AIM:To investigate the spatial distribution patterns of anorectal atresia/stenosis in China.METHODS:Data were collected from the Chinese Birth Defects Monitoring Network(CBDMN),a hospital-based congenital malformation...AIM:To investigate the spatial distribution patterns of anorectal atresia/stenosis in China.METHODS:Data were collected from the Chinese Birth Defects Monitoring Network(CBDMN),a hospital-based congenital malformations registry system.All fetuses more than 28 wk of gestation and neonates up to 7 d of age in hospitals within the monitoring sites of the CBDMN were monitored from 2001 to 2005.Two-dimensional graph-theoretical clustering was used to divide monitoring sites of the CBDMN into different clusters according to the average incidences of anorectal atresia/stenosis in the different monitoring sites.RESULTS:The overall average incidence of anorectal atresia/stenosis in China was 3.17 per 10000 from 2001 to 2005.The areas with the highest average incidences of anorectal atresia/stenosis were almost always focused in Eastern China.The monitoring sites were grouped into 6 clusters of areas.Cluster 1 comprised the monitoring sites in Heilongjiang Province,Jilin Province,and Liaoning Province;Cluster 2 was composed of those in Fujian Province,Guangdong Province,Hainan Province,Guangxi Zhuang Autonomous Region,south Hunan Province,and south Jiangxi Province;Cluster 3 consisted of those in Beijing Municipal City,Tianjin Municipal City,Hebei Province,Shandong Province,north Jiangsu Province,and north Anhui Province;Cluster 4 was made up of those in Zhejiang Province,Shanghai Municipal City,south Anhui Province,south Jiangsu Province,north Hunan Province,north Jiangxi Province,Hubei Province,Henan Province,Shanxi Province and Inner Mongolia Autonomous Region;Cluster 5 consisted of those in Ningxia Hui Autonomous Region,Gansu Province and Qinghai Province;and Cluster 6 included those in Shaanxi Province,Sichuan Province,Chongqing Municipal City,Yunnan Province,Guizhou Province,Xinjiang Uygur Autonomous Province and Tibet Autonomous Region.CONCLUSION:The fi ndings in this research allow the display of the spatial distribution patterns of anorectal atresia/stenosis in China.These will have important guiding significance for further analysis of relevant environmental factors regarding anorectal atresia/ stenosis and for achieving regional monitoring for anorectal atresia/stenosis.展开更多
Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic...Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscularthickening, tracheobronchial remnants(TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography(EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.展开更多
BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-yea...BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed.Diagnosis was made based on the patient's clinical symptoms,signs,imaging showing the fistula,X-ray and magnetic resonance imaging results.The preoperative examination was improved.Anorectoplasty was performed.The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up.CONCLUSION Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.展开更多
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and ...Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still contro-versial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.展开更多
Anorectal stenosis(ARS)is a rare complication of Crohn's disease(CD)and a risk factor for both fecal diversion(FD)and proctectomy,[1,2]especially in patients who failed anti-tumor necrosis factor-α(anti-TNF)thera...Anorectal stenosis(ARS)is a rare complication of Crohn's disease(CD)and a risk factor for both fecal diversion(FD)and proctectomy,[1,2]especially in patients who failed anti-tumor necrosis factor-α(anti-TNF)therapy)[3,4]Herein,we presented a case of a 17-year-old female diagnosed with CD at age 7,with inflammation located in the small bowel and colon(Paris classification:L3L4b).Due to corticosteroid dependency,infliximab with concomitant azathioprine was introduced at age 9 and discontinued two years after,because of the appearance of a 3 cm tumor-mass,which resembled a lymphoma,in the oral cavity.展开更多
基金Supported by The National Science & Technology Pillar Program during the Eleventh Five-year Plan Period, Grant No. 2006BAI05A01
文摘AIM:To investigate the spatial distribution patterns of anorectal atresia/stenosis in China.METHODS:Data were collected from the Chinese Birth Defects Monitoring Network(CBDMN),a hospital-based congenital malformations registry system.All fetuses more than 28 wk of gestation and neonates up to 7 d of age in hospitals within the monitoring sites of the CBDMN were monitored from 2001 to 2005.Two-dimensional graph-theoretical clustering was used to divide monitoring sites of the CBDMN into different clusters according to the average incidences of anorectal atresia/stenosis in the different monitoring sites.RESULTS:The overall average incidence of anorectal atresia/stenosis in China was 3.17 per 10000 from 2001 to 2005.The areas with the highest average incidences of anorectal atresia/stenosis were almost always focused in Eastern China.The monitoring sites were grouped into 6 clusters of areas.Cluster 1 comprised the monitoring sites in Heilongjiang Province,Jilin Province,and Liaoning Province;Cluster 2 was composed of those in Fujian Province,Guangdong Province,Hainan Province,Guangxi Zhuang Autonomous Region,south Hunan Province,and south Jiangxi Province;Cluster 3 consisted of those in Beijing Municipal City,Tianjin Municipal City,Hebei Province,Shandong Province,north Jiangsu Province,and north Anhui Province;Cluster 4 was made up of those in Zhejiang Province,Shanghai Municipal City,south Anhui Province,south Jiangsu Province,north Hunan Province,north Jiangxi Province,Hubei Province,Henan Province,Shanxi Province and Inner Mongolia Autonomous Region;Cluster 5 consisted of those in Ningxia Hui Autonomous Region,Gansu Province and Qinghai Province;and Cluster 6 included those in Shaanxi Province,Sichuan Province,Chongqing Municipal City,Yunnan Province,Guizhou Province,Xinjiang Uygur Autonomous Province and Tibet Autonomous Region.CONCLUSION:The fi ndings in this research allow the display of the spatial distribution patterns of anorectal atresia/stenosis in China.These will have important guiding significance for further analysis of relevant environmental factors regarding anorectal atresia/ stenosis and for achieving regional monitoring for anorectal atresia/stenosis.
文摘Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscularthickening, tracheobronchial remnants(TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography(EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.
文摘BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed.Diagnosis was made based on the patient's clinical symptoms,signs,imaging showing the fistula,X-ray and magnetic resonance imaging results.The preoperative examination was improved.Anorectoplasty was performed.The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up.CONCLUSION Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.
基金Supported by Fundao de Amparo à Pesquisa do Estado de Minas Gerais, BrazilConselho Nacional de Desenvolvimento Científico e Tecnológico, Brazil+1 种基金FAPEMIG: CBB-APQ-00075-09/ CNPq 573646/2008-2Programa de Grupos de ExcelênciaFINEP, Brazil
文摘Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still contro-versial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.
文摘Anorectal stenosis(ARS)is a rare complication of Crohn's disease(CD)and a risk factor for both fecal diversion(FD)and proctectomy,[1,2]especially in patients who failed anti-tumor necrosis factor-α(anti-TNF)therapy)[3,4]Herein,we presented a case of a 17-year-old female diagnosed with CD at age 7,with inflammation located in the small bowel and colon(Paris classification:L3L4b).Due to corticosteroid dependency,infliximab with concomitant azathioprine was introduced at age 9 and discontinued two years after,because of the appearance of a 3 cm tumor-mass,which resembled a lymphoma,in the oral cavity.
