Section of Child Neurology, Women’s and Children’s Hospital, Charleston Area Medical Cen- ter, Charleston, West Virginia, USAUnited States
Texila American University, Woolford Ave, Georgetown, GuyanaGuyana
Department of Physiology, P.D.U. Government Medical College, Rajkot, Gujarat, IndiaIndia
Also available as PDF (figures and tables appear in the PDF).
Group A Streptococcus pyogenes meningitis rarely results in central nervous system complication. Chronic hydrocephalus is a well-known complication of bacterial meningitis. To date, no reports have focused on acute central nervous system complications and neurological outcome of Group A Streptococcus pyogenes meningitis. An unusual association of acute hydrocephalus requiring emergent neurosurgical intervention in a 6-year-old girl with Group A Streptococcus meningitis is presented. Based on reported cases since 1966, authors present the results of a comprehensive acute neurological complication and outcome. Our report highlights a need for vigilance and timely needed emergent surgical intervention in minimizing acute hydrocephalus in children with Group A Streptococcus meningitis.
Keywords: Streptococcus pyogenes meningitis; Central Nervous System complication; Acute hydrocephalus; Surgical intervention; Children; Streptococcus pyogenes meningitis; Central Nervous System complication; Acute hydrocephalus; Surgical intervention; Children
Streptococcal infections are common in children. Unlike Group B Streptococci septicemia,1 Group A Streptococcus (S. pyogenes) typically affects children beyond the neonatal period. Occasionally, this causes acute meningitis, glomerulonephritis, or rheumatic heart disease.2 Group A Streptococcus infection is a rare cause of bacterial meningitis. Hydrocephalus is a relatively rare complication of acute bacterial meningitis in children. Nonetheless, it has
They have been reported in children with viral and other bacterial meningitis.
Group A Streptococcal meningitis and provide the results of a comprehensive literature review on acute CNS complications and neurological outcome in children 17years and under.
A 6-year-old girl presented to the Emergency Department with vomiting and neck pain for 24 hours. She had headache for five days and intermittent fever and sore throat for the past two weeks. She had no seizures and had received no antibiotics. A non-contrast Computerized Tomography (CT) scan of the brain prior to the lumbar puncture was normal. Treatment with intravenous vancomycin and ceftriaxone was initiated.
On admission to the Pediatric Intensive Care Unit, her vital signs were stable and temperature was 37.6 degree C. Her throat was red and congested but the tympanic membranes were normal. Her skin had no lesions. She had decreased alertness and her neck was hyper-extended in a fixed position. Pupils were 4 mm and reactive to light. Fundoscopic examination revealed no papilledema. Deep tendon reflexes were hyperreflexic and on plantar stimulation, her toes were up going.
The Cerebrospinal fluid (CSF) studies revealed an elevated intracranial pressure, a white blood cell count of 3 3 7644 mm3, red blood cells 10,000 mm3, protein 440 mg/dl and glucose 20 mg/dl. Serum glucose was 102 mg/dl. CSF smears revealed Gram-positive cocci in pairs and chains. The blood and CSF culture yielded S. pyogenes. Genotyping for the streptococcal isolates was not obtained. The CSF polymerase chain reaction for herpes simplex virus type 1 and 2 DNA for both, was negative.
Ten hours after admission, she had an acute increase in heart rate from 80 to 133 beats per minute and blood pressure from 124/69 to 156/47 mm Hg. Her pupils became asymmetric and reacted sluggishly to light. Her Glasgow Coma Scale score declined from 13 to 9. Following endotracheal intubation, mechanical ventilation, and administration of 1 gram/kilogram of mannitol, a contrast-enhanced brain CT scan was performed which revealed communicating hydrocephalus with no herniation or abscess (Figure 1A). An urgent frontal external ventricular drain was placed. A follow up brain CT scan revealed relief of the hydrocephalus (Figure 1B).
The ventriculostomy tube was clamped on day 12 and was removed on day 15. Intravenous ceftriaxone was continued for 21 days. She received physical, occupational, and speech therapies throughout the hospitalization. She was rd discharged on the 23rd day of admission with no focal motor, cerebellar, or sensory deficits.
ventricular drain shows a decrease in ventricular size, reappearance of basal cisterns (arrows heads), and also the disappearance of the left parietal hypodensity suggesting no parietal infarction.
Increased intracranial pressure caused by communicating hydrocephalus may be the result of long standing hydrocephalus, increased CSF production, decreased CSF absorption, or tonsillar herniation. Streptococcal infection produces many virulence factors and toxins.
