Marion et al developed the Fetal AIDS syndrome scoring system to describe dysmorphic features in children with congenital HIV infection. The authors are from Albert Einstein College of Medicine in New York City.
Parameters:
(1) growth deficiency in height and weight
(2) microcephaly
(3) forehead
(4) nasal bridge
(5) prominence of eyes
(6) sclerae
(7) hypertelorism
(8) obliquity of eyes
(9) columella nasi (the fleshy distal margin of the nasal septum)
(10) philtrum
(11) lips
|
Parameter |
Finding |
Points |
|
growth deficiency |
height AND weight > 3rd percentile |
0 |
|
|
height OR weight < 3rd percentile |
1 |
|
|
height AND weigh < 3rd percentile |
2 |
|
microcephaly |
head circumference > 3rd percentile for chronological age |
0 |
|
|
head circumference > 3rd percentile for height age but < 3rd percentile for chronological age |
1 |
|
|
head circumference < 3rd percentile for height age |
2 |
|
forehead |
not prominent |
0 |
|
|
slightly prominent |
1 |
|
|
very prominent, boxlike |
2 |
|
nasal bridge |
not flattened |
0 |
|
|
slightly flattened |
1 |
|
|
very flattened |
2 |
|
prominence of eyes |
not prominent |
0 |
|
|
very prominent |
1 |
|
sclerae |
white or yellow |
0 |
|
|
blue |
1 |
|
hypertelorism |
inner and outer canthal distances < 95% for head circumference age |
0 |
|
|
inner and outer canthal distances > 95% for head circumference age |
1 |
|
obliquity of eyes |
none |
0 |
|
|
upward |
1 |
|
|
downward |
1 |
|
columella (nasi) |
normal |
0 |
|
|
flattened |
1 |
|
philtrum |
flattened |
0 |
|
|
triangular and prominent |
1 |
|
lips |
thin upper vemilion |
0 |
|
|
patulous |
1 |
|
|
normal |
1 |
where:
• I would have thought that a normal upper lip should be scored 0.
• The ranges for height, weight and canthal distances do not cover the equal condition. I will use <= rather than < in the implementation.
• I don't think I have ever seen a table showing head circumference for height age.
total score =
= SUM(points for all 11 parameters)
Interpretation:
• minimum score: 0
• maximum score: 15
• The higher the score the more severe the dysmorphic features.
|
Total Score |
Fetal AIDS Syndrome |
|
0 to 8 |
none or mild |
|
9 to 11 |
moderate |
|
12 to 15 |
severe |
Differential diagnosis: The fetal AIDS syndrome needs to be distinguished from fetal alcohol syndrome and other dysmorphic syndromes.
Purpose: To evaluate a child for the fetal AIDS syndrome using the score of Marion et al.
Specialty: Pedatrics, Pharmacology, clinical
Objective: criteria for diagnosis, severity, prognosis, stage, comorbid conditions
ICD-10: O98.7, B24,
