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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 814-816
Dicephalus dipus tribrachius: A case report of unusual conjoined twins


1 Department of Pathology, Guntur Medical College, Guntur, Andhra Pradesh, India
2 Department of Paediatric Surgery, Guntur Medical College, Guntur, Andhra Pradesh, India

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Date of Web Publication27-Oct-2010
 

   Abstract 

A conjoined twin is a rarity. It occurs 1 in 50,000 to 1 in 2,00,000 fetuses. Forty percent of the conjoined twins are stillborn and an additional one-third die within 24 h of birth. They result from late twinning events about 14th day after fertilization. We report a case of stillborn conjoined twins sent for autopsy. The bodies of the fetuses were fused from the thorax to the pelvis. There were two heads, three upper limbs and two lower limbs, with fusion of the thoracic, abdominal and pelvic regions. On systemic examination, some organs were fused and some were separate. A multilocular cyst with milky fluid was seen in the pelvic region. This case is reported in view of its rarity.

Keywords: Conjoined twins, dicephalis, fetu

How to cite this article:
Aparna C, Renuka I V, Sailabala G, Nayudamma Y. Dicephalus dipus tribrachius: A case report of unusual conjoined twins. Indian J Pathol Microbiol 2010;53:814-6

How to cite this URL:
Aparna C, Renuka I V, Sailabala G, Nayudamma Y. Dicephalus dipus tribrachius: A case report of unusual conjoined twins. Indian J Pathol Microbiol [serial online] 2010 [cited 2020 Nov 24];53:814-6. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/814/72100



   Introduction Top


Conjoined twins result from late twinning events when the body axes have been molecularly specified and are beginning to separate. [1] The study of conjoined twins is important because they may be diagnosed prenatally and may be surgically separable. Patterns of coalescence of the body plans seen in clinical practice are limited because, in most of the cases, early fetal demise occurs. There are only two major methods of classification of conjoined twins: according to the orientation of the body axes and according to their degree of complete development. The usual types of conjoined twins are thoracopagus, xiphipagus, pygopagus, craniopagus and ischiopagus.

In the present case of Dicephalus dipus tribrachius, the bodies of the fetuses were fused from the thorax to the pelvis. There were two heads, three upper limbs and two lower limbs.


   Case Report Top


We received stillborn conjoined twins for autopsy. The mother was a fullterm primigravida aged 25 years and had a normal vaginal delivery. There was no history of diabetes, hypertension, use of any drugs during pregnancy or consanguinity. External examination revealed that the weight of the fetuses together was 2.9 kg. There were two heads, three upper limbs and two lower limbs, with fusion of the thoracic, abdominal and pelvic regions [Figure 1]. Both fetuses showed overlapping of the skull bones. There was a single anal opening and an ill-formed external genitalia. A skin tag was seen at the coccygeal region. A single umbilical cord of length 2 cm was present. The placenta was monochorionic, weighing 500 g. Radiological examination revealed one upper limb with fused humerus and three bones in the forearm, six metacarpals, one extra digit and fusion of the 11 th and 12 th ribs. The other two independent upper limbs were normal. Scapulae were separate, with two separate vertebral columns and sacrum. There was partial fusion of the iliac bones.
Figure 1: Conjoined twins with three upper and two lower limbs

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Systemic examination showed one large thymus in each neck.

In the thoracic cage, each fetus had two lungs. In the left fetus, right lung lobation was not well developed and the lungs were congested with a frothy fluid and the floatation test was positive. There was a single pericardial sac with two hearts [Figure 2]. In the right fetus, the right ventricle was atretic, the left ventricle was normal and the venacavae and great vessels were also normal.
Figure 2: Two hearts

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In the left fetus, both the ventricles were normal. There was a patent ductus arteriosus, the venacavae were well formed and the great vessels were normal. The abdominal aortae were fused just below the diaphragm. There was a single diaphragm.

