DOI: 10.7860/JCDR/2014/7012.

3965
Case Report

Twin Reversed Arterial Perfusion

Pathology Section

Syndrome (TRAP or Acardiac Twin)-

A Case Report
Kalyani R.1, Mandeep S. Bindra2

ABSTRACT
Twin reversed arterial perfusion (TRAP) syndrome is a rare condition (0.3:10,000 births) that occurs in monochorionic twin pregnancies,
resulting in coexistence of a normal “pump” twin and an acardiac twin. The acardiac twin is dependent upon the normal twin to provide
circulation by means of vascular anastomosis. Many of the bizarre defects are felt to be caused by low oxygen tension and this causes
dramatic alteration in the twin fetal physiology and high prenatal mortality. The acardiac twin is a parasite, putting the pump fetus at risk
of high output cardiac failure. Overall only 50% of pump twins survive. We present a case in a twenty year female, gravida two, para one,
living one, full term normal pregnancy, ultrasound showed twin pregnancy. Following delivery, the first fetus survived only for one day and
the second fetus showed features of TRAP syndrome.

Keywords: Acardiac twin, fetal anomaly, twin reversed arterial perfusion syndrome

Case report containing about 2000 ml of straw colour fluid. Internal examination
Twenty year old female with gravida two, para one, living one, non- showed rudimentary cardia with absence of lungs, liver, gall bladder
consanguious marriage, seven months amenorrhoea presented and spleen [Table/Fig-2]. Microscopy showed focal calcification
with over distended abdomen. Examination showed multiple fetal in rudimentary heart [Table/Fig-3], pancreas, adrenals and skin
parts, fetal heart sound not localised, cervix 30% effaced and 0.5 showed oedematous dermis. Umbilical cord measured 8 cms in
cms dilated. Ultrasound examination showed twin monochorionic length and cut section showed three vessels. Microscopy showed
diamniotic intrauterine pregnancy with viable first twin. The second one artery, two veins and one very small (rudimentary) artery [Table/
twin showed large cystic lesion around head & neck, upper Fig-4]. Cranium showed only membranous structures grossly [Table/
extremities, chest and abdomen with no cardiac activity. Anatomy Fig-5] and microscopy showed multiple dilated blood vessels. Final
of the fetus was distorted. A provisional radiological diagnosis Diagnosis was TRAP syndrome.
of Cystic Hygroma was made. Patient underwent therapeutic
amniocentesis and was discharged. Two weeks later, on second
DISCUSSION
TRAP syndrome is a rare obstetric condition unique to monozygotic
admission patient underwent emergency caesarean section for
monochorionic twin pregnancies in which there is co-existence of a
severe oligohydramnious of first fetus.
normal pump (donor) twin and an acardiac recipient (perfuse) twin
The first fetus was male (pump twin) which survived for 24 hours [1-4]. The incidence of TRAP syndrome is 1 in 35,000 pregnancies,
and the second was still born. A diagnosis of fetal papyraceous / 1 in 100 monozygotic monochorionic twin gestation and 1 in 30
massive cystic hygroma (second fetus) with fetal (first twin) demise monochorionic triplet gestation [1-6]. Most cases are seen in twins
was made. The consent for autopsy of first twin was not obtained. with only 8% in triplets [5]. The risk in multiple pregnancies of a
Consent for autopsy of second twin was obtained. higher order is ever greater [6].
The second male twin at autopsy weighted 2.3 Kg. A provisional The acardiac twin is dependent upon the normal twin to provide
clinical diagnosis of cystic hygroma was made. External examination blood by means of anomalous reversal of umbilical arterial flow
showed a cystic mass around head and neck to sacrum involving which results from umbilical artery to artery or venous to venous
proximal 3/4th of upper limb with flap like distal 1/4th of upper anastomosis between two circulations of twins which develops
limb having two fingers [Table/Fig-1]. Both feet showed 4 toes at 3-4 weeks of gestation [1,3,4,6]. Exact pathogenesis is not
and absence of small toe. Depressed nasal bridge and cleft palate known. One theory explains that umbilical vascular anastomosis
was noted. The consent for autopsy obtained. Cystic mass on in early embryogenesis in fused placenta resulting in vascular
dissection showed multiloculated subcutaneous cystic spaces disruption, early tissue hypoxia in one twin giving rise to atrophy of

