Page 27 - GP Fall 2024

A sealant was then placed over the glass Treatment plan: poor crown-to-root ratio due to blunted root
ionomer to mask the dark appearance of the Phase one of the treatment plan involves formation and root resorption. Patients may
SDF. An orthodontic consultation was also treating gingivitis to reduce inflammation. also present with taurodontism and delayed
performed, and the patient was diagnosed Oral hygiene instructions with modified tooth eruption. Kabuki syndrome patients
with a Class 3 malocclusion, constricted Bass Technique and flossing will be re- exhibit missing, fused, or unusually shaped
arch, and a crossbite on #10. In 2016, the viewed at each visit. Three-month recall ap- teeth. The oral alterations of high-arched
orthodontic department prescribed and de- pointments along with fluoride application palate, malocclusion, severe maxillary reces-
livered a maxillary Schwarz appliance, and are planned. Self-applied topical neutral flu- sion, and midfacial hypoplasia, small dental
in 2017, orthodontic fixed appliances were oride dentifrice containing 1.1% (w/w) so- arch, hypodontia, particularly the absence of
placed. Endodontic two-year follow-up for dium fluoride and 5% potassium nitrate will incisors and premolars, and diastema are fre-
#9 reevaluated the tooth as vital. The 2019 be prescribed for home care. A referral to quent findings (Figure 4).
7,9
re-care appointment by the pediatric dental the oral surgery department will be made for
department resulted in the extraction of #T exposure and eruption of second permanent
to allow for the eruption of #29. In 2023, molars. Radiographs will be taken at 12-18
the patient presented to the general dental month intervals.
department following the debonding of or-
thodontic appliances. However, since root Discussion: Kabuki Syndrome is a rare
resorption was observed, the patient was genetic condition affecting multiple organ
referred to oral surgery for evaluation of the systems. Identification of Kabuki Syndrome
possible exposure of impacted second per- patients occurs using the five cardinal fea-
1,4
manent molars. tures. These features are facial, skeletal,
dermatoglyphic, mild to moderate mental
Extraoral Exam: Extra-orally, the patient retardation, and postnatal growth deficien-
displayed the cardinal signs of Kabuki Syn- cy. 1,2,5 Due to the genetic variations of Ka- Figure 4. High-arched palate, malocclu-
drome: long palpebral fissures, depressed buki syndrome, a variety of physical pheno- sion, small dental arch and hypodontia.
nasal tip, and prominent ears. types were observed in the patient involving
the brain, heart, and endocrine system. His maxillary third molars are congenital-
11
Intraoral Exam: Intraorally, the patient The expression of the two genes responsible ly missing. His mandibular third molars are
demonstrated edematous and erythematous for Kabuki Syndrome is evident in the den- unerupted, and his second molars are im-
buccal gingiva which was diagnosed as tal epithelium in the early stages of human pacted.
moderate to severe generalized gingivitis tooth development suggesting their roles in
(Figure 2). odontogenesis, which explains the variety Ocular Manifestations: Kabuki syndrome
8
of dental findings in Kabuki syndrome pa- patients often report ophthalmologic issues.
tients. Strabismus is detected in 20-50% of KS pa-
tients. The patient had right eye strabismus,
Craniofacial and Oro-Dental Features: The which was surgically repaired in 2015. He
patient presents with radiographic evidence presently wears corrective lenses and has a
of dens invaginatus on #8 and #9, which blue sclera. 3,9,13
have a conical appearance. He also shows a

Figure 2. Patient KS demonstrates edema-
tous and erythematous buccal gingiva with
moderate to severe gingivitis.

Probing depths ranged from 0-3mm and
there was zero clinical attachment loss.
Visible copious amounts of plaque and in-
flammation were recorded on several sites.
Bleeding sites were 72 with 50% inflamma-
tion noted (72/144). Caries risk was moder-
ate due to visible plaque on multiple tooth
surfaces. Radiographic analysis revealed a
malformation of teeth and roots. Dental in-
vaginatus was noted on #8 and #9. Mandib-
ular third molars were unerupted, maxillary
third molars were congenitally missing, and
second molars were impacted (Figure 3). Figure 3. Radiographic analysis shows malformation of teeth and roots.
Dental invaginatus is visible on #8,#9. Unerupted 2nd maxillary molars (#2,15).
www.nysagd.org l Fall 2024 l GP 27