ABSTRACT
Peutz-Jeghers syndrome is a rare autosomal dominant disorder defined by harmatomatous polyps of the gastrointestinal tracts and the occurrence of melanin spots on the lips(oral mucosa), hands and feet. This syndrome is associated with a variety of gastrointestinal and extraintestinal neoplasms, both benign and malignant. A 28-year old male was admitted to our hospital with abdominal pain and nausea for two months. He had melanin spots involving his lips, buccal mucosa, hands and feet. A small bowel series showed luminal obstruction at the forth portion of the duodenum. And abdominal CT scan showed target-like mass suggesting intussusception on the small bowel. On laparotomy, there was an annular constricting mass adherent to retroperitoneum with metastasis to preaortic lymph nodes. Palliative duodeno-jejunostomy was performed. The histologic finding disclosed signet-ring cell type adenocarcinoma.
Subject(s)
Adult , Humans , Male , Abdominal Pain , Adenocarcinoma , Duodenum , Foot , Gastrointestinal Tract , Hand , Intestinal Obstruction , Intussusception , Laparotomy , Lip , Lymph Nodes , Melanins , Mouth Mucosa , Nausea , Neoplasm Metastasis , Peutz-Jeghers Syndrome , Phenobarbital , Polyps , Tomography, X-Ray ComputedABSTRACT
Chylothorax is a debilitating condition to the point of threatening life. Diagnosis and subsequent management present significant problems for the clinician and the adverse effects of chyle loss on nutrition status and immune function result in devastating consequences for the patient. prompt diagnosis is essential to institute an effective therapeutic regimen. we reported a patient of idiopathic bilateral chylothorax treated by conservative approach with review of literatures.