[Table/Fig-1]: Gross photograph of fetus showing cystic dilated sac around head, neck & upper half of the body with deformed face and upper limb.
[Table/Fig-2]: Gross photograph showing loculated cystic spaces around upper half of the body with thorax & abdominal contents having rudimentary
heart, absence of lung, liver & spleen. [Table/Fig-3]: Microphotograph of rudimentary heart showing cystic spaces with myocardium. (H&E x 100)
[Table/Fig-4]: Microphotograph of umbilical card showing two veins, one artery and one rudimentary artery. (H&E x 100) [Table/Fig-5]: Gross photograph
showing cranial cavity having only membranous vascular structure.

166 Journal of Clinical and Diagnostic Research. 2014 Jan, Vol-8(1): 166-167
www.jcdr.net Kalyani R. and Mandeep S. Bindra, Twin Reversed Arterial Perfusion Syndrome

heart and other organs [1-4]. Other theory postulates that severe polyhydramnios and skin oedema which are features of congestive
genetic/other primary cardiac embryogenesis defect first cause the heart failure [2,4]. In the present case the consent for autopsy of
failure of cardiac development in one twin followed by anastomosis pump twin was not given.
between umbilical vessels allowing survival of affected twin [1-6]. In Prenatal diagnosis by ultrasound is suspected when a twin gestation
one case report, there is association of TRAP with VACTERL which presents with discordance and bizarre malformations with retrograde
indicates defect in early embryogenesis [7]. Chromosomal disorder blood flow in acardiac twin demonstrated by pulsed/colour flow
has been reported in 50% of cases of TRAP syndrome [1,4]. A Doppler [1,2]. Anencephaly, cystic hygroma, conjoined twins, twin
case of TRAP syndrome reported in triamniotic dichorionic triplet demise and intramniotic placental tumours are differential diagnosis
pregnancies conceived by in-vitro fertilization [6]. Monochorionicity to be ruled out. In our case ultrasound diagnosis of cystic hygroma
always highlights risk of TRAP. In our case it was monochorionic was made.
and diamniotic twins. Chromosomal analysis was not done in our
Recurrence of TRAP syndrome is unknown/not increased, likely to
case.
be low and the couples can therefore be counselled optimistically
The acardiac twin becomes dependent on the perfusion of the for future pregnancy [2,5].
“Pump” twin. The upper half of the body of an acardiac twin is
The prognosis in TRAP syndromes is lethal for acardiac twin [1,2].
extremely poorly developed and sometimes not developed at all.
The fetal mortality of the pump twin is extremely high [50-75%]
Head, cervical spine and upper limbs are usually absent. Oedema
because high output cardiac failure [1-6]. A case of survivor pump
in the upper body consistent with cystic hygroma is common. In
twin with TRAP sequence is reported who had poor neurological
contrast the lower half of the body although malformed is better
outcome on long them follow-up [5]. In our case, the pump twin
developed. This is because of mechanism of perfusion of the acardiac
survived for 24 hours and acardiac twin was still born.
twin where blood enters via abdominal aorta is deoxygenated blood
that left the normal twin. So the oxygen and nutrition available is
extracted allowing some development of caudal aspect. Once
conclusion
The obstetrician and radiologist should be aware of this anomaly
blood enters upper torso in retrograde fashion oxygen saturation is
especially in twin/multiple pregnancies so that timely proper
extremely low, halting development of heart, head and upper torso
measures can be taken to survive the pump twin. The pathologist
giving rise to severe deficits. The pump twin suffers from high output
