Fourth ventricular subarachnoid stent for Chiari malformation type I-associated persistent syringomyelia. 2023

Rachael K Han, and Mauricio P Medina, and Alexandra M Giantini-Larsen, and John K Chae, and Amanda Cruz, and Andrew L A Garton, and Jeffrey P Greenfield

Syringomyelia (syrinx) associated with Chiari malformation type I (CM-I) is commonly managed with posterior fossa decompression, which can lead to resolution in most cases. A persistent syrinx postdecompression is therefore uncommon and challenging to address. In the setting of radiographically adequate decompression with persistent syrinx, the authors prefer placing fourth ventricular subarachnoid stents that span the craniocervical junction particularly when intraoperative observation reveals arachnoid plane scarring. The objective of this study was to evaluate the safety and efficacy of a fourth ventricle stent for CM-I-associated persistent syringomyelia, assess dynamic changes in syrinx dimensions, and report stent-reduction durability, clinical outcomes, and procedure-associated complications. The authors performed a single-institution, retrospective review of patients who underwent fourth ventricular subarachnoid stent placement for persistent CM-I-associated syringomyelia following a prior posterior fossa decompression. The authors' institutional Chiari database contains 600 cases with 149 decompressions for CM-I-associated syringomyelia, of which 13 met criteria for inclusion. Data on patient demographics, clinical presentation and outcomes, and MRI findings were collected. The maximal syrinx diameter was estimated by calculating the area of an elliptical cross-section in the largest axial plane from preoperative, immediately postoperative, and late postoperative T2-weighted MR images. All 13 patients experienced a significant decrease in mean syrinx area from the preoperative to the late postoperative MRI (mean syrinx diameter 114.1 ± 81.8 mm2 vs 24.5 ± 23.8 mm2, p < 0.001). The mean time until late postoperative MRI was 19.7 months (range 2.0-70.7 months). The syrinx area reduced on average by 75.0% ± 23.9% at the time of the last postoperative scan. Syrinx resolution was variable, with 4 patients (30.8%) achieving near-complete resolution (> 90%, grade III reduction), 7 patients (50%) having 50%-90% reduction (grade II), and 2 patients (14.3%) having < 50% decrease (grade I). One patient experienced catheter migration into the left brachium pontis with an associated cyst at the tip of the catheter that decreased in size on follow-up imaging. Placement of fourth ventricular subarachnoid stents spanning the craniocervical junction in patients with persistent CM-I-associated syringomyelia after posterior fossa decompression is a safe therapeutic option and significantly reduced the mean syrinx area, with a greater reductive effect seen over longer follow-up periods.

UI MeSH Term Description Entries
D003560 Cysts Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues. Cyst
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001139 Arnold-Chiari Malformation A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46) Arnold-Chiari Deformity,Arnold-Chiari Malformation, Type 1,Arnold-Chiari Malformation, Type 2,Arnold-Chiari Malformation, Type 3,Arnold-Chiari Malformation, Type 4,Arnold-Chiari Malformation, Type I,Arnold-Chiari Malformation, Type II,Arnold-Chiari Malformation, Type III,Arnold-Chiari Malformation, Type IV,Arnold-Chiari Syndrome,Chiari Malformation Type 2,Chiari Malformation Type I,Chiari Malformation Type II,Malformation, Arnold-Chiari,Type I Arnold-Chiari Malformation,Type II Arnold-Chiari Malformation,Type III Arnold-Chiari Malformation,Type IV Arnold-Chiari Malformation,Arnold Chiari Deformity,Arnold Chiari Malformation,Arnold Chiari Malformation, Type 1,Arnold Chiari Malformation, Type 2,Arnold Chiari Malformation, Type 3,Arnold Chiari Malformation, Type 4,Arnold Chiari Malformation, Type I,Arnold Chiari Malformation, Type II,Arnold Chiari Malformation, Type III,Arnold Chiari Malformation, Type IV,Arnold Chiari Syndrome,Deformity, Arnold-Chiari,Malformation, Arnold Chiari,Syndrome, Arnold-Chiari,Type I Arnold Chiari Malformation,Type II Arnold Chiari Malformation,Type III Arnold Chiari Malformation,Type IV Arnold Chiari Malformation
D013595 Syringomyelia Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269) Hydrosyringomyelia,Morvan Disease,Morvan's Disease,Myelosyringosis,Syringomyelus,Hydrosyringomyelias,Morvan Diseases,Morvan's Diseases,Morvans Disease,Myelosyringoses,Syringomyelias
D057785 Catheters A flexible, tubular device that is used to carry fluids into or from a blood vessel, hollow organ, or body cavity. Catheter
D020546 Fourth Ventricle An irregularly shaped cavity in the RHOMBENCEPHALON, located between the MEDULLA OBLONGATA; the PONS; and the isthmus in front, and the CEREBELLUM behind. It is continuous with the central canal of the cord below and with the CEREBRAL AQUEDUCT above, and through its lateral and median apertures it communicates with the SUBARACHNOID SPACE. 4th Ventricle,Quarto Ventricolo,4th Ventricles,Fourth Ventricles,Quarto Ventricolos,Ventricle, 4th,Ventricle, Fourth,Ventricles, 4th,Ventricles, Fourth,Ventricolo, Quarto,Ventricolos, Quarto

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