Suboccipital extracranial and intracranial abscess of a neonate: an exceedingly rare occurrence
- Leopoldo Mandic Furtado 1 , 2,
- José Aloysio Costa Val Filho 3,
- Rayane Toledo Simas 3 and
- João Paulo Uviera Ferreira 4
- 1 Neurosurgery, Fundacao Hospitalar do Estado de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
- 2 Pediatric Neurosurgery, Hospital Vila da Serra, Nova Lima, Minas Gerais, Brazil
- 3 Pediatric Neurosurgery, Vila da Serra Hospital, Nova Lima, Minas Gerais, Brazil
- 4 Neurosurgery, Beneficência Portuguesa de Ribeirão Preto, Sao Paulo, Brazil
- Correspondence to Leopoldo Mandic Furtado; lmandicster@gmail.com
Abstract
Intracranial abscess is a life-threatening disease that is uncommon in paediatric populations. Although there have been few reports in the literature, some aetiologies, such as mastoiditis and sinusitis, have been proposed. The pathophysiology is not completely known, and there are no data regarding the long-term follow-up of these patients. Herein, we present a case of a newborn affected with a mass in the suboccipital region due to an extracranial and intracranial abscess that had no clear association with infections except for a transient fever episode 1 month earlier. Isolation of Staphylococcus aureus from an open-surgery sample identified the cause of the mass. The patient achieved excellent recovery with no recurrence even after 8 years of follow-up. To our knowledge, this rare pattern of infection highlights the importance of early diagnosis in combination with a surgical approach as an effective diagnosis and treatment approach that provided a good outcome.
Background
Intracranial abscess is a rare condition affecting children. Reportedly, the subdural space is the preferred site for identifying this infection. Some risk factors have been described, such as prior sinusitis, otitis media, dermoid cyst and head trauma. Another unexpected factor associated with these infections was iatrogenic complications after development of a line vein on the scalp.1–7 Currently, the extradural space is considered the rarest region where such intracranial abscesses can occur, and the classic entity is referred to as a Pott’s puffy tumour is the most recognisable example in which the abscess simultaneously affects the extradural and extracranial spaces on the frontal region as a complication of sinusitis.3 However, this kind of abscess has not been described in the posterior fossa previously.
We report a case of a newborn with a mass in the suboccipital region caused by an extracranial and intracranial abscess that should be considered as an important differential diagnosis of a suboccipital cutaneous mass in newborns.
Case presentation
A 2-month-old boy was brought to our hospital due to a rapidly growing oedematous formation in the suboccipital region. The mother reported that the child had a 3-day fever 1 month earlier without an identifiable cause, and spontaneous remission was observed. No other symptoms and no use of antibiotics were reported. Moreover, this boy had no history of head trauma, congenital diseases, otitis or skin infections. Additionally, the mother reported an uneventful pregnancy with a delivery at full term and a weight of 3500 g.
On physical examination, the patient showed a soft mass occupying the suboccipital region without pain or skin abnormalities while maintaining normal behaviour without irritability (figure 1). In addition, no compromise of the child’s motor function or feeding capacity was noted. During observation, the mass enlarged considerably, so we performed neuroimaging.
A soft mass (thick white arrow) not associated with pain or skin alterations was observed in a physical examination of the child (A). The mass was positioned on the suboccipital region (pointed square) below the inion and deep into the suboccipital muscles (thin write arrow) displayed on skull CT with three-dimensional reconstruction allowing the observation of the soft structures of the suboccipital region (B). A small hole (thin black arrow) on the right suboccipital region is revealed on skull CT. OC, occipital region. (*), External occipital protuberance (Inion)
Investigations
Head CT with three-dimensional reconstruction was performed, which showed an intracranial component in continuity with an extracranial cystic aspect. Moreover, fluid in the intracranial space was compressing the right cerebellar hemisphere, which appeared to have caused erosion of the occipital bone (figures 1 and 2). To obtain more information, MRI was performed and showed peripheral gadolinium enhancement (figure 3).