should also be aware of this entity so that proper autopsy report can
cardiac failure which is directly proportional to size/weight of the
be given to the parents so that they can plan their future pregnancy.
acardiac twin [2].
This also highlights the importance of fetal autopsy.
The anomalies in acardiac twin are partial/total absence of cranial
vault, holoprosencephaly, absent facial structures, anophthalamia, References
micro-ophthalmia, cleft lip, cleft palate, absent or rudimentary limbs, [1] Chia-Jen Wu, Dah-Ching Ding, Shi-Shih Ren, Cheng-Chang Chang, Jueng-
diaphragmatic defects, absent lungs & heart, oesophageal atresia, Tsueng Weng, Kwei-Shai Hwang. Prenatal diagnosis and management of Twin
Reversed Arterial Perfusion (TRAP) syndrome. Taiwan J Obstet Gynecol. 2008;
ventral wall defects, ascites, absent liver & gall bladder, oedema of 47(1): 126-128.
the skin, single umbilical artery (75% cases), markedly different sized [2] Steffensen TS, Gilbert-Barness E, Spellacy W, Quintero RA. Placental Pathology
umbilical artery, inconsistent membrane development between the in TRAP sequence: clinical and pathologic implications. Fetal Pediatr Pathol.
twins and occasionally umbilical artery drawn directly into superior 2008; (1): 13-29.
[3] Hanafy A, Peterson CM. Twin Reversed arterial perfusion ( TRAP) sequence: case
mesentric artery [2,3]. Depending on anomalies the acardiac twin report and review of literature. Aust N Z J Obstet Gynaecol. 1997 May; 37(2):
are classified as hemiacardius (if the heart is incompletely formed) 187-91.
and holocardius (if the heart is absent). Other classification is [4] Kariappa TM, Chidananda HT, Mamatha R. Acardiac twin: an unusual case
report. Indian J Pathol Microbiol. 2007Oct; 50(4): 801-3.
acephalus (if head is absent), anceps (if head is poorly formed),
[5] Chih-Chen Chen, Yu-Hsin Hsu, Te-Fu Chan, Shyng-Shiou F Yuan, Jinu-Huang
acormus (presence of head only) and amorphous (unrecognizable Su. Poor long-term outcome in a survivor presenting with the twin reversed
amorphous masses) [2-5]. The classifications have little significance arterial perfusion sequence In-utero: A case report. Kaohsing J Med Sci. 2003;
as acardiac fetus is invariably fatal and the importance of survival of 19(10): 526-30.
[6] Chaliha C, Schwarzler P, Bookes M, Battash MA, Ville Y. Case report: Trisomy 2 in
pump twin is the priority [2]. In our case the acardiac fetus showed an acardiac twin in a triplet in-vitro fertilization pregnancy. Human Reproduction.
calcification in heart, pancreas, adrenals and umbilical cord showed 1999; 14 (5): 1378-80.
four vessels with membranous structures in the cranium.. [7] Sharan Athwal, Katherine Millard, Kokila Lakhoo. Twin reversed arterial perfusion
(TRAP) sequence in association with VACTERL association: a case report.
Congenital anomalies are present in about 9% of pump twins; Journal of Medical Case Reports. 2010; 4: 411.
however pump twin presents with ascites, pleural effusion,

PARTICULARS OF CONTRIBUTORS:
1. Associate Professor, Department of Pathology, ESIC Medical College & PGIMSR, Bangalore, Karnataka, India.
2. Assistant Professor, Department of Pathology, CMC, Vellore, Tamil nadu, India.

NAME, ADDRESS, E-MAIL ID OF THE CORRESPONDING AUTHOR:

Dr Kalyani R., Date of Submission: Jul 18, 2013
H.No: 127/13, “Sri Ganesh”, 4th main, 4th cross, P. C. Extension, Kolar-563101. Karnataka, India. Date of Peer Review: Oct 31, 2013
Phone: 9448402775, E-mail: drkalyanir@rediffmail.com Date of Acceptance: Nov 28, 2013
Financial OR OTHER COMPETING INTERESTS: None. Date of Publishing: Jan 12, 2014

Journal of Clinical and Diagnostic Research. 2014 Jan, Vol-8(1): 166-167 167