Conjoint twins - case n review

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1. Lord Brahma – Hindu mythology 2. “SIAMESE TWINS” 3.  Conjoint twins born in Siam ‘Siamese twins’ 1811 -1874 Considered inoperable Lived…
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  • 1. Lord Brahma – Hindu mythology
  • 2. “SIAMESE TWINS”
  • 3.  Conjoint twins born in Siam ‘Siamese twins’ 1811 -1874 Considered inoperable Lived without separation Fathered 21 children Lived life as ‘entertainers’ Died at the age of 63
  • 4.  Among the 13,418 consecutive stillborns surveyed, 6 cases of conjoined twins were identified giving a frequency of 44.72 per 100,000. 11 pairs were identified among the 2,425,583 total live births, a frequency of 0.45 per 100,000. The frequency among stillborn infants is 99.34 times higher than that observed among live births1 Martinez-Frias et al. J Pediatr Surg. 2009
  • 5. Thoraco-omphalopagus
  • 6.  Found to be conjoint (Thoraco-Omphalopagus) intra- op during Cesarean section T1 required bag & mask for 30 seconds Respiratory distress in both soon after birth Both put on NPCPAP CXR at CLR No sharing of bony chest wall USG at CLR did not reveal any organ sharing : 2 distinct livers and hearts.
  • 7.  Shifted to Pediatric surgery at 6 hrs of life Planned for elective surgery Were taken off Nasopharyngeal CPAP on D2 and kept on free flow oxygen. Continued to have minimal distress. T1 was diagnosed to have # of shaft of left femur. Ortho advised conservative Mx. Started on T/F which were gradually being hiked.
  • 8.  Planned for CECT. PAC obtained. Had worsening in the form of not maintaining saturation on D3. Intubated and put on manual IPPR and then on SIMV mode of ventilation. Cause kept as: ?Pneumonia ? Aspiration syndrome ?HMD. Antibiotics upgraded. Worsening clinical course. Suffered cardiac arrest on D6 of life from which the babies could not be revived.
  • 9. Twin 1 Twin 2 Dextrocardia  Levocardia AV-VA concordance  Single ventricle 6 mm displacement of  Single AV valve STL towards RV   No PS/PDA EBSTEIN’S ANAMOLY  8 mm OS ASD with LR Mild TR shunt No  Malposed Great vessels ASD/VSD/PDA/LVOTO/ RVOTO No vessel / Chamber sharing seen between twins
  • 10. DiscussionSaptharishi L G
  • 11.  Incomplete embryonic division occurs late At around day 13 - 14 of conception1 Chorion and amnion have already formed by that time Conjoint twins  monochoionic monoamniotic twinsKaufman MH. The embryology of conjoined twins. Childs Nerv Syst. 2004
  • 12.  The Fission theories  Well established  Numerous studies  The “Fusion” theory1&2  Animal model in Triton embryos + study of more than 1800 reported cases of conjoint twins  Fusion – ventral or dorsal: at sites of absence of ectodermSpencer. Theoretical and analytical embryology of conjoined twins. Clin Anat 2000
  • 13. Points against Points for "the same parts are  No theoretical "fission" of the vertebrate embryo at always united to the any stage of development, same parts“ in any plane, in any direction can explain Ectoderm is non-sticky (1)the selection of the Zona pellucida: observed sites of fusion hydrophobic (2)the details of the union (3) the limitation to the specific areas in which the twins are found to be joined.
