Clinics and Practice 2012; volume 2:e23

Undiagnosed xiphopagus twins: in the family. At admission her blood pressure was Correspondence: Gowri Dorairajan, No. 68, First a perinatal malady 140/100 mm in the right arm supine position. Cross, Nanbargal Nagar Oulgaret, She had mild anemia. There was mild pedal Puducherry-605010, India. Gowri Dorairajan edema. Abdomen was over distended with mul- E-mail: [email protected] Department of Obstetrics and tiple fetal parts. The first twin presented as Key words: conjoined twins, labor dystocia, Gynaecology, Jawaharlal Institute of vertex (left occipito -transverse). The second monoamniotic twins, perinatal ultrasound, Postgraduate Medical Education and head was felt in the fundus of the uterus. Both xiphopagus twins. Research, Puducherry, India fetal heart sounds were good. Uterus was act- ing mildly. The expected combined fetal weight Conflicts of interests: the authors declare no was 5 kg. There was no polyhydramnios. On potential conflicts of interests. vaginal examination, the cervix was 3 cm dilat- ed, fully effaced and vertex was at -3 station. Received for publication: 11 November 2011. Abstract Revision received: 13 December 2011. Membranes were absent and clear liquid was Accepted for publication: 17 December 2011. draining. Pelvis was normal gynecoid. Conjoined twins are a very rare entity. It is The patient was monitored. An intravenous associated with poor survival rate in the pres- This work is licensed under a Creative Commons access was secured and she was sedated. ence of vital organ sharing. The entity can be Attribution NonCommercial 3.0 License (CC BY- Investigations revealed normal bleeding time, NC 3.0). diagnosed as early as the first trimester. A con- clotting time and platelet count. Blood urea joined twin diagnosed late in labor is a malady and creatinine levels were within normal lim- ©Copyright G. Dorairajan, 2012 with high perinatal mortality and maternal Licensee PAGEPress, Italy its and there was no albuminuria. Her blood morbidity. We present one such case of Clinics and Practice 2012; 2:e23 pressure and progress of labor was monitored. xiphopagus twins. The management of a case doi:10.4081/cp.2012.e23 Uterine contractions were regular and optimal diagnosed late in labor can be very challeng- in intensity. Her cervical dialation and descent ing. Such obstetric challenges can be avoided of the first vertex progressed satisfactorily by a meticulous early scan with a high index of a problematic post-operative period with with the partograph line to the left of the alert only suspicion, especially in the absence of separat- pyrexia and wound infection. The wound was line. The fetal heart sounds were well pre- ing membrane while scanning multiple preg- re-sutured after controlling infection with served. Her diastolic blood pressure varied nancies. antibiotics as per the culture report. She was between 90-100 mm of Hg. The labor was discharged from the hospital in good condition uneventful till the second stage. Perineal useinfil- after 21 days. tration was given and episiotomy was made. Introduction The head of the first twin delivered sponta- neously followed by shoulders and upper limbs. The chest appeared till the lower part of ster- Conjoint twins are very rare. The incidence num. Thereafter the labor came to a standstill. Discussion 1 is about 1 in 60,000 to 1 in 100,000 deliveries. The umbilical cord was not felt. A cartilaginous In a large multicenter worldwide research that stalk was felt from the lower end of the ster- Conjoint twinning occurs in monozygotic studied the largest sample of conjoined twins, num. Traction on the delivered part did not twinning when the embryo divides 13-14 days the total prevalence was 1.47 per 100,000 cause any further descent. Neither could the after fertilization. Xiphopagus twins are 2 births. The condition can be diagnosed early limits of the stalk be made out from the vaginal joined at the xiphoid process (part of the ster- in pregnancy. Conjoined twins recognized late approach. Unfortunately, by this time none of num) and usually linked only by cartilage and in labor is an unfortunate malady associated the fetal heart sounds could be clinically aus- soft tissue. These twins share no vital organs with very high perinatal mortality and mater- cultated. Suspecting conjoined twins, she was but often have conjoined livers. This is a type nal morbidity. We report one such case of prepared for abdominal delivery. Lower seg- of ventral union. Xiphopagus is terata anacata- xiphopagus