Congenital absence of sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava

IF 0.5 Q4 PEDIATRICS Egyptian Pediatric Association Gazette Pub Date : 2023-12-18 DOI:10.1186/s43054-023-00216-4
Santosh Chavan, Nilima Narkhede, Rajaram Weling
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Abstract

Congenital complete absence of sternal bone or cleft sternum or absent sternum is a very rare and potentially life-threatening birth defect. An association of cleft sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava is extremely rare, and not many cases have been reported. A 2-year-old child was brought for routine checkup. He was a known case of congenital heart disease and midline thoracic defect at age of 3 months. Further investigations revealed absent sternum with tetralogy of Fallot, large cono-ventricular defect with bidirectional shunt, severe infundibular pulmonary stenosis, superiorly located right aortic arch, and bilateral superior vena cava. Congenital absence of the sternum with intracardiac defects like tetralogy of Fallot with right aortic arch and bilateral superior vena cava is extremely rare malformation. Early diagnosis and surgical correction give the infant the best chance of survival.
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先天性胸骨缺失伴法洛氏四联症、右主动脉弓和双侧上腔静脉
先天性胸骨完全缺失或胸骨裂或胸骨缺失是一种非常罕见且可能危及生命的出生缺陷。胸骨裂伴有法洛氏四联症、右主动脉弓和双侧上腔静脉的病例极为罕见,报道的病例也不多。一名两岁的儿童被带去做常规检查。他在 3 个月大时就患有先天性心脏病和中线胸廓缺损。进一步检查发现,患儿胸骨缺失并伴有法洛四联症、伴有双向分流的巨大圆锥心室缺损、严重的肺底狭窄、右主动脉弓上位和双侧上腔静脉。先天性胸骨缺失伴心内膜缺损,如法洛四联症伴右主动脉弓和双侧上腔静脉,是极其罕见的畸形。早期诊断和手术矫正可为婴儿带来最佳的存活机会。
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来源期刊
自引率
0.00%
发文量
32
审稿时长
9 weeks
期刊介绍: The Gazette is the official journal of the Egyptian Pediatric Association. The main purpose of the Gazette is to provide a place for the publication of high-quality papers documenting recent advances and new developments in both pediatrics and pediatric surgery in clinical and experimental settings. An equally important purpose of the Gazette is to publish local and regional issues related to children and child care. The Gazette welcomes original papers, review articles, case reports and short communications as well as short technical reports. Papers submitted to the Gazette are peer-reviewed by a large review board. The Gazette also offers CME quizzes, credits for which can be claimed from either the EPA website or the EPA headquarters. Fields of interest: all aspects of pediatrics, pediatric surgery, child health and child care. The Gazette complies with the Uniform Requirements for Manuscripts submitted to biomedical journals as recommended by the International Committee of Medical Journal Editors (ICMJE).
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