Maroteaux-Lamy Syndrome (Mucopolysaccharidosis Type VI) Follow-up

Updated: Mar 20, 2017
  • Author: Paul R Harmatz, MD; Chief Editor: Maria Descartes, MD  more...
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Follow-up

Further Outpatient Care

Physical therapy to maximize joint mobility is beneficial.

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Further Inpatient Care

Obstructive airway disease can result from narrowing of the trachea, enlarged tongue, and redundant tissue in patients with mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome. Tracheostomy has been performed in some patients. Tonsillectomy and adenoidectomy are also frequently performed to relieve obstruction.

Many patients develop carpal tunnel syndrome, which may require nerve decompression.

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Inpatient & Outpatient Medications

Patients with cardiac disease may require pharmacological therapy to manage cardiac failure. Monitor these patients closely with a pediatric cardiologist.

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Transfer

Transfer patients with MPS VI who require general anesthesia for any procedure to a facility equipped to handle the substantial anesthetic risks.

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Deterrence/Prevention

In most cases, a history of MPS VI is not present, and the affected child is the first affected individual in the family.

In future pregnancies, prenatal diagnosis is possible.

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Complications

Vision loss due to corneal clouding is common. Rapid vision loss may occur related to increased intracranial pressure or compression of optic nerve.

Hearing loss is common and may require hearing aids.

Carpal tunnel syndrome is a common complication.

Obstructive airway disease can result in hypoventilation and pulmonary hypertension.

Valvular heart disease is common and may lead to congestive heart failure.

Spinal cord compression with damage and paralysis may occur.

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Prognosis

MPS VI is a progressive disorder with significant morbidity and early mortality. As with many genetic inborn errors of metabolism, considerable variation exists among individual patients. Therefore, the prognosis for a particular patient must be determined after consideration of the presentation and complications.

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Patient Education

Counsel patients and their families about the autosomal recessive inheritance pattern of MPS VI, the risk for occurrence in future pregnancies, and the availability of prenatal diagnosis.

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