We are reporting the first documented case of an abdominal desmoid tumor presenting primarily after liver transplantation. This tumor, well described in the literature as occurring both in conjunction with familial ad...We are reporting the first documented case of an abdominal desmoid tumor presenting primarily after liver transplantation. This tumor, well described in the literature as occurring both in conjunction with familial adenomatous polyposis as well as in the postsurgical patient, has never been noted after solid organ transplantation and was therefore not included in our differential upon presentation. Definitive diagnosis required the patient to undergo surgical excision and immunochemical staining of the mass for confirmation. A review of the literature showed no primary tumors after transplantation. In a population of patients who received a small bowel transplant after they developedshort gut post radical resection of aggressive fibromatosis, only rare recurrences were seen. No connection of tumor development with immunosuppression or need to decrease immunosuppressant treatment has been demonstrated in these patients. Our case and the literature show the risk of this tumor presenting in the post-transplantation patient and the need for a high index of suspicion in patients who present with a complex mass after transplantation to prevent progression of the disease beyond a resectable lesion. Results of a thorough search of the literature are detailed and the medical and surgical management of both resectable and unresectable lesions is reviewed.展开更多
BACKGROUND Intussusception is a primary cause of intestinal obstruction in young children.Delayed diagnosis is associated with increased morbidity.Ultrasonography(USG)is the gold standard for diagnosis,but it is opera...BACKGROUND Intussusception is a primary cause of intestinal obstruction in young children.Delayed diagnosis is associated with increased morbidity.Ultrasonography(USG)is the gold standard for diagnosis,but it is operator dependent and often unavailable in limited resource areas.AIM To study the clinical characteristics of intussusception including management and evaluation of the diagnostic accuracy of abdominal radiography(AR)and the promising parameters found in the pediatric intussusception score(PIS).METHODS Children with suspected intussusception in our center from 2006 to 2018 were recruited.Clinical manifestations,investigations,and treatment outcomes were recorded.AR images were interpreted by a pediatric radiologist.Diagnosis of intussusception was composed of compatible USG and response with reduction.The diagnostic value of the proposed PIS was evaluated.RESULTS Ninety-seven children were diagnosed with intussusception(2.06±2.67 years,62.9%male),of whom 74%were<2 years old and 37.1%were referrals.The common manifestations of intussusception were irritability or abdominal pain(86.7%)and vomiting(59.2%).Children aged 6 mo to 2 years,pallor,palpable abdominal mass,and positive AR were the parameters that could discriminate intussusception from other mimics(P<0.05).Referral case was the only significant parameter for failure to reduce intussusception(P<0.05).AR to diagnose intussusception had a sensitivity of 59.2%.The proposed PIS,a combination of clinical irritability or abdominal pain,children aged 6 mo to 2 years,and compatible AR,had a sensitivity of 85.7%.CONCLUSION AR alone provides poor screening for intussusception.The proposed PIS in combination with common manifestations and AR data was shown to increase the diagnostic sensitivity,leading to timely clinical management.展开更多
文摘We are reporting the first documented case of an abdominal desmoid tumor presenting primarily after liver transplantation. This tumor, well described in the literature as occurring both in conjunction with familial adenomatous polyposis as well as in the postsurgical patient, has never been noted after solid organ transplantation and was therefore not included in our differential upon presentation. Definitive diagnosis required the patient to undergo surgical excision and immunochemical staining of the mass for confirmation. A review of the literature showed no primary tumors after transplantation. In a population of patients who received a small bowel transplant after they developedshort gut post radical resection of aggressive fibromatosis, only rare recurrences were seen. No connection of tumor development with immunosuppression or need to decrease immunosuppressant treatment has been demonstrated in these patients. Our case and the literature show the risk of this tumor presenting in the post-transplantation patient and the need for a high index of suspicion in patients who present with a complex mass after transplantation to prevent progression of the disease beyond a resectable lesion. Results of a thorough search of the literature are detailed and the medical and surgical management of both resectable and unresectable lesions is reviewed.
基金Thailand Science Research and Innovation Fund Chulalongkorn University,No.HEA663000047.
文摘BACKGROUND Intussusception is a primary cause of intestinal obstruction in young children.Delayed diagnosis is associated with increased morbidity.Ultrasonography(USG)is the gold standard for diagnosis,but it is operator dependent and often unavailable in limited resource areas.AIM To study the clinical characteristics of intussusception including management and evaluation of the diagnostic accuracy of abdominal radiography(AR)and the promising parameters found in the pediatric intussusception score(PIS).METHODS Children with suspected intussusception in our center from 2006 to 2018 were recruited.Clinical manifestations,investigations,and treatment outcomes were recorded.AR images were interpreted by a pediatric radiologist.Diagnosis of intussusception was composed of compatible USG and response with reduction.The diagnostic value of the proposed PIS was evaluated.RESULTS Ninety-seven children were diagnosed with intussusception(2.06±2.67 years,62.9%male),of whom 74%were<2 years old and 37.1%were referrals.The common manifestations of intussusception were irritability or abdominal pain(86.7%)and vomiting(59.2%).Children aged 6 mo to 2 years,pallor,palpable abdominal mass,and positive AR were the parameters that could discriminate intussusception from other mimics(P<0.05).Referral case was the only significant parameter for failure to reduce intussusception(P<0.05).AR to diagnose intussusception had a sensitivity of 59.2%.The proposed PIS,a combination of clinical irritability or abdominal pain,children aged 6 mo to 2 years,and compatible AR,had a sensitivity of 85.7%.CONCLUSION AR alone provides poor screening for intussusception.The proposed PIS in combination with common manifestations and AR data was shown to increase the diagnostic sensitivity,leading to timely clinical management.
