Treatment and Standard of Care for DGI-II

Because Dentinogenesis Imperfecta Type 2 (DGI-II) causes teeth to wear down much faster than normal, the standard of care focuses on early, proactive protection ^[1][2]. Waiting for symptoms to appear is often risky, as the soft dentin can be lost rapidly once the protective enamel flakes away ^[3][1].

When to See a Dentist

For children with a family history of DGI-II, the first dental visit should happen as soon as the first baby tooth erupts (usually around 6 months of age). Early evaluation allows the dental team to plan protective treatments before rapid wear begins ^[2].

Protecting Baby (Primary) Teeth

In young children, the goal is to keep the baby teeth functional until the adult teeth are ready to come in ^[2].

• Stainless Steel Crowns (SSCs): These are often the “gold standard” for baby teeth in children with DGI-II ^[2]. They provide full-coverage protection, meaning they encase the entire tooth to prevent any further wear or chipping ^[2][4].
• Preventing “Nerve” Exposure: Because the teeth wear down so fast, the center of the tooth (the pulp) can become exposed or infected ^[5]. Crowns act as a shield to keep the tooth sealed and healthy.

Transitioning to Adult (Permanent) Teeth

As adult teeth emerge, the strategy shifts toward long-term durability and aesthetics ^[4].

• Full-Coverage Crowns: Just like with baby teeth, adult teeth usually require full-coverage restorations ^[6]. Materials like zirconia, nanoceramics, or CAD/CAM (computer-aided design) resin crowns are frequently used because they are strong enough to withstand chewing forces and can be matched to a natural tooth color ^[4][6].
• Avoiding Simple Fillings: Using standard “white fillings” (composite resin) for large areas of wear is often considered a temporary or substandard fix in DGI-II ^[7]. Because the underlying dentin is weak, these fillings often fail to stay bonded or don’t provide enough strength to stop the tooth from continuing to wear down underneath them ^[8][3].

Preserving the Vertical Dimension

One of the most critical goals of treatment is preserving the Vertical Dimension of Occlusion (VDO) ^[5]. This is essentially the “height” of your face when your teeth are biting together ^[5].

If the teeth are allowed to wear down significantly, the VDO drops, making the lower face look shorter and potentially causing jaw joint (TMJ) pain or making future dental work much more difficult ^[5][9]. By placing crowns early, dentists can “lock in” the correct bite height, ensuring there is enough space for adult teeth and future restorations ^[5].

A Team Approach

Managing DGI-II is a multidisciplinary effort, meaning it often requires several types of specialists working together over many years ^[2][10].

1. Pediatric Dentist: Typically leads the early care, focusing on the baby teeth and monitoring growth ^[2].
2. Prosthodontist: A specialist in restoring and replacing teeth, who often takes over when it is time for complex adult crowns or full-mouth rehabilitation ^[4][9].
3. Orthodontist: May be needed to help manage tooth spacing and ensure the bite is properly aligned as adult teeth come in ^[11].
4. Endodontist: If a tooth becomes infected, an endodontist specializes in navigating the “obliterated” (filled-in) pulp canals that are common in DGI-II ^[12].