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Progressive melorheostosis in the peripheral and axial skeleton with associated vascular malformations: imaging findings over three decades.
A 28-year old woman presented with Léri'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.
Renal lymphangiectasia: radiologic diagnosis and evolution.
Renal lymphangiectasia is a rare renal entity. Symptoms and radiologic findings are well defined, but its clinical evolution is not totally well known. We report a severe case of renal lymphangiectasia with a large right perirenal collection and ascites. Both resolved 6 years later on computed tomography, but right intrarenal involvement worsened. To our knowledge, this form of evolution has not been described.
Drainage of subcutaneous lymphatic fluid for the management of respiratory distress in a case of generalized lymphangiectasia in an infant.
A 10-month-old girl was referred to our hospital because of congenital and persistent bilateral chylothorax and generalized lymphedema as well as long-standing respiratory disturbance. Radiological studies showed a diffuse network of superficial lymphatic vessels without major trunks throughout her entire body as well as the lung. She was diagnosed with systemic lymphangiomatosis complicated with pulmonary lymphangiectasia. Percutaneous puncture in the lower leg was performed to discharge the lymphatic fluid and proved to be effective for the respiratory disturbance. This procedure is safe and easy and effectively improves the quality of life of the patient and the family in case of such a persistent disease.
Unusual vascular dysplasia presenting as an intra-abdominal catastrophe.
A case of severe, generalised vascular dysplasia presenting as an intra-abdominal catastrophe is reported. Associated clinical features included cutis marmorata telangiectatica congenita, congenital glaucoma, hypertension, and focal fits. The case has been reported in view of its rarity and nature of presentation.
An unusual presentation of intestinal lymphangiectasia.
Intestinal lymphangiectasia is a rare disorder, usually presenting with protein-losing enteropathy leading to oedema and ascites. We describe a case that presented as an acute surgical emergency with pain and a mass in the right iliac fossa, which was successfully treated by resection of the involved segment of ileum.
Familial non-immune hydrops fetalis and congenital pulmonary lymphangiectasia.
UNLABELLED: We report on three siblings with non-immune hydrops fetalis. Congenital pulmonary lymphangiectasia was diagnosed in two of them. One of these, a girl still alive and suffering from frequent airway infections, has bilateral pleural effusions and distal congenital lymphoedema. CONCLUSION: To our knowledge, this is the first report of non-immune hydrops fetalis and congenital pulmonary lymphangiectasia occurring in siblings.
Cystic renal lymphangiectasia presenting as renal insufficiency in childhood.
Cystic renal lymphangiectasia is an unusual cause of cystic renal disease in childhood. We present a case of bilateral cystic renal lymphangiectasia in a 7-year-old boy who presented with asymptomatic renal insufficiency and anemia with decreased erythropoietin production. The clinical features of this condition and the diagnostic approach are reviewed. Although rare, this disorder should be considered in the differential diagnosis of cystic renal disease.
End-stage renal disease in a patient with congenital lymphangiectasia and lymphedema.
Congenital lymphangiectasia with lymphedema is a disorder constituting the main defect in many different genetic syndromes. Herein we describe a 23-year-old male patient with congenital lymphangiectasia and severe lymphedema of the right leg, scrotum, and abdominal wall, who presented with end-stage renal disease, presumably due to cystic renal lymphangiectasia, and is undergoing chronic hemodialysis treatment.
Primary intestinal lymphangiectasia successfully treated with octreotide.
A 21-year-old man with diarrhea and edema was admitted to our hospital and diagnosed with protein-losing enteropathy caused by primary intestinal lymphangiectasia. He was placed, in turn, on a low-fat diet, an elemental diet, and, subsequently, fasting therapy with total parenteral nutrition (TPN) support. However, his symptoms were not relieved, but, rather were exacerbated. On the 45th day of hospitalization, octreotide therapy was initiated. After 2 weeks of treatment, his clinical symptoms, as well as hypoproteinemia and hypoalbuminemia, gradually became alleviated. The improvement was confirmed in terms of scintigraphy, endoscopy, and histology of the duodenum. The patient remained healthy until 6 months after the commencement of octreotide treatment, when he discontinued octreotide at his own discretion, at which point the symptoms recurred. Resumption of the drug, however, again brought about remission, which has continued until the present, March 2000. Thus, octreotide therapy is one modality which may be useful for refractory primary intestinal lymphangiectasia.
What are the objectives of treatment for intestinal lymphangiectasia?
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Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition.
Intestinal lymphangiectasia (IL) is a rare disease requiring oral fat restriction. The aim of this study was to evaluate the efficacy of enteral nutrition compared to that of total parenteral nutrition (TPN). We retrospectively reviewed nine patients with IL presenting with protein-losing enteropathy. Of these, seven patients not responding to a low-fat diet were treated with elemental diet (ED), polymeric diet (PD) containing medium-chain triglycerides, or TPN. Improvement in serum total protein was observed in two of three on ED and in one of two on PD, compared with three of three on TPN. Enteric protein loss was improved in two of two on ED, one of two on PD, and two of two on TPN. Outpatients who continued to receive enteral nutrition maintained a total protein level. Enteral nutirition appears to be as effective as TPN for patients with IL, and it may provide a valid and safe alternative therapy.
Unilateral renal lymphangiectasia: imaging appearance on sonography, CT and MRI.
Lymphangiomas are benign tumors of unknown etiology often seen in children, involving the neck (75%), and axillary (20%) region, but rarely the kidney [Zapzalka et al. (2002) Lymphangioma of the renal capsule. J Urol 168:220.]. We report a case of unilateral renal lymphangiectasia, where the diagnosis was made on imaging.
Isolated massive chylopericardium.
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Kerley B lines in total anomalous pulmonaru venous connection below the diaphragm (type 3).
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Apolipoprotein B subspecies in chylomicrons isolated from a patient with chyluria.
The diagnosis and the clinical course of a 17-year-old white male with chyluria are reported. Cloudy, milky urine appeared spontaneously, in the absence of edema or any signs or symptoms of parasitic infection. Pedal lymphangiography demonstrated the presence of a lymphatic renal fistula, and digital subtraction angiography showed aneurysmal dilatation of the aorta at the level of the renal arteries. This case provided an opportunity to ascertain which of the forms of apolipoprotein B were present in lymph chylomicrons. Apolipoprotein B is needed for chylomicron secretion. It exists in several forms--B-100, B-74, B-48, and B-26. After a meal consisting of fat, chylomicrons in which apolipoprotein B-48 was virtually the only apolipoprotein B present appeared in the urine, while apolipoprotein B-100 was the only apolipoprotein B present in the plasma very low-density lipoproteins. Chyluria disappeared two weeks after institution of a low-fat diet. This case illustrates an interesting, rare cause of chyluria. Because of the presence of chyluria, it was also demonstrated that chylomicrons in which apolipoprotein B-48 is virtually the only apolipoprotein B present are a physiologically normal product of the intestine.
Congenital bronchopulmonary malformations.
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Brugia pahangi: infections and their effect on the lymphatic system of Mongolian jirds (Meriones unguiculatus).
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