A Rare Case Report of Total Aplasia of Paranasal Air Sinuses with Posterior Fossa Malformation (Dandy Walker)

ABSTRACT

Background: Total aplasia of paranasal air sinuses is a very rare condition. Dandy Walker malformation is the most common posterior fossa malformation involving cerebellum and IV ventricle. We present herewith a rare case of aplasia of paranasal sinuses with Dandy Walker malformation. There is no parallel case reports of these combined anomalies found in one patient available to our knowledge. Case report A mentally retarded 7 years old male child was referred for CT scan study of P.N.S. and Brain. The C.T. findings of P.N.S. and Brain showed aplasia of all paranasal air sinuses and characteristic of Dandy Walker malformation such as Hypoplasia of cerebellar vermis, Dilated IV ventricle, Mega cistern magna etc. Conclusions: Total aplasia of paranasal sinuses is a very rare condition. Computed Tomography (CT) scan remains the gold standard in revealing anatomic details of paranasal sinuses. Dandy-Walker malformation is a rare congenital anomaly of posterior fossa involving cerebellum and IVth ventricle. Treatment depends upon the severity of abnormality and status of patient at the time of its detection.