Australian Dental Journal
The official journal of the Australian Dental Association
Australian Dental Journal 2015; 60: 255–259 doi: 10.1111/adj.12319
Atraumatic restorative technique: case report on dental management of a patient with Moebius syndrome A Pradhan,*† M Gryst† *Australian Research Centre for Population Oral Health, The University of Adelaide, South Australia. †Special Needs Unit, Adelaide Dental Hospital, Adelaide, South Australia.
ABSTRACT Background: Clinical challenges associated with Moebius syndrome, a rare congenital neuromuscular disorder, include orofacial anomalies like microstomia, limited mouth opening and severe gag reflex. Methods: This case report presents the dental management of an anxious female with Moebius syndrome. For restorations, atraumatic restorative technique (ART), using glass ionomer cement was used with hand instruments. Results: All necessary dental treatment was completed in the dental chair, thus avoiding the need for a general anaesthetic, and associated complications with airway management due to orofacial abnormalities. Conclusions: ART is an appropriate technique for people with microstomia and/or limited mouth opening, such as those with Moebius syndrome. The application of ART can be extended to other special needs patients (people with physical and intellectual disabilities, extreme anxiety, needle phobia and the frail elderly in aged care facilities), where access or cooperation is difficult or limited. Keywords: Atraumatic restorative technique, Moebius syndrome, orofacial anomalies, special needs. Abbreviations and acronyms: ART = atraumatic restorative technique; GIC = glass ionomer cement; MbS = Moebius syndrome; OPG = orthopantomograph; SNU = Special Needs Unit. (Accepted for publication 27 July 2014.)
INTRODUCTION Moebius syndrome (MbS) is an extremely rare congenital neuromuscular disorder, characterized by bilateral or unilateral, complete or partial paralysis of the VIth (abducens) and VIIth (facial) cranial nerves.1,2 with the VIth cranial nerve affected, there is eye sensitivity, strabismus, motor delays and reduction of lateral gaze. With the VIIth cranial nerve affected, bilateral facial palsy may occur with mask-like expressionless face with inability to smile, often with the upper lip retracted due to muscle shrinkage. Occasionally, the Vth (trigeminal), Xth (vagus), XIth (accessory) and XIIth (hypoglossal) cranial nerves are involved, resulting in difficulty in chewing, swallowing and coughing, which often leads to respiratory complications.1 Occasionally, if the VIIIth (auditory) cranial nerve is affected, there may be hearing problems, impaired listening ability and speech difficulties.3 Limbs may also be affected resulting in clubfoot and missing or webbed fingers.4,5 MbS has also been linked to increased occurrence of the symptoms of autism.6,7 While some are mistaken to have intellectual disability, due to their expressionless faces, © 2015 Australian Dental Association
strabismus and frequent drooling, mild to moderate intellectual disability has only been reported to occur in 10–15% of cases based either on conclusions of history taking and observations.5,8 However, a more recent Dutch study on adults with MbS, using the Groninger Intelligence Test, reports that there were no differences in intelligent quotient or attention and memory capacity between the normal population and individuals with the syndrome.9 Verzijl et al.10 estimate that on average there are 2 to 20 cases of MbS per million births. It affects both males and females equally.11 Although there are multiple theories regarding the aetiology and pathogenesis of MbS, the aetiology is not yet fully understood. Some believe that the primary pathology is manifested in the brain stem nuclei,10 while others relate to genetic factors like reciprocal translocation between chromosomes 1 and 13.12 Others believe it to be related to environmental factors like maternal trauma resulting in ischaemia or hypoxia to the developing foetus, or use of the drugs like thalidomide during pregnancy4 or cocaine13 or misoprostol.14 MbS is also thought to result from a vascular disruption in the brain during prenatal development.3 In a systematic review, Dal Pizzol 255
A Pradhan and M Gryst et al.15 concluded that the odds of intrauterine exposure to misoprostol were 25 times greater in cases of MbS than in cases without anomalies. MbS also presents with multiple oral complications,16 including microstomia, microglossia, high arched palate or cleft palate, cleft lip, bifid uvula and dental malocclusion.17,18 Hypodontia (both deciduous and permanent),2 and hypoplastic teeth19 have also been reported. Lack of lower lip support and excessive drooling may cause angular cheilitis. Lack of a good oral seal from open bite can result in dry mouth and lips. There may be paralysis and hypoplasia of the tongue with limited movement.1 Management for people with MbS includes supportive treatment in accordance with symptoms. The most life-threatening problems of patients with MbS are