Epidemiological and clinical analysis of a consecutive series of conjoined twins in Spain

• Published in: Journal of pediatric surgery 2009-04, Vol.44 (4), p.811-820
• Main Authors: Martínez-Frías, María Luisa, Bermejo, Eva, Mendioroz, Jacobo, Rodríguez-Pinilla, Elvira, Blanco, Manuel, Egüés, Javier, Félix, Valentín, García, Angel, Huertas, Héctor, Nieto, Carmen, López, José Antonio, López, Santiago, Paisán, Luis, Rosa, Alejandro, Vázquez, María Socorro
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - diagnosis; Abnormalities, Multiple - epidemiology; Abnormalities, Multiple - surgery; Abortion, Therapeutic; Cause of Death; Conjoined twin; Diprosopus pair; Epidemiology; Female; Humans; Incidence; Infant, Newborn; Male; Pediatrics; Prenatal Diagnosis; Registries; Retrospective Studies; Spain - epidemiology; Stillbirth - epidemiology; Surgery; Survival Analysis; Thoracopagus; Twins, Conjoined - pathology; Twins, Conjoined - physiopathology
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2008.07.002

Abstract Purpose The aim of the study was to analyze the frequency and certain epidemiological characteristics of a consecutive series of conjoined twins born in Spain. Material and Methods We used data from the Spanish Collaborative Study of Congenital Malformations for the period April 1976 to 2006. Because the Spanish law permitting voluntary termination of pregnancies (TOP) when the fetus presented malformations was effective by the end of 1985, we analyzed the data in 4 periods, 2 before 1986 and 2 after. During the first period (1976-1979) only live births were recorded, whereas both still and live births were included in the other three (1980-1985, 1986-1995, and 1996-2006). In the present study, the cases were classified as symmetrical (16 pairs) and asymmetrical (1 pair) conjoined twins. Each pair of conjoined twins was considered as only one case for calculations, regardless of the type of union. Results Among a total of 2,281,604 consecutive births between 1980 and 2006, there were a total of 15 cases of symmetrical conjoined twins giving a frequency of 0.70 per 100,000 (1/152,107), whereas there was only 1 stillborn asymmetrical conjoined twin pair (0.04/100,000). Among the 13,418 consecutive stillborns surveyed, 6 cases of conjoined twins were identified (either symmetrical or asymmetrical) giving a frequency of 44.72 per 100,000, and 11 pairs were identified among the 2,425,583 total live births surveyed during the first period 1976 to 1979, a frequency of 0.45 per 100,000. Thus, the frequency among stillborn infants is 99.34 times higher than that observed among live births. However, the frequency for the total births (3 last periods) showed a decreasing trend from 1.47 per 100,000 birth in the first period (1980-1985) when TOP was illegal, to a value of 0.09 per 100,000 in the last period, more than 16-fold lower, probably because of the TOP of affected fetuses. Therefore, we consider that the frequencies observed in the period 1980 to 1985 are the basal values in our population. The most frequent type observed was thoracopagus, with an overall prevalence at birth of 0.44 per 100,000 (1/228,160) from 1980 to 2006, representing 58.82% of the total population of symmetric conjoined twin pairs. Diprosopus pairs were the next most common group (11.76%). Most of the cases were females (4 males/11 females), and although this appeared to be mainly because of the thoracopagus pairs (males-females, 2:8), in such a small number of cases, it is not po