspiration. We emphasize the radiological findings of this rare observation. The CT-associated lymphangiography showed mediastinal lymphangiectasis with retrograde opacification of mediastinal and hilar lymph nodes as well as submucosal lymphatic vessels protruding into the lumen of the tracheo-bronchial tree without evidence of thoracic duct obstruction as well as a "crazy-paving appearance." Congenital incompetence of the valves of the lymphatic vessels originating from the thoracic duct is held to be the cause. Chyloptysis and pulmonary lymphatic disorder should be sought in cases of bronchial cast expectoration.
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