Case Report - PDF document

Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: 30 December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Duane syndrome is a rare retraction anomaly characterized by congenital non - progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered a bout oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock - wise mandibul ar rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero - anterior x - ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. © 2019 Edoardo Sicurezza. Hosting by Science Repository. All rights reserved Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized by difficulty abducting and adducting one or both eyes. DRS is due to miswiring of the eye muscles and in patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was to describe the oral signs of a patient affected by DRS. Case Report Our patient was a little male child. He was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected by oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. At the age of 6 years old he underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. On examination, his height was found to be 110 cm, with a weight of 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the Korkhaus’ index was performed on patient’s dental casts showing a considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com © 2019 Edoardo Sicurezza. This is an open - access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and re production in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository 2 Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report morphology of the mandibular condyles and multiple decay lesions. Postero - anterior x - ray showed an asymmetrical craniofacial structure. Figure 1: Frontal extra - oral view. Figure 2: Lateral extraoral view. Figure 3: Cephalometric analysis. Table 1: SNA, SNA angle; SNB, SNB angle; ANB, ANB angle; FMIA, Frankfort – Mandibular Incisor Angle; FA, Facial Axis angle; LFH, Lower Facial Height; FD, Facial Depth angle; II, Interincisive angle. Discussion Duane’s Retraction Syndrome (DRS), also named Stilling - Turk - Duane syndrome, is a congenital oculomoto r disease caused by the hypoplasia or the absence of the abducens nucleus. DRS is a rare syndrome (represent approximately 1% of total cases of strabismus). Previous studies associated this syndrome with ocular (nystagmus, anisocoria, ptosis, optic nerve c olobomas, etc.), cardiac (auricular septal defect), skeletal (scoliosis, spina bifida, limb hypoplasia, etc.), genitourinary (renal agenesis and vesicoureteral reflux) anomalies [3, 4]. DRS has also been associated with a number of specific congenital synd romes including Klippel - Feil syndrome, Wildervanck syndrome (Duane syndrome, Klippel - Feil anomaly, and deafness), Holt - Oram, morning - glory syndrome, Goldenhar syndrome, Okihiro syndrome (Duane syndrome and radial ray defects), and Usher Syndrome Type 2. N o oral manifestation related to DRS have been described earlier in literature. Our patient presented stomatognathic defects as micrognatia, teeth agenesis, maxillary and mandibular prognathism (probably due to the hypoplasia of frontal sinus that retruded the nasion and mycrocephaly) and short tongue frenulum. The Korkaus’ Index was performed to verify micrognatia and, despite the teeth defects, reported a great reduction of premaxilla diameter. Dental panoramic x - ray showed an abnormal growth of mandible and particularly of mandibular branch with also an alteration of TMJ heads (Figure 4 & Figure 5). Figure 4: Right mandibular branch. Figure 5: Left mandibular branch. Case Reports in Dental & Oral Biology doi:10.31487/j.CRDOB.2019.01.02 Volume 1(1): 2 - 3 VARIABLE NORMAL REPORTED DIFFERENCE SNA 82  2 86.1 4.1 SNB 80  2 87.1 7.1 ANB 2  2 - 1 - 3 FMIA 67  3 91 24 FA 90  3 96.6 6.6 LFH 47  4 39.8 - 7.2 FD 89  3 102.7 13.7 II 130  8 158.3 28.3 3 Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report Conclusion DRS is a genetic condition present from birth, and usually recognized in early childhood. It shows usually audiologic, oto logic and oculars signs and symptoms. Our observation of oral manifestations in a boy with DRS may prompt a prospective and retrospective review of other patients affected by this syndrome in order