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What is Cranio-cephalopagus?




AHMED ZAKI, MRCOG
Head of OB/GYN Department, Bakhsh Hospital
Chairman of Scientific Committee
Twins (MZ/DZ)
Conjoined twins

 • Are rare and complex complication of
   monozygotic twinning, which is associated with
   high perinatal mortality. (1:100,000 deliveries)

 • Represent a unique challenge to pediatric surgeons
   and radiologists.

 • Each set of conjoined twins is unique. An imaging strategy to
   accurately define anatomic fusion, vascular anomalies, and
   other associated abnormalities is important for surgical
   planning and prognostic information.
Such twins are classified according to
the site of connection:

 • the thorax (thoracopagus)
 • abdomen (omphalopagus),
 • sacrum (pygopagus),
 • pelvis (ischiopagus),
 • skull (craniopagus),
 • brain (cephalopagus),
 • or back (rachipagus).

 • Cephalopagus is a very rare form of conjoined
   twins.
Conjoined Twins
Double-headed monsters (1:85 conj
twins)
With introduction of high-resolution imaging, accurate
prenatal diagnosis of conjoined twins is possible early in
pregnancy
cephalopagus
History

 • 28 Y, G3P2. H/O consanguinity
 • 1st delivery SVD (normal girl)
 • 2nd delivery , preterm with multiple cong. Abns
   died at birth. This was followed by PPH, DVT
 • This pregnancy, booked at 10 weeks
 • At 22 Weeks, she had an anomaly scan which
   showed no abnormalities ,
 • At 34 weeks, polyhydramnios..GTT..Diabetic..Diet
   control (her Bl. Gluc was always within normal)
•   At 36 weeks, she came in labour
•   Her CTG showed typical picture of fetal distress
•   & the os was 3 cm dilated, head floating
•   She was taken to theatre for C/S
Delivery
:ήθ ϨθΦέΎ
         ϟϳ Φ Η                                              :ϰϟ
                                                               ·
1 1429/11/16ΔόϤ ϟ ήϗ ϟ
ϫ              ΠΠ  ϮϤή            Γ Ϲ β β Ϡβ β έ /Γ Ύ
                                   έέΩΩ Πϣ β
                                     ϴ                Ω όγ   -
  ϡ2008/11/14 Í ϟ
                     ϮϤή
                   ήϗ ϟ       Γ Ϲ β β Ϡβ ϧβ ΐ Ύ/Γ Ύ
                                έέΩΩ Πϣ βέΐ Ύ Ω
                                  ϴ                     όγ   -
                    ϮϤή
                  ήϗ ϟ      Γ ΩΩ β Ϡβ β έΪϋΎ ϣ /Γ Ύ
                             έέ Ϲ β Πϣ β δ
                                ϴ                     Ω όγ   -
                    ϮϤή
                  ήϗ ϟ                  ϡόϟ ΐΐήϤή /Γ Ύ
                                         Ύόΐ     ϟ
                                                 ϗ Ω    όγ   -
                    ϮϤή
                  ήϗ ϟ          ήή ϟϳ ς ϴ Θ ήή Ϯ ϭΩ
                                  ήτΘ τΨϟ Ϊ /Γ Ύ
                                      Θ        Θ        ϳϣ
                                                        όγ   -
  2968 :Ω ϟ Ϣ
        Ϊόό ϗέ      ϮϤή
                  ήϗ ϟ                   ΓΎ Ϸ Βϛ Ω
                                            Β σήήϴ Ύ
                                            σ       /Γ όγ    -     29 :Δ ϔ ϟ
                                                                        Τμ μ
                    ϮϤή
                  ήϗ ϟ                  ϱ έέΩΩ ΪϳϤ /Γ Ύ
                                             ϟ
                                             Ϲ ήή ΪΪ Ω  όγ   -
                  Ϧϳ ήϗ ϟ ζ Ψ .Ω ϴ δ ϣϡ ϋϋϋέΪϣ/Γ Ύϟ -
                      ϮϤή    Α ΕΎϔθΘ ϮϤ         Ω δ
                                                 δ




