Background: Twin reversed arterial perfusion sequence is a rare and potentially lethal condition affecting approximately 1% of monochorionic twin pregnancies and 1 in 35,000 pregnancies overall. An apparently normal (pump) twin perfuses its severely malformed cotwin with deoxygenated blood via retrograde flow in direct arterioarterial anastomoses between the umbilical arteries of each twin. Fetal intestinal volvulus is a rare condition usually manifesting after birth. We report a unique case of twin reversed arterial perfusion sequence in association with intestinal volvulus in the surviving pump twin.
Case presentation: A 32-year-old Hispanic primigravida was referred to our clinic after a fetoscopy procedure of laser photocoagulation of anastomoses at 18 weeks of gestation. Follow up scans in the ex-pump twin revealed dilated bowel loops and a typical "whirlpool sign" at 26 weeks of gestation, and intrauterine intestinal volvulus was suspected. At 29 weeks of gestation, preterm premature rupture of membranes occurred, and an emergency cesarean section was performed. The newborn was diagnosed in the early neonatal period with intestinal perforation. The diagnosis was postnatally confirmed by surgery and histopathology.
Conclusions: The type of fetal intervention and late gestational age of the procedure increase the risk of complications. This case alerts health providers to be vigilant in the follow-up of patients with complicated monochorionic pregnancies.
Keywords: Fetal therapies; Intestinal volvulus; Prenatal diagnosis; TRAP sequence; Twin pregnancy.