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Current Paediatrics (2006) 16, 517 519

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journal homepage: www.elsevier.com/locate/cupe

SELF-ASSESSMENT
Question 1
A 5-year-old girl is referred to the emergency department by her GP. She has recently had a viral upper respiratory tract infection and presented to her GP that day as her mother was concerned that her mouth appeared to droop on the right side and her smile was asymmetrical. On further history-taking, you discover that the symptoms were only noted today and are not causing her any problems. She has previously been t and well. She has never had chicken pox and is fully immunised. She is afebrile with normal observations. On examining her face, you see that when she smiles her mouth droops on the right, she is unable to squeeze her right eye shut, and she is also unable to raise her right eyebrow. Further examination of the remaining cranial nerves is normal. 4. What is the name given to a recurrent unilateral or bilateral facial nerve palsy associated with chronic facial oedema? (a) Bells palsy (b) GuillainBarre syndrome (c) Melkersson syndrome (d) Moebius syndrome (e) RamsayHunt syndrome 5. In a child with Bells palsy, what is the most important treatment? (a) Articial tears (b) Gentamicin ear drops (c) Ibuprofen (d) Oral aciclovir (e) Prednisolone

Answers
1. What is the diagnosis? (a) Left lower motor neurone lesion of the facial nerve (b) Left-sided Bells palsy (c) Left upper motor neurone lesion of the facial nerve (d) Right lower motor neurone lesion of the facial nerve (e) Right upper motor neurone lesion of the facial nerve 1. (d) Right lower motor neurone lesion of the facial nerve An upper motor neurone lesion will affect the contralateral side; as the lesion occurs above the level of decussation of the tracts, and only involves the lower face; this is due to the fact that the forehead receives bilateral innervation. A lower motor neurone lesion, i.e. one involving the facial nerve nucleus in the brainstem or anywhere along the facial nerve itself, will cause symptoms on the same side of the face and involve the forehead. A Bells palsy is an isolated facial nerve lower motor neurone lesion. 2. (d) Parasympathetic supply to the lacrimal gland 3. When examining a child with a facial nerve palsy, it is important to think about the course of the facial nerve as this directs clinical examination. Which one of the following structures does the facial nerve not pass through? (a) External auditory canal (b) Internal auditory canal (c) Parotid gland (d) Stylomastoid foramen (e) Temporal bone
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2. The facial nerve is responsible for which of the following? (a) Hearing (b) Lateral eye gaze (c) Innervation to the muscles of mastication (d) Parasympathetic supply to the lacrimal gland (e) Taste sensation to the posterior one-third of the tongue

The facial nerve (VIIth cranial nerve) is composed of a motor, a sensory and a parasympathetic division. The motor division supplies all the muscles of facial expression. Asymmetry of the face therefore indicates a unilateral lesion, and this may present as loss of the nasolabial fold, drooping of the mouth and drooling. On further examination, a loss of forehead wrinkling and an inability to raise the eyebrow and close the eye tight may be seen.

