The natural history of prenatally diagnosed conjoined twins

• Published in: Journal of pediatric surgery 2002-03, Vol.37 (3), p.303-309
• Main Authors: MacKenzie, Tippi C., Crombleholme, Timothy M., Johnson, Mark P., Schnaufer, Louise, Flake, Alan W., Hedrick, Holly L., Howell, Lori J., Adzick, N.Scott
• Format: Article
• Language: English
• Subjects: Female; Fetal Diseases - diagnostic imaging; Fetal Diseases - surgery; Humans; Pregnancy; Prenatal Diagnosis - methods; Retrospective Studies; Twins, Conjoined - surgery; Ultrasonography, Prenatal - methods
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1053/jpsu.2002.30830

Background/Purpose: Accurate prenatal diagnosis of complex anatomic connections and associated anomalies has only been possible recently with the use of ultrasonography, echocardiography, and fetal magnetic resonance imaging (MRI). To assess the impact of improved antenatal diagnosis in the management and outcome of conjoined twins, the authors reviewed their experience with 14 cases. Methods: A retrospective review of prenatally diagnosed conjoined twins referred to our institution from 1996 to present was conducted. Results: In 14 sets of conjoined twins, there were 10 thoracoomphalopagus, 2 dicephalus tribrachius dipus, 1 ischiopagus, and 1 ischioomphalopagus. The earliest age at diagnosis was 9 weeks' gestation (range, 9 to 29; mean, 20). Prenatal imaging with ultrasonography, echocardiography, and ultrafast fetal MRI accurately defined the shared anatomy in all cases. Associated anomalies included cardiac malformations (11 of 14), congenital diaphragmatic hernia (4 of 14), abdominal wall defects (2 of 14), and imperforate anus (2 of 14). Three sets of twins underwent therapeutic abortion, 1 set of twins died in utero, and 10 were delivered via cesarean section at a mean gestational age of 34 weeks. There were 5 individual survivors in the series after separation (18%). In one case, in which a twin with a normal heart perfused the cotwin with a rudimentary heart, the ex utero intrapartum treatment procedure (EXIT) was utilized because of concern that the normal twin would suffer immediate cardiac decompensation at birth. This EXIT-to-separation strategy allowed prompt control of the airway and circulation before clamping the umbilical cord and optimized control over a potentially emergent situation, leading to survival of the normal cotwin. In 2 sets of twins in which each twin had a normal heart, tissue expanders were inserted before separation. Conclusions: Advances in prenatal diagnosis allow detailed, accurate evaluations of conjoined twins. Careful prenatal studies may uncover cases in which emergent separation at birth is lifesaving.