Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort

Yates, Charles; Campbell, Robert M.; Wood, Martin; Chaseling, Raymond; Tollesson, Gert; Ma, Norman

doi:10.1007/s00381-020-04556-z

Cited by 7 publications

(3 citation statements)

References 28 publications

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“…Of these, 44 were rejected for not meeting the inclusion criteria or for being off-topic. Thirty-five were selected 7-40 for this systematic review (Appendix III, https://www.cns.org/guidelines/browse-guidelines-detail/2-symptoms).…”

Section: Resultsmentioning

confidence: 99%

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Symptoms

Jackson,

Jernigan,

Raskin

et al. 2023

Neurosurgery

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BACKGROUND: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians. OBJECTIVE: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM. METHODS: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines. RESULTS: The literature search yielded 430 abstracts, of which 79 were selected for full-text review, 44 were then rejected for not meeting the inclusion criteria or for being off-topic, and 35 were included in this systematic review. CONCLUSION: Four Grade C recommendations were made based on Class III evidence, and 1 question had insufficient evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/2-symptoms.

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Section: Resultsmentioning

confidence: 99%

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Symptoms

Jackson,

Jernigan,

Raskin

et al. 2023

Neurosurgery

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“…Previous studies have characterized the nature and incidence of postoperative complications after PFDD for Chiari I malformation. 6,7,9,11,13,[15][16][17][18][19][20][21][22][23][24][25][26][27] A recent systematic review and meta-analysis of 27 studies of PFDD for Chiari I malformation combining adult and pediatric patients showed an overall complication rate of 16.1%, with a CSF leak occurring in 3.6% of patients and a combined wound infection/dehiscence rate of 1.7%. 17 Large series of only pediatric patients have reported that nearly 1 in 4 patients undergoing PFDD will experience a postoperative complication, with 6.1% experiencing CSF leak and 3.3% requiring wound revision for either infection or dehiscence.…”

Section: Discussionmentioning

confidence: 99%

Above-The-Hairline Suboccipital Transverse Approach for the Treatment of Chiari I Malformation

Naylor

Rotter

Proctor

et al. 2023

Operative Neurosurgery

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BACKGROUND: Chiari I malformation is a common pediatric neurosurgical disorder with an established treatment paradigm. Posterior fossa decompression and duraplasty (PFDD) is associated with symptom improvement but it carries postoperative risk, particularly cerebrospinal fluid (CSF) leak and wound complications. In addition, the cosmetic outcomes of PFDD have been overlooked in the literature. OBJECTIVE: To describe a novel approach for PFDD in which the transverse surgical incision is completely hidden above the hairline and to report early outcomes in a prospective patient cohort. METHODS: Clinical and cosmetic outcomes were recorded for 15 consecutive pediatric patients who underwent PFDD for Chiari I malformation via the above-the-hairline transverse suboccipital approach. RESULTS: The median clinical follow-up time was 6 months (range 1-12 months), and the majority of patients experienced significant improvement of their preoperative symptoms. Three patients (20%) experienced complications associated with surgery, which included injury to the greater occipital nerve, CSF hypotension and subfascial pseudomeningocele, and superficial wound dehiscence that resolved spontaneously with oral antibiotics. Zero patients (0%) returned to the operating room for persistent CSF leak, deep wound infection, or revision decompression. An excellent cosmetic outcome was achieved in 12 patients (80%). No patient had a poor cosmetic outcome. CONCLUSION: The above-the-hairline transverse suboccipital approach for PFDD in patients with Chiari I malformation offers favorable cosmetic outcomes and fascial closure while permitting adequate decompression.

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“…Approximately two-thirds of patients with CM-1 are asymptomatic and most do not require surgical intervention [7,8]. Of those patients with CM-1 who are symptomatic, presenting complaints vary widely.…”

Section: Introductionmentioning

confidence: 99%

Surgical Treatment of Upper Extremity Segmental Myoclonus in an Adolescent with Chiari Malformation and Cervicothoracic Syrinx

et al. 2021

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Background: Myoclonus is an involuntary movement disorder characterized by semirhythmic jerking movements of muscle groups but is rarely seen in association with Chiari malformation type I (CM-1). CM-1 is a frequently encountered clinical entity in pediatric neurosurgery characterized by caudal displacement of the cerebellar tonsils with or without syringomyelia. We report a pediatric patient who presented with upper extremity myoclonus and was found to have CM-1 and a complex septated cervicothoracic syrinx eccentric to the left. Case Presentation: A 12-year-old female presented with 6 months of headaches and upper extremity paresthesias who subsequently developed a left upper extremity segmental myoclonus after a fall. MRI demonstrated a CM-1 and a large complex cervicothoracic syrinx with a midline and left paracentral cavities. Her myoclonus was nonepileptic and refractory to clonazepam, cyclobenzaprine, and gabapentin. She underwent an intradural Chiari decompression and duraplasty. Postoperatively, she had complete resolution of her segmental myoclonus. Discussion: This case demonstrates a durable resolution of posttraumatic upper extremity segmental myoclonus after surgical decompression of a CM-1 with syringomyelia. Thus, Chiari decompression should be considered in cases of myoclonus with CM-1 and syringomyelia.

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Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort

Cited by 7 publications

References 28 publications

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Symptoms

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Symptoms

Above-The-Hairline Suboccipital Transverse Approach for the Treatment of Chiari I Malformation

Surgical Treatment of Upper Extremity Segmental Myoclonus in an Adolescent with Chiari Malformation and Cervicothoracic Syrinx

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