Case 1: An eight-year-old girl with short stature

Clinician’s Corner
Case 2: A nine-year-old girl
with prolonged fever and
headache
A
previously healthy nine year-old girl presented to the authors’
institution (BC Children’s Hospital, Vancouver, British
Columbia) with a four-week history of fevers, vomiting, headache
and abdominal pain. She was first seen in a community hospital
following a 24 h history of fever followed by headache, emesis and
a generalized erythematous, macular rash. Petechiae were not
noted. A diagnosis of viral gastroenteritis was made. The rash subsided quickly but the remainder of her symptoms persisted.
Ten days later, she presented again to the same hospital with
daily fevers, daily episodes of emesis and debilitating headaches.
Her investigations included a normal complete blood cell count,
and her urine culture was positive for Escherichia coli. She was discharged on cephalexin for five days; however, her symptoms failed
to resolve.
She was subsequently referred and admitted to the authors’
tertiary care centre. Review of her history revealed daily fevers
accompanied by severe nausea and emesis, along with severe headaches without photophobia. There was no history of recent travel
or ill contacts. She denied night sweats, neck stiffness or joint
pain. Her records indicated a 5 kg weight loss over four weeks.
Physical examination revealed a stable patient with normal
vital signs. General examination was unremarkable and no neck
stiffness or neurological deficit was detected.
Laboratory investigations on admission showed leukocytosis
(white blood cell count 11.2×109/L) and thrombocytosis (platelet
count 423×109/L [normal range 150×109/L to 400×109/L]).
Erythrocyte sedimentation rate was elevated (76 mm/h). Urine
and blood cultures were negative.
Abdominal ultrasound revealed a large echogenic right kidney,
suggesting possible pyelonephritis. A computed tomography scan
of the head and a chest radiograph were normal.
A further investigation was performed to reveal the diagnosis.
Correspondence (Case 2): Dr Keyvan Hadad, Division of General Pediatrics, BC Children’s Hospital, Room 2N-11 – 4480 Oak Street, Vancouver,
British Columbia V6H 3N1. Telephone 604-602-0931, e-mail [email protected]
Case 1 accepted for publication November 8, 2013. Case 2 accepted November 12, 2013
Paediatr Child Health Vol 19 No 4 April 2014
©2014 Pulsus Group Inc. All rights reserved
177
Clinician’s Corner
case 2 Diagnosis: Chronic
Meningococcal Meningitis
Given the ultrasound results, a provisional diagnosis of partially
treated urinary tract infection was made. To complete the diagnostic workup, a lumbar puncture was performed at 12 h of admission
and before initiation of antibiotic therapy.
The cerebrospinal fluid (CSF) was cloudy, with an opening
pressure of 38 cmH2O. Analysis of the CSF revealed a glucose
level of <1.1 mmol/L, a protein level of 2.33 mg/L, white blood cell
count of 1740×106/L and red blood cell count of 2×106/L.
Polymerase chain reaction performed on the CSF was positive for
Neisseria meningitidis serogroup B. The patient was started on intravenous cefotaxime and became afebrile within 12 h of therapy. To
facilitate comprehensive evaluation of exposure and possible
chemoprophylaxis of close contacts, the case was reported to the
BC Centre for Disease Control (Vancouver, British Columbia).
All symptoms resolved within 24 h to 48 h. She was discharged
after completion of a seven-day course of intravenous cefotaxime.
Of note, the patient was subsequently shown to have no complement deficiency, nor was she asplenic. At discharge, she was found
to have moderately severe left-sided sensorineural hearing loss;
this remained unchanged six weeks later.
Infection with Neisseria meningitidis remains an important cause
of morbidity and mortality. Prompt recognition of symptoms and
initiation of therapy are essential to prevent possibly serious morbidity and mortality. The infection may be limited to blood or to
the meninges, or may involve a combination of both.
The most common findings of an acute meningeal infection
are neck stiffness, fever and altered mental status. Here, however,
we present a case of chronic meningococcal meningitis. Chronic
and/or recurrent infections with N meningitidis are exceedingly
rare, and are mostly confined to patients with deficiencies of
terminal complement components (C5 to C9), C3 or properdin,
or with anatomical or functional asplenia. Chronic meningococcemia presents as a triad of spiking fever, vasculitic rash and largejoint arthralgia. The diagnosis is challenging because bacterial
cultures are frequently negative, at least in the initial stages of the
illness. Meningeal involvement in chronic meningococcemia can
occur as a late complication.
178
Our patient was both young and fully immunocompetent, in
contrast to reports of this condition in the elderly (1) and in a
patient with a complement deficiency (2-5). Additionally, our
patient had no clinically detectable neurological findings; this differed from previous case reports (1,2,6) and increased the diagnostic challenge. Our case did, however, resemble other published
reports on chronic meningitis due to N meningitidis in that the
blood cultures were negative and the response to appropriate antibiotic treatment was rapid. The outcomes of published cases
appear to be highly variable, ranging from none to mild gait ataxia
or sensorineural hearing loss, as demonstrated in our patient.
Clinical Pearls
• Chronic meningococcal meningitis can exist in the absence of
acute or chronic meningococcemia.
• Patients with chronic meningococcal meningitis may not
always demonstrate classic meningeal signs or neurological
deficits. A lumbar puncture is, thus, essential for the diagnosis.
• Chronic meningococcal meningitis should be considered in
the differential diagnosis of fever or headache associated with
emesis.
Tahara Bhate BSc
Division of General Pediatrics
Tobias R Kollmann MD PhD
Divison of Infectious Diseases
Keyvan Hadad MD MHSc FRCPC
Division of General Pediatrics
Department of Pediatrics, University of British Columbia
Vancouver, British Columbia
Paediatr Child Health Vol 19 No 4 April 2014