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518 The sensory division supplies taste to the anterior twothirds of the tongue. The parasympathetic division supplies the lacrimal gland. The glossopharyngeal nerve supplies taste bres to the posterior third of the tongue. Lateral eye gaze is brought about by the lateral rectus muscle, which is supplied by the abducens nerve. Branches of the mandibular portion of the trigeminal nerve supply the muscles of mastication. Hearing is supplied by the vestibulocochlear nerve. A branch of the facial nerve supplies the stapedius muscle in the tympanic cavity. The stapedius responds to high-intensity sounds and acts by damping down vibrations before they reach the internal ear, but is not responsible for hearing.1 3. (a) External auditory canal The facial nerve arises near the pons. On emerging from the brain, the facial nerve enters the internal acoustic meatus. It then passes from the lateral end and enters the facial canal before descending to the stylomastoid foramen. Emerging from here, it runs forward in the parotid gland, crosses the styloid process and divides behind the neck of the mandible into branches that further distribute to the facial muscles.1 4. (c) Melkersson syndrome RamsayHunt syndrome is herpes zoster infection of the geniculate ganglion of the facial nerve. It is usually unilateral. Examination may reveal vesicles on the tympanic membrane. It should be treated with aciclovir. Moebius syndrome is a congenital condition characterised by a bilateral asymmetrical lower motor neurone facial nerve palsy associated with VIth nerve palsies. It is thought to be caused by underdevelopment of the cranial nerve nuclei. GuillainBarre syndrome is characterised by an ascending symmetrical accid paralysis. It generally occurs after a viral illness. Bells palsy is an isolated facial nerve palsy of unknown pathology. Melkersson syndrome is a rare disorder involving recurring facial paralysis, swelling of the face and lips, and the development of folds and furrows in the tongue. There may be a genetic predisposition.2 5. (a) Articial tears More evidence is needed before antiviral medication can be recommended as being effective in aiding recovery from Bells palsy.3 There is no evidence that prednisolone improves the outcome in children with Bells palsy.4 Articial tears are important to prevent corneal ulceration. In cases in which the eye will not close at night, a lubricant gel or patch should be worn. Gentamicin ear drops are not indicated. Ibuprofen could be used for its anti-inammatory properties, but there is no evidence to support its routine use. SELF-ASSESSMENT

References
1. Williams PL, Warwick R, Dyson M, Bannister L, editors. Grays Anatomy, 37th ed. Edinburgh: Churchill Livingstone; 1989. p. 110711, 1228. 2. Brett EM. Neuromuscular disorders: II. Peripheral neuropathy. In: Brett EM, editor. Paediatric Neurology. Edinburgh: Churchill Livingstone; 1985. p. 124. 3. Allen D, Dunn L. Aciclovir or valaciclovir for Bells palsy. Cochrane Database of Systematic Reviews 2006; Issue 1. 4. Ashtekar C, Joishy M, Joshi R. Do we need to give steroids in children with Bells palsy? Emerg Med J 2005;22:5057.

Question 2
A 10-day-old boy presents to the emergency department with a 1-day history of lesions on his lips. He was born at 38 weeks by Caesarean section because his mother had previously had a Caesarean section. The pregnancy was uneventful; in particular there was no history of Group B Streptococcus or herpes simplex virus infection. The baby was discharged home on day 2, feeding well. He developed sticky eyes on day 4, which his mother treated with overthe-counter medication. Otherwise, there had been no concerns and he continued to feed well. On the morning of presentation, his mother had noticed yellow crusty lesions on his upper lip and at the angle of his mouth. Although feeding well, he seemed more unsettled than normal. At triage, his observations were as follows: Axillary temperature 36.4 1C Heart rate 140 beats per minute Central capillary rell time less than 2 s Respiratory rate 35 breaths per minute. Clinical assessment revealed the baby to be unsettled, and he disliked being examined. Cardiovascular, respiratory and abdominal examination was unremarkable. He had a yellow crusty lesion at the angle of his mouth and a small one on his central upper lip. There was yellow discharge from both eyes, but his conjunctivae were normal. His skin was diffusely erythematous and seemed to be tender. He was well hydrated. 1. What is the most likely diagnosis? (a) Herpes simplex infection (b) Neonatal bullous impetigo (c) Neonatal erythema toxicum (d) Staphylococcal scalded skin syndrome (e) Toxic epidermal necrolysis 2. What is the most likely pathogen? (a) Group B Streptococcus (b) Human herpes virus 6 (c) Staphylococcus aureus enterotoxin (d) Staphylococcus aureus epidermolytic toxins A and B (e) Streptococcus pyogenes exotoxin

