REVISTA DE ORTOPEDIA INFANTIL DEL
HOSPITAL DE NIÑOS RICARDO GUTIERREZ

Editor: Dr.Néstor Vallejos Meana

PAPER Nº 3

Osteogénesis imperfecta.

Update: classification; diagnosis and treatment

O.I. is a congenital disease of type I collagen with a main sympton: bone fragility

Dr. Stefano M.D.

These patients suffered a multiorganic collagen affection such as dentinogénesis imperfecta, deafness, hyperlaxity, blue sclerae, short statue, ecc.

CLASSIFICATION

Congenita (Vrolik)

Tarda (Lobstein): Tarda gravis : with a lot of angulated bones

Tarda levis.

But Sillence four groups classification based on symptoms and inheritance is the most useful and accepted one:

Orthopedic management of fractures in O.I.. We must provide:

The best alignement: avoid angulation that trend to refractures.

Short time inmobilization: avoid disuse osteopenia and muscular athrophy

Easy to apply and to remove: Orthesis or splints.

Avoid unneccesary hospytalization

Consider I.M. rods since the first years ;Telescopic (Bailey-Dubow) or overlaping Rush nails

General Treatment.

The intravenous administration of dipphosphonats during the last

Decade has demostrated a lower rate of fractures and some improved calcification Psychosocial treatment with parents association, regional groups or virtual groups are also effective.

Permanent stimulation and rehabilitation is needed, to recover movement and improve muscular imballance.

Protection of long bones with special Orthesis and equipments is mandatory.

Coments: edstef@sinectis.com.ar