  • 14.  Factors that induce calcium depression and delayed implantation encourage uniovular duplication in general and CJ twinning in particular1. Steinman G. Mechanism of twinning.V. J Reprod Med 2002
  • 15.  Very rare 1 in 50,000 to 100,000 births Indian incidence ( 1 in 50,000) Note : Live born twins are rare as most of them get spontaneously terminated in utero
  • 16.  Broad categories:  Ventral fusion: 87%  Dorsal fusion: 13% Most common sites of fusion1:  Thoraco/omphalopagus : 73%  Pyopagus 19%  Ischiopagus 6%  Craniopagus 2%Hoyle RM. Surgical separation of conjoined twins. Surg Gynecol Obstet. 1990
  • 17. UK study Spanish study Thoraco-omphalopagus  Thoracopagus (58.82%) (28%)  Diprosopus (11.7%) Thoracopagus (18.5%) Omphalopagus (10%) Parasitic twins (10%) Craniopagus (6%) Kaufman MH. The embryology of conjoined twins. Childs Nerv Syst. 2004 Martinez-Frias et al.J Pediatr Surg. 2009
  • 18.  Embryological Classification* Ventral Union (87%)  Rostral (48%) Cephalopagus (11%, top of head to umbilicus) Thoracopagus (19%, conjoined heart) Omphalopagus (18%, including lower thorax)  Caudal (11%)  Ischiopagus (lower abdomen and genitourinary system)  Lateral (28%) Parapagus (pelvis and variable trunk) Dorsal Union (13%)  Craniopagus (5%, cranial vault) Rachipagus (2%, vertebral column) Pygopagus (6%, sacrum) *Adapted from Spencer
  • 19.  Conjunction never involving heart or umbilicus:  Craniopagus: Cranial union only, about 2% of all conjoined twins.  Pygopagus: Posterior union of the rump, about 19% of all conjoined twins. Rare forms of conjoined twins, having different patterns:  Parasitic twins: Asymmetrical conjoined twins, one twin being small, less formed, and dependent upon the other.  Fetus in fetu: Situation in which an imperfect fetus is contained completely within the body of its sibling.
  • 20.  Conjunctions always involving the umbilicus:  Thoracopagus: Anterior union of the upper half of the trunk. The most common form of conjoined twins (about 35%), it always involves sharing the heart.  Cephalopagus: Anterior union of the upper half of the body with two faces on opposite sides of a conjoined head. Extremely rare. The heart is sometimes involved. A combination of types 3 and 4 is called cephalothoracopagus.  Parapagus:(sometimes balled diprosopus): lateral union of the lower half, extending variable distances upward, about 5% of all conjoined twins. Heart sometimes involved.  Ischopagus: Anterior union of the lower half of the body, about 6% of all conjoined twins. Heart not involved.  Omphalopagus: Anterior union of the midtrunk, about 30% of conjoined twins.
  • 21.  Female siblings seem to have a better shot at survival than their male counterparts. Although more male twins conjoin in the womb than female twins, females are three times as likely as males to be born alive. Approximately 70 percent of all conjoined twins are girls. Overall female conjoint twins 3 to 10 times more common than male conjoint twins. Why?? University of Maryland Medical centre website.
  • 22.  Early as the 12th week of gestation on prenatal USG Suspicious features  Lack of a separating membrane between the twins  Iinability to separate fetal bodies and skin contours  Constant position of the fetal heads. USG ,ECHO- at 18 to 20 weeks - anatomy of the shared organs, associated anomalies, and cardiac status Serial scans  to accurately define the extent and nature of the union using CT and 3-dimensional volume rendering or ultrafast magnetic resonance imaging Monitor for polyhydramnios or hydrops, (50% of cases)**Spielmann AL MRI of conjoined twins J Comput Assist Tomogr. 2001
  • 23.  Polyhydraminos (50%)  Requiring amnioreduction Conjoint twins are at a risk for significant congenital anamolies. Look for them actively.  Numerous case reports where such anamolies were picked up early and pregnancy terminated Expect difficulties in delivery and resuscitation Based on the type of conjoint twins, the pediatric surgeon must have a fair idea of the organs they are likely to share
  • 24.  Prenatal MRI  Exact details of organ sharing  Planning EXIT procedure / immediate separation Elective Cesarean section close to term Experienced team of Obstetricians, Neonatologists, Anesthetists, Cardiologists, CTV surgeons and Pediatric surgeons Full team effort with co-ordination
  • 25.  EXIT procedure (Ex-utero Intrapartum Treatment) Difficulty in Airway management Problems with providing positive pressure ventilation  B&T superior to B&M All drugs to be calculated on combined weight (100% conjoint twins share circulation*. So ??) Index case did not share circulation. Remember: If one of them dies, the only way to save the other is * to separate them immediately.
