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The literature on conjoinedtwins emphasizes the needfor careful investigation and
planning for the separation proce-dure.1,2 Surgical separation has beencarried out on 7 pairs of conjoinedtwins at National Taiwan UniversityHospital since 1979. We report thesuccessful separation, at 7 days of age,of xiphoomphalopagus conjoinedtwins who had a total body weight ofonly 3502 g.
CASE REPORT
A pair of female conjoined twinswere identified by prenatal sonogra-phy at 32 weeks’ gestation. They weredelivered by emergency cesarean sec-tion at 35 weeks’ gestation after themembranes ruptured prematurely.The combined body weight of thetwins was 3502 g. Both babies hadgood Apgar scores. They were joinedface to face from the lower sternum to
the infraumbilical area. An omphalo-cele, 3 × 3 cm in dimension, wasnoted on the lower part of the con-joined bridge (Fig. 1).MRI and aortography showed a
fused liver with no major vascularconnection. Echocardiography indi-cated a patent ductus arteriosus ineach baby. No other major anomalieswere found. The patent ductus arte-riosus had disappeared in twin A by3 days of age but persisted in twin B.
Case ReportÉtude de cas
SUCCESSFUL EARLY SEPARATION OF A PREMATUREXIPHO-OMPHALOPAGUS CONJOINED TWINS: A CASE REPORT
Hong-Shiee Lai, MD, PhD; Po-Huang Lee, MD, PhD; Shu-Hsun Chu, MD; Ming-Ting Chen, MD; Tsuo-Wu Lin, MD; Yuan-Chao Duh, MD; Wei-Jao Chen, MD, DMSc
From the Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan, ROC
Accepted for publication Sept. 25, 1996
Correspondence and reprint requests to: Dr. Hong-Shiee Lai, Department of Surgery, National Taiwan University Hospital, No 7, Chung-Shan South Rd. Taipei, Taiwan,ROC; fax: 886-2-3412969, e-mail: [email protected]
© 1997 Canadian Medical Association (text and abstract/résumé)
Premature xiphoomphalopagus conjoined twins were successfully separated at 7 days of age. The totalbody weight of the twins before separation was 3502 g. One twin had persistent patent ductus arteriosusand signs of cardiac failure at 4 days of age. This was managed with indomethacin. Thirty-six hours laterthe second twin became anuric, necessitating early emergency separation. The twins were joined from thelower sternum to the infraumbilical area. The liver was fused, but there was no major vascular connection.There were no other major anomalies. The babies stood the procedure well and were healthy at follow-up8 months after separation.
Des jumeaux prématurés joints au niveau xipho-omphalopage ont été séparés avec succès à l’âge de septjours. Les jumeaux avaient une masse corporelle totale de 3502 g avant la séparation. Un jumeau avait unepersistance du canal artériel et a présenté des signes d’insuffisance cardiaque à l’âge de quatre jours. On atraité le problème avec de l’indométhacine. Trente-six heures plus tard, le deuxième jumeau est devenuanurique, et c’est pourquoi il a fallu procéder à une séparation d’urgence précoce. Les jumeaux étaientjoints de la partie inférieure du sternum jusqu’à la zone sous-ombilicale. Il y avait fusion des foies, sansconnexion vasculaire importante, toutefois. Il n’y avait pas d’autres anomalies majeures. Les bébés ont trèsbien survécu à l’intervention et étaient en bonne santé au suivi, huit mois après la séparation.
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The hourly urine excretion of thetwo babies was quite different: 1.1mL/kg in twin A and 7.6 mL/kg intwin B. Tachypnea and tachycardiawere noted in twin B on the fourthday. Indomethacin (0.4 mg/12 h for3 doses) was given to twin B for herpersistent patent ductus arteriosus
and early signs of heart failure on thefifth day. Twin B’s patent ductus ar-teriosus was closed 2 days later.However, twin A was anuric for 30hours after indomethacin was givento twin B. As a result, an emergencyseparation was necessary on the sev-enth day.
SEPARATION OF PREMATURE CONJOINED TWINS
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FIG. 1. Xiphoomphalopagus conjoined twins showing the fused lower sternum, omphalo-bridge and asmall omphalocele (arrow).
