Multidisciplinary management of Hunter syndrome.

Hunter syndrome is a rare, X-linked disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase. In the absence of sufficient enzyme activity, glycosaminoglycans accumulate in the lysosomes of many tissues and organs and contribute to the multisystem, progressive pathologies seen in Hunter syndrome. The nervous, cardiovascular, respiratory, and musculoskeletal systems can be involved in individuals with Hunter syndrome. Although the management of some clinical problems associated with the disease may seem routine, the management is typically complex and requires the physician to be aware of the special issues surrounding the patient with Hunter syndrome, and a multidisciplinary approach should be taken. Subspecialties such as otorhinolaryngology, neurosurgery, orthopedics, cardiology, anesthesiology, pulmonology, and neurodevelopment will all have a role in management, as will specialty areas such as physiotherapy, audiology, and others. The important management topics are discussed in this review, and the use of enzyme-replacement therapy with recombinant human iduronate-2-sulfatase as a specific treatment for Hunter syndrome is presented.

Polydiaxonon prevents adhesions in the rabbit model: a pilot report.

PURPOSE: Restrictive strabismus is a common and difficult problem confronted by strabismologists. Several materials have been used to minimize the formation of adhesions after strabismus surgery with varying degrees of success. Polydiaxonon (PDS, Ethicon) is an absorbable, nontoxic, nonporous material. We used it as 25 and 50 microm thick sleeves to study its effectiveness in the prevention of adhesions. METHOD: Eight eyes of four adult New Zealand White rabbits were used. To simulate the adhesions, a raw scleral bed was created under the superior rectus insertion in study animals and the muscle capsule facing the sclera was opened. Four study eyes had PDS sleeves inserted around the superior rectus; the other four served as controls. After 4 months the animals were killed. The surgical sites were inspected for adhesions. Light microscopy was also performed. RESULTS: Virtually no adhesion formation was noted in the study eyes. In the control group, however, dense adhesions were seen. Light microscopy confirmed these results. No significant amount of foreign material was found. There was no toxicity resulting from PDS. CONCLUSIONS: This demonstrated nearly complete prevention of adhesions in the rabbit model. PDS sleeves appear to have potential in surgery for restrictive strabismus.