dysphagia and aspiration, and a special diet is often suggested to avoid aspiration.20 Infants may require feeding tubes or special bottles to maintain sufficient nutrition. Surgery may be necessary to correct strabismus, protect the cornea (via frequent lubrication or tarsorraphy), and improve limb and jaw deformities. For jaw deformities, while interceptive orthodontics has an important role to expand the upper arch and correct malocclusion during mixed dentition, orthognathic surgery could be considered in adults. Grascillis muscle surgery (‘smile surgery’) pioneered by Dr RM Zuker in 1997 can be performed.21 Physical and speech therapy is necessary to improve motor skills and coordination, and for better control of speaking and eating abilities. The orofacial anomalies frequently manifested in MbS have a negative impact on the quality of life leading to emotional or social adjustment issues and affected individuals may suffer from depression, guilt, anger and denial.22 High caries rates and prevalence of Bell’s palsy among patients with MbS have been reported.20 Considering the oral manifestations of the syndrome, it is evident that dentistry plays a fundamental role in caring for these individuals. Microstomia makes dental treatment extremely difficult as it restricts the use of standard handpieces and instruments. The limited opening also does not allow adequate light in the working field of the oral cavity. Limitations in opening the mouth due to the size of the oral cavity and the lack of muscle elasticity represent the greatest difficulties regarding dental treatment, as reported in earlier studies.18,23 This case report presents the dental management of an anxious female with MbS, using the atraumatic restorative technique (ART), placing glass ionomer cement (GIC) with hand instruments.
and Children’s Hospital for continuation of dental management. At eight years of age, she had dental treatment under general anaesthesia. An increase in palatal smooth surface caries was noted between 1996 and 1998, ‘from her habit of holding coke in the mouth before swallowing’. Fluoride trays were made but not used due to gagging problems. Two per cent neutral sodium fluoride gel was preferred over mouthwash as she could not swish due to facial paralysis. Between 2000 and 2001, she had dental restorations, dietary advice and 2% neutral sodium fluoride was continued, after which she did not seek any dental treatment for six years. In 2007, she was referred back to the SNU from a community dental clinic, with ‘a severely broken down dentition’. Presenting problem The patient lives independently, but is on a disability support pension. She was seeking psychiatric counselling but was not on any medication. She has a blank expression, which is characteristic of a patient with MbS. As indicated in the referral, she presented with multiple carious teeth: Quadrant 1: 13 (incisal), 12 (mesial), 11 (mesiopalatal). Quadrant 2: 21 (mesial), 22 (disto-palatal), 23 (disto-palatal), 24 (occluso-buccal), 25 (occlusal), 26 (occlusal), decayed roots of 27 and 28. Quadrant 3: 32 (lingual), 33 (lingual), 34 (occlusobuccal), 35 (occlusal), 36 (occlusal), 37 (occlusal). Quadrant 4: 46 (mesio-occlusal), hypoplastic 44 (disto-occlusal). An orthopantomograph (OPG) shows the multiple carious lesions and decayed roots (Fig. 1). The main challenge was to perform routine dental procedures like restorations and extractions but in a very small inelastic mouth with a maximum width of 38 mm and opening of 18 mm (Fig. 2 and Fig. 3), making all intraoral procedures challenging due to extremely difficult access and restricted visibility. In addition, the patient had poor masticatory and swallowing capabilities and severe gag reflex.
CASE DESCRIPTION AND RESULTS A 19-year-old female with MbS was referred to the Special Needs Unit (SNU) in 1999 from the Women’s 256
Fig. 1 OPG showing multiple carious lesions and decayed roots. © 2015 Australian Dental Association
Atraumatic restorative technique
Fig. 2 Maximum width of 38 mm.
for 15–20 seconds to improve the chemical and mechanical bonding of the GIC to dentine. The cavity is then washed and dried but not dehydrated. Isolation is achieved with cotton rolls instead of rubber dam. Due to the inadequate intraoral space in this case, a ball burnisher was used to condense the glass ionomer cement instead of the ART ‘press-finger technique’.25 Likewise, other clinical procedures can be varied according to the equipment available and patient compliance. For example, a local anaesthetic can be used, a rubber dam can be placed, and a rotary instrument can be used to gain minimal access to the body of the lesion. The washing and drying can be done using a triple-syringe or using a wet and dry cotton pellet, thereby avoiding the use of a triplex and suction in extremely anxious patients. Reinforcement of preventive measures included instructions on daily oral hygiene care, dietary counselling and continuation of 2% neutral sodium fluoride gel. Follow-up
Fig. 3 Maximum opening of 18 mm.
