Alstrom syndrome surgery

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]; Raviteja Guddeti, M.B.B.S. [3]

Surgery

Sensorineural Hearing Loss

Myringotomy has been helpful in individuals with recurrent otitis media (‘glue ear’). Hearing can be maximized with bilateral digital hearing aids. Cochlear implantation has benefited some patients.

Cardiomyopathy

Cardiac transplantation has been successful in isolated cases.

Urological Disorders

Urinary diversion or self-catheterization to manage voiding difficulties are required in some individuals.

Renal Failure

Renal transplantation is indicated in patients with ESRD refractory to medical therapy. But it can be contraindicated in the presence of other complications including morbid obesity, uncontrolled diabetes, and cardiomyopathy.

Liver Dysfunction

Patients who fail to respond to medication and banding are candidates for a transjugular intrahepatic portosystemic shunt (TIPS) to decrease risk of variceal bleeding caused by portal hypertension. Patients with significant portal hypertension should be evaluated early for liver transplantation.

References

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