Page 28 - GP Fall 2024

Cardiovascular: Approximately 70% of with caries were noted. The patient was re- nent molars have been lost. Sealants were
individuals with KS have a congenital ferred to the endodontic department to exca- replaced since oral hygiene did not improve.
heart defect. Many heart defects have vate #9, but no pulpal exposure was noted. Caries can develop in the irregularly shaped,
10
been described in association with KS, but Tooth #9 was treated with calcium hydrox- deep grooves of the molars and premolars.
left-sided obstructive lesions, especially ide and flowable composite. Tooth #8 was The patient stated that he would like to, “Fix
coarctation of the aorta, are the most com- subsequently treated in the pediatric clinic my front teeth”. Teeth #8 and #9 will be
evaluated for permanent restorations. How-
mon. Other defects may include (alone or with SDF, Fuji IX, and a sealant to mask the ever, KS is being referred back to the or-
10
in combination): septal defects, bicuspid discoloration caused by the SDF (Figure 5). thodontic and oral surgery departments for
aortic valve, mitral valve anomalies, trun- potential exposure of the permanent second
cal heart defects, and hypoplastic left heart molars to bring them into proper occlusion.
syndrome. Hypertrophic cardiomyopathy
and aortic root dilatation are common. The Conclusions: Kabuki syndrome is a rare
patient is followed by a pediatric cardiol- condition. First identified in Japan, many
ogist for trivial aortic valve regurgitation. other ethnic groups have manifested with
Screening by a pediatric cardiologist and similar reports of the craniofacial, skeletal
echocardiograms should be performed on and mental condition. Therefore, we have
presented this case study to list some evi-
these patients at the time of diagnosis. It dence-based management of Kabuki syn-
should be emphasized that the existence of drome a dental healthcare professional may
congenital cardiac abnormalities in KMS encounter. The dental management is a mul-
patients make them high risk for bacterial tidiscipline approach involving the follow-
endocarditis and, therefore, administration ing disciplines: orthodontic, pediatric, and
of an antibiotic prophylaxis before invasive general dentistry.
dental procedures is a highly advisable ap-
proach. 5 References
1. Kuroki, Y., Suzuki, Y., Chyo, H., Hata,
Anesthesia Medications: The patient was A., & Matsui, I. (1981). A new malforma-
found to be allergic to many anesthesia tion syndrome of long palpebral fissures,
medications including propofol and sevo- large ears, depressed nasal tip, and skeletal
anomalies associated with postnatal dwarf-
flurane. There are several reports of compli- ism and mental retardation. The Journal
cations of Kabuki Syndrome patients during Figure 5. Teeth #8 and #9 show deep invag- of Pediatrics, 99(4), 570–573. https://doi.
inations of lingual groove. #8 was treated
surgical procedures. The anesthesiologist with SDF/Fuji IX/sealant to mask the dis- org/10.1016/s0022-3476(81)80256-9.
11
needs to be aware of structural malforma- coloration. 2. Niikawa, N., Matsuura, N., Fukushima,
tions, congenital heart defects, narrowing Y., Ohsawa, T., & Kajii, T. (1981). Kabuki
of airways, and anatomical variations of the Sealants were also applied to teeth make-up syndrome: A syndrome of men-
bronchial tree affecting intubation and skel- #3,7,10,14,19,30. Orthodontic referral was tal retardation, unusual facies, large and
etal anomalies. The patient had a diagnosis made due to Class II malocclusion and high protruding ears, and postnatal growth de-
of asthma nine years ago, which is also an arch palate which are hallmarks of Kabuki ficiency. The Journal of Pediatrics, 99(4),
anesthetic risk. However, the patient has not syndrome oral manifestations. At various 565–569. https://doi.org/10.1016/s0022-
needed a rescue inhaler for nine years. re-care visits, it was noted that the child 3476(81)80255-7.
had poor oral hygiene and a high plaque in- 3. do Prado Sobral S, Leite AF, Figueire-
Developmental and Social Delay: The dex. The visits for recall were every three do PT, Ferrari I, Safatle HP, Córdoba MS,
patient has a developmental delay and he months as a result of these findings. In 2023, Versiani BR, Acevedo AC, Mestrinho HD.
Craniofacial and dental features in kabuki
primarily engages with friends via video the patient was transferred to the Compre- syndrome patients. Cleft Palate Craniofac J.
games. Improved communication with the hensive Care and General Dentistry Clinic. 2013 Jul;50(4):440-7. doi: 10.1597/11-052.
patient and guardian regarding oral hygiene Earlier in 2023, the potential impaction of Epub 2011 Oct 24. PMID: 22023252.
and home care is stressed. Early establish- all permanent second molars was noted. The 4. Rocha, C.T., Peixoto, I.T.A., Fer-
ment of a dental home is imperative to fur- teeth appear to demonstrate hypocalcifica- nandes, P.M., Torres, C.P. and De Queiroz,
tion, possibly from the skeletal dysplasia
ther engagement with the patient as he ap- associated with Kabuki syndrome. Teeth #8 A.M. (2008), Dental findings in Kabuki
proaches adulthood. Positive reinforcement and #9 remained asymptomatic and were make-up syndrome: a case report. Spe-
is necessary during dental visits including continually observed for any changes. The cial Care in Dentistry, 28: 53-57. https://
repetition of oral hygiene instructions. patient returned in May and August of 2023 doi-org.ezproxy.med.nyu.edu/10.1111
Three-month recall appointments are sched- for three-month recalls including fluoride /j.1754-4505.2008.00011.
5. Barbosa-Limar R., Lopes A., de Moura J.,
uled for the patient, which will evaluate his application and he had copious amounts of S.N.T. Cardoso M., 2020: Dental Findings
understanding of homecare. plaque, with bleeding and inflamed gingiva. in Kabuki Syndrome: A Systematic Review
His parents have admitted that it is difficult for Dentistry-ODVOS-Int. J. Dental Sc.,
Dental Management: Kabuki syndrome to instruct the patient in proper oral hygiene. 22-2 (May-August): 61-69.
patients present with abnormal dentition Oral hygiene instructions are reinforced to 6. Adam, M., & Hudgins, L. (2004). Kabuki
in 83% of all patients. When our patient the guardians and the patient at every visit. syndrome: A Review. Clinical Genetics,
14
first presented to the pediatric dental clinic The most recent three-month recall in May 67(3), 209–219. https://doi.org/10.1111/
in 2015, malformations of teeth #8 and #9 2024 revealed moderate to severe gingival j.1399-0004.2004.00348.x
with deep invaginations of lingual grooves inflammation. The sealants on the perma-
www.nysagd.org l Fall 2024 l GP 28