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A Unique Case with Tracheal Atresia Among Published Literature on TACRD and VACTERL Associations [J Behcet Uz Child Hosp]
J Behcet Uz Child Hosp. 2023; 13(2): 142-145 | DOI: 10.4274/buchd.galenos.2023.76736  

A Unique Case with Tracheal Atresia Among Published Literature on TACRD and VACTERL Associations

Sabri Cansaran, Cengiz Gül, Shukri Said Mohamed, Ayşenur Celayir
University of Health Sciences Turkey, Zeynep Kamil Maternity and Children's Diseases Health Training and Research Center, Department of Pediatric Surgery, İstanbul, Turkey

Tracheal atresia/agenesis (TA) is associated with other congenital anomalies (TACRD and/or VACTERL). In this study, a female newborn with TA who had all the elements associated with TACRD and VACTERL was reported. The patient with TA, tracheo-esophageal fistula (TEF) connecting to the esophagus at the level of the carina, persistent left superior vena cava, duodenal atresia, vertebral and limb defects, bilateral hydronephrosis, and persistent cloaca was operated on postnatal 5th day. Band ligation to the abdominal esophagus, gastrostomy, duodenal atresia repair and diverting colostomy due to persistent cloaca were performed. The lungs were bilaterally expanded in the first postnatal, preoperative and postoperative chest radiographs. Oxygen saturation remained above 90% until the patient died suddenly on the postnatal 8th day. TA is a very rare congenital anomaly that causes postnatal respiratory distress. This case is unique among the literature on TACRD and VACTERL associations for many reasons. Esophageal band ligation in TA cases with TEF is a method that keeps the pressure in the esophagus at an appropriate level and provides air passage to the trachea via fistula.

Keywords: Duodenal atresia, newborn, TACRD, tracheal atresia/agenesis, VACTERL


TACRD ve VACTERL Birliktelikleri Hakkında Yayınlanmış Literatürler Arasında Trakeal Atrezili Özgün Bir Olgu

Sabri Cansaran, Cengiz Gül, Shukri Said Mohamed, Ayşenur Celayir
Sağlık Bilimleri Üniversitesi, Zeynep Kamil Kadın ve Çocuk Hastalıkları Sağlık Uygulama ve Araşırma Merkezi, İstanbul

Trakeal atrezi/agenezi (TA) diğer konjenital anomalilerle (TACRD ve/veya VACTERL) ilişkilidir. Bu çalışmada, TACRD ve VACTERL ile ilişkili tüm özelliklere sahip TA’lı bir kız yenidoğan raporlandı. TA, özofagusa karina seviyesinde bağlanan trakeo-özofageal fistül (TEF), persistan sol superior vena kava, duodenal atrezi, vertebra ve ekstremite defektleri, bilateral hidronefroz ve persistan kloaka tanılı hasta postnatal 5. günde opere edildi. Abdominal özofagusa bant ligasyonu, gastrostomi, duodenal atrezi onarımı ve persistan kloaka nedeniyle diverjan kolostomi uygulandı. Postnatal ilk, preoperatif ve postoperatif çekilen grafilerde akciğerler bilateral ekspanseydi. Hastanın oksijen satürasyonu, postnatal 8. gündeki ani ölümüne kadar, %90’ın üzerinde kaldı. TA, postnatal solunum sıkıntısına neden olan, çok nadir görülen bir konjenital anomalidir. Bu olgu, birçok nedenden dolayı, TACRD ve VACTERL birliktelikleri ile ilgili literatür arasında benzersizdir. TEF’li TA olgularında özofagus bant ligasyonu özofagustaki basıncı uygun seviyede tutan ve fistül yoluyla trakeaya hava geçişini sağlayan bir yöntemdir.

Anahtar Kelimeler: Duodenal atrezi, yenidoğan, TACRD, trakeal atrezi/agenezi, VACTERL


Sabri Cansaran, Cengiz Gül, Shukri Said Mohamed, Ayşenur Celayir. A Unique Case with Tracheal Atresia Among Published Literature on TACRD and VACTERL Associations. J Behcet Uz Child Hosp. 2023; 13(2): 142-145

Corresponding Author: Sabri Cansaran, Türkiye


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