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48 rev port estomatol med dent cir maxilofac. 2021;62(1):42-49

ly monitored and advised about the need for thorough oral reported the improvement in the patient’s quality of life after
hygiene. 21 dental treatment.
The presence of caries and gingivitis are common findings, There is no report of dental care in a patient with this syn-
and their severity can be attributed to the patient’s cognitive drome in the literature, making it difficult to compare the ob-
22
impairment. Motor and behavioral difficulties make dental stacles encountered and the protocols adopted in the present
treatment difficult. In this case, non-pharmacological behav- case report. CSS has a variable expression in different individ-
ioral techniques were used, such as tell-show-do, positive re- uals. For the patient described in our report, dental treatment
inforcement, and protective stabilization. Parents and caregiv- was possible using non-pharmacological behavior manage-
ers of children with mental disabilities can help manage these ment techniques. In individuals who are less cooperative or
patients in the dental office. Protective stabilization to ensure need more extensive treatment, pharmacologic techniques,
safety during procedures can be useful for patients when be- such as conscious sedation or general anesthesia, might be
havioral management techniques fail. 17 necessary.
The fluoride varnish use in a patient with hypomineraliza- The dental surgeon’s knowledge of CSS’s clinical charac-
tion is essential to increase calcium and fluoride ion release teristics will lead to the early diagnosis of oral problems and
16
and control dental caries development. Moreover, the use of improve the quality of life of the syndromic patient. The pa-
fluoride varnish is important to prevent enamel breakdown tient is being monitored, and her oral conditions have im-
and control the hypersensitivity associated with the hypomin- proved.
eralization. 23
We opted for the extraction of the mandibular first perma-
nent molar because the child’s behavior would not have al- Ethical disclosures
lowed endodontic treatment followed by placement of a stain-
less-steel or similar crown, which could be replaced by a Protection of human and animal subjects. The authors
24
porcelain crown at an older age. Root canal therapy requires declare that no experiments were performed on humans or
rubber dam isolation, extended working time, radiographic animals for this study.

acquisition, and substantial cooperation, all of which might
prove challenging to non-collaborative individuals with special Confidentiality of data. The authors declare that no patient
data appear in this article.
25
needs. Operative dental treatments, especially complex res-
torations and root canal therapy, are time-consuming and re- Right to privacy and informed consent. The authors have
quire patient compliance; therefore, dentists must tend to obtained the written informed consent of the patients or sub-
provide tooth extraction if patients with specific disabilities jects mentioned in the article. The corresponding author is in
do not have cooperative behavior due to their developmental possession of this document.
delay. 26
GICs combine fluoro-alumina silicate glass and polyalke-
noic acids. Resin-modified glass ionomers share this same Conflict of interest
chemistry but with additional resin, which reinforces the ma-
terial and adds an aesthetic dimension. Used widely world- The authors have no conflicts of interest to declare.
wide as definitive restorative materials for deciduous teeth,
the resin-modified GICs present mechanical properties com-
patible with composite resins, being as efficient as the conven- references
27
tional technique. Besides offering favorable work time, easy
application, and release fluoride, another advantage of GICs is 1. Ordóñez MPT, Oyervide JAJ. Síndrome de Coffin-Siris. Rev Fac
Cienc Méd Univ Cuenca. 2016;34:69-75.
that their longevity is not influenced by the use of a dental 2. Mannino EA, Miyawaki H, Santen G, Vergano SAS. First
dam, making its use even easier, both in children and spe- data from a parent-reported registry of 81 individuals
cial-needs patients. Providing increased aesthetics and with Coffin-Siris syndrome: Natural history and
strength, the indications for resin-modified glass ionomers are management recommendations. Am J Med Genet A.
non-stress bearing class I (as in this clinical report) or II resto- 2018;176:2250-8.
rations for deciduous teeth. The encapsulated GICs present 3. Curcio MR, Ferranti S, Lotti F, Grosso S. Coffin-Siris syndrome
superior resistance to compression compared to the powder/ and epilepsy. Neurol Sci. 2020.
liquid system because the proportions are pre-balanced in the 4. Nemani L, Barik R, Patnaik AN, Mishra RC, Rao AM, Kapur P.
Coffin-Siris syndrome with the rarest constellation of
factory. 28 congenital cardiac defects: A case report with review of
The use of restorations with fluoride-releasing materials, literature. Ann Pediatr Cardiol. 2014;7:221-6.
such as GICs, can be a useful therapeutic and preventive ap- 5. Vergano SS, Deardorff MA. Clinical features, diagnostic
17
proach for patients with special needs, in which we have criteria, and management of Coffin-Siris syndrome. Am J
difficulty maintaining a dry field or must work very quickly. Med Genet C Semin Med Genet. 2014;166C:252-6.
These patients require home care and periodic dental consul- 6. Schrier SA, Bodurtha JN, Burton B, Chudley AE, Chiong MA,
tations for preventive and systematic dental treatment. Pa- D’Avanzo M G, et al. The Coffin-Siris syndrome: a proposed
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13
two to three months, or more often if indicated. In this case 7. Sonmez FM, Uctepe E, Gunduz M, Gormez Z, Erpolat S, Oznur
report, the patients’ father, responsible for her, very clearly M, et al. Coffin-Siris syndrome with café-au-lait spots,