Rare Association of Semilobar Holoprosencephaly
and Frontonasal Encephalocoele in a
Neonate of Twin Pregnancy
Published: March 1, 2021 | DOI: https://doi.org/10.7860/JCDR/2021/46820.14678
Varsha Rangankar, Anmol Singh, Sanjay Khaladkar
1. Professor, Department of Radiology, Dr DY Patil Medical College, Pune, Maharashtra, India.
2. Junior Resident, Department of Radiology, Dr DY Patil Medical College, Pune, Maharashtra, India.
3. Professor, Department of Radiology, Dr DY Patil Medical College, Pune, Maharashtra, India.
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.
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