Congenital Odontoid Abnormalities

Ossification

 

JAAOS 2019

 

Atlas

 

Three 1° Ossification centres

- two lateral masses with posterior arch in between

- anterior arch which appears at 1 year

- posterior arch fuses at 4 years

- anterior & posterior fuses to body at 7 years

 

Axis

 

Five 1° Ossification centres

- two neural arches / lateral masses

- two 1/2 dens

- body

 

Five 2° Centres

- Os Terminale (Appears 3, fuses 12)

- inferior ring apophysis

- 2 transverse processes

- 1 spinous process

 

Body of odontoid fuses by birth from two centres

- failure = Dens Bicornis

 

Dens separated from body by Dentocentral Synchondrosis

- present at birth

- fuses by 6 years

 

Tip of Odontoid not ossified at birth

- ossifies by age 3

- fuses by age 12

- Ossicum Terminale Persistens if doesn't fuse

 

Deformities

 

1. Os Odontoideum

- failure of fusion of base of odontoid

- normally fuses by age 6

- appears like Type II fracture

 

2. Odontoid Hypoplasia

- congenitally small odontoid

- asssociated with Morquio Syndrome & SED

- can get AAI 2° Hypoplasia 

 

3. Odontoid Aplasia

- absent odontoid

 

4. Os Terminale Persistens

- unfused terminal apophysis

- normally fuses by age 12

 

Os Ondontoideum

 

Theories

 

Congenital

 

Developmental

- ? os odontoideum is a traumatic event

 

Clinical Features

 

Usually asymptomatic

 

May present in adulthood with

- pain (Usually dull ache)

- neurologic symptoms

- Lhermitte sign / lightning pain

- long tract signs

- risk of sudden death

 

X-ray

 

Open-mouth view

 

Instability

- > 3.5 mm translation on FE views

- ADI > 5 mm

- SAC also important if < 14 mm

 

Surgical Indications

 

Non syndromic

 

1.  > 10 mm FE motion

2.  Painful neck with instability 

3.  Neurology

 

Prophylactic neck fusion is controversial

- if asymptomatic avoid contact sport

 

SED /  Morquio

 

ADI > 5mm = Fusion

 

Technique

 

Gallie Fusion

- involves using notched bone graft

- sitting it between the posterior arch of C1 and the spinous process and medial lamina arch of C2

- secured by sublaminar wires 

- this is not inherently rotationally stable

- usually requires halo immobilisation