Treatment After obtaining consent, treatment included extractions of the upper left second molar (27) and upper left third molar (28) for relief of pain, which were simple and uneventful. The decayed teeth were then restored over several visits. Restorations were completed using ART as there was inadequate intraoral space for the conventional use of standard size handpieces. ART refers to a minimal intervention technique for caries, originally developed in response to a need to provide effective preventive and restorative treatment in underserved communities where running water and electricity might not always be available. It involves minimum cavity preparation using only hand instruments (small and large spoon excavators for caries removal) to remove infected dentine, followed by restoration of the cavity, with an adhesive filling material (GIC).24,25 Local anaesthetic is not necessary as only soft, completely demineralized carious tooth tissue is removed and remaining sound tissue is retained. Care must be taken in deep cavities to avoid pulp exposure. The resultant cavity is then washed. The resultant smear layer from the use of hand instruments on the dentine surface is removed by applying 10% polyacrylic acid dentine conditioner to the cavity © 2015 Australian Dental Association
As is routine at SNU, the patient was placed on a sixmonthly recall list. However, in spite of reminder calls prior to every appointment and a new appointment arranged following every appointment that was unattended, she failed recall visits in 2009. Reasons given over the phone included ‘unwell’ or ‘not ready for the dental visit’. In 2010, she returned with pain on 36 and 37, which had to be surgically extracted. Since then she has maintained good oral hygiene and dental health (Fig. 4). DISCUSSION In contrast to past literature about the association with intellectual disability or autism,6,7 this patient did not present with either of these problems, conforming with the more recent Dutch study.9 Orally, the patient presented with full dentition, including all
Fig. 4 OPG showing maintenance of dental health. 257
A Pradhan and M Gryst third molars, but did have microstomia and gag reflex, which made all intraoral procedures extremely difficult. Restrictions in tongue and masticatory muscle functions were most likely to have added difficulties in performing oral hygiene procedures. Therefore, past caries may be partly attributed to insufficient oral hygiene, made worse by dry mouth and inappropriate diet. In addition, the patient had poor masticatory and swallowing capabilities, both of which are associated with this syndrome. As a result of these problems, her need for soft diet and the prolonged intraoral food retention could have increased the risk of caries. While other case reports have presented cases about children and young adults between the ages of 2 years and 18 years, with treatment under conscious sedation26 or general anaesthetic20 the patient in this case was a young adult who was treated in the chair, using an alternate technique like ART that avoided the need for a general anaesthetic. Although local anaesthetic can be avoided for restorations, it is necessary for the extraction of teeth. However, if ART is used in the first instance for the restoration of teeth, good rapport and trust can be developed over time with the dentist. At the least, ART can save teeth, whilst patients are on the waiting list for dental extractions under general anaesthetic and minimize the time which would otherwise have to be spent for the general anaesthetic. For patients with MbS, there are several risks associated when treated under a general anaesthetic, mainly difficult or even failed intubation.27 In addition, there is a risk for tracheomalacia, palatal and uvular weakness leading to loss of airway and respiratory failure. The presence of associated hypotonia of the skeletal musculature, and gastro-oesophageal reflux may increase the risk of perioperative aspiration. Associated orofacial abnormalities may make routine airway management difficult. Therefore, if a general anaesthetic was necessary, the anaesthetist would most likely consult the patient’s ear, nose and throat surgeon about the possibility of the need for a tracheostomy to ensure airway maintenance during the procedure. Prevention of oral health problems is always best, but when intervention is necessary, ART is appropriate for treating special needs patients with limited oral opening. To date, ART has been promoted by WHO for application in developing countries where there is no access to electricity for the use of handpieces. This case report highlights the application of ART in the treatment of patients not only with limited oral opening, but suggests that the application can be extended to other special needs patients (people with physical and intellectual disabilities, extreme anxiety, needle phobia and the frail elderly in aged care facilities), where access or cooperation is diffi258
