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CASE REPORT J Neurosurg Pediatr 21:471–477, 2018 I NTRACRANIAL aneurysms are not common in the pe- diatric age group, with pediatric cases accounting for only 0.5% to 4.5% of all aneurysms encountered. 7,18,23, 28,29 These cases may be further divided into 3 periods: neonatal (involving patients younger than 4 weeks), in- fancy (involving patients 4 weeks–2 years of age), and childhood (involving patients 2–18 years of age). Neonatal intracranial aneurysms are exceedingly uncommon, and to date, only 36 cases have been reported. They represent a unique subset of intracranial aneurysms that differ from those encountered in the adult population. Hypertension, atherosclerosis, and smoking are risk factors for the development of aneurysms in adults. The etiology of intracranial aneurysms in neonates is less clear, although trauma, infection, connective tissue disorders, and congenital vessel abnormalities have been suggested as potential contributors. 36 In the current study, we present the case of a 28-day-old neonate admitted with seizures from a ruptured left middle cerebral artery (MCA) aneu- rysm. A comprehensive literature review was performed to investigate the clinical characteristics of neonatal in- tracranial aneurysms, the modality used to diagnose the intracranial aneurysm, treatment characteristics, and clini- cal outcomes. ABBREVIATIONS CTA = computed tomography angiography; ICA = internal carotid artery; MCA = middle cerebral artery; MRA = magnetic resonance angiography; TOF = time of flight. SUBMITTED May 1, 2017. ACCEPTED October 18, 2017. INCLUDE WHEN CITING Published online March 2, 2018; DOI: 10.3171/2017.10.PEDS17226. Neonatal intracranial aneurysms: case report and review of the literature Jeewaka E. Mohotti, MBBS, Grad Dip Anat, 1 Nicole S. Carter, 2,3 Victor Jia Wei Zhang, 2 Leon T. Lai, MBBS, PhD, FRACS, 1,2,4 Christopher Xenos, MBBS, FRACS, 1 Hamed Asadi, MD, PhD, FRANZCR, CCINR, 3,5,6 and Ronil V. Chandra, MBBS, MMed, FRANZCR, CCINR 2–4 1 Department of Neurosurgery, 2 Faculty of Medicine, Nursing, and Health Sciences, Monash University; 3 Neurointerventional Service, Monash Imaging, Monash Health; 5 Neurointerventional Service, Department of Radiology, Austin Health; 4 Monash Neurovascular Institute, Melbourne; and 6 School of Medicine, Faculty of Health, Deakin University, Geelong, Victoria, Australia Intracranial aneurysms in the neonate, presenting in the first 4 weeks of life, are exceedingly rare. They appear to have characteristics, including presentation and location, that vary from those found in adults. The authors present a case of a 28-day-old neonate with a ruptured distal middle cerebral artery (MCA) aneurysm. Initial noninvasive imaging with transfontanelle ultrasound and CT confirmed intraparenchymal and subarachnoid hemorrhage. Contrast-enhanced MRI revealed a 14-mm ruptured fusiform MCA aneurysm that was not identified on time-of-flight magnetic resonance angiography (MRA). Microsurgical treatment was performed with partial neurological recovery. A comprehensive review of the literature from 1949 to 2017 revealed a total of 40 aneurysms in 37 neonates, including the present case. The most common presenting symptom was seizure. Although subarachnoid hemorrhage was the most common form of hemorrhage, 40% had intraparenchymal hemorrhage. The median aneurysm size was 10 mm (range 2–30 mm) and the most common location was the MCA, with two-thirds of cases involving the distal intracranial vasculature. Over the last 10 years, there has been a trend of increasing noninvasive diagnosis of ruptured cerebral aneurysms in neonates, with CT angiography and contrast-enhanced MRI being the most useful diagnostic modalities. The use of contrast-enhanced MRI may improve sensitivity over time-of-flight MRA. Microsurgical treatment was the most common treatment modality overall, with increased use of endovascular treatment in the last decade. Most patients underwent microsurgical vessel ligation or endovascular parent vessel occlusion. There were high rates of neurological recovery after microsurgical or endovascular treatment, particularly for patients with distal aneurysms. https://thejns.org/doi/abs/10.3171/2017.10.PEDS17226 KEY WORDS intracranial aneurysms; neonate; clipping; endovascular embolization; coiling; vascular disorders J Neurosurg Pediatr Volume 21 • May 2018 471 ©AANS 2018, except where prohibited by US copyright law

