Rare Association of Semilobar Holoprosencephaly
and Frontonasal Encephalocoele in a
Neonate of Twin Pregnancy
TD01-TD03
Correspondence
Anmol Singh,
Apartment 701, Blue Bells-1 Building, Sukhwani Campus,
Vallabh Nagar, Pimpri Colony, Pune, Maharashtra, India.
E-mail: anmolsinghmzn2009@gmail.com
Holoprosencephaly is a rare intracranial malformation seen in newborns, resulting from a failure of separation of the forebrain into separate cerebral hemispheres. It is divided into three subtypes-alobar, semilobar and lobar. Encephalocele is a rare neural tube defect characterised by a defect in the cranial vault with herniation of brain parenchyma along with the overlying meninges. Author has reported a case of a male neonate born of a twin pregnancy to a 29-year-old primigravida presenting with a midline swelling in the frontal region. On imaging studies, the patient was diagnosed with semilobar holoprosencephaly and frontonasal encephalocele with dural arteriovenous fistula. Holoprosencephaly and encephalocoele are important congenital malformations; however association of the two conditions has been rarely described in the literature.