Table 5. [Treatment of Manifestations in Individuals with FLNB Disorders]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Cervical spine instability	Asymptomatic infants: Early intervention to improve cervical spine stability using posterior arthrodesis is successful. Infants w/myelopathic signs: Function can be stabilized &/or improved by combination of anterior decompression & circumferential arthrodesis. ¹	Care must be taken to minimize extension of cervical spine intraoperatively.
Scoliosis	Medical treatment per orthopedist	No effective surgical intervention has been described.
Large joint dislocations	Operative reduction is usually required.	Conservative, nonsurgical mgmt of hip dislocation in Larsen syndrome is often unsuccessful.
Clubfeet	Routine mgmt per orthopedist
Laryngotrach-eomalacia	Anesthetic agents that exhibit more rapid induction & recovery are preferred.	Due to ↑ risk for airway complications in persons w/Larsen syndrome
Cleft palate	Treated by multidisciplinary craniofacial team when possible
Hearing loss	Possible treatments incl: hearing aids, vibrotactile devices, & cochlear implantation (See Hereditary Hearing Loss and Deafness Overview).	Ideally, by ENT & audiologist w/expertise in early-childhood otologic disorders The expertise of an educator of the Deaf may be required. An important part of eval is determining appropriate habilitation option.

Manifestation/
Concern

Treatment

Considerations/Other

Cervical spine
instability

Asymptomatic infants: Early intervention to improve cervical spine stability using posterior arthrodesis is successful.
Infants w/myelopathic signs: Function can be stabilized &/or improved by combination of anterior decompression & circumferential arthrodesis. ¹

Care must be taken to minimize extension of cervical spine intraoperatively.

Scoliosis

Medical treatment per orthopedist

No effective surgical intervention has been described.

Large joint
dislocations

Operative reduction is usually required.

Conservative, nonsurgical mgmt of hip dislocation in Larsen syndrome is often unsuccessful.

Clubfeet

Routine mgmt per orthopedist

Laryngotrach-eomalacia

Anesthetic agents that exhibit more rapid induction & recovery are preferred.

Due to ↑ risk for airway complications in persons w/Larsen syndrome

Cleft palate

Treated by multidisciplinary craniofacial team when possible

Hearing loss

Possible treatments incl: hearing aids, vibrotactile devices, & cochlear implantation (See Hereditary Hearing Loss and Deafness Overview).

Ideally, by ENT & audiologist w/expertise in early-childhood otologic disorders
The expertise of an educator of the Deaf may be required. An important part of eval is determining appropriate habilitation option.

From: FLNB Disorders

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

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