rare case in congenital anomalies

1. What is Cranio-cephalopagus?
AHMED ZAKI, MRCOG
Head of OB/GYN Department, Bakhsh Hospital
Chairman of Scientific Committee

2. Twins (MZ/DZ)

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.

5. Conjoined Twins

12. Double-headed monsters (1:85 conj
twins)

13. With introduction of high-resolution imaging, accurate
prenatal diagnosis of conjoined twins is possible early in
pregnancy

15. cephalopagus

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

20. Delivery

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Ύόϼϭ ϟ έέ ·ΕΎΤΗ Χ
Θ ϟ

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

41. Never take yourself for granted