On opening the abdomen, esophagus, stomach and intestines up to the distal ileum were separate for each fetus. There was fusion at the distal ileum with a common caecum, single appendix, colon and rectum [Figure 3]. Intestines contained meconium. Liver was single and large, with two gallbladders. Spleen was single and large. Two pancreatic glands were seen.
Figure 3: Fusion of intestines at the distal ileum

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In the right fetus, the kidney was large with fetal lobulations and a single ureter. One adrenal gland was found. In the left fetus, two kidneys with a fused ureter were seen and two adrenals were found. The urinary bladder was single, large and muscular, with narrowed lumen and two ureteric openings. There was a cyst of size 6 cm x 4 cm x 3cm, located just above the left kidney. It was multiloculated with milky fluid. Two gonads with ducts were found attached to the cyst. Another gonad was found in the pelvis [Figure 4]. Prostates were not identified.
Figure 4: Arrows pointing to the three gonads

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Microscopic examination revealed normal thymi. All four lungs showed diffuse alveolar congestion and foci of compensatory emphysema. The liver was normal, with extramedullary hematopoiesis. The spleen showed congestion. Intestines showed atrophic mucosal lining. Both pancreas were normal. All three kidneys were normal. Adrenals showed congestion. Bladder showed hypertrophy of muscle layer with atrophy of lining epithelium. All three gonads revealed structure of testes. Ducts were not patent and ends could not be traced. The lining epithelium was not identified. Cyst was lined by squamous epithelium, suggesting an epidermal cyst. No heterologous elements were seen. Both brains were autolysed. Placenta and umbilical cord were normal.

Our final diagnosis was Dicephalus dipus tribrachius with a pelvic epidermal cyst.


   Discussion Top


Conjoined twins are particularly important because they may be diagnosed prenatally [2] and can be surgically separable. They result from late twinning events when the body axes have been molecularly specified and are beginning to separate. These events occur about the 14th day after fertilization. Thus, two major body plans are laid down in one body delineated by the ectoderm and with complex relationships to amnion and intraembryonic and extraembryonic coelom. Patterns of coalescence seen in clinical practice are limited because others would probably interfere with development. Thus, early fetal demise occurs.

Classification of conjoined twins is according to the orientation of the two body axes and their degree of complete development. Most of the conjoined twins have two almost complete notochordal axes with minimal mutual interference with development. In the second type, the two notochords are closely side by side with closest apposition caudally. In the sagittal plane, the duplicated twins face in the same direction and in the coronal plane, the twins face either toward or away from each other.

Conjoined twins show extensive sharing of the common viscera. [3] The placenta of conjoined twins is always monochorionic [4] : there may be a single cord from the placental surface that may divide before inserting into the twins or two cords that may originate separately and fuse close to the infant. A single umbilical artery is common, but not universal. In our case, the placenta was monochorionic and the umbilical cord was single. Currently, the role of HOX and PAX genes in early embryogenesis are being studied.


   Conclusion Top


This case is highlighted to illustrate the rare anomaly of conjoined twins Dicephalus dipus tribrachius, which is associated with many fused pelvic organs. Hence, separation could not be attempted.

 
   References Top

1.GeoffreyA Machin, multiple pregnancies and conjoined twins. In: Enid Gilbert- Barness, editors. Potter's Pathology of the fetus and infant, 4 th ed. St.Louis: Mosby; 1995. P. 307-17.  Back to cited text no. 1
    
2.Hammond DI, Okun NB, Carpenter BF, Martin DJ, Kizaniak S. Prenatal ultrasonographic diagnosis of dicephalic conjoined twins. Can Assoc Radiol J 1991;42:357-9.  Back to cited text no. 2
    
3.Groner JI, Teske DW, Teich S. Dicephalus dipus dibrachius: an unusual case of conjoined twins. J Pediatr Surg 1996;31:1698-700.  Back to cited text no. 3
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4.Fox H, Sebire N J. The placenta in multiple pregnancy.In: Fox H, editor. Pathology of the placenta, 3rd ed. Philadelphia: Saunders Elsevier; 2007. P. 370.  Back to cited text no. 4
    

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Correspondence Address:
C Aparna
Associate Professor, Department of Pathology, Guntur Medical College, Guntur, Andhra Pradesh - 522 004
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72100

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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