Material and Method: Down Syndrome patients underwent dental treatment under general anesthesia were evaluated retrospectively. General anesthesia method, demographic variables, ASA scores, Oral Hygiene Index, Frankl scale, Houpt Scale, systemic diseases of the patients, antibiotic prophylaxis and dental treatments were examined as data.

Results: Female/male ratio was 62/51, mean age was 14.14±10.28. Most patients were ASA II with 67.3%. The oral hygiene index was poor (%38). While 84.81% of the patients were sedated, 15.2% were given general anesthesia. Tooth extraction with tooth filling was the most performed dental treatment. The most common systemic disease was of cardiac origin. Antibiotic prophylaxis was administered to 82 of the patients.

Conclusion: To treat patients with Down Syndrome, frequent use of general anesthesia and sedation, as well as frequent antibiotic prophylaxis, is required. In terms of these applications, it is necessary to create a treatment plan with the least risk to the patient.

Down syndrome (DS) is one of them, first described by Langdon Down in 1866, and has an incidence of 1.5 per 1000 live births ². It is the most common congenital mental anomaly, including several mental and behavioral alterations and physical malformations ^3,4. Moreover oral health of children with Down's syndrome; can be adversely affected due to medications, treatments, or difficulties in daily basic tooth cleaning ⁵. It requires particular relevance and professional care due to mental disability and health problems ⁶.

Current dental problems can be treated with general anesthesia due to the lack of cooperation of patients, excessive dental procedures, and accompanying systemic diseases ^1,7. Depending on the patient's condition, procedures ranging from minimal sedation to deep general anesthesia are performed ^8,9.

Children with Down syndrome are born with certain anatomical characteristics specific to the condition, including atypical fingerprints, small teeth, stunted growth, droopy ears, hypotonia, and a flattened nose ^8,10. As these patients age, they often experience increased obesity, which can pose challenges for vascular access ¹¹. Moreover, these individuals commonly exhibit enlarged tonsils and adenoids, a narrowed subglottic area, a prolapsed epiglottis, and an enlarged tongue ^10,12. Specifically, their tonsils and adenoids are larger, the subglottic area is smaller, the tongue is enlarged and the epiglottis may droop (10, 12). App-roximately 10% to 40% of individuals with Down syndrome also have atlantoaxial instability ¹³. There-fore during treatment it is crucial to minimize head extension to prevent subluxation. Due to these unique characteristics, advanced airway devices should be readily available for intubation, as airway management can be complex ¹⁴. It is not recommended to administer sedation to these patients in office settings and more specialized operating rooms are required ¹⁵.

Individuals with Down syndrome are at a higher risk of developing various systemic conditions, including cardiovascular diseases, leukemia, infections, osteoporosis, and endocrine, neurological, orthopedic, dental, auditory, and ophthalmic disorders. Approximately 40-50% of them exhibit ventricular septal defects, a type of congenital heart defect, which may necessitate antibiotic prophylaxis for invasive dental treatments ¹⁶. Differences in their immune systems, zinc deficiency and anatomical variations make these individuals more susceptible to infections ¹⁷. Consequently, compromised dental health can pose a systemic disease risk, especially in those with concurrent medical issues. Therefore prophylactic regimens should be implemented to mitigate potential risks for the patients ^17,18.

First, when treating children with DS, the need for subacute bacterial endocarditis prophylaxis should be determined. The clinical importance of bacteremia in healthy patients is low. However, in a patient prone to local bacterial colonization following a bacterial infection, microorganisms may colonize through circulation and become a life-threatening condition such as infective endocarditis ¹⁷. İE prophylaxis should be given before dental treatment of such diseases to obtain an appropriate serum antibiotic concentration.

In this retrospective study, considering all the above-mentioned risky situations, we aimed to determine the limits of when and how antibiotic prophylaxis and general anesthesia should be used in patients undergoing dental treatment with Down syndrome.

Inclusion criteria: patients who underwent dental treatment with general anesthesia and only those with Down's syndrome. Dental treatment cannot be performed with local anesthesia due to exclusion criteria (patients undergoing local anesthesia and patients wit-hout information in their medical records).