cult or limited. ART rarely requires a local anaesthetic, it is well accepted by patients. Moreover, as handpieces are not used with ART, the threatening drilling sound is avoided and the necessary highvolume suction is not needed. It can be used both in the traditional dental clinic setting and for outreach situations like aged care facilities and remote areas where dental facilities do not exist. Given these advantages, and the effectiveness and survival rates of ART restorations,28,29 ART should be accepted by the dental profession and included in the dental education system for the dental management of people with special needs. ACKNOWLEDGEMENTS The authors would like to thank the South Australian Dental Service (SADS) for their approval to publish this case report and for the use of their facilities. The patient has given informed consent for the publication of this case report. REFERENCES 1. Kumar D. Moebius syndrome. J Med Genet 1990;27:122–126. 2. Rizoz M, Negr on RJ, Serman N. M€ obius syndrome with dental involvement: a case report and literature review. Cleft Palate Craniofac J 1998;35:262–268. 3. Briegel W. Neuropsychiatric findings of M€ obius sequence: a review. Clin Genet 2006;70:91–97. 4. Rogers GL, Hatch GF Jr, Gray I. Mobius syndrome and limb abnormalities. J Pediatr Ophthalmol 1977;14:134–138. 5. Lin KJ, Wang WN. Moebius syndrome: report of case. J Dent Child 1997;64:64–67. 6. Bandim JM, Ventura LO, Miller MT, Almeida HC, Costa AES. Autism and M€ obius sequence. Arq Neuropsiquiatr 2003;61:181–185. 7. Gillberg C, Steffenburg S. Autistic behaviour in Moebius syndrome. Acta Paediatr Scand 1989;78:314–316. 8. Koroluk LD, Lanigan DT. Moebius and Poland syndromes: a report of a case. Can Dent Assoc J 1989;55:647–648. 9. Verzijl HTFM, van Es N, Berger HJC, et al. Cognitive evaluation in adult patients with M€ obius syndrome. J Neurol 2005;252:202–207. 10. Verzijl HTFM, van der Zwaag B, Cruysberg JRM, et al. M€ obius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology 2003;61:327–333. 11. Str€ omland K, Sj€ ogreen L, Miller M, et al. M€ obius sequence – a Swedish multidiscipline study. Eur J Paediatr Neurol 2002;6:35–45. 12. Ziter FA, Wiser WC, Robinson A. Three-generation pedigree of a Mobius syndrome variant with chromosome translocation. Arch Neurol 1977;34:437–442. 13. Kankirawatana P, Tennison MB, D’Cruz O, Greenwood RS. Mobius syndrome in infant exposed to cocaine in utero. Pediatr Neuro 1993;19:71–72. 14. Shepard TH. Mobius syndrome after misoprostol: a possible teratogenic mechanism. Lancet 1995;346:780. 15. Dal Pizzol TS, Knop FP, Mengue SS. Prenatal exposure to misoprostol and congenital anomalies: systematic review and meta-analysis. Reprod Toxicol 2006;22:666–671. © 2015 Australian Dental Association
Atraumatic restorative technique 16. Wedgwood DL. Mobius syndrome. J Can Dent Assoc 1978;9:417–420. 17. Sj€ ogreen L, Andersson-Norinder J, Jacobsson C. Development of speech, feeding, eating, and facial expression in M€ obius sequence. Int J Pediatr Otorhinolaryngol 2001;60:197–204. 18. De Serpa Pinto MVX, Magalh~aes MHCG, Nunes FD. Moebius syndrome with oral involvement. Int J Paediatr Dent 2002;12:446–449. 19. Legum C, Godel V, Nemet P. Heterogeneity and pleiotropism in the Moebius syndrome. Clin Genet 1981;20:254–259. 20. Ha CY, Messieha ZS. Management of a patient with Mobius syndrome: a case report. Spec Care Dentist 2003;23:111–116. 21. Zuker R, Goldberg C, Manktelow R. Facial animation in children with Mobius syndrome after segmental gracilis muscle transplant. Plast Reconstr Surg 2000;106:1–8. 22. Pedraza S, Gamez J, Rovira A, et al. MRI findings in Mobius syndrome: correlation with clinical features. Neurology 2000;55:1058–1060. 23. Domingos AC, Lopes SLCP, Almeida SM, Boscolo FN, Whaites EJ. Poland-Moebius syndrome: a case with oral anomalies. Oral Dis 2004;10:404–407. 24. Frencken JE, Pilot T, Songpaisan Y, Phantumvanit P. Atraumatic restorative treatment (ART): rationale, technique, and development. J Public Health Dent 1996;56:135–140.
