Evidence map›Paper›PMID 41219586›Full record

ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2025

Posterior fossa decompression in syndromic children with chiari-like posterior fossa crowding: a nationwide US-based study.

Victor Gabriel El-Hajj, Josué Aganze Mwambali, Ihab Ahmad Al-Rikabi, Erik Öhlen, Maria Gharios, Victor E Staartjes, Joanna M Roy, Basel Musmar, Pascal Jabbour, Erik Edström and 1 more

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Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

11 authors.

Victor Gabriel El-HajjDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Josué Aganze MwambaliFaculty of Medicine, Université Catholique de Bukavu, Bukavu, Democratic Republic of Congo.
Ihab Ahmad Al-RikabiDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Erik ÖhlenDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Maria GhariosDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Victor E StaartjesDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Joanna M RoyDepartment of Neurosurgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
Basel MusmarMachine Intelligence in Clinical Neuroscience & Microsurgical Neuroanatomy (MICN) Laboratory, Department of Neurosurgery, University Hospital Zurich, Clinical Neuroscience Center, University of Zurich, Zurich, Switzerland.
Pascal JabbourMachine Intelligence in Clinical Neuroscience & Microsurgical Neuroanatomy (MICN) Laboratory, Department of Neurosurgery, University Hospital Zurich, Clinical Neuroscience Center, University of Zurich, Zurich, Switzerland.
Erik EdströmDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Adrian Elmi-TeranderDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden. adrian.elmi.terander@ki.se.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionPosterior fossa crowding, due to cerebellar tonsil herniation, often requires surgery with posterior fossa decompression (PFD). Although most cases are due to a Chiari-1 malformation (CM1), some are due to concomitant congenital conditions, mimicking a radiological CM1. The aim of this study was to compare PFD outcomes between CM1 and the syndromic Chiari-like crowding of the posterior fossa. A national pediatric surgical database was used to compare baseline characteristics and short-term postoperative outcomes.

methodsPediatric patients undergoing PFD (2012-2021) were identified in the ACS NSQIP-P database. Baseline characteristics and 30-day outcomes were compared between syndromic and non-syndromic cases. Multivariate logistic regression identified factors associated with readmission and reoperation.

resultsAmong 6,910 patients who underwent PFD, 752 (11%) had associated syndromes or conditions. The most common syndromes were chondrodystrophies (28%), fetal alcohol syndrome (14%), and Klippel-Feil syndrome (10%). Syndromic patients were younger, had lower BMI, and exhibited a significantly higher prevalence of comorbidities, including pulmonary, gastrointestinal, cardiac, and neurological conditions (all p < 0.001). They also experienced longer hospital stays (mean 5.17 vs. 3.64 days; p = 0.010), higher rates of 30-day complications (12% vs. 8.7%; p = 0.003), readmissions (9.4% vs. 7.1%; p = 0.021), and reoperations (6.6% vs. 2.9%; p < 0.001). In adjusted analysis, syndromic cases were not independently associated with increased 30-day complications or readmissions but remained significantly associated with 30-day reoperation (OR 1.51; 95% CI 1.03-2.19; p = 0.032).

conclusionThe syndromic Chiari-like condition is associated with distinct baseline characteristics and higher short-term complication rates following PFD compared to true CM1. While syndromic status was independently associated with reoperation, it was not an independent predictor of overall complications or readmissions. These findings suggest that patients with syndromes mimicking CM1 represent a clinically and etiologically distinct entity, warranting tailored management and further investigation.

Indexed as

Arnold-Chiari MalformationCranial Fossa, PosteriorDecompression, SurgicalNeurosurgical ProceduresAdolescentChildChild, PreschoolFemaleHumansMaleChiari malformationPediatricPosterior fossaSyndromic

Identifiers

PMID41219586
PMCPMC12605545

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.