                           ϰϟϷ Δ μ ϟήΒ ϟϔϟ έΎ Ϲ
                            ϭ ΤΤ Ύ ΨΨ ·Γ η η
                                    Α    ϰ




              Δ ΎόΕΎ όϗ ϖϳϮδΘ Γ Ω ϴ κ κΎϊ ϣ
               ϣόϟ Ύ ϟ
                    Ύόϼϭ    ϟ έέ ·ΕΎΤΗ Χ
                             Θ      ϟ
Diagnosis

 • Cranio-Cephalopagus twins are misdiagnosed as
   a singleton pregnancy because of the extreme
   degree of fusion which makes accurate
   demonstration of the abnormalities very difficult

 • Ultrafast magnetic resonance imaging (MRI) has
   been used successfully in antenatal diagnosis.

 • ITS TOO RARE TO BE MIS S ED
• Ultrafast MR imaging can provide image quality superior to
  two dimensional ultrasonography and should be considered
  an adjunct to ultrasound for antenatal characterization of
  some anomalies.



• where 3D imaging was used indicates that this modality
  does not improve on the diagnosis made by 2D ultrasound.
  Overall, very early prenatal diagnosis and first-trimester 3D
  imaging provide very little additional practical medical
  information compared to the 11-14 weeks' ultrasound
  examination.
•
Diagnosis

 • Although first-trimester diagnosis of conjoined
   twins is feasible, false-positive cases are common
   before 10 weeks because, earlier in gestation,
   fetal movements are limited and monoamniotic
   twins may appear conjoined.
Outcome
• As most parents opt for immediate
  termination of pregnancy at confirmation of
  the diagnosis, there are limited data on the
  prenatal follow-up of conjoined twins.

• When the parents opt for conservative
  management, half of the fetuses die in utero
  and another 44% will die during the neonatal
  period.
• The prognosis for cephalopagus twins is
  extremely poor because surgical separation is not
  an option. Thus early prenatal diagnosis of
  cephalopagus twins is important to provide an
  opportunity for pregnancy termination if desired.
• I am presenting an extremely rare case of
  cephalo-carniopagus



• To the best of my knowledge, this is an extremely
  rare case in the whole literature
Never take yourself for granted

