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Official Journal of the European Paediatric Neurology Society

Case study

Skull base osteomyelitis leading to lateral medullarysyndrome in a child

Joanne Nga, Daniel J.A. Connollyb, Christopher D. Ritteya, Santosh R. Mordekara,�

aDepartment of Paediatric Neurology, Sheffield Children’s Hospital, Sheffield, UKbDepartment of Neuroradiology, Sheffield Children’s Hospital, Sheffield, UK

a r t i c l e i n f o

Article history:

Received 31 May 2006

Received in revised form

5 November 2006

Accepted 5 November 2006

Keywords:

Skull base osteomyelitis

Sinusitis

Headache

Lateral medullary syndrome

nt matter & 2006 Europe06.11.003

thor. Tel.: +44 1142260675santosh.mordekar@sch.n

A B S T R A C T

Skull base osteomyelitis (SBO) arising from the sphenoidal paranasal air sinus infection

without associated external otitis is rare. Initially SBO may have headache as the only

symptom with cranial neuropathies occurring later. We report a 10-year-old immunocom-

petent girl with headache and chronic sinusitis, who developed a lateral medullary

syndrome following streptococcal milleri sphenoidal osteomyelitis.

& 2006 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

1. Introduction

Typical cases of skull base osteomyelitis (SBO) are initiated by

ear infections in older diabetic or immunocompromised

patients, with Pseudomonas aeruginosa as the usual pathogen.1

SBO rarely originates from paranasal infection, and in these

cases is usually caused by Aspergillus, Pseudomonas, Salmonella,

and Staphylococcus species.2 Patients initially may present with

headache as the only symptom, with cranial neuropathies

occurring later, which makes early clinical diagnosis difficult.

We report an immunocompetent 10-year-old girl with a SBO

due to chronic sphenoidal sinusitis, who developed a lateral

medullary syndrome (LMS).

2. Case study

A 10-year-old girl presented with vomiting, neck pain and

worsening headaches. She had suffered recurrent sinusitis

an Paediatric Neurology S

; fax: +44 1142678296.hs.uk (S.R. Mordekar).

and otitis media previously and had an atrial septal defect

repair at the age of 7 years. On examination she was febrile

with neck stiffness. The rest of her neurological examination

was normal at this point.

Her initial assessment included a septic screen excluding a

lumbar puncture. Full blood count showed haemoglobin

12.1 g/l; white cell count 21.6�109/l with neutrophils

19.2�109/l and platlets 217�109/l. Her C-reactive protein

was 191 g/l but her blood and urine cultures were negative.

Immunoglobulin levels including sub-classes were normal.

Cardiac echocardiogram was normal. She was empirically

started on intravenous Cefatoxime 200 mg/kg/day. Subse-

quently she developed a right motor facial palsy, left

ptosis and dysarthria. Twenty-four hours later she developed

right-sided hemiparesis with left-sided complete third

nerve palsy. A computed tomography (CT) scan of her brain

showed marked meningeal enhancement with no space

occupying lesion. Based on her focal neurological signs and

neurological deterioration she was started on intravenous

ociety. Published by Elsevier Ltd. All rights reserved.

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Acyclovir 500 mg/m2/day whilst microbiology results were

awaited.

Twenty-four hours later, she became encephalopathic and

developed profound dysarthria with an absent gag reflex. She

was intubated, ventilated and transferred to a paediatric

intensive care unit. An MRI of her brain demonstrated right

medullary high T2 signal changes, right petrous apex

enhancement, right spenoidal and maxillary sinusitis with

increased meningeal enhancement post-gadolinium. (Figs. 1

and 2) T1 images showing a normal left clivus whilst the right

clivus shows signal changes suggestive of base of skull

osteomyelitis (Fig. 3a and b). She underwent an inferior

meatus antrostomy and right maxillary sinus washout that

revealed a thick purulent fluid. This grew Streptococcus milleri

on culture. Following surgery her Glasgow coma scale (GCS)

was 15, right facial palsy and left third nerve palsy and her

right hemiparesis improved; however, her gag reflex re-

mained absent. She underwent a tracheostomy and was

extubated. A speaking valve tracheostomy was performed

and she was nasojejunally fed. She received intravenous

Cefatoxime 200 mg/kg/day and Metronidazole 15 mg/kg/day

for 8 weeks. She did extremely well and after 4 months, her

tracheostomy tube was removed without any problems. At 6

months follow-up she fed orally, has a very mild hemiparesis

on right side, but is completely well and is being gradually

integrated in school with good progress. Follow-up MRI at 6

Fig. 1 – Axial T2 through the cerebellopontine angle

demonstrating right medullary high T2 signal, right

petrous apex enhancement, right spenoidal and maxillary

sinusitis.

