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Journal of the Anatomical Society of India

Omphalopagus Ischiopagus Tetrapus Conjoined Twins - A Case Report

Author(s): Sangari, S.K., Khatri, K. & Pradhan, S.

Vol. 50, No. 1 (2001-01 - 2001-06)

Department of Anatomy, University College of Medical Sciences & G.T.B. Hospital, Shahdara, Delhi - 10095, INDIA

For Reprints, request the first author.

Abstract

The conjoined ischiopagus female twins (one well developed and the other underdeveloped) were delivered normally per vaginum. They were put on tube feeding and were passing urine and stool. After 5 days, clinical condition of the small sized child (A) deteriorated and had to be sacrificed in emergency to save the apparently well developed child (B). Initially, the child B tolerated surgery very well but after 3 days showed signs of peritonitis and died. Detailed postmortem study was performed which showed multiple cardiorespiratory, gastro intestinal, genitourinary and skeletal abnormalities.

Key words: omphalopagus ischiopagus, twins.

Introduction :

The birth of conjoined twins is rare, occuring in 1:50,000 - 1:60,000 live births. Eades and Thomas, 1966; Milham, 1966). Teratologists have proved that congenital abnormalities in animals can be induced but no one has been able to prevent the occasional occurrence of conjoined twins. Untill this is achieved, surgeons will be concerned with repair procedures, primarily depending upon the morphology of the abnormality. The opportunity to dissect ischiopagus, whose incidence among conjoined twins is very low (6%) is worth reporting.

Case report :

A 26 year old female with no previous or family history of twinning, delivered normally per veginum, full-term omphalopagus, ischiopagus tetrapus conjoined female twins (Fig. 1) weighing 4.8 kg. There was no history of taking drugs or oral contraceptives. Both babies were alive and one child looked apparently well developed (Child B) than the other one (Child A).

Child A was very thin, emaciated and showed feeble reflexes. Child B was active and responding to painful stimuli all over the body except the right leg which showed movements on pinching the child A near the site of union. Both children were put on tube feeding and passed urine and stool normally from the common external openings. After 5 days, condition of child A started deteriorating,showing signs of sepsis with a distinct line appearing on the skin at the site of union. Thus a team of surgeons decided to sacrifice this child to save child B. After opening the abdomen, it was found that two ureters, belonging one to each of the twins were opening into a single urinary bladder. Sigmoid colons of both the babies had a common rectum in the pelvis of child

B. The ureter and sigmoid colon of child A were tied and resected while no other corrective surgery was possible. Initially child B tolerated surgery very well except that the right lower limb showed signs of paralysis. After 3 days, this child showed signs of peritonitis and died.

Detailed anatomical dissection showed many cardio-respiratory, genitourinary, gastrointestinal and skeletal abnormalities.

External appearance : Both the babies showed normal features above the umbilicus except that the anterior and posterior-fontanelle of child A were much large measuring 7.5 ' 5.5 cm and 2 ' 2 cm. respectively, and right pinna of child A was in the form of hillocks. The lower trunk showed three apparently well developed lower limbs while the fourth limb was rudimentary. Both the babies showed common perineum having one external opening each of urethra, vagina and anal canal in normal sequence.

Observations on Dissection :

  Child B Child A
Cardiovascular system
Heart Normal with a small foramen Enlarged
Large foramen ovale Defective membranous part of interventricular septum with over-riding of aorta, patent ductus arteriosus.
Blood vessels Normal Normal except abdominal aorta showed anastomosis with branches of abdominal aorta of Child B
Respiratory system
Lungs Normal Small, flappy, crepitating at the peripheral part only.
Gastrointestinal system
  Normal Disposition of the Viscera was normal but under developed.
  Both the babies had common rectum and anal canal with a single external opening.
Urinary System
Left kidney and Ureter Well developed Rudimentary
Right Kidney and Ureter Rudimentary Well developed
Urinary bladder Well developed with two ureteric openings belonging one each to Child B and Child A Absent
Genital System
Uterus with Fellopian tubes Normal Rudimentary ending blindly and attached (Fig. 2) to urinary bladder by a fibrous band.
Vagina Normal Absent
Ovaries Absent Absent
Endocrine system (Gross and microscopic)
Hypophysis cerebri Normal Normal
Thyroid gland Normal Normal
Adrenal gland Left well developed, Right absent Left absent, Right well developed
Radiological observations :
Upper part of body Skeleton of Upper part of the body was normal except that 11th and the 12th left ribs showed synostosis Skeleton of upper part of the body was normal and the vertebral column was joined to the common sacrum.
Pelvis Normal but tilted by 90° to the left because of attachment of Child A on the right side One hip bone was visible which was attached in the angle between the two vertebra columns.
Lower limbs Normal; Lower end of femora had secondary centre of ossification . One limb partially developed showed normal hip bone, normal femur and an undifferentiated mass of bone in the region of leg and foot. The other limb stump was in the form of a limb bud with no bones developed in it.

