We report the case of a 25-year-old male with Neurofibromatosis type I (NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstructi...We report the case of a 25-year-old male with Neurofibromatosis type I (NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient’s symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen’s disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease.展开更多
The extrinsic compression of the third part of the duodenum as it passes through the aorto-mesenteric angle is known as the superior mesenteric artery syndrome(SMAS).This syndrome is a rare mechanical cause of upper i...The extrinsic compression of the third part of the duodenum as it passes through the aorto-mesenteric angle is known as the superior mesenteric artery syndrome(SMAS).This syndrome is a rare mechanical cause of upper intestinal obstruction,with a reported incidence of between 0.2% and 0.78%.Clinical manifestations of SMAS may be chronic or acute;chronic symptoms include intermittent gastric pain,fullness and occasional episodes of postprandial vomiting,while acute symptoms include incoercible vomiting,oral intolerance,mainly epigastric abdominal distension and abdominal pain.Surgery is recommended only when initial conservative treatment fails.Here,we report what appears to be the third published case of SMAS associated with hereditary motor and sensory neuropathy or Charcot Marie Tooth disease.展开更多
文摘We report the case of a 25-year-old male with Neurofibromatosis type I (NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient’s symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen’s disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease.
文摘The extrinsic compression of the third part of the duodenum as it passes through the aorto-mesenteric angle is known as the superior mesenteric artery syndrome(SMAS).This syndrome is a rare mechanical cause of upper intestinal obstruction,with a reported incidence of between 0.2% and 0.78%.Clinical manifestations of SMAS may be chronic or acute;chronic symptoms include intermittent gastric pain,fullness and occasional episodes of postprandial vomiting,while acute symptoms include incoercible vomiting,oral intolerance,mainly epigastric abdominal distension and abdominal pain.Surgery is recommended only when initial conservative treatment fails.Here,we report what appears to be the third published case of SMAS associated with hereditary motor and sensory neuropathy or Charcot Marie Tooth disease.