© 2015 Australian Dental Association
25. Holmgren CJ, Roux D, Domejean S. Minimal intervention dentistry: Part 5. Atraumatic restorative treatment (ART) – a minimum intervention and minimally invasive approach for the management of dental caries. Br Dent J 2013;214: 11–18. 26. Shashikiran ND, Subba Reddy VV, Patil R. Moebius syndrome: a case report. J Indian Soc Prev Dent 2004;22:96–99. 27. Ferguson S. Moebius syndrome: a review of the anaesthetic implications. Paediatr Anaesth 1996;6:51–56. 28. Frencken JE, Van’t Hof MA, Van Amerongen WE, Holmgren CJ. Effectiveness of single-surface ART restorations in the permanent dentition: a meta-analysis. J Dent Res 2004;83:120– 123. 29. Mickenautsch S, Yengopal V, Banerjee A. Atraumatic restorative treatment versus amalgam restoration longevity: a systematic review. Clin Oral Investig 2010;14:233–240.
Address for correspondence: Dr Archana Pradhan Special Needs Unit Adelaide Dental Hospital Adelaide SA 5000 Email: [email protected]
259
The official journal of the Australian Dental Association
Australian Dental Journal 2015; 60: 255–259 doi: 10.1111/adj.12319
Atraumatic restorative technique: case report on dental management of a patient with Moebius syndrome A Pradhan,*† M Gryst† *Australian Research Centre for Population Oral Health, The University of Adelaide, South Australia. †Special Needs Unit, Adelaide Dental Hospital, Adelaide, South Australia.
ABSTRACT Background: Clinical challenges associated with Moebius syndrome, a rare congenital neuromuscular disorder, include orofacial anomalies like microstomia, limited mouth opening and severe gag reflex. Methods: This case report presents the dental management of an anxious female with Moebius syndrome. For restorations, atraumatic restorative technique (ART), using glass ionomer cement was used with hand instruments. Results: All necessary dental treatment was completed in the dental chair, thus avoiding the need for a general anaesthetic, and associated complications with airway management due to orofacial abnormalities. Conclusions: ART is an appropriate technique for people with microstomia and/or limited mouth opening, such as those with Moebius syndrome. The application of ART can be extended to other special needs patients (people with physical and intellectual disabilities, extreme anxiety, needle phobia and the frail elderly in aged care facilities), where access or cooperation is difficult or limited. Keywords: Atraumatic restorative technique, Moebius syndrome, orofacial anomalies, special needs. Abbreviations and acronyms: ART = atraumatic restorative technique; GIC = glass ionomer cement; MbS = Moebius syndrome; OPG = orthopantomograph; SNU = Special Needs Unit. (Accepted for publication 27 July 2014.)
INTRODUCTION Moebius syndrome (MbS) is an extremely rare congenital neuromuscular disorder, characterized by bilateral or unilateral, complete or partial paralysis of the VIth (abducens) and VIIth (facial) cranial nerves.1,2 with the VIth cranial nerve affected, there is eye sensitivity, strabismus, motor delays and reduction of lateral gaze. With the VIIth cranial nerve affected, bilateral facial palsy may occur with mask-like expressionless face with inability to smile, often with the upper lip retracted due to muscle shrinkage. Occasionally, the Vth (trigeminal), Xth (vagus), XIth (accessory) and XIIth (hypoglossal) cranial nerves are involved, resulting in difficulty in chewing, swallowing and coughing, which often leads to respiratory complications.1 Occasionally, if the VIIIth (auditory) cranial nerve is affected, there may be hearing problems, impaired listening ability and speech difficulties.3 Limbs may also be affected resulting in clubfoot and missing or webbed fingers.4,5 MbS has also been linked to increased occurrence of the symptoms of autism.6,7 While some are mistaken to have intellectual disability, due to their expressionless faces, © 2015 Australian Dental Association
strabismus and frequent drooling, mild to moderate intellectual disability has only been reported to occur in 10–15% of cases based either on conclusions of history taking and observations.5,8 However, a more recent Dutch study on adults with MbS, using the Groninger Intelligence Test, reports that there were no differences in intelligent quotient or attention and memory capacity between the normal population and individuals with the syndrome.9 Verzijl et al.10 estimate that on average there are 2 to 20 cases of MbS per million births. It affects both males and females equally.11 Although there are multiple theories regarding the aetiology and pathogenesis of MbS, the aetiology is not yet fully understood. Some believe that the primary pathology is manifested in the brain stem nuclei,10 while others relate to genetic factors like reciprocal translocation between chromosomes 1 and 13.12 Others believe it to be related to environmental factors like maternal trauma resulting in ischaemia or hypoxia to the developing foetus, or use of the drugs like thalidomide during pregnancy4 or cocaine13 or misoprostol.14 MbS is also thought to result from a vascular disruption in the brain during prenatal development.3 In a systematic review, Dal Pizzol 255
