Paediatrica Indonesiana

VOLUME 48 November ‡ NUMBER 6

Case Report

Separation of ischiopagus tetrapus conjoined twins in Dr. Sardjito Hospital, Yogyakarta

Rochadi

onjoined twins are one of the greatest delivered spontaneously with the combined birth challenges to modern pediatric surgery. ZHLJKWRIJUDPV7KHPRWKHUGLHGDIWHUGHOLYHU\ Despite extensive preoperative investi- GXHWRXQFRQWUROOHGEOHHGLQJ:HODEHOHGWKHILUVWEDE\ CJDWLRQVWKHSUHFLVHGHVFULSWLRQRIWKH DV%DE\$DQGWKHVHFRQGRQHDV%DE\<7KH\ZHUH conjoined anatomy is often only possible during conjoined at the pelvic region with their lower limbs surgery.1-3 SDUDOOHORQERWKVLGHVDULVLQJIURPODWHUDOSDUWRIWKHLU 6RFLDODWWUDFWLRQPHGLFDOHWKLFDOSUREOHPV bodies. The anterior abdominal wall ran from one to and sociological impacts are three characteristics the other without clear demarcation. There was only IDFHGE\FRQMRLQHGWZLQVRU6LDPHVHWZLQVVRFDOOHG one umbilical cord located exactly in the middle of the due to the famous conjoined twins Chang and Eng DQWHULRUDEGRPLQDOZDOOWZRDQXVHVDQGWZRYXOYDH %XQNHUERUQLQLQ6LDP7KH\ZHUHXQLWHGDW (Figures 1 and 2). WKHXPELOLFXVE\FPZLGHEULGJLQJWLVVXHWKH\ $OORUJDQV\VWHPVZHUHHYDOXDWHGZLWK[UD\V had never undergone surgery and lived together for UHQRJUDPXUHWHURF\VWRJUDPEDULXPHQHPDDQG 63 years.4-6 The existence of conjoined twins was CT scan. The bony pelvis formed a ring and joined mentioned in the old Egyptian records. The first with the normal lower extremities. The upper and successful separation was performed in Bazilea by lower gastrointestinal studies demonstrated normal -RKDQQHV)RWLR7KHWZLQVZHUHMRLQHGDWWKHUHJLRQ DQGVHSDUDWHVWRPDFKVVPDOOLQWHVWLQHVDQGDVLQJOH between the xiphoid process and the umbilicus. Fotio FHFXPVLQJOHDVFHQGLQJFRORQZLWKVLQJOHDSSHQGL[ VXFFHHGHGLQVHSDUDWLQJWKHXPELOLFDOYHVVHOVDVZHOO The two small intestines joined at the terminal ileum as tying and transecting the bridging tissue. This case and entered the solitary cecum. Each baby had two ZDVSUHVHQWHGLQE\.RHQLJ This paper reports a case of conjoined twins ZKRZHUHVXFFHVVIXOO\VHSDUDWHGLQ6DUGMLWR+RVSLWDO

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Paediatr Indones, Vol. 48, No. 6, November 2008‡381 Rochadi: Separation of ischiopagus tetrapus conjoined twins

Figure 1. The anterior abdominal walls Figure 2. The posterior side with 2 anuses kidneys in the shared pelvis with two ureters which There were two separate kidneys lying in the emptied into two bladders that was attached one to SHOYLVDQGWKHEODGGHUZDVVHSDUDWHG$*RUH²7H[ another. The functions of micturition were normal patch was used to reconstruct the distal part of the except for Baby A who had hydroureter with reflux anterior abdominal wall. A thick layer of subcutaneous and mild hydronephrosis caused by occlusion of the fat was spread over the patch and the skin layer was urethral orifice. Each of them had one normal uterus. finally reconstructed (Figure 4). Operation was performed when they were seven months old to prevent further damage to Baby A’s NLGQH\7KHLUFRPELQHGZHLJKWZDVJUDPV 3UHSDUDWLRQVIRUVXUJHU\ZHUHLQFOXGLQJIRUPLQJ DWHDPFRQVLVWHGRIH[SHUWVZKRZHUHDQHVWKHWLVWV VXUJHRQVLQWHQVLYHFDUHVSHFLDOLVWVHOHFWULFDO HQJLQHHUVEORRGEDQNDQGODERUDWRU\VWDIIV7KHPDMRU VXUJLFDOFRQVLGHUDWLRQVLQFOXGHGGUDSLQJSURFHGXUH RSHUDWLYHDSSURDFKWKHVHTXHQFHRIRUJDQVHSDUDWLRQ SUHVHUYDWLRQRIXULQDU\WUDFWSHOYLFUHFRQVWUXFWLRQ and the closure of the huge abdominal wounds in both twins. $ODUJHWUDQVYHUVHLQFLVLRQQHDUO\FPLQ Figure 3. The transverse colon was divided and an length was made across the anterior abdominal wall. ileotransversostomy anastomosis was performed Exploration confirmed the accuracy of the preoperative HYDOXDWLRQ(DFKLQIDQWKDGDQRUPDOVWRPDFK GXRGHQXPOLYHUJDOOEODGGHUSDQFUHDVVSOHHQDQGVPDOO intestines. Two small intestines were in normal length DQGMRLQHGIRUDERXWFPIURPWKHVLQJOHFHFXP %HVLGHVWKHVLQJOHFHFXPZLWKVLQJOHDSSHQGL[WKHUH ZHUHDOVRVLQJOHDVFHQGLQJFRORQVLQJOHWUDQVYHUVH FRORQDQGWZRVLJPRLGVZLWKWZRUHFWXPV The two small intestines were transected at the junction where they joined the common terminal ileum. The transverse colon was then divided and an ileotransversostomy anastomosis was performed Figure 4. The reconstruction of the distal part of the (Figure 3). anterior abdominal wall using Gore-Tax patch

