Esophageal atresia and associated anomalies

German, John C.; Mahour, G. Hossein; Woolley, Morton M.

doi:10.1016/s0022-3468(76)80182-0

Cited by 102 publications

(36 citation statements)

References 17 publications

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“…The risk of CHD is high among EA/TEF patient groups. Although all CHD's may occur in EA/TEF patients, VSD, PDA, and ASD are the most frequent ones (5,11,12). In the present study, ASD, VSD, and PDA were the most frequent anomalies in the EA/TEF patients.…”

Section: Discussionsupporting

confidence: 47%

Gastrointestinal system malformations in children are associated with congenital heart defects

Örün¹,

Bilici²,

Demirçeken³

et al. 2011

Anadolu Kardiyol Derg

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ÖZETAmaç: Gastrointestinal sistem malformasyonlu (GİSM) çocuklarda doğumsal kalp hastalıklarının sıklığını ve mortalite ile ilişkisini saptamak. Yöntemler: Hastanemizin, Çocuk Cerrahisi Kliniğinde GİSM nedeniyle izlenen ve kardiyolojik inceleme yapılan ardışık 242 çocuk hastanın kayıt-ları retrospektif olarak incelenerek, GİSM olan çocuk hastalarda doğumsal kalp defektlerinin (DKH) insidansı araştırılmıştır. Verilerin istatistiksel değerlendirmesinde Ki-kare testi kullanılmıştır. Bulgular: GİSM saptanan, yaşları 0 gün ile 15 yıl arasında değişen 242 hasta değerlendirildi. Hastaların 135'i (%55.8) erkek, 107'si kız (%44.2) idi. En sık görülen gastrointestinal malformasyonları anorektal malformasyon (%43.2), mide, ileum veya kolon atrezisi (%21) ve özofajiyal atrezi/ trakeoözo-fajiyal fistül (%18.3) idi. Hastaların %28.5'inde doğumsal kalp hastalığı saptanmış olup, en sık gözlenen anomaliler; olguların 31'inde (%44.9) atriyal septal defekt, 17'sinde (%24.6) ventriküler septal defekt, 5'inde (%7.2) patent duktus arteriyozus idi. GİSM nedeni ile ameliyat edilen doğumsal kalp hastalığı olan (%16.7) ve olmayan (%13.3) hastalar arasında mortalite açısından anlamlı fark saptanmadı (p>0.05). Sonuç: Gastrointestinal sistem malformasyonu saptanan hastaların erken dönemde kardiyolojik açıdan değerlendirilmesi gerektiği vurgulanmak istenmiştir. (Anadolu Kardiyol Derg 2011; 2: 146-9) Anahtar kelimeler: Gastrointestinal sistem malformasyonu, doğumsal kalp defekti, ekokardiyografi, çocuk, hastalık insidansı ABSTRACT Objective: To determine the frequency of congenital heart defects (CHD) in children with gastrointestinal malformations (GISM) and mortality rates in patients with GISM. Methods: Two hundred and forty two consecutive children patients with GISM followed up in Pediatric Surgery Clinics of our hospital were examined for cardiovascular anomaly by the Department of Pediatric Cardiology, and the CHD incidence was investigated by examining the records of the patients retrospectively. Chi-square test was used for the statistical analysis of data. Results: Two hundred and forty two patients with gastrointestinal system malformations were included in the study. Of 242 patients, 135 (55.8%) were male and 107 (44.2%) were female, and their age range was 0-15 years. The most frequent GISM were anorectal malformations (43.2%), atresia involving stomach, ileum or colon (21%) and esophageal atresia/tracheoesophageal fistula (18.3%). Congenital heart defects were observed in 28.5% of the participants. The most frequent defects were as follows; atrial septal defect (31 patients, 44.9%) a, ventricular septal defect (17 patients, 24.6%) and patent ductus arteriosus (5 patients, 7.2%). There was no significant difference (p>0.05) in mortality rate in patients with CHD (16.7%) and without CHD (13.3%) undergoing operations for GISM. Conclusion: We would like to emphasize the importance of the earliest possible cardiological evaluation of all patients with gastrointestinal system malformations. (Anadolu Kardiyol Derg 2011; 2: 146-9)

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Section: Discussionsupporting

confidence: 47%

Gastrointestinal system malformations in children are associated with congenital heart defects

