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Clinical Risk Factors of Khosrotehrani for Mortality in Patients with Neurofibromatosis Type 1

Description

Khosrotehrani et al identified risk factors observable on clinical examination that are associated with an increased risk of mortality in patients with neurofibromatosis type 1. This can help identify a subset of patients who may benefit from closer monitoring. The authors are from Hopital Henri-Mondor in Paris.


 

Parameters:

(1) subcutaneous neurofibromas (firm, beadlike nodules arising along the length of a nerve)

(2) cutaneous neurofibromas

(3) facial symmetry

Parameter

Findings

Risk Factor

subcutaneous neurofibromas

0 or 1

0

 

>= 2

1

cutaneous neurofibromas

>= 1

0

 

0

1

facial symmetry

symmetrical

0

 

asymmetrical

1

 

where:

• Facial asymmetry was associated with bone dysplasia, plexiform neurofibromas and intracranial tumors.

 

Risk Factor

Odds Ratio

95% CI

p value

subcutaneous neurofibromas

10.8

2.1 – 56.7

< 0.001

no cutaneous neurofibromas

5.3

1.2 – 25.0

< 0.03

facial asymmetry

11.4

2.6 – 50.2

< 0.01

 

number of risk factors =

= SUM(points for all 3 risk factors)

 

Interpretation:

• minimum number of risk factors: 0

• maximum number of risk factors: 3

• A patient with 1 or more risk factors is at increased risk for mortality and should be evaluated more carefully.

 

Causes of mortality:

(1) malignant nerve sheath tumors

(2) spinal cord compression

(3) hydrocephalus

(4) complications of hypertension

(5) severe seizures

 


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