Table 4. [Treatment of Manifestations in Individuals with Congenital Insensitivity to Pain]. - GeneReviews®

Manifestation/Concern	Treatment	Considerations/Other
Dental & oral lesions	Tooth extraction &/or filing (smoothing) of sharp incisal edges [Bodner et al 2002]; use of a mouth guard [Hutton & McKaig 2010]
Bone fractures	Standard treatment w/careful & regular review, assuming healing may not occur, & low threshold for repeat radiological imaging until normal	Treatment w/an external fixator may → potentially serious infectious complications.
Bone & joint deformity	Corrective osteotomy	Prolonged & intensive monitoring is necessary to avoid deformity or incomplete healing.
Leg length discrepancy	Shoe lift or epiphysiodesis [Bar-On et al 2002]	The value of surgical intervention needs to be weighed against nonsurgical approaches incl close monitoring [Kim et al 2013].
Corneal ulceration/ perforation/infection	Standard treatment w/careful & regular review, assuming healing may not occur	Corneal transplants will have increased risk of failure.
Dry eyes	Lubricating eye drops or ointments	Surgical treatment of neurotrophic keratitis has not been successful [Yagev et al 1999].
Long-standing infections	Wide surgical debridement	Prevention may be difficult but is important (See Prevention of Primary Manifestations and Prevention of Secondary Complications.)
Ulcerating foot lesions	Standard treatment w/careful & regular review, assuming healing may not occur	Appropriate footwear & ankle support & periods of non-weight-bearing may be appropriate.
Hyperthermia	Direct cooling in bath or w/cooling blanket	Control of environmental temperatures is essential.
Hypothermia	Warming by blanket
Skin dryness & cracking	Topical moisturizer (lotion or cream)	Untreated dry skin can → skin infections, which ↑ risk for serious infections (cellulitis or osteomyelitis).
Developmental delay / Intellectual disability	See Developmental Delay / Intellectual Disability Management Issues.

Manifestation/Concern

Treatment

Considerations/Other

Dental & oral lesions

Tooth extraction &/or filing (smoothing) of sharp incisal edges [Bodner et al 2002]; use of a mouth guard [Hutton & McKaig 2010]

Bone fractures

Standard treatment w/careful & regular review, assuming healing may not occur, & low threshold for repeat radiological imaging until normal

Treatment w/an external fixator may → potentially serious infectious complications.

Bone & joint deformity

Corrective osteotomy

Prolonged & intensive monitoring is necessary to avoid deformity or incomplete healing.

Leg length discrepancy

Shoe lift or epiphysiodesis [Bar-On et al 2002]

The value of surgical intervention needs to be weighed against nonsurgical approaches incl close monitoring [Kim et al 2013].

Corneal ulceration/
perforation/infection

Standard treatment w/careful & regular review, assuming healing may not occur

Corneal transplants will have increased risk of failure.

Dry eyes

Lubricating eye drops or ointments

Surgical treatment of neurotrophic keratitis has not been successful [Yagev et al 1999].

Long-standing infections

Wide surgical debridement

Prevention may be difficult but is important (See Prevention of Primary Manifestations and Prevention of Secondary Complications.)

Ulcerating foot lesions

Standard treatment w/careful & regular review, assuming healing may not occur

Appropriate footwear & ankle support & periods of non-weight-bearing may be appropriate.

Hyperthermia

Direct cooling in bath or w/cooling blanket

Control of environmental temperatures is essential.

Hypothermia

Warming by blanket

Skin dryness & cracking

Topical moisturizer (lotion or cream)

Untreated dry skin can → skin infections, which ↑ risk for serious infections (cellulitis or osteomyelitis).

Developmental delay /
Intellectual disability

See Developmental Delay / Intellectual Disability Management Issues.

From: Congenital Insensitivity to Pain Overview

GeneReviews^® [Internet].

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

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