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Year : 2015  |  Volume : 4  |  Issue : 2  |  Page : 142-144

Situs inversus and dextrocardia in epigastric heteropagus twins: An uncommon entity explored!!

1 Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
2 Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Date of Web Publication6-Apr-2015

Correspondence Address:
Dr. Harish Chellani
Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4847.154129

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Epigastric heteropagus twin is a rare form of parasitic twins. Though heart defects may be found in 28% of these babies, an association with situs inversus and dextrocardia is extremely uncommon. We present a rare case of epigastric heteropagus twinning with dextrocardia and situs inversus.

Keywords: Autosite, dextrocardia, epigastric heteropagus twin, parasitic twin

How to cite this article:
Mittal A, Gupta S, Arya S, Dawson L, Chellani H. Situs inversus and dextrocardia in epigastric heteropagus twins: An uncommon entity explored!!. J Clin Neonatol 2015;4:142-4

How to cite this URL:
Mittal A, Gupta S, Arya S, Dawson L, Chellani H. Situs inversus and dextrocardia in epigastric heteropagus twins: An uncommon entity explored!!. J Clin Neonatol [serial online] 2015 [cited 2020 Apr 5];4:142-4. Available from: http://www.jcnonweb.com/text.asp?2015/4/2/142/154129

  Introduction Top

Conjoined twinning is a rare entity while heteropagus twinning is still rarer. When the parasite (the anatomically incomplete twin) is attached to the autosite (the anatomically normal twin) at the epigastrium it is known as epigastric heteropagus twinning which is extremely rare. [1]

  Case Report Top

A 2.4 kg girl child was born vaginally to a primigravida mother whose periconceptional and antenatal period was uneventful. The baby cried immediately after birth and required nursery admission for management of conjoined twin. The parasitic twin was attached to the epigastrium of the child. It consisted of a pair of lower limbs, pelvis and female external genitals from which urine was being passed, however the anal opening was imperforate [Figure 1]. Bowel was palpated in the tissue connecting the two. The knee and the ankle joint was fixed and there was bilateral congenital talipes equino varus in the parasitic twin. The parasitic portion however had no spontaneous movement and no response to painful stimulus. Bilateral syndactyly of the fourth and fifth digits were also noted.
Figure 1: (a) Epigastric heteropagus twin attached to the autosite (arrow). (b) Parasitic twin having an imperforate anus (arrow), bilateral syndactyly of fourth and fifth digits is also appreciated (black arrow heads)

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On examination of the autosite, there was no respiratory distress, child was hemodynamically stable, but the heart sound in the autosite was heard on the right side. The child passed meconium and urine. The child was started on cup feeding, which the child tolerated well initially, but on 4 th day baby developed respiratory distress and cardiac failure, the condition deteriorated rapidly and baby expired within 5 h of symptoms.

Autopsy findings

To evaluate the exact anatomical connection of the twins and the cause of death an autopsy was performed after taking consent from the parents.

The lungs were normally developed with dextrocardia with situs inversus. Heart had a single ventricle with both aorta and pulmonary artery arising from the ventricle. The small intestine was duplicated after the duodeno-jejunal junction and intestine was seen connected to the anus in the parasitic lower segment which was imperforate [Figure 2]. In the autosite the bowel was complete with well-developed small and large intestine and ended as a patent anal opening with normal function and tone. A Meckel's diverticulum with omphalocele was present. The liver in the main body was enlarged.
Figure 2: Autopsy showing dextrocardia with situs inversus (white arrow head), an enlarged central liver (black arrow head), bowel communicating with parasitic twin (white arrow) and the kidney of the parasite (black arrow)

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It was found that the parasite had an independent genitourinary system with an extra kidney and ureter draining into the bladder with no connection to autosite. The genital system consisted of a separate pair of ovaries, fallopian tubes and uterus in the parasite. The kidneys and other organs in the autosite were unremarkable.

The sections from both lungs show normally inflated alveoli with severe congestion of septal blood vessels and few foci of hemorrhages. The sections from liver were suggestive of sinusoidal congestion.

  Discussion Top

Parasitic twins account for 1-2% of all conjoined twins. The dependent undeveloped twin, the parasite, is attached to independent developed twin called autosite at different sites. Parasite attached to host's epigastrium is rare and is called epigastric heteropagus.