to establish if these anomalies are part of it. R EFERENCES 1. Yalcin SS, Cagdas D, Sener EC, Yurdakok K (2003) Right - sided Duane retraction syndrome associated with multiple malformations. Pediatr Int 45: 577 - 579. [ Crossreff ] 2. Varma C, Aroor S , Mundkur SC, Annamalai K (2012) Bilateral type - I duane syndrome with multiple anamolies: a case report. J Clin Diagn Res 6: 1435 - 1436. [ Crossreff ] 3. Marshman WE, Schalit G, Jones RB, Lee JP, Matthews TD et al. (2000) Congenital anomalies in patients with Duane retraction syndrome and their relatives. J AAPOS 4: 106 - 109. [ Crossreff ] 4. Ruttum M, Alcorn D (2000) 10 - year - ol d boy with Duane syndrome OS. J Pediatr Ophthalmol Strabismus 37: 107 - 110. [ Crossreff ] 5. Calabrese G, Telvi L, Capodiferro F, Morizio E, Pizzuti A et al. (2000) Narrowing the Duane syndrome critical region at chromosome 8q13 down to 40 kb. Eur J Hum Genet 8: 319 - 324. [ Crossreff ] 6. Louly F, Nouer PR, Janson G, Pinzan A (2011) Dental arch dimension s in the mixed dentition: a study of Brazilian children from 9 to 12 years of age. J Appl Oral Sci 19: 169 - 174. [ Crossreff ] 7. Defraia E, Baroni G, Marinelli A (2006) Dental arch dimensions in the mixed dentition: a study of Italian children born in the 1950s and the 1990s. Angle Orthod 76: 446 - 451. [ Crossreff ] 8. Lindsten R (2003) Secular changes in tooth size and dental arch dimensions in the mixed dentition. Swed Dent J Suppl 1 - 89. [ Crossreff ] 9. El Hayeck E, Bassil Nassif N, Mouhanna Fattal C, Bouserhal J (2017) Skeletal and dento - alveolar norms: Conventional and based on true vertical in a Lebanese population. Int Orthod 15: 180 - 198. [ Crossreff ] 10. Tehranchi A, Motamedian SR, Saedi S, Kabiri S, Shidfar S (2017) Correlation between frontal sinus dimensions and cephalometric indices: A cross - sectional study. Eur J Dent 11: 64 - 70. [ Crossref f ] Case Reports in Dental & Oral Biology doi:10.31487/j.CRDOB.2019.01.02 Volume 1(1): 3 - 3 Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Duane syndrome is a rare retraction anomaly characterized by congenital non - progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered a bout oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock - wise mandibul ar rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero - anterior x - ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com © 2019 Edoardo Sicurezza. This is an open - access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and re production in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Duane syndrome is a rare retraction anomaly characterized by congenital non - progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered a bout oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock - wise mandibul ar rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero - anterior x - ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com © 2019 Edoardo Sicurezza. This is an open - access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and re production in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com © 2019 Edoardo Sicurezza. This is an open - access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and re production in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6

years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com © 2019 Edoardo Sicurezza. This is an open - access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and re production in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. © 2019 Edoardo Sicurezza. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. © 2019 Edoardo Sicurezza. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class II