Skull CT scan without contrast depicting a mass on the posterior fossa that has compressed the cerebellum and has hypodense (*) and isodense (thin arrow) intracranial and extracranial components (thick arrow) in the sagittal (A) and axial (B) views of the parenchyma phase. The bone window shows a decreased width of the right suboccipital bone (double-thin arrows) in (C).
Sagittal gadolinium-enhanced MRI showing a peripherally enhanced lesion (thick white arrow) (A). The free communication (thin black arrow) between the extracranial and intracranial components is seen on MRI sagittal T2-weighted short TI inversion recovery (STIR) imaging (B). The lesion (*) shows enhancement on diffusion-weighted imaging (C) and no signs of bleeding are observed on axial STIR sequence (D).
Blood sampling of the suboccipital mass with subsequent laboratory testing showed the following findings: erythrocyte sedimentation rate, 83 mm3/hour (reference=10 mm3/hour); erythrocyte count: 2.67×106/mm3 (reference=3.8×106/mm3); haemoglobin, 76 g/L (reference=11.5 g/dL); platelet count, 0.451/L (reference=150–400/mm3) and leucocyte count, 9.130 /mm3 (reference=5–17 000/mm3). The relative counts were as follows: neutrophils, 46.3%; lymphocytes, 42.8%; monocytes, 10%; eosinophils, 0.9% and C reactive protein, 3.9 mg/dL (reference=<1 mg/dL).
Differential diagnosis
First, considering the rapid growth of the mass without other patient symptoms, the main diagnostic hypothesis was subcutaneous infection. However, the patient had no skin inflammatory symptoms.
Second, posterior fossa lesions affecting children usually have a neoplastic nature until proven otherwise. Considering this premise, our other hypothesis was a neoplastic disease, such as haemangiopericytoma and pilocytic astrocytoma. However, the extracranial component led us to suppose unusual bone tumours, such as osteosarcoma, or tumours that arise from muscles, such as rhabdomyosarcoma.
Finally, the infection hypothesis was considered because of the rapid growth of the lesion and its unusual behaviour, which compressed the surface of the dura and cerebellum and led to erosion of the occipital bone.
Treatment
The open surgical approach was chosen, and under general anaesthesia, the boy was placed on the ventral decubitus position, and a linear incision was made above the mass. A massive collection of pus was observed after the suboccipital muscles were incised, and a syringe was used to collect an aliquot of the infectious fluid (figure 4).
The neurosurgical approach performed drainage of the extracranial component of the abscess on the suboccipital region (left), and the intracranial component was assessed through the bone erosion (*); after that, irrigation and curettage of the capsule adhering to the dura were performed (right).
After the extracranial pus was drained, a small hole on the right side of the suboccipital bone was confirmed, and the intracranial component was evacuated. Curettage was performed on the surface of the dura to remove the residual inflammatory cells, and a dural tear was avoided, which minimised dissemination of the infection to the central nervous system. A small bone sample was removed from the border of the skull hole for analysis. No drains were inserted, and non-absorbable stitches were used to close the skin.
Postoperatively, the culture stain of the pus and bone samples was positive for Staphylococcus aureus, and the patient was administered antibiotic therapy with vancomycin for 40 days without complications.
Outcome and follow-up
After discharge from the hospital, the patient achieved normal growth and development milestones without neurological alterations. No abscess recurrence was observed during the 8-year follow-up, and no cerebellar signs, such as ataxia, dysdiadochocinesia, speech alterations or dysmetria, were observed throughout the follow-up, and the boy had acceptable school performance.
Contrast-enhanced CT performed on the final follow-up showed total recovery of cerebellar morphology without parenchymal abnormalities (figure 5). Furthermore, the eroded bone was not ossified.
Eight years after the procedure, skull CT without contrast shows the recovery of cerebellar compression without parenchymal or dural (thick white arrow) abnormalities (A), and NO abnormal contrast enhancement is observed (B). Normal pneumatisation of right mastoid cells (double-thin arrows) is observed (C) and there is no ossification on the small hole observed on CT with three-dimensional reconstruction (*).
