Poland综合征常见的上肢及手畸形的临床特征

Poland 综合征又称胸大肌缺如短指并指综合征，是一种少见的先天性胸壁及上肢畸形。早于1841年由Poland在尸体解剖中发现[1]，1962年Clarkson遇到同样的病例并将此先天畸形命名为Poland综合征[2]。有关其临床特点及治疗策略的研究和报道不断增多，文献报道发病率为1∶7000到1∶100000，男女比例为2∶1到3∶1，右侧发病占60%～75%，累及左侧及双侧的患者少见[3-4]，目前还未发现有明显的家族遗传性，但偶有家族性发病的病例报道[5-6]。

Poland综合征常见的上肢及手畸形的临床特征

Poland 综合征又称胸大肌缺如短指并指综合征，是一种少见的先天性胸壁及上肢畸形。早于1841年由Poland在尸体解剖中发现[1]，1962年Clarkson遇到同样的病例并将此先天畸形命名为Poland综合征[2]。有关其临床特点及治疗策略的研究和报道不断增多，文献报道发病率为1∶7000到1∶100000，男女比例为2∶1到3∶1，右侧发病占60%～75%，累及左侧及双侧的患者少见[3-4]，目前还未发现有明显的家族遗传性，但偶有家族性发病的病例报道[5-6]。

Computed tomography versus transthoracic echocardiography in the detection of complex congenial heart diseases in china:a meta-analysis

Background: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns, and accounting for nearly half of all cases of neonatal intestinal obstruction. This study aimed to review our single-center experience in managing congenital duodenal obstruction while evaluate the outcomes.
Methods: We conducted a retrospective analysis of the records of all neonates dianogsed with congenital duodenal obstruction admitted to our center between January 2003 and December 2012. We analyzed demographic criteria, clinical manifestations, associated anomalies, radiologic findings, surgical methods, postoperative complications, and fi nal outcomes.
Results: The study comprised 287 newborns (193 boys and 94 girls). Birth weight ranged from 950 g to 4850 g. Fifty-three patients were born prematurely between 28 and 36 weeks' gestation. Malrotation was diagnosed in 174 patients, annular pancreas in 66, duodenal web in 55, duodenal atresia or stenosis in 9, preduodenal portal vein in 2, and congenital band compression in 1. Twenty patients had various combinations of these conditions. Presenting symptoms included bilious vomiting, dehydration, and weight loss. X-rays of the upper abdomen demonstrated the presence of a typical doublebubble sign or air-fl uid levels in 68.64% of patients, and confi rmatory upper and/or lower gastrointestinal contrast studies were obtained in 64.11%. Multiple associated abnormalities were observed in 50.52% of the patients. Various surgical approaches were used, including Ladd's procedure, duodenoplasty, duodenoduodenostomy, duodenojejunostomy, or a combination of these. Seventeen patients died postoperatively and 14 required re-operation.
Conclusions: Congenital duodenal obstruction is a complex entity with various etiologies and often includes multiple concomitant disorders. Timely diagnosis and aggressive surgery are key to improving prognosis. Care should be taken to address all of the causes of duodenal obstruction and/or associated alimentary tract anomalies during surgery.