Collet-Sicard Syndrome

Collet-Sicard Syndrome
Other Names	Condylar Jugular Syndrome
Identifiers
ICD-9	352.6
ICD-10	G52.7
Outside Resources
Radiopaedia	Collet-Sicard Syndrome

Overview

Collet-Sicard Syndrome, also known as Condylar Jugular Syndrome, describes a collection of ipsilateral cranial nerve palsies (IX, X, XI, and XII) as a result of injury or mass effect at the skull base.

History

Frederic Collet first described a World War I patient in 1915 with this constellation of cranial nerve palsies following a bullet injury. This case presentation was seen again by Jean Sicard in 1917.

Pathophysiology

Relevant Anatomy

Collet-Sicard syndrome is a result of injury or mass effect to cranial nerves IX, X, and XI at the jugular foramen (in isolation known as Vernet Syndrome), with the additional involvement of CN XII (most commonly at the hypoglossal canal). Readers should be familiar with the anatomy of the cranial nerves and their respective skull base foramina.

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  Skull base foramina, superior and inferior views

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  Skull base foramina

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  Cranial Nerves IX, X, and XII exiting the skull base

Disease Etiology

The etiology of Collet-Sicard syndrome can be divided into neoplastic and non-neoplastic causes:

Neoplastic

• Jugular Paraganglioma
• Meningioma
• Schwannoma
• Metastatic Disease

Non-Neoplastic

• Trauma
• Paget Disease
• Osteomyelitis of the Skull Base
• Vascular Disease

Diagnosis

Patient History

Patient should be screened for the following symptoms:

• Dysphagia
• Voice changes
• Aspiration
• Shoulder / arm weakness
• Altered taste

Pertinent history to assess for:

• History of head trauma
• History of malignancy
• History of aspiration pneumonia
• Prior head and neck radiation
• Family history of glomus tumors / paragangliomas

Physical Examination

Tongue deviation from hypoglossal nerve injury

All cranial nerves should be assessed in the workup of Collett-Sicard to rule out other cranial nerve involvement (potentially leading to a different diagnosis). Cranial nerves IX, X, XI, and XII are expected to be weak or paralyzed.

• Altered sense of taste (CN IX)
• Diminished gag reflex (CN IX, X)
• Vocal cord weakness / immobility (CN X)
• Weakened shoulder shrug (CN XI)
• Weakened arm abduction over 90 degrees (CN XI)
• Tongue deviation to the side of weakness on tongue protrusion (CN XII)

Laboratory Tests

Laboratory testing may not be necessary in patients with a low index of suspicion for infectious causes. In patients where you suspect an infectious etiology, such as osteomyelitis of the skull base, routine infectious laboratory workup is warranted:

• Complete blood count (CBC) with differential
• C-Reactive Protein (CRP) / Erythrocyte Sedimentation Rate (ESR)
• Blood cultures

Imaging

In many instances, patients will already have a CT Brain at the time of ENT consultation to rule out a central cause of their cranial nerve deficits. MRI of the skull base with and without contrast will be the ideal imaging modality in most neoplastic causes of Collet-Sicard syndrome. For patients with a traumatic etiology, CT skull base will identify any fractures or bone chips resulting in the nerve palsies. Consider CT angiogram of the carotid system if there is trauma to the skull base as there may be concomitant injury to the carotid canal.

Differential Diagnosis

There are several named syndromes differentiating the various cranial nerve deficits that can result from skull base masses and lesions. These should be considered based on cranial nerve involvement.

• Vernet Syndrome
• Villaret Syndrome
• Tapia Syndrome
• Jackson Syndrome
• Schmidt Syndrome

These syndromes and their respective cranial nerve involvement are outlined in the table below.

Cranial Nerve Involvement in Skull Base Masses

Syndrome	CN IX	CN X	CN XI	CN XII	Sympathetics
Vernet Syndrome	✔	✔	✔
Collet-Sicard Syndrome	✔	✔	✔	✔
Villaret Syndrome	✔	✔	✔	✔	✔
Tapia Syndrome		✔	±	✔	±
Jackson Syndrome		✔	✔	✔
Schmidt Syndrome		✔	✔