Case Report
Pediatr Neurosurg 2001;35:94–98

Received: September 13, 2000
Accepted: May 14, 2001

Ruptured Intracranial Mycotic Aneurysm:
An Unusual Infectious Complication following
Craniofacial Surgery
Christopher M. Uchiyama Douglas L. Brockmeyer W. Bruce Cherny
John J. Jacobs Marion L. Walker
Department of Neurological Surgery, University of Utah School of Medicine and Primary Children’s Medical Center,
Salt Lake City, Utah, USA

Key Words
Mycotic aneurysm W Infectious aneurysm W Craniofacial
surgery W Apert syndrome

Abstract
The case of a child with Apert syndrome is presented in
which the development and rupture of an intracranial
mycotic aneurysm occurred secondary to multiple infectious complications following craniofacial surgery. An
endovascular procedure was utilized in an attempt to
embolize the aneurysm and parent vessel. The patient
recovered from her infections, but retained a residual
right hemiparesis and left cranial nerve III palsy at the
time of discharge. To our knowledge, this is the first
report of a mycotic aneurysm developing after a craniofacial procedure. Risk factors leading to aneurysm formation in this case are presented, as well as a literature
review of neurological complications following craniofacial surgery.
Copyright © 2001 S. Karger AG, Basel

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Introduction

The term mycotic aneurysm refers to a saccular or fusiform-like arterial dilatation resulting from an infectious
process affecting an arterial wall. Mycotic aneurysms tend
to be located on vessels distal to the circle of Willis with
branches of the middle cerebral artery most commonly
affected [1–4]. They presumably arise as the result of one
of the following four mechanisms: lodging of septic emboli into the lumen of an intracranial vessel, hematogenous seeding from a distant infection to a vessel via the
vasa vasorum or rete vasorum, local invasion of an intracranial vessel by direct spread of an adjacent infection, or
direct microbial contamination of an arterial wall following penetrating trauma [3, 5–11]. Invasion of the affected
vessel by a pathogen subsequently causes weakening or
destruction of the elastica and media resulting in an aneurysm defect [12]. Contrary to what its name may suggest,
bacterial rather than fungal etiologies of mycotic aneurysms predominate with Staphylococcus and Streptococcus species being the most common pathogens associated
with this disease [2, 13–15].
In this report, risk factors leading to mycotic aneurysm
development following a craniofacial procedure are discussed. The endovascular approach for treating this aneurysm is described and a brief literature review of neurological complications following craniofacial surgery will
be presented.

Christopher M. Uchiyama, MD, PhD
Department of Neurological Surgery, University of Utah
50 North Medical Drive, Suite 3B409 SOM
Salt Lake City, UT 84132 (USA)
Tel. +1 801 581 6908, Fax +1 801 581 4385