A Pradhan and M Gryst et al.15 concluded that the odds of intrauterine exposure to misoprostol were 25 times greater in cases of MbS than in cases without anomalies. MbS also presents with multiple oral complications,16 including microstomia, microglossia, high arched palate or cleft palate, cleft lip, bifid uvula and dental malocclusion.17,18 Hypodontia (both deciduous and permanent),2 and hypoplastic teeth19 have also been reported. Lack of lower lip support and excessive drooling may cause angular cheilitis. Lack of a good oral seal from open bite can result in dry mouth and lips. There may be paralysis and hypoplasia of the tongue with limited movement.1 Management for people with MbS includes supportive treatment in accordance with symptoms. The most life-threatening problems of patients with MbS are dysphagia and aspiration, and a special diet is often suggested to avoid aspiration.20 Infants may require feeding tubes or special bottles to maintain sufficient nutrition. Surgery may be necessary to correct strabismus, protect the cornea (via frequent lubrication or tarsorraphy), and improve limb and jaw deformities. For jaw deformities, while interceptive orthodontics has an important role to expand the upper arch and correct malocclusion during mixed dentition, orthognathic surgery could be considered in adults. Grascillis muscle surgery (‘smile surgery’) pioneered by Dr RM Zuker in 1997 can be performed.21 Physical and speech therapy is necessary to improve motor skills and coordination, and for better control of speaking and eating abilities. The orofacial anomalies frequently manifested in MbS have a negative impact on the quality of life leading to emotional or social adjustment issues and affected individuals may suffer from depression, guilt, anger and denial.22 High caries rates and prevalence of Bell’s palsy among patients with MbS have been reported.20 Considering the oral manifestations of the syndrome, it is evident that dentistry plays a fundamental role in caring for these individuals. Microstomia makes dental treatment extremely difficult as it restricts the use of standard handpieces and instruments. The limited opening also does not allow adequate light in the working field of the oral cavity. Limitations in opening the mouth due to the size of the oral cavity and the lack of muscle elasticity represent the greatest difficulties regarding dental treatment, as reported in earlier studies.18,23 This case report presents the dental management of an anxious female with MbS, using the atraumatic restorative technique (ART), placing glass ionomer cement (GIC) with hand instruments.
and Children’s Hospital for continuation of dental management. At eight years of age, she had dental treatment under general anaesthesia. An increase in palatal smooth surface caries was noted between 1996 and 1998, ‘from her habit of holding coke in the mouth before swallowing’. Fluoride trays were made but not used due to gagging problems. Two per cent neutral sodium fluoride gel was preferred over mouthwash as she could not swish due to facial paralysis. Between 2000 and 2001, she had dental restorations, dietary advice and 2% neutral sodium fluoride was continued, after which she did not seek any dental treatment for six years. In 2007, she was referred back to the SNU from a community dental clinic, with ‘a severely broken down dentition’. Presenting problem The patient lives independently, but is on a disability support pension. She was seeking psychiatric counselling but was not on any medication. She has a blank expression, which is characteristic of a patient with MbS. As indicated in the referral, she presented with multiple carious teeth: Quadrant 1: 13 (incisal), 12 (mesial), 11 (mesiopalatal). Quadrant 2: 21 (mesial), 22 (disto-palatal), 23 (disto-palatal), 24 (occluso-buccal), 25 (occlusal), 26 (occlusal), decayed roots of 27 and 28. Quadrant 3: 32 (lingual), 33 (lingual), 34 (occlusobuccal), 35 (occlusal), 36 (occlusal), 37 (occlusal). Quadrant 4: 46 (mesio-occlusal), hypoplastic 44 (disto-occlusal). An orthopantomograph (OPG) shows the multiple carious lesions and decayed roots (Fig. 1). The main challenge was to perform routine dental procedures like restorations and extractions but in a very small inelastic mouth with a maximum width of 38 mm and opening of 18 mm (Fig. 2 and Fig. 3), making all intraoral procedures challenging due to extremely difficult access and restricted visibility. In addition, the patient had poor masticatory and swallowing capabilities and severe gag reflex.
