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Vascular anomalies (birthmarks) commonly involve the feet and ankles. Little is known about these anomalies among practicing physicians. In this article, vascular anomalies are described, and detailed information is presented regarding appropriate diagnostic work-up and treatment strategies.
Cystic hygromas are benign lesions arising due to an abnormality in lymphatic development and only rarely present in adults. Adequate radiological imaging prior to surgery is important as incomplete excision often leads to recurrence. Several adjunctive therapies have been shown to be beneficial in recurrent or inaccessible lesions but these are not in common use. We describe the case of a cystic lymphangioma appearing in adulthood that presented a diagnostic and therapeutic challenge.
Infections, sepsis and multiple organ failure syndrome are associated with high morbidity and mortality in human and experimental small bowel transplantation (SBTx). These complications are attributed to bacterial translocation demonstrated in animal and human studies. Bacterial translocation (BT) is defined as the passage of viable bacteria from the intestinal lumen to other tissues or organs. BT has been associated with different clinical and experimental situations, hemorrhagic shock, trauma, bowel obstruction, immunodepression, total parenteral nutrition, antibiotics. Although BT has been investigated in several small and large animal models of SBTx, precise information on the mechanisms involved are not available. It is possible that the operative procedure by itself may promote BT for the interaction of a number of factors such as preservation, ischemia/reperfusion, abnormal motility, lymphatic disruption and aberrant systemic venous drainage, acute or chronic rejection and antibiotic therapy. Furthermore, the potent immunosuppressive therapy used in these patients may augment the deleterious effects caused by BT. In this review we examined the existing literature concerning BT with particular regard to intestinal transplantation, to better understand the alterations in the symbiotic relationship between immunocompromised host and his gut microflora after SBTx.
Aneurysms, hemangiomas, congenital arteriovascular and lymphatic fistulas often formed as a result of genetically determined defects in the vessel wall structure. They contribute to the development of sysytemic diseases like hypertension or circulatory failure. The author concentrates on the definition of malformation and its types, the review of clinical studies and guidelines for diagnosis and treatment.
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Explore the source record for details and available documents.
To evaluate the clinical effect of microwave on commonly seen benign tumors or malformations of the oral cavity, 146 patients were randomly selected. There were 78 patients with mucous cyst, 22 patients with papilloma, 24 patients with hemangioma or malformation, 6 patients with lymphatic malformation and 16 patients with epulis. All the patients were treated by ECO-100 microwave. It is concluded that 126 patients were cured (86.3%), 20 patients had good response (13.7%) after treatment.
According to the classification of ISSVA, Waner and Suen, the traditionally called lymphangiomas are now referred to as lymphatic malformations, including both macrocystic and microcystic lesions. They are commonly seen vascular anomalies, and most frequently diagnosed at birth and most often occur in the head and neck area. The etiology of lymphatic malformations is still unknown, resulting in a variety of treatment modalities. This paper reviewed the recent literatures with respect to the indications, contraindications, advantages and disadvantages of surgical excision, sclerotherapy and laser ablation of lymphatic malformations of the head and neck, with detailed discussion of the advances in molecular biology and clinical treatment prospects. It is concluded that although lymphatic malformations are benign lesions, they seldom involute spontaneously. Of all vascular malformations, lymphatic malformations are the most difficult to be eradicated. Their infiltrating nature coupled with the difficulty in distinguishing involved important structures of the head and neck from adjacent normal tissues makes complete surgical extirpation even more difficult. The likelihood of postsurgical recurrence and complications is thus higher than other vascular lesions. Although many treatment protocols are available in clinic, indications, contraindications, advantages and disadvantages exist in each modality. The selection of treatment modalities should depend on the patients' status and techniques available. The treatment protocol should be individualized and comprehensive as well as sequential, in order to obtain the best treatment outcomes. In general, treatment will vary according to the depth and the extent of the lesions. Superficial mucosal microcystic lesions and cervicofacial macrocystic lesions are amenable to ablation with sclerotherapy using Bleomycin and OK-432 with the advantages of the absence of a surgical scar. The sclerosing agent OK-432 is effective for macrocystic lymphatic malformations but showed less promise for microcystic lesions, mixed lesions, and lesions outside the head and neck region. In addition, superficial mucosal microcystic lesions are also amenable to CO2 laser therapy. Deeper microcystic lesions are still challenging head and neck surgeons, which are usually extensive and sometimes need to be resected in stages, and even may be impossible to be completely removed. Somnoplasty shows promise for reduction of tongue lymphatic malformations. Surgical excision, staged when necessary, continues to be integral to management in many cases, but should not be overused without consideration of the histologic types and extent. Localized mucosal microcystic lesions and major cervicofacial macrocystic lesions are amenable to primary excision. Care should be exercised in identifying and preserving important cervical and facial structures because anatomical planes are often distorted. Combined sequential approach is recommended for mixed lesions as well as extensive lesions involving both the mucosa and soft tissues.
