i T i t PiP arasitic Twins - SUNY Downstate Medical...
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P iti T iParasitic Twins
SUNY Downstate Medical CenterSUNY Downstate Medical CenterPediatric Surgery
J i K MDJamie Kang, MD
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CaseCase
A full-term baby girl born to a xx yo healthy female via c-y g y ysectionMaternal history: - P2042- HIV/ Hep B/ VDRL negative, Rubella immuned
prenatal sonogram showed possible meningocele and- prenatal sonogram showed possible meningocele and left lower extremity agenesis
- amniocentesis analysis: 46: XX: gene deletion at locusamniocentesis analysis: 46: XX: gene deletion at locus22 of X chromosome
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CaseCase
Apgars score: 1min – 4, 5 min – 6, 10min – 7pg , ,No spontaneous breathingHR < 100, sat 50%A large defect and a soft tissue mass noted at left lower quadrantImperforate anusImperforate anus2 vessels cord
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CaseCasewww.downstatesurgery.org
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CaseCasewww.downstatesurgery.org
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Pre-op Differential DiagnosesPre op Differential Diagnoses
Cloacal exstrophy MeningomyeloceleMeningomyeloceleIncomplete conjoined twins
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OperationOperation
Part I: Excision of meningocele
Part II: Exploratory laparotomy
Part III: GU system identification
Part IV: Excision of malformed tissue
Part V: End colostomy creation
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Part II: Exploratory Laparotomy
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Part II: Exploratory laparotomyPart II: Exploratory laparotomywww.downstatesurgery.org
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Part II: Exploratory laparotomyPart II: Exploratory laparotomywww.downstatesurgery.org
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Part III: GU system identification
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Part III: GU system identificationPart III: GU system identificationwww.downstatesurgery.org
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Part III: GU system identificationPart III: GU system identificationwww.downstatesurgery.org
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Part IV: Excision of the malformed tissuePart IV: Excision of the malformed tissuewww.downstatesurgery.org
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Part IV: Excision of the malformed tissuePart IV: Excision of the malformed tissuewww.downstatesurgery.org
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Part V: Creation of colostomyPart V: Creation of colostomywww.downstatesurgery.org
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PathologyPathology
Tissue source Final Pathology
Unknown foreign tissue Fragments of skin (scalp), bone and glial tissue
r/o ectopic kidney tissue Fragments of glial vascular tissuer/o ectopic kidney tissue Fragments of glial vascular tissue with squamous epithelium, ependymal lining and papillary structure lined by simple columnarstructure lined by simple columnar epithelium
r/o ectopic adrenal tissue Ectopic adrenal glandFragments of thymus pancreaticFragments of thymus, pancreatic, gastric, small intestine and esophageal tissueFragments of tissue from theFragments of tissue from the respiratory tract
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PathologyPathology
Tissue source Final Pathology
r/o bladder tissue Fragments of glial tissue, cartilage with bronchial type glands and gastrointestinal mucosagastrointestinal mucosa
Distal colon Segment of colon, meconium filledFragments of fat with adjacent fibrous tissue
Meninges Fragments of glial tissue with squamous mucosa
Tooth Tooth
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Post-op CoursePost op Course
Resolving renal failure, not necessitating dialysisg , g yCr improved to 0.6 before being discharged homeSeen by orthopedics surgery for evaluation of left lower extremity prosthesis
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Q ti ?Questions?