382‡Paediatr Indones, Vol. 48, No. 6, November 2008 Rochadi: Separation of ischiopagus tetrapus conjoined twins

The difficulties during operation were when There was no problem in nutrition and they began the separation of the bridging vascular system in the to walk at 13 months. Their mental development is SUHVDFUDOUHJLRQVHSDUDWLRQRIWKHIXVHGERQHVDQG excellent; there were no ambulatory problems and UHFRQVWUXFWLRQRIWKHSHOYLVWKHSHOYLFIORRUDQGWKH they speak fluently (Figure 6). anterior abdominal wall were performed (Figure 5). There was no hemodynamic instability during the operation. There was only minimal blood los. Ten Discussion PLQXWHVDIWHUWKHVHYHQKRXUVRIVXUJHU\ERWKWZLQV moved their limbs and opened their eyes. Three days The exact incidence of Siamese twins varies from one ODWHU%DE\$GHYHORSHGSHULWRQLWLVFDXVHGE\OHDNDJH SHUOLYHELUWKVWRRQHSHUOLYHELUWKV which was successfully treated by colostomy and re- $SSUR[LPDWHO\WZRWKLUGVDUHIHPDOHDQGDOOSDLUVDUH anastomosis. of the same sex. There are five main types of conjoined WZLQVWKRUDFRSDJXV  RPSKDORSDJXV   S\JRSDJXV  LVFKLRSDJXV  DQGFUDQLRSDJXV   ,QWKRUDFRSDJXVWZLQVWKHMRLQLQJLQFOXGHVWKH thorax and may extend from manubrium to umbilicus. Seventy–five percent of the twins have varying degrees RIFDUGLDFIXVLRQ,QRPSKDORSDJXVWKHMRLQLQJLVDW the abdominal wall and fusion of the liver must be anticipated when the junction of the twins includes WKHXPELOLFDODUHD,QS\JRSDJXVWKHUHLVDWWDFKPHQW DWWKHEXWWRFNDUHDDQGYHUWHEUDOFROXPQVXVXDOO\LQ continuity but spinal cords are usually separated. In LVFKLRSDJXVWKHIXVLRQH[WHQGVIURPWKHXPELOLFDODUHD to include the lower trunk and pelvis with three or Figure 5. After separation four limbs. Craniopagus twins might be partial or total FRQMRLQLQJIRUPVKDYLQJDMXQFWLRQDWEURZYHUWH[ or parietal bone.  Identical twins develop from the division of a single fertilized ovum. By the sixth day after IHUWLOL]DWLRQWKHKXPDQ]\JRWHVEHFRPHEODVWRF\VWV $WRQHSROHRIWKHEODVWRF\VWVRPHFHOOVDJJUHJDWH NQRZQDVWKHLQQHUFHOOPDVV)URPWKLVFHOOPDVV WKHHPEU\RDPQLRQDQG\RONVVDFGHYHORS,QQHUFHOO mass is totipotent and may split to form two germinal discs which can develop into two identical individuals. Division of the zygote within the first seven days after fertilization yields monozygotic identical twins. 7KHXOWUDVRXQGH[DPLQDWLRQLQWKHth week of gestation is very useful to detect polyhydramnios with RQHSODFHQWDRQHXPELOLFDOFRUGZLWKPRUHWKDQWKUHH EORRGYHVVHOV3UHQDWDO&7H[DPLQDWLRQRUPDJQHWLF resonance imaging may detect the exact anatomy of the malformation and the presence of other associated anomalies. 3UHRSHUDWLYHH[DPLQDWLRQVDUHSHUIRUPHGWR Figure 6. The twins at the age of two evaluate the internal anatomy and to determine