CASE DESCRIPTION AND RESULTS A 19-year-old female with MbS was referred to the Special Needs Unit (SNU) in 1999 from the Women’s 256
Fig. 1 OPG showing multiple carious lesions and decayed roots. © 2015 Australian Dental Association
Atraumatic restorative technique
Fig. 2 Maximum width of 38 mm.
for 15–20 seconds to improve the chemical and mechanical bonding of the GIC to dentine. The cavity is then washed and dried but not dehydrated. Isolation is achieved with cotton rolls instead of rubber dam. Due to the inadequate intraoral space in this case, a ball burnisher was used to condense the glass ionomer cement instead of the ART ‘press-finger technique’.25 Likewise, other clinical procedures can be varied according to the equipment available and patient compliance. For example, a local anaesthetic can be used, a rubber dam can be placed, and a rotary instrument can be used to gain minimal access to the body of the lesion. The washing and drying can be done using a triple-syringe or using a wet and dry cotton pellet, thereby avoiding the use of a triplex and suction in extremely anxious patients. Reinforcement of preventive measures included instructions on daily oral hygiene care, dietary counselling and continuation of 2% neutral sodium fluoride gel. Follow-up
Fig. 3 Maximum opening of 18 mm.
Treatment After obtaining consent, treatment included extractions of the upper left second molar (27) and upper left third molar (28) for relief of pain, which were simple and uneventful. The decayed teeth were then restored over several visits. Restorations were completed using ART as there was inadequate intraoral space for the conventional use of standard size handpieces. ART refers to a minimal intervention technique for caries, originally developed in response to a need to provide effective preventive and restorative treatment in underserved communities where running water and electricity might not always be available. It involves minimum cavity preparation using only hand instruments (small and large spoon excavators for caries removal) to remove infected dentine, followed by restoration of the cavity, with an adhesive filling material (GIC).24,25 Local anaesthetic is not necessary as only soft, completely demineralized carious tooth tissue is removed and remaining sound tissue is retained. Care must be taken in deep cavities to avoid pulp exposure. The resultant cavity is then washed. The resultant smear layer from the use of hand instruments on the dentine surface is removed by applying 10% polyacrylic acid dentine conditioner to the cavity © 2015 Australian Dental Association
As is routine at SNU, the patient was placed on a sixmonthly recall list. However, in spite of reminder calls prior to every appointment and a new appointment arranged following every appointment that was unattended, she failed recall visits in 2009. Reasons given over the phone included ‘unwell’ or ‘not ready for the dental visit’. In 2010, she returned with pain on 36 and 37, which had to be surgically extracted. Since then she has maintained good oral hygiene and dental health (Fig. 4). DISCUSSION In contrast to past literature about the association with intellectual disability or autism,6,7 this patient did not present with either of these problems, conforming with the more recent Dutch study.9 Orally, the patient presented with full dentition, including all
Fig. 4 OPG showing maintenance of dental health. 257
A Pradhan and M Gryst third molars, but did have microstomia and gag reflex, which made all intraoral procedures extremely difficult. Restrictions in tongue and masticatory muscle functions were most likely to have added difficulties in performing oral hygiene procedures. Therefore, past caries may be partly attributed to insufficient oral hygiene, made worse by dry mouth and inappropriate diet. In addition, the patient had poor masticatory and swallowing capabilities, both of which are associated with this syndrome. As a result of these problems, her need for soft diet and the prolonged intraoral food retention could have increased the risk of caries. While other case reports have presented cases about children and young adults between the ages of 2 years and 18 years, with treatment under conscious sedation26 or general anaesthetic20 the patient in this case was a young adult who was treated in the chair, using an alternate technique like ART that avoided the need for a general anaesthetic. Although local anaesthetic can be avoided for restorations, it is necessary for the extraction of teeth. However, if ART is used in the first instance for the restoration of teeth, good rapport and trust can be developed over