The Dental Aspects of X-Linked Hypophosphataemia

“Spontaneous” abscesses are a common dental consequence associated with XLH

Patients with XLH have an increased risk of developing periodontal disease with more severe consequences, such as premature tooth loss, even in young adults.

You may develop infections in the bone around the teeth, without any clinical or X-ray changes (no cavities or injury), contrary to what is conventionally observed. The barrier function of the enamel and dentine is compromised and bacteria can easily reach the pulp.

Once the pulp is infected, an ABSCESS (collection of pus) or a FISTULA (hollow tunnel that allows pus to escape) may develop.

Infection can sometimes spread rapidly to the facial tissue causing CELLULITIS, a swelling of the face with an alteration in the overall condition, but without any obvious clinical dental signs.

In case of an ABSCESS or FISTULA, you should URGENTLY see a dentist.
In case of CELLULITIS, you should see your dentist IMMEDIATELY, or go to the nearest emergency unit, to start antibiotics

Anatomy of a normal tooth

Covers the crown of the tooth and acts as a protective barrier; it is a highly mineralised semi-transparent layer.

A less dense layer of hard (calcified) tissue beneath the enamel; the composition is similar to bone. The cohesion with enamel helps to cushion pressure on the tooth, especially when chewing, and it serves as a protective barrier for the pulp.

Tissue that is located in the tooth core, it is not calcified and contains the vessels and nerves of the tooth.

A calcified layer of tissue that covers the root of the tooth and anchors it in the jaw bone.

The specialised tissues that surround and support the teeth, maintaining them in the jaw bones.

Anatomy of an XLH affected tooth with dental abscess

The enamel is thinner and more prone to being worn down. Microscopic cracks are often present and allow bacteria to enter the pulp without the presence of cavities.

Dentine is less mineralised and may have structural defects allowing bacteria to enter the pulp; the barrier function is not very effective.

The pulp is generally enlarged and closer to the external surface of the tooth. Therefore, it is more vulnerable and susceptible to infections.

The pulp horns are more prominent.

It is thinner.

There may be abnormalities in the jaw bone, i.e. the support bones of the teeth.