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An infant with posterior fossa hemangioma with aortic stenosis: Case report and review of the literature

1 Department of Neurosurgery, Ankara Bilkent City Hospital, Ankara, Turkey
2 Department of Pathology, Ankara Bilkent City Hospital, Ankara, Turkey

Date of Submission16-Jan-2021
Date of Acceptance27-Mar-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Yigit Can Senol,
Department of Neurosurgery, Ankara Bilkent City Hospital
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_15_21



We presented a unique case of infantile type hemangioma corresponding with aortic stenosis. Therefore, we accepted this case as a PHACES syndrome. This case will be the 4th case of the literature. The most remarkable feature of PHACES syndrome is infantile hemangioma, but abnormalities in the arteries which are not obvious on the clinical examination have considerable potential morbidity. We have certain diagnosis with pathology and no conflicts of interest disclosure.

Keywords: Aortic stenosis, infantile hemangioma, PHACES syndrome, posterior fossa hemangioma

How to cite this URL:
Senol YC, Daglioglu E, Basaran OA, Belen AD. An infant with posterior fossa hemangioma with aortic stenosis: Case report and review of the literature. J Pediatr Neurosci [Epub ahead of print] [cited 2023 Sep 26]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=335189

   Introduction Top

The most prevalent benign neoplasm of infancy is the infantile hemangioma[1] with an incidence of 5–10% in pediatric population.[2] These tumors usually do not present with systemic involvement. Primarily located in skin, whereas intracranial hemangiomas less frequently observed pathologies. We presented a unique case of posterior fossa hemangioma with aortic stenosis in a 1-year-old infant. Including aortic stenosis as an aortic defect with a posterior fossa hemangioma, we can count this condition as a PHACE syndrome. The most remarkable feature of PHACE syndrome is infantile hemangioma but abnormalities in the arteries which are not obvious on the clinical examination have considerable potential morbidity.[3],[4] PHACE syndrome is an acronym of Posterior fossa malformations, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities. It was first described in 1978 by Pascual-Castroviejo.[5] In 1996, Frieden et al.[6] created the acronym. Subsequently, sternal malformations were added and the acronym became PHACES.[7]

   Case Report Top

In this study, a 1-year-old infant who was a close follow-up patient of our hospital’s pediatric cardiology department with an aortic stenosis planning a balloon-valvuloplasty was studied. He was admitted to the pediatric emergency service with a 2-day onset of right hemiparesis without any traumatic injury. According to the patient’s perinatal and medical history, he was born after 39 weeks pregnancy with a birth weight of 3125 g. There was no positive family history of hemangiomas. On examination, he was awake, alert, positive pupillary reflexes, no facial paresis, with a 4/5 right hemiparesis. Neither syncope nor seizure had happened. The ophthalmological evaluation showed that both eyes were equal in size and eye motility was normal. There were no other symptoms of acute hydrocephaly and had subfebril fever of 37.8ºC, without neck stiffness.

Blood tests were evaluated, and no pathologic value was seen. Magnetic resonance imaging (MRI) and angiography (MRA) were obtained which revealed a pontin mass along the dorsal aspect of the posterior fossa. The lesionwas heterogeneously hyperintense on T1 and T2 weighted sequences [Figure 1] and [Figure 2]. The mass was well-circumscribed and heterogeneous, and had evidence of previous hemorrhage within it. He was consulted to our department with preliminary diagnosis of pontine cavernoma and hemangioma. The differential diagnoses of an intracranial vascular lesion in this 1-year-old infant included hemangioma, hemangioblastoma, vascular malformation, and other neonatal tumor types, such as soft-tissue sarcoma.[8]
Figure 1: MR angiography showed a non-vascular lesion narrowing forth ventricle on the left part of pons

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Figure 2: Diffusion sequence of the lesion

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He required hospitalization with focal neurologic deficit and admitted to the pediatric ICU service to complete preoperative studies. After 12 h of admission, our team was ready for surgery but on the operation table he had fever of 38.5°C. The anesthetist wanted to exclude COVID-19 infection and sent him back to ICU. Blood and urine cultures were made and nasopharyngeal swab culture for COVID-19 was sent to the public health laboratory of Turkish Health Ministry. Prophylactic antibiotics were started. Tests were revealed after 2 days and showed no infection and his fever was improved. Two days later we operated on the patient. A suboccipital craniectomy was performed. The lesion appeared vascular and clearly separated from the normal glial tissue without involvement. Because of the patient’s size and low circulating blood volume, minimizing blood loss was imperative. Blood products were on standby but neither of it was used. Hemangioma was gross totally resected and no complication was made. After surgery to exclude intracranial hemorrhage, a diffusion MRI was obtained and revealed the lesion was totally resected. No hemorrhage was seen [Figure 3]. On his postoperative day 1, his hemiparesis was 4+/5 but had a facial paresis, so we immediately started 2 mg/kg/h prednisolone and continued for 24 h. In his postoperative day 2, his facial paralysis was recovered and we stopped prednisolone infusion. He was transferred to infantile service, near his parents. We checked his neurologic examination on our daily routines and his right paresis was totally improved on postoperative day 5 and he was discharged with outpatient polyclinic control.
Figure 3: Postoperative 24 h MRI, no hemorrhage was seen on the MRI