Edema due to increased capillary permeability (ICP) may be diffuse or localized. Local edemas (Quincke edema, angioneurotic edema) are most often allergic or very rarely due to a defect in C1-inhibitor. Generalized edemas due to ICP share the following clinical features: Fluid retention (subcutaneous edema and diffused swelling) is predominant in lower limbs; it is worsened by orthostatism and warmth and alleviated by decubitus and cold, with important weight variations between morning and evening. It is associated with enhanced thirst, hypotension, oliguria, headaches and blood volume reduction; secondary hyperaldosteronism is the main mechanism. These troubles are due to ICP, associated with lymphatic drainage abnormalities; ICP is measured by the isotopic Landis Test. This abnormality is present in several diseases. Idiopathic orthostatic edema (IOE) is frequent and often unrecognized, occurring mainly in women, often associated with luteal insufficiency. Iatrogenic complications (diuretic and laxative abuses) are frequently superimposed. ICP may be corrected by vitamins P (rutin, anthocyanosides, diosmin, Ginkgo biloba extracts...) Cyclic shock due to ICP is rare. It is characterized by cyclic edema and shock with hypovolemia, hypoproteinemia; the mechanism of shock is a severe loss of fluid and protein from the vascular bed. It is often associated with monoclonal gammapathy and complement activation. In our personal case, the trouble in CP was present all along the disease with permanent edema and low blood pressure (especially in orthostatism). Vit "P" and Ginkgo biloba extracts were able to partially improve CP and the clinical troubles. However, in spite of this treatment a fatal shock occurred after ten years follow-up. Episodic angioedema associated with eosinophilia was first described by Gleich.(ABSTRACT TRUNCATED AT 250 WORDS)
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Radionuclide lymphoscintigraphy is a useful technique for differentiating lymphedema from other causes of swelling, and may sometimes be useful for delineating collateral lymphatics, the level of obstruction, and the presence of lymphoceles or abnormal collections of lymphatic vessels, if they communicate sufficiently with normal lymphatic vessels. Standardization of technique is important to provide better intrapatient and even interpatient comparison. Symmetry, numbers, and locations of lymphatic vessels, lymph nodes, abnormal collections, and dermal collaterals are helpful in the qualitative assessment of lymphoscintigraphy. In addition, region-of-interest analysis may be used to quantitate the clearance of the radiopharmaceutical from the injection site and its accumulation in draining lymph nodes. The constellation of findings may be used to assess the severity of the lymphatic obstruction, the involvement of clinically normal limbs, and to plan therapy.
The pattern and prevalence of neuropsychological abnormalities in children receiving combination CNS prophylaxis (2000 rads cranial irradiation and intrathecal methotrexate) during therapy for acute lymphoblastic leukemia (ALL) were studied. Thirty five children (25 boys) in the age group 5-15 years (mean 9.3) with no evidence of CNS leukemia were included and 20 age matched normal siblings served as controls. Neuropsychological parameters of general intelligence (Malin's modification of WISC test); attention and concentration (colour cancellation test); memory (modified PGI memory test) and visuomotor perception (Bender Gestalt test) were evaluated at least 6 months after CNS prophylaxis. Six (17.1%) patients had mean intelligence quotients (IQ) less than 85, while all controls had IQ > 85 (p < 0.05). The mean IQ of the patient population (93.4 +/- 11.9) was significantly lower than the control group (107 +/- 8.4) (p < 0.001). Scores on the colour cancellation test were lower in the patients as compared to controls (148.7 +/- 27.7 versus 184.9 +/- 23.9; p < 0.01). The mean memory quotient in the patient population was also lower than in controls (74.5 +/- 12 versus 93.6 +/- 9.2; p < 0.001). Scores on the Bender-Gestalt test did not show a significant difference. The presence of significant neuropsychological abnormalities in patients of ALL indicates the need for modification of the schedule of CNS prophylaxis. A comprehensive psychometric evaluation at regular intervals is essential for longterm rehabilitation.
We report on two cases, one with acute lymphoblastic leukemia and a second with lymphatic blastic phase of Philadelphia chromosome-positive chronic myelogenous leukemia, cytogenetically characterized by ider(9)(q10)t(9;22)(q34;q11). Our findings and the data of the 4 cases previously published indicate that ider(9)(q10)t(9;22)(q34;q11) represents a rare but recurrent chromosomal abnormality occurring in hematological malignancies with lymphoid differentiation, namely acute lymphoblastic leukemia and lymphatic blastic phase of chronic myelogenous leukemia, and most likely evolves from a preexistent der(9) involved in the standard t(9;22).
A patient showing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous angle. Chylous effusions and subcutaneous chyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The "pseudo"-storage disease is regarded as secondary to the backflow of chylus into the tissues.
Lymphangioma circumscriptum (LC) is a defect of lymphatics in deep dermis. This abnormal saccular dilatation of lymphatics occurs in dermal papillae visible as vesicles clinically. The entity may be acquired or congenital. The occurrence of congenital variety of LC in vulva is extremely rare, with only few cases reported in the literature. We communicate one such case presenting as recurrent vulval growth along with a review of literature and treatment modalities.
Myotonic dystrophy is an hereditary disorder of several organ systems. Skeletal muscle is a principal target organ, but abnormalities also occur in the peripheral microcirculation. Because morphological and functional changes in the dermal blood microcirculation may affect interstitial fluid drainage of the skin, we examined dermal lymphatic morphology in adult patients with myotonic dystrophy. Skin biopsies were taken from the big toe from patients with myotonic dystrophy (age 18-50 years) and subjected to light and electron microscopy; five healthy subjects served as controls. The salient findings in myotonic dystrophy were ultrastructural changes of the lymphatic endothelial cells and the fibrillar elements that surround the lymphatic wall. These abnormal lymphatic findings are interpreted in light of changes in the blood microvasculature and loose connective tissue in this disorder.
Multiple organs cooperate to regulate appetite, metabolism, and glucose and fatty acid homeostasis. Here, we identified and characterized lymphatic vasculature dysfunction as a cause of adult-onset obesity. We found that functional inactivation of a single allele of the homeobox gene Prox1 led to adult-onset obesity due to abnormal lymph leakage from mispatterned and ruptured lymphatic vessels. Prox1 heterozygous mice are a new model for adult-onset obesity and lymphatic vascular disease.