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cranio-Cephalopagus

  • 1. What is Cranio-cephalopagus? AHMED ZAKI, MRCOG Head of OB/GYN Department, Bakhsh Hospital Chairman of Scientific Committee
  • 3. Conjoined twins • Are rare and complex complication of monozygotic twinning, which is associated with high perinatal mortality. (1:100,000 deliveries) • Represent a unique challenge to pediatric surgeons and radiologists. • Each set of conjoined twins is unique. An imaging strategy to accurately define anatomic fusion, vascular anomalies, and other associated abnormalities is important for surgical planning and prognostic information.
  • 4. Such twins are classified according to the site of connection: • the thorax (thoracopagus) • abdomen (omphalopagus), • sacrum (pygopagus), • pelvis (ischiopagus), • skull (craniopagus), • brain (cephalopagus), • or back (rachipagus). • Cephalopagus is a very rare form of conjoined twins.
  • 6.
  • 7.
  • 8.
  • 9.
  • 10.
  • 11.
  • 13. With introduction of high-resolution imaging, accurate prenatal diagnosis of conjoined twins is possible early in pregnancy
  • 14.
  • 16.
  • 17. History • 28 Y, G3P2. H/O consanguinity • 1st delivery SVD (normal girl) • 2nd delivery , preterm with multiple cong. Abns died at birth. This was followed by PPH, DVT • This pregnancy, booked at 10 weeks • At 22 Weeks, she had an anomaly scan which showed no abnormalities , • At 34 weeks, polyhydramnios..GTT..Diabetic..Diet control (her Bl. Gluc was always within normal)
  • 18. At 36 weeks, she came in labour • Her CTG showed typical picture of fetal distress • & the os was 3 cm dilated, head floating • She was taken to theatre for C/S
  • 19.
  • 21. :ήθ ϨθΦέΎ ϟϳ Φ Η :ϰϟ · 1 1429/11/16ΔόϤ ϟ ήϗ ϟ ϫ ΠΠ ϮϤή Γ Ϲ β β Ϡβ β έ /Γ Ύ έέΩΩ Πϣ β ϴ Ω όγ - ϡ2008/11/14 Í ϟ ϮϤή ήϗ ϟ Γ Ϲ β β Ϡβ ϧβ ΐ Ύ/Γ Ύ έέΩΩ Πϣ βέΐ Ύ Ω ϴ όγ - ϮϤή ήϗ ϟ Γ ΩΩ β Ϡβ β έΪϋΎ ϣ /Γ Ύ έέ Ϲ β Πϣ β δ ϴ Ω όγ - ϮϤή ήϗ ϟ ϡόϟ ΐΐήϤή /Γ Ύ Ύόΐ ϟ ϗ Ω όγ - ϮϤή ήϗ ϟ ήή ϟϳ ς ϴ Θ ήή Ϯ ϭΩ ήτΘ τΨϟ Ϊ /Γ Ύ Θ Θ ϳϣ όγ - 2968 :Ω ϟ Ϣ Ϊόό ϗέ ϮϤή ήϗ ϟ ΓΎ Ϸ Βϛ Ω Β σήήϴ Ύ σ /Γ όγ - 29 :Δ ϔ ϟ Τμ μ ϮϤή ήϗ ϟ ϱ έέΩΩ ΪϳϤ /Γ Ύ ϟ Ϲ ήή ΪΪ Ω όγ - Ϧϳ ήϗ ϟ ζ Ψ .Ω ϴ δ ϣϡ ϋϋϋέΪϣ/Γ Ύϟ - ϮϤή Α ΕΎϔθΘ ϮϤ Ω δ δ ϰϟϷ Δ μ ϟήΒ ϟϔϟ έΎ Ϲ ϭ ΤΤ Ύ ΨΨ ·Γ η η Α ϰ Δ ΎόΕΎ όϗ ϖϳϮδΘ Γ Ω ϴ κ κΎϊ ϣ ϣόϟ Ύ ϟ Ύόϼϭ ϟ έέ ·ΕΎΤΗ Χ Θ ϟ
  • 22.
  • 23.
  • 24.
  • 25.
  • 26.
  • 27.
  • 28.
  • 29.
  • 30.
  • 31.
  • 32.
  • 33.
  • 34. Diagnosis • Cranio-Cephalopagus twins are misdiagnosed as a singleton pregnancy because of the extreme degree of fusion which makes accurate demonstration of the abnormalities very difficult • Ultrafast magnetic resonance imaging (MRI) has been used successfully in antenatal diagnosis. • ITS TOO RARE TO BE MIS S ED
  • 35. • Ultrafast MR imaging can provide image quality superior to two dimensional ultrasonography and should be considered an adjunct to ultrasound for antenatal characterization of some anomalies. • where 3D imaging was used indicates that this modality does not improve on the diagnosis made by 2D ultrasound. Overall, very early prenatal diagnosis and first-trimester 3D imaging provide very little additional practical medical information compared to the 11-14 weeks' ultrasound examination. •
  • 36. Diagnosis • Although first-trimester diagnosis of conjoined twins is feasible, false-positive cases are common before 10 weeks because, earlier in gestation, fetal movements are limited and monoamniotic twins may appear conjoined.
  • 37. Outcome • As most parents opt for immediate termination of pregnancy at confirmation of the diagnosis, there are limited data on the prenatal follow-up of conjoined twins. • When the parents opt for conservative management, half of the fetuses die in utero and another 44% will die during the neonatal period.
  • 38. • The prognosis for cephalopagus twins is extremely poor because surgical separation is not an option. Thus early prenatal diagnosis of cephalopagus twins is important to provide an opportunity for pregnancy termination if desired.
  • 39. • I am presenting an extremely rare case of cephalo-carniopagus • To the best of my knowledge, this is an extremely rare case in the whole literature
  • 40.
  • 41. Never take yourself for granted