time with the dentist. At the least, ART can save teeth, whilst patients are on the waiting list for dental extractions under general anaesthetic and minimize the time which would otherwise have to be spent for the general anaesthetic. For patients with MbS, there are several risks associated when treated under a general anaesthetic, mainly difficult or even failed intubation.27 In addition, there is a risk for tracheomalacia, palatal and uvular weakness leading to loss of airway and respiratory failure. The presence of associated hypotonia of the skeletal musculature, and gastro-oesophageal reflux may increase the risk of perioperative aspiration. Associated orofacial abnormalities may make routine airway management difficult. Therefore, if a general anaesthetic was necessary, the anaesthetist would most likely consult the patient’s ear, nose and throat surgeon about the possibility of the need for a tracheostomy to ensure airway maintenance during the procedure. Prevention of oral health problems is always best, but when intervention is necessary, ART is appropriate for treating special needs patients with limited oral opening. To date, ART has been promoted by WHO for application in developing countries where there is no access to electricity for the use of handpieces. This case report highlights the application of ART in the treatment of patients not only with limited oral opening, but suggests that the application can be extended to other special needs patients (people with physical and intellectual disabilities, extreme anxiety, needle phobia and the frail elderly in aged care facilities), where access or cooperation is diffi258
cult or limited. ART rarely requires a local anaesthetic, it is well accepted by patients. Moreover, as handpieces are not used with ART, the threatening drilling sound is avoided and the necessary highvolume suction is not needed. It can be used both in the traditional dental clinic setting and for outreach situations like aged care facilities and remote areas where dental facilities do not exist. Given these advantages, and the effectiveness and survival rates of ART restorations,28,29 ART should be accepted by the dental profession and included in the dental education system for the dental management of people with special needs. ACKNOWLEDGEMENTS The authors would like to thank the South Australian Dental Service (SADS) for their approval to publish this case report and for the use of their facilities. The patient has given informed consent for the publication of this case report. REFERENCES 1. Kumar D. Moebius syndrome. J Med Genet 1990;27:122–126. 2. Rizoz M, Negr on RJ, Serman N. M€ obius syndrome with dental involvement: a case report and literature review. Cleft Palate Craniofac J 1998;35:262–268. 3. Briegel W. Neuropsychiatric findings of M€ obius sequence: a review. Clin Genet 2006;70:91–97. 4. Rogers GL, Hatch GF Jr, Gray I. Mobius syndrome and limb abnormalities. J Pediatr Ophthalmol 1977;14:134–138. 5. Lin KJ, Wang WN. Moebius syndrome: report of case. J Dent Child 1997;64:64–67. 6. Bandim JM, Ventura LO, Miller MT, Almeida HC, Costa AES. Autism and M€ obius sequence. Arq Neuropsiquiatr 2003;61:181–185. 7. Gillberg C, Steffenburg S. Autistic behaviour in Moebius syndrome. Acta Paediatr Scand 1989;78:314–316. 8. Koroluk LD, Lanigan DT. Moebius and Poland syndromes: a report of a case. Can Dent Assoc J 1989;55:647–648. 9. Verzijl HTFM, van Es N, Berger HJC, et al. Cognitive evaluation in adult patients with M€ obius syndrome. J Neurol 2005;252:202–207. 10. Verzijl HTFM, van der Zwaag B, Cruysberg JRM, et al. M€ obius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology 2003;61:327–333. 11. Str€ omland K, Sj€ ogreen L, Miller M, et al. M€ obius sequence – a Swedish multidiscipline study. Eur J Paediatr Neurol 2002;6:35–45. 12. Ziter FA, Wiser WC, Robinson A. Three-generation pedigree of a Mobius syndrome variant with chromosome translocation. Arch Neurol 1977;34:437–442. 13. Kankirawatana P, Tennison MB, D’Cruz O, Greenwood RS. Mobius syndrome in infant exposed to cocaine in utero. Pediatr Neuro 1993;19:71–72. 14. Shepard TH. Mobius syndrome after misoprostol: a possible teratogenic mechanism. Lancet 1995;346:780. 15. Dal Pizzol TS, Knop FP, Mengue SS. Prenatal exposure to misoprostol and congenital anomalies: systematic review and meta-analysis. Reprod Toxicol 2006;22:666–671. © 2015 Australian Dental Association
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Address for correspondence: Dr Archana Pradhan Special Needs Unit Adelaide Dental Hospital Adelaide SA 5000 Email: [email protected]
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