Berry-Treacher Collins Syndrome With Congenital Bell's Palsy and Unilateral Anotia: Tongue-Tie Release Under General Anesthesia

Abstract

The inherited disease of unilateral anotia and ipsilateral Bell's palsy is exceedingly uncommon, but it has a few other clinical manifestations. The prevalence of anotia in combination with congenital Bell's palsy is well-known by Berry-Treacher Collins and Goldenhar syndrome. Despite the prevalence of anotia in combination with Bell's palsy, there have been relatively very few case reports about the corresponding conditions in India. The aim of the paper is to discuss the anesthesia plan for a seven-year-old boy who underwent surgery for tongue-tie release.

Keywords: anotia; berry-treacher collins syndrome; congenital bell’s palsy; difficult airway; general anesthesia; goldenhar syndrome; shared airway; tongue – tie release.

Figure 1. Physical characteristics of Bell's palsy

Figure 2. CT scan for identification of lack of pneumatized mastoid and missing left ear

(A): the image shows a lack of pneumatized mastoid air cells, left lateral canal vestibular deformity (LCVD), and anomalous left facial never course. (B): the image shows a complete absence of the left ear, missing anterior and posterior mandibular bones on OPG, left EAC atresia (Altmann’s Group 3, Schuknecht Type D), hypoplastic left middle ear cavity with soft tissue and wall erosion, absent left middle ear ossicular system, and absent left cardio canal CT: computed tomography; OPG: orthopantomogram; EAC: external auditory canal