A clinical and epidemiologic study for the last 45 years reported the incidence of GAS meningitis in pediatric population 0.06 cases per 100,000 children per year. The case fatality rate was 43%. Neonatal age and the presence of an associated toxic shock syndrome were identified as risk factors for death. A distant focus of infection was present 36% in the Brazilian case series which were more than half of the patients in the literature. No single virulence determinant could be associated with death.5
We performed PubMed online search for English literature for Streptococcal pyogenes meningitis in children 0-17 years and under. We used terms “Group A Streptococcal meningitis”, “Streptococcal pyogenes meningitis”, “with and without” and “CNS complications” and “acute and chronic hydrocephalus” with a variable combination. Bibliography of the reports was searched for additional information.
The results of our literature search with CNS complications are shown in Table 1.
With the exception of 7 neonates, the mean age for children with CNS complications was 6.1 years. Of 44 children, 30 (68%) children had a known focus of infection. In fourteen (32%) children the focus of infection was unknown
1 6 d/M Erysipelas Seizures
2 8 d/M Unknown Seizures
3 13 d/F Cellulitis of both Focal Seizures Death 19848
4 14 d/F Cystic gingival Seizures,
5 24 d/F Unknown Seizures, Brain Ab- Death 200010
6 26 d/M Paronychia Seizures, Communicat- No sequellae 19848
7 28 d/M Unknown Seizures and
8 1 m/F Unknown Seizures
9 1 m/F Unknown Meningitis and
10 1.5 m/M Otitis Media Seizures 11 NA/NA Unknown NA
12 2 m/M Tonsillitis Seizures and Subdural Moderate Motor Deficit, 199815
13 2 m/NA Ulcerated and Infantile spasm and Encephalomalacia, 200416
14 2.5 m/M None Seizures and SIADH Seizure Disorder, Optic 19839
15 2.5 m/F Infected heman- Seizures
16 2.5 m/M Pharyngitis Focal Seizures Hearing loss 198818
17 3 m/M Infected BCG Generalized Seizures No sequelae 200019
18 3 m/F Chicken pox Hydrocephalus No sequelae 200320
19 7 m/F None Seizures
20 11 m/NA Unknown NA
21 17 m/M Otitis media Generalized Seizure, No sequelae 200623
22 18 m/M Dermal sinus of Recurrent Meningitis No sequelae 197624
No sequellae 19666
Death 19677
feet No sequellae 19839 lesion
scesses
ing Hydrocephalus No sequellae 200811 Brain Abscess Death 19677
a 12 Multiple NAa 198812 Brain Abscess Severe Motor Deficits 197913 Hydrocephalus, Poren- 198614 cephalic cyst
effusions Cystic Hygroma
Infected heman- Seizures Infantile spasms,Profound gioma Developmental Delay
atrophy, Microcephaly, Psychomotor Retardation Psychomotor Retardation 198717 gioma
cscar
Death 199221
Bilateral Subdural Hy- 197622 groma
Brain Abscess
nose and Otitis Media
23 2.5 yr/M None Seizures and Increased Death 200125
24 3 yr/M Otitis media Seizures
25 3.5 yr/M Pharyngitis and Cerebral Abscess Left sensorineural hearing 201227
26 4 yr/M Otitis media SIADH 27 4.5 yr/NA Cochlear implan- Hearing Loss
28 5 yr/NA Right Mastoiditis Right Facial Nerve No sequelae 200729
29 6 yr/NA Unknown NA 30 6 yr/F Pharyngitis Communicating hydro- No sequelae Present
31 8 yr/M Otitis media, Seizures
32 8 yr/M Sore throat Seizures 33 8 yr/F Unknown Coma 34 8 yr/F Unknown (Toxic Shock-Like Syn- Death 199431
35 8 yr/M Otitis Media, Subdural effusion, Headaches and Attention 201032
36 10 yr/M Unspecified ill- Brain Abscess
37 11 yr/NA Multiple Otitis Cranial Nerve III Cranial Nerve III Palsy, 200134
38 12 yr/F Pharyngitis, Coma and
39 12 yr/F None Brain Abscess
40 13 yr/F Sinusitis Seizures
41 14 yr/NA Posttraumatic Cerebrospinal fluid leak Severe Psychomotor 199037
42 15 yr/M Occipital Fracture Cerebrospinal fluid leak No sequelae 199938
43 15 yr/F Tonsillitis Seizures and Cerebral No sequelae 201032
44 17 yr/M Pharyngitis, Cavernous Sinus No sequelae 199938
Table 1. Lists increasing age-based case Reports of central nervous system (CNS) complicationsin children with GroupA Streptococcal meningitis since 1966 [6-38]. d: days; M: male; F: female; m: month; yr: year ; aNA, data not available; bSIADH, Syndrome of Inappropriate Anti-Diuretic Hormone; cBCG, Bacillus Calmette–Guérin The table is based on the results of our search on PubMed for English literature for by Streptococcal A meningitis. Our search identified 40 studies with 43 children, excluding the current report (Case # 30). Twenty seven (68%) studies were isolated
case reports. Six reports reference number 7, 9, 22, 32, 38 and 8, had2 cases each. The study number8 included6 cases. The children who presented with menigeal manifestations of headache, photophobia, and neck stiffness, but no CNS complications were excluded from this report.
