Table 6.

Treatment of Manifestations in Individuals with Noonan Syndrome with Multiple Lentigines

Manifestation/ConcernTreatmentConsiderations/Other
Short stature Growth hormone therapy may be considered, although no data on use of growth hormone therapy in persons w/NSML exist.
  • Growth hormone therapy may be contraindicated in persons w/HCM.
  • Prior to instituting growth hormone therapy, cardiac eval for HCM is recommended; continued surveillance for development of HCM while on growth hormone therapy is reasonable.
HCM Standard treatment per cardiologist & cardiovascular surgeon
Structural heart defects
Hearing Hearing aids may be helpful; per otolaryngologist.Community hearing services through early intervention or school district
Consider cochlear implantation for persons w/profound deafness.See Hereditary Hearing Loss and Deafness Overview.
Colobomata, stereopsis,
& abnormal eye mvmts 		Standard treatment per ophthalmologist
Seizures Standard treatment per neurologist
Cryptorchidism /
Genitourinary anomalies 		Standard treatment per urologist
Developmental delay /
Intellectual disability 		See Developmental Delay / Intellectual Disability Management Issues.
Family/Community
  • Ensure appropriate social work involvement to connect families w/local resources, respite, & support.
  • Coordinate care to manage multiple subspecialty appointments, equipment, medications, & supplies.
  • Ongoing assessment of need for palliative care involvement &/or home nursing
  • Consider involvement in adaptive sports or Special Olympics.

HCM = hypertrophic cardiomyopathy; NSML = Noonan syndrome with multiple lentigines

From: Noonan Syndrome with Multiple Lentigines

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