General anesthesia can be difficult and risky due to the anatomical changes, including short neck, jaw immobility and upper airway obstruction, seen in Hunter syndrome (MPS II) patients.


Cardiac involvement is present in nearly all Hunter syndrome patients and is a major cause of death in this population. Cardiac disease can present as early as 5 years of age.

Ear, nose and throat

Normal breathing is inhibited because of the skeletal manifestations of Hunter syndrome, such as stiffness of the chest wall, abnormally shaped ribs and restricted movement of the temporomandibular joints and also as a result of the abdominal distention caused by an enlarged liver and spleen.


Depending on the disease severity, neurological manifestations may include developmental delay, cognitive impairment, behavioral problems, seizures, spinal cord compression, carpal tunnel syndrome (CTS) and communicating hydrocephalus.

Pediatric surgeon

As a pediatric surgeon, you may be one of the first physicians to suspect Hunter syndrome (MPS II). In the Hunter Outcomes Survey (HOS), the majority of patients (56.8%) underwent at least one surgical intervention before Hunter syndrome was diagnosed.

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MPS Day 2017

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