LAI ET AL
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142 JCC, Vol. 40, No 2, avril 1997
Surgical technique
A circumflex incision line was madewith 2-cm deviation to the right sideof each baby. The fused lower sternumwas divided sharply with scissors, andthe common peritoneal cavity wasopened. Each baby had a normalstomach, duodenum, gallbladder, pan-creas, spleen, small intestine and colon.However, the left lobes of the two liv-ers were fused. The central part of theengorged fused area was divided by ul-trasound aspirator. A 7 × 5 cm2 fusingsurface of the livers was demonstrated.Multiple cross-circulating vessels werefound in the conjoined portion of theliver. After checking bleeding and plac-ing drainage tubes (2 for twin B, 1 fortwin A), the abdominal wall defect wasclosed in layers. The skin flap was ade-quate. The immediate postoperativebody weights were 1890 g for twin Aand 1558 g for twin B. Both twinsstood the procedure well. The totaloperation time was 3 hours and 47minutes. Each baby received 60 mL offresh blood during the operation.The postoperative course was un-
complicated. The urine of twin Astarted to flow 4 hours postopera-tively. The twins were discharged 24days after the operation. At follow-up8 months after separation, both babieswere healthy, thriving and developingwell.
DISCUSSION
The reported incidence of con-joined twins is extremely variable.1,3
Prenatal sonography can identify theconjoined anomaly early in the secondtrimester of pregnancy. The pregnancycan be terminated if the anomaly isfound by prenatal examination. How-ever, the anomaly may be missed by a
poorly performed sonographic exami-nation. Conjunctions may be eithersymmetric or asymmetric. The resultsof separation depend on the form ofconnection and the associated anom-alies.4 The separation of ischiopagusconjoined twins is a surgical challenge,yet the success rate is usually high inomphalopagus conjunctions.5
The optimum age for surgical sepa-ration depends on the conjoined form.In the more complex forms, a delay isrecommended to achieve a better nu-tritional state. Otherwise, early separa-tion is common practice to minimizecomplications such as thoracolumbarscoliosis, growth retardation or urinarytract infection. However, separationfor conjoined twins is usually per-formed after the neonatal stage exceptin emergency situations. Sometimes,emergency separation must be per-formed to salvage the healthier twin ifthe other twin is severely malformed.6,7
Our case may represent the smallestxiphoomphalopagus conjoined twinsseparated successfully. Originally, theseparation of the twins was scheduledto take place when they were at least 1 month old. However, it became anearly emergency separation because ofthe anuric status of twin A. The opera-tion improved the anuric status of twinA, because it prevented the unbal-anced cross-circulation from twin A totwin B and it cancelled the side effecton renal function of indomethacinwhich had been given to twin B.If 1 twin is expected to die as a re-
sult of severe congenital abnormalities,abdominal wall and skin closure shouldfavour the more viable twin.1 Birmoleand associates7 reported an urgent sep-aration of omphaloischiopagus tetrapusconjoined twins on day 14, when thesurvival of one was threatened by theseverely malformed counterpart.
In our report, a fair circumflex inci-sion was made, and the conditions ofboth twins were stable after separa-tion. This experience showed that anemergency separation procedure insmall, premature, xiphoomphalopagusconjoined twins is feasible, and a suc-cessful result can be expected.
References
1. Spitz L, Capps SN, Kiely EM.Xiphoomphaloischiopagus tripus con-joined twins: successful separation fol-lowing abdominal wall expansion. J Pediatr Surg 1991;26:26-9.
2. Chadha R, Bagga D, Dhar A, MohtaA, Malhotra CJ, Taneja SB. Epigas-tric heteropagus [review]. J PediatrSurg 1993;28(5):723-7.
3. Hanson JW. Incidence of conjoinedtwinning [letter]. Lancet 1975;2(7947): 1257.
4. Gerlis LM, Seo JW, Ho SY, Chi JG.Morphology of the cardiovascular sys-tem in conjoined twins: spatial and se-quential segmental arrangements in36 cases [review]. Teratology 1993;47(2):91-108.
5. Walton JM, Gillis DA, Giacomanto-nio JM, Hayashi AH, Lau HY. Emer-gency separation of conjoined twins[review]. J Pediatr Surg 1991;26(11):1337-40.
6. Chen WJ, Chen KM, Chen MT, LiuTK, Chu SH, Tsai TC, et al. Emer-gency separation of omphaloischiopa-gus tetrapus conjoined twins in thenewborn period. J Pediatr Surg 1989;24(12):1221-4.
7. Birmole B, Kulkarni B, Shah R, Kara-purkar S, Vaidya A, Vaidya M, et al.Xiphoomphalopagus twins: separa-tion in the newborn. J Postgrad Med1993;39(2):99-101.