Paediatr Indones, Vol. 48, No. 6, November 2008‡383 Rochadi: Separation of ischiopagus tetrapus conjoined twins whether one or both twins can survive after separation. LQWUDRSHUDWLYHDQGSRVWRSHUDWLYHSUREOHPVLQWHQVLYH 7KHDQDWRP\WKHIXQFWLRQRIDOOPDMRURUJDQV\VWHPV intraoperative monitoring and good management. and the skeleton are needed to be evaluated. In ,QFRQFOXVLRQLVFKLRSDJXVWHWUDSXVLVDYHU\UDUH WKRUDFRSDJXVFRQMRLQHGWZLQV&7VFDQRIFKHVW YDULDWLRQRIFRQMRLQHGWZLQV:LWKDWHDPDSSURDFK DQGOLYHULVRWRSLFELOLDU\WUDFWVFDQFRQWUDVWVWXGLHV DQGJRRGSUHSDUDWLRQWKH\FDQEHVXFFHVVIXOO\ of the gastrointestinal tract and ECG are needed. separated. Omphalopagus needs CT and isotopic scans of the OLYHUDQGELOLDU\WUDFW+HDG&7VFDQDUWHULRJUDP and EEG are important in craniopagus conjoined References WZLQV&7VFDQRIWKHDEGRPHQDQGSHOYLVFRQWUDVW VWXGLHVRIJDVWURLQWHVWLQDODQGXULQDU\WUDFWDEGRPLQDO  +ROFRPE*:2·1HLOO-$Conjoined twins in pediatric arteriography and genitography are needed in VXUJHU\ndHGLWLRQ3KLODGHOSKLD:%6DXQGHUVS LVFKLRSKDJXVFRQMRLQHGWZLQV3\JRSDJXVFRQMRLQHG  twins need CT scan of abdomen and pelvis.  6FKQDXIHU8*&RQMRLQHGWZLQVLQ6ZHQVRQCVSHGLDWULF The separation of conjoined twins is a surgical VXUJHU\thHG1RUZDON&RQQHFWLFXW$SSOHWRQ /DQJH FKDOOHQJHHVSHFLDOO\WKDWRILVFKLRSDJXVW\SH7KH S planning for eventual surgical separation should  6SHQFHU5&RQMRLQHGWZLQVLQ$VKFUDIW3HGLDWULF6XUJHU\ be unhurried. It needs multidisciplinary team. The 3rdHG3KLODGHOSKLD:%6DXQGHUVS main goal is to achieve life as normal as possible. A 4. Stauffer UG. Conjoined twins in neonatal surgery. 3rd ed. variety of diagnostic studies is necessary to create %XWWHUZRUWK &R/WGS diagrams of organ sharing and demonstrate possible  6WULQJHU0'&DSSV61-&RQMRLQHGWZLQVLQVXUJHU\RIWKH coexisting congenital anomalies. Separation is best QHZERUQ(GLQEXUJ/RQGRQ0DGULG0HOERXUQH&KXUFKLOO SHUIRUPHGRQDQHOHFWLYHEDVLVZKHQWKHEDELHVDUH /LYLQJVWRQHS WRPRQWKVRIDJHEXWWKHWLPLQJGHSHQGVRQWKH  9RWWHOHU73&RQMRLQHGWZLQVLQSHGLDWULFVXUJHU\th ed. DEQRUPDOLWLHV2QWKHRWKHUKDQGXUJHQWVHSDUDWLRQ &KLFDJR/RQGRQtwin suffering from brain damage. terminal ileum and transverse colon; separating the -3HGLDWU6XUJ bridging vascular system in the pre-sacral region;  0HOLNRJOX0$VODQ$0HWH$2]ND\QDN&,QDQ0DQG separating the fused pelvic bones; and reconstructing .DUDYHOL6$&DVHRIWKRUDFRRPSKDORLVFKLRSDJXVELSXV RIWKHSHOYLVWKHSHOYLVIORRUDQGWKHDQWHULRU FRQMRLQHGWZLQV-3HGLDWU6XUJ abdominal wall. Factors that make this separation  6SLW]/&UDEEH'&*.LHO\(06HSDUDWLRQRIWKRUDFR successful were careful preoperative planning and omphalopagus conjoined twins with complex hepato-biliary GHWDLOHGLQYHVWLJDWLRQUHFRJQLWLRQRISRWHQWLDO DQDWRP\-3HGLDWU6XUJ

384‡Paediatr Indones, Vol. 48, No. 6, November 2008