Newborn with congenital facial palsy and bilateral anotia/atresia of external auditory canal: Rare occurrence.

Congenital facial palsy (CFP) is clinically defined as facial palsy of the seventh cranial nerve which is present at birth or shortly thereafter. It is generally considered to be either developmental or acquired in origin. Facial palsy of developmental origin is associated with other anomalies including those of pinna and external auditory canal, which range from mild defects to severe microtia and atresia. We report a 2-day-old male newborn that had right CFP with bilateral anotia and atresia of external auditory canals which is rare.

Introduction

Congenital facial palsy (CFP) is clinically defined as facial palsy of the seventh cranial nerve which is present at birth or shortly thereafter. It is generally considered to be either developmental or acquired in origin.[1] Facial palsy of developmental origin is associated with other anomalies including those of pinna and external auditory canal, which range from mild defects to severe microtia and atresia.[2] Acquired causes of facial palsy include perinatal trauma, intrauterine posture, and intrapartum compression. Here, we report a 2-day-old male newborn that had right CFP with bilateral anotia and atresia of external auditory canals which is rare.

Case Report

A 2-day-old male newborn born out of nonconsanguineous parentage, full term normal vaginal delivery, who cried immediately after birth, was found to have facial asymmetry with absent ears. The child was unable to close his right eye with the reduced blink of the right eye. There was no history suggestive of intrauterine infection or drug intake during pregnancy. There were no other congenital anomalies of eyes or limbs. On examination, child had bilateral anotia, preauricular tags and atresia of both external auditory canals [Figure 1]. There was right lower motor neuron type facial palsy evident in the form of inability to close the right eye. There was no other cranial nerve palsy and the remainder of the neurological examination was normal. Baby was intubated for airway protection. Magnetic resonance imaging brain was normal [Figure 2]. Echocardiography did not reveal any cardiac abnormalities. Brainstem auditory evoked responses were normal.

Figure 1

(a) Anotia of right side with skin tags. External auditory meatus is not seen; (b) anotia of left side with skin tags. External auditory meatus is not seen; (c) lower motor neuron type of facial palsy on the right side

Figure 2

(a and b) Magnetic resonance imaging brain T1-weighted imaging axial view showing normal pons and medulla; (c) T1-weighted imaging coronal view showing presence of both cochlea (red arrow)

Discussion

The incidence of CFP was found to be 2.1/1000 live births and is an infrequent condition.[3] Perinatal trauma, intrauterine posture, and intrapartum compression are the most frequent cause for CFP. The extracranial facial nerve is susceptible to be damaged during birth due to its relatively superficial course. Following mechanisms have been suggested: Instrumentation in assisted delivery, intrauterine posture, wherein pressure from the shoulder produces a notable displacement of the jaw and the periaural portions of the head, resulting in the pressure on the peripheral portion of the facial nerve to cause facial weakness, and intrapartum compression, wherein the foetal head is compressed against a maternal bony prominence such as the pubic rami, the ischial spines, or the sacral prominence.[4] Our patient did not have any such event. Congenital aplasia of the facial nerve nucleus is the most frequently reported aetiology for bilateral congenital facial palsy. Syndromes such as Moebius, Poland's, and Goldenhaar's have CFP as part of their symptoms.[2] Moebius syndrome is a bilateral facial and abducens nerve palsy due to defects ranging from hypoplasia to agenesis of the respective cranial nuclei.[5] Poland's syndrome comprise of unilateral absence of the pectoralis muscles, upper limb defects, and unilateral CFP. Goldenhaar's syndrome patients have unilateral facial hypoplasia, cervical vertebral defects, epibulbar dermoid, and preauricular skin tags.[5]

Agenesis of the petrous portion of the temporal bone, with resulting agenesis of the facial and auditory nerves, the external ear and the mastoid region have been described.[6] Developmental facial palsy is associated with abnormalities of the pinna and external auditory canal, ranging from milder defects to microtia and atresia. Microtia and atresia can be inherited as a part of several syndromes or acquired due to intrauterine infections (rubella, syphilis), toxin exposure (thalidomide, Isotretinoin) or ischemic injury (hemifacial microsomia). Fault in the canalization process of external auditory canal leads to stenosis, canal tortuosity or fibrosis/osseous obliteration. Defects in the canalization process may also be associated with the faulty formation of the pinna. The development of middle ear structures occurs independently to that of the external ear.[7] Hence, the tympanic cavity and ossicles may be normal. Gathwala et al., had reported a child with congenital right facial palsy associated with bilateral anotia and atresia of the right external auditory canal.

There is divided opinion among the clinicians regarding the timing of the intervention - preschool versus postadolescence surgery. Muscle transplantation is effective surgical option. Traumatic facial palsy in neonates carries good prognosis as compared to developmental facial palsy that carries poor functional outcome.

Conclusion

CFP can be developmental or acquired. One of the developmental causes for CFP is the occurrence of CFP in association with bilateral anotia and external auditory canal atresia which is rare.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.