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On his postoperative day 12, we took out the sutures and no cerebral spinal fluid collection was seen. We planned a control magnetic resonance angiography and obtained it on his postoperative day 30 [Figure 4]. No residue lesion was revealed.
Figure 4: Postoperative day 30 control MRI, lesion was totally removed. T1, TOF, and T2 flair sequences of MRI images

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   Discussion Top

One percent of infantile hemangiomas involve the central nervous system, and approximately half of these are intracranial.[8] Most intracranial infantile hemangiomas classically involve the extra-axial basal cisterns, subarachnoid and ventricular spaces, and cavernous sinus.[9] Our case was demonstrating classical findings of infantile hemangiomas [Figure 5], but confirmation of hemangioma was made by immunohistochemical staining. Pathologic specimen was positive staining with GLUT1 and SMA which are reliable markers for infantile hemangioma. GLUT1 [Figure 6] and [Figure 7], a glucose transporter present in normal capillary endothelial cells constituting the blood–brain barrier, is a particularly useful marker of infantile hemangiomas because it differentiates those from other vascular tumors and malformations that have been shown to lack GLUT1.[10] Several other associated abnormalities have been observed in infants with infantile hemangioma, including arterial anomalies, ipsilateral cerebellar hypoplasia, Dandy–Walker malformation, Moya Moya-like proliferation of the internal carotid artery terminus.[11]
Figure 5: Pathology prepared slides showed congested vascular lesions which has thin walls with hematoxylin eosin dye

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Figure 6: Hematoxylin eosin dying of the pontine mass. Specimen showed a vascular lesion within slight walls

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Figure 7: İmmunohistochemically, Glut-1 dye showed expressions of the vascular wall and it is compatible with the infantile type hemangioma

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Despite the benign nature of infantile hemangioma, in our case, the patient had focal neurologic deficits because of the mass effect. These lesions can cause considerable and permanent injury to the developing brain. Therefore, intervention is required such as these mass effect lesions of infantile hemangiomas. Besides, Propanol is a common treatment protocol of infantile hemangiomas whereas extra-cranial lesions, there is no evidence of using it for intracranial lesions. On the other hand, as a second option of medical therapy, steroids were generally used, mostly in benign skin lesions with biopsy confirmed. Regression of lesions were reported.[1],[8] But also no evidence was found in the literature of using steroids for intracranial lesions. Embolization can be an additional therapy to surgical resection. But as an infant patient, small vessels can be a handicap to make angiography without complication.[12] Three of Dural-based infantile hemangiomas were reported in the literature.[11] Our case can be the 4th case in the literature, which included the PHACES symptoms [Table 1].
Table 1: Case reports of dural-based infantile hemangiomas

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Conflicts of interest

There are no conflicts of interest.

   References Top

Daenekindt T, Weyns F, Kho KH, Peuskens D, Engelborghs K, Wuyts J. Giant intracranial capillary hemangioma associated with enlarged head circumference in a newborn. J Neurosurg Pediatr 2008;1:488-92.  Back to cited text no. 1
Kilcline C, Frieden IJ. Infantile hemangiomas: how common are they? A systematic review of the medical literature. Pediatr Dermatol 2008;25:168-73.  Back to cited text no. 2
Garzon MC, Epstein LG, Heyer GL, Frommelt PC, Orbach DB, Baylis AL, et al. PHACE syndrome: consensus-derived diagnosis and care recommendations. J Pediatr 2016;178:24-33.e2.  Back to cited text no. 3
Bracken J, Robinson I, Snow A, Watson R, Irvine AD, Rea D, et al. PHACE syndrome: MRI of intracerebral vascular anomalies and clinical findings in a series of 12 patients. Pediatr Radiol 2011;41:1129-38.  Back to cited text no. 4
Pascual-Castroviejo I. Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas. Neuroradiology 1978;16:82-4.  Back to cited text no. 5
Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996;132: 307-11.  Back to cited text no. 6
Metry DW, Dowd CF, Barkovich AJ, Frieden IJ. The many faces of PHACE syndrome. J Pediatr 2001;139:117-23.  Back to cited text no. 7
Viswanathan V, Smith ER, Mulliken JB, Fishman SJ, Kozakewich HP, Burrows PE, et al. Infantile hemangiomas involving the neuraxis: clinical and imaging findings. AJNR Am J Neuroradiol 2009;30:1005-13.  Back to cited text no. 8
Philpott C, Wray A, MacGregor D, Coleman L. Dural infantile hemangioma masquerading as a skull vault lesion. AJNR Am J Neuroradiol 2012;33:E85-7.  Back to cited text no. 9
North PE, Waner M, Mizeracki A, Mrak RE, Nicholas R, Kincannon J, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol 2001;137:559-70.  Back to cited text no. 10
Shakir HJ, McBride P, Reynolds RM. Dural-based infantile hemangioma of the posterior fossa: case report. Surg Neurol Int 2016;7. https://doi.org/10.4103/2152–7806.181827  Back to cited text no. 11
Willing SJ, Faye-Petersen O, Aronin P, Faith S. Radiologic-pathologic correlation. Capillary hemangioma of the meninges. AJNR Am J Neuroradiol 1993;14:529-36.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]

  [Table 1]


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