ABSTRACT
The blue rubber nevus syndrome consists of multiple venous malformations in the skin and gastrointestinal tract associated with intestinal hemorrhage and iron deficiency anemia. Other organs may be involved. The causes of this syndrome are unknown. Its most common presentation is in the form of sporadic cases, but dominant autosomal inheritance has been described. It is a condition that affects both sexes equally, and its occurrence is rare in the black race. We present a case of this syndrome diagnosed in a 11-year-old patient. He had severe anemia and a venous swelling on the trunk. Similar lesions were found in the stomach, bowel, and on his foot. We emphasize the main clinical aspects: intestine, eyes, nasopharynx, parotids, lungs, liver, spleen, heart, brain, pleura, peritoneum, pericardium, skeletal muscles, bladder, and penis lesions, systemic complications that may occur to these patients which are thrombosis and calcification, as well as consumptive coagulopathy and thrombocytopenia that may occur within the nevi.
Subject(s)
Humans , Male , Child , Nevus, Blue , Skin Neoplasms , Anemia, Iron-Deficiency/diagnosis , Digestive System/pathology , Gastrointestinal Hemorrhage/diagnosis , Nevus, Blue/complications , Nevus, Blue/diagnosis , SyndromeABSTRACT
Säo apresentadas duas crianças com dilataçäo da via biliar em decorrência de anomalia de junçäo entre o colédoco e Wirsung. Forma-se um ducto comum longo, antes da penetraçäo na aprede duodenal, com a possibilidade de refluxo de suco pancreático para a árvore biliar e conseqüente dilataçäo da via biliar. O quadro clínico consistiu de dor no abdome superior, vômitos ou icterícia. O diagnóstico foi feito pela ultra-sonografia e colangiografia endoscópica ou intra-operatória. O tratamento de eleiçäo, realizado em ambos os casos, foi a anastomose biliodigestiva em Y de Roux. O seguimento revelou que as crianças estäo bem, sem sintomas