Surya N. Gupta, MD
Associate Professor, Department of Pediatric Neurology, Section of Child Neurology, Women’s and Children’s Hospital, Charleston Area Medical Center 415 Morris Street, Suite 300, Charleston, WV 25301, USA; Tel. 304-388-6441; Fax: 304-388-6445; E-mail: suryangupta@rediffmail.com
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 been reported in 20% of infants and children. Acute Central Nervous System (CNS) complications including hydrocephalus are not unique to Stretococcus pyogenes causing meningitis. They have been reported in children with viral and other bacterial meningitis.3
We report an unusual association of acute hydrocephalus in a 6-year-old girl with 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 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.
Hospital Course
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).
Figure 1: An axial Computerized Tomography (CT) of the brain at the fourth ventricle level shows communicating hydrocephalus, figure A was obtained before and figure B was after surgical placement of a ventricular drain which is not seen at this level. A. Contrasted CT scan of the brain 10 hours after initiating parenteral antibiotics shows a global enlargement of the ventricular system. Note the complete effacement of the basal cisterns.
B. A follow up non-contrasted CT scan of the brain 8 hours after surgical placement of a 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.
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 discharged on the 23rd day of admission with no focal motor, cerebellar, or sensory deficits.
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.4
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
Literature Search and the Results
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.
| Table 1: Lists increasing age-based case Reports of central nervous system (CNS) complicationsin children with Group A Streptococcal meningitis since 1966 [6-38]. | |||||
| Case # | Age/ Sex | Infection Focus | Central Nervous System Complication | Year [Reference] | |
| Acute | Chronic or Outcome | ||||
| 1 | 6 d/M | Erysipelas | Seizures | No sequellae | 19666 |
| 2 | 8 d/M | Unknown | Seizures | Death | 19677 |
| 3 | 13 d/F | Cellulitis of both feet | Focal Seizures | Death | 19848 |
| 4 | 14 d/F | Cystic gingival lesion | Seizures, | No sequellae | 19839 |
| 5 | 24 d/F | Unknown | Seizures, Brain Abscesses | Death | 200010 |
| 6 | 26 d/M | Paronychia | Seizures, Communicating Hydrocephalus | No sequellae | 19848 |
| 7 | 28 d/M | Unknown | Seizures and
Brain Abscess |
No sequellae | 200811 |
| 8 | 1 m/F | Unknown | Seizures | Death | 19677 |
| 9 | 1 m/F | Unknown | Meningitis and Multiple Brain Abscess | NAa | 198812 |
| 10 | 1.5 m/M | Otitis Media | Seizures | Severe Motor Deficits | 197913 |
| 11 | NA/NA | Unknown | NA | Hydrocephalus, Porencephalic cyst | 198614 |
| 12 | 2 m/M | Tonsillitis | Seizures and Subdural effusions | Moderate Motor Deficit, Cystic Hygroma | 199815 |
| 13 | 2 m/NA | Ulcerated and Infected hemangioma | Infantile spasm and Seizures | Encephalomalacia,
Infantile spasms,Profound Developmental Delay |
200416 |
| 14 | 2.5 m/M | None | Seizures and SIADH | Seizure Disorder, Optic atrophy, Microcephaly, Psychomotor Retardation | 19839 |
| 15 | 2.5 m/F | Infected hemangioma | Seizures
|
Psychomotor Retardation | 198717 |
| 16 | 2.5 m/M | Pharyngitis | Focal Seizures | Hearing loss | 198818 |
| 17 | 3 m/M | Infected BCG cscar | Generalized Seizures | No sequelae | 200019 |
| 18 | 3 m/F | Chicken pox | Hydrocephalus | No sequelae | 200320 |
| 19 | 7 m/F | None | Seizures | Death | 199221 |
| 20 | 11 m/NA | Unknown | NA | Bilateral Subdural Hygroma | 197622 |
| 21 | 17 m/M | Otitis media | Generalized Seizure,
Brain Abscess |
No sequelae | 200623 |
| 22 | 18 m/M | Dermal sinus of nose and Otitis Media | Recurrent Meningitis | No sequelae | 197624 |
| 23 | 2.5 yr/M | None | Seizures and Increased intracranial pressure | Death | 200125 |
| 24 | 3 yr/M | Otitis media | Seizures | Partial blindness, Deafness, Mental Retardation | 198426 |
| 25 | 3.5 yr/M | Pharyngitis and Mastoiditis | Cerebral Abscess | Left sensorineural hearing Loss | 201227 |
| 26 | 4 yr/M | Otitis media | SIADH | No sequelae | 19839 |
| 27 | 4.5 yr/NA | Cochlear implantation | Hearing Loss | Language Delay | 200528 |
| 28 | 5 yr/NA | Right Mastoiditis | Right Facial Nerve Palsy | No sequelae | 200729 |
| 29 | 6 yr/NA | Unknown | NA | Cranial Nerve IV Palsy | 197622 |
| 30 | 6 yr/F | Pharyngitis | Communicating hydrocephalus | No sequelae | Present |
| 31 | 8 yr/M | Otitis media, Pneumonia | Seizures
|
Neuropsychological deficit | 198130 |
| 32 | 8 yr/M | Sore throat | Seizures | No sequelae | 19839 |
| 33 | 8 yr/F | Unknown | Coma | No sequelae | 19839 |
| 34 | 8 yr/F | Unknown | (Toxic Shock-Like Syndrome) | Death | 199431 |
| 35 | 8 yr/M | Otitis Media, Pharyngitis | Subdural effusion, Hemiparesis, and
Seizures |
Headaches and Attention Deficit Disorder | 201032 |
| 36 | 10 yr/M | Unspecified illness | Brain Abscess | No sequelae (abscess required surgical drainage ) | 200133 |
| 37 | 11 yr/NA | Multiple Otitis media | Cranial Nerve III
Palsy |
Cranial Nerve III Palsy, Subdural Hygroma | 200134 |
| 38 | 12 yr/F | Pharyngitis,
Otitis media |
Coma and
Hemianopia |
Persistent
Hemianopia |
19839 |
| 39 | 12 yr/F | None | Brain Abscess | No sequelae | 198835 |
| 40 | 13 yr/F | Sinusitis | Seizures | Moderate Motor Deficit | 199236 |
| 41 | 14 yr/NA | Posttraumatic meningitis | Cerebrospinal fluid leak | Severe Psychomotor Retardation | 199037 |
| 42 | 15 yr/M | Occipital Fracture | Cerebrospinal fluid leak | No sequelae | 199938 |
| 43 | 15 yr/F | Tonsillitis | Seizures and Cerebral abscess | No sequelae | 201032 |
| 44 | 17 yr/M | Pharyngitis, sinusitis | Cavernous Sinus
Thrombosis |
No sequelae | 199938 |
| 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 children age 17years and under with central nervous system complications caused 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, had 2 cases each. The study number 8 included 6 cases. The children who presented with menigeal manifestations of headache, photophobia, and neck stiffness, but no CNS complications were excluded from this report. |
|||||
Clinical Demography
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 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.
Central nervous system complication
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.
Neurological Outcome
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?
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.
Bar Graph: The bar graph shows age-based CNS complications of Group A 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 Group A w.
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.
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