Case Report
A 5-year-old female with Apert syndrome presented with midface
deficiency and craniosynostoses. She underwent a differential frontoorbital, intracranial LeFort III and LeFort I advancement for correction of her craniofacial abnormalities. Intravenous cefuroxime
(25 mg/kg every 8 h) was administered throughout the case. Her
intraoperative course was complicated by a maxillary artery laceration causing hemorrhage requiring a 4,000-ml transfusion of whole
blood. Additionally, the loss of an adequate endotracheal airway
necessitated the emergent placement of a tracheostomy during surgery. Bilateral subgaleal Jackson-Pratt drains were placed at the conclusion of the case. Postoperatively, the patient developed a bleeding
diathesis which resolved following administration of fresh-frozen
plasma within the first 24 h after surgery. The patient remained on
cefuroxime for several days postoperatively.
On postoperative day 4, the patient became febrile to 39.5 ° C and
blood cultures obtained at that time grew Candida albicans. Amphotericin B (0.9 mg/kg) was started but she continued to remain febrile
over the next few days despite negative cultures from blood, urine
and pulmonary sources. A contrast-enhanced computer tomography
(CT) scan was then performed on postoperative day 6 which showed
a left-sided subgaleal fluid collection with minimal dural enhancement. Due to concerns of an infected subgaleal hematoma and possible meningitis, the subgaleal hematoma was tapped. Culture results
demonstrated the presence of Streptococcus viridans and group G
Streptococcus species. The cefuroxime was discontinued and the
patient was started on broad-spectrum antibiotics.
The patient was taken back to the operating room on postoperative day 7 where she underwent an evacuation of her infected subgaleal fluid collection, debridement of the operative site and removal of
an infected frontal bone flap. Cultures of the infected subgaleal
hematoma obtained at surgery revealed a multitude of aerobic,
anaerobic and fungal organisms. Besides the two previously cultured
Streptococcus species, other organisms including Escherichia coli,
Candida glabrata, Veillonella species, Bacteroides gracillus and Peptostreptococcus organisms were also identified. Postoperatively the
patient was placed on appropriate intravenous broad-spectrum antibiotics based on organism sensitivities and on postoperative days
11/41 also began a 10-day course of continuous bacitracin irrigations
(500 units/ml at 50 cm3/h) to the operative site via bilateral, subgaleal Jackson-Pratt drains. Following the wound debridement procedure, the patient had episodes of hemodynamic instability due to
septic shock requiring fluid resuscitation and pressor support from
which she was eventually withdrawn. The patient continued to spike
fevers and sequential workups demonstrated a C. albicans urinary
tract infection. Additionally, a recurrence of C. albicans line sepsis
was treated appropriately with combinations of antifungal agents.
Despite these infectious episodes, the patient remained neurologically stable and continued to follow commands with symmetrical
motor function. A follow-up head CT scan on postoperative days
13/61 showed no new abnormalities. On postoperative days 16/91,
the patient acutely developed a fixed dilated left pupil, was unresponsive to commands, and had a dense right-sided hemiparesis. An
emergency head CT scan showed a 5 ! 5 ! 5 cm left temporal lobe
intracerebral hematoma with left lateral ventricle compression and

Fig. 1. CT scan performed at the time of acute neurological deterioration demonstrates large, left temporal intraparenchymal hemorrhage from ruptured mycotic aneurysm causing mass effect and
uncal herniation.

The numerator denotes postoperative day from initial craniofacial procedure and the denominator indicates postoperative day from wound debridement procedure.

mass effect causing uncal herniation (fig. 1). The patient was immediately taken to the operating room where she underwent a craniotomy and evacuation of the hematoma. Due to the patient’s multitude
of antecedent infections and location of clot on CT scan, a ruptured
intracranial mycotic aneurysm as the etiology of her hemorrhage was
suspected. This was subsequently confirmed on a postoperative cerebral angiogram showing a 4- to 5-mm aneurysm arising from an early
anterior temporal branch of the left middle cerebral artery (fig. 2a).
An attempt to embolize the vessel was then undertaken with the goal
of occluding the aneurysm and the proximal anterior temporal artery
branch.
A 5-Fr Berenstein catheter was positioned into the left internal
carotid artery, and under live subtraction with ‘road-mapping’, a
Tracker 10 microcatheter was advanced into the vessel supplying the
aneurysm. A suspension of 0.5 cm3 of N-butyl-cyanoacrylate glue
mixed with 0.25 cm3 of Ethiodol and a small amount of tantalum
powder for opacification was then injected. During the procedure,
occlusion of the left middle cerebral artery occurred due to an intimal
flap and/or vasospasm. Multiple attempts to angioplasty the affected
vessel were unsuccessful. However, at the conclusion of the procedure, a repeat angiogram demonstrated spontaneous recanalization
of the proximal aspect of the middle cerebral artery and no filling of
the aneurysm. Therefore, the presumed mechanism for middle cerebral artery occlusion appeared to be acute vasospasm and not dissection or emboli. An angiogram performed the following day demonstrated a patent middle cerebral artery and obliteration of the vessel
supplying the lesion with no detectable aneurysm (fig. 2b). No additional aneurysms were noted in the remainder of the cerebral vascu-

Mycotic Aneurysm

Pediatr Neurosurg 2001;35:94–98

1

95

lature. Early in the postembolization period, the patient developed a
left hemispheric infarction in the distribution of the left middle cerebral artery. The patient eventually recovered to her baseline level of
cognitive function. She continued to make clinical improvements
daily, but retained a left cranial nerve III palsy and a right hemiparesis prior to her discharge home.

Discussion

Fig. 2. a Cerebral angiogram following left internal carotid artery

injection reveals 4- to 5-mm saccular aneurysm arising distal on an
early anterior temporal branch of the left middle cerebral artery
(black arrow). b Postembolization angiogram showing obliteration of
mycotic aneurysm and distal segment of parent vessel (double
arrows). Following procedure, bilateral anterior cerebral arteries
showed loss of perfusion via left carotid artery (heavy black arrow),
but showed adequate cross-filling from the contralateral side (not
shown).

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Pediatr Neurosurg 2001;35:94–98

In this patient, the multiplicity of bacterial and fungal
infections arising in the subgaleal compartment, lungs,
urinary tract and blood of this patient, was a major factor
in suspecting a ruptured mycotic aneurysm at the time of
her acute neurological changes. Several factors contributed to the development of infections and a mycotic aneurysm in this patient. First, the large intraoperative blood
loss and multiple blood transfusions she received placed
her in an immunocompromised state predisposing her to
subsequent postoperative infections. Secondly, the craniofacial procedure performed violates the mucoperiosteal barrier, exposes the intracranial compartment to
oronasal flora, and increases the duration of surgery.
These are known to be associated with a higher risk of
infection compared to staged procedures where extra- and
intracranial compartments are generally kept separate,
and the duration of surgery is shorter [16]. The need for
an emergent intraoperative tracheostomy further contributed to exposure of intracranial compartments to microbial flora of the upper airway. Not surprisingly, organisms
normally found in the oro- and nasopharynx were isolated
from the subgaleal cultured specimens.
In reviewing the radiographic findings and the favorable location of the aneurysm, treatment options considered were continued antibiotic therapy plus either surgical management by ligation of the parent vessel proximal
to the aneurysm, or endovascular embolization of the
aneurysm and proximal artery. The latter approach was
utilized but the procedure was complicated by acute vasospasm. The aneurysm and affected parent vessel were
occluded and patency of the middle cerebral artery was
re-established as demonstrated by the angiogram obtained the following day. The patient subsequently convalesced from her left middle cerebral artery stroke and
retained the cranial nerve and motor deficits which were
preoperatively observed at the time of her acute hemorrhage.
A number of general, retrospective reviews address
complications following craniofacial surgical procedures.
Murray et al. [17] summarized a 20-year experience on
385 craniofacial operations performed on 264 patients for

Uchiyama/Brockmeyer/Cherny/Jacobs/
Walker

a variety of congenital deformities. An overall complication rate of 30% was reported, mostly due to infection or
loss of bone grafts and 1 patient (0.38%) died from respiratory arrest. Neurological complications encountered included cerebrospinal fluid (CSF) leaks in 6 patients
(2.2%), and unilateral blindness in 2 patients (0.75%).
Matthews [18] reported his complications following
craniofacial surgery on 46 patients over a 6-year period.
An overall complication rate is unable to be ascertained
from this report. However, of the neurological complications described, persistent CSF leaks were seen as late
complications in 3 patients, with subsequent development of meningitis in 1 of these patients. Additionally,
anosmia in 1 patient, and diabetes insipidus in 2 patients
were observed. Cerebral edema was the reported cause of
death in 1 of 2 mortalities encountered, and was apparently due to SIADH (syndrome of inappropriate secretion
of antidiuretic hormone) from brain retraction.
Siegel and Israele [19] reported their experience in 219
children undergoing either oromaxillary or craniofacial
surgery for congenital anomalies over a 10-year period. Of
the infectious complications involving the central nervous
system per se, 1 patient (0.45%) developed an epidural
abscess, 1 patient (0.45%) developed a frontal abscess,
and 4 patients (1.8%) developed either meningitis, ventriculitis or a combination of both. Like our patient, 3 of
their 5 patients with subgaleal fluid infection developed
sepsis, meningitis or osteomyelitis. This anecdotal evidence may imply additional serious infectious risks in
patients with subgaleal infections following craniofacial
surgery. Also similar to our own experience, surgical
removal of infected bone grafts was necessary to resolve
osteomyelitis in 6 of their 10 patients.
Although Siegel and Israele [19] focused on infectious
complications following surgery, noninfectious complications were briefly noted as well. Neurological complications were seen in 19 patients (8.7%) with the majority of
these being comprised of CSF leaks (13 patients). The
remainder included 2 patients with seizures (due to cerebral edema in 1 patient and SIADH-induced hyponatremia in the other), diabetes insipidus in 1 patient, 2
additional patients with SIADH-induced hyponatremia,
and a hypotonic bladder in 1 patient. Unrelated to the
initial craniofacial procedure was the development of an
intracranial hemorrhage following ventriculostomy in
another patient who had required intrathecal access to
treat his meningitis and ventriculitis.
In the largest review of complications associated with
craniofacial surgery, Whitaker et al. [16] summarized
their experience with 793 surgical procedures in 6 centers

over a 10-year period. An overall 16.5% complication rate
and a 1.6% death rate was noted. Of these cases, the following neurological complications were noted: CSF leaks
in 9 patients (1.1%), visual changes (i.e., transient and
permanent blindness and decreased visual acuity) in 3
patients (0.38%), sixth nerve palsies in ‘several’ patients,
seventh nerve palsy in 1 patient (0.13%), and meningitis
in 1 patient (0.13%). Of note, there were no morbid complications of ‘brain damage’ following these 793 procedures, however, 1 of the 13 mortalities encountered was
attributed to cerebral edema.
Finally, Wolfe et al. [20] reviewed complications in 29
patients (17 of which were Apert’s patients) who underwent either a midfacial or frontofacial advancement. In
his series, no CSF leaks were encountered and the only
significant neurological complication was a fungal epidural abscess in 1 patient. Furthermore, no instances of
intracranial hemorrhage from a ruptured mycotic aneurysm following craniofacial surgery have been described
making this report the first of such an occurrence. In
short, this literature review suggests that the majority of
complications following craniofacial surgery are due to
CSF leakage and the remainder are relatively uncommon
in occurrence.

Mycotic Aneurysm

Pediatr Neurosurg 2001;35:94–98

Conclusion

This case illustrates the difficult and complex nature of
managing intracranial mycotic aneurysms, particularly in
children. It also emphasizes the need for vigilant neurological exams in the setting of a critically ill, surgical
patient with prolonged infectious complications. This is
often difficult since many patients may be intubated,
heavily sedated and chemically paralyzed, thus prohibiting even the most basic of neurological exams. Furthermore, the necessary practice of tarsorrhaphy during some
craniofacial procedures, unfortunately, eliminates the
ability to perform a simple but informative pupillary
exam in the postoperative period. The importance of the
pupillary exam is illustrated in this report as the recognition of an acute intracranial hemorrhage was promptly
made in a sedated, intubated patient when she was found
to have a unilateral fixed and dilated pupil. Intracranial
hemorrhages and ruptured mycotic aneurysms will no
doubt continue to constitute a rare, postoperative risk following craniofacial procedures. However, because patients can quickly deteriorate, die, or sustain irreversible
neurological injury following these events, their prompt
recognition and treatment is essential.

97

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Uchiyama/Brockmeyer/Cherny/Jacobs/
Walker

Copyright: S. Karger AG, Basel 2001. Reproduced with the permission of S. Karger AG, Basel. Further
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