Cocaine-induced midline destructive lesion (CIMDL), a consequence of snorting cocaine powder, begins with necrosis of the nasal pyramid and can spread to involve adjacent structures including the clivus, craniocervical junction, anterior and middle cranial fossae.

Terminology

Snorting refers to the practice of forcibly inhaling cocaine hydrochloride powder (‘snow’) through the nose ¹.

Epidemiology 

Cocaine use is widespread and increasing ¹; the drug is addictive and tolerance drives dose increases ⁶. Sporadic use is common and can cause myocardial infarction, arrhythmias and stroke. Regular use can cause CIMDLS, which is dose and duration-dependent. A history of illegal drug use can be difficult to obtain, so the true incidence is unknown.

Diagnosis

If a history is unavailable, blood tests can remain positive for 2 days, urine tests for 2-4 days, and hair for 3 months ⁶. Patients may test ANCA positive, and p-ANCA autoantibody (especially to human neutrophil elastase) is particularly helpful in distinguishing CIMDLS from the pattern seen in granulomatosis with polyangiitis (GPA).

Clinical presentation 

Symptoms are due to destruction or secondary infection and include disfigurement, oronasal regurgitation, altered sense of smell and taste, epistaxis and dysphonia.

Pathology

Cocaine is a sodium channel blocker with local anesthetic action, an irritant, and a potent vasoconstrictor ⁶. Microvascular ischemia can lead to mucosal cell apoptosis with inflammation and immunological changes and a predisposition to secondary infection. Necrosis and destruction spread to contiguous tissue and can continue for years following cessation.

Radiographic features

CT

CT demonstrates bone and soft tissue destruction ²:

• nasal pyramid bone and cartilage including septum and turbinates

• paranasal sinuses

• clivus ³ and craniocervical junction ⁵

• pneumocephalus

MRI 

MRI optimally demonstrates ⁴:

• invasion of the skull base and anterior and middle cranial fossae

• frontal lobe syndrome ⁴

• spread of secondary infection 

Treatment and prognosis

• cessation is an essential first step but destruction can progress for years afterwards

• infection control, frequently anaerobic, multi-drug resistant, sometimes fungal

• prosthetic occlusion of the palate, which increases the risk of infection

• saline spray to moisturize

• lubrication with hyaluronate spray 

• surgical correction is delayed for at least one year following cessation

Differential diagnosis

A number of conditions could present with midline facial destruction ¹:

• granulomatous disease:

  • granulomatosis with polyangiitis (affects heart, lungs and kidneys; often c-ANCA positive)

  • sarcoidosis

• bacterial infections:

  • tuberculosis

  • leprosy

  • syphilis

  • rhinoscleroma

  • actinomycosis

• fungal disease:

  • histoplasmosis

  • blastomycosis

  • coccidioidomycosis

  • mucormycosis

• parasites:

  • leishmaniasis 

  • myiasis

• neoplastic disease: extranodal T-cell lymphoma