From the Department of Paediatric Ophthalmology and Strabismus (BM, VS, AS, NA), Moorfields Eye Hospital; and the Department of Paediatrics (AS), Homerton University Hospital, London, United Kingdom.
The authors have no financial or proprietary interest in the materials presented herein.
The authors thank Dr. I. O. Kudayah at the Lifeline Children’s Hospital, Lagos, Nigeria, for the provision of the medical notes of the patient while under investigation in Nigeria.
Address correspondence to Bita Manzouri, MRCP, FRCOphth, PhD, Department of Paediatrics & Strabismus, Moorfields Eye Hospital Foundation NHS Trust, 162 City Road, London EC1V 2PD, United Kingdom. E-mail: email@example.com
Bell’s palsy, named after the eminent Scottish anatomist Charles Bell, is defined as an idiopathic unilateral partial or complete facial nerve paralysis of rapid onset that is usually self-limiting. Bell’s palsy accounts for almost three-quarters of acute facial palsies1 and is a diagnosis of exclusion, made following appropriate examination and investigation. The highest incidence is noted in the 15- to 45-year-old age group.2 It is much less common in children, with an annual incidence of 2.7 per 100,000 children younger than 10 years and 10.1 per 100,000 for those between 10 and 20 years of age.3
A previously healthy, term-born female infant of Nigerian origin aged 3 months developed drooping of the left side of the face over the course of 1 day, so that by the evening she had an asymmetrical smile. Her family was visiting Lagos, Nigeria, and the infant had been receiving chloroquine syrup malarial prophylaxis prior to leaving the United Kingdom a month before. There was no preceding history of fever or illness.
She was examined the same day by a pediatrician, who found her to be afebrile with normal general and developmental examination. Neurological examination was normal apart from a unilateral left lower motor-neuron facial palsy. Blood tests (full blood count, HIV testing, and erythrocyte sedimentation rate) and cranial computed tomography were undertaken and she began a course of oral acyclovir and prednisolone. The only abnormal result was a marginally raised erythrocyte sedimentation rate of 14 mm in the first hour.
On return to London 1 month later, the facial palsy was noted to be much less marked by a consultant pediatrician. She had mild exposure keratopathy and topical ocular lubricants were commenced. Repeat HIV testing remained negative, and complete clinical recovery of the facial muscle function and the exposure keratopathy was observed when she was examined 2 months after onset of her symptoms.
Bell’s palsy is an idiopathic peripheral neuropathy of the seventh cranial or facial nerve,4 and this case is, to the best of our knowledge, the youngest yet reported. The prognosis for children with idiopathic Bell’s palsy is good, with more than 90% showing some degree of recovery, in part because of the high frequency of partial paralysis.2 Children with complete palsies may suffer poorer outcomes,5 such as exposure keratopathy of the affected eye.
Possible causes of a unilateral facial palsy are listed in Table 1, and it is important that all children are carefully examined to exclude them (Table 2). This is probably best undertaken, as in this case, by the pediatric ophthalmologist working in conjunction with a pediatrician.
Table 1: Etiology of Unilateral Facial Nerve Palsy in Children
Table 2: Assessment of Unilateral Facial Nerve Palsy in Children
Most children (approximately 60%)4 with Bell’s palsy have a history of a preceding illness, usually an upper respiratory tract infection. Viral infection is suspected as a causative factor because isolates of herpes and varicella zoster viruses have been found in many affected patients.6 The virus is presumed to cause inflammation of the facial nerve, which then becomes compressed in the bony facial canal and palsy ensues.7 In our case, given the high prevalence of HIV infection in sub-Saharan Africa, retroviral testing was performed. Similarly, in endemic areas, it is important to exclude Lyme disease.
The use of oral steroids for the treatment of Bell’s palsy in adults is common and more than 200 non-controlled studies have been published.8,9 A Cochrane meta-analysis of a sample of 8 studies concluded that treatment with oral steroids (prednisolone) reduces the time to recovery of facial nerve function compared with placebo or no specific treatment in adults.10 The only randomized controlled trial in the pediatric age group reported no short-term benefit of treatment with methylprednisolone over placebo.9 However, this study was small, involving a total of 42 children, all of whom had the most severe form of Bell’s palsy.
Given the putative involvement of herpes viruses, the use of antiviral drugs is not uncommon (Table 3). Antivirals (acyclovir or valacyclovir) are less effective at 12 weeks in producing full recovery of facial function than oral steroids.11 However, it is thought that there may be a benefit in the use of antiviral agents in combination with a corticosteroid compared to a steroid alone in adults.11 A review of available research suggests that there is insufficient data to make definitive statements about the best treatment in children and that 95% of cases will resolve without treatment.12
Table 3: Treatment of Unilateral Idiopathic Facial Nerve Palsy in Children
Bell’s palsy in children is a diagnosis of exclusion. We have presented the youngest case of Bell’s palsy reported in the literature, highlighting the importance of investigations tailored to the patient and his or her presenting history and some of the controversies as to the best management of cases of pediatric Bell’s palsy.