Discussion :

Robertson in 1953 studied conjoined twins and determined the incidence of various types as being 73% thoracopagus, 19% pygopagus, 6% ischiopagus and 2% craniopagus. The female conjoined twins are two to three times more common than male twins.

The term ischiopagus is used to indicate a connection by any part of the pelvic girdle, usually they are joined by inferior margin of sacrum and coccyx. In the present case, both the children had common sacrum and coccyx and the hip bone of one child was joined to the vertebral column of the other child by fibrous ligaments. Developmentally the conjoined twinning in this case can be explained due to the split of embryonic disc upto the body stalk where it has got arrested. The undivided caudal part of the disc has resulted in cloacal and various other abnormalities. The common abnormalities seen in conjoined twins are pulmonary atresia,transposition of great vessels, defect or absence of atrial or ventricular septae, many intestinal abnormalities including imperforate anus and severe scoliosis. (Golladay et al, 1982) Some of these quoted abnormalities were seen in the present case.

In conjoined uniovular twins,human anatomy is essentially symmetrical or one is a mirror image of the other, likewise in this case the left kidney and the left suprarenal were well developed in child B while the right kidney and the right suprarenal were well developed in child A. Asymmetry of vanae cava, heart, aortic arches, liver, gall bladder,spleen, stomach, large and small intestine is seen frequently (Golladay et al, 1982).

Unless a life threatening situation at birth necessitates emergency evaluation or surgical separation,it is better to study electively and plan for surgery in later infancy (Paradowska et al 1969). Abnormalities occuring in ischiopagus which demand immediate correction at birth includes significantly obstructed gastrointestinal and urinary tract (Poradowska et al 1969; Bankole et al 1972) imperforate anus (Riker & Traisman 1964) and rectal atresia with vesical fistula (Remakrishnan et al 1967). In the present case surgery had to be done because the child A started deteriorating and showed signs of sepsis. Separation is successful in50% cases which depends on the degree of congenital abnormalities (Kiesewetter, 1966). In the present case, surgery in a very small child and spread of infection from the other child may be the cause of failure for successful separation.

Antenatal diagnosis of conjoined twins can be made by radiology (Borden et al, 1974) and by ultrasonography (Schmidt et al, 1981; Fagan, 1977). If abnormalities incompatible with life are found, pregnancy should be terminated.

Acknowledgement :

We owe our thanks to Dr. Raj. K. Gupta,Obstetrician and Gynaecologist in Private Practice, New Delhi, India for providing the material for this study.

References :

  1. Bankole, M. A; Oduntan, S. A; Oluwasanmi, J.O; Itayami,S,O; Khwaja, S. (1972) : The conjoined twins of Warri, Nigerian Report of an ischiopagus tetrapus with a review of the literature. Archieves of surgery 104 : 294-301.
  2. Borden, S; Robert,F; Rider, Pollard, J. J. and Mendren,W.H. (1974) : Radiology of conjoined twins, Intrauterine diagnosis and post natal evaluation American Journal of Roentgenology, 120: 424-430.
  3. Eades, J; Thomas, C. (1966) : Successful separation of ischiopagus tetrapus-conjoined twins. Annals of Surgery 164 : 1059-1072.
  4. Fagan, J.; Charles (1977) : Antepartum diagnosis of conjoined twins by ultrasonography. American Journal of Roentgenology 128 (406) : 921-922.
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  6. Kiesewetter, W. B. (1966): Surgery on conjoined (Siemese) twins Surgery 59 : 860-871.
  7. Milham, S (1966) : Symmetrical conjoined twins : an analysis of the birth records of twenty-two sets. Journal of Pediatrics 69 : 643-647.
  8. Poradowska, W; Miezyslawa, J; Stefanmia, R; Lodzinaki, K.(1969): Conjoined twins and twin parasites : clinical analysis of three examples. Journal of Pediatric Surgery 4 : 688-693.
  9. Ramakrishnan, M; Subbiah N; Saradha, A; (1967) : Conjoined twins. Report of a case.Indian Journal of Pediatrics 34 :83-87.
  10. Riker, W; Traisman, H; (1964): Conjoined twins : report of three cases III. Medical Journal 126 : 450-454.
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  12. Schmidt, W; Heberliong, D; Kubli, F; (1981): Antepartum pregnancy, American Journal of Obstetrics and Gynaecology 139 (8) : 961-963.

J. Anat. Soc. India 50(1) 40-42 (2001) Fig.1. Omphalopagus, Ischiopagus, tetrapus conjoined twins.

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Fig.2. Disposition of Viscera in pelvis. U-common urinary bladder, UB-uterus of Child B, UA-uterus of child A, R-common rectum, F-fibrous band connecting the uterus of child A to urinary bladder

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