Fig. 2 – Axial T1 (post-gadolinium) through the

cerebellopontine angle demonstrating empyema with

meningeal enhancement.

months showed minimal residual change in the right lateral

medulla (Fig. 4).

3. Discussion

SBO is an uncommon condition that is potentially life

threatening if not promptly recognised and properly treated.

It often presents subtly and non-specifically with a persistent

headache and an eventual development of cranial neuro-

pathy.1 Other presenting features include conductive hearing

loss, venous sinus thrombosis, intracerebral abscess, menin-

gitis and internal carotid artery infiltration.3 Baring the first

cranial nerve, all other cranial nerves can be involved in

extensive BSO.4 It is usually due to contiguous spread of

invasive or necrotising infection from the ear canal. Although

the exact mechanism of spread is unknown, venous spread

(thrombophlebitis) could be a possibility in our case. Patients

are usually adults with a predisposition to infection such as

diabetes mellitus, corticosteroid use immunosuppression or a

HIV infection. Our case is rare as our patient is an

immunocompetent child with normal immunoglobulin levels

and BSO presenting without otitis externa is extremely rare.2

Various organisms have been implicated in BSO, with

Pseudomonas species being the most common.2,4 The organ-

ism isolated in the sinus fluid in our case was Streptococcus

milleri.

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Fig. 3 – (a and b) Unenhanced coronal T1 images through the

skull base. There is normal high T1 signal in the left

occipital condyle (figure 3a) and the left side of the clivus

(figure 3b) but low T1 signal in the right side of the skull

base. An associated right sided soft tissue inflammatory

mass which is also of low signal on T1 is demonstrated

below the bony skull base on the both figures.

Fig. 4 – Axial T2 follow-up scan through the lateral medulla.

E U RO P E A N J O U R NA L O F PA E D I AT R I C N EU RO L O G Y 11 (2007) 111 – 114 113

MRI brain is superior to CT brain for diagnosis of SBO as it

provides superior soft tissue discrimination and is useful in

assessing the soft tissue planes around the skull base and

abnormalities of the medullary bone.5 As seen in our case, CT

scan can sometimes be normal, especially if done in the early

stages of the illness. In cases where CT/MRI findings are not

conclusive, increased uptake on gallium 67 scintiscan with

high inflammatory markers would be highly suggestive of

SBO.4 Treatment is by long-term intravenous antibiotics

usually for 8–12 weeks.4,6 There is a high morbidity and

mortality associated with this condition, especially as diag-

nosis is delayed.

LMS or Wallenberg’s syndrome is a vascular syndrome

of the posterior circulation. Symptoms depend upon the level

of medulla involved. The three main neurological sequelae of

LMS are sensory symptoms, dizziness/sense of imbalance,

and dysphagia, in that order of frequency.7 Headaches,

dysphagia and dysarthria are more common in caudal lesions

rather than rostral lesions, which presents with sensory

involvement.8 In our case, the symptoms were suggestive of a

caudal rather than rostral lesion. The association of LMS with

SBO has not been previously described. The precise patho-

genesis of LMS in our case is unknown. It is likely be due to

direct extension of the SBO causing a thrombophlebitis

vascular occlusion leading to a lateral medullary infarct,

rather than a thromboembolic event that is usually seen with

LMS. In our case, aggressive treatment with antibiotics and

surgery helped reverse the neurological sequelae of SBO.

Antibiotics constitute the mainstay of medical treatment, and

in our case, intranasal antrostomy proved to be both

diagnostic as well as therapeutic in rationalising antibiotic

therapy. In more recent years endoscopic sinus surgery has

been increasingly used, although its role in paediatric practice

still remains controversial.9,10 As the child remained clinically

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well and neurologically improved conservative management

was pursued. Should her clinical condition have deteriorated,

possible craniotomy with abscess drainage would have been

an option. However, the progression of the disease to LMS was

responsible for the prolonged morbidity in this child.

SBO is a serious condition that requires prompt diagnosis

and aggressive management. Diagnosis can be challenging,

especially in a child, where this condition is extremely rare.

A high index of suspicion should be considered in cases with

the following signs and symptoms even in an immunocom-

petent patient: (1) persistent/unremitting headache (2) ear

and/or paranasal infections (3) presence of lower cranial

nerve deficits. Early diagnosis and treatment is important to

prevent progression and decrease the mortality and morbid-

ity associated with this condition.

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