Twin pregnancy complicated by esophageal atresia, duodenal atresia, gastric perforation, and hypoplastic left heart structures in one twin: a case report and review of the literature.
BACKGROUND
The antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy. Therefore we believe this is the first case report of an antenatal diagnosis of combined pure esophageal and duodenal atresia in a twin gestation.


METHODS
We present a case of a 30-year-old G1P0 white woman at 22-week gestation with a monochorionic-diamniotic twin pregnancy discordant for esophageal atresia, duodenal atresia with gastric perforation, hypoplastic left heart structures, and significant early gestation maternal polyhydramnios. In this case, fetal magnetic resonance imaging was able to depict additional findings including area of gastric wall rupture, hiatal hernia, dilation of the distal esophagus, and area of duodenal obstruction and thus facilitated the proper diagnosis. After extensive counseling at our multidisciplinary team meeting, the parents elected to proceed with radiofrequency ablation of the anomalous twin to maximize the survival of the normal co-twin. The procedure was performed successfully with complete cessation of flow in the umbilical artery and complete cardiac standstill in the anomalous twin with no detrimental effects on the healthy co-twin.


CONCLUSIONS
Prenatal diagnosis of complex anomalies in twin pregnancies constitutes a multitude of ethical, religious, and cultural factors that come into play in the management of these cases. Fetal magnetic resonance imaging provides detailed valuable information that can assist in management options including possible prenatal intervention. The combination of a cystic structure with peristalsis-like movement above the diaphragm (for example, "the upper thoracic pouch sign"), polyhydramnios, and progressive distention of the stomach and duodenum should increase suspicion for a combined pure esophageal and duodenal atresia.
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA.
Mohamad K
MK
Abou Chaar
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA.|Department of Pediatric Radiology, University of Colorado Denver, Anschutz Medical Center, Denver, USA.
Mariana L
ML
Meyers
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA.|Department of Pediatrics, University of Colorado Denver, Anschutz Medical Center, Denver, USA.
Bethany D
BD
Tucker
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA.|Department of Obstetrics and Gynecology - Maternal Fetal Medicine, University of Colorado Denver, Anschutz Medical Center, Denver, USA.
Henry L
HL
Galan
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA.|Department of Pediatric Surgery, Children's Hospital Colorado, University of Colorado Denver, Anschutz Medical Center, Denver, USA.
Kenneth W
KW
Liechty
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA.|Department of Pediatric Surgery, Children's Hospital Colorado, University of Colorado Denver, Anschutz Medical Center, Denver, USA.
Timothy M
TM
Crombleholme
Colorado Fetal Care Center, Colorado Institute of Maternal and Fetal Health, University of Colorado Denver, Anschutz Medical Center, Denver, USA. rony.marwan@childrenscolorado.org.|Department of Pediatric Surgery, Children's Hospital Colorado, University of Colorado Denver, Anschutz Medical Center, Denver, USA. rony.marwan@childrenscolorado.org.|Surgery and Pediatrics, Colorado Fetal Care Center - Children's Hospital Colorado, 13123 East 16th Avenue, 328, Aurora, Colorado, 80045, USA. rony.marwan@childrenscolorado.org.
Ahmed I
AI
Marwan
D000328:Adult; D004380:Duodenal Obstruction / Q000000981:diagnostic imaging / Q000196:embryology*; D004933:Esophageal Atresia / Q000000981:diagnostic imaging / Q000196:embryology*; D005260:Female; D005817:Genetic Counseling; D006801:Humans; D007231:Infant, Newborn; D007409:Intestinal Atresia; D006831:Polyhydramnios; D011247:Pregnancy; D011248:Pregnancy Complications / Q000000981:diagnostic imaging / Q000523:psychology / Q000628:therapy*; D011256:Pregnancy Outcome; D018607:Pregnancy Reduction, Multifetal / Q000523:psychology; D059285:Pregnancy, Twin / Q000523:psychology; D011296:Prenatal Diagnosis; D014427:Twins
Case report; Duodenal atresia; Esophageal atresia without tracheoesophageal fistula; Fetal MRI; Gastric perforation; Monochorionic diamniotic twins