Thus, the cryptogenic suboccipital abscess hypothesis was confirmed.
Discussion
Intracranial abscess affecting the paediatric population is a rare event, and infections occupying the extradural space are especially rare with a paucity of studies reported in the literature.8–13 Legrand et al 4 conducted a retrospective study in France to assess the epidemiology and outcomes of paediatric neurosurgery, and during the 13-year study period, 38 patients with intracranial abscesses were considered for analysis. Five of the 38 patients had extradural empyema, whereas the remaining 33 patients presented with fluid collection in the subdural space. Two patients had infections located in the posterior fossa, and most patients (34) underwent surgery. Regarding aetiology, the five patients with the extradural variant presented with fewer neurological deficits and had experienced a prior infection, such as otitis or sinusitis. In the present case, no causes of infection were identified, so we surmised that the transient fever 1 month before the onset of the abscess could have been associated with bacteraemia, which could have implanted bacteria into the bone surface in a haematogenous path. The local osteomyelitis results in the suppurative infection may have progressed simultaneously intracranially and extracranially.
Similar to the anatomical presentation of the abscess in our case in which the pus accumulated simultaneously in the extradural and extracranial compartments through bone erosion, the Pott’s puffy tumour is a type of suppurative infection that originates from a frontal sinusitis that progresses to the frontal bone leading to bone erosion and accumulates inflammatory cells extracranially and intracranially.3 14 However, the pathophysiology of the Pott puffy tumour differs from that in the present case in which the presumed apparent cause was haematogenous dissemination after bacteraemia and implantation in the suboccipital bone. Furthermore, this pattern of pus accumulation affecting the posterior fossa has been described in the literature as an iatrogenic complication due to the vein line on the scalp of the occipital region and is evaluated by compression of the torcula2 and parietal region.12 No other causes have been proposed for this kind of abscess, which reinforces the rarity of this anatomical behaviour of the abscess.
The suboccipital region (figure 1), defined as the occipital bone located below the inion (the point at which the superior nuchal lines merge in the external occipital protuberance), constitutes the posterior limit of the posterior fossa, and the growth of masses inside this compartment has enormous potential for neurological complications due to the compression of structures, such as the cerebellum, fourth ventricle and brain stem, leading to cerebellar compromises, blockage of the fourth ventricle, hydrocephalus and even coma and death. Therefore, paediatricians should be aware of this risk.
The patient in our case was remarkable because he did not show any recurrence of this infection during the long-term follow-up, which corroborates the lack of a propensity of this patient to develop otitis or sinusitis. Interestingly, this patient did not have epilepsy or other signs of brain compromises due to infection, which is consistent with the effectiveness of the early diagnosis and surgical approach. Furthermore, this case sheds light on the physiopathology of the potential risk of transient bacteraemia affecting newborns and the potential prevention using antibiotic therapy, which we did not provide because of the spontaneous remission of the fever reported by the mother.
Therefore, given the importance of early diagnosis of this type of abscess, paediatricians should be aware of the meaningful role of neuroimaging in investigating a skull mass even if the patient does not show neurological signs. Neuroimaging can avoid underestimating this physical finding as a simple skin infection.
Learning points
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Intracranial abscess in children is a rare condition, and the extradural variation is very rarely reported in the literature.
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The most recognisable risk factors are prior sinusitis, otitis and head trauma, which lead to infection due to contiguity.
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In the patient in this case, transient bacteraemia was the potential cause of osteomyelitis and progression to intracranial infection.
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The occurrence of a soft mass on the skull along with inflammatory symptoms could be associated with intracranial infection, and brain imaging is mandatory.
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Early diagnosis and treatment are critical for successful treatment and avoidance of complications, such as brain abscess and brain injury.
Ethics statements
Patient consent for publication
Footnotes
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Contributors The design of this case report as well as the obtaining the consent of patient and the description per se was performed by LMF. RTS and JPUF were responsible for the write the manuscript and JACVF performed the revision before submission.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
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