2. Evaluation of general health
Due to the increase in life expectancy in patients with Down Syndrome, dental treatments are also a serious public health problem that is frequently on the agenda. Social awareness is essential to solve the problem wit-hout further difficulty. Dental treatment of DS patients is started before, especially by the pedodontics clinic, by screening the schools for disabled patients. These are the ones detected due to these scans in some patients who are operated under general anesthesia.

3. Evaluation of oral health
Routine examinations of the patients were carried out, whether they applied to the clinic themselves or were determined by the health screenings. Oral and dental examinations and radiological imaging were performed. Especially incompatible patients who could not be thoroughly examined and whose imaging could not be performed to the desired extent were sent to the anesthesia evaluation polyclinic. Patients planned to be operated on under general anesthesia were evaluated according to Frankle (F) and Houpt (H) scales. Patients with F1-2 and H1-2 were included in the appointment list for general anesthesia or sedation ^19,20.

4. Dental treatments
Although periodontological disease is a common problem in DS patients, our patients received general anesthesia or sedation to treat the following diseases.

Preventive treatment includes systematic scaling/cleaning, fluoride application, and placement of sealants where appropriate. Restorative treatment: It is the treatment that includes the restoration of material losses in the hard tissues of primary teeth and permanent teeth.

Surgical procedures: The surgical treatment comprises various procedures such as extractions of both simple and impacted teeth, gingivectomies, frenectomies, and other minor oral surgeries. Additionally, it involves surgical interventions for dental implants, jaw fracture repair, removal of cysts in the jawbones, maxillary expansion through surgical implantation, as well as plaque placement and removal operations.

Examination under anesthesia (without intervention)

5. Preoperative evaluation
The patients were evaluated preoperatively, and a physical examination was performed. Blood tests and consultation were required, if necessary. Comorbidities and drugs used were recorded, and necessary precautions were taken. All patients were fasting for at least 6 hours for sedation and general anesthesia. Information was given about the anesthesia procedure, and written informed consent was obtained from the legal guardians of the patients after the necessary explanations about possible risks.

6. Antibiotic proflaxia
Prophylaxis was given to those with cardiac disease and immune problems. Usually, penicillin was used. Cephalosporins were used for allergy sufferers.

7.Intra-operative period
During the dental procedure, peripheral oxygen saturation, heart rate, blood pressure, and endtidal carbon dioxide levels were continuously monitored in accordance with the guidelines set by ASA. Based on the patients' medical condition and their ability to cooperate, various anesthetic agents and techniques were employed to ensure optimal safety and comfort.

8. Postoperative period
At the end of the procedure, the patients were monitored for about 20 minutes in the recovery unit. The patients were taken to the inpatient unit without any decrease in vital functions. Oral nutrition started after 1 hour in patients undergoing sedation with an inhalation agent, after 2 hours for those undergoing sedation with an intravenous agent, and after 4 hours, respectively, for general anesthesia. Patients who tolerated oral intake were discharged the same day. The patients who could not take oral treatments because of nausea and vomiting or those who needed time to recover were followed up and treated in the inpatient service for 24 hours

9. Statistical analysis
The data obtained from the study was recorded in Microsoft Excel 2010. Subsequently, the data were subjected to analysis using the SPSS software package (version 17.0, SPSS Inc., Chicago, Illinois, USA). Descriptive statistics, including frequencies, percentages, and mean±standard deviations, were calculated. The study variables were subjected to a descriptive analysis, wherein arithmetic means, frequencies, and percentages were computed and presented in the form of tables.

Click Here to Zoom

Table 1: General Characteristics of Patients with Down syndrome.

The most common systemic disease was of cardiac origin. The most common of these was the operated VSD (Table 2).

Click Here to Zoom

Table 2: Concomitant diseases.

According to the Frankl scale, 21% of the patients were F1(n= 24), 79% were F2 (n= 89), according to the Houpt Scale, 33% were H1 (n= 37), and 67% were H2 (n= 76). In the absence of ASA1 patients, ASA2 was 67.3% (n= 76), and ASA3 was 32.7% (n= 37) (Table 3).

Click Here to Zoom

Table 3: Frankl scale, Houpt Scale and ASA classification 20,21.

When the patients were evaluated in terms of general anesthesia indications, 33% were Group A, 17% were Group B, and 50% were Group C (Table 4).

Click Here to Zoom

Table 4: Indications for the use of general anaesthesia for dental treatment.