- Lunan R, Nagarajan L. Bell’s palsy: a guideline proposal following a review of practice. J Paediatr Child Health. 2008;44:219–220. doi:10.1111/j.1440-1754.2007.01245.x [CrossRef]
- Peitersen E. Bell’s palsy: the spontaneous course of 2,500 peripheral facial nerve palsies of different etiologies. Acta Otolaryngol Suppl. 2002;(549):4–30.
- Katusic SK, Beard CM, Wiederholt WC, Bergstralh EJ, Kurland LT. Incidence, clinical features, and prognosis in Bell’s palsy, Rochester, Minnesota, 1968–1982. Ann Neurol. 1986;20:622–627. doi:10.1002/ana.410200511 [CrossRef]
- Chen WX, Wong V. Prognosis of Bell’s palsy in children: analysis of 29 cases. Brain Dev. 2005;27:504–508. doi:10.1016/j.braindev.2005.01.002 [CrossRef]
- Prescott CA. Idiopathic facial nerve palsy in children and the effect of treatment with steroids. Int J Pediatr Otorhinolaryngol. 1987;13:257–264. doi:10.1016/0165-5876(87)90106-6 [CrossRef]
- Murakami S, Mizobuchi M, Nakashiro Y, Doi T, Hato N, Yanagihara N. Bell palsy and herpes simplex virus: identification of viral DNA in endoneural fluid and muscle. Ann Intern Med. 1996;124:27–30.
- Adour KK, Byl FM, Hilsinger RL Jr, Kahn ZM, Sheldon MI. The true nature of Bell’s palsy: analysis of 1,000 consecutive patients. Laryngoscope. 1978;88:787–801.
- Lagalla G, Logullo F, Di Bella P, Provinciali L, Ceravolo MG. Influence of early high-dose steroid treatment on Bell’s palsy evolution. Neurol Sci. 2002;23:107–112. doi:10.1007/s100720200035 [CrossRef]
- Salinas RA, Alvarez G, Alvarez MI, Ferreira J. Corticosteroids for Bell’s palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2002;CD001942.
- Salinas RA, Alvarez G, Ferreira J. Corticosteroids for Bell’s palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2004;CD001942.
- Allen D, Dunn L. Aciclovir or valaciclovir for Bell’s palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2004;CD001869.
- Ashtekar CS, Joishy M, Joshi R. Best evidence topic report: do we need to give steroids in children with Bell’s palsy?Emerg Med J. 2005;22:505–507. doi:10.1136/emj.2005.026567 [CrossRef]
Etiology of Unilateral Facial Nerve Palsy in Children
Varicella zoster virus, herpes simple type 1 or type 2 virus, HIV, mumps, rubella, Epstein–Barr virus, hepatitis (A, B, C), cytomegalovirus
Lyme disease, Mycoplasma, meningitis (infectious or infiltrative)
Otitis media, mastoiditis, cholesteatoma
Polyneuritis, Guillain–Barre syndrome, Melkersson–Rosenthal syndrome
Facial trauma, forceps delivery, post-surgery
Hypertension, aneurysm of vertebral, basilar, or carotid arteries
Leukemia, tumor (eg, parotid, base of skull, intracranial), sarcoid
Assessment of Unilateral Facial Nerve Palsy in Children
Obtain a thorough history from the parent or guardian to determine whether there are associated systemic features
Complete a full systemic, neurological, and cranial nerve examination, including auriscope examination and blood pressure estimation
Perform blood tests (full blood count, film, erythrocyte sedimentation rate); urea, electrolytes, and liver function tests; C-reactive protein
Consider HIV, cytomegalovirus, Epstein–Barr virus, rubella, herpes simplex virus types 1 and 2, hepatitis A, B, or C, varicella zoster virus, Mycoplasma pneumoniae, or Lyme disease (in endemic area)
Consider storing a serum sample for paired testing
Consider neurological imaging (computed tomography or magnetic resonance imaging)a
Consider referral to a pediatrician, ear-nose-throat surgeon, or pediatric neurologist
Treatment of Unilateral Idiopathic Facial Nerve Palsy in Children
|Eye protection||Regular lubrication with artificial tear drops during the day. Artificial tear ointment and taping of the eye shut overnight.|
|Steroids||Consider the use of oral steroids: oral prednisolone 1 to 2 mg/kg (max 60 mg) for 7 to 14 days.|
|Acyclovir||Indicated if there are signs of herpes simplex or herpes zoster infection. Oral dose: age 1 month to 2 years = 100 mg 5 times a day, age 2 to 18 years = 200 mg 5 times a day. Consider the need for the parenteral route. Consider even if herpes virus is not proven at the time of presentation.|