1 - Department of Pediatric Surgery, Hospital Dona Estefânia (HDE), Centro Hospitalar Universitário Lisboa Central (CHULC), Lisboa, Portugal
2 - Department of Pediatrics/ Neonatology, HDE, CHULC, Lisboa, Portugal
3 - Department of Pediatric Cardiology, Hospital Santa Marta (HSM), CHULC, Lisboa, Portugal.
- Artigo publicado no Journal of Neonatal Surgery em Julho 2022, vol 11: 22
- DOI: https://doi.org/10.47338/jns.v11.1091
- Caso apresentado no 50º Congresso Português de Neonatologia em Novembro 2022
Introdução: Heteropagus, or “parasitic twins,” is an anomaly of asymmetrical conjoined twins in which an incompletely formed twin (parasite) is attached to and dependent upon a fully developed twin (autosite). The authors report a case of omphalopagus twins with omphalocele and congenital heart disease.
Caso clínico: A 3250g, female second twin, known antenatally to have an omphalocele and interventricular communication, was delivered by elective cesarean section (pelvic 1st twin) at 36 weeks’ gestation. It was a monochorionic diamniotic pregnancy through a donor in vitro fertilization. Examination of the second, conjoined twin found an acardiac acephalic parasitic twin with dysmorphic legs and arms, pelvis, buttocks, genitalia, and a soft tissue pedicle emanating from the xiphoid region of the primary twin. The parasite’s urethra showed some urine flow. Postnatal imaging revealed perimembranous ventricular septal defect, muscular ventricular septal defect, patent foramen ovale, also that both intestine and liver were present in the omphalocele and that the blood supply to the parasitic twin was originated from an aberrant branch of the left iliac artery of the autosite twin. The twins were sucessfully separated on 3rd postnatal day with primary closure of the omphalocele. Postoperative period was uneventfull.
Discussão: The authors describe a case of female heteropagus twins, in which the junction is above the umbilicus, and the connection between them was a tubular structure formed by a rudimentary articulation, umbilical vessel and cord (omphalopagus twins). Omphalocele is the most associated anomaly in the autosite, followed by cardiac anomalies as in this case. Most parasitic twins are diagnosed with prenatal ultrasound and since their prognosis is favorable, there is no need to end the pregnancy. As there is great anatomical variation among cases, preoperative planning and subsequent operative approach must be tailored to the unique presentation of the patients. Different surgical techniques are available, depending on the sharing of organs. In this case, surgery was simple and uncomplicated, as there were no bony and/or visceral communications between twins. Repairing omphalocele and closing the defect created by the removal of the parasite was also straightforward. The patient got complicated later with mild congestive heart failure, a main prognostic factor.
Palavras-chave: parasitic twins; omphalocele; cardiac disease; surgery; neonatology