Translate this page into:
A rare case of bilateral nasal block
†For correspondence: senthilkumarpgi@yahoo.co.in
This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
This article was originally published by Medknow Publications & Media Pvt Ltd and was migrated to Scientific Scholar after the change of Publisher.
A seven year old male child was referred to the Radiology department of Saveetha Medical College and Hospital, Thandalam, Kanchipuram, India in January 2014, for evaluation of abnormal facies and gradually increasing bilateral nasal block for the last two years. According to parents, perinatal period was uneventful. The child was normal till six months of age, after which facial deformity started appearing. Examination showed prominent forehead, wide set eyes and broad nasal root (Fig. 1A). X-rays and computed tomography (CT) of skull showed marked thickening and sclerosis of all craniofacial bones (Figs 1B, 1C, 2) with dolichocephalic shape of skull. Marked compromise of bilateral nasal cavities was noted due to thickening of nasal bones, nasal septum and wall of paranasal sinuses (Fig. 2). X-rays of bilateral femur showed widened metaphysis with Erlenmeyer flask deformity (Fig. 3). All laboratory investigations were within normal limits except for increase in serum alkaline phosphatase (399 IU/l). Diagnosis of craniometaphyseal dysplasia was made. Surgical correction for bilateral nasal block was advised, however, his parents refused. The patient is being managed conservatively with low calcium diet and calcitriol. There has been no progression of his symptoms till six months of follow up.
-
(A). Clinical picture of the patient showing prominent forehead, broad nasal root (black arrow) and wide set eyes. 1. (B) Anteroposterior and (C) lateral views of skull radiograph showing thickened craniofacial bones with dolichocephalic shape of skull.
-
(A). Oblique axial CT showing markedly thickened bilateral nasal bones (black asterisks), nasal septum (white arrow), and nasal turbinates (black arrows) causing significant compromise of both nasal cavities. Thickened skull bones are also seen (curved white arrow). Thickened maxillary bone is also seen with absence of maxillary sinus cavities (dotted white arrows). 2. (B) Volume rendered CT of the face showing thickened craniofacial bones especially both nasal bones (black arrow).
- X-rays of bilateral femur (anteroposterior view) showing widening of metaphysis (black arrows) with narrow diaphysis (white arrows) giving Erlenmeyer-flask appearance.
Acknowledgment
Authors thank Dr Radha Kumar, Head, Department of Pediatrics, for clinical workup of the patient.