A Rare Case Report of Epigastric Heteropagus: Autosite with Cyanotic Congenital Heart Disease
Amar Taksande1*, Akashi Gandhi2, Rewat Meshram3 and Amol Lohakare4
1 Professor, Department of Pediatrics, Jawaharlal Nehru Medical College, India
2 Resident, Department of Pediatrics, Jawaharlal Nehru Medical College, India
3 Associate Professor, Department of Pediatrics, Jawaharlal Nehru Medical College, India
4 Senior Resident, Department of Pediatrics, Jawaharlal Nehru Medical College, India
Submission:April 26, 2018; Published: June 06, 2018
*Corresponding author: Amar M Taksande, Department of Pediatrics, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha, Maharashtra-442004, India, Email: firstname.lastname@example.org
How to cite this article: Amar T, Akashi G, Rewat M, Amol L. A Rare Case Report of Epigastric Heteropagus: Autosite with Cyanotic Congenital Heart Disease.
J Cardiol & Cardiovasc Ther. 2018; 11(1): 555802. DOI: 10.19080/JOCCT.2018.11.555802
Heteropagus, or “parasitic,” twins are asymmetric conjoined twins, in which the host (autosite), is near normal and bears the parasite, which is totally dependent for growth on it. A parasitic twin who is attached to the epigastrium of the autosite is called as Epigastric heteropagus conjoined twins (EHTs) which are extremely rare. An associated congenital cardiac malformations, has been described in autosites. Here we report a rare case of epigastric heteropagus conjoined twins with cyanotic congenital heart disease (CHD) in the autosite.
Conjoined twins are the rarest form of monochrionic monoamniotic twins. Conjoined twins are usually of the symmetrical variety but at times they may be asymmetrical called heteropagus or parasitic twins. Parasitic or heteropagus twinsare an extremely rare condition, with an estimated incidence of 1-2 million live births . The parasitic twin usually has supernumerary limbs without a functional heart or brain. In epigastric heteropagus conjoined twins, the parasite is attached to the autosite in the epigastric region mostly to the xiphi-sternal area. Here we report a rare case of epigastric heteropagus conjoined twins with Tetralogy of Fallot in the autosite.
A 17-day-old full term boy was born to a 26yrs old mother was referred with epigastric parasitic twins. The baby was initially treated in other hospital but developed severe infection and then referred to our hospital. The mother had no history of drug intake, radiation exposure or illness during pregnancy. There was no history of twin delivery in the family. The antenatal
period was uneventful. The parasite twin was attached to the epigastrium of the autosite. The parasite possessed rudimentary upper limbs, well developed lower limbs, pelvis, and genitals (Figure 1). However, the limbs of the parasite had neither active movement nor response to tactile stimulus. No head, neck or thoracic structures present. Omphalocele was present in the autosite as the attachment of parasite. The autosite twin was well developed, with intact bowel and bladder function (Figure 2). The autosite had a systolic cardiac murmur suggesting congenital heart disease. Infantogram was done suggestive of normal bowel shadows of the autosite twin and abdomen viscera was connected with the parasite twin. Ultrasonography of the autosite suggested normal genitourinary system. Echocardiography of the autosite showed tetralogy of fallot, having large ventricular-septal defect (right to left shunt), overriding of aorta and severe infundibular stenosis (gradient 60mmHg). The baby died after a days of admission due to fulminant sepsis. The parents refused for a post-mortem examination and thus detail evaluation was not done.
Conjoined twins are rarely observed, but heteropagus
conjoined twins occur even less frequently. Heteropagus twins
are a rare form of conjoined twins where one of the twins is fully
formed (autosite) and other is incomplete (parasite). Asymmetric
and parasitic conjoined twins are anomalies of monochorionic
monoamniotic twins [1,2]. The etiopathogenesis of conjoined
twins are not very clear. There are two possible theories of which
first is the fission theory in which incomplete fission due to division
of the blastocyst beyond the 13th day of fertilization. The second
theory is the fusion hypothesis in which an incomplete division
of a single zygote, the formation of two cellular masses within
the blastocyst at the end of the second week post conception. In
consequence, a sole embrionary structure is formed, but with
a single yolk sac. The another theory mentioned was ischemic
atrophy, in which the parasite twin is the result of an ischemic
accident in the uterus, followed by the death and resorption of one
of the foetuses [3,4].
Conjoined twins are classified by the site of union and their
symmetry and asymmetry. Symmetrical conjoined twins have two
identically formed twins which are fused to each other at a certain
site on both bodies. They may share viscera at that site and the
nomenclature is according to the site of fusion i.e. craniopagus
(Cranium), thoracopagus (fusion at chest), omphalopagus (fusion
at anterior abdominal wall), is chiopagus (Pelvis/ischium),
pyopagus (Buttocks/sacrum), or a combination of any of the
above fusion. Heteropagusor parasitic twins are also called as
asymmetrical conjoined twins in which the incompletely formed
foetus ‘parasite’ is attached to the normally formed foetus or the
‘autosite’ at a particular point. The parasite is not completely
formed and may only be evident by supernumerary limbs. The
parasite usually does not have a functional brain or heart and does
not share the host twin’s gastrointestinal tract or liver but may
have a rudimentary gastrointestinal tract or urinary system of its
own with connection to that of the host .
Sharma et al.  reviewed the 39 cases of parasitic twins
reported in 10-year interval and found that epigastric heteropagus
was the most common anatomical type (59%). Various congenital
defects such as anencephaly, cleft lip, cleft palate, lumbosacral
meningomyelocele, cardiac anomalies, gastrointestinal and
urogenital anomalies may be associated with conjoined twins.
Abdominal wall and spinal defects are commonly seen in
omphalopagus and rachipagus twins, respectively. An associated
congenital cardiac malformations, has been described in autosites.
However, a single-ventricle heart anomaly in the autosite has been
reported in a very few cases [4-7]. The cardiac anomalies was
found in the autosite index case. Previously, only single reported
cases of epigastric heteropagus twins with TOF inparasite,
however this is the rare case of associated TOF in autosite . Of
all the heteropagus twins, epigastric heteropagus twins (EHT) are
among the least documented cases in literature. In contrast to the
female predominance of symmetrically conjoined twins, a male
predominance is observed in EHT [6-9]. Antepartum diagnosis
of parasitic twinning plays an important role in management.
Therefore, heteropagus twins should be distinguished from
more severe congenital malformations to avoid unnecessary
termination. The diagnosis of Conjoined twins should be
considered in any twin pregnancy that has single placenta, no
visible separating amniotic membrane and polyhydramions that
occurs frequently in conjoined twin pregnancy as compared
with normal twin pregnancy . Early referral and intensive
management can help in achieving better survival in these babies.
The ultimate outcome however will depend upon the associated
malformation especially cardiac [9,10]. So, the improvement in
paediatric cardiac care services can improve survival in babies
having cardiac anomalies.
A parasitic twin in which one of the twins is severely defective
and is depended on the vascular supply from its counterpart for its
growth. To the best of our knowledge, this is the rare case report
of Epigastric heteropagus case in the literature with a tetralogy of
fallot in the autosite.