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Parasitic TwinsParasitic Twinswww.downstatesurgery.org
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ContentsContentsEarly Human EmbryologyConcept of twinning IntroductionEtiology and pathogenesisEtiology and pathogenesisDiagnosisCase reportsManagementManagementPrognosis Conclusions
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Early Human Embryology (week-1)Early Human Embryology (week 1)
Fertilization Cleavage Blastocyst Formation ImplantantionFertilization Cleavage Blastocyst Formation Implantantion
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Early Human EmbryologyEarly Human Embryologywww.downstatesurgery.org
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Concept Of TwinningConcept Of Twinning
ZygosityChorionicityAmniocity
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Concept of TwinningConcept of Twinning
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Concept of Twinningwww.downstatesurgery.org
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Concept Of TwinningConcept Of Twinning
TwinsTwinsMonozygotic Dizygotic
Dichorionic MonochorionicDiamniotic Monoamniotic
Monochorionic Conjoined twinsDiamniotic
Symmetrical Asymmetrical
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Symmetrical Conjoined Twinswww.downstatesurgery.org
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Conjoined TwinsConjoined Twins
Ischiopagus tripus
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Asymmetrical Conjoined TwinsAsymmetrical Conjoined Twins
Parasitic Twins Fetus-in-fetuParasitic Twins Fetus in fetu
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IntroductionIntroduction
The overall incidence of twinning about 1:87 of all birthsgWide variations in the incidence of dizygotic twinning but constant in monozygotic twinning, 3.5 per 1,000
ipregnanciesMonozygotic twins account for one third of twin births Conjoined twins account for 1% of monozygotic twinsConjoined twins account for 1% of monozygotic twinsParasitic/ heteropagus twins are estimated to account <5% of conjoined twins
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IntroductionIntroduction
Parasitic twins occurs when there is a parasite tt h t i d li t d f hi t t f thattachment in a nonduplicated fashion to any part of the
body of the autositeIncidence 1 in 50,000 to 100,000 live births
M. H. Kaufman, The embryology of conjoined twins. Children’s Nervous System, 2004, 20: 508-525 Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin.
Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39.
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Etiology and Pathogenesis
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Fission or Fusion?Fission or Fusion?
Fission FusionPrimary hypothesisFailure of a single zygote to divide completely
Union of two initially distinct embryos that took place during earlyto divide completely place during early embryonic periodLogrono et al: DNA study demonstrated dizygositydemonstrated dizygosity in a pair of parasitic twins
M. H. Kaufman, The embryology of conjoined twins. Children’s Nervous System, 2004, 20: 508-525. M. H. Kaufman, The embryology of conjoined twins.
Child ’ N S t 2004 20 508 525y , ,
Children’s Nervous System, 2004, 20: 508-525.
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PathogenesisPathogenesis
It is postulated that parasitic twin occur as a result of p pselective ischemic damage in uteroResulting in death and partial resorption of one of the t i d t t i i l t iti t itwins and eventuates in an incomplete parasitic twin attached to a fully developed twinsMechanism maybe similar to that which producesMechanism maybe similar to that which produces acardiac twins
Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin. Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39
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Acardiac TwinsAcardiac Twinswww.downstatesurgery.org
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DiagnosisDiagnosis
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DiagnosisDiagnosis
Prenatalusually by obstetric sonogram
Postnatalcombination of different modalities
Cxray, sonogram, MRI, CT
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Case Reportsp
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Lumbosacral Parasitic Rachipagus (2006)New Delhi, India
A 2.3 kg full term baby girl was born by C-section to g y g yhealthy parentsNo fetal abnormality suspected prenatallyNo h/o maternal illness or drug intake during pregnancyNo h/o congenital anomalies in familyHas three other healthy siblingsHas three other healthy siblingsAt birth, a 11 x 8 cm mass was attached to the lumbosacral region with bony structure underneathg y
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Lumbosacral Parasitic Rachipagus (2006)New Delhi, India
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Lumbosacral Parasitic Rachipagus (2006)New Delhi, India
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Lumbosacral Parasitic Rachipagus (2006)New Delhi, India
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Asymmetric Conjoined Twins: Atypical I hi P it (2007)Ischiopagus Parasite (2007)Chandigarh, India
A 2.7kg full-term baby girl was born to a young healthy woman via normal vaginal deliveryNo h/o maternal illness or drug intake during pregnancyNo h/o maternal illness or drug intake during pregnancyNo h/o congenital anomalies in familyAt birth, a parasitic twin was found attached to her leftAt birth, a parasitic twin was found attached to her left lumbar regionAlso found to have a major exomphalos with a t t d th hi h ti b ltransparent sac, underneath which necrotic bowel was visible
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Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite (2007)Ischiopagus Parasite (2007)Chandigarh, India
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Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite (2007)Ischiopagus Parasite (2007)Chandigarh, India
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Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite (2007)Ischiopagus Parasite (2007)Chandigarh, India
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Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite (2007)Ischiopagus Parasite (2007)Chandigarh, India
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ManagementManagementwww.downstatesurgery.org
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ManagementManagement
Recognition Xray, sonogram, CT, MRI
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Management
U lik t i l j i d t i iti t i ftUnlike symmetrical conjoined twins, parasitic twins often do not shared major organsThus surgical separation for parasitic twins is less complicatedcomplicated
Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin. Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39.
