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. 2018 Oct 29;8(4):e264–e276. doi: 10.1055/s-0038-1675353

Table 1. Reported fetal findings by diagnosis.

OEIS complex/cloacal exstrophy
Diagnostic criteria Four key criteria in the acronym
 Omphalocele
 Exstrophy of the bladder
 Imperforate anus
 Spina bifida
Proposed common findings Kidney malformations (up to 60% of cases 49 )
 Hydroureter 49 50
 Hydronephrosis 49 50 51
 Congenital megaureter 16 52
 Pelvic kidney 16 53 54
 Duplex kidney 16 54
 Renal dysplasia 51 55
 Cystic dysplasia 16 49 50
 Renal agenesis 49 50 52 55 56 57
Limb anomalies
 Clubfoot with vertical talus 50
 Limb length discrepancy 53
 Bilateral clubfeet 16 53 56
Neural tube defects 57 58 (70% of cases 49 )
 Spina bifida, myelocystocele 49
 Terminal myelocystocele 16 53 54
 Lumbosacral spina bifida 51 56
 Hindbrain herniation, lipomyelomeningocele 16
Omphalocele 16 52 54 55 56
Spine abnormalities
 Kyphoscoliosis 49 50
 Sacral hemivertebra 55
 Pubic diastasis 51
Missing bladder 16 51 52 53 54 56
Single umbilical artery 16 52 54 55
Abnormality of external genitalia 16 54
 Hypospadias 55
 Bifid corporal bodies 53
 Duplicated vagina, bifid scrotum, micropenis 56
 Ambiguous genitalia 51
Case reports Intrinsic cardiac abnormalities
 Moderate PDA, small PFO, mild tricuspid regurgitation 54
 ASD, atrioventricular valve, unroofed coronary sinus, persistent L SVC, LVH 55
 Single right ventricle 56
Pentalogy of Cantrell
Diagnostic criteria Five distinct criteria, partial cases may have fewer than five
 Intracardiac abnormalities
 Anterior pericardial defects
 Lower sternal defects
 Anterior diaphragmatic defects
 Supraumbilical abdominal wall defects 12
Proposed common findings Ectopia cordis 4 8 22 24 26 59
Intrinsic cardiac malformations
 ASD 4 26 50
 VSD 4 8 24 50
 Tetralogy of Fallot 4 22 24 26 50
 Left ventricular diverticulum 26 50
 Transposition of the great vessels 26
 Tricuspid atresia, dextrocardia, anomalous cardiac venous return 8
Neural tube defects 26 58
Midline supraumbilical abdominal wall defect
 Omphalocele 4 8 22 26 50 59
 Diastasis recti abdominis, umbilical hernia, epigastric hernia 50
 Ventral hernia, open defect 26
 Supraumbilical hernia 24
Sternal defects
 Bifid sternum 4 26
 Cleft sternum 4 22 24
 Absence of xiphoid 8
 Split sternum 26
Diaphragmatic defects
 Anterior diaphragmatic hernia 4 24 26 59
Pericardial defects
 Pericardial hernia 4 50
 Absent pericardium 26 59
Umbilical cord defects
 Single umbilical artery 8 22 60
 Short cord, hypercoiled cord associated with POC + ectopia cordis 8
Facial defects
 Cleft lip and/or palate 26
Case reports Aplastic left limb 61
Spinal defect 62
Hypoplastic lung 26
Bronchopulmonary dysplasia, hypoplastic kidney, cleft lip and palate, pulmonary atresia 4
Gastroschisis, twin reversed arterial perfusion sequence 8
Nonrotation of the midgut, accessory spleen 22
Limb–body wall complex
Diagnostic criteria Two of the following
 Exencephaly or encephalocele with facial clefts
 Thoraco- and/or abdominoschisis
 Limb defects 7 63
Two distinct phenotypes
 Craniofacial defects often with cranioplacental adhesion and or amniotic bands
 No craniofacial defects but abdominal–placental attachment with short/absent umbilical cord and urogenital anomalies 35
Body stalk anomaly
 Large abdominal wall defect with herniation of abdominal contents into the extraembryonic coelom
 Absent or rudimentary umbilical cord 34
Proposed common findings Absent/rudimentary/short umbilical cord 32 34 47 58
Limb anomalies
 Pseudosyndactyly without amniotic bands, oligodactyly, polydactyly, split hand and foot, single bone forelimb, forebone abnormalities, absent limb and limb girdle, absent muscles and arthrogryposis 7 63
 Clubbed feet, single lower limb 34
 Bilateral clubbed feet 32
 Club foot, rocker bottom foot 47
Neural tube defects 46 58
 Exencephaly or encephalocele 7 63
Abdominal wall defects
 Abdominoschisis 34 64
 Omphalocele 32 39 47
Skeletal abnormalities
 Kyphoscoliosis 32 34
 Scoliosis 46 47 64
Craniofacial abnormalities 28 34
Case reports Encephalocele, anophthalmia, bilateral cleft lip, thoracic cleft with ectopia cordis, omphalocele, short umbilical cord, single umbilical artery 39
Ectopia cordis 11
Anencephaly 32
Abdominoschisis, diaphragmatic defect 39
Agenesis of the anal canal, agenesis of the genitourinary tract, hypoplastic lungs 64
Absent diaphragm, bowel atresia, renal agenesis, anal atresia, no external genitalia 44
Anal atresia, no external genitalia, no urinary bladder, hypoplastic lungs 47

Abbreviations: ASD, atrial septal defect; OEIS, omphalocele, exstrophy, imperforate anus, spina bifida; PDA, patent ductus arteriosus; PFO, patent foramen ovale; SVC, superior vena cava.