Childs Nerv Syst (2008) 24:265–268 DOI 10.1007/s00381-007-0454-y CASE REPORT Infectious aneurysmal rupture presenting as massive intracerebral hemorrhage in a preterm baby Hyun-Seung Kang & So Dug Lim & Young-Cho Koh Received: 22 May 2007 / Published online: 7 November 2007 # Springer-Verlag 2007 Abstract Introduction Infectious intracranial aneurysms in infantile period are very rare, and there is no report of infectious intracranial aneurysm in preterm infant Case report A 43-day-old female infant, delivered at 29 weeks of gestation, presented with massive intracranial hemorrhage. Eighteen days after diagnoses of necrotizing enterocolitis, sepsis, and meningitis, the baby became lethargic with rigid left limbs. Imaging studies revealed a large hematoma in the right temporoparietal region and adjacent lateral ventricle. During removal of fresh hematoma, an actively bleeding nodular mass, contiguous with the distal middle cerebral artery branch, was found and excised from the parent vessel. Pathologic examination revealed a ruptured infectious intracranial aneurysm. After surgery, her neurologic status improved and she was doing well at 12 months’ follow-up with mild spastic hemiparesis. Conclusion To our knowledge, this is the first reported case of infectious intracranial aneurysm in the preterm infant, which was successfully treated with hematoma evacuation and resection of the aneurysm. Keywords Infectious aneurysm . Intracranial aneurysm . Intracerebral hemorrhage . Necrotizing enterocolitis . Preterm infant H.-S. Kang : Y.-C. Koh (*) Department of Neurosurgery, Konkuk University Hospital, 4-12 Hwayang-dong, Gwangjin-gu, Seoul 143-729, South Korea e-mail: yckoh@kuh.ac.kr S. D. Lim Department of Pathology, Konkuk University Hospital, Seoul, South Korea Introduction Annual incidence of spontaneous intracranial hemorrhage ranges from 1.5 to 2.5 cases per 100,000 per year in the pediatric population under 15 years of age [5, 22]. Causes of intracerebral hemorrhage in childhood include germinal matrix hemorrhage in the preterm infants, vascular diseases (arteriovenous malformation, arterial aneurysms, mycotic aneurysms, and moyamoya disease), and disorders of coagulation (hemophilia, disseminated intravascular coagulation, idiopathic thrombocytopenic purpura, and leukemia) [10]. Infectious intracranial aneurysm is a rare cause of intracranial hemorrhage in infancy, and fewer than ten cases have been reported up to the year 2000 [19]. In this paper, the authors report a case of infectious intracranial aneurysm in a preterm infant, which presented with massive intracerebral hemorrhage and was successfully treated with hematoma evacuation and resection of the aneurysm. Case report A 43-day-old female infant presented with intracranial hemorrhage caused by ruptured intracranial infectious aneurysm. The baby had been delivered by cesarean section at 29 weeks of gestation because of preterm labor and cephalopelvic disproportion from her mother with gestational diabetes mellitus. Her birth weight was 1,580 g. The Apgar scores were 4 and 7 at 1 and 5 min, respectively. After delivery, she was fed intravenously with total parenteral nutrition and underwent phototherapy for neonatal jaundice. At 2 weeks after birth, enteral feeding became possible and she gained weight well. At 25 days after birth, the baby had necrotizing enterocolitis, sepsis, and meningitis: blood cultures grew Klebsiella pneumoniae and 266 Childs Nerv Syst (2008) 24:265–268 Fig. 1 a A coronal scan of transfontanelle ultrasonography showing an echogenic mass-like lesion, intracerebral hematoma (ICH), with intraventricular hemorrhage (IVH) in the lateral and the third ventricles. Midline shifting is also noted. b An axial scan of computed tomography (CT) revealing ICH at the right cerebral hemisphere and accompanying IVH and dilated ventricles, similar to the features in ultrasonography Candida albicans, and cerebrospinal fluid culture grew Enterococcus faecium. Her condition improved with antibacterial and antifungal therapy. Brain ultrasonographic examinations at 2-week interval were not remarkable. At 43 days after birth the baby became suddenly lethargic and pale. Her left limbs were rigid. Laboratory examination revealed decreased hemoglobin and hematocrit from 16.0 g/dl and 50%, a week ago, to 3.2 g/dl and 10%. Ultrasonography and computed tomography (CT) revealed a large hematoma in the right temporoparietal region and adjacent lateral ventricle (Fig. 1). No remarkable finding was seen on echocardiography. At surgery, an actively bleeding nodular mass was found during dissection and removal of the hematoma. It was contiguous with the middle cerebral artery branch and was excised from the parent vessel. There was no evidence of residual vascular lesion under surgical microscope. After operation, her respiration became regular and the left motor weakness improved gradually. Pathologic