Cases reported "Lymphatic Abnormalities"

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1/4. Use of the deep inferior epigastric perforator flap in a 15-month-old boy after resection of lymphatic malformation.

    Lymphatic malformations are benign tumors that arise from congenital defects of the lymphatic system. Despite the fact that all lymphatic malformations develop embryologically, their presence may not be detected until the tumor enlarges from hemorrhage or infection. Typical of these tumors is a slow-growing, asymptomatic phase with the potential for rapid and massive expansion and a tendency to infiltrate into surrounding tissues. These tumors are associated with a high rate of recurrence, especially with incomplete excisions. To minimize the chance of recurrence, a complete resection of a groin microcystic lymphatic malformation was performed on this 15-month-old boy, creating a significant defect in the upper thigh. Immediate coverage was felt to be the best option for reducing any associated morbidity. We used a skin-soft tissue flap supplied by perforators of the deep inferior epigastric artery to repair the wound. Using this technique, the musculofascial structures in the abdominal wall were completely spared, and the skin and fat were transferred on a vascular leash to the desired location. The donor site was closed by approximating the fascial edges without tension. The defect on the thigh was filled with tissue of similar thickness and characteristics, avoiding potential contour irregularities and color mismatch. The deep inferior epigastric perforator (DIEP) flap is a useful option for groin defects and can be applied safely in the pediatric population. ( info)

2/4. Lymphatic malformation of the sphenoid sinus in a pediatric patient.

    Lymphatic malformation is a benign vascular lesion resulting from lymphatic tissue being isolated from the remainder of the lymphatic system. They are present at birth and up to 90% are diagnosed by 2 years of age. More aggressive lesions are usually diagnosed earlier, with low-grade lesions presenting later with fewer complications. These lesions are hamartomas and not true neoplasms. The term hamartoma is used to describe an abnormally large mass of histologically normal tissue in a normal location. The lymphatic malformation is composed of lymph-filled channels lined with a single layer of flat endothelial cells on a basement membrane. They present with either generalized edema and poorly defined borders (microcystic) or a localized area of multilocular cysts (macrocystic). The term lymphatic malformation has replaced many other outdated terms, such as lymphangioma, cystic hygroma, lymphangioma circumscriptum, and lymphangiomatosis. In this study, we present a case report of a pediatric lymphatic malformation of the sphenoid sinus. To our knowledge, this lesion has not been described in the pediatric population and has been described only once in an adult in the German literature. EBM rating: C. ( info)

3/4. Nonimmune hydrops fetalis due to generalized lymphatic dysplasia in an infant with Robertsonian trisomy 21.

    We report the first case of generalized lymphatic dysplasia and trisomy 21 presenting with nonimmune hydrops fetalis. This infant showed intractable chylothorax, chylous ascites, and periodic bouts of edema. A karyotype analysis revealed Robertsonian trisomy 21: 46,XY,t(14q21q)(q10;q10) 21. This patient died of multiple organ failure at 400 days of life, despite the management of chylous effusions. The lymphoscintigraphy and histopathological findings led to the final diagnosis of generalized lymphatic dysplasia, which might also contribute to the development of hydrops. Refractory chylothorax in trisomy 21 patients may emphasize the need for intensive scrutiny of lymphatic disorders. ( info)

4/4. Gorham's disease: an autopsy report.

    We present the case report of a 35-year-old man with Gorham's disease (disappearing bone disease, massive osteolysis) with initial clinical findings of small bowel lymphangiomatosis and multicentric osteolysis. The patient, who otherwise was healthy, had a chylothorax develop and he died 9 months later of thoracic and pulmonary complications. An autopsy revealed absence of the proximal thoracic duct and significant lymphangiectatic abnormalities of the pleural, peritoneal, diaphragmatic, splenic, and small bowel tissue with lymphangiomatous masses in the thoracic and mediastinal regions. The findings suggest a subtype of Gorham's disease characterized by a dysplastic lymphatic system. osteolysis was correlated anatomically with lymphangiectatic tissue, suggesting mediation of osteoclastic resorption via local lymphatic tissue factors. ( info)

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