Aim: To evaluate the anomalies associated with congenital duodenal obstruction and the factors affecting short-term survival.
Material and method: Age, gender, pathology and associated congenital anomalies of the patients with congenital duodenal obstruction, operated between 2006-2017 were retrospectively evaluated.
Results: Forty-seven patients who were diagnosed as congenital duodenal obstruction were included in the study. 32 (68%) of the patients were female and 15 (32%) were male (K / E = 2.1; 1). 18 (38%) of the patients had prematurity. The mean age was 8.6 days (1-73 days). In 31 (66%) patients, extrinsic anomalies (29 annular pancreas, 2 congenital band) and in 15 patients (32%) intrinsic anomalies (9 duodenal web, 6 duodenal atresia) were observed, whereas one patient had annular pancreas and duodenal web together. 11 (23%) patients had Down syndrome. There were congenital heart anomalies in 27 (57%) patients, including those with Down syndrome and those without Down syndrome. 2 (4,2%) patients had anal atresia, 5 (10%) patients had malrotation, 1 (2%) patient had internal cloaca and 1 (2%) patient had right renal agenesis. 8 of the patients passed away. Mortality was defined as sepsis in 4 patients and congenital heart disease in 4 patients. Six of them (75%) were premature.
Conclusion: Down syndrome is the most common accompanying chromosomal anomaly in congenital duodenal obstruction. Congenital heart disease, congenital duodenal obstruction together are the most common anomaly independent of chromosomal anomaly. Prematurity has a significant negative effect on the prognosis.
Keywords: duodenal obstruction, anular pancreas, down syndrome, newborn
Amaç: Konjenital duodenal obstrüksiyon ile ilişkili anomalileri ve kısa süreli sağkalımı etkileyen faktörleri değerlendirmek.
Konjenital duodenal obstrüksiyon tanısıyla opere edilen 47 hasta çalışmaya alındı. Hastaların 32’si (%68) kız, 15’i (%32) erkekti (K/E=2.1;1). 18 (%38) hastanın prematuritesi mevcuttu. Ameliyat yaşı ortalama 8,6 gündü (1-73 gün). Hastalardan 31 ‘inde (%66) ekstrensek anomali (29 ‘unda anüler pankreas, 2’sinde konjenital band), 15’inde (%32) intrensek anomali (9’unda duodenal web, 6’sında duodenal atrezi), 1’inde anüler pankreas ve duodenal web birlikteliği görüldü. 11 (%23) hastada Down sendromu mevcuttu. Down sendromlu olanların hepsinde ve olmayan 16 hastada olmak üzere 27 (%57) hastada konjenital kalp anomalisi mevcuttu. 2 (%4,2) hastada anal atrezi, 5 (%10) hastada malrotasyon, 1 (%2) hastada internal kloaka, 1 (%2) hastada sağ renal agenezi mevcuttu. Hastalardan 8’i kaybedildi. Mortalite nedeni 4 hastada sepsis, 4 hastada konjenital kalp hastalığı olarak belirlendi. Kaybedilen hastalardan 6’sı (%75) prematur doğumdu.
Sonuç: Konjenital duodenal obstrüksiyonlara en sık eşlik kromozom anomalisi Down sendromudur. Konjenital kalp hastalığı, konjenital duodenal obstrüksiyon birlikteliği kromozom anomalisinden bağımsız olarak en sık anomalidir. Prematuritenin prognoz üzerinde önemli etkisi vardır.
Anahtar Kelimeler: duodenal obstruksiyon, anüler pankreas, down sendromu, yenidoğan