diagnosis was a ruptured infectious intracranial aneurysm, having the features of fresh blood clots intermixed with small amount of brain parenchyme, numerous neutrophils in fresh blood, and degenerated vascular wall structures with granulation tissue (Fig. 2). No microorganism was seen. She underwent ventriculoperitoneal shunt for posthemorrhagic hydrocephalus 3 months after the hematoma evacuation and the aneurysm excision, and discharged home. A repeat CT at 9 months later showed tissue loss at the previous hematoma site and decreased sizes of ventricles (Fig. 3). She was doing well at 12 months’ follow-up although spastic hemiparesis on the left side remained. Discussion Intracranial aneurysms of any type are not common in infants [14]. For example, there was only 1 infant case Fig. 2 Photomicrographs demonstrating necrotizing inflammation with the ruptured elastic fibers (a) and septic embolus (b) in the aneurysmal artery (Verhoff’s elastic tissue stain) Childs Nerv Syst (2008) 24:265–268 267 Fig. 3 a–b Axial CT scans at 9 months after surgery showing tissue loss at the previous hematoma site and decreased sizes of ventricles. The shunt catheter tip is also noted (b) among 32 pediatric patients (less than 18 years of age) more than 27 years in a single institution [21]. The youngest case reported to the present is an 8-day-old girl with a dissecting aneurysm at the anterior communicating artery, which presented with subarachnoid hemorrhage [13]. Furthermore, infectious aneurysms are also rare, accounting for 2–6% of all intracranial aneurysms [25]. Their incidence is often said to be higher (10%) in children [25]. In a number of pediatric aneurysm series, the incidence ranged from 3.7 to 24% [1, 2, 12, 13]. In a review, infections aneurysms accounted for 9.9% (13/131) of intracranial aneurysms in children less than 1 year of age [6]. As a result, infectious intracranial aneurysm is one of the rarest causes of intracranial hemorrhage in infancy [9, 14, 15, 18, 23, 24]. Infectious intracranial aneurysms are most often found in the setting of infective endocarditis with consequent septic embolization [11, 16]. Other associated conditions include meningitis, cavernous sinus thrombophlebitis, cerebral abscess, and subdural empyema [19]. Our case demonstrates infectious aneurysms can be related to septicemia and meningitis in preterm infants. To our knowledge, this is the first reported case of ruptured infectious aneurysm in the preterm baby. The most frequent causative organisms in infectious aneurysms are Staphylococcus (aureus, epidermidis) and Streptococcus (viridans, sanguis, morbidiformis, pneumoniae) species [16]. Among fungi, Aspergilli account for half the cases of fungal aneurysms, and C. albicans is the second most common cause [16]. In cases of infectious aneurysms, culture of multiple organisms is rare [16]. In our case, blood cultures grew K. pneumoniae and C. albicans, and cerebrospinal fluid culture grew E. faecium. In a previously reported case [19], C. albicans and Pseudomonas maltophilia grew in blood cultures 18 days before development of intracranial hemorrhage due to rupture of an infectious aneurysm. In other cases of 24-day old girl and 7-month-old boy, Staphylococcus aureus and Streptococcus salivarius was grown on blood cultures and aneurysm contents, respectively [14, 24]. Time interval between the infectious emboli and infectious aneurysm development, including rupture, can be as short as 24–48 h [8]. Moreover, the hemorrhagic or neurologic manifestations of the infectious aneurysm may occur before manifestations of endocarditis [4]. In our case, there was an interval of 18 days between septicemia and intracranial bleed. Repeat cerebral angiography to detect morphologic changes of the aneurysm or development of new lesions would be necessary especially in cases on medical treatment [3, 20]. Although it may fail to detect new small distal aneurysm, our case underwent magnetic resonance angiography a month after operation (not shown), revealing no new arterial lesion. Treatment of infectious aneurysm should be individualized depending on ruptured or unruptured aneurysm, presence of intracranial hematoma with raised intracranial pressure, eloquence of the corresponding brain region, and effectiveness of medical treatment [7, 17]. In the past, the mortality rate up to 80% has been reported with rupture of an infectious aneurysm [4], while a mortality of 10% was reported among 20 patients of infectious aneurysms recently [7]. Our case demonstrate even a preterm infant with a ruptured infectious aneurysm can be successfully treated with evacuation of hematoma and resection of the aneurysm. References 1. Allison JW, Davis PC, Sato Y, James CA, Haque SS, Angtuaco EJ, Glasier CM (1998) Intracranial aneurysms in infants and children. Pediatr Radiol 28:223–229 2. 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