I with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. © 2019 Edoardo Sicurezza. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. Case Report Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report E. Sicurezza 1* , V. Nicotra 2 , T. Mattina 2 and G. Palazzo 1 1 Department of Orthodontics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy 2 Department of Genetics, Policlinico, University of Catania, via Santa Sofia 78, Catania, Italy A R T I C L E I N F O Article history: Received: 25 November, 2019 Accepted: 17 December, 2019 Published: December 2019 Keywords: Duane syndrome oculomotor anomaly dental agenesis asymmetrical craniofacial structure A B S T R A C T Introduction The Duane Retraction Syndrome (DRS) is a congenital disorder typically characterized difficulty abducting and adducting one both eyes. DRS due miswiring the eye muscles and patients affec ted by this syndrome, the sixth cranial nerve (that controls the lateral rectus muscle) does not develop properly. This condition was first described by Sinclair, Turk and Stilling in 1895 and in literature it is reported a frequency of 1 - 5% in general population with strabismus [1]. Previous study associated DRS with multiples anomalies such as crocodile tears, mental retardation and spina bifida occul ta [2 - 4]. No data has been yet registered about oral manifestation of Duane syndrome. The aim of this case report was describe the oral signs a patient affected DRS. Case Report Our patient was a little male child. was 6 years old, born out o f a non - consanguineous marriage with an euthocic birth after a pregnancy affected oligoamnio and old placenta. During the perinatal period the patients reported cardio - respiratory depression, cyanosis, transitory itterus. the age 6 years old underwent the genetic evaluation that putted in light auxological parameters 10°pc, facial dimorphism, ipoacusia and no cardiovascular affectio ns. SNP - Array underlined two microdeletions: one located in the long arm of chromosome 2 of 2q22.3 region and one located in the long arm of chromosome 17 of 17q23.1q23.2 region. These two chromosomal defects confirmed the genetic diagnosis of Duan Syndrome [5]. examination, his height was found 110 cm, with a weight 18 kg. Extra - oral exami nation revealed facial asymmetry with reduced intercanthal distance and facial dimorphism (Figure 1 & Figure 2). Intraoral examination revealed bald and fissured tongue with short lingual frenulum and hypotrophic gingiva. The patient presented microdontia with oligodontia; teeth presented severe enamel defects as amelogenesis imperfecta that conduced to destructive decay lesions of deciduous teeth. In order to evaluate dental arch dimension, the .RUNKDXV¶LQGH[ZDVSHUIRUPHGRQSDWLHQW¶VGHQWDOFDVWVVKRZLQJD considerable reduction of pre - maxilla diameter [6 - 8]. The patient underwent to X - rays ortopantomography and teleradiography exams in order to assess cephalometric evaluation. Cephalometric analysis was conducted according the Ricketts and Tweed norms (Table 1 & Figure 3) [9, 10]. His skeletal pattern was skeletal Class III with both maxillary and mandibular protrusion (probably due to the point Nasion retrusion), counterclockwise mandibular rotation, lingualized maxillary and mandibular incisors. Dental x - ray showed first mandibular molar agenesis, asymmetric * Correspondence to: Edoardo Sicurezza, Department of Orthodontics, University of Catania, via Santa Sofia 78, Policlinico, Cat ania 95123, Italy; E - mail: edoardo.sicurezza@gmail.com C ASE R EPORTS IN D ENTAL & O RAL B IOLOGY Available online at www.sciencerepository.org Science Repository © 2019 Edoardo Sicurezza. Hosting by Science Repository. Duane syndrome is a rare retraction anomaly characterized by congenital non-progressive horizontal ophthalmoplegia and other systemic signs. No data has been yet registered about oral manifestation of Duane syndrome. In this article we present a six years old male patient was diagnosed as having Duane retraction syndrome. He presented skeletal Class III with both maxillary and mandibular protrusion, counter clock-wise mandibular rotation, lingualized maxillary and mandibular incisors, first mandibular molar agenesis, asymmetric morphology of the mandibular condyles and multiple decay lesions. Postero- anterior x-ray showed an asymmetrical craniofacial structure. The aim of this case report was to describe the oral signs of a patient affected by DRS. © 2019 Edoardo Sicurezza. