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Twin reversed arterial perfusion sequence: a rare entity.

Khanduri S, Chhabra S, Raja A, Bhagat S - J Clin Imaging Sci (2015)

Bottom Line: She delivered successfully at term a normal live baby and an acardius acephalus fetus.Plain X-ray of the acardius acephalus fetus confirmed the absence of cephalic structures.The perinatal mortality of the pump twin ranges from 35 to 55%; hence, it is essential to diagnose the presence of a pump twin at an early gestational age through improved imaging techniques, so that intervention can be planned early in the pregnancy for a better outcome of the pump twin.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh, India.

ABSTRACT
Twin reversed arterial perfusion (TRAP) sequence is an extremely rare complication of monochorionic multi-fetal pregnancy, occurring once in 35,000 births. This condition is characterized by a malformed fetus without a cardiac pump being perfused by a structurally normal (pump) twin via an artery-to-artery anastomosis in a reverse direction. We report a case of a primigravida, who came for routine antenatal checkup to our hospital at 31 weeks gestational age. Ultrasound imaging and magnetic resonance imaging revealed twin monochorionic intrauterine pregnancy with a viable, normal-appearing first twin and amorphous structured second twin connected by umbilical vessels. The patient was monitored with weekly ultrasonography, echocardiography, and Doppler ultrasound examination to ascertain the well-being of the pump twin. She delivered successfully at term a normal live baby and an acardius acephalus fetus. Plain X-ray of the acardius acephalus fetus confirmed the absence of cephalic structures. The perinatal mortality of the pump twin ranges from 35 to 55%; hence, it is essential to diagnose the presence of a pump twin at an early gestational age through improved imaging techniques, so that intervention can be planned early in the pregnancy for a better outcome of the pump twin.

No MeSH data available.


Related in: MedlinePlus

22-year-old pregnant asymptomatic female who came for routine antenatal checkup, diagnosed with twin reversed arterial perfusion (TRAP) sequence pregnancy. (a and b) Magnetic Resonance Imaging T2-weighted coronal images of abdomen for fetal assessment show normal fetus (arrowheads) in cephalic presentation, with abnormal fetus demonstrating lower limbs with feet (arrows); absent head, upper limbs, thorax; and a single placenta (curved arrow) in the left lateral region of the upper uterine segment.
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Figure 4: 22-year-old pregnant asymptomatic female who came for routine antenatal checkup, diagnosed with twin reversed arterial perfusion (TRAP) sequence pregnancy. (a and b) Magnetic Resonance Imaging T2-weighted coronal images of abdomen for fetal assessment show normal fetus (arrowheads) in cephalic presentation, with abnormal fetus demonstrating lower limbs with feet (arrows); absent head, upper limbs, thorax; and a single placenta (curved arrow) in the left lateral region of the upper uterine segment.

Mentions: A 22-year-old, 7-months pregnant primigravida visited the Department of Obstetrics and Gynecology of our hospital for routine antenatal checkup. Physical examination revealed distended abdomen with fundal height corresponding to 30–32 weeks of gestation. Multiple fetal parts were felt and a single fetal heart sound was auscultated below the umbilicus toward the left of midline. On per vaginal examination, the os was found to be closed with uneffaced cervix. Three months earlier, her first ultrasound scan taken at a peripheral hospital was misdiagnosed as a single live intrauterine fetus of 18 weeks gestational age. Later she was referred to the Department of Radiology, and the ultrasound images showed twin monochorionic, diamniotic intra-uterine pregnancy with a viable, normal-appearing first twin and amorphous structured second twin [Figure 1]. The placenta was located in the left lateral region of the upper uterine segment [Figure 1]. On further examination, the first twin showed normal morphology and growth with gestational age of 31 weeks in cephalic presentation. Fetal Doppler and echocardiography revealed normal parameters without any signs of cardiac failure in the first twin. The second twin showed disrupted growth and incomplete formation with absent head, upper limbs, heart, and thoracic structures. It had well-developed lower limbs and poorly developed lower abdomen with an abrupt ending at the top to form a blunt end. There was gross, diffuse soft tissue edema in the second twin [Figure 2]. Umbilical cord was seen entering the second twin with intact blood flow and pulsed Doppler study revealed reversal of the flow on the spectral pattern [Figure 3]. Ultrasound findings were consistent with TRAP sequence twin with an acardius acephalus which was confirmed on Magnetic Resonance Imaging. Magnetic resonance imaging showed twin monochorionic, diamniotic intrauterine pregnancy with the normally developed first twin in cephalic presentation on the left and malformed, small, grossly edematous second twin on the right with absent head, heart, upper limbs, and thoracic structures. The second twin had well-developed lower limbs and a part of lower abdomen with an abrupt ending at the top forming a blunt end. Also, a single placenta located in the left lateral region of the upper uterine segment was confirmed on Magnetic resonance imaging [Figure 4]. The patient was monitored with weekly ultrasonography, echocardiography, and Doppler examination for assessing the well-being of the pump twin. At no time during the follow-up did the fetal assessment demonstrate any evidence of complications like cardiac failure, hydrops, and polyhydramnios in the pump twin. She underwent cesarean section at 36 weeks of gestational age and delivered a normal live baby and another acardius acephalus twin. The acardius acephalus fetus was grossly edematous and showed tuft of coarse and lusterless hair in the midline at supraumbilical region [Figure 5]. Plain X-ray of the acardius acephalus fetus revealed normal lower limb bones, pelvic bone, lower vertebrae with the last four paired ribs, and an abrupt ending of bones above it confirming the absence of cephalic structures (i.e. head, heart, upper limbs, and thorax) [Figure 6].


