Dermatologic Manifestations of Proteus Syndrome Treatment & Management

Updated: Sep 16, 2022
  • Author: Shekhar Neema, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Treatment

Surgical Care

Preoperative evaluation should include assessment of the airway anatomy and pulmonary reserve because of the frequent presence of tonsillar hypertrophy and pulmonary cysts. [40]

Plastic surgeons and orthopedic surgeons can correct some skeletal defects. Epiphysiodesis can help correct asymmetric epiphyseal growth, and reduction osteotomy can be used to shorten long bones. Spinal fusion for overgrown vertebrae may prevent the development of severe kyphoscoliosis and the risk of pulmonary compromise. [22, 8]

Subcutaneous tumors should be removed in the early stages. [41]

Gonadal deformities (ovarian or testicular) should be managed aggressively because of the high incidence of neoplastic transformation. [40] The Medscape Testicular Cancer Resource Center and Ovarian Cancer Resource Center may be of interest.

Next:

Consultations

Consult a dermatologist.

Consult a plastic surgeon for the correction of defects.

Consult an orthopedic surgeon for the correction of skeletal deformities.

Ophthalmologists and neurologists may be helpful because many ocular manifestations and CNS anomalies associated with seizures or developmental delays are reported in patients with Proteus syndrome.

Referral to psychologist or psychiatrist is important for patients and for their families. The disease creates an isolating social stigma associated with having a very rare, progressive, disfiguring condition. Symptoms of depression have been reported in 23% of parents of affected children. [42]

Previous