Örün¹,

Bilici²,

Demirçeken³

et al. 2011

Anadolu Kardiyol Derg

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“…In con trast, associated chromosomal abnormalities were reported in only 3-4% of live births with oesophageal atresia [29,30], One possible ex planation for this apparent discrepancy, and for the finding that in more than 90% of infants with oesophageal atresia there is an associated in tracheo-oesophageai fistula [31], is that trisomic fetuses are more likely to die in utero, due to other malformations and intra-uterine growth retardation, but also to be born at previable gestations due to the polyhydramnios, because their oesophageal atresia develops in the absence of tracheo-oesophageai fistula.…”

Section: Discussionmentioning

confidence: 99%

Fetal Gastro-Intestinal and Abdominal Wall Defects: Associated Malformations and Chromosomal Abnormalities

et al. 1992

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During an 8-year period (1983–1991), blood karyotyping was performed in 235 fetuses with abdominal wall or gastrointestinal tract defects. The overall incidence of chromosomal abnormalities was 29% (trisomy 21, n = 12; trisomy 18, n = 44; trisomy 13, n = 7; deletion of the short arm of chromosome 5, n = 1; unbalanced translocation involving chromosomes 4 and 15, n = 1; triploidy, n = 1; Klinefelter’s syndrome, n = 1; and Beck-with-Wiedemann syndrome with mosaic duplication 1 lpl5, n = 1). The karyotype was abnormal in 42 (36%) of the 116 fetuses with exomphalos, in none of the 26 with gastroschisis, in 10 (43 %) of the 23 with duodenal atresia, in 18 (75 %) of the 24 with lack of visible stomach, in 1 (4%) of the 24 with dilated bowel and in 2 (7 %) of the 27 with echogenic hepatic nodules or abdominal cysts. Abnormal karyotypes were more commonly encountered when there was ultrasonographic evidence of multiple malformations (43%) compared to isolated defects (2%). Survival in fetuses with exomphalos (33%), absent stomach (4%), and large bowel obstruction (13%) was poor, whereas in those with gastroschisis (73%) or abdominal cysts (88%) survival was high; in small bowel obstruction and in duodenal atresia, survival was 65 and 57%, respectively.

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“…Cardiovascular anomalies occur most frequently (11-49%) followed by genitourinary 24%, gastrointestinal 24% and skeletal 13%. [1][2][3] Neural tube defects 2.3%, hydrocephalus 5.2%, abdominal wall defects 4.3% and Diaphragmatic hernia 2.9% have been reported Harris et al nursing, neonatal ventilators, newer antibiotics, advancement in neonatal anaesthesia, the survival in EA with TEF is approaching about 100% especially in Western countries. But in our country mortality is still very high, due to many factors.…”

Section: Introductionmentioning

confidence: 99%

Esophageal atresia and Tracheoesophageal fistula with associated anomalies in a tertiary care hospital of north India

Prasad¹

2017

Int Surg J

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Background: Esophageal atresia (EA) with or without Tracheoesophageal fistula (TEF) has been described as the epitome of pediatric surgery and the management of these neonates require high degree of skill, manpower and tertiary care. This clinical study was undertaken to identify the EA and TEF cases along with the pattern of associated anomalies causing mortality in Varanasi .Methods: This prospective study included 53 patients of EA with TEF who underwent primary esophageal anastomosis. Their clinical profile, time of presentation, various associated anomalies, sex distribution, and their effect on mortality and morbidity were observed.Results: Mortality was high (41.64%) among patients who reached the tertiary centre late i.e. >24 hours. The incidence of low birth weight babies is approximately 75% and mortality rate increased with low birth weight. Almost 2/3 of patients had clinical or radiological evidence of pneumonia. The mortality was very high in severe pneumonia 66.7% as compared to 15% patients without pneumonia. Septicaemia and leak were most common complications. The mortality in septicemic patients is high (77.8%). The overall incidence of associated anomalies was 47%. Early surgical intervention improved the survival of EA with TEF with major GIT associated anomalies. The overall survival rate was 60.37% and mortality was 39.67%.Conclusions: Although we have improved a lot in managing these cases of EA with TEF, but we have to do much more to achieve our goal of near 100% survival.

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Esophageal atresia and associated anomalies

Cited by 102 publications

References 17 publications

Gastrointestinal system malformations in children are associated with congenital heart defects

Gastrointestinal system malformations in children are associated with congenital heart defects

Fetal Gastro-Intestinal and Abdominal Wall Defects: Associated Malformations and Chromosomal Abnormalities

Esophageal atresia and Tracheoesophageal fistula with associated anomalies in a tertiary care hospital of north India

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