Twin reversed arterial perfusion sequence: a rare entity.

Khanduri S, Chhabra S, Raja A, Bhagat S - J Clin Imaging Sci (2015)

22-year-old pregnant asymptomatic female who came for routine antenatal checkup, diagnosed with twin reversed arterial perfusion (TRAP) sequence pregnancy. (a and b) Magnetic Resonance Imaging T2-weighted coronal images of abdomen for fetal assessment show normal fetus (arrowheads) in cephalic presentation, with abnormal fetus demonstrating lower limbs with feet (arrows); absent head, upper limbs, thorax; and a single placenta (curved arrow) in the left lateral region of the upper uterine segment.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4374195&req=5

Figure 4: 22-year-old pregnant asymptomatic female who came for routine antenatal checkup, diagnosed with twin reversed arterial perfusion (TRAP) sequence pregnancy. (a and b) Magnetic Resonance Imaging T2-weighted coronal images of abdomen for fetal assessment show normal fetus (arrowheads) in cephalic presentation, with abnormal fetus demonstrating lower limbs with feet (arrows); absent head, upper limbs, thorax; and a single placenta (curved arrow) in the left lateral region of the upper uterine segment.
Mentions: A 22-year-old, 7-months pregnant primigravida visited the Department of Obstetrics and Gynecology of our hospital for routine antenatal checkup. Physical examination revealed distended abdomen with fundal height corresponding to 30–32 weeks of gestation. Multiple fetal parts were felt and a single fetal heart sound was auscultated below the umbilicus toward the left of midline. On per vaginal examination, the os was found to be closed with uneffaced cervix. Three months earlier, her first ultrasound scan taken at a peripheral hospital was misdiagnosed as a single live intrauterine fetus of 18 weeks gestational age. Later she was referred to the Department of Radiology, and the ultrasound images showed twin monochorionic, diamniotic intra-uterine pregnancy with a viable, normal-appearing first twin and amorphous structured second twin [Figure 1]. The placenta was located in the left lateral region of the upper uterine segment [Figure 1]. On further examination, the first twin showed normal morphology and growth with gestational age of 31 weeks in cephalic presentation. Fetal Doppler and echocardiography revealed normal parameters without any signs of cardiac failure in the first twin. The second twin showed disrupted growth and incomplete formation with absent head, upper limbs, heart, and thoracic structures. It had well-developed lower limbs and poorly developed lower abdomen with an abrupt ending at the top to form a blunt end. There was gross, diffuse soft tissue edema in the second twin [Figure 2]. Umbilical cord was seen entering the second twin with intact blood flow and pulsed Doppler study revealed reversal of the flow on the spectral pattern [Figure 3]. Ultrasound findings were consistent with TRAP sequence twin with an acardius acephalus which was confirmed on Magnetic Resonance Imaging. Magnetic resonance imaging showed twin monochorionic, diamniotic intrauterine pregnancy with the normally developed first twin in cephalic presentation on the left and malformed, small, grossly edematous second twin on the right with absent head, heart, upper limbs, and thoracic structures. The second twin had well-developed lower limbs and a part of lower abdomen with an abrupt ending at the top forming a blunt end. Also, a single placenta located in the left lateral region of the upper uterine segment was confirmed on Magnetic resonance imaging [Figure 4]. The patient was monitored with weekly ultrasonography, echocardiography, and Doppler examination for assessing the well-being of the pump twin. At no time during the follow-up did the fetal assessment demonstrate any evidence of complications like cardiac failure, hydrops, and polyhydramnios in the pump twin. She underwent cesarean section at 36 weeks of gestational age and delivered a normal live baby and another acardius acephalus twin. The acardius acephalus fetus was grossly edematous and showed tuft of coarse and lusterless hair in the midline at supraumbilical region [Figure 5]. Plain X-ray of the acardius acephalus fetus revealed normal lower limb bones, pelvic bone, lower vertebrae with the last four paired ribs, and an abrupt ending of bones above it confirming the absence of cephalic structures (i.e. head, heart, upper limbs, and thorax) [Figure 6].

Bottom Line: She delivered successfully at term a normal live baby and an acardius acephalus fetus.Plain X-ray of the acardius acephalus fetus confirmed the absence of cephalic structures.The perinatal mortality of the pump twin ranges from 35 to 55%; hence, it is essential to diagnose the presence of a pump twin at an early gestational age through improved imaging techniques, so that intervention can be planned early in the pregnancy for a better outcome of the pump twin.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh, India.

ABSTRACT
Twin reversed arterial perfusion (TRAP) sequence is an extremely rare complication of monochorionic multi-fetal pregnancy, occurring once in 35,000 births. This condition is characterized by a malformed fetus without a cardiac pump being perfused by a structurally normal (pump) twin via an artery-to-artery anastomosis in a reverse direction. We report a case of a primigravida, who came for routine antenatal checkup to our hospital at 31 weeks gestational age. Ultrasound imaging and magnetic resonance imaging revealed twin monochorionic intrauterine pregnancy with a viable, normal-appearing first twin and amorphous structured second twin connected by umbilical vessels. The patient was monitored with weekly ultrasonography, echocardiography, and Doppler ultrasound examination to ascertain the well-being of the pump twin. She delivered successfully at term a normal live baby and an acardius acephalus fetus. Plain X-ray of the acardius acephalus fetus confirmed the absence of cephalic structures. The perinatal mortality of the pump twin ranges from 35 to 55%; hence, it is essential to diagnose the presence of a pump twin at an early gestational age through improved imaging techniques, so that intervention can be planned early in the pregnancy for a better outcome of the pump twin.

No MeSH data available.


Related in: MedlinePlus