Conjoined twins are monozygotic twins in which the inner cell mass does not completely split. Incomplete division of the embryonic disk after 13 th day post conception results in the formation of conjoined twins. The two embryos are joined by a tissue bridge. Spencer proposed an alternative theory of fusion of two originally separate monozygotic embryonic disks, to explain the conjoined twin etiology. Some authors suggested that parasitic twin occur as a result of selective ischemic damage in-utero leading to death or partial resorption of, one of the twins, resulting in an incomplete parasitic twin attached to a fully developed twin. The parasitic twin is also sometimes referred to as asymmetrical conjoined twin. [2]

Epigastric conjoined twins are predominantly seen among males. In epigastric heteropagus; the parasite has duplication of the pelvis and pelvic organs, such as the complete urinary tract, external and internal genitalia. Omphalocele is present in most of the cases. [3] In the present case the twins were female, with complete well developed independent female genitourinary system and external genitals. The twins however shared a common gut. The parasitic twin in this case was acephalic and acardiac as reported in many other cases. [2],[3],[4]

Laterality defects in conjoined twins have been studied widely on animal models. Levin et al. in 1996 proposed various theories on laterality based on a study on 167 pairs of conjoined twins obtained from the literature. In cases (69) where the twins were joined obliquely at the chest and/or abdomen (thoracopagi) or laterally at the chest (dicephali), one of the twins in 33 pairs had reversal in heart situs; however none of the twins (of 98 remaining) joined only at the head (craniopagi) or pelvis (ischiopagi) exhibited laterally defects. Strikingly, when laterally defects are present, they occur most frequently in the twin on the right side in dicephalus (86%) and thoracopagus (71%) twins. This may explain the occurrence of situs inversus with dextrocardia in our case as well. [5]

Epigastric heteropagus conjoined twins, is an extremely rare condition, and only 44 cases have been previously reported in the world literature. [3],[4] There may be an associated heart disease in 28-30% cases. In this case the autosite had a dextrocardia with a single ventricle. It has been described by Ohye et al. that among 164 deaths of babies with single ventricle there were 29 sudden unexpected deaths. We postulate that sudden death due to cardiac disease was the cause of death in the present case as well [6],[7] There have been only three reported cases of epigastric heteropagus twins with single ventricle previously, however this is the 1 st time where it is being reported to be associated with situs inversus. [8]

  References Top

Xie JT, Zhou L, Yang ZL, Sun HY. Epigastric heteropagus conjoined twins: Two case studies and associated DNA analysis. Clinics (Sao Paulo) 2012;67:527-9.  Back to cited text no. 1
Qasim M, Shaukat M. Epigastric heteropagus twin. APSP J Case Rep 2011;2:24.  Back to cited text no. 2
Kesan K, Gupta A, Gupta RK, Kothari P, Ranjan R, Karkera P, et al. Gluteal flap for omphalocele repair in a case of epigastric heteropagus: A novel approach for surgical management. Indian J Plast Surg 2013;46:127-9.  Back to cited text no. 3
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Abubakar AM, Ahidjo A, Chinda JY, Tahir C, Abubakar S, Adamu SA, et al. The epigastric heteropagus conjoined twins. J Pediatr Surg 2011;46:417-20.  Back to cited text no. 4
Levin M, Roberts DJ, Holmes LB, Tabin C. Laterality defects in conjoined twins. Nature 1996;384:321.  Back to cited text no. 5
Raynal P, Petit T, Ravasse P, Herlicoviez M. A rare case of epigastric heteropagus twinning. J Gynecol Obstet Biol Reprod (Paris) 2001;30:65-9.  Back to cited text no. 6
Ohye RG, Schonbeck JV, Eghtesady P, Laussen PC, Pizarro C, Shrader P, et al. Cause, timing, and location of death in the single ventricle reconstruction trial. J Thorac Cardiovasc Surg 2012;144:907-14.  Back to cited text no. 7
Ozkan-Ulu H, Yilmaz Y, Sari FN, Altug N, Uras N, Dilmen U. An unusual case of heteropagus: Autosite with a complex cardiac malformation. Pediatr Neonatol 2011;52:358-60.  Back to cited text no. 8


  [Figure 1], [Figure 2]

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