Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report

Brain stem cavernomas are exceedingly rare in pediatric populations, with limited literature addressing their natural history, treatment guidelines, and counseling. We report the case of a 5-year-old girl presenting with acute neurological symptoms, including diplopia, gait ataxia, headache, and alt...

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Main Authors: Matthias Krause, Armin-Johannes Michel, Johannes Koch, Johann Gradl, Johannes A. R. Pfaff, Christoph J. Griessenauer, Lorenz Stana-Hackenberg
Format: Article
Language:English
Published: MDPI AG 2025-04-01
Series:Children
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Online Access:https://www.mdpi.com/2227-9067/12/5/564
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author Matthias Krause
Armin-Johannes Michel
Johannes Koch
Johann Gradl
Johannes A. R. Pfaff
Christoph J. Griessenauer
Lorenz Stana-Hackenberg
author_facet Matthias Krause
Armin-Johannes Michel
Johannes Koch
Johann Gradl
Johannes A. R. Pfaff
Christoph J. Griessenauer
Lorenz Stana-Hackenberg
author_sort Matthias Krause
collection DOAJ
description Brain stem cavernomas are exceedingly rare in pediatric populations, with limited literature addressing their natural history, treatment guidelines, and counseling. We report the case of a 5-year-old girl presenting with acute neurological symptoms, including diplopia, gait ataxia, headache, and altered consciousness. Initial imaging revealed obstructive hydrocephalus caused by a hemorrhagic lesion near the pineal region. After emergency external ventricular drainage (EVD), most symptoms resolved except for diplopia. A subsequent MRI suggested a space-occupying hemorrhagic cyst in the tectal lamina, leading to endoscopic third ventriculostomy (ETV). During ETV, a large hemorrhagic mass at the aqueduct entrance was identified but not removed due to its fragility. Following ETV, the patient improved rapidly and was discharged. However, she was readmitted with recurrent symptoms and altered consciousness. An emergency MRI indicated a progressive hemorrhagic mass lesion compressing the midbrain, necessitating surgical intervention. The patient underwent suboccipital craniotomy using a telovelar approach. The intraoperative findings included cavernoma-like tissue within the aqueduct, which was successfully resected. Histopathology confirmed hemorrhagic and angiomatous tissue, excluding a primary brain tumor. Postoperatively, the patient showed significant, progressive neurological improvement, with mild internuclear strabism, trunk ataxia, and fatigue at the last follow-up. Six months later, a follow-up MRI and cerebral angiography showed no cavernoma remnants but identified a midbrain deep venous anomaly. This case underscores the feasibility of the microsurgical resection of midbrain cavernomas in symptomatic pediatric patients, highlighting the importance of the thorough assessment of atypical hemorrhagic midbrain lesions to exclude rare vascular malformations from differential diagnoses.
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spelling doaj-art-2f501a81f52048ea99a2a7dc4938fa672025-08-20T03:47:48ZengMDPI AGChildren2227-90672025-04-0112556410.3390/children12050564Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case ReportMatthias Krause0Armin-Johannes Michel1Johannes Koch2Johann Gradl3Johannes A. R. Pfaff4Christoph J. Griessenauer5Lorenz Stana-Hackenberg6Department of Neurosurgery, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaDepartment of Pediatric Surgery, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaDepartment of General Pediatrics, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaDepartment of Radiology, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaDepartment of Neurosurgery, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaDepartment of Neurosurgery, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaDepartment of General Pediatrics, Paracelsus Medical Private University Salzburg, 5020 Salzburg, AustriaBrain stem cavernomas are exceedingly rare in pediatric populations, with limited literature addressing their natural history, treatment guidelines, and counseling. We report the case of a 5-year-old girl presenting with acute neurological symptoms, including diplopia, gait ataxia, headache, and altered consciousness. Initial imaging revealed obstructive hydrocephalus caused by a hemorrhagic lesion near the pineal region. After emergency external ventricular drainage (EVD), most symptoms resolved except for diplopia. A subsequent MRI suggested a space-occupying hemorrhagic cyst in the tectal lamina, leading to endoscopic third ventriculostomy (ETV). During ETV, a large hemorrhagic mass at the aqueduct entrance was identified but not removed due to its fragility. Following ETV, the patient improved rapidly and was discharged. However, she was readmitted with recurrent symptoms and altered consciousness. An emergency MRI indicated a progressive hemorrhagic mass lesion compressing the midbrain, necessitating surgical intervention. The patient underwent suboccipital craniotomy using a telovelar approach. The intraoperative findings included cavernoma-like tissue within the aqueduct, which was successfully resected. Histopathology confirmed hemorrhagic and angiomatous tissue, excluding a primary brain tumor. Postoperatively, the patient showed significant, progressive neurological improvement, with mild internuclear strabism, trunk ataxia, and fatigue at the last follow-up. Six months later, a follow-up MRI and cerebral angiography showed no cavernoma remnants but identified a midbrain deep venous anomaly. This case underscores the feasibility of the microsurgical resection of midbrain cavernomas in symptomatic pediatric patients, highlighting the importance of the thorough assessment of atypical hemorrhagic midbrain lesions to exclude rare vascular malformations from differential diagnoses.https://www.mdpi.com/2227-9067/12/5/564midbrain cavernomaendoscopic third ventriculostomymicrosurgical resectiontectal cavernoma
spellingShingle Matthias Krause
Armin-Johannes Michel
Johannes Koch
Johann Gradl
Johannes A. R. Pfaff
Christoph J. Griessenauer
Lorenz Stana-Hackenberg
Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report
Children
midbrain cavernoma
endoscopic third ventriculostomy
microsurgical resection
tectal cavernoma
title Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report
title_full Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report
title_fullStr Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report
title_full_unstemmed Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report
title_short Treatment of a Large Hemorrhagic Midbrain Cavernoma Within the Silvian Aqueduct in a Five-Year-Old Girl—A Case Report
title_sort treatment of a large hemorrhagic midbrain cavernoma within the silvian aqueduct in a five year old girl a case report
topic midbrain cavernoma
endoscopic third ventriculostomy
microsurgical resection
tectal cavernoma
url https://www.mdpi.com/2227-9067/12/5/564
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