twins diagnosed in advanced labor. ment was opened; the second twin presenting didyma type of twins (Guttamacher’s classifi- Such a malady can be easily prevented if a as breech was held. There was a cartilaginous cation). Three percent of conjoined twins are checklist is followed while performingNon-commercial an ultra- stalk connecting the twins from the xiphi-ster- xiphopagus. They are more common in sound for a multiple pregnancy. num to the mid-abdomen. The conjoined twins females (3:1).3 were carefully delivered intact from the uterus Chang and Eng Bunker (1811-1874) were after reverse manipulation from the vagina the first known xiphopagus twins with con- aided by traction on the second twin from joined livers. Their autopsy had revealed a Case Report above. Both the fetuses were born dead. There band of liver along with the xiphoid with a was no extension of the uterine incision or median bursa like sac. The synchondrosis had A 32-year-old, nulliparous woman with one post partum hemorrhage. been stretched by these active twins to enable previous abortion was admitted at 40 weeks of Xiphopagus twins, both female, with a com- them to stand side by side.4 pregnancy with labor pains. She had had a bined weight of 5.4 kg were still born (Figure Harper et al.5 (1980) studied 36 pairs of non-consanguineous marriage two years earli- 1). There was a single placenta and single xiphopagus twins over 300 years. They er. She was admitted to the labor room at term umbilical cord attached to the middle of the observed that 69% were female, 10% were with pains and draining for half hour. She had stalk. The cord revealed two arteries and one associated with polyhydramnios, 50% had been already diagnosed to have a twin preg- vein with a velamentous insertion (Figure 2). cephalic-breech presentation, 36% had dysto- nancy at 30 weeks of gestation by a general Postnatal skeletal survey showed a cartilagi- cia and the still birth rate was 19%. They fur- practitioner. There was no history of prior nous connection, normal thoraces and normal ther reported that 6% had only a skin bridge, usage of contraceptive pills or ovulation induc- skeletal morphology (Figure 3). The relatives 81% had conjoined liver, 56% had conjoined ing agents. There was no history of twinning refused autopsy of the fetuses. The patient had sterna cartilage, 17% had conjoined

[page 52] [Clinics and Practice 2012; 2:e23] Case Report diaphragm, 3% had conjoined genitor-urinary nature and extent of organ sharing and pres- separating membrane indicates monoamniotic system and 33% had associated other malfor- ence of other malformations. Hoyle6 (1990) twins. In such cases detailed, patient and mations. Twenty-seven of these thirty-six pairs analyzed all attempts at surgical separation of repeated scans may be necessary to rule out had been operated upon of which 18 sets and 7 conjoined twins reported in the literature until fixed relationship of fetus to each other and singles survived. The neonatal complications 1987. Surgical separation had been attempted hence the possibility of conjoined twins. and mortality increased as the number of on 167 occasions. The overall survival rate in The Canadian Task Force on Preventive organs shared increased. this group of twins was 64%. The highest mor- Health Care has made recommendations for Prognosis of these twins depends on the tality occurred among thoracopagus (51%), the best use of ultrasound in twin pregnancies craniopagus (48%), and omphalopagus (32%). based on the study by Morin and Lim.16 The Mortality was lower with ischiopagus (19%) authors emphasize the need to adhere to these and pygopagus (23%). He also noted a signifi- recommendations to reduce such preventable cant increase in mortality for emergency sepa- maladies. ration (70%) compared with elective proce- Early diagnosis with scans keeping the dures (20%). checklist in mind can avert unwarranted chal- In a recent study,7 36 twin pregnancies with lenge and dilemma to the obstetrician in the conjoined twins seen over a period of 12 years labor room, and morbidity and extreme pain in a single tertiary hospital were analyzed. Of and psychological anguish to the mother. these, 69.4% were thoracopagus and 5.6% were Planned delivery in appropriate centers with omphlaopagus. Cardiac defects were the most expertise for separation and post-operative frequent, present in 91.6% of twin pairs. support once the extent of organ sharing is Associated malformations were present in known can reduce the perinatal mortality and 61.8% of the cases. Twelve pregnancies with maternal morbidity. poor prognosis were terminated and the remaining cases were delivered by cesarean section. Five sets of twins underwent surgical separation and 6 children survived. Overall Referencesonly survival in these was only 8.3%. Xiphopagus twins with only soft tissue 1. Rees AE, Vujanic GM, Williams WM. bridge, as in our case, carry a very good prog- Epidemic of conjoined twins in Cardiff. Br nosis after separation, with the earliestuse sur- J Obstet Gynaecol 1993;100:388-91. 