Haberland Syndrome Treatment & Management

Updated: Jun 25, 2018
  • Author: Sergiusz Jozwiak, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Approach Considerations

There are no effective treatment modalities for encephalocraniocutaneous lipomatosis (ECCL).

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Medical Care

Therapy of cutaneous and subcutaneous lesions, especially on the face and cranium, is challenging. Zielinska-Kazmierska et al reported mandibular reconstruction after resection of a large osteoma. [26]

Antiepileptic treatment is administered in patients with clinical epileptic fits.

Antiarrhythmic treatment may be required in patients with cardiac rhythm abnormalities.

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Surgical Care

Surgical treatment may be necessary in patients with extensive skin lesions. [27]

Selected patients with drug-resistant epilepsy may benefit from neurosurgical intervention, as reported by Roszkowski et al. [28]

Most spinal lipomas are asymptomatic, and surgery is usually not required. However, in some cases they may be causing neurological deficits and require surgical removal. [29] Surgical treatment may also be necessary if low-grade gliomas develop.

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Consultations

Consultation with a neurosurgeon, neurologist, ophthalmologist, and cardiologist is warranted as determined by history and physical examination findings.

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Activity

No special activity limitations are required for most patients.

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Long-Term Monitoring

Periodic neurologic and cardiologic assessment with echocardiography and electrocardiography may be indicated because of an anticipated progressive course.

Owing to reported midline low-grade gliomas in the suprasellar region in four encephalocraniocutaneous lipomatosis (ECCL) patients, some authors also recommend ophthalmologic and endocrinologic control in order to quickly discover any warning signs.

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