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository. http://dx.doi.org/10.31487/j.CRDOB.2019.01.02 2 Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report morphology of the mandibular condyles and multiple decay lesions. Postero - anterior x - ray showed an asymmetrical craniofacial structure. Figure 1: Frontal extra - oral view. Figure 2: Lateral extraoral view. Figure 3: Cephalometric analysis. Table SNA, SNA angle; SNB, SNB angle; ANB, angle; FMIA, Frankfort ± Mandibular Incisor Angle; FA, Facial Axis angle; LFH, Lower Facial Height; FD, Facial Depth angle; II, Interincisive angle. Discussion 'XDQH¶V5HWUDFWLRQ6\QGURPH'56\fDOVRQDPHG6WLOOLQJ - Turk - Duane syndrome, is a congenital oculomoto r disease caused by the hypoplasia or the absence of the abducens nucleus. DRS is a rare syndrome (represent approximately 1% of total cases of strabismus). Previous studies associated this syndrome with ocular (nystagmus, anisocoria, ptosis, optic nerve c olobomas, etc.), cardiac (auricular septal defect), skeletal (scoliosis, spina bifida, limb hypoplasia, etc.), genitourinary (renal agenesis and vesicoureteral reflux) anomalies [3, 4]. DRS has also been associated with a number of specific congenital synd romes including Klippel - Feil syndrome, Wildervanck syndrome (Duane syndrome, Klippel - Feil anomaly, and deafness), Holt - Oram, morning - glory syndrome, Goldenhar syndrome, Okihiro syndrome (Duane syndrome and radial ray defects), and Usher Syndrome Type 2. N o oral manifestation related to DRS have been described earlier in literature. Our patient presented stomatognathic defects as micrognatia, teeth agenesis, maxillary and mandibular prognathism (probably due to the hypoplasia of frontal sinus that retruded the nasion and mycrocephaly) and short tongue frenulum. The .RUNDXV¶ Index was performed verify micrognatia and, despite the teeth defects, reported a great reduction of premaxilla diameter. Dental panoramic x - ray showed an abnormal growth of mandible and particularly of mandibular branch with also an alteration of TMJ heads (Figure 4 & Figure 5). Figure 4: Right mandibular branch. Figure 5: Left mandibular branch. VARIABLE NORMAL REPORTED DIFFERENCE SNA r 2 86.1 4.1 SNB r 2 87.1 7.1 2 r 2 - 1 - 3 FMIA r 3 r 3 96.6 6.6 LFH r 4 39.8 - 7.2 r 3 102.7 13.7 130 r 8 158.3 28.3 2 Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report morphology of the mandibular condyles and multiple decay lesions. Postero - anterior x - ray showed an asymmetrical craniofacial structure. Figure 1: Frontal extra - oral view. Figure 2: Lateral extraoral view. Figure 3: Cephalometric analysis. Table SNA, SNA angle; SNB, SNB angle; ANB, angle; FMIA, Frankfort ± Mandibular Incisor Angle; FA, Facial Axis angle; LFH, Lower Facial Height; FD, Facial Depth angle; II, Interincisive angle. Discussion 'XDQH¶V5HWUDFWLRQ6\QGURPH'56\fDOVRQDPHG6WLOOLQJ - Turk - Duane syndrome, is a congenital oculomoto r disease caused by the hypoplasia or the absence of the abducens nucleus. DRS is a rare syndrome (represent approximately 1% of total cases of strabismus). Previous studies associated this syndrome with ocular (nystagmus, anisocoria, ptosis, optic nerve c olobomas, etc.), cardiac (auricular septal defect), skeletal (scoliosis, spina bifida, limb hypoplasia, etc.), genitourinary (renal agenesis and vesicoureteral reflux) anomalies [3, 4]. DRS has also been associated with a number of specific congenital synd romes including Klippel - Feil syndrome, Wildervanck syndrome (Duane syndrome, Klippel - Feil anomaly, and deafness), Holt - Oram, morning - glory syndrome, Goldenhar syndrome, Okihiro syndrome (Duane syndrome and radial ray defects), and Usher Syndrome Type 2. N o oral manifestation related to DRS have been described earlier in literature. Our patient presented stomatognathic defects as micrognatia, teeth agenesis, maxillary and mandibular prognathism (probably due to the hypoplasia of frontal sinus that retruded the nasion and mycrocephaly) and short tongue frenulum. The .RUNDXV¶ Index was performed verify micrognatia and, despite the teeth defects, reported a great reduction of premaxilla diameter. Dental panoramic x - ray showed an abnormal growth of mandible and particularly of mandibular branch with also an alteration of TMJ heads (Figure 4 & Figure 5). Figure 4: Right mandibular branch. Figure 5: Left mandibular branch. VARIABLE NORMAL REPORTED DIFFERENCE SNA r 2 86.1 4.1 SNB r 2 87.1 7.1 2 r 2 - 1 - 3 FMIA r 3 r 3 96.6 6.6 LFH r 4 39.8 - 7.2 r 3 102.7 13.7 130 r 8 158.3 28.3 Case Rep Dent Oral Biol doi:10.31487/j.CRDOB.2019.01.02 Volume 1(1): 2-3 3 Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report Conclusion DRS a genetic condition present from