SPECIAL ANNUAL ISSUE Management of Chiari I malformation in children: personal opinions Joanna Gernsback 1 & Tadanori Tomita 1 Received: 22 March 2019 /Accepted: 28 April 2019 # Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Purpose This manuscript describes our management philosophy of Chiari I malformation in children based on a single neuro- surgeon’ s personal experience. Methods Based on 61 infants and children with Chiari I malformation treated from 2007 to 2017, typical symptoms, surgical indications, types of surgery, and evaluation of surgical decompression are reviewed. Results Sixty-one patients had 69 decompressions, with 90% having symptom improvement. Seven (11.5%) needed reoperation, 1 of which needed 2 reoperations for recurrence. The recurrence rates were 20% (5 of 25) after dural scoring and 5.6% (2 of 36) after duraplasty (p = 0.1116, Fisher’ s exact test). Six (16%) of 36 patients developed pseudomeningocele or CSF leak. Conclusions We recommend surgical intervention for Chiari I malformation for clearly symptomatic patients and those with significant hydromyelia regardless of symptoms. A bony decompression with dural scoring is recommended for patients with typical occipital headaches with a lesser degree of tonsillar descent, while an expansile duraplasty is standard for those with high- grade tonsillar descent, medullary kink, or hydromyelia. Intraoperative ultrasound is often helpful to ensure the adequacy of the decompression. Most patients will have improvements in symptom and imaging after either type of decompressive surgery. Keywords Chiari I malformation . Children . Craniotomy . Posterior fossa decompression Introduction The Chiari I malformation (CMI), with a displacement of the cerebellar tonsils below the foramen magnum, remains perplexing both in terms of its pathophysiology and its ideal treatment. While there are ongoing randomized studies and patient registries designed to determine the ideal treatment paradigm, current management generally follows institutional bias and personal experience. Typical symptoms The most common presentation of Chiari I malformation is headaches, particularly in the midline sub-occipital region. These headaches are due to an overcrowded craniocervical junction and lack of free CSF flow between the intracranial and spinal subarachnoid spaces. They are aggravated by Valsalva maneuvers such as coughing, sneezing, and laughing. Non-verbal infants and toddlers present with irrita- bility, nighttime waking, and arching back as a sign of dis- comfort [1]. Neurological disturbances are uncommon though some present with increasing gait unsteadiness or paresthesia, in the extremities associated with hydromyelia. In infants and young children, oropharyngeal symptoms such as snoring, gagging, and sleep apnea are common [1]. Thoracolumbar scoliosis is usually associated with holocord hydromyelia. Surgical indications Surgical indications are based on presenting symptoms. Patients with above-described symptoms usually need sur- gical decompression. On MRI, pointy cerebellar tonsils descending to or below the C1 arch or with a medullary kink are considered to be of more indicated. The presence of hydromyelia is usually an indication for surgical decom- pression. Watchful waiting was recommended for minimal- ly symptomatic patients and those with atypical headaches. This is in keeping with current global attitudes toward * Tadanori Tomita ttomita@luriechildrens.org 1 Division of Pediatric Neurosurgery, Ann and Robert H. Lurie Children’ s Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Avenue, Chicago, IL 60611, USA Child's Nervous System https://doi.org/10.1007/s00381-019-04180-6