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PrognosisPrognosis
The outcome of the autosite after surgical intervention is gusually favorableNo occurrence of recurrence
Nisreen M Khalifa, Doaa W Maximous, Alaa A Abd-elsayed. Department of Pediatric Oncology/ Surgical oncology/ Public Health and Community Medicine, South Egypt Cancer Institute, Asdiut University, Egypt. Fetus in fetu: A case report. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2253549, January 10, 2008.p p g g , y ,
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ConclusionsConclusions
Parasitic twins are rare, account for less than 5% of ,conjoined twinsTheory of “fission” or “fusion”Occur as a result of selective ischemic damage in uteroPrenatal diagnosis is possible by sonogram Surgery is the mainstay treatment to separate theSurgery is the mainstay treatment to separate the parasite from the autosite, less complicated than conjoined twins separationThe prognosis of the autosite after surgical intervention is excellent
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References
1. Phillippe Jeanty, Kay Caldwell, Patricia Dix. Department of Radiology, Vanderbilt University. Fetus-in-fetu. http://www.thefetus.net/page.php?id=290, December 6, 2001.p p g p p , ,
2. Nisreen M Khalifa, Doaa W Maximous, Alaa A Abd-elsayed. Department of Pediatric Oncology/ Surgical oncology/ Public Health and Community Medicine, South Egypt Cancer Institute, Asdiut University Egypt Fetus in fetu: A case reportAsdiut University, Egypt. Fetus in fetu: A case report. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2253549, January 10, 2008.
3. 22q11.2 deletion syndrome. http://en wikipedia org/wiki/22q11 2 deletion syndromehttp://en.wikipedia.org/wiki/22q11.2_deletion_syndrome
4. Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin. Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39.
5. Rajiv Chadha, Prabha Lal, Dharmendra Singh, Akshay Sharma, S. Roy Choudhury, Lumbosacral parasitic rachipagus twin. Journal of Pediatric Surgery, volume 41, 2006, pages e45-e48.
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ReferencesReferences
1. J.K. Mahajan, Kumar Devendra, Deb Mainak, and K.L.N Rao, Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite. Journal of Pediatric Surgery. Volume 37, October 2002, page e33.
2. M. H. Kaufman, The embryology of conjoined twins. Children’s Nervous System, 2004, 20: 508-525.
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AssessmentAssessment www.downstatesurgery.org
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Question 1Question 1
Which of the following is true?g
(a) Fusion is the primary theory for conjoined twins(b) The latter the cleavage, the higher chance of conjoined
embryos in early human embryology( ) Asymmetrical conjoined twins are more common than(c) Asymmetrical conjoined twins are more common than
symmetrical conjoined twins(d) Separation surgery is more complicated in ( ) p g y p
asymmetrical conjoined twins
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Question 2Question 2
What is the estimated incidence of parasitic twins?p
(a) 1 in 100 live births(b) 1 in 1000 live births(c) 1 in 10,000 live births(d) 1 in 100,000 live births
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Question 3Question 3
What is the postulated mechanism of parasitic twins?p p(a) Maternal malnutrition(b) Maternal trauma(c) Fetal neoplastic growth(d) Twins Reversed Arterial Perfusion syndrome in acardic
twinstwins
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Question 4Question 4
Which is the most common type of conjoined twins?yp j(a) Thoragopagus(b) Craniopagus(c) Pygopagus(d) Omphalopagus
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Question 5Question 5
What is the recurrence rate for parasitic twins after surgical p gIntervention?(a) None(b) < 2%(c) 10%
%(d) 50%
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