Rare Association of Bilateral Anotia with Congenital Facial Palsy

Prem Lochan Prasad,Ruchika Bhatnagar

doi:10.3126/jnps.v35i2.13361

Abstract

Anotia is a birth defect and is associated with facial palsy and congenital heart defect as various presentation of cardiofacial syndrome. Herein we report a case of bilateral anotia and unilateral congenital facial palsy without congenital heart disease. It may occur due to single gene abnormality, teratogenic effect of maternal ingestion of thalidomide, retinoic acid, misoprostol or maternal diabetes, dietary lack of carbohydrates and folate.J Nepal Paediatr Soc 2015;35(2):195-197

Highlights

Ano a or micro a, defined as absent or miniature external ears, is known to be a feature of cardiofacial syndrome and seen to be associated with various other congenital anomalies like renal anomalies, short stature,small patella syndrome Unilateral atresia is more common Congenital facial palsy with bilateral ano a has not been seen so o en
It is generally considered to be either developmental or acquired in origin Developmental facial paralysis is associated with other anomalies including those of pinna and external auditory canal, ranging from mild defects to severe micro a and atresia[1]
MRI Brain was done to evaluate the cause for cong. facial palsy but, normal study warranted the need for HRCT Temporal bone which showed agenesis of petrous part of right sided temporal bone along with agenesis of facial and auditory nerve on right side only, non-visualiza on of bilateral external ears, 1Dr

Summary

Introduction

Ano a or micro a, defined as absent or miniature external ears, is known to be a feature of cardiofacial syndrome and seen to be associated with various other congenital anomalies like renal anomalies, short stature,small patella syndrome Unilateral atresia is more common Congenital facial palsy with bilateral ano a has not been seen so o en. A late preterm male with gesta onal age to 36 weeks and four days, a product of non-consanguineous marriage was delivered normally and cried soon a er birth. His general physical examina on was conspicuous by bilateral absence of external ear which was replaced by preauricular skin tag on the right side only (Figure 1). Facial palsy but, normal study warranted the need for HRCT Temporal bone which showed agenesis of petrous part of right sided temporal bone along with agenesis of facial and auditory nerve on right side only, non-visualiza on of bilateral external ears, 1Dr. Ruchika Bhatnagar, MBBS, MD, Assistant Professor, Department of Paediatrics, 2Dr. Prem Lochan Prasad, MBBS, MD, Professor and Head. Hearing assesment done through BERA at the me of discharge revealed mixed type of hearing loss in right ear unlike conduc ve hearing loss alone in le ear

Discussion

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Conclusion