Abstract
Purpose
This paper reviews the plausible etiological mechanisms, clinical features, preoperative analysis, and documented modern-day craniopagus parasiticus surgical separation attempts as well as an historical review of the few cases documented in the literature.
Methods
We describe the successful separation of a 28-week preterm newborn from its parasite sibling twin bearing lethal congenital anomalies associated to Cantrell’s pentad and sirenomelia. Description of the case, plausible explanations on the mechanisms of conjointment along with the associated congenital abnormalities of the deceased twin are examined along with an historical revision of craniopagus parasiticus and their separation attempts with special attention to the previously undocumented attempt of the Dominican CP separation surgery by Lazareff et al.
Results
The use of the deceased twin cranial vault tissues (skin, bone, and duramater) as an autologous implant due to the identical genetical profile served to remodel and close the skull of the surviving twin with good esthetic results and no tissue rejection. To our knowledge, this is the youngest preterm set of craniopagus parasiticus separated in an emergency fashion with good functional and esthetic outcome.
Conclusions
Craniopagus parasiticus is an infrequent subvariant of this rare form of twin conjointment which may require urgent separation due to the associated malformations of the parasitic twin; therefore, the fact that both siblings are genetically identical may prove as an advantage to use duramater, bone, and soft tissues from the parasitic twin as ideal grafts for covering the resultant defect after the separation has been performed.
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Acknowledgements
The authors would like to express deep gratitude to Dr. Jorge Lazareff for providing his comments and imaging studies from the previously unpublished first modern-day neurosurgical attempt of craniopagus parasiticus separation which took place in the Dominican Republic in 2004.
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Caceres, A., Segura-Masis, J.L., Caceres-Alan, A. et al. Craniopagus parasiticus: successful separation of a 28-week preterm newborn from parasite sibling twin bearing lethal congenital anomalies associated to Cantrell’s pentad and sirenomelia—case-based review of the literature. Childs Nerv Syst 37, 2139–2146 (2021). https://doi.org/10.1007/s00381-021-05179-8
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DOI: https://doi.org/10.1007/s00381-021-05179-8