birth, and usually recognized in early childhood. It shows usually audiologic, oto logic and oculars signs and symptoms. Our observation oral manifestations a boy with DRS may prompt a prospective and retrospective review of other patients affected this syndrome order establish these anomalies are part R EFERENCES Yalcin SS, Cagdas D, Sener EC, Yurdakok K (2003) Right - sided Duane retraction syndrome associated with multiple malformations. Pediatr Int 45: 577 - 579. [ Crossreff ] Varma C, Aroor S , Mundkur SC, Annamalai K (2012) Bilateral type - duane syndrome with multiple anamolies: a case report. J Clin Diagn Res 6: 1435 - 1436. [ Crossreff ] Marshman WE, Schalit Jones Lee JP, Matthews al. (2000) Congenital anomalies in patients with Duane retraction syndrome and their relatives. J AAPOS 4: 106 - 109. [ Crossreff ] Ruttum Alcorn D (2000) - year - d boy with Duane syndrome OS. J Pediatr Ophthalmol Strabismus 37: 107 - 110. [ Crossreff ] Calabrese Telvi Capodiferro Morizio Pizzuti A al. (2000) Narrowing the Duane syndrome critical region at chromosome 8q13 down to 40 kb. Eur J Hum Genet 8: 319 - 324. [ Crossreff ] Louly Nouer PR, Janson Pinzan A (2011) Dental arch dimension in the mixed dentition: a study of Brazilian children from 9 to 12 years of age. J Appl Oral Sci 19: 169 - 174. [ Crossreff ] Defraia Baroni Marinelli A (2006) Dental arch dimensions the mixed dentition: a study of Italian children born in the 1950s and the 1990s. Angle Orthod 76: 446 - 451. [ Crossreff ] Lindsten R (2003) Secular changes in tooth size and dental arch dimensions the mixed dentition. Swed Dent J Suppl 1 - 89. [ Crossreff ] El Hayeck E, Bassil Nassif N, Mouhanna Fattal C, Bouserhal J (2017) Skeletal and dento - alveolar norms: Conventional and based on true vertical in a Lebanese population. Int Orthod 15: 180 - 198. [ Crossreff ] 10. Tehranchi A, Motamedian SR, Saedi S, Kabiri S, Shidfar S (2017) Correlation between frontal sinus dimensions and cephalometric indices: A cross - sectional study. Eur J Dent 11: 64 - 70. [ Crossref f ] 3 Oral Manifestation in a Patient with Bilateral Duane Syndrome: A Case Report Conclusion DRS a genetic condition present from birth, and usually recognized in early childhood. It shows usually audiologic, oto logic and oculars signs and symptoms. Our observation oral manifestations a boy with DRS may prompt a prospective and retrospective review of other patients affected this syndrome order establish these anomalies are part R EFERENCES Yalcin SS, Cagdas D, Sener EC, Yurdakok K (2003) Right - sided Duane retraction syndrome associated with multiple malformations. Pediatr Int 45: 577 - 579. [ Crossreff ] Varma C, Aroor S , Mundkur SC, Annamalai K (2012) Bilateral type - duane syndrome with multiple anamolies: a case report. J Clin Diagn Res 6: 1435 - 1436. [ Crossreff ] Marshman WE, Schalit Jones Lee JP, Matthews al. (2000) Congenital anomalies in patients with Duane retraction syndrome and their relatives. J AAPOS 4: 106 - 109. [ Crossreff ] Ruttum Alcorn D (2000) - year - d boy with Duane syndrome OS. J Pediatr Ophthalmol Strabismus 37: 107 - 110. [ Crossreff ] Calabrese Telvi Capodiferro Morizio Pizzuti A al. (2000) Narrowing the Duane syndrome critical region at chromosome 8q13 down to 40 kb. Eur J Hum Genet 8: 319 - 324. [ Crossreff ] Louly Nouer PR, Janson Pinzan A (2011) Dental arch dimension in the mixed dentition: a study of Brazilian children from 9 to 12 years of age. J Appl Oral Sci 19: 169 - 174. [ Crossreff ] Defraia Baroni Marinelli A (2006) Dental arch dimensions the mixed dentition: a study of Italian children born in the 1950s and the 1990s. Angle Orthod 76: 446 - 451. [ Crossreff ] Lindsten R (2003) Secular changes in tooth size and dental arch dimensions the mixed dentition. Swed Dent J Suppl 1 - 89. [ Crossreff ] El Hayeck E, Bassil Nassif N, Mouhanna Fattal C, Bouserhal J (2017) Skeletal and dento - alveolar norms: Conventional and based on true vertical in a Lebanese population. Int Orthod 15: 180 - 198. [ Crossreff ] 10. Tehranchi A, Motamedian SR, Saedi S, Kabiri S, Shidfar S (2017) Correlation between frontal sinus dimensions and cephalometric indices: A cross - sectional study. Eur J Dent 11: 64 - 70. [ Crossref f ] Case Rep Dent Oral Biol doi:10.31487/j.CRDOB.2019.01.02 Volume 1(1): 3-