Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report
Syringohydromyelia, syringobulbia, and syringocephaly are rare manifestations of Chiari-I malformation (CM-I), each resulting from disrupted cerebrospinal fluid dynamics. While syringomyelia is commonly associated with CM-I, the rostral extension of syrinx cavities into the brainstem (syringobulbia)...
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Elsevier
2025-08-01
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| Series: | Radiology Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S193004332500367X |
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| author | Nebil A. Shukralla Etsehiwot Demeke Fitsum A. Gemechu Michael A. Negussie Abebe Mekonnen Mersha A. Woldemariam Kibruyisfaw Z. Shumbash |
| author_facet | Nebil A. Shukralla Etsehiwot Demeke Fitsum A. Gemechu Michael A. Negussie Abebe Mekonnen Mersha A. Woldemariam Kibruyisfaw Z. Shumbash |
| author_sort | Nebil A. Shukralla |
| collection | DOAJ |
| description | Syringohydromyelia, syringobulbia, and syringocephaly are rare manifestations of Chiari-I malformation (CM-I), each resulting from disrupted cerebrospinal fluid dynamics. While syringomyelia is commonly associated with CM-I, the rostral extension of syrinx cavities into the brainstem (syringobulbia) and cerebral parenchyma (syringocephaly) is very rare, with limited cases reported globally. This case report describes a 40-year-old woman presenting with progressive neurological deficits, including hemiparesis, sensory disturbances, and bulbar symptoms. Magnetic resonance imaging revealed CM-I with a syringohydromyelic cavity extending from the cervicomedullary junction to the thoracic spinal cord, syringobulbia involving the brainstem, and syringocephaly affecting the left cerebral hemisphere, internal capsule, and basal ganglia. Following posterior fossa decompression, the patient experienced significant neurological improvement, including restored motor function and resolution of intracranial edema, although the cervical syrinx cavity remained stable. This case demonstrates the importance of early diagnosis and surgical intervention in managing these rare yet debilitating complications of CM-I, highlighting the potential for improved outcomes despite the complex and extensive nature of these lesions. |
| format | Article |
| id | doaj-art-e2cca6e2d3f64e97ab2aa85f738b328e |
| institution | OA Journals |
| issn | 1930-0433 |
| language | English |
| publishDate | 2025-08-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj-art-e2cca6e2d3f64e97ab2aa85f738b328e2025-08-20T02:30:55ZengElsevierRadiology Case Reports1930-04332025-08-012083793379710.1016/j.radcr.2025.04.068Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case reportNebil A. Shukralla0Etsehiwot Demeke1Fitsum A. Gemechu2Michael A. Negussie3Abebe Mekonnen4Mersha A. Woldemariam5Kibruyisfaw Z. Shumbash6School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia; Corresponding author.Department of Radiology, College of Health Sciences, Addis Ababa University, Addis Ababa, EthiopiaSchool of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, EthiopiaSchool of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, EthiopiaDepartment of Radiology, College of Health Sciences, Addis Ababa University, Addis Ababa, EthiopiaDepartment of Surgery, College of Health Sciences, Addis Ababa University, EthiopiaDepartment of Surgery, College of Health Sciences, Addis Ababa University, EthiopiaSyringohydromyelia, syringobulbia, and syringocephaly are rare manifestations of Chiari-I malformation (CM-I), each resulting from disrupted cerebrospinal fluid dynamics. While syringomyelia is commonly associated with CM-I, the rostral extension of syrinx cavities into the brainstem (syringobulbia) and cerebral parenchyma (syringocephaly) is very rare, with limited cases reported globally. This case report describes a 40-year-old woman presenting with progressive neurological deficits, including hemiparesis, sensory disturbances, and bulbar symptoms. Magnetic resonance imaging revealed CM-I with a syringohydromyelic cavity extending from the cervicomedullary junction to the thoracic spinal cord, syringobulbia involving the brainstem, and syringocephaly affecting the left cerebral hemisphere, internal capsule, and basal ganglia. Following posterior fossa decompression, the patient experienced significant neurological improvement, including restored motor function and resolution of intracranial edema, although the cervical syrinx cavity remained stable. This case demonstrates the importance of early diagnosis and surgical intervention in managing these rare yet debilitating complications of CM-I, highlighting the potential for improved outcomes despite the complex and extensive nature of these lesions.http://www.sciencedirect.com/science/article/pii/S193004332500367XChiari-I malformationMagnetic resonance imagingSyringobulbiaSyringocephalySyringohydromyelia |
| spellingShingle | Nebil A. Shukralla Etsehiwot Demeke Fitsum A. Gemechu Michael A. Negussie Abebe Mekonnen Mersha A. Woldemariam Kibruyisfaw Z. Shumbash Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report Radiology Case Reports Chiari-I malformation Magnetic resonance imaging Syringobulbia Syringocephaly Syringohydromyelia |
| title | Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report |
| title_full | Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report |
| title_fullStr | Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report |
| title_full_unstemmed | Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report |
| title_short | Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report |
| title_sort | syringohydromyelia syringobulbia and syringocephaly associated with chiari i malformation a case report |
| topic | Chiari-I malformation Magnetic resonance imaging Syringobulbia Syringocephaly Syringohydromyelia |
| url | http://www.sciencedirect.com/science/article/pii/S193004332500367X |
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