DS patients often suffer from periodontal disease due to poor oral hygiene, a ketogenic diet, and inadequate oral care. They need restorative treatment more often than their peers. Some patients need orthodontic treatment due to tooth deficiencies and anomalies, and they may be indicated for prosthesis applications at an earlier age. It has become commonplace under today's conditions to talk about dental implant treatments in these patient groups. All these treatments are performed in our clinic under general anesthesia or sedation ²³. Consistent with the literature, most patients (80%) underwent conservative treatments, including restorative and tooth extractions. While patients who apply to the oral diagnosis department of the faculty of dentistry are directed to the relevant departments according to the treatment requirement, these patients, most of whom are in the pediatric age group, are treated in the general operating room, especially because of difficulty in cooperation ²².

Preoperative evaluation in patients to be treated under general anesthesia or sedation; The duration of the procedure, the intensity of systemic problems, and difficult airway control are considered. While general anesthesia is preferred for long-lasting procedures, the patient may decide to divide the procedures and perform them under sedation instead of general anesthesia, which may pose a high risk due to serious systemic problems. Or in a patient with a difficult airway, even if the procedure is short, if it is a problem to protect the airway safely during sedation, intubation can be preferred, and the patient can be directed to general anesthesia ⁸. Dental treatments can be postponed in patients with ASA III and above because they are not so important that they do not require taking a life risk. Our study shows that the sedation/general anesthesia rate is approximately 85/15%. For the reasons mentioned above, sedation preference is more important. The available data are compatible with the literature.

In particular, general anesthesia offers a relatively safe option for managing patients with behavioral and adjustment problems ²⁴. Although it suggests that these patients are at higher risk of perioperative complications due to the presence of medical comorbidities, the available literature reports that these occur at a similar rate or less frequently than in the general population ⁹. In addition, even minor morbidities may occur more prominently in patients with special needs ¹⁰. Therefore, comprehensive preoperative evaluations are crucial to reduce the risk of surgery-related complications, especially in this cohort.

Around 50% of infants with Down syndrome are born with some form of heart abnormality. Among adults with Down syndrome, approximately 50% exhibit Mitral Valve Prolapse (MVP) and may require prophylactic antibiotics prior to undergoing dental procedures ¹⁷. According to our study, heart disease is the most common systemic disease accompanying DS. Among these, VSD is the most common. IE prophylaxis is recommended, especially in problems involving the heart valves. In surgical interventions, antibiotic prophylaxis is performed to provide sufficient antibiotic levels in the tissue during and immediately after surgery, reduce bacterial flora, and reduce the possible contamination to the level the host can resist. The high need for antibiotic prophylaxis is closely related to cardiac diseases ²⁵. These indications can be listed as follows: History of previous infective endocarditis, Prosthetic Heart Valves, hypertrophic cardiomyopathy, Acquired heart valve diseases, Complex congenital cyanotic heart diseases Surgically corrected systemic or pulmonary shunts, Non-cyanotic congenital heart diseases (except secundum type ASD), Mitral valve prolapse with valvular insufficiency or severe valve thickening ²⁶.

Invasive procedures that may cause bleeding should be considered when performing prophylaxis. While there is a prophylaxis indication for tooth extraction, prophylaxis is not applied for a simple procedure such as fissure sealant in the same patient. Antibiotics used in prophylaxis and their doses are given in table 5.

Click Here to Zoom

Table 5: Recommended prophylaxis in dental procedures 18.

The greatest risk that may arise due to antibiotics is anaphylaxis, which resolves with medical treatment. However, if IE occurs, it is necessary to struggle with a seriously complicated situation, which may have mortal consequences ¹⁸. Antibiotic prophylaxis was required in 73% of the patients treated in our clinic. We did not see any side effects from antibiotics in these patients. Penicillin was used frequently. Cephalosporin was preferred in patients with a history of allergy. We have also experienced that patients with DS need prophylaxis more frequently for dental procedures than healthy patients of the same age group.

The sensitive issue here is to take adequate precautions to solve the problem smoothly or with fewer problems. While the intensity of medical problems in DS patients who will receive dental treatment poses a risk regarding general anesthesia, antibiotic prophylaxis increases some risks due to cardiac disease and immune problems. For this reason, dentists should focus on solving the problem with a holistic approach. Dentists should address these patients as both a public health problem and a personal problem requiring individual treatment outside their routine practice ²⁷. When the subject is handled from this point of view, the issues we draw attention to as two main topics in our study come to the fore.