intracranial pressure Partial blindness, Deaf- 198426 ness, Mental Retardation
Mastoiditis Loss No sequelae 19839 Language Delay 200528 tation
Palsy Cranial Nerve IV Palsy 197622
cephalus Neuropsychological deficit 198130 Pneumonia No sequelae 19839 No sequelae 19839
drome)
Pharyngitis Hemiparesis, and Deficit Disorder Seizures No sequelae (abscess re- 200133 ness quired surgical drainage )
media Palsy Subdural Hygroma Persistent 19839 Otitis media Hemianopia Hemianopia No sequelae 198835
Moderate Motor Deficit 199236
meningitis Retardation
abscess
sinusitis Thrombosis
children age 17years and under with central nervous system complications caused
or was not described. Amongst children with known focus, 13 (43%) had otitis media and/or pharyngitis. Tonsillitis, infected hemangioma, cellulitis, or erysipelas, and 1 patient had sore throat. Other minor but significant foci of infection included skull fracture, infected dermal sinus, and mastoiditis.
The most common acute CNS complication secondary to Streptococcal meningitis was seizure 24/44 (55%) followed by brain abscess 8/44 (18%). Other acute complications included hydrocephalus, CSF leak, meningitis, cranial nerve palsy and subdural effusion, except one case, the complication was unknown.
Regarding outcome, 18/44 (41%) patients including ours had no sequelae. 10/44 (23%) cases developed a slowing of speech or physical movement with or without a decreased cognitive ability, 3/44 (7%) cases with hearing loss, 2/44 (4.5%) cases with cranial nerve palsy, 2/44 (4.5%) cases developed partial or complete blindness, only 1/44 (2.3%) developed chronic hydrocephalus. In one case the outcome was unknown (See Bar Graph).
In addition, 7/44 (16%) children died from complications of Group A Streptococcus meningitis, secondary to cardio respiratory insufficiency, Waterhouse Friderichsen syndrome, and Toxic shock-like syndrome. It is unclear why previously healthy children with Group A streptococcus meningitis developed significant CNS complications?
Bar Graph: The bar graph shows age-based CNS complications of GroupA Streptococcal meningitis in children age 0-17 years. Others complications reported were meningitis, subdural effusions, infantile spasms, Syndrome of Inappropriate Antidiuretic Hormone secretion (SIADH), increased intracranial pressure, hearing loss, third and forth cranial nerve palsies, coma, and cavernous sinus thrombosis. Seizure, the most common CNS complication, occurred between births to 12 months. Hydrocephalus and cerebral abscess occurred between births to 10 years. Cerebrospinal fluid leak exclusively occurred between 10 to17 years of age. There were no reports of intracranial hemorrhage secondary to
In contrast to our case, two previous cases of acute hydrocephalus were reported during early infancy. Acute onset hydrocephalus in our case was likely due to the combined effect of untreated prolonged pharyngitis, and initial delay in diagnosis and treatment. We suggest that a high CSF protein together with pleocytosis impeded absorption of CSF by arachnoid villi at the superior sagittal sinus. Such functional blockage causing chronic hydrocephalus has been reported in the presence of proteinaceous and cellular debris in CSF39 and also after intracranial hemorrhage.40
Alternatively, the term “communicating hydrocephalus” which could be acute or chronic is defined to mean that the CSF within the ventricles communicating with subarachnoid space CSF. But the CT scan panel A shows CSF within the ventricles and an obliterated subarachnoid space. Arguably, there is no evidence that the ventricles and subarachnoid space are in communication with each other (a broader definition of obstructive hydrocephalus), and therefore, this is not “communicating hydrocephalus”. Additionally, hydrocephalus is not caused by increased CSF production or by tonsillar herniation. Instead, in our case, the non-communicating hydrocephalus is caused by ventriculitis resulting in obstruction to the outflow of CSF through the fourth ventricle.
Our case illustrates that Group A Streptococcal meningitis induced acute hydrocephalus is rare. A timely instituted surgical intervention may prevent other CNS complication. The review of published cases reveals that central nervous system complications including hydrocephalus are
rare. Since prompt antibiotic therapy can minimize risk for acute CNS complications, prompt identification and emergent medical and neurosurgical interventions should be undertaken for an optimum outcome in children with Group A Streptococcal meningitis.
Author thanks Jeffery Maisels, MD for his review of this manuscript.
The authors have no financial considerations to disclose or competing interests in relation to this article.