8 Figure 1. The xiphopagus twins. vivors operated way back in 1952. Shukla and 2. Mutchinick OM, Luna-Muñoz L, Amar E, et colleagues reported the case of a pair who was al. Conjoined twins: A worldwide collabora- delivered by cesarean for prolonged labor.9 In tive epidemiological study of the this case, also the diagnosis had been missed International Clearinghouse for Birth in the previous scans performed during preg- Defects Surveillance and Research. Am J nancy. One of the twins had multiple malfor- Med Genet C Semin Med Genet mations and died after birth. In this xiphopa- 2011;157C:274-87. gus pair, liver was shared. Emergency separa- 3. Edmonds LD, Layde PM. Conjoined twins tion had been undertaken as it was threaten- in the United States, 1970-1977. ing the survival of the live fetus. Teratology 1982;25:301-8. Various techniques have been used to delin- 4. Spencer R. Chapter I. History, conjoined eate the hepatobiliary and vascular sharing twins: developmental malformations and amongst the xiphopagus twins before planning clinical implications. 1st edition. separation surgery. Intravenous cholangiogra- Baltimore: The Johns Hopkins Press; 2003. phy has been used to delineate the biliary tree pp. 2-3. and gall bladder.10,11 Upadhyay10 used Iodine-I 5. Harper RG, Kenigsberg K, Sia CG, et al. Figure 2. The velamentous insertionNon-commercial of the 31 rosebengal. Kling et al.11 used umbilical Xiphopagus conjoined twins: a 300-year single umbilical cord. vein angiography. Sequential scintiangiogra- review of the obstetric, morphopathologic, phy technique using Tc-99m sulfur colloid was neonatal, and surgical parameters. Am J used by Margouleff et al.12 for the delineation Obstet Gynecol 1980;137:617-29. of the size, configuration, anatomic point of 6. Hoyle RM. Surgical separation of con- fusion, and quantity of cross circulation in joined twins. Surg Gynecol Obstet xiphopagus conjoined twins. 1990;170:549-62. In our case, it was extremely unfortunate 7. Brizot ML, Liao AW, Lopes LM, et al. that, in spite of two scans performed on two Conjoined twins pregnancies: experience different occasions by two different observers with 36 cases from a single center. Prenat in the second trimester, this problem was Diagn 2011;31:1120-5. missed. The scan reports did not make any 8. Reitman H, Smith EE, Geller JS. comment on the separating membranes. Separation and survival of xiphopagus Presence of separating membrane should form twins. J Am Med Assoc 1953;153:1360-2. an important checklist while scanning twins. 9. Shukla RM, Mukhopadhyay B, Mandal KC, Diamniotic placentation almost always rules et al. Emergency separation of a xipho- Figure 3. X-ray of the twin showing normal out conjoined twins with the exception of 3 omphalopagus twin in a developing coun- skeletal survey and separate thoracic cages. cases reported in literature.13-15 The absence of try. Afr J Paediatr Surg 2010;7:197-9.

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10. Upadhyaya P. Surgical separation of hepato-splenic system of xiphopagus con- joined twins: a case report. Am J Med xiphoomphalopagus conjoined twins. J joined twins. J Nucl Med 1980;21:246-7. Genet 1990;37:558-61. Pediatr Surg 1971;6:462-5. 13. Kapur RP, Jack RM, Siebert JR. Diamniotic 15. DeStephano CC, Meena M, Brown DL, et 11. Kling S, Johnston RJ, Michalyshyn B, et al. placentation associated with omphalopa- al. Sonographic diagnosis of conjoined Successful separation of xiphopagus con- gus conjoined twins: implications for a diamniotic monochorionic twins. Am J joined twins. J Pediatr Surg 1975;10:267- contemporary model of conjoined twin- Obstet Gynecol 2010; 203:e4-6. 71. ning. Am J Med Genet 1994;52:188-95. 16. Morin L, Lim K. Ultrasound in twin preg- 12. Margouleff D, Harper RG, Kenigsberg K, et 14. Weston PJ, Ives EJ, Honore RL, et al. nancies. J Obstet Gynaecol Can 2011;33: al. Sequential scintiangiography of the Monochorionic diamniotic minimally con- 643-56.

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