  • 26.  Management  Prenatal & Postnatal Prenatal:  Elective termination is recommended where there is cardiac or cerebral fusion. (Only 2 successful separations of conjoined hearts)  Elective interruption of the pregnancy particularly when the anticipated severity of deformity following separation would be unacceptable Postnatal: Emergent separation Vs Elective separation Absolute indication for emergent separation  Death of one of the twins  One twin has a major anamoly incompatible with life Elective:  2 to 4 months of age  Advantages
  • 27.  Choice of imaging study will depend on the area of union. For thoraco-omphalopagus twins:  Essential investigations – ECHO, CT & MRI.  Where the livers are fused, it is important to document the presence of separate gall bladders and hepatic veins.  Not possible to define biliary anatomy before separation and this should be addressed during the procedure.  Gastrointestinal contrast studies and angiography have not been helpful.
  • 28.  Two sets of anesthesiologists Essential monitoring - arterial and central venous catheters, electrocardiogram, pulse oximetry, capnography, and urinary output. Regular blood gas analyses to be undertaken throughout the procedure. All drugs and intravenous fluids calculated on a total weight basis cross-circulation  drugs given intravenously have an unpredictable effect. Particular care is essential when administering drugs such as opioids, which should be given incrementally.
  • 29.  ‘Swab technique’ ?! As per plan: based on imaging investigations Unexpected findings are not uncommon and the operative plan may have to be varied accordingly Assignment of organs, such as intestine, will be equal unless 1 twin is nonviable. In ischiopagus, parapagus, and pygopagus twins, urological anatomy is often complex with 1 ureter from each twin frequently crossing to enter the contralateral bladder.
  • 30. Type Degree of fusion SeparabilityI No significant fusion EasyII Fusion of the great vessels EasyIII Atrial fusion PossibleIIIa Mirror image right atrial Possible fusionIIIb Other type of atrial fusion PossibleIV Atrioventricular fusion Not possibleV Single heart in one of the Not possible twins
  • 31.  Following prolonged operative procedures, it is necessary to electively paralyze and mechanically ventilate the infants for 48 to 72 hours postoperatively. The infants require meticulous monitoring in the intensive care unit, paying particular attention to cardiac underperformance (poor cardiac output). Fluid and electrolyte replacement should be accurately administered as there will be huge losses when large prosthetic closure has been used. Strict infectious precautions must be exercised to avoid sepsis, particularly where there are large skin defects.
  • 32.  Survival rare when there is cardiac or cerebral fusion One case series of conjoint twins:  28% died in utero  54% died immediately after birth  18% survived Hoyle et al analyzed all attempts at surgical separation until 1987 and found:  Surgical separation attempted on 167 occasions  Overall survival – 64%  Mortality among various subgroups: Thoraco (51%), cranio (48%) and omphalo (32%). Mortality with ischio (19%) and pyo (23%) was lower.  Mortality 70% for emergent procedures and 20% for electiveHoyle RM. Surgical separation of conjoined twins. Surg Gynecol Obstet. 1990
  • 33. Major outcome Spitz and Kiely. JAMA 2004
  • 34.  Whether to sacrifice one for the other??
  • 35. Is conjoint status more physiological than separation??
  • 36. Why are there very fewreported cases of conjoined triplets or quadruplets?
  • 37.  Kendra and Maliyah Herrin >>>>>http://www.youtube.com/watch?v=5gn 2009HTtcxoPA
  • 38. • Loice and Christine >>>>>BEFORE: Two girls wereconnected from the breastbone to the navel, and shareda liver and a main bloodvessel that connected theirhearts. 2002
  • 39. Group member:CHENG Tsz Yan 6S (2)TONG Carmen 6S (23)
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