Table 5. [Treatment of Manifestations in Individuals with Osteopathia Striata with Cranial Sclerosis]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Scoliosis	Management per orthopedic surgeon
Joint contractures	PT may be helpful.
Orofacial clefting	Management per ENT surgeon
Hearing loss	Management per audiologist Speech-language therapy Surgical management per otolaryngologist should be considered on a case-by-case basis if underlying pathology involves foraminal compression.	Community & support groups for hearing loss Sclerotic bone poses surgical challenges [Katsevman et al 2016], and the success of surgical decompression of cranial nerve foramina in OS-CS is not established.
Vision loss	Management per ophthalmologist	Community & support groups for vision loss
Surgical management per neurosurgeon should be decided on a case-by-case basis.	Sclerotic bone poses surgical challenges [Katsevman et al 2016], and the success of surgical decompression of cranial nerve foramina in OS-CS is not established.
Cognition	Early intervention services & special education services as indicated
Cardiac anomalies	Treatment per cardiologist
Genitourinary anomalies	Treatment per urologist &/or nephrologist
Gastrointestinal anomalies	Treatment per gastroenterologist &/or surgery
Wilms tumor &/or other malignancy	Standard treatment

Manifestation/
Concern

Treatment

Considerations/Other

Scoliosis

Management per orthopedic surgeon

Joint contractures

PT may be helpful.

Orofacial clefting

Management per ENT surgeon

Hearing loss

Management per audiologist
Speech-language therapy
Surgical management per otolaryngologist should be considered on a case-by-case basis if underlying pathology involves foraminal compression.

Community & support groups for hearing loss
Sclerotic bone poses surgical challenges [Katsevman et al 2016], and the success of surgical decompression of cranial nerve foramina in OS-CS is not established.

Vision loss

Management per ophthalmologist

Community & support groups for vision loss

Surgical management per neurosurgeon should be decided on a case-by-case basis.

Sclerotic bone poses surgical challenges [Katsevman et al 2016], and the success of surgical decompression of cranial nerve foramina in OS-CS is not established.

Cognition

Early intervention services & special education services as indicated

Cardiac anomalies

Treatment per cardiologist

Genitourinary
anomalies

Treatment per urologist &/or nephrologist

Gastrointestinal
anomalies

Treatment per gastroenterologist &/or surgery

Wilms tumor &/or
other malignancy

Standard treatment

From: Osteopathia Striata with Cranial Sclerosis

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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