As emphasized in the literature in our study, taking adequate precautions and working with the right team and equipment provided good results contrary to fears. While the anesthetic literature does provide assessments regarding patients with special needs, clinicians must acknowledge the challenges associated with evaluating such individuals ²⁰. Furthermore, the presence of multiple disorders, medical conditions, and anatomical and physiological changes can considerably complicate this process, particularly when comprehensive information is not readily accessible.

1) Carvalho B. Down’s syndrome. In: Oxford Hand-book of Anaesthesia. Ed: Allman KG, Wilson IH Oxford University Press 2003; 212-4.

2) Roizen MF. Anesthetic Implications of Concurrent Diseases. In: Anesthesia Ed: Miller RD. Churchill Livingstone 2000; 5th edition: 974.

3) Jeng W, Wang T, Cher T et al. Strategies for oral health care for people with disabilities in Taiwan. J Dent Sci 2009; 4: 165-72.

4) Rahman, M M and Fatema K. Seizures in Down Syndrome: An Update. Mymensingh Med J 2019; 28: 712-5.

5) Marta SN. Program of dental assistance to special patients: a 13-year experience. Rev Gaúcha Odontol 2011; 59: 379-85.

6) Frankl SN, Shiere FR, Fogels HR. Should the parent remain with the child in the dental operatory. J Dent Child 1962; 29: 150-3.

7) de Nova-García MJ, Gallardo-López NE, Martín-Sanjuán C et al. for selecting children with special needs for dental treatment under general anaesthesia. Med Oral Patol OralCir Bucal 2007; 12: 496- 503.

8) Glassman P. A review of guidelines for sedation, anesthesia, and alternative interventions for people with special needs. Spec Care Dentist 2009; 29: 9-16.

9) Wang YC, Lin IH, Huang CH, Fan SZ. Dental anesthesia for patients with special needs. Acta Anaesthesiol Taiwan 2012; 50: 122-5.

10) Kobel M, Creighton RE, Steward DJ. Anaesthetic considerations in Down's syndrome: experience with 100 patients and a review of the literature. Can Anaesth Soc J 1982; 29: 593-9.

11) Sulemanji DS, Donmez A, Akpek EA et al. Vascular catheterization is difficult in infants with Down syndrome. Acta Anaesthesiol Scand 2009; 53: 98e100.

12) Sedaghat AR, Flax-Goldenberg RB, Gayler BW et al. A case control comparison of lingual tonsillar size in children with and without Down syndrome. Laryngoscope 2012; 112: 1165e9.

13) Cremers MJ, Bol E, de Roos F, van Gijn J. Risk of sports activities in children with Down’s syndrome and atlantoaxial instability. Lancet 1993; 342: 511e4.

14) Antonarakis SE, Skotko BG, Rafii MS et al. Down syndrome. Nat Rev Dis Primers 2020; 6: 9.

15) Hennequin M, Faulks D, Veyrune J, et al. Significance of oral health in persons with Down synd-rome: a literature review. Dev Med Child Neurol 1999; 41: 275-83.

16) American Academy of Pediatric Dentistry. Guideline on management of dental patients with special health care needs. Pediatr Dent 2012; 34: 160e5.

17) Ram G, Chinen J. Infections and immunodeficiency in Down syndrome. Clin Exp Immunol 2011; 164: 9-16.

18) Goldie MP. New evidence on bacteraemia. Int J Dent Hyg 2010; 8: 317-8.

19) Jeng W, Wang T, Cher T et al. Strategies for oral health care for people with disabilities in Taiwan. J Dent Sci 2009; 4: 165-72.

20) Polli VA, Sordi MB, Lisboa ML et al. Dental management of special needs patients: a literature review. Glob J Oral Sci 2016; 2: 33-45.

21) de Nova García MJ, Gallardo López NE, Martín Sanjuán C et al. Criteria for selecting children with special needs for dental treatment under general anaesthesia. Med Oral Patol Oral Cir Bucal 2007; 12: E496-03.

22) Akpınar H. Genel anestezi altında dental tedavi yapılan olguların değerlendirilmesi. Çukurova Tıp Dergisi 2019; 44: 341-6.