Chiari Malformation (Arnold-Chiari Malformation)

Downward herniation of the cerebellum through the opening at the skull base - a congenital structural anomaly

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Pediatric Neurosurgery

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1Overview

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What is this Condition?

Chiari malformation is a congenital structural anomaly characterized by herniation of the cerebellum and sometimes the brainstem through the large opening at the base of the skull (foramen magnum) into the spinal canal. In the most common Type 1, only the lower tips of the cerebellum descend somewhat and symptoms typically appear at a more advanced age; in many individuals there are no symptoms and the condition is incidentally discovered on MR performed for another reason. Type 2 occurs together with spinal dysraphism (spina bifida), is more prominent, and is usually recognized at birth. This herniation can obstruct the free flow of fluid surrounding the brain and spinal cord (cerebrospinal fluid), leading to development of a fluid-filled cavity within the spinal cord (syrinx) or fluid accumulation in the brain (hydrocephalus). In symptomatic patients, surgery to decompress this region (posterior fossa decompression) relieves fluid flow and attempts to reduce symptoms. Early diagnosis is important in preventing permanent spinal cord damage.

Symptoms

Headache - particularly pain that worsens with coughing, sneezing, or straining, originating from the occiput

Neck pain and neck stiffness

Balance and coordination disturbance, clumsiness

Numbness, tingling, and weakness in the hands

Dysphagia and sensation of choking

Diplopia, blurred vision, and abnormal eye movements

Hearing loss, tinnitus, and vertigo

Sleep apnea and irregular breathing during sleep

Back and arm pain (when a syrinx develops in the spinal cord)

Spinal curvature (scoliosis) - particularly in Type 2

Diagnosis Methods

Diagnosis is established through neurological examination and imaging. The most reliable method is MR, which clearly demonstrates how far the cerebellum has descended and whether a syrinx (fluid-filled cavity) is present in the spinal cord. A special MR study evaluating cerebrospinal fluid flow (cine MR) reveals the extent of flow obstruction in this region and provides valuable information for surgical planning. When detailed visualization of bone structures is needed, CT can be added. Tests measuring nerve and spinal cord function (EMG and evoked potential studies) are performed when indicated. If hydrocephalus is suspected, additional evaluations may be requested.

Treatment Methods

Treatment varies depending on whether symptoms are present and their severity. Asymptomatic Type 1 patients who are incidentally discovered generally do not require surgery; they are monitored with annual MR and clinical examination. In symptomatic patients, surgery to decompress this region (posterior fossa decompression) is performed: a small piece of bone is removed from the posterior-inferior skull and, if necessary, from the uppermost cervical vertebra to create space for the cerebellum and brainstem. In most cases, the dura is also opened and enlarged with a patch (duraplasty) to provide greater decompression. If a syrinx (fluid-filled cavity) is present in the spinal cord, it often shrinks on its own once fluid flow is restored; if necessary, it is also drained separately. Type 2 typically requires repair of the spinal dysraphism at birth and placement of a shunt to relieve fluid accumulation. After surgery, many patients experience reduction or resolution of symptoms. Physical therapy, pain management, and regular follow-up are part of the process.

Posterior fossa decompression (surgery to create space in the posterior-inferior skull)

Bone removal from the uppermost cervical vertebra (to create space)

Dural enlargement with patch (duraplasty)

Drainage of syrinx (fluid-filled cavity in the spinal cord) if present

Procedures directed at the cerebellar tonsils (in rare cases)

Shunt placement if hydrocephalus is present to drain fluid

Physical therapy and rehabilitation

Pain management and neurology follow-up

Treatment Path

Related Treatments

The procedures and interventions most commonly explored with this condition.

4 links

Oksipitoservikal posterior dekompresyon + duraplasti

Oksipitoservikal posterior dekompresyon + duraplasti procedure

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Oksipitoservikal stabilizasyon ve greft

Oksipitoservikal stabilizasyon ve greft procedure

Open treatment detail

Endoscopic Arachnoid Cyst Fenestration

Endoscopic Arachnoid Cyst Fenestration procedure

Open treatment detail

External Ventricular Drainage

External Ventricular Drainage procedure

Open treatment detail

Editorial Notes

References

Greenberg MS. Handbook of Neurosurgery. 10th ed. Thieme; 2023.
Chiari Malformations. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 6th ed. Elsevier; 2011.
Chiari I Malformation and Associated Syringomyelia. In: Di Rocco C, Pang D, Rutka JT, eds. Textbook of Pediatric Neurosurgery. Springer; 2020.
Tavallaii A. Chiari Malformation. In: Pediatric Neurosurgery for Clinicians. Cham: Springer; 2022.

This content is for informational purposes and based on academic sources; consult your physician for diagnosis and treatment.

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Chiari Malformation (Arnold-Chiari Malformation)

Pediatric Neurosurgery

1Overview

What is this Condition?

Symptoms

Diagnosis Methods

Treatment Methods

Related Treatments

Oksipitoservikal posterior dekompresyon + duraplasti

Oksipitoservikal stabilizasyon ve greft

Endoscopic Arachnoid Cyst Fenestration

External Ventricular Drainage

Related Blog Posts

Chiari Malformation: When Is Surgery Considered for Headache Worsened by Coughing?

References

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Related Treatments

Oksipitoservikal posterior dekompresyon + duraplasti

Oksipitoservikal stabilizasyon ve greft

Endoscopic Arachnoid Cyst Fenestration

External Ventricular Drainage

Related Blog Posts

Chiari Malformation: When Is Surgery Considered for Headache Worsened by Coughing?

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