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SELF-ASSESSMENT 3. What would be the most appropriate treatment? (a) High-dose oral ucloxacillin (b) Intravenous benzylpenicillin and ucloxacillin (c) Intravenous aciclovir (d) A swab lip lesion and await sensitivities before commencing the appropriate antibiotic (e) Topical fucidin 4. What is the usual natural progression of this condition if treated appropriately and promptly? (a) Drying of the erosions followed by desquamation and healing within 14 days (b) The formation of pustules that burst, leaving hyperpigmented macules (c) Septicaemia leading to circulatory collapse, with a high rate of morbidity and mortality (d) Stripping of the epidermis followed by severe scarring with loss of the nails and hair (e) Uncomplicated healing but a recurrence of the lesions precipitated by minor illness 519 epidermolytic toxins A and B are responsible for epidermal splitting and exfoliation, and it is these which, when disseminated, haematogenously cause staphylococcal scalded skin syndrome. Staphylococcus aureus enterotoxin causes diarrhoea and vomiting. Streptococcus pyogenes exotoxin is responsible for scarlet fever. 3. (b) Intravenous benzylpenicillin and ucloxacillin Systemic anti-staphylococcal antibiotics are appropriate in this situation. Swabs and cultures will often be negative given that the skin involvement is toxin-mediated. It is appropriate to cover for Streptococcus as wellin the above case, our patient grew Streptococcus pneumoniae from eye swabs and Staphylococus aureus from his lip. The Staphylococcus was conrmed as toxins A- and B-positive. 4. (a) Drying of the erosions followed by desquamation and healing within 14 days Stripping of the epidermis and scarring is often the outcome in toxic epidermal necrolysis, which has a much higher mortality and morbidity. In staphylococcal scalded skin syndrome, a localised staphylococcal infection may be the initial pathology. A few days later, there is a widespread erythematous eruption and skin tenderness. This is followed by blister formation. The tender skin then wrinkles and shears, leaving raw and painful areas that dry and heal, generally within 414 days, with no sequelae. It is important to monitor uid balance in these children; circulatory collapse and staphylococcal septicaemia are rare but important complications.

Answers
1. (d) Staphylococcal scalded skin syndrome Clinically, the lesions described are characteristic of Staphylococcus infection. Yellow crusting is commonly described. Staphylococcal scalded skin syndrome is an exfoliative dermatosis that initially presents with diffuse erythema and skin tenderness. There is usually an isolated minor local infection, which in this case was the lip. Neonatal bullous impetigo can be caused by Staphylococcus, Streptococcus or the two together. It is a supercial infection caused by the bacteria and generally leads to lesions that are vesicular and then pustular. These rupture dry and form shiny crusts. Oral antibiotics generally sufce. Although lesions may spread, the whole skin is not involved. Erythema toxicum is a benign rash of neonates. It is generally seen between 24 h and 14 days of life. It consists of poorly demarcated erythematous macules with central pale papules or pustules. It is self-limiting, and its aetiology is unknown. There is no history of herpes simplex infection, and lesions tend to be vesicular and present on the presenting part. The lesions may become pustular and then burst to form erosions. Toxic epidermal necrolysis is generally a drug reaction affecting adults and causes full-thickness damage to the epidermis. 2. (d) Staphylococcus aureus epidermolytic toxins A and B. Group B Streptococcus is an important pathogen in neonates, generally causing meningitis and septicaemia. Human herpes virus 6 causes roseola infantum, a selflimiting vial exanthema with a characteristic rash and fever. Staphylococcus aureus forms a large number of extracellular toxins and enzymes. Staphylococcus aureus

Further reading
Hay RJ, Adriaans BM. Bacterial infections. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Textbook of dermatology. 6th ed., vol. 2. Oxford: Blackwell Science; 1998. p. 11256. Rogers M. Neonatal dermatology. In: Rennie JM (ed.) Robertons textbook of neonatology. 4th ed. Edinburgh: Elsevier Churchill Livingstone; 2005. p. 81921. Sleigh JD, Timbury MC. Notes on medical bacteriology. 4th ed. Edinburgh: Churchill Livingstone; 1995. p. 623. This self-assessment was compiled by: Anne Ingram Specialist Registrar in Paediatrics Ashley Reece Consultant Paediatrician Department of Paediatrics Watford General Hospital, Watford Hertfordshire WD18 0HB, UK E-mail address: ashley.reece@nhs.net