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Developmental unilateral facial palsy in a newborn: six cases and literature review

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Abstract

Unilateral facial palsy in a newborn is rarely caused by a developmental defect. It occurs either isolated or in the context of a syndrome. This article describes a multidisciplinary approach towards unilateral, isolated congenital facial palsy along with a literature review. We report six patients, three boys and three girls, who presented with a unilateral facial palsy at birth. Clinical assessment was performed by an ear-nose-throat (ENT) surgeon, a pediatric neurologist, and an ophthalmologist. Magnetic resonance imaging (MRI) of the posterior fossa and computerized tomography (CT) of the temporal bone were requested to exclude structural anomalies of the facial nerve. Imaging revealed the underlying cause in five patients out of six (80%), showing an ipsilateral facial nerve aplasia or hypoplasia. These findings point towards an underlying developmental defect and underscore the importance of MRI in the diagnostic work-up. Surgical and non-surgical therapies were discussed with the parents.

Conclusion: Congenital unilateral facial palsy caused by a developmental defect outside the context of a syndrome is rare. A multidisciplinary approach is recommended to differentiate between various causes and to initiate timely treatment.

What is Known:

• Congenital facial palsy is mostly caused by environmental/external fcators.

• However in rare cases it can be developmental defect.

What is New:

• This paper describes 6 cases of isolated congenital facial palsy related to a developmental defect and presents the largest case series in the literature caused by aplasia/hypoplasia of the facial nerve.

• MRI and CT-imaging allow for an assessment of the facial nerve at the root entry zone of the brainstem and along its course through the middle ear or the face. Moreover, they proved to be helpful in differentiating between several causes of congenital facial palsy.

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Abbreviations

3D-CISS:

Three-dimensional constructive interference steady state

AICA:

Anterior inferior cerebellar artery

CPAP:

Continuous positive airway pressure

CT:

Computed tomography

Dr.:

Doctor

EMG:

Electromyography

ENT:

Ear-nose-throat

GSPN:

Greater superficial petrosal nerve

HB:

House-Brackmann

IAC:

Internal acoustic canal

M.:

Musculus

MRI:

Magnetic resonance imaging

N.:

Nervus

UMCG:

University Medical Center Groningen

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Acknowledgments

We would like to acknowledge the contributions of Professor Dr. P. Werker, plastic surgeon, UMCG, Groningen, The Netherlands, for sharing practical expertise and execution of the surgical procedure of patient one. The authors have stated that they had no conflict of interests that could be perceived as posing a conflict or bias.

Author information

Author notes

  1. An Boudewyns and Berten Ceulemans contributed equally to this work.

Authors and Affiliations

  1. Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, Belgium

    Laura Decraene, An Boudewyns & Berten Ceulemans

  2. Department of Otorhinolaryngology, Antwerp University Hospital, Wilrijkstraat 10, 2650, Edegem, Belgium

    An Boudewyns

  3. Department of Radiology and Imaging, Antwerp University Hospital, Wilrijkstraat 10, 2650, Edegem, Belgium

    Caroline Venstermans

  4. Department of Pediatric Neurology, Antwerp University Hospital, Wilrijkstraat 10, 2650, Edegem, Belgium

    Berten Ceulemans

Authors
  1. Laura Decraene
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  2. An Boudewyns
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  3. Caroline Venstermans
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  4. Berten Ceulemans
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Contributions

Authors LD, AB and BC are responsible for content and scripture of manuscript. Author CV is responsible for neuroimaging evaluations. Author AB is responsible for ENT evaluations. Authors BC is repsonsible for clinicals evaluations. All authors have read and agreed to the content of the manuscript.

Corresponding author

Correspondence to Berten Ceulemans.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Communicated by Peter de Winter

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Appendix

Appendix

Fig. 1
figure 2

MRI images of patient 2 performed in 2012. a Axial 0.4-mm 3D-constructive interference in steady state (3D-CISS) sequence shows an aplasia of the right facial nerve (white arrowhead). b Parasagittal 0.5-mm reconstructions through the right internal auditory canal (IAC) show an aplastic facial nerve (small white arrow). c Parasagittal 0.5-mm reconstructions through the left internal auditory canal (IAC) show a normal facial nerve (thick white arrow). A, anterior; L, left; P, posterior; R, right

Full size image
Fig. 2
figure 3

MRI of patient 6 performed in 2013. a Axial 0.4-mm 3D-constructive interference in steady state (3D-CISS) sequence shows a hypoplastic right facial nerve (white arrowhead). It is important not to confuse the anterior inferior cerebellar artery (AICA) loop in the internal auditory canal (IAC) with a nerve. b Parasagittal 0.5-mm reconstructions through the right IAC show a hypoplastic facial nerve (small white arrow). c Parasagittal 0.5-mm reconstructions through the left IAC show a normal facial nerve (thick white arrow). A, anterior; L, left; P, posterior; R, right

Full size image

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Decraene, L., Boudewyns, A., Venstermans, C. et al. Developmental unilateral facial palsy in a newborn: six cases and literature review. Eur J Pediatr 179, 367–375 (2020). https://doi.org/10.1007/s00431-019-03484-8

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  • DOI: https://doi.org/10.1007/s00431-019-03484-8

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