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Page 1: Neonatal intracranial aneurysms: case report and review of

CASE REPORTJ Neurosurg Pediatr 21:471–477, 2018

IntracranIal aneurysms are not common in the pe-diatric age group, with pediatric cases accounting for only 0.5% to 4.5% of all aneurysms encountered.7,18,23,

28,29 These cases may be further divided into 3 periods: neonatal (involving patients younger than 4 weeks), in-fancy (involving patients 4 weeks–2 years of age), and childhood (involving patients 2–18 years of age). Neonatal intracranial aneurysms are exceedingly uncommon, and to date, only 36 cases have been reported. They represent a unique subset of intracranial aneurysms that differ from those encountered in the adult population.

Hypertension, atherosclerosis, and smoking are risk

factors for the development of aneurysms in adults. The etiology of intracranial aneurysms in neonates is less clear, although trauma, infection, connective tissue disorders, and congenital vessel abnormalities have been suggested as potential contributors.36 In the current study, we present the case of a 28-day-old neonate admitted with seizures from a ruptured left middle cerebral artery (MCA) aneu-rysm. A comprehensive literature review was performed to investigate the clinical characteristics of neonatal in-tracranial aneurysms, the modality used to diagnose the intracranial aneurysm, treatment characteristics, and clini-cal outcomes.

ABBREVIATIONS CTA = computed tomography angiography; ICA = internal carotid artery; MCA = middle cerebral artery; MRA = magnetic resonance angiography; TOF = time of flight.SUBMITTED May 1, 2017. ACCEPTED October 18, 2017.INCLUDE WHEN CITING Published online March 2, 2018; DOI: 10.3171/2017.10.PEDS17226.

Neonatal intracranial aneurysms: case report and review of the literatureJeewaka E. Mohotti, MBBS, Grad Dip Anat,1 Nicole S. Carter,2,3 Victor Jia Wei Zhang,2 Leon T. Lai, MBBS, PhD, FRACS,1,2,4 Christopher Xenos, MBBS, FRACS,1 Hamed Asadi, MD, PhD, FRANZCR, CCINR,3,5,6 and Ronil V. Chandra, MBBS, MMed, FRANZCR, CCINR2–4

1Department of Neurosurgery, 2Faculty of Medicine, Nursing, and Health Sciences, Monash University; 3Neurointerventional Service, Monash Imaging, Monash Health; 5Neurointerventional Service, Department of Radiology, Austin Health; 4Monash Neurovascular Institute, Melbourne; and 6School of Medicine, Faculty of Health, Deakin University, Geelong, Victoria, Australia

Intracranial aneurysms in the neonate, presenting in the first 4 weeks of life, are exceedingly rare. They appear to have characteristics, including presentation and location, that vary from those found in adults. The authors present a case of a 28-day-old neonate with a ruptured distal middle cerebral artery (MCA) aneurysm. Initial noninvasive imaging with transfontanelle ultrasound and CT confirmed intraparenchymal and subarachnoid hemorrhage. Contrast-enhanced MRI revealed a 14-mm ruptured fusiform MCA aneurysm that was not identified on time-of-flight magnetic resonance angiography (MRA). Microsurgical treatment was performed with partial neurological recovery. A comprehensive review of the literature from 1949 to 2017 revealed a total of 40 aneurysms in 37 neonates, including the present case. The most common presenting symptom was seizure. Although subarachnoid hemorrhage was the most common form of hemorrhage, 40% had intraparenchymal hemorrhage. The median aneurysm size was 10 mm (range 2–30 mm) and the most common location was the MCA, with two-thirds of cases involving the distal intracranial vasculature. Over the last 10 years, there has been a trend of increasing noninvasive diagnosis of ruptured cerebral aneurysms in neonates, with CT angiography and contrast-enhanced MRI being the most useful diagnostic modalities. The use of contrast-enhanced MRI may improve sensitivity over time-of-flight MRA. Microsurgical treatment was the most common treatment modality overall, with increased use of endovascular treatment in the last decade. Most patients underwent microsurgical vessel ligation or endovascular parent vessel occlusion. There were high rates of neurological recovery after microsurgical or endovascular treatment, particularly for patients with distal aneurysms.https://thejns.org/doi/abs/10.3171/2017.10.PEDS17226KEY WORDS intracranial aneurysms; neonate; clipping; endovascular embolization; coiling; vascular disorders

J Neurosurg Pediatr Volume 21 • May 2018 471©AANS 2018, except where prohibited by US copyright law

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Case ReportHistory

A 28-day-old female neonate was admitted to our in-stitution with irritability, decreased oral intake, and recur-rent right-sided upper and lower limb focal seizures over 2 days. There was no history of trauma or recent infection. The patient was born at 38 weeks’ gestation through an emergency caesarean section in the setting of poor pro-gression of labor, which had been induced due to mild maternal preeclampsia. The perinatal period was normal and the Apgar score was 9 at 1 and 5 minutes. During the early neonatal period, the patient suffered mild jaundice that required phototherapy, but she was discharged clini-cally well 5 days after birth.

Initial ExaminationOn initial examination, the patient was drowsy and

lethargic with a tense anterior fontanelle. Her vital signs were within the normal ranges, and her breathing was spontaneous. She opened her eyes in response to pain and moved all 4 limbs spontaneously with moderate strength. Her pupils measured 2 mm bilaterally and were reactive to light. There were no clinical findings suggestive of in-fection.

InvestigationsNo evidence of infection was revealed on full blood

examination, inflammatory markers, chest radiograph, or urine or blood cultures. Initial cranial investigation was with transfontanelle ultrasound (Fig. 1 left) followed by CT (Fig. 1 right). These revealed extensive left-sided pa-renchymal hemorrhage. MRI confirmed considerable as-sociated vasogenic edema and mass effect, characterized by midline shift and subfalcine and uncal herniation (Fig. 2A and B). Time-of-flight (TOF) magnetic resonance an-giography (MRA) did not reveal a structural cause to the hemorrhage (Fig. 2C). However, the contrast-enhanced MRI revealed a vividly enhancing oval mass (14 × 8 mm) located directly adjacent to the parenchymal hemorrhage with distal MCA branches leading up to it, compatible with a ruptured distal MCA aneurysm (Fig. 2D). All ra-diological investigations were performed within 10 hours of initial after-hours hospital presentation.

While under assessment, the patient was noticed to stop moving her right upper and lower limbs. Her left pupil be-came progressively dilated and fixed at 4 mm. Intravenous mannitol was administered immediately, and she was tak-en to the operating theater for urgent craniotomy.

OperationBefore anesthesia, the patient’s heart rate was 157

beats/minute, her blood pressure was 81/37 mm Hg, and her hemoglobin level was 79 g/L. During the 4 hours of anesthetic time, fluid balance and hemoglobin levels were monitored by a pediatric anesthesiologist, with adminis-tration of 100 ml total of normal saline and 75 ml of red blood cells in 25-ml aliquots during surgery. Intraopera-tively, the aneurysm arose from the trunk of an M3 branch, and not from a bifurcation. There was a vessel exiting the aneurysm in continuity and a daughter sac that pointed

medially into the hematoma, which was the rupture point. Clipping of the daughter sac alone was not feasible, and thus the branch proximal to the aneurysm was secured with a 7-mm Sugita aneurysm clip and the vessel distal to the aneurysm was fulgurated with bipolar diathermy and divided. The feeding vessel was divided immediately dis-

FIG. 1. Diagnosis of intracranial hemorrhage. Left: Coronal transfonta-nelle ultrasonographic image showing large hyperechoic mass in the left cerebral hemisphere causing midline shift. Right: Coronal noncontrast CT image showing left intraparenchymal hemorrhage with surrounding edema and midline shift. There is also left subarachnoid hemorrhage.

FIG. 2. Utility of contrast-enhanced MRI. A and B: Sagittal (A) and axial (B) T2-weighted MR images showing a large intraparenchymal hemorrhage (central area of reduced signal intensity) with surrounding edema (peripheral area of increased signal intensity). Note the small round dark flow void along the left lateral margin of the hematoma on the axial image. C: TOF MR angiogram showing poor visualization of the left MCA branches and no visualization of the ruptured aneurysm. D: Contrast-enhanced T1-weighted axial MR image showing a vividly enhancing oval mass located directly adjacent to the parenchymal hem-orrhage, the ruptured distal left MCA aneurysm.

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tal to the clip, and the aneurysm was removed and sent for analysis. The hematoma was evacuated, and hemostasis was achieved. There were no intraoperative complications.

Postoperative CoursePostoperative radiological assessment demonstrated

satisfactory evacuation of the intraparenchymal hematoma and no residual aneurysm. Microscopy and culture of the aneurysm dome did not reveal evidence of infection. The etiology remained unknown, with no history of trauma or collagen vascular disease or other congenital syndrome associated with cerebral aneurysms. In the postopera-tive period, the patient suffered ongoing right hemipa-resis and seizures. She required long-term levetiracetam and was eventually discharged to pediatric rehabilitation. At 15 months’ follow-up, the patient had persistent right hemiparesis and delays in reaching gross and fine motor movement milestones. Despite high-dose levetiracetam, she continued to experience recurrent seizures. Follow-up MRI of the brain showed left MCA territory cystic en-cephalomalacia and gliosis.

Literature ReviewSearch Strategy

A comprehensive literature search was performed to identify reports of neonatal aneurysms. We searched PubMed, Ovid MEDLINE, and Embase databases from database inception until December 2016 to identify rel-evant articles. Appropriate key words and MeSH terms were used to identify all studies: “neonate”, “newborn”, “aneurysm”, “cerebral”, and “intracranial.” The reference lists of the final included articles were also searched to identify additional data sources.

Selection Criteria and Data ExtractionStudies eligible for inclusion were those that reported

intracranial arterial aneurysms in children aged 1 month or younger. All studies selected are in English. Proxi-mal aneurysms were defined as those arising from arter-ies of the circle of Willis (including the M1, A1, and P1 segments of the middle, anterior, and posterior cerebral arteries), the cavernous internal carotid artery (ICA), or the tip of the basilar artery. Intracranial aneurysms aris-ing outside of these arteries were considered distal. Aneu-rysm location was, as per these definitions, dichotomized as proximal or distal. Clinical outcomes were classified into 3 groups: death, partial recovery, and full recovery. Patients with no residual neurological deficit were consid-ered to have reached full recovery, while partial recovery was considered to denote survival with some neurological deficit. Two reviewers (J.E.M. and N.S.C.) independently appraised studies and extracted relevant clinical, imaging, and treatment characteristics. Discrepancies between re-viewers were resolved by discussion, and consensus was reached; the pooled cohort was analyzed descriptively.

Study SelectionA total of 510 studies were identified through database

searches and underwent title, abstract, and/or full-text

review. Four hundred seventy-six studies were excluded. Reasons for exclusion included duplication and/or irrel-evance to the present study. After comprehensive review, 34 studies were included in the analysis.

Study Characteristics and OutcomesOur literature review yielded an additional 36 reported

cases of neonates presenting with symptoms that resulted in the diagnosis of an intracranial aneurysm. The patients’ demographic characteristics and the clinical features, di-agnostic modality, treatment, and outcomes of each case, including our present case, are given in Table 1.

Including the present case, our literature analysis yield-ed a total of 40 aneurysms in 37 neonatal patients reported between June 1949 and December 2016. The patients’ me-dian age at diagnosis was 20 days, and 58% were female. Almost all (95%) of the neonates were diagnosed with a ruptured aneurysm; the most common clinical symptoms were seizures (19 cases) and irritability (16 cases). Other presenting features included cyanosis, apnea, tachypnea, vomiting, decreased state of consciousness, and fever. The median aneurysm size was 10 mm (range 2–30 mm). Two-thirds of aneurysms arose in the distal intracranial vessels; almost half were in the MCA branches (Fig. 3).

Treatment decisions and outcomes are summarized in Table 2. Of the 37 patients, 8 died prior to treatment. Of the remaining 29 patients, 20 underwent surgical treat-ment with clipping, excision, or ligation of their aneu-rysms; 6 underwent endovascular treatment; and a further 3 cases were managed conservatively. Most (89%) of the patients treated surgically had a full or partial recovery; there were 2 deaths. The use of endovascular coiling was first reported in 2005,4 and all patients treated with that technique had full or partial recovery.

DiscussionNeonatal intracranial aneurysms are a rare pathology

and are infrequently reported. In this paper, we analyzed data from 37 cases involving neonates with intracranial aneurysms (including our own case as well as those iden-tified in our literature search). Although the literature is limited to case reports, we can draw some conclusions from this comprehensive pooled series regarding clinical presentation, imaging, and treatment. In contrast to adult cases, the most common symptom in neonatal cases of in-tracranial aneurysms is seizure. Given the relatively com-mon occurrence of seizures in the neonatal period, cranial imaging is critical to identify intracranial hemorrhage. Since 1980, noninvasive imaging with transfontanelle ul-trasound and CT have been increasingly used to diagnose intracranial hemorrhage. Although subarachnoid hemor-rhage was the most common form of hemorrhage identi-fied, almost 40% of patients had intraparenchymal hemor-rhage, and 20% had no subarachnoid hemorrhage. Intra-ventricular hemorrhage and/or hydrocephalus was present in 30% of patients. While transfontanelle ultrasound of-fers an attractive first-line imaging option for identifica-tion of large hematomas or hydrocephalus, in the last 10 years, most patients received MRI to diagnose intracranial hemorrhage.

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TABLE 1. Overview of neonatal intracranial aneurysm cases reported in the literature

Authors & Year Age, SexHemorrhage

TypeModality of Dx* Side Site

Distal or Prox

Max Size (mm) Tx Outcome

Newcomb et al., 1949 23 days, M SAH, SDH Autopsy — CoW Prox NR — Death0 days, M SAH Autopsy Rt PCA/PCoA

junctionProx 2 — Death

Jones et al., 1961 20 days, F SAH, IVH Angio Rt MCA Prox NR MS clip Full recovWierdis et al., 1965 0 days, NR SAH Autopsy Rt MCA Distal NR — DeathPickering et al., 1970 1 mo, M SAH Autopsy Rt PICA Distal 25 — DeathLee et al., 1978 0 days, F SAH, IVH Angio — Basilar bifurc Prox 30 — DeathLipper et al., 1978 19 days, F SAH, IVH Autopsy Lt ICA/PCoA Prox 21 — DeathGrode et al., 1978 8 days, F SAH Angio Lt MCA Distal NR MS lig Partial recovHungerford et al., 1981 22 days, F ICH Angio Rt MCA Distal 15 MS lig Full recovThrush & Marano, 1988 1 mo, F ICH Angio Lt MCA Distal 6 MS clip Full recovShimauchi et al., 1989 19 days, F SAH, ICH Angio Rt MCA Distal 10 MS lig Full recovBoop et al., 1991–1992 23 days, M ICH, IVH Intraop Lt MCA Distal 20 MS lig Partial recovPiatt & Clunie, 1992 1 day, M SAH, ICH Angio Lt SCA Distal 15 Conserv Full recovKuchelmeister et al., 1993 4 days, M SAH, IVH Autopsy — ACoA Prox 10 — DeathHosotani et al., 1995 24 days, M SAH Angio Lt PICA Distal 15 MS lig Full recovOzek et al., 1996 17 days, F ICH MRA Rt MCA NR 5 MS lig Full recovAllison et al., 1998 1 mo, M SAH, ICH Intraop Lt MCA Distal 20 MS† NRTan et al., 1998 9 days, M SAH, IVH MRA Lt ACA Prox 10 MS failed DeathJansen et al., 2000 1 mo, F SAH, ICH CTA Lt PICA Distal 8 MS† Full recovKourtopoulos et al., 2000 13 days, F SAH, ICH Angio Lt MCA Distal 3 MS ligation Full recovMaroun et al., 2003 2 days, M SAH Angio Lt MCA Distal 10 MS ligation DeathMotohashi et al., 2004 1 mo, F SAH CTA Rt ACA Distal 7 MS ligation Full recovGallia et al., 2005 21 days, F — Angio Lt Cav ICA Prox 20 EV occl Partial recovLasjaunias et al., 2005 28 days, F — Angio NR MCA Distal NR Conserv Full recov

8 days, F SAH Angio — ACoA Prox NR MS† Partial recovSong et al., 2005 11 days, F SAH, ICH Angio Multi ACoA, RAH, lt

ICA2 prox, 1

distalNR EV occl Partial recov

Rana et al., 2007 21 days, M SDH MRA Rt MCA Distal 5 EV occl Full recovWong & Fong, 2007 1 mo, M SAH, ICH Intraop Rt MCA Distal 10 MS excision w/

anastomosisFull recov

Kasliwal et al., 2008 14 days, M SAH MRA Rt Cav ICA Prox 26 Conserv Full recovVan Raay et al., 2009 7 days, F SAH, IVH MRI Rt PICA Distal 5 MS lig Full recovVizcaíno-Díaz et al., 2009 26 days, M ICH, IVH MRI Lt MCA Distal 20 — DeathTai et al., 2010 7 days, M SAH, IVH CTA Both rt Both PICA 2 distal Both 3 EV occl Full recovChoi & Lee, 2013 26 days, F SAH, ICH, IVH CTA Lt MCA Distal 7 MS clip Partial recovRao et al., 2013 21 days, F SAH, SDH CTA Rt MCA Distal 15 EV occl Partial recovKim et al., 2014 11 days, F ICH CTA Lt MCA Distal 8 MS† Full recovYatomi et al., 2014 1 mo, F SAH MRI — ACoA Prox NR EV occl Full recovPresent case 26 days, F ICH, SAH MRI Lt MCA Distal 14 MS lig Partial recov

ACA = anterior cerebral artery; ACoA = anterior communicating artery; angio = angiography; bifurc = bifurcation; cav = cavernous; conserv = conservative; CoW = circle of Willis; CTA = computed tomography angiography; Dx = diagnosis; EV = endovascular; ICA = internal carotid artery; ICH = intracerebral hemorrhage; IVH = intraven-tricular hemorrhage; lig = ligation; MCA = middle cerebral artery; MRA = magnetic resonance angiography; MRI = magnetic resonance imaging (protocol not stated); MS = microsurgical; multi = multiple; NR = not reported; occl = occlusion; PCA = posterior cerebral artery; PCoA = posterior communicating artery; PICA = posterior inferior cerebellar artery; prox = proximal; RAH = recurrent artery of Heubner; recov = recovery; SAH = subarachnoid hemorrhage; SCA = superior cerebellar artery; SDH = subdural hemorrhage; Tx = treatment.* Modality used to diagnose aneurysm.† Microsurgical technique not reported.

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Between 1980 and 2005, the noninvasive diagnosis of a ruptured aneurysm as the cause of intracranial hemor-rhage was uncommon (22%); the diagnosis was generally made with invasive preoperative cerebral angiography or intraoperatively. However, this has changed significantly in the last 10 years, with 90% of neonatal patients diag-nosed with a ruptured aneurysm with computed tomog-raphy angiography (CTA), MRI, and/or MRA. There was 1 patient with an intraoperative diagnosis of a ruptured distal MCA aneurysm;39 however, in retrospect the aneu-rysm was evident on MRI as a 5-mm contrast-enhancing mass along the lateral margin of the hematoma.39 In our patient, and in other patients reported in the literature,36,39 the diagnosis could be made on contrast-enhanced MRI, as a contrast-enhancing mass on the margin of the hema-toma. Notably in 2 of the 3 cases in which both MRA and contrast-enhanced MRI were performed, TOF MRA was negative, while contrast-enhanced MRI revealed the aneurysm. This may be related to lack of sensitivity of the TOF MRA technique with slow or reduced intracranial vascular flow, which is not uncommon in cases of severe subarachnoid hemorrhage.32 CTA is less sensitive to al-terations in vascular flow and was the diagnostic modality for 4 of the 11 neonatal cases reported in the last decade.

However, MRI with contrast and MRA offer advantages over CTA with the lack of ionizing radiation, an important consideration in this patient cohort.

Most neonatal aneurysms arose in the distal circula-tion, mainly in the MCA territory. This contrasts with ce-rebral aneurysm occurrence in adults and can explain the high (40%) rate of intraparenchymal hemorrhage. Main-taining a high index of suspicion for a ruptured aneurysm is important, since 1 in 5 patients did not have any sub-arachnoid hemorrhage. The etiology of the aneurysm was unknown in most cases (70%); it was considered congeni-

TABLE 2. Management of neonatal cerebral aneurysms and patient outcomes

ManagementNo. of Patients

Full Recov Partial Recov Death NR Total

Microsurgical 12 5 2 1 20Endovascular 3 3 0 6Conservative 3 0 0 3Died prior to Tx 8 8Total 18 8 10 1 37

FIG. 3. Distribution of diagnosed neonatal aneurysms from 1949 to 2017. ACoA = anterior communicating artery; CoW = circle of Willis; ICA = internal carotid artery; PCA = posterior cerebral artery; PCoA = posterior communicating artery; PICA = posterior inferior cerebellar artery; RAH = recurrent artery of Heubner; SCA = superior cerebellar artery.

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tal in 4 cases, traumatic in one, mycotic in one, associated with an AVM in one, and not reported in the remainder. In our case, the etiology remained unknown. There was no history of trauma, collagen vascular disease, or congenital syndrome. In addition, culture of the aneurysm did not re-veal evidence of infection.

Once the diagnosis is made, treatment can be offered. Spontaneous occlusion of the aneurysm is rare but was reported in a patient with a ruptured superior cerebellar artery aneurysm,24 and in another patient with a giant ICA aneurysm.11 Both neonates made a full recovery de-spite asymptomatic complete right ICA occlusion in the second patient. Treatment was carried out in 26 neonates, with approximately one-quarter receiving endovascular treatment. Our review of all the available published cases demonstrates an increasing rate of active treatment—with almost 90% of patients in last the 2 decades receiving active treatment compared with only 50% prior to 1997. The mainstay of treatment in the cases identified by our literature review (85%) was microsurgical vessel ligation or endovascular parent vessel occlusion. The rate of full neurological recovery was higher in neonates who were treated with distal aneurysms (67%) compared with those with proximal aneurysms (33%), with an overall posttreat-ment mortality rate of 8%. Despite the high rate of initial seizure presentation, only 1 patient experienced recurrent seizures requiring ongoing anticonvulsants.

The major limitation of our article is that the literature is limited to published case reports, which introduces se-lection bias. Moreover, previous publications have varied in the level of detail included regarding clinical presen-tation, treatment, and follow-up. Nonetheless, due to the rarity of the condition, large series are unlikely to be published. Thus, this pooled case series provides unique clinical insights from a large pooled analysis of reports of neonatal aneurysms.

ConclusionsNeonatal intracranial aneurysms are exceedingly rare.

The most common presenting symptom was seizure. Al-though subarachnoid hemorrhage was most common, some neonates had intraparenchymal hemorrhage without subarachnoid hemorrhage, indicating a need to maintain a high index of clinical suspicion for a ruptured aneurysm in any neonate with intracranial hemorrhage. Over the last 10 years, there has been increasing noninvasive diagnosis of ruptured neonatal cerebral aneurysms, with most found in the distal intracranial vasculature, particularly in the MCA territory. Microsurgical and endovascular treatment both represent reasonable therapeutic strategies with high rates of neurological recovery, particularly for patients with dis-tal aneurysms.

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DisclosuresThe authors report no conflict of interest concerning the materi-als or methods used in this study or the findings specified in this paper.

Author ContributionsConception and design: Chandra, Mohotti, Xenos, Lai. Acqui-sition of data: Mohotti, Zhang. Analysis and interpretation of data: Mohotti, Carter, Zhang. Drafting the article: Mohotti, Carter, Zhang. Critically revising the article: Mohotti, Carter, Lai, Chandra, Asadi. Reviewed submitted version of manuscript: all authors. Administrative/technical/material support: Mohotti, Carter. Study supervision: Lai, Chandra.

CorrespondenceRonil